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Blueprints - Pediatrics

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tingtingcrazy
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© © All Rights Reserved
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2K vistas315 páginas

Blueprints - Pediatrics

Cargado por

tingtingcrazy
Derechos de autor
© © All Rights Reserved
Nos tomamos en serio los derechos de los contenidos. Si sospechas que se trata de tu contenido, reclámalo aquí.
Formatos disponibles
Descarga como PDF, TXT o lee en línea desde Scribd

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cu!ccv [IInu !ngcv :vcc I: convuInuIcucu
Iccuu:c u ovcIgn Iouy nuy Ic ovccu uvt!cv
uovn !c ovo!uvyn)
IcnIng c uIvvuy vIu !c juv-!vu: ov c!In-!It
nuncuvcv und vcIIcvIng uny oI:vucIon cuu:cu Iy
!c onguc ov :ot ::uc: o !c ncck
IIucIng c !cuu In !c nIuIInc ":nI!ng o:Ion,"
otcn vIu u vo!Icu-u ovcI Icncu! !c occIu
[nycvccn:Ion o !c ncck nuy vc:u! In oI::uc-
on o !c uIvvuy)
IvovI:Ion o I00% oygcn vIu ucc nu:k
I Inucucu, !uccncn o un ovuI ov u nu:o!u-
vyngcuI uIvvuy
Incc un uIvvuy I: c:uIII:!cu, uIv cc!ungc
[breathing} :!ouIu Ic cvuIuucu. IunInuIon o
c!c: vuII ccuv:Ion vcvcu!: !c vc:cncc unu c!lcc-
vcnc:: o :onuncou: vc:Ivuon:. I :onuncou:
vc:Ivuon : vc:cn vI! uucquuc oygcnuIon,
InuIuIon I: no InuIcucu. | c!c: vuII ecuv:Ion I:
no uucquuc, cnuovuc!cuI uIc !uccncn I: InuI-
cucu [I no u!vcuuy In Iucc o :ccuvc !c uIvvuy).
I c cIIu I: youngcv !un 8 ycuv:, un uncu!lcu uc
:!ouIu Ic u:cu o vcuucc !c vI:k o :uIgIoIc cucnu
unu :cno:I:. [ In c!IIuvcn youngcv !un 8 ycuv:, !c
cvIcoId rIng I: !c nurvovc: uv o !c uIvvuy unu
rovIuc: !c :cuI ov !c un cucu uIc.) Jhe size oI
he endotracheaI tube chosen shouId equaI 4 + [age
in years 4}. BIoou oygcnuIon [vIu uI:c oIncvy
ov uvcvIu! IIoou gu: ncu:uvcncn) unu IIoou CI,
IcvcI [y uvcvIuI ov vcnou: IIoou gu: ncu:uvcncn)
:!ouIu Ic u::c::cu unu vI!I guIuc :c:Ivuovy !cvuy.
conuuI InuIuIon I: :uuIIonuIIy cvovncu
vI!ou vcncuIcuon, Iu InuIuon o !c Inun
2 |
<

not, o

i } < nc rn noa i >


< cx v cxann-ronnccun: nvcm o- on-. JV >
* ucrrIO Cat Cs
1PLL 11
1he Oefent OgnOSS Of Lh0fen Wth
Lf0OumOnf PffeSt
KeSQrt0r
ppCr alrwayCbstruCtlCn
LCwCralrwayCbstruCtlCn
HCstrlCtlvC|ungOlsCasC
InsuIhClCntgastransICr
Lr0c
LCngCnlta|hCartOlsCasC
|rlmaryOysrhy\hmla
MyCCarOltls
|CrlCarOltls
LarOlaCtampCnaOC
LCngCstlvChCartIalIurC
Letr NerV0uS bStem
MCnlngltls
LnCCphaIltls
PCutChyOrCCCphaIus
HCaOtrauma
5ClZurC
1umCr
HypCxlC-lsChCmlClnjury
OStr0teSt
PbOCmlnaItrauma
bCwCIpCrICratlCnCr
CbstruCtlCn
|CrltCnltls
LChyOratlCn
NetD0c
LlabCtlCkCtCaClOCsls
POOlsCn`sOlsCasC
HypCrthyrClOlsm
HypCgIyCCmla
HypCrkaICmla
HypCCaICCmla
HypCnatrCmla
NutSStem
5uOOCnlnIantOCath
synOrCmC
Lrug lntCxlCatlCn*
MuItlpIC trauma
PnaphyIaxls
HypCthCrmla
5CptlCshCCk
Ke
PCutC anO ChrCnlCrCnaI
IalIurC
NarcoIcs, Ir|cyc|c anIdepressanIs, barbIuraIes, benzod|azep|nes.
ov c!IIu I: uonc vI! vcncuIcuon In !c oIIovIng
vuIu-:cqucncc u:!Ion.
I. Ivcoygcnuon vI! !00% oygcn
2. unInI:vuon o u vugoIyIc uvug [c.g., u:oInc).
J. unInI:t:uIon o u :cuutvc, !ynoc, unu/ov
oIoIu uvug [c.g., !IocnuI, vcv:cu, cnunyI).
4. IIcuIon o cvIcoIu vc::uvc.
S. unInI:vuIon o u uvuIyzIng uo:c o u ncuvo-
nu:cuIur IIockng ugcn [c.g., uncuvonIun ov
vccuvonIun, u nonucoIuvIzIng ugcn, ov :uccInyI-
c!oIInc, u ucoIuvIzIng ugcn). I :uccInyIc!oIInc
I: u:cu, u ucu:cIcu!uIng uo:c o u ncuvonu:-
cu!uv IIockng ugcn :!ouIu Ic gIvcn Icovc
uunInI::uIon o :uccInyIc!oIInc [c.g., uncuvo-
nIun ov vccuvonIun).
In !c !yocn:Ivc, !cnouynunIcuIIy un:uIIc, ov
uncon:cIou: uIcn, rcncuIcuIon I: no InuIcucu.
Frmary BurvCy
l |vy
U|OI|llQ
Ll|CUlIlOl
Ul5OlllIy
LjO5U|O
|lO-I||OIOll|Q COlOlIlOl:
YO5
.

HCBuBclallon UCcondary BurvCy


l| |vy |lOUVO|5 LO|jlOIO O|||IlOl
Ujjl O|OlIl L_lO ll |OOIOIOO5|lOl
VOlI|lIO|y 5UjjO|L l5IO|yO l|llO55O|
VOlOU5CCO55 lO I|U|OVOlI
5|OCb |lQO|OlI |5I |OOlCl |l5IO|y
|OllIO|llQ VlI| 5Ql5, LOO|IO|y |O5UlI5lG
LLL, jUl5O Ol |OI|y,
|O|OQ|j|lC IO5I5
U|l ||y CI|OIO| O|OQ
lO-OVlUIlOllO
L|OQ5I|lC IUOO 5IOllZIlOl
||OOlO| ll5I
W
l
DCflnlllvC carC
IlgurCT-T PIgCrlthmCI thClnltlaIassCssmentCIthepCOlatrlC
patlent.
NoC!eC rCm Nchols LL, YasICr N, LaC LL, eI a|. Lo|deO Hour. 1e
HanCLook o ACvaned PeCa\rC L|e 5uCrt. 5I. LCU|s: NosDy YCarbook,
''`V,V.
CvIcou vc::uvc :!ouIu Ic uIIcu, unu !c ucn
:!ouIu Ic InuIucu. RuvcIy, u uIcn cunno Ic
InuIucu ov vcnIucu vI! u Iug unu nu:k, unu un
cncvgcncy nccuc cvIconyvoony I: vcquIvcu o
c:uIII:! un uIvvuy.
CircuIation nuy Ic u::c::cu Iy cvuIuung uI:c:
[ccn:uI unu cvI!cvuI), cuIIIuvy vc!II, unu IIoou
vc::uvc. J!c uI:cncc o u uI:c In !c Iuvgc uvtcvIc:
o un uncon:cIou: ucn v!o I: no Ivcu!Ing
uc!nc: u cuvuIuc uvvc:. In c!IIuvcn, !cuvt vuc : !c
no: :cn:IIvc ncu:uvc o n:uvu:cuIuv voIunc
:uu:. CuI!!uvy vc!!I I: !c no: :cn:IIvc ncu:uvc
o uucquuc cIvcuIuon. BIoou vc::uvc !.ucuuIon:
< nor foz saJeI > < p)

I >
< cra u Deaazxotaejcn: MOAsr r jy :
atC mCrQCnyNanaQCmCnt:LvaIUattCnCIthCC taI|y III OrInjUrCO ChlIO 3
Infanl Older ClId
Alrway
LOIO|||lO Ul|O5jOl5lVOlO55
Lll O| |O| j
|O5lIlOl jIlOlI 5UjllO
UjjO|L |OOlO lOCb
mOO IllIlC|ll llL O| jv I||U5I
lO OlllO llQO| 5vOCj5
BrCallng
L lllIll O|OI|5
1|Ol. Z O|OI|5}|ll 1|O|. 1c O|OI|5}|ll
ClrcuIallon
LIOC| O|C|tl jU|5O L|OC|C|O|tO jU|5O
lCl|VIO L| y5O|
LO|j|O55lO| |OCIlOl' LO|j|O55iOl lOCLlOl.
J !llQO| O|OOI| OOlOv lOvO| J}H Ol 5IO|lU
llIO|||||y lllO Ol
5LO|lU
LOj|O55lOl|OI|OO.
llO5 OlCl|ClO C|O5I O|
Z llQO|5 Ol 5IO|lU|
LO|j|O55lOl OOjI|.
.c-1
LO|j|O55tO| |lO. 1l|l|
LO|j|O55lOlOI|OO.
J O| L |lO5 O| 5IO|lU|
LO|j|O55lOl OOjI|.
11
LO|j|O55|O| |IO.
J}|l |
LO|j|O55lOl.VOlLl lIlOl |IlO c.J
lO55O55|OlI. |ljIO jUl5O OVO|y 1 CyClO5
IlgurO 1-2* bSC L|H G GG1S GOC|O|CG.
NCUJ!CU rCm N|cC|s LL, YastCr N, LaC LL, Ct a|. LC|UCn HCUr: C
|aOCDCCk C ACvaOCCU |CU|atr|C L||C 5uCrt. 5t. LCU|s: NCsDy YCarDCC|,
''.V,V.
uc un In:cn:ItIvc nucuto, Iccuu:c !yotcn:Ion I: u
Iuc hnuIng In !yovoIcnIu. C!IIucn uc noc IIkcIy
to c:cnt vIt! u:y:to!c t!un vIt! un u!yt!nIu,
unIc:: t!cy !uvc un unucIyIng cuuIuc cIcctIcuI
uInonu!Ity. CuuIoc:Iutoy nonIto: uc !cIuI
o :ccI]Ing t!c cIcctIcuI uctIvIty o t!c !cut.
l uI:c!c::nc:: I: notcu on cxunInutIon o i!c
Iuc!IuI uI:c In t!c Inunt o t!c cuotIu uI:c In t!c
c!IIu, c!c:t conc::Ion: :!ouIu Ic :tutcu. Vu:cuIu
uccc:: nunugcncnt uuIng cuuIouInonuy c:u:cI-
tutIon I: ou\!Incu In !Iguc I-3. Incc uccc:: !u: Iccn
c:uII:!cu, InItIuI h uIu c:u:cIuIon vIt! Iucucu
Rngc': :oIuton o nonuI :uIIne :!ouIu Ic gIvcn u:
u 2J nIkg IoIu: u: quIc!y u: o::IIIc. l nccc::uy,
t!c:c IoIu:c: :!ouIu Ic ccutcu. Hovcvc, I t!cc
I: no c:on:c o t!c utIcnt !u: :uccu ucutc IIoou
Io::, con:Iuc u Un1g InuIon o uIIunIn, cy:-
tu!IoIu, o tyc I-ncgutIvc v!oIc IIoou. I !yo-
tcn:Ion uuc to !cno!ugc I: :u:cctcu, guInIng
oInuI conto! o t!c !cno!ugc I: cItcuI.
ItInuIIy, u uII :ct o :cccnIng tc:t: [Inc!uuIng
FCrculanCous pCrlpCraI IV
YCs No [IOU5OC)
l
l |I|O55OOU5 lOOOlO
[J5IC|OlCOOl* cy.O.)
O|
j|OlOU5 VO|l CUIOOvl
O|
LO|I|' VOlOU5CCO55
1
LCIIOO lllQO|5 1 Z |l}bQ lO
IlI|IO U|I|OI VOlU|OO|lll 5I|IlOl
lO5U5ClIIlOlO|UQ5
IlgurC 1-3 VSCU| CCCSS HGQCDCG1 OU|GQ C|OOjU-
HOG|y |CSUSC11OG.
con!ctc IIoou count, vcnou: IIoou gu:, cIcct:oIytc
unu c!cnI:t:y uncI, unu IIoou g!uco:c) I: oItuIncu
ut t!c tInc o vu:cuIu uccc::. I Ingc:tIon P u o::I-
lIIIty, :cun unu uInc toIcoIog, unu un ucctu-
nIno!cn unu :uIIcyIutc IcvcI nuy Ic oItuncu.
|n t!c utcnt vIt! tuc!yuy:!y\!nIu: [5V, V)
t!cucutc uccI:Ion: uc Iu:cu on v!ct!c t!c
utcnt I: !cnouynunIcuIIy :tuIIc o un:tuIIc.
Sunuentt|cu/ot 7ocbycotdio [S!]
Hemodynomico//y sto//e. Vugu! nuncuvc:, uucno-
:Inc [1V ccocutIng tuc!ycuuIu), unIouuonc
[uutonutc tuc!ycuuu).
Hemodynomico//y unsto//e ot S\| te|roctot to
medicotions. 5ync!onIzcu cuuovc:on J.SJ to
!.JJ/kg, Inccu:cu to 2J/g I IntIuI cuuIovc:Ion
: un:uccc::u!.
\entrcu/ot 7ocbycotdio [v|)
Hemo[nomico//y su//e. lIuocutnc, unIouuonc, o
ocuInunIuc, unu t:cut !yonugnc:cnIu unu/o
!yokuIcnIu. 1nIouuonc unu ocuInunuc
:!ouIu not Ic u:cu togct!c Iccuu:c t!cy Iot!
oIong t!c J intcvuI unu Iot! nuy cuu:c
!yotcn:Ion.
Hemodynomico//y unsto//e ot \7 te]rocto h
medicotions. 5ync!onIzcu cuuIovc:Ion 0.SJ to
l.UJ/g; Inccu:cu to 2J/kg I InItIu! cuuIovc:Ion
I: un:uccc::uI.
4
< not foz! < ue pLn njopa ! >
< cx v pexaak~xouaejmn: HCA sT QJouT. jy >
||ucr|rI I'Cat Cs
IuI:cIc:: VJ ov V!. on:ync!vonIzcu uc!IvIIIu-
Ion [21/g, oIIovcu ly 41/g I un:uccc::uI,
oI!ovcu ly 4 1/g I un:uccc::uI) I: InuIcucu.
FInc!vInc I: uunInI:cvcu I vc:u:cIuon :
un:uccc::uI u!cv t!vcc cIcctvIcuI :!ocJ:, oIIovcu
Iy :!ock uguIn, 4J/g. Ivcccuc :uI:cqucn uc!I-
vIIIuon ucn: vI! In:uvcnou: IIuocuInc,
unIouuvonc, ov cInc!vInc.
!ov u !uII uI:cu::Ion o uvug !y:IoIog,, InuIcu-
Ion:, uo:ugc, vouc o uunInI:vuon, ctcc:, unu
::uc c!lcc:, :cc Tbe Hor|et Ione Hond/oo/ ov
Co/den Hout. Tbe Hond/oo/ o| .duonced |ediotr|c
Ii|e Su_ott. JuI!c !-2 uc:cvIIc: !c InuIcuIon: unu
c!lcc: o cuc! u:ug.
!ov disabiIity, u ruIu :cvccnIng ncuvoIogc cun-
1PLL 1Z
InuIon I: cvovncu o noc uIIIuvy vc:on:c, IcvcI
o con:cIou:nc::, unu IocuIIzIng !nung:.
Secondary Survey
J!c secondary survey IncIuuc: u !cuu-o-oc !y:I-
cuI cunInuon In ovucv o uccvnInc !c ccn o
njuvy unu uv!cv vIovIIzc :cuncn. J!c uIcn`:
IcvcI o con:cIou:nc:: I: u::c::cu u:Ing !c CIu:gov
Conu 5cuIc [:cc JuIIc I5-S). In vcuvuIon ov !c
:cconuuvy :uvvcy, !c ucn :!ou!u Ic unuvc::cu.
Bccuu:c o c!IIuvcn': Iuvgc :uvucc-o-Iouy nu::
vuo, !cy cooI vuIuIy, unu u::Ivc !cu !o:: cun Ic
voIIcnuIc. lxposure [!yo- ov !ycvt!cvnIu)
nu: Ic uccccu unu ucuI vI! von!y.
OfugS USe0 n le0tfC Lf0OfeSftOf HeSuSCttOn
Lru@
PtrCplnC
BlCarbCnatC
LICmCntaICa|Clum
(CaIClumg|uCCnatCCr
CaIClum Ch|CrlOC}
UCxtrCsC
LpnCphrlnC(T:T0,000J
LplnCphrlnC (1 .1 000]
LlOCCalnC
PmlCOarCnC
NarCan
lnUICatlon
BraOyCarOaanO
atrlCvCntrlCuIar bICCk
5CvCrC rCIraCtCrymCtabC|lC
aClOCslsanO/Cr hypCrka|Cm|a
HypCCaICCmla,hypCrkaICmla,
hypCrmagnCsCmla,anOCaIClum
ChannC|bICCkCrCvCrOCsC
HypCgIyCCmla
PsystC|C,braOyCarOla,puIsCICss
V1, V|
|uIsCICssarrCstaItCrabCvC
OCsC CrashrstOCsC OCwn
CnOCtraChCaI tubCl!nC
vasCuIaraCCCssavalIabIC
VCntrlCuIarCCtCpy,V1,V|
PtrlaI(rCIraCtCry5V1} anO
vCntrlCuIar arrhy\hmlas
(rCIraCtCry puIsC|CssV1,
rC!raCtCryV|,hCmCdynamlCaIIy
stabICV1}
|rCsumCOCrknCwnCplatC
lntCxlCatlCn
LUCt
|nCrCasCs hCart ratC anO CCnOuCtlCn thrCugh thC
atrlCvCntrlCuIarnCOCby OCCrCaslng vagaItCnC
InCrCasCs b|CCO pH
InCrCasCsmyCCarOlaICCntraCtlIlty,lnCrCasCs
vCntrlCuIar CxCltablIIty,anOlnCrCasCsCCnOuCtlCn
vCICCltythrCugh thCmyCCarOlum
InCrCasCs bICCO gIuCCsC ICvCI
InCrCasCs systCmlC vasCuIar rCslsUnCC,ChrCnCtrCQ,
anO lnCtrCpy,thCrCby lnCrCaslng CarOlaC Cutput
anOb|CCO prCssurC (lnCrCaslng OlastCIlC bICCO
prCssurC lnCrCasCs CCrCnary artCry pCrlusICn
prCssurC}
5amC asabCvC
HCIpsmakCrCIraCtCry pu|sCICssV1 anOV| mCrC
susCCptlbIC tC CarOlCvCrslCn,may supprCss
hCmCOynamlCa|Iy stabICV1,anO OCCrCasCs thC
IlkCIlhCCO CI rCCurrCnCC CI vCntrlCuIar CCtCpy
B|CCksNa,anOLa ChannCIs anObCta-rCcCptCrs ln
thCmyCCarOlumaswCIIasaIpha- anO
bCta-rCCCptCrs lnthCvasCu|ar pCrlphCry
HaplOrCvCrsa|Cl CplatCClICCt
Orugs tat can De g|ven by endotracea| tube |nc|uCe ||Coca|ne, atro|ne, Narcan, anC epner|ne l!g! dose).
SV,supravenlrcuIar taccard|a;V|, ventrcular hbrI\atCn, V,ventr|cuIar tachycarda.
< nor tor saJeI > < ' ?
< ca M e
a

e
*
CrCnCyNaragCmCnt. aatt o |C rlaI Cr rrCO ChIO

5
%
hN LW

1. |C mattCt whattC CausC OI CarOOtCspttatCty


attCst,tC aIgOrtmsOut|tnCOlOrpCOtatttC OastC
anOaOvanCCO CarOaC|tICsuppOttsOu|ObC
IO||OwCO.P pttmatysurvCy(Ptway,tCathng,
LtCu|atOn,Usabt|ty,LxpCsutC}tsIOI|CwCObya
seCOnOatysurvCy.
2. PpptOxtmatC|yII OltCCausCsOI pCOtatrC
artCsttCOuCtOtCspttatCtyattCst,wtC CanbC
btCuQhtabOutbyuppCtatrwayCOsttuCttOn,IOwCt
arway OOsttuCttOn,tCstttCttvC|ung OtsCasC,Cran
CtOIOgytatrCsu|tsnnaOCquatCQasCxCangC.
d. 1hCL|HaIgOrtmts summarZCOn |gutC 14.
4. |rCsusCtatOn OOCs nOtCstaOItshCatOaCOutput,
tClO||Owng mCChanCaIOrmCtaOOIC CausCs
shCu|ObCtnvCsttQatCO.hypOtCtmta,tCnsCn
pnCumOthOrax,CmOthOrax,CatOaCtampOnaOC,
prCIOunOhypOvO|Cma,ptOIOunOmCtabCItC
mOa|anCC,tCxntnQCsttOn,anOCICsCO hCaOtn]ury.
SHDCK
5hock I: u :ynuonc c!u:uccIzcu Iy !c InuIIIIy o
!c cIcuIuoy :y:cn o ovuc uucquuc nu:cn:
o ncc !c Iouy': ncuIo!Ic ucnunu:. C!I!u:cn,
c:ccIuI!y nconuc:, vIII inIuIIy :y o concn-
:uc Iy IcconIng uc!ycuuIc. Iyocn:Ion, u Iuc
hndIng, Icud: o ccIIuIu !yoc:u:Ion ncuIoIc
ucIuo:I:, unu ccIIuIu ucu!. J!cc vc!uIon:!I:
cIuIn !yocn:Ion n :!ock.
Iood pressure [cuuuc ouu :y:cnc vu:cu-
Iu rc::uncc)
Cardiac Out[u [:t:okc voIunc X !cut utc)
5troke voIume [uccnIncu Iy c!ouu jvcn-
:IcuIu cnu uIu:oIIc voIunc], u!tcIouu
j:y:tcnIc vu:cuIu c:I:uncc, unu nyocuuIu!
cont:uctIy)
J!c !cc :ugc: o :!ock uc concn:ucu,
unconcn:ucu, unu Icvcr:IIIc. |n !c compensated
stage, !onco:uIc ncc!unI:n: nunuIn c::cnuI
ogun cu:Ion. BIoou c::uc, uInc ouu, unu
cuuuc uncIon uI! :ccn o Ic nonuI. In !c uncom-
pensated stage, !onco:uIc ncc!unI:n: uI! Iccuu:c
o I:c!cnIu, cnuo!cIIu! Injuy, unu t!c c!uIouIon o
toIc nucIuI:. Ivcnuu!Iy, ccI!uIu uncIon uccIo-
:uc: unu nuIogun :y:cn uy:!uncIon rc:uI..
V!cn !I: vocc:: !u: cuu:cu IvcuvuIIc unctIonu!
Io:: In c::cnuI orgun:, !c cnInuI o irreversibIc
stage o :!ocI I: cuc!cu.
J!c yc: o :!ock Inc!uuc !yovo!cnIc, cuuIo-
gcnIc, uI::Iuvc, unu :cc. lypovoIemic shock
c:uI: on uccrcu:cu n:uvu:cu!u voIunc, v!c!
c:uI: In uccu:cu vcnou: |cun unu nyocuuu!
cIouu. Bccuu:c o !c :cuucIon In nyocuuIu!
cIouu, !cc I: u c:uIun ucccu:c n :t:oIc
voIunc, cuuuc ouu, unu IIoou c::uc. J!I: I:
t!c no:t connon cIoIogy o :!ock In c!IIucn.
CurdiOgenIC shock : !c c:uI o "un uI!u:c.
|nuucquuc :tokc vo!unc c:u!: In uInIn:!cu
curuIuc ouu unu !yocn:Ion.
OistriIutive shock c:uI: ron un uInonu!Iy n
vu:onoo onc !u Icuu: o nuIuI:IIuon o u
no:nu! ccu!uoy voIunc unu u :uc o cIuvc
!yovo!cnIu. Bccuu:c o cI!cuI ooIIng, cIouu
I: cuuccu, cuu:ng u ucccu:c in :rokc vo!unc,
cuuIuc ouu, unu I!oou c::uc. 5y:cnIc vu:cu-
I c:I:uncc I: uI:o ucccu:cu duc o vu:onoo uy:-
uncon. Bccuu:c lo! :y:cnc vu:cuIu c:I:uncc
unu cuvuIuc ouu uc cuuccu, :cvcrc !yocn:Ion
rc:uI:.
5eptic shock :c:uI: v!cn ccvuIn u!ogcn: Incc
!c IIoou. J!c curIy concn:ucu :ugc o :cIc
:!oc : c!uuccIzcu Iy ucccu:cu vu:cuIu c:I:-
uncc [uI:IIuIvc :!ock), v!ccu: In !c Iuc
unconcn:ucu !u:c, !yovoIcnIu on nIu
:ucIng unu un uI!uc uuc o nyocuuuI ucrc:-
:on lcconc: noc uucn. Concn:ucu :cc
:!ock I: cuI!cu "vuvn" :c:I:, unu unconcn:utcu
:cIc :!ock I: cccu o u: "coIu :c:I:.
J!c no:t connon cIo!ogIc: o cuc! yc o
:!ock u:c I:cu In JuIIc -J.
CI|n|caI Man|festat|ons
M5fOly DD0 Dy5CD LXDHDDfOD
J!c !I:oy :!ouIu |ocu: on otcntIuI cuu:c:. Con-
:Iuc !yovoIcnIc :!ock I !cc I: u !I:toy o von-
Ing, uIu!cu, oIyuIu, Iun:, uunu, :ugc:
gu::oInc:nuI IIccung, inc:InuI oI::ucIon, Iong
cou: In !c :un, o: unccuI:. !I:oy o con-
gcnIuI !cu uI:cu:c, ur!yt!nIu:, o c!cno!cuy
[u:IunycIn) uunIn:uIon nuy inuIcuc curuo-
gcnc :!ock. II:IIuvc :!ock :!ouIu Ic concn-
!ucu v!cn !ec I: u !I:oy o oIc Ingc:Ion,
unu!yIuI:, o !cuu o :InuI cou njuy. In uuuI-
on, uny InnunocononI:cu utcn v!o c:cn.
*
not o ai

< nc D ma=
HCS
i >
< crau u cxanrronnccun: nvcm o- on-. y >
lO
l
LjOl llvy
|OUI| IO |OUl|
LllO| |Olj
I
LOllj5OO C|llO
O|OI|||Q:

YO5
H|OI||lQ:

YO5
|llIlllIWy
LlVO L_
VO|I||I|O|OOQUIO:
L|O5I |l5O5 vlI| lO |lOUVO|5O|
|OUI| lO |OUI|:
OO5I|UCIOO llvy5

[C|O|llQ}l|ylQO5COjy)
LOlIllUOVOlIl l IlOl """"""
LlVO L_, OQ-|5|
lO
LlOOI|C|Ol llIUOIlOl
l
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_
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ljO UOlOlO:

LOv lO||l
L|O5I CO|j|O55lOl5
LlVO VOlU|O
C|llOlQO
l
|l5U|O OOUCyOlVOlIllIOl
LlO5O |OllIO|llQ
Ll!O|OlIll OlQlO5l5:
5I2Ol|5| lV}CUIOOv|
||ItVO|OU5||5|IU: |O I|It2O55OOU5 ||U5|O|
l
j|Oj||llO}OlC|OOlIO
YO5
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lO|OOyl|lClly Ul5IO|O}
5IOlO V |O|2CIO|y
IO|OO5
|O|OOyl|IC||y
lO|OOyl|lClly Ul5IOlO}
5IOlO V1 |O|2CIO|y
IO |OO5
|lOOClO
l|lOO|OlO
||OCll|lOO
1|OI |yjO|QlO5O|l
1|OI|yjO|lO|l
L|OlOVO|5lOl LOllO|ll |IO V2Ql |lOUVO|5
lOOlO5|lO
l|lOO|O|O
L|OlOVO|5IO|
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5y5IOlO

lI|OjllO
LjllOj|||lO
|CO|2|O|
gUtH 1 -4 L|O|OjUHOG|y |CSUSC1I|OG QO|1|O.V,VCG1|CU|IC|yC|O,VVCGI|CU|DO|1OG,5V,SUj|VCG1|CU|
1C|yC|O.
< not foz zac
_
< __ _
_ < can u rcxannonncc: ucm o- on-. JV
_
.
atC CrgCncyNanagCmCrt:LvaIuatCn CIthCCrtcaIIyIIIor InjurCOChIO 7
1PLL 15
1he LtOOgeS O bhOck
HQ0V0emc
WatCranO CICctrOIy\CIOssCs
HCmOrthagC
|IasmaIOssCs (thrOspacng}
Cr0Ugec
LOngCntaIhCartOsCasC
IschCmchCartOsCasC
LatOlOmyOpathCs
Prrhy\hmas
InICctlOns
StrDutVe
PnaphyIaxs
NCurOIOgcnjury(hCaO
Orspna|cOrO}
UrugtOxcty
beQtc
InlCctOn
NSceeUuS
|u|mOnaryCmbOIsm
POrCna|nsuIhcCncy
vI! u !I:ovy o cvcv unu : III ucuvIng nuy Ic In
:cIc :!ock.
5cvIuI vIuI :Ign: uvc cvIcuI In !c uIugno:I: unu
nunugcncn o c!I!u:cn vI! :!ock. In cuvIy vuvn
concn:ucu :ctc :!ock, vu:ouIIuIon, vuvn
cvcnIc:, uc!ycuvuIu, u vIucncu uI:c :c::uvc,
unu uucquuc u:Inc ouu uvc :ccn. In convu:,
:ynon: o !yovoIcnIc, cuvuIogcnIc, unu Iuc
"coIu" unconcn:ucu :cIc :!ock incIuuc vu:ocon-
:vIcIon, uc!ycuvuu, coIu cvcnIIc:, oov cvI-
!cvu! u!:c:, uIcvcu con:cIou:nc::, uIIov, :vcung,
IIcu:, unu oIIguvIu.
D|agnost|c cvaIuat|on
IuvIng !c :uIIIIzuIon cvIou, !c cIInIcIun nu:
uccvnInc no v!Ic! cucgovy o :!ock !c ucn:
IIInc:: uII:. ny ucn vI! :!ock :!ouIu Ic Iuccu
on u cuvuuc nonIov. J!c Icvc! o uc!ycuvuIu I: !c
Ic: uccvnInun o !c !cvcI o Invuvu:cuIuv ucIc-
Ion ov vu:onoov uInovnuIy. yocn:Ion I: u Iuc
!nuIng unu occuv: on!y u!tcv 40% o !c Invuvu:cuIuv
voIunc !u: Iccn uc!ccu. IIugno:c c:: uvc uccv-
nIncu on !c Iu:I: o !c :ccI!c cuu:c: :u:cccu.
reatment
J!c :cuncn o :!ock : uIncu u cn:uvIng cvu-
:Ion o vIcuI vu:cuIuv Icu: [covonuvy, ccvcIvuI,
!cuIc, vcnuI) unu vcvcnIng o: covvccIng ncu-
IoIIc uInovnuIIIc: uvI:Ing :on ccIIuIuv !yocvu-
:Ion. unugcncn o !yoIu vcuucc: t!c IcvcI o
ncuIoIIc ucIuo:I:. Covvccng ncuIoIIc ucIuo:I:
vc:uI: In Iccv ccIIuIuv uncon, nyocuvuIuI cvov-
nuncc, unu uccvcu:cu :y:cnIc unu uInonuvy vu:-
cuIuv vc:I:uncc.
HyovoIcnIc :!ock I: vcucu vI! novnuI :uInc
ov Iucucu RIngcv': :oIuon [:cc C!ucv 7 ov
ucuII:). I !cnovv!ugc I: !c cuu:c o !c !yo-
voIcnIu, yc I-ncguvc, cvo::-nuc!cu v!oIc
IIoou ov uckcu vcu ccII: nuy Ic gIvcn. In cuvuo-
gcnIc :!ock vc:uIng :on u congcnuI !cuv uccc,
:uvgcvy, Iu!Ioon ungIoIu:y ov vuIvuIo!u:y, :uvgcuI
vuIvoony, ov Ino:oIc :uov nuy Ic nuIcucu.
C!IIuvcn vI! :cvcvc I:c!cnIc Injuvy o !c !cuv,
uIIucu cu:uIonyou!y, ov nyocuvuII: nuy nccu u
!cuv vun:Iun. In uI:vIIuvc :!ock uuc o unu-
!y!u:, nvuvcnou: :cvoIu:, cnuuvyI [u!cn!y-
uvunInc), :uIcuuncou: cInc!vInc, unu u!Iucvo!
ncIuIIzcv: uvc cnIoycu. 5oncnc: InuIuon ov
Iuvyngo:u:n unu vu:ovc::ov: ov IntvucuIIc !yo-
cn:Ion uvc nccucu. 5cIc :!ock I: vcucu vt!
vu:ovc::ov:, !.uIu:, unu Ivouu-:cc:un unIIoIc:.
nIIIoc: uvc con:Iucvcu u vc:u:cIuIon ncuIcuIon
|ov :cc :!ock.
.
hN LW
M
1 . LClCtmtnC tCcatCQOtyCIsCckanO wCthCt lhC
patlCnl as CarIy Ct |alC mantICstatOns.
2. HypOvCICmcsOck accOunlsIOtmOstCasCsOl
sOck.
3. In ypOvOICmcsCck,O|OOO prCssutCOCprCsstCn
tsa |atCDnOlng,anO tC|CvCI OltacycatOtatsthC
mOst sCnsttlvC mCasurC Oltntrvascu|atlutO
status.
4. InsCplc sOck,anttbtOlcsarC a rCsusctlattOn
mCOtcatOn anO tCtt OmInlstratIOn shOuIO nOt
OC OCIayCO.
ov!cvc uoc: !c oIu uuugc "un ouncc o vcvcnIon
I: vovt! u ounu o cuvc" vc:onuc novc uc !un
n cuIuIc:. Jogc!cr, uccIucn: unu InjuvIc: uvc !c
Iuvgc: cuu:c o novIuIy unu novtuIIy In c!IIucn.
Vncn un unovuvu cvcn occuv:, ncIy cvuIuuon
unu vcuncn nuy IIn uI:uIiIIy unu vc:cvvc
quuIIty o! IIc.
$ __ACJ]O|SDN|NG
- -- -
IoI:onIng I: onc o !c oovc connon cuuIc
ncucuI cncvgcncIc:, rc:uIng In ovcv 2 nIIIIon
cncvgcncy vI:I: u ycuv. Iou S0% o c!IIu!oou oI-
:onIng: occuv In c!IIuvcn younger dvan b years.
J!c:c cnu o InvoIvc onIy onc :uI:uncc unu nuy
ucnoc cI!cv accidentaI Ingc:Ion ov [novc vuvcIy)
uIu:c Iy cuvcukcv:. AdoIescents uccoun ov !c
vcnuInIng 207; :uc! Ingc:on: uvc u:uuIIy inten-
tionaI, vcvc:cn u :uIcIuc uttcn ov gc:uc, unu
nuy InvoIvc nuIIc :uI:uncc:. RccvcuonuI uvug
u:c In !I: oIucv ouIuIon cun vc:uI in unIncn-
onuI Iu uuI ovcvuo:c:.
CI|n|caI Man|festat|ons
M5fOly DD0 Dy5CD LXDHDDfOD
J!c !I:ovy :!ouIu IncIuuc !c :uI:uncc ingc:cu,
v!cn, !ov nuc!, unu :uI:cqucn Ic!uvIov. J!c
c!uvuccvI:c cIInIcuI nunIc:uIon: unu cuncn
o !c no: connon oI:onIng: In c!IIuvcn unu
uuoIc:ccn. uvc u:cu::cu n JuIIc Z-I.
D|herent|aI D|agnos|s
J!c o::IIIIIy o oIcoIogIc ingc:Ion :!ouIu Ic
con:Iucvcu In uny ucn vc:cnng vI! uIcvcu
ncnuI :uu:, ucuc Ic!uvIov c!ungc:, :cIzuvc:,
uv!yt!nIu:, ov conu.
D|agnost|c cvaIuat|on
5cvccnIng :uuIc: :!ouIu IncIuuc u uI:c oygcnuon
c!cck, uco:c-:ck, cIccvocuvuIogvun, :cvun
cIccvoIytc: unu o:noIuvIy, unu H vcnou: IIoou
gu: o uccvnInc . BIoou unu uvInc oIcoIogy
:cvccn: uvc vuvIuIIy !cIuI; !c cIInIcun :!ouIu
inquIvc v!Ic! :uI:uncc: In uvIcuIuv uvc :cvccncu
|o:
Treatment
Jvcuncn :!ouIu Ic Iu:cu on !c c:nucu
nuInuI ocnIuI uo:c Ingc:cu. C!IIuvcn vI! :Ig-
nI!cun Ingc:on: unu ucn. v!o uvc ncuIcuIIy
un:uIIc vcquIvc uIIIgcn oI:cvvuon unu nunugc-
ncn o !cIv uIvvuy, Ivcu!Ing, unu cIvcuIuon.
InuucIon o cnc:I: v! syrup ol ipecac I: uvo-
vuc In :onc cu:c: n !c :uI:uncc vu: vcccnIy
Ingc:cu. Lastric Iavage Iot! rcnovc: unu uIuc:
:onuc! concn:. IIII ugncn. vccovcvcu Iy cI!cv
nc!ou nuy uIu In uIugno:I:. Activated charcoaI Iy
nou! ov nu:ogu:Ic uIc nnInIzc: uI:ovon Iy
IInung !c :uI:uncc unu !u:cnIng : cIInInuon,
pccac is spccihcally contraindicatcd lor ingcstions oI
hydtocarbons andcaustic acids/Lascs
* not tor saJe! > < ue rn oa ! >
< M

o-


-

.
aCr / !'Cr,s,afU!hUyrCvCntCr
#
V
bgnS, bmtOmS, n0 fetment O bechc le0tfc lOSOnngS
buUSlnCC
PCCtamnOphCn
PntChOInCtgCs
[atrOpnC,trCyCIC
antOCprCssants,
anthstamnCs,
phCnOthazOCs}
Psprn
ChOInCrgCs
[OrganOphOsphatCs
anOOthCrpCstCOCs}
HyOrOCatbOns
|tOn
OpatCs
5COatvCs/hypnOtCs
5ympathOmmCtCs
[amphCtam|nCs,
COCanC,
thCOphyIInC}
LlnCal NnlCStatonS
NausCa/vOmtng,anOrCxa,paIIOr,
OaphOtCss,may prOgrCss OvCrOays
lO jaunOCC,abOOmnaI pan,IvCr
IaIurC
|CvCr,myOrass,Ilushng,Oryskn,
taChyCarOa,hypCrtCnsOn,CarOaC
arthyIhmas,OCIrum,psyChOss,
COnvuIsOns,COma
|CvCr,hypCrpnCa,vOmtng,tnntus,
ICthatgy,COma
NausCa/vOmtng, swCat|ng,mCOss,
sa|vatOn,IaCrmatOn,brOnChOrrhCa,
urnatOn,OCICCatOn,wCaknCss,
musC|CIasCCuIatOns,paraIyss,
COnIusOn,COma
|CvCr,nausCa/vOmtng,
gastrOntCstnaIbICCOng,
COnIusOn,COma
VOmtng,O|arrCa,
gastrOntCstnaIbICCOng,
CyanOss,sCZurCs,COma,
mCtabOICaCOOss
|npOntpup|s,btaOypnCa,
hypOtCnsOn,hypOthCtma,
stupOr,COma
Nystagmus,mCOssOrmyOrass,
hypOthCrma,hypOtCnsOn,
btaOypnCa,COnIusOn,ataxa,COma
|CvCr,myOrass,taChyCarOa,
hypCttCnsOn,swCatng,OCIrum,
psyChOss,trCmOr,myOCIOnus,
COnvu|sOns
Pnt0olCfeatment
P. N-aCCtyICystCnC
1.gastrCCmptyngI<2hr
snCCngCstOn,aCtvatCO
CharCOaII<4hrsnCCngCstOn.
UrawbIOOOICvCIat4 ranOusC
avaIaOIC nOmOgramtOassCss
rskOIhCpatOtOxCty.IItOxC,
startOraIN-aCCtyICystCnCanO
COntnuCkr72hr
P. physOstgmnC IOratrOpnC
anOanthstamnCs
A. NaCOMg5IOrtrCyCIC
antOCprCssants
1.gastrCCmptyngI<ht
snCCngCstOn,aCtvatCO
CharCOaI,CathartCs,IluOanO
C|CCtrOIy\CmanagCmCnt
P. ptaIOOxmCChIOrOC
1.gastrCIavagC,aCtvatCO
CharCOaI,prOphyIaCtCatrOpnC
1. fCvenl Splfton
[PspratOnrCsuItsn ChCmCaI
pnCumOntsanOsgnhCantIung
tssuCOamagC!} NCgastrC
CmptyngtCChnquCsarC
nCCssaty.
P. OCIerOxamnCCCIatOn
1.CmCss nOuCtOn,gastrC
IavagC,CathartCs
P. naIOxOnC
1.CvaIuatCanO sCurC arway
asnCOCO,gasttOntCstnaI
OCCOntamnatOnIapprOpratC,
naIOxOnC
P. IlumaZCnI IOr
bCnZOOaZCpnCs
1.CvaIuatC anOsCCutCarwayI
nCCOCO,mantanhCmOOynamC
stabIty,aCtvatCOCarCOaIw|th
CathattC,suppOrtvC CarC
1.gastrC Cmptyng,aCtvatCO
CatCOa|,CathartCs,sCOatvCs
krsCvCrC agtatOn,COntrOI
OIhypCrtCnsOn,amp|CIluOs
1

o < nc rn nora i >


< cx v cx anz-onnccn: ucm o- pon-. y >

I
!ovcvcv, uctIvutcu c!uvcouI I: Inc!lcctvc In Ingc:-
ton: vIt! uIco!oI, !yuvocuvIon:, Ivon, unu IIt!Iun.
5ccI!c untuotc: cxI:f ov :cvcvuI connonIy
ingc:tcu uvug: [:cc JuIIc 2-I).
Prevent|on
IcuIutvicIun: !uvc Iuycu u nujov voIc In uccvcu:Ing
t!c nunIcv unu :cvcvIty o oI:onIng:, IncIuuIng
IoIIyIng ov c!IIu-vc:I:tunt mcuIcInc IottIc cu: unu
incovovutIng untcIutovy guIuuncc Into vcII-c!IIu
vI:It.. 5ccI!c toIc: IncIuuc "c!IIuvoo!ng" t!c
!onc, kccng ncuIcInc: In u Iock Io, vcnovIng
cIcunIng vouuct: :on c!IIu:cn': vcuc!, unu t!c juuI-
cIou: u:c oI :yvu o Iccuc.
Lc0D P D|SDN|NG
lcuu oi:onIng i: onc o t!c mo:t Imovtunt
vcvcntIvc !cuIt! I::uc: In vInuvy cuvc cuIut:Ic:.
J!c cIIninutIon o Icuu In !ou:c uInt [In I977) unu
gu:oIInc [In !9SS) !u: uccvcu:cu t!c uvcvugc IIoou
IcvcI o Icuu Iy 7S%. J!c vInuvy :ouvcc o Icuu
touuy Icuu-contuInIng uInt vc:cnt in IuIung:
con:tvuctcu Icovc !9SJ. C!IIu:cn Ivcut!c In Icuu
uu:t, Ingc:t uInt c!I:, unu Iuy In Icuu-contunI-
nutcu :oII. 1t!oug! t!cvc I: no uIvcct covvcIuton
Ictvccn IIoou IcvcI: unu movIIuty, IcvcI: o IJ to
I9g/ul uvc con:Iucvcu IovucvIInc, unu t!c tcvm
Iead poisoning" I: vc:cvvcu ov IcvcI: o Z0gldl ov
gvcutcv.
CI|n|caI Man|festat|ons
Fuvy :ynton: o Icuu oI:onIng ncuuc IvvItuIII-
Ity, !ycvuctvIty, uut!y, uccvcu:cu Iuy, unovcIu,
uIuonInuI uIn, con:tIutIon, unu Intcvnttcnt von-
Itng. C!IIu:cn vIt! c!vonIcuIIy cIcvutcu Icuu IcvcI:
nuy nunIc:t ucvcIoncntuI ucIuy, Ic!uvIovuI voI-
Icn:, uttcnton uI:ovucv:, unu oov :c!ooI cvov-
nuncc. 1cutc cncc!uIout!y : tc no:t :cviou:
conIicuton o Icuu oI:onIng unu : c!uvuctcvzcu
Iy Incvcu:cu intvucvunIuI vc::uvc, vonItIng, utuIu,
conu:Ion, :cIzuvc: unu conu.
reatment
J!c no:t clcctvc t!cvuy InvoIvc: removing the
poison vom t!c c!IIu': cnvIvonncnt. lcuucu uInt
:!ouIu Ic :tvicu unu :uvucc: cIcuncu vIt! !Ig!-
!o:!utc uctcvgcnt unu u :ccIuI !Ig!-clcIcncy
uvtIcIc uccunuIutov vucuun. 5uc! un ovcv!uuI
InvuvIuIIy Incvcu:c: t!c unount o Icuu uu:t In t!c
uIv, :o t!c In!uIItunt: nu:t Ic tcnovuviIy !ou:cu
cI:cv!cvc.
5yntomutc c!Iuvcn :!ouIu Ic ImmcuIutcIy
vcnovcu to u Icuu-vcc cnvIvonncnt unu t:cutcu
vIt! Intvunu:cuIuv dimercaproI [B1l) oIIovcu Iy
Int:uvcnou: edetate caIcium-dis odium [FIJ1). traI
succimer [I^51) I: un uItcvnutvc in u:yntonutIc
c!IIuvcn vit! IcvcI: IcIov 6Jg/u!.
Prevent|on
Rcccnty nouIlcu guiucIInc: ov :cvccnng vc!cct
t!c contnuIng uccvcu:c In t!c ncIucncc o cIcvutcu
IIoou Icuu IcvcI: In c!IIu:cn. Juvgctcu :cvccnIng I:
Iu:cu on :I:k u::c::ncnt Inovnuton gut!cvcu uuvIng
vcII-c!Iu vi:it:. J!c Ccntcv: ov II:cu:c Cont:o unu
Ivcvcnton vcconncnu: Icuu :cvccnIng ut !2 unu 24
mont!: ov uticnt: IivIng in uvcu: vIt! muny vc-
| 9SJ !onc: unu unu:uuIIy hig! cvccntugc: o
cIcvutcu IIoou Icuu IcvcI:.
MDDR VcH|CL 0CCl DcNS
^otov vc!Icc injuvic: vcnuin t!c Icuuing cuu:c o
uccIucntuI ucut! In uII ugc gvou:. ^o:t Inunt:
unu uuoIc:ccnt: :u:tuIn tvuunu u: vc!IcIc occuunt.,
v!cvcu: :c!ooI-ugc c!IIuvcn tcnu to Ic injuvcu u:
cuc:tvIun:. !uctov: u::ocIutcu vtI un Incvcu:cu
vI:k o uutonoIIIc injuvy unu ucut! IncIuuc nuc
gcnucv, ugc Ictvccn IS unu I9 ycuv:, vuvn ov In-
cIcncnt vcut!cv, nIg!t ov vcckcnu u:IvIng, unu
uIco!oI IntoIcuton.
J!c voutInc u:c o seat beIts unu chiId car :eat:
!u: Iccn :!ovn to Ic !Ig!Iy c!lcctIvc n vcuuc-
Ing t!c Inciucncc o :cvcvc Injuvy unu ucut!. 1II
:tutc: vcquivc cuv :cut vc:tvunt o u::cngcv: unucv
4J ounu:. C!IIuvcn 2J ounu: ov !cuvIcv unu
ycuv o ugc ov oIucv nuy vIuc ucIng ovvuvu, v!cvcu:
IIg!tcv Inunt. nu:t ucc t!c vcuv. IIucv c!IIu:cn
:!ouIu vcnuIn lctcu vIt! u unu :!ouIucv :tvu:
ut uII tInc:. |ccuu:c uiv Iug: uvc uc:Igncu vInuvIIy
ov uuuIt:, c!IIuvcn :!ouIu vIuc IcItcu In t!c Iuck
:cut v!cncvcv o::iIIc. 1here is no evidence that
driver education programs are an eHective deterrent
to accidents invoIving teenage drivers.
ike heImets uccvcu:c t!c v:k o :IgnI!cunt cIo:cu
!cuu tvuumu uuc to tvumc uccIucnt: InvoIvIng
IIcycc:. In nuny juvI:ucton:, uv nunuutc: t!cIv
u:c Iy c!IIuvcn. C!iIuvcn youngcv t!un IJ ycuv:
< not tor zac i > < nc rn noa i >
< v c

nnc

o-

n-

> .
1C| S| ||S| || |CC|1o|
#
1 1
:!ouIu Ie :uevvI:eu v!IIe vuIkIng ov !uyIng neuv c!IIuven ut uuuIt.onuI vI:!. Puts uccount ov ovev S0%
:tveet:. o oveIgn Iouy u:Ivut.on:.
DRDWN|NG
IvovnIng I: u :equent cuu:e o novIIuIty unu nov-
tuIIty In t!e euIutvIc ou!ut.on. lncIuence eu!:
Ieveen l unu S yeuv: unu uguIn In uuoIe:cence.
Rue: uve tvIce u: Ig! in IIuc!: unu tvee Ine:
!Ig!ev In Ioy:. athtubs uve ue no: connon :Ie
o u:ovnIng In t!e Iv: yeuv o IIe. Luvge Iuc!et:
unu ve:IuentIuI ooI: uvc uvt.cuIuvIy uungevou: ov
touuIev:, v!eveu: nutuvuI vutev :ouvce: uccount ov
no: uuo!e:cen InjuvIe:. ReIIuI!e veuIctov: o out-
cone IncIuue vutev tenevutuve, tIne o :uInev-
:Ion, uegvee o uInonuvy uunuge, unu e!ectIvene::
o euvIy ve:u:cItutIon e!ovt:.
5uInev:Ion ov nove t!un S nInute: In vuvn
vutev u::ocIuteu vIt! :IgnI!cun u:Ivut.on unu
nInInuI ve:on:e to InItIuI cuvuIouInonuvy ve:u:cI-
tut.on [C|R) vIvtuuIIy uIvuy: ve:uIt: In nujov uI:-
ulIIIty ov ueut!.
Jouuev: unu young c!IIuven nu:t Ie :uevvI:eu
ut uII tIne: v!IIc In t!c Iut!tuI ov uvounu oo!: ov
ot!ev IouIe: o vutev. Re:IuenIuI unu connevcIuI
:vInnIng ooI: :!ouIu Ie enceu In unu !uve Ioc!eu
gute:. CIR tvuInIng I: uvuIIuIIe to uvent: !voug! t!e
nevIcun euvt ::ocIutIon unu nuny uveu !o:Iul:.
LeuvnIng to :vIn I: un Inovtunt vevent.ve neu:uve
Iut uoe: not tu!e t!e Iuce o c!o:e :uevvI:Ion .
DR|GN DDY 0SP|R0| DN
. . . . . a . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
J!e nutuvuI cuvIo:Ity o c!IIuven couIeu vIt! t!c
touuIev`: tenuency to ut evevyt!Ing In t!e nout!
nu!e loreign body aspiration u vequent occuvvence
In t!e euIut:Ic ouIutIon. Mo:t oIjec: unu oou-
:tu: uve InneuIuteIy eeI!eu hon t!e t:uc!eu Iy
coug!Ing. InovtunuteIy, oveIgn IouIe: t!ut Iouge
In t!e uev o: Iovev ve:Ivutovy t:uct uve nove
voI!enut.c.
cp|dem|o|ogy
J!e !Ig!e:t IncIuence I: noteu In c!I!uven 6 to
36 nont!: oIu. :IvutIon Into t!e !ovev uIvvuy: I:
nuc! nove connon t!un tvuc!euI oI::uct.on,
nuny oIject: Iouge In t!e righI main stem bronchus
Iecuu:e o Ivonc!Iu! unutony. lnuuequute :uevvI-
:Ion unu InuvovIue oou c!oIce: ov uge Iuce
D|fferent|aI D|agnos|s
|utIent: v!o uo not ucuteIy oI:tvuct t!eIv uIvvuy:
nuy ve:en u to u vee! ultev t!e InIt.uI event
vIt! no vItne::eu eI:oue o c!okIng. V!eezIng unu
ve:Ivutovy uI:t:e:: nuy Ie nI:tu!en ov u:t!nu,
neunonIu L u con:IuevutIon v!en Iveut! :ounu:
uve uecveu:eu. I note, InuIng: on uu:cuItutIon In
cu:e: o oveIgn Iouy u:IvutIon uve IocuIIzeu to one
:Iue o t!e c!e:t onIy.
C||n|ca| Man|festat|ons
|ie:entutIon vuvIe: ueenuIng on v!eve the oveIgn
Iouy Iouge: In t!e ve:Ivutovy tvee [JuIIc 2-2). I t!e
oI::ucIon I: compIeIc, t!e c!e:t vuuIog:u! uenon-
:vute: :IgnIIcunt one-:Iueu ute!ectu:I: unu t!e !euvt
I: uvuvn tovuvu !e u!ecteu !ung t!voug!out t!c
cntIve ve:Ivutovy cycIe. Movevev, u partiaI oI:tvuc-
tIon uIIov: uI: to entev uuvIng In:IvutIon, unu it
Iecone: t:ueu [LuII-vuIvc oI:tvuctIon). In t!e:e
cu:e:, t!e In:Ivutovy !In nuy ueuv novnuI, ut he
-vuy ultev eIvutIon vIIl :!ov u !yevInLuteu
oI:t:ucteu Iung vIt! neuIu:tInul :!Ilt uvuy :on t!e
IIoc!uge [!Iguve 2- I).
Igure 2-1 LXj |1O|y UH G O|CQG DOOy Sj|IOG w1|
j|1 ODSI|UC1OG, 1|C ODS1|UC1CO C! UGQ S |yjC|Gl 1CG,
w|C|CS 1|C |C|1 [GO HCO|S1 GUH) |C S|!CO1O 1|C |Q||.
1 2
not ,tor > .< nc Dm noa i >
< cx v cxanr-ronncjcun: nvcm o- on-. jy >
uc
[
rn1O |'CC|at cs
1PLL Z~Z
bgnS n0 bDtOmS O Ofegn bO0 PSftOn
LoCaton o1 LUSt|uCton PSSoCatC0 bgnS an0
b_mptomS
1rachea
1Ota|CbstructlCn PcutCasphyxla,sCvCrC
tetractlOns wlth pOOt
chCstwa|ImOvemCnt
ExtrathOraclc,partla| |nsplratOryandCxplratOry
sttlOOr,tCtractOns
IntrathOracc,partaI LxplratOrywhCCzC,
thCrC|sIrCquCnt|y
lnsplratOrystrdOras
wCI|
ManstCm brOnchus LOughand CxpratOry
wheeze,thCrCmay be
b|OOd-tngCO sputum
LObat/sCgmCnta| brOnchus UCcrCased breathsOunOs
OvCtaI!CctCd IObC,
whCCzng,rhOnch
reatment
ForcignlodicsnusflcrcnovcoIron fhcairvayo
aIIcviatesynfons. Rigid bronchoscopy isfhctrcat
ncnf oI choicc. JhcrcaItcr, rognosis dccnds or
hc degrec oIIung danagc, vhich isdirccf!yrcIatcd
o ine incrvaI fo diagnosis. Most aicns rccovcr
quicIlyvith nininaI scqucIac.
Prevent|on
InIanfs arc nofdcvcIoncnfaIIyrearcdfo rocc
fhcir airvays Iron snaII norscIs oI Iood, incIud-
ing hardcandy, nufs, andocorn. SnaIIoys,coins,
lufons, and laIIoons shouId lc Ict ouf oI thc
foddIer's rcach. ChoIing is covcred in lasic CIR
cIasscs, hovcvcr, fhc cIIccfivcncss oI fhc IcinIich
nancuver or lacI lIovs is Iinifed in insanccs oI
lovcr rac asirafion.
URNS
Burns arcfhc fhird Icading causc oIinjury in chiI-
drcn,lehindnoorvchicIcaccidcnsanddrovnings,
and arehc sccondnost Ircqucnfcausc oIaccidcn-
fal dcah.n csinafcd IS% oIlurns arc hc rcsuII
oIabuse. ForfunatcIy,hcgrcatrajorifyoIlurnsarc
not IiIe-thrcacning. Iaficns vho survivc scvcrc
lurns are olcn IeIt vih signiIcan scarring and
disaliIiy.
cp|dem|oIogy
JhcnajoriyoIlurnsarcscaId injuries.Iame lurns,
usualIy acconanicd ly snoIc inhaIafion, arc Icss
Ircqucn lu accoun Ior nos deafhs. fyicaI
sccnario Ior an eIectric lurn invoIves a young chiId
uffing conducfivcnacriaIinoa vaIIsocIcor an
inIanfsucIing onthcconncccdcndoIan cxcnsion
cord. Contact lurns rcsuIf Iron direct confacf vifh
a hof surIacc.
R|sk actors
Boys and chiIdrcn youngcr fhan S ycars arc af fhc
grcatcst risIIorlurn injury.
C||n|caI Man|festat|ons
Jhc cvaluafion oI scvcrity is lased on lody sur
Iacc area and dch. IariaI-thickness lurns arc
oiviocd into Irst-dcgrcc and sccond-dcgrcc lurns.
irst-degree lurns involvc onIy he cidcrnis, hc
sIin is red and cndcr lut docs not lIiscr. |irs-
dcgrcc lurns usuaIIy hcaI vifhin a vccI vifh no
rcsiduaIscarring.5econd-degree lurnsnay lc sucr-
IciaI [lesshanhalIhcdchoIfhcdcrnis) ordcc
[invoIvingnosoIhcdcrnisluIcavingacndagcs
suchassveafgIandsandhairIoIIicIesinfac) . Sucr
Icial artiaI-thickncss lurns rcsoIve in a Icv vccIs
vihIif!escarring,dccsccond-dcgreeinjuricsrcsuIt
in signiIcan scarring andnayrcquire skin graIting.
FuII-hickncss lurns cxcnd ino fhe sulcufancous
fissuc and arc dividcd ino third-degree and lourth-
degree burns. ourth-degree lurns invoIvc Iascia,
nuscIc,lonc,orjoinfissuc. |ofharenonfcndcrduc
o scnsory ncrvous issuc Ioss. |ccausc oIthc con-
Icc desfrucfion oI fhc cithcIiun, hcsc lurns
rcquirc sIin grals. ScciIc injury sics and afcrns
arc characcristic oIalusc [Figurc 2-2).
reatment
Burncd arcas shouId lc Iaccd inncdiafcIy in
IuIcvarnvacrorcovcrcdvihvctgauzcorcIofh.
Minorlurnsrcsondo gcnIc cIcansing, siIvcrsuI-
Iadiazine [SiIvadcnc), and drcssing changcs vicc a
day uniI rc-cifhcIiaIizafion occurs. |urns fhaf arc
< not tor zac i > < nc rn rqoa i >
<
;an cnnn_n ^ o- n > .
BC| | SG| G ||B|| G U| |CC|1o|
4
1 3
Ll Q|OIIO

W
|||O|5O| 5ClO
LlQ|IOtl O
Lt|lllQ l|Ol
Igure 2-2 bU|G G]U|yj11C|GS COGSS1C|1 w1| DUSC.
scvcrc, circunIcrcnfiaI, cxfcnsivc [norc han 0%
fo l S% oIhclody), orfhainvolvcuclacc, hands,
crincun, or Icc rcquirc norc scciaIizco carc.
Jrcaucnf incIudcs aroriafc nanagcncnt oI
airvay, lrcafhing, and circuIafion issucs, cIccivc
cIccroIytcandIluidhcrayo accounfIorincrcascd
IuioIoss,rcvcnfionoIinIccion,ainnanagcncnf,
ofinization oIcosncfic rccovcry, and carly nolil
i andrchaliIifafion.
Prevent|on
InsfaIIing and naintamug smoke detectors ard
dccrcasing vater heater thermostat scfings arc fhc
fvo oost succcssIuI rcvcnivc ncasurcs Ior avoid
inglurn injury.lI sIccvcarIor chiIdrcn shouId lc
consfruccd oI Ianc-rcfardan natcrial. Snoking
ccssafion dccrcascs fhc IiIclihood fhai nafchcs or
Iighcrs viII lcIcI:vhcrc chiIdrcn can cxcrincn
vih hcn. Parcns shouId lc counscIcd o racicc
cscacroufcs andrcinIorccfhc"sfo, dro, anJ roII"
fcchniquc Ior cxfinguishingIrc.
CH|LD 0USc 0ND NcGLcC
Injurics infcnionaIIycrcfratcdlyacarcfaIcrfha
rcsuIf ir norlidi or nortaliy consfifufc physicaI
a|use. 5euaI abuse isdcIncoasfhcinvoIvcncnfoI
a chiIdin any acivi ncanIo rovidc scxual gra
iIcafion o an aouIf. |aiIurc o rovidc a child vifh
aroriac Iood, clofhing, ncdicaI carc, schooIing,
and a saIccnvironncnfconsfiucs negIect.
cp|dem|oIogy
lnos baII fhc chiIdrcn vho arc lrough Ior
ncdicaI acnfion as a rcsuI oI hysicaI alusc arc
undcr I ycaroIagc,fhcgrcafnajoriarcrcschool-
crs.IfiscsfinacdfhaI0% oIcncrgcncyroonvisifs
invoIving chiIdrcn youngcr han 5 ycars rcsulfIron
alusc. Parcnfs, fhc nofhcr's loyIricnd, and scar
cnts arcthcnosIrcqucncrcrators.
Rcors oI scxuaI alusc havc sIyrocIccd ovcr
hcasIcvdccadcs.Jhcaluscnayoccuraanyagc.
Rclafivcs andIanilyacquairanccs accounfIornosf
cascs,noIcsfafionlysfrangcrsisunconnon.InS0%
oIrcorts, fhc vicfins arc girIs, nos arc aluscdly
sfcIafhcrs, Iafhcrs, or ofhcr naIc IaniIy ncnlcrs.
MaIc scxuaI alusc isrolalIyundcr-rccognizcd.

cglccfrcsulfs in norc dcafhs fhan hysicaI and


scxuaIaluscconlincd.Ifisfhcnosfconnoncausc
oIIaiIurc fo hrivcin chiIdhood.
Coninuco alusc occurs ir a Iourfh oI chiIdrcn
rcurncdfofhcir honcs.Jhcnortaliyrac isS%.
R|sk |actors
lusc ano ncgIcci occur af aII socioccononic
lcvcIs lufarcnorcrcvalcnfanongfhcoor. Chil-
drcnvifhscciaInccds [ncnfalrcfardafion,ccrclraI
aIsy, rcnauriy, chronic iIIncss) arc af arficuIar
risI.CarcaIcrsvhohavc fhcnscIvcssulIcrcdalusc,
vho arc aIcohoI or sulsfancc aluscrs, or vho atc
undcr cxucnc sfrcss arc norc IiIcIy fo alusc or
ncglccf.
D|fferent|aI D|agnos|s
Mosf cascs oI susccfco alusc arc sulscqucntIy
sulsanfiacd ly chiId roccfivc scrviccs. Cufs and
lruiscsarcnorcIiIcIy fo rcrcscnfalusciIIounoin
lovrauna arcas, such asfhclufocIs orlacI. Carc
shouIo lc faIcn fo dilIcrcnfiafc lruiscs Iron
MongoIian sofs, vhich connonIy occur in hcsc
arcas. Ftacturcs fha occur lclorc anluIatior arc
usuaIIy inIicfcd. CccasionaIly, osfcogcncsis incr-
Iccfa has lccn nisfaIcn lor alusc. Skin condifions
such asluIIous incigo nayninic cigarcfclurns
or ohcr Iorns oIalusc.
CI|n|ca| Man|festat|ons
M5fOly
ninjuryhatisinconsistent vith the stated history
couIcdvifhdeIay in ohtaining apptopriate medicaI
caIc strongIy suggcsfs alusc. gc-inaroriac
scxuaI lchavior and knovIcdgc arc consisfcnf vith
scxuaIalusc.\icinsoIhysicaIorscxuaIaluscnay
< not )or > nc rn njoa i >
14 || uc
[
rn!O Catcs
< cx v )caanr-rnncjcun: nvcm o- jon-. jy>
acoulyalusingofhcrs,acnfingsuicidc,running
avay,orengaginginhigh-risIlchaviors.luscIaccs
children a anincrcascdrisIIor oor schooI crIor-
nance, Iov scII-csccn, and dcression.
Dy5CD LXDHDDfOD
svifhlurns,fhcIocafionandaernoIinjurynay
srongIysuggcsalusc[Figurc2-J). Bruiscs,Iracfurcs,
or Iacerafions in diIlcrcnf sagcs oIhcaIing occur in
chronic or rccacd alusc. 5pira| hactures in young
chiIdren arc virtuaIIy diagnosfic, ril and sIuII Irac-
urcs IrequcnIyrcsuIfIron aluse asvcII.\|gorous
shaIingnay Icado acccIcrafion/deccIcrafion injury,
incIuding sulduraI hcnorrhagc. |ighy-Ivc crccnf
oIinlanfs vhohavclccnshaIcnviII havcrcfinaIor
vircoushcnorrhagcs,orlofh. IcrnancnfvisionIoss
nayresuIIiIfhcsc hcnorrhagcs are IcItunrcafcd.
D|agnost|c cva| uat|on
sIeIea| survcy and lonc scan rcvcal arcas oI
as injury ha nay no lc cviden on hysicaI
cxaninafion.ChiIorcnvifhcxfcnsivelruisingshouIo
undcrgocoaguIafionsudics o ruIeouhcnaoIogic
alnornaIifics.VhcnscxuaIaluscissuscccd,rccaI,
oraI, vaginaI, and urchraI sccincns shouId lc
LOOjCO
ClCc!|lc3l cO|O
\O3I |3|QC|
LClI
P
#
1
t
1 f
* #
m w
||IC
l3l|O|u5|
Ilc|O||OjC
l3|O

L
L
w ~
d
Luc|lC
|l5I
F|gure2-3 * BOdymarkscOnslstCntwithabusC.
cxanined Ior leissero gonotrboeoe, Cb/odio tr-
cbomotis, and ofhcr scxualIy ransnicd discascs.
Chcr sudics incIudc lIood csfs Ior syhiIis and
hunaninnunodcIcicncyvirus.
reatment
IcaIfh carc vorIcrs arc required by Iav fo rcorf
any susicion oI chiId alusc or ncgIcc fo safc
rofccfion agcncics. \icfins shouId lc inncdiafcly
rcnovcdIronfhcir honcs andlaced in rofccivc
cusody a a hosiaI or a sfafc IaciIiy. Many IaniIy
infcrvcnion rograns fha Iocus on sociaI suort
and arenfing sIilIs arc lcing cvaIuacd across hc
counfry inanafcnorovidcchiIdrcnvifhsalcr
honc cnvironncns.
SUDDN |N0N D0H
SYNDRDM
BydcInifion, suddcninIanfdcafh syndronc [SIIS)
consisfs oI fhc uncxccfcd dcafh oI an inIanf Ior
vhich he cfioIogy rcnains unclcar dcsic a hor-
ough hisfory andosnorfcncvaIuafion.Jhc causc
oI SIIS rcnains unlnovn lu is fhough o lc
rcIacd o dcIaycd naurafion oIlrainsfcn rcsira-
oryconroI and arousaI ncchanisns.
R|sk |actors
lfhoughnuIfiIcIacors havclccnassociafcdvifh
an increased risI Ior SIIS, nonc has rovcn rog
nosfic vaIuc [JalIc 2-J). Morc cases arc rcortcd
duringfhevinfcrnonfhs.Irican-ncrican inIanfs
arc fvicc as IiIcIy [and ncrican !ndians fhrcc
fincs as IiIcly) o dic oI SIIS fhan fhc gcncraI
ouIaion.
' 1PLL Z5
bOb. HSk COfS
|rOnCs|eCplng pcsltlOn
|rematur|ty
Ppnea
MaternaIsmOklng
PgC2mO
IntrautCrlneOrugexpOsure
UChc|Cnt prenata|carC
LOwsOcOeCOnOmlcstatus
|Crlnata|asphyxla
< not tor zac i > < nc rn noa i >
< o M c

oncn o-

on )v>
=
1 5
_
aptCr |CGU|G ar| |]U|y |CVCG1|C|
D|herent|aI D|agnos|s
Cascs that initiaIIy acar to lc SIIS nay ir Iact
rcsultIron inIcction,congcniaIhcaridiscasc, ncta-
loIic disordcrs, accidcntaI trauna, or alusc.
Prevent|on
Sincc hc "BacI Jo SIcc" canaign vas initiatcd
lyhcMationaIInstitucsoIIcaIth,hcincidcnccoI
SIIShasdccrcascdly4J%. !nIantsshouIdlclaccd
on thcir lacIs vhiIc sIccing. Contrary o oular
lcIic[honcancanonitorsdonotdccrcaschcliIc-
IihoodoISIIS.
hN LW
1. OQCtCr,acCtUCn!SnU tn|urtCS |C!CmOS!
COmmOn cuSCOI pCUttrlC mOrblUlty arO
mO|!IIty.
2. |OtsOrlnQtS uSuI|y cctOCnlI |n tOUUICtSanU
lntCn!IOnaItn UO|CScCntS.
d. LCaO pOtsOnlnQ p|CCS ctIO allQ rlsklO|
OCVCIOpmCnlaIUC|y anObCvlOrprCb|CmS.
4. C|OutInCuSCOl sCa!bCItSnU C|SCtSlS
QIyCl!CcttvC ln rCOuCInQ tCInCUCncCCl
sCvCrCtn]utyanU UCt.
b. CrC ls nO CvIUCncCtt OtlvCr COucatOn prO-
Qrams rC an Cl!CctIVCUCtCrrCnttO acClOCntS
InvOIvtnQ!CCnaQCUrlvCrs.
. LCrtalnpattCrnSOl ln|uryOrburnSsuQQCst abuSC.
. bblCs sOu|U bCputtO bCU OntClrbCkS.
. HOmC apnC mCnltO|S OC nOtUCcrCasClCIlkC|l-
OOU Cl b|b.
V0LU0|DN D Hc
CY0NDT|C Nc DN0Tc
Cyanosis is a hysicaI sign characcrizcd ly lIuc
nucous ncnlrancs, naiI lcds, ard skin. Cyanosis
rcsuIs Iron an alsoIuc conccnraion oI dcoxy-
gcnacdhcnogIolinoIaIcasf J. 0g/dI.Facorsha
inhucncc vhchcr cyanosis viII appcar inc|udc hc
hcnaocri,vhichrcIccshcalsoIucconccnraion
oI hcnogIolin, and he Iacors ha aIcc hc C,
dissociaion curvc [I, )tt cncraurc, IcvcI oI
2, J-dihoshogIyccrac, and raio oI aduI o IcaI
hcnogIolin) . Cyanosis shouId nolcconIuscdvih
acrocyanosis, vhich is lIucncss oI hc cxrcniics
duc o crihcraI vasoconsricion nocd in hc Irs
24 o 4S hours oI IiIc. lconacs vih acrocyanosis
havc inknucosaIncnlrancs.
D|fferent|aI D|agnos|s
JhccauscsoIcyanosisinhcncvlornarcoIcardiac,
uInonary, ncuroIogic, or hcnaoIogic origin. Jhc
incidcncc oI srucuraI hcar discasc is alou S in
I 000Iivc lirths, and scvcrc congcniaI hcart discasc
occurs in aroxinacIy in 400 Iivc lirhs. IuI-
nonarydisordcrs nay Icad o cyanosis as a rcsuI oI
rinaryIungdiscasc,airvayolsrucion,orcxrinsic
conrcssion oI hc Iung. lcuroIogic causcs oI
cyanosis inc|udc ccnraI ncrvous syscn dysIuncion
andrcsiraoryncuronuscuIardysIuncion.JalIcJ-
I dcIincacshc causcsoIcyanosisinhc nconac.
CI|n|caI Man|festat|ons
Ml5fOly DD0 Dy5lC0 LXDHlDDflOD
/ conIcc lirh hisory ha incIudcs nacrnaI
hisory, rcnaaI, crinaaI, and osnaaI conlica-
ions, hisory oI Ialor and dcIivcry, and nconaaI
coursc shouId lc olaincd. Fxac!y vhcn hc chiId
dcvcIocd cyanosis is criicaI, lccausc ccrtain con
gcniaI hcart dcIccs rcscn a lirh, vhiIc ohcrs
nayakcasIongasoncnonhorcscnhcnscIvcs.
Jhc iniiaI hysicaI cxaninaion shouId Iocus on
hcviaIsigns and cardiac and rcsiraory cxanina-
tions, looIingIor cvidcnccoIright,lcIf,orlivcntric-
uIarcongcsivchcarIailurc andrcsiraory disrcss.
Bluc or dusky nucous ncnlrancs arc consiscn
vih cyanosis. FvaIuac Ior raIcs, sridor, gruning,
Iaring, rcracions, and cvidcncc oIconsoIidaion or
cIIusion on uInonary cxaninaion. Cn cardiovas-
cular cxaninaion, hc rccordiaI inulsc is aI-
aicd, and hc cIinician shouId cvaIuac Ior sysoIic
ordiasoIic nurnurs, hcincnsiyoI>j , >, sIiing
alnornaIiics,andhcrcscnccoIanzor>, gaIIo,
cjccion cIick opcning sna, or rul. Fxaninaion oI
hc cxrcniics shouId Iocus on hc srcngh and
synncry oI hc uIscs ir hc ucr and Iovcr
cxrcniics, cvidcncc oI cdcna, and cyanosis oIhc
naiI lcds. IcaosIcnoncgaIy nay lc consiscnf
vih righvcnricuIar orlivcnricuIar hcar IaiIurc.
D|agnost|c cvaIuat|on
Jhc goaI oI hc iniiaI cvaIuaion oI hc cyanoic
nconac is o dccrninc vhchcr hc cyanosis is
cardiacornoncardiacinorigin./ncIccrocardiogran
[FCC), chcs radiograh, and hycroxia cs shouId
lc crIorncd. In addiion, rcducfaI andosducaI
oxygcn sauraion, and Iour cxrcniy lIood rcs
surcsshouIdlc docuncncd.
/hycroxiacsshouIdlccarricdouinnconacs
vih arcsinguIsc oxincry rcading Icsshan 9S%,
visilIccyanosis,orcircuIaorycoIIasc.Jhchycroxia
cs consiss oI olaining a lascIinc righ radiaI
* nor tor zac i > < nc rn noa i >
< cx V rcano-onncjcn:
o

_,_:
O
.
I apr ar lL Cgy
#
1 7
" PLL 5~1
Oefent OgnOSS O LDOSS n the NeOnte
La|U|aC
UuCtaI-lnOCpCnOCntmlx|ng|CslOns
1runCusarICrlCsus
1OtaI anOma|Ous puImOnary vCnOusrCturnwlthOutObstruCtlOn
|-transpOsltlOnOIthCgrCatartCrlCs
|CslOnswlthOuCtaI-OepCnOCnt |b
1Ctra|OgyOIa|ICtwlthpuImOnaryatrCsla'
LbstCln'sanOmaIy
Crlt|CaI pu|mOnlC stCnOsls
1rlCusplOvaIvCatrCs|a'wlthnOrma||yrCIatCOgrCatartCrCs'
|u|mOnlC va|vC atrCslawlth |ntaCt vCntrlCuIarsCptum
HetCrOtaxy
|CslOns wlth OuCtaI-OCpCndCnt5bF
HypOp|astlCICIIhCartsynOrOmC
IntCrrupteOaCrtlC arCh
LrltlCaI COarCtatlOn OIthCaOrta
CrltlCaIaOrtlCstCnOsls
1rlCusplOvaIvCatrCslawlthtranspOsltlOnOIthCgrCatartCrlCs'
ulmona|_
IlOOIy /UOg Ol5O5 suChas rCsplratOryOlstrCss
synOrOmC,meCOnlumasplratlOn,pnCumOnla,
OrpCrslstCnt pu|mOnary hypCrtCnslOnOIthC
nCwbOrn
PlIWOy OD5fIUCflOO suChasChOanaIatrCsla,vOCaI
COrO paraIysls,OrIaryngOtraCheOmaIaCla
XfIlOSlC COOIS5lOO O f /UOgS suCh as
pnCumCthOrax,Chy|OthOrax,OrhemOthOrax
Neu|ologC
LN5 Oy5UOCflOO suChasOrug-lnOuCCOOCprCsslOn
OIrCsplratOryOr|vC,pOstasphyxlaICCrCbraI
OysIunCtlOn,OrCCntraI apnCa
hC5lHfOly OUIOOU5CU/Ol Oy5UOCflOO suChas
splnaImusCuIaratrOphy,lnIantbOtu|lsm,Or
nCOnataI myasthCnla gravls
HematologC
MCthCmOg|OblnCmlaOrpOIyCyIhCmla
A paIenI ducIus arIercsus may |mprcve m|ng, especa||y wIh an |nIacI ventr|cu|ar septum.
"Mcst lcrms.
|B|, pu|mcnary b|ccd llcw; SBF, sysIem|c b|ccd !Icw.
M PLL 5~Z
nteffettOn O the HefOX 1eSt
aO_ (mm Hg) at O_ .Z1 aO_ (mm Hg) at |O_ " 1 . aLO_
(%baturaton) (%batu|aton) (mmH@)
NOrmaI 70(95) >300( J 00) 35
|u|mOnaryOlsCasC 50(85) >J 50( J 00) 50
NCurOIOglC OlsCasC 50(85) >J 50(J 00) 50
MCthemOgIOb|nemla 70(95) >200(J 00) 35
CarOlaC OlsCasC
|araIIC|C|rCu|atlOn <40( 75) <50( 85) 35
MlxlngwlthrCstrlCtCO |B' <40( 75) <50( 85) 35
MlxlngwlthOutrCstrlCtCO |b' 4060(75-93) <J 50( J 00) 35
L-1rnspcsI|cn c! Ihe great arIenes w|Ih nIacI venIr|cu|ar sepIum, L-IranspcsI|cn c! Ihe greaI arIeres w|th venIr|cu|ar sepIa| de!ecI.
"1rcusp|d aIres|a wIh pu|mcnary stencss cr aIres|a, pu|mcnary atresa cr cr|Ica| pu|mcnry sIencss wIh nIacI venIr|cu|ar sepIum, IeIra|cgy c! |aI|cI,
cr LbsIe|n's ancmaIy.
'1runcus arIer|csus; Icta| ancma|cus pu|mcnary vencus return, sng|e vntrcle, hypp|ast|c IelI heart syndrcme.
|Bl, pu|mcnary b|ccd llcw.
[rcducfaI) artcriaI lIood gas ncasurcncnfvifh fhc
chiIdlrcafhingroonair,Fio,0. 2I , andfhcnrccaf
ing fhc ncasurcncnf vifh fhc chiId insiring 00%
oxygcn,Iio,I .00.InfcrrcfafionoIfhchycroxiafcsf
isdcIincafcdinJalIcJ-2.IaC,grcafcrfhan200nn
Ig on I 00% oxygcn naIcs congcnifaI hcarf discasc
vcry unIiIcly. IaC, Icss fhan l 50nnIg on I 00%
oxygcnsuggcsfsacardiacIcsioncharacfcrizcolycon-
Icfcnixinguitbout rcsfricfcduInonarylloodIov
IaC, lcssfhan50nnIgon I 00%oxygcnindicafcs
a cardiac Icsion vifh araIIcI circuIafion, or a nixing
Icsion uitb rcs

ricfcduInonarylIoodIov.
1

o o > 5 nc rn njora i >


< cx v cxanr-nncjcn: ucm o- qjon-. jy >

JhcIaC, shouId lc ncasurcd dirccf!y via arfcriaI
uncfurc, fhougI rocrIy acquircd franscufancous
oxygcn nonifor [JCCM) vaIucs Ior IaC, arc aIso
acccfalIc. PuIse oimetry shouId not be used tor
interpretation ot the hyperoia test, lecausc a
nconafc givcn I 00v insired oxygcn nay havc a
IaC, oI80nnIgvifh a uIsc oxincfcr rcading oI
I 00v [alnornaI),oraIaCgrcafcrfhanJ00nnIg
vifh a uIsc oxincfcrrcading oII 00v [nornaI) . II
a cardiaccauseisdccncdIiIcIy, olfainancchocar-
diogran and a cardioIogy consuIfafion.
IuIsc oxincfry shouId lc docuncnfed af rc-
ducfaI and osfducfaI sifcs fo asscss Ior diIcrcnfiaI
or rcvcrsc diIlcrcnfiaI cyanosis. II fhc rcducfaI
safurafion is highcr fhan fhc osfducfaI safura-
fion, diIlcrcnfiaI cyanosiscxisfs,vhich rcsuIfs vhcn
fhcrc arc nornaIIy rcIafed grcaf arferics and
dcoxygcnafcd lIood Iron fhc uInonary circuIa-
fion cnfcrs fhc dcsccnding aorfa fhrough a afcnf
ducfus arfcriosus. IiIlcrcnfiaI cyanosis |s sccn in
crsisfcnf uInonary hycrfcnsion oI the ncvlorn
[IIIl) and in Icsions vifh IcIt vcntricuIar oufIov
fracf olsfrucfion such as |nfcrrufcd aorfic arch,
crifical coarcfafion oI fhc aorfa, and crificaI aorfic
sfcnosis.
In rarc cases oI rcvcrsc diIcrcnfiaI cyanosis, fhc
osfducfaIsafurafionishigherfhanfhcrcducfaIsaf-
urafion. Jhis occurs onIy in chiIdren vith transposi-
tion ot the great arteries vifhIcItvcnfricuIaroufIov
olsfrucfion [i.c., crificaI coarcfafion oI fhc aorfa,
intcrrufcd aorfic arch, crificaI aorfic sfenosis) or
IIIl. Cxygenafcd lIood Iron fhc uInonary cir-
cuIafioncnfcrsfhcdcsccndingaortafhroughaafcnf
ducfusarfcriosus.
Vhcn cifher thc hycroxia fcsforfhc rcducfaI/
osfducfaI oxygcn safurafion ncasurcrcnf, orlofh,
indicafc cardiac discasc, fhe chcsf radiograh and
FCCnay lc uscdfo dcIineafc vhich cardiac sfruc-
uraI dcIccfis fhc nosfIiIcIy.Jhc chcsf radiograh
is olfaincd fo dcfcrninc thc sizc oI fhc hcarf and
vhcfhcr fhc uInonary vascuIarify is incrcascd or
dccrcascd. Jhc ECC cvaIuafcs fhc bcarf rafc,
rhyfhn, axis, infcrvaIs, R-vavc rogrcssion, and I-
vavc and SJ/J vavc norhoIogy and heIs dcfcr-
ninc |I ischenia, afriaI diIafation, or vcnfricuIar
hycrfrohy is rcscnf.
Jo diIlcrcnfiafc anong cyanofic congcnifaI bcart
dcIccfs fhafrcscnfvifha IaC, Icss fhan 50nnIg
on fhc hyeroxia fcsf, fhc cIinician shouId Irsf
cxaninc fhc chcsf radiograh. II nassivc cardiac
enIargcncnfisnofcd, |lsfein'sanonaIy is fhc nosf
IiIcIy diagnosis. Cncc nassivc cardiac cnIargcncnf
bas lccn ruIcd ouf, fhc uInonary vascuIariiy
leconcs fhc Iocus. IncrcascduInonarylIoodIov
suggcsfs fhcrescnccoIl-fransosifionoIfhc grcaf
artcrics [l-JC/) vifh infacf vcnfricuIar scfun,
vhcrcas uInonary cdcna is a oaniIcsfafion oI
totaI anonaIous uInonary vcnous rcfurn vifh
olsfrucfion.
Jhc rcnaining diagnoscs [fricusid afresia vifh
nornaIIyrcIafcdgrcafarfcrics,uInonic afresia vifh
infacfvcnfricuIarscfun, crificaI uInonicsfcnosis,
and fcfraIogy oI FalIof vifh or vifhouf uInonary
afrcsia) aIIroducc dccrcascduInonaryvascuIarify
andnornaIoronIysIighfIycnIargcdhcarisizc.Jhcsc
deIccfs arc diIlcrcnfiafcd ly fhcir axis on FCC
andfhc rcscnceoralscncc oIa nurnur.Jricusid
afrcsiavifhuInonarysfcnosisoruInonaryafrcsia
|s nofcd Ior ifs sucrior axis, Iying in fhc 270- fo
0-dcgrcc quadranf. CrificaI puInonic sfcnosis and
uInonary afresia vifh infacf vcnfricuIar scfun
lofhhavcaxcsirfhc0- fo90-dcgrccquadranf.Jhcy
arcdiIcrcnfiatedlyfhcrcsenccoIfhcIoudsystoIic
cjccfionnurnurhcardIroncrificaIuInonicsfcno
sis.SiniIarIy,fcfraIogyoIFaIIofandfcfraIogyoIFaIIof
vifh uInonary afrcsialofh havc axcsinfhc 90- fo
I 80-dcgrcc quadranf, fhcy arc disfinguished Iron
cach ofhcr ly fhc uInonic stcnosis nurnur nofcd
|nfcfraIogyoIFaIlof.
reatment
|evlorns vifh nixmg Icsions vifhouf adcquafc
oixing [l-JC/vifh infacfvenfricuIar scfun and
resfricfivc atenf Iorancn ovaIc) or dcIecfs thaf
havc ducfaI-dccndcnf uInonary lIood Iov
or ducfaI-dcendcnf sysfcnic lIood Iov nay
requircrosfagIandinFj [ICF,)inIusiontonainfain
afcncy oI fhe ducfus arteriosus unfiI dcInitivc
surgicaI frcatnenf car lc acconlishcd. RarcIy, fhc
aficnf vifh congcnifal hcarf discasc nay lcconc
rogrcssivcIy norc unsfalIc alcr fhc insfifufion oI
ICF, fhcray.Jhis cIinicaI detcriorafion ahcr insfi-
tufionoIICF, |saninorfanfdiagnosficIndingfhaf
|dcnfiIcs fhccongcnifaIhcar:dcIcct asonc fhafhas
olsfrucfcdlIoodIovoufoIfhcuInonaryvcinsor
Iel afriun. Icsions fhafhavc inaircd lIood Iov
Iror fhcIcIt afriun incIude hyoIasfic Iel hcart
syndronc vifh rcsfricfivc or infacf loraner ovaIc,
ofhcr variarfs oI nifraI afrcsia vifh resfricfivc
IorancnovaIc,fransosifionoIfhcgrcafarfericsvifh
aninfacfvcnfricuIarscfun andrcsiricfivcIorancn
ovaIc,andtofaIanonaIousuInonaryvcnousrcfurn
vifh olsfrucfion.
< not tor zac i > < nc rn noa i >
< cx M cxanr-ronncjcn:
h
o

U` | V r r lL CQy 1
hN PC|NT5

1 . C absO|utC cOncCnt|ltCn CI UCOxyQCntCU


hCmOQ|Ob|n,anc nOt tC|ttCCICxyQCnlCO tC
UCOxyQCntCOCmOQ|Obtn,OCtCrm|nCs lCp|Cs-
CnCC CCynCss.
2. LncCcyanCs|shsbCCntUCnttDCU,stb|||ZClhC
|nInt,qutck|ytn|lttClCp|C|tmtn|ywOrkup
(CCstrUtOQ|p,C|CctrCCrU|CQrm,anU ypCr-
Cxt tCst) anO OCDnC wCthCr thC |CstCn |scrUtac
C|nCncarU|ac |n CrQtn.
d. |I ca|UtC |CstCn s suspCClCU,UCtCrmtnCwCtCr
thC OCICct tsOuct|UCpCnUCnlICrsyslCmtcOr
pu|mOn|yctrCu|ttCn Or OuCt|-|nOCpCnOCnt
m| x|nQ |Cs|Cn.
4. LncCtC |nIan!|sstb|tZCO,Cbltnn CmCrQCnl
CrUO|CQy COnsu|tat|On,nCchOC|OtOQram,nU,
tI|nU|ctCO,bCQ|n |L, tCrapy |n p|CprattOn IO|
surQ|c| p|ltCnCr CCrrCcl|Cn.
CY0NDT|C CDNGcN|0L Hc 0RT
D|Sc 0S. DUC0L-|NDPcNDcNT
M|X|NG LS| DNS
runcus 0rter|osus
Jruncusartcriosus[FigurcJ- I ) isararcIornoIcyan-
otic congcnitaI hcart discasethatconsists oIa singIc
artcriaIvcsscI arisingIronthcbaseo|thchcartIron
vhich arisc thc coronary, systcnic, and uInonary
artcries. ln this disordcr, thcrc lsconIctcnixingoI
systenicanduInonaryvcnousbIoodinthctruncus.
Jhis Iesion, aIong vith othcr conotruncaI anonaIics
[tctraIogy oI FaIIot, interrutcd aortic arch, \SI,
isoIated arch anonalics, andvascuIar ring), is associ-
atcdvith nicrodcIction oIchronosonc 2 [2q!
dcIction) .
LDCD NDDE5fDfOD5
Moderatccyanosis lsrcscntatbirth,and congcstivc
hcartIaiIurcdcvcIojsinanattcroIvccIsasthcuI
nonary vascuIar rcsistancc IalIs and shunting across
thcventricuIarsctaIdcIcctbcgins. Cncxanination,
asystoIicc]cctionnurnurishcardatthcIcItstcrnaI
lorder, a vidcncd uIsc rcssurc is rcscnt, and
bounding artcriaI uIscs arc alatcd. Jhcrc is a
singIe Ioud sccond hcart sound or cardiovascuIar
cxan.ScvcntycrccntoIchiIdrenvithtruncusaric-
Ilgre d-1 1rUncUs r!erCsus (v!h rgh! CrIc rch).1ypc|
ntCmc nO hemOOynmDL hnOngs nC|UOe: () sng|e
r!eryrses!rCm!hecCnC!rUnCusgvngrse!CCCrCnryr!er-
es |nC! shCvn), pU|mCnry r!eres, nO Cr!c vesse|s, |b)
bnCrm| !runc| v|ve |quOrcuspO shCvn) v!h s!enCs|s
nO/Cr regurg!!Cn s cCmmCn, |C) rgh!-sOeO Cr!C rch
|CCCUrs n pprCxm!e|y J0' C! Cses), |O) |rge cCn-
Cven!riCU|r ven!rcUlr sep!| Oe!ec!, |e) pu|mCnry r!ery
hyper!ensCn wth |rge |e!I-!C-rghI shUn! |nC!e superCr
ven cv| Cxygen s!Ur!Cn C! 60' nO pU|mCnry r!ery
Cxygen s!ur!Cn C 85'), |!) cCmp| e!e mxng |C! Ihe
sys!emC nO pU|mCnry venCUs re!Urn) Cccurs ! Ihe gre!
vesse| |eve|,
L|oherty !P 51ark AH. Nanua| ol Neona1aI Lare, 4" e. Ft|aUelphta.
LppnCo!\haven, 1 . V.
riosus havc bivcntricuIar hycrtrohy on FCC Cn
chcst radiograh, narIcd cardioncgaIy, incrcascd
uInonary vascuIarity, and rightaorticarch nay bc
sccn. LiCcorgc's syndronc rcIated to thc 22qI I
nicrodelction nay rcsuIf ir hyocaIccnia.
lEDfHEDf
/t nost ccntcrs nconataI surgicaI rcair is cr-
Iorncd. SurgicaI rcair invoIvcs cIosing thc vcntric-
uIarsetaIdcIcctsothcoxygcnatedbIoodinthcIcIt
vcntricIc is baIlcd through thc \SI to thc truncaI
vaIvc and a conduit is intcroscd lctvccn thc right
vcniricIc arduInonary artcrics, vhich arc discon-
ncctcd Iron thc truncaIvcsscI.
Z
o ;or > < nc rn noa i >
< cx v )caanr-rnncjcun: nvcm o- jon-. jy >
ucrnIO e !Cs
Ilgure d-Z 1rnspCs|!Cn C!!hegre! ner|es v!h n n!C!
ven!rCU|r sep!um, |rge p!en! OUC!us r!er|CsUs (Cn |GL,)
nO!r|sep!|Oe!eC!(s!!Us pCs!b||CCn!r| sep!Cs!Cmy).
NCIe!he!Cl|Cv|ng.()1heCr! rsesrCm!hen!CmCrgh!
ven!rC|e,nO!hepU|mCnryr!ery!rCm!hen!CmC|e!|ven-
!rC|e,(b)"!rnspCs!iCn physC|Cgy,"v!h h gherCxygens!-
Ur!Cn n !he pU|mCnryr!ery!hn n !he Cr!, (C)"mxng"
be!veen !he pr||e| CrCU|!Cns (see !ex!) I !he !r|
(\erb||CCn!r|sep!Cs!Cmy) nO OUCIl|eve|s,(O)shun!|ng
rCm !he |e! !rUm!C !he righ!!rUm v !he Ir| sep!|
Oe!eC! (nC! shCvn) v|!h equ| zICn C! !r|| pressUres, (e)
shun!|ngrCm!heCr!!C!hepU|mCnryr!eryvi!heOUC!us
rIer|Csus, () pu|mCnry hyper!ensCn OUe !C |rge OuC!us
rIerCsUs.
L|ohe|\y 1|, 5ta|k Ah. NanUa| o Neonata| L|e. 4' ed. |h||ade|ph|a:
L|ppnCott-|aven, ''. V.
Lranspos|t|on of the Great 0rter|es
l-JransosiLion oI Lhe grcaL arteries [Figurc J-2)
accounLsIor5voIcongcniLalhcartdcIccLsandisLhc
nost connon Iorn oI cyanotic congcniLaI heart
discase rcscnting in thc nconataI criod. In Lhis
dcIccL,thcaorLaariscsanLcriorIyIron LhcrighLvcn-
tricIc, and thc ulnonary artcry riscs ostcriorIy
IronthcIcItvcnLricIc.JhcrcarcLhrccbasicvariants.
l-JC/ viLh inLact vcntricuIar seLun [60v), l-
JC/ viLh vcnLricuIar scLaI dcIecL [20v) , and l-
JC/ viLh vcntricuIar scLaI dcIect and uInonic
sLcnosis [20v) .
InLhisdcIccL,LhcuInonaryandsysLcniccircuiLs
arcaraIIeIraLhcrLhaninscrics.JhesysLcniccircuiL
[dcoxygenatcd bIood) ls rccircuIated Lhrough Lhc
lody, vhercas Lhc uInonary circuiL [oxygcnatcd
lIood) recircuIaLcs Lhrough Lhc Iungs. / nixing
Icsion such as an atriaI scLaI deIect, vcnLricuIar
scLaI deIcct, and/or atcnL ducLus arLcriosus LhaL
aIIovs nixing oILhc sysLcnic anduInonary circu-
IaLions is ncccssary Ior survivaI.
LDCD NDDE5fDfOD5
Cyanosis is rcscnI Iron lirLh, the dcgrcc varying
vith the associatcdnixing Icsions. In thc abscnccoI
nxing Iesions, thcrc is ronounccd cyanosis, right
vcntricuIar hcavc, and a singIc Ioud S, on cxanina-
Lion.JhercscnccoIasystoIicnurnurindicatcsLhc
rcscnceoIa\SIoruInonicsLcnosis.Jhc FCC is
nornalinLhcncvlorn,hovcvcr,right-axisdcviaLion
and right vcntricuIar hycrtrohy arc cvcntuaIly
sccn. Jhe chcsL radiograh rcvcaIs incrcascd uI-
nonaryvascuIarnarkingsinl-transosiLionviLhor
viLhout vcnLricuIar sctaI dcIcct, buL iI uInonic
stcnosis is criticaI, dccrcascd uInonary vascuIar
narkings nay bc rcscnt. CardionegaIy vith cgg-
shacdsiIhoucLtc"isoItcnsccnonchesLradiograh.
lEDfHEDf
IniLiaI nanagcncnt nay incIudc ICF, Lo Icc the
aLcnf ductus arLcriosus ocn and incrcasc aorta
[dcoxygenaLcd) Lo uInonary artery [oxygcnaLcd)
shunLing. IInccdcd,Lhc Rashkind laIloon aLriaI sc-
tosLonycanbcuLiIizcdLoinrovcatriaInixingand
rcIicvc sevcrchyoxia. SurgicaI rcair, utiIizing Lhc
arLcriaI svitch roccdurc, is gcneraIIy crIorncd
during theIrsLvccIoIliIc.
otaI 0nomaIous PuImonary
Venous Connect|on
JoLal anonaIous uInonary vcnous connccLion
[J/I\ [Figurc J-J) |s a rarc Icsion in vhich thc
uInonary vcnous rcturn is dirccLed Lo Lhc righf
atriun eithcr dircct!y or indircct!y Lhrough vcnous
channcIs.Jhcrc arc Iour varianfs
5upracardiac [50v oI cascs). BIood drains via a
vcrLicaI vcin into Lhc innoninaLe vcin or into Lhc
sucriorvcnacava
Cardiac [20voIcascs) .BIooddrainsinLoLhccoro-
narysinus or dircctIy intoLhcrighLaLriun
< not tor zaci > < nc rn njoa i >
IIgure d-d | n!rOiphrgm!c !C!| nCn|Cus pu| mCnry
venCus cCnnec!iCn.NC!e!he!C||Cvng:|) pu| nCnryvenCus
cCn!'uenceOCesnC!cCnneC!v!h!he|e!!r un, bu!OescenOs
!C cCnneC! v!h !he pCr!| crcu|!Cn be|Cv !he Ophrgm.
1hscCnneC!Cns!requen!|ysevere|yCbs!ruc!eO sshCvn,|b)
Cbs!ruc!Cn!Cpu|mCnryvenCusre!urnresu|!snsgncn!|y
e|ev!eO pu|mCnry venCus pressures, OeCreseO pu| mCnry
b|CCO !'Cv,pu|mCnryeOemnO pu| mCnryvenCusOes!u
r!Cn[92'),|c)sys!emc!Csuprsys!emlcpressuren!hepu|
mCnryr!ery |n !hebsenCeC p!en! Ouc!us r!erCsus,
pu| nCnry r!ery pressures my exceeO sys!emc pressures
vhen severepu| mCnry venCus Cbs!ruc!Cn s presen!),|O) | |
sys!encb|CCO!'Cvmus!beOerveOv| rgh!-!C-| e!!shun!!
!he !Crmen Cv| e,|e) ner|y equ| Cxygen s!ur!Cns n | |
ChmbersC!!heher!|e., cCmp|e!emxing!rgh!!r||eve|),
v!h severe hypCxem |sys!em|cCxygen s!ur!Cn 60') nO
|CvcrOcCu!pu!|mxeOvenCusCxygen s!ur!Cn25').
L|ODCf1y 1| b1fK F|. OU| O COO1| LfC, `
h
CO. D|OCjD.
LjjOCO1l|VCO, ''! Z.
lnradiaphragmatic [20v ol cascs). BIood drains
via a vcrficaI vcin infofhcorfaI orhcafic vcins
Nied [I 0v oIcascs) . BIood rcLurnsLo Lhc hcarL
viaa conlination olLhc alovc roufcs
J/I\C can occur viLh or viLhouf olsirucLion.
Clsfrucfion occurs vhcn fhc anonaIous vcin
cnLcrs a vcsscl af an acufc anglc. Jhc rcscncc or
alscncc oI olsfrucfion dctcrnincs vhcLhcr fh:rc
is uInonary vcnous hycrfcnsion and scvcrc
cyanosisorincrcascdulrooary loodlovandn|IJ
cyanosis.
LlDCD NDDE5fDfOD5
ViLhoufolsirucLion, clinicaI Indings arc sinilar io
LhoseolanairialscLaldcIcct.Jhcreisanacfivc prc-
cordiun vifh a righL vcnLricuIar Ieavc, a vide and
Ixcdsl|IS,viLhalouduInonarycononcnL,and
a sysfoIic c]cction nurnur affhclcItucr sfcrnal
lordcr. Cn chcsf radiograh, cardioncgaIy is nofcd
vith incrcascd uInonary vascularity. Cn FCC,
righf-axis dcviafion and righI vcnLr|cuIar hycrLro-
hy arc sccn./ nconaLc viLhJ/I\CviLh olsfruc-
Lion rcscnLs cxLrcncly cyanoLic, fachyncic, and
dysnc|c. Fxaninafion rcvcals a right vcnfricular
hcavc, a narrovly sliL S,, and a vcnLricular galIo
[S,).
lEDfHEDf
ln J/I\C vifhouL olsfrucLion, trcafncnL ol con-
gcsLivc hcartlaiIurc is nccdcd iniLially, and surgical
rcdircct|onoIalcrranL v:sscls info thc IcItafriun is
ncccssary in thc IrsL nonfh ollilc. InJ/I\Cvifh
olsfrucfion, fhc nconafc should le LaIcn io surgcry
cncrgcnLIy|orcorrccfion.ICF, shouldnoLlcgivcn
lccausc fhc afcnf ducfus arLcriosus adds norc
lIood voIunc fo an alrcady loodcd ulnonary
circuiL.
CY0NDT|C CDNGcN|0L
Hc 0RT D|Sc 0Sc. LcS| DNS W|TH
DUCT0L-DcPcNDcNT PULMDN0RY
LDDD |LDW
Tr|cusp|d 0tres|a
Jricusidatrcsiav|fhnorraIIyrclaLcdgrcaLarLcrics
[Figurc J-4) isararcdcIcctthatconsisfsolconIcfc
alscnce ol right afriovcnfricular connccfion, vhich
lcads Lo scvcrc hyoIasia or alscncc oI fhc righf
vcnfricIc.lincfycrccnf olcascs oILricusid atrcsia
havc an associatcd vcnfricuIar scfal dcIcct. !n chiI-
drcn vifh iricusid aircsia vifh nornaIly rclafcd
grcaf arfcrics, fhc vcnfricuIar scfal dcIcci allovs
lloodfo assIronLhcIcILvcnLriclcfofhcrighLv:n-
fricuIar ouflov and uInonary arLcrics. 1hc vasf
na]orifyolaticnfs vifh fricusid afrcsia vifh nor
naIly rcIatcd grcaL arLcrics aIso havc ulnonary
sfcnosis. In Lricusid afrcsia Lhc sysfcnic vcnous
ZZ

* nc J7R njoDa

| |
i >
Ilgure 3-4 1rcuspO!resv!h nCrm||yre|!eO gre!r!er-
es nO sm|| p!en! OuC!us r!erCsus.1ypc| n!CmcnO
hemCOynmcnO|ngsnc|uOe:[)!resC!!he!rcuspOv|ve,
[b)hypCp|s C!!he rgh!ven!rc|e,[c)res!rc!Cn!Cpu|mCnry
b|CCO !'Cv ! IvC |eve|s: [usu||y) sm|| ven!rcu|r sep!|
Oe!ec! nO s!enC!c pu| mCnryv|ve,[O)| | sys!emcvenCus
re!um mus! pss !hrCugh !hep!en!!Crmen Cv|e!C rech
!he|e!\ven!rc|e,[e)cCmp|e!emxng!!he|e!\!r| |eve|,v!h
sys!emC Cxygen s!ur!CnC!78' [n |OC!0:21 ),sugges!ng
b|nceO sys!emcnO pu|mCnry b|CCO!'Cv.
L|ODC|1y ![ b1fK F. OU O COO L|C, `
'
CO. |DOCjD!
LjQ|OCO1VCO, ''. V.
rcfurn is shunfcd Iron fhc righf afriun to Lhc lcl
afriunfhroughfhcafcnfIorancnovaIcoranafriaI
sofaI dcIccf, and Lhc Icl atriun and Icl vontricIc
handIolofhsysfonic anduInonaryvcnous rcfurn.
CxygcnafodanddcoxygonatodlIoodisnixodinLhc
IoItafriun. Cyanosis issovcrcinLhe noonataI criod
and is roorLionaIIy rcIafcd fo Lho anounf oIuI-
nonary llood Iov. In J0v oIcascs, fhoro is frans-
osifion oIfhc grcafarforios,vhichrosuIfs in lIood
assingIronLhoIolvcnfricIofhroughLhc vcnfricu-
larsotaIdcIcctto fhc righfvonfricuIaroufIov and
fhcascondingaorfa.Jricusidafrosia viLhfransosi-
tion oI Lhc groat artcrics is olcn associafcd viLh
coarctafion oI fhc aorfa or aortic arch hyoIasia.
\nIiIc iricusid aLrcsiaviLh nornalIy rcIafod groat
arfcrics iL isa cyanotic IosionvifhducfaIdoondcnf
sysfonic lIood Iov.
LlDlCD NDDlE5fDflOD5
lconafcsviLhfricusidafrcsiaviLhnornaIlyrcIatcd
grcatartcricsrcscnLvifhrogrcssivccyanosis,oor
Iooding, andtachyncaovorfhcIrsf2vooIsoIIiIo.
Cr cardiac oxanination, tho harsh hoIosysfoIic
nurnur oI a vontricuIar sotaI doIoct af Lhc lcIt
Iovor stornaI lordcr and thc continuous nurnur
oI a atcnt ducfus artcriosus nay lc hcard. Cr
FCC, Lhcro is a sucrior axis and Icl vonfricular
hyorfrohy. Findings on chcsf radiograh includo
nornaI hoartsizo and dccrcascd uInonaryvascuIar
narlngs.
lEDfHEDf
/ chiId viLh fricusid afrcsia vifl nornalIy rcIatcd
grcaI arfcricsshouId havo PCF, sfarfodfo nainfain
uInonary Iov, and a laIIoon atriaI sofosLony
should lo crIornod iILho afriaI doIoct is not adc-
quafc. Surgical nanagcncnf Ior fricusid afrcsia
invoIvcsIacing anodiIcdBIaIocI-Jaussig shuntto
naintain uInonary lIood Iov. Jhc nodiIcd
BIaIocI-Jaussig shuni is a Coricx conduit Iaccd
lotvocr fhc sulcIavian arfcry and fhc uInonary
artcry. \IfinafoIy, a cavouInonary anasfonosis
[hcni-Fonfan or lidirccfionaI CIcnn) is crIorncd
fo rovidc sfalIc uInonary lIood Iov. In nosL
ccnfcrs,anodiIcdFonfanroccdurciscrIorncdfo
rcdirccffhcinIcrior vcnacava andhcaficvcir Iov
info fhouInonarycircuIation.
Pu|mon|c 0tres|a w|th |ntact
Ventr|cuIar Septum
PuInonic afrcsia viLh infacf vcnfricuIar scfun
[Figuro J-5)isa rarcdcIcctconsisting oIuInonary
valvuIarandinIundiluIarafrosiaandvaryingdcgrocs
oIrighfvcnfricularandfricusidvalvohyoIasia.In
Lhis disordcr, Lhcrc is an olIigato afriaI shunt Iron
righffoIcIt, anduInonarylloodIovisdccndcnL
on a afont ductus arLcriosus. Sincc fhcrc isno ul
nonary oufIov, fhc right vcntricIc is hycrtcnsivo
andfhcrcisolonnodcrafcfoscvcrcfricusidrcgur-
gifafion. PuInonary afrcsia vith intact vcntricuIar
scfun nay aIso lo associatcd viLh coronary
arforynyocardial sinusoid connunication. Jhc
coronaryarfcrics naylc quifc alnornaI,viLh arcas
oIstonosis or conIcfc afrosia. Insonc casos, coro-
nary crIusior nay lodccndcnf or Lhc hycrtcn-
sivc righI vonfricIo. II Lhc coronarics arc righf
vcnfricIc [R\ doondcnf, any aIIiativc roccduro
fhaI dcconrcssos fho righf vcnfricIc nay Ioad fo
nyocardiaI inIarction anddcafh.
< not tor zaci > < nc rn njoa i >
< cran n cxanr-ronocjcun: n

O | Zd r r | C CQy
|Igu|e db |u|mCnry!res v!h n!c! ven!rcu|rsep!um
||A/|V5) n neCn!ew!h nCnres!rc!ve p!en!Ouc!usr!e-
rCsus vhi|e reCevng |GL,.Typc| an!Cmc nO hemCOy-
nmC nOngs nc|uOe. [) hyper!rCpheO, hypCp|s!c rgh!
ven!rC|e, [b) hypCp|s!c !rcuspO v|ve nO pu|mCnry
nnu| us,[c!!resC!!hepu|mCnryv|vevIh nC n!egrOe
!'Cv, [O) suprsys!emC rgh! ven!rcu|r pressure, [e) pu|-
nCnry b|CCO!'Cvv!hep!en!Ouc!us,[!) rgh!-!C-|e!\shun!
! !he !r| |eve| v!h sys!emc Oes!ur!Cn. Nny p!en!s
hve sgncn! cCrCnrybnCrm|!es v!h snusCO| Crs
!u|Cus CCnneC!Cns !C !he hyper|ensve rgh! ven!rc|e Crsg
ncn!cCrCnrys!enCss|nC!shCvn).
LODCfIy !P bIfK F|. OU| O |COO L|C, 4'" CO. lOC|l.
LljjlOCOI\~VC, ''. V.
LlDCD NDDE5fDfOD5
lconafcs rcscnL af lirfh cxfrencIy cyanofic and
fachyncic. Cardiac cxaninatior reveaIs a tricusid
rcgurgifafiornurnurirflclclIovcrsfcrnallordcr
and fhe confinuous nurnur oI a afcnf ducfus
arLeriosus.Cn FCC,IenventricuIarhyerfrohyand
a IeILvard axis arc seen. Cn cIest radiograh,
dccreascd ulnonary narkings and IcIL vcntricuIar
hyerfrohyarc sccn.
lEDfHEDf
PCF, shouId lc sfarfcd Lo cnsure uInonary lIood
Iov |niLiaIly. Prior fo any surgery fo rovidc norc
sfalIeulnonaryIov,acardiaccafhcfcrizaLionnusf
le erIorned to asscss fhc coronary arLcrics. IIthc
coronary circulaLion is nof R\ deendcnf, Lhcn a
righf venfric|e fo u|nonary arfery conduif or ul
nonaryvaIvoLonyiscrIornedforovidcanfcgradc
uInonary lIood Iov. / nodiIed BlaIocI-1aussig
shunI is aIso fyicaIIy crIorncd Lo augncni uI-
nonarylIoodIovIurLhcr.!ccndingorfhcgrovfh
oIfherighfvcnfricIcandfricusidvalvc, asinglcvcn
fricIc,oncandahaIIvcnfricIc,orfvovcnfricIcrcair
nay le ossilIc. II Lhc coronary circuIation is R\
dccndenf, fhc R\ is nof dcconresscdand a nod
iIedBlalocI-Jaussigshunt |s crIorncd.^ernod
ihed BlalocI-Jaussigshunflacenenf, aticnfsvifh
a righf venfriclc dccndcnt coronary circulafion arc
cithcr Iisfcd Ior hcarf franslanLafion or stagcdLo a
Fontan alIiafion.
Tetra|ogy of |a|Iot
JefraIogy oI FaIlof [Figurc J-6) |s fhe Lhird nosL
rcvaIenfcyaroficcongcnifaIhcarfIesionduringthc
nconafal criod and alcr fhe third vccI oI IiIe
|ccones fhe Icading causc oI cyanosis duc io cor
genitaI hearfdiscasc in childhood.Jhe Iour dcIccfs
FaIIof nofcd incIude an antcrior naIaIignncnt vcn-
tricuIar scfaI dcIccf, right vcnfricuIar ouLIov fracI
olsfrucfion [50v inIundiluIar sfenosis, 20v ul-
nonary vaIvc sLcnosis, and J0v inIundiluIar sfcno-
sis and uInonary vaIvc sfcnosis), right vcnfricuIar
hyerfrohy, and an ovcrriding" largc asccnding
aorfa.
LDCD NDDE5fDfOD5
lconafes vith fctraIogy oI FaIIof arc cyanoiic
lccauseoIrighL-to-IclshunfingacrossLhcvcnfricu
Iar sefaI dcIccf and dccrcased ulnonary Iov.
Shunfing occurs vhcn Lhc conlination oIfhc uI
nonaryvascuIarrcsisfanccandtheresisfancccrcatcd
ly fhe righf vcnfricular oufIov fract olsfrucfion
cxccedfhccrihcraIvascuIarrcsisfancc.Jhcdcgrcc
oIcyanosis isroorfionaItoLlcseverifyoILhcrighf
venfricularoufIovfracf olsfrucfion. Blood shuntcd
Iron fhe aoria io fhc uInonary artery fhroughfhe
atcnf ducfus arfcriosus provides addifionaI uI-
nonary llood lov. lconafcs resenf vifh cyanosis
oIvaryingscvcriLy and nayhavc characfcrisfic cri-
odic eisodic cyanosis and agifation.Jhcsc cisodcs
oI cyanosis arc knovr as "fcf seIIs."Jcf sclIs arc
caused ly an incrcasc in right venfricuIar oufIov
fracf resisfancc, Icading fo an increasc in Lhc righf-
lelshunf. Such scIIs nayIasfninufesLohours,nay
i >
< cran u cxanr-ronncjcun: nvcm o- jon-. jy >
IIgure d- 1e!r|CgyC! |||C!.Typc|n!CmcnO hemCOy-
nmchnOngsnc|uOe:()nnterCr|yOsp|ceO nunObuIr
sepIum,resu|Ingnsubpu|mCnrys!enCss,|rgeven!rcu|r
sep!| Oe!ec! nO CverrOng C! !he Cr! CverIhe muscu|r
sep!um, |b) hypCp|s C! !he pu|mCnry v|ve, mn nO
brnch pu|mCnryr!eres, |c)equ| rghInO |e!! ven!rcu|r
pressures,|O)rgh!-!C| e!\shunI!ven!rcu|r|eve|,v!hsys
\emc Cxygen s!urICnC!82'.
LO|Cf1y 1| bfK F|. OU| O COO| LfC, 4'
'
CO. D|OCjD!
LjjOCO11|VCO, ''. V.
rcsoIvc sonfancousIy, or nay lcad Lo rogrcssivc
hyoxia, acidosis, and dcafh. Cn cardiac cxanina-
fion, arighfvcniricuIarhcavcisolcn |cIfandaIoud
sysfoIic cjccfion nurnur is hcard in Lhc Icl ucr
sfcrnaI lordcr duc fo righf vcnfricuIar oufIovfracf
olsfrucfion.JhcFCCrcvcaIsrighfairiaI diIafionand
righI vcnLricuIar hycrfrohy, vhcrcas Lhc chcsf
radiograh shovs nornaI hcarL sizc vifh dccrcascd
uInonaryvascuIarnarlngs.Jvcnfy-IvccrccnfoI
chiIdrcn viLh fcfraIogy oI FalIoL havc a righf-sidcd
aorficarch.
lEDfHEDf
Jhc frcafncrf oIfcf scIIs is aincd af dininishing
righf-fo-Iclshunfingly incrcasingsysfcnicvascuIar
rcsisfancc and dccrcasing uInonary vascuIar rcsis
fancc. Tcf scIIs naylc frcafcd viLh suIcncnfaI
oxygcn,vagaInancuvcrs,norhincsuIIafc,vasocon-
sfricfors, lcfa-lIocIcrs, and voIunc adninisfrafion.
IoIding fhe inIanf ovcr fhc shouIdcr and Iacing
Lhc chiId in a kncc-chcsf osifion dccrcascs rcIoad
andincrcascs sysfcnicvascuIarrcsisfancc.Morhinc
suIIafc surcsscs fhc rcsiraLory ccnfcr, sfos
hycrnca, and diIafcsLhcuInonaryaricrics.\aso
consfricfors raisc fhc sysfcnic vascuIar rcsisfancc,
vhcrcas lcfa-lIocIcrs arc fhoughf fo nininizc
inIundiluIarsasn.\oIuncisaddcdfoincrcasc fhc
sysfcnic lIood rcssurc, vhich niinizcs righf-fo-
Icl shunfing. McfaloIic acidosis nusf lc corrccfcd,
lccausc if incrcascs uInonary vascuIar rcsisfancc
and fhcrcly ronofcs righf-fo-Icn shunfing across
fhc vcnfricuIar scfaI dclccf. ln nosf insfifufions,
surgicaI rcair is crIorncd during fhc Irsf J fo 6
nonfhsolIilc,oralcrLhcIrsfhycrcyanoLiccisodc
[fcf scII).
cbste|n's 0noma|y
Flsfcin's anonaIy [FigurcJ-7) is an cxfrcncIy rarc
anonaIy in vhich fhc scfaI Icalcf oIfhc fricusid
vaIvcisdisIaccdinfofhcrighfvcnfricuIarcaviLyand
fhc anfcriorIcaIcf oIfhc fricusid vaIvc is saiI-IiIc
and rcdundarf.Jhis rcsuIfs ir a orfion oIfhc righf
vcnfricIc lcing incororafcd info fhc righf afriun.
FuncLionaI hyoIasia oIfhc righf vcnfricIc rcsuIfs,
asvcII asiricusidrcgurgiLaLionorsLcnosis orloLh.
AafcnfIorancnovaIcisrcscnfinS0voInconaLcs
vifh fhc anonaIy, and Lhcrc is a righf-fo-Icl shunf
af thc afriaI IcvcI. Jhc righf afriun is nassivcIy
diIafcd, vhich nayrcsuIf in suravcnfricuIar fachy
cardia. \oIIl-Parkinson-Vhifc [\P\) syndronc is
associafcdvifh Flsfcin's anonaIy. lnscvcrc cascs oI
Flsfcin's anonaIy, Lhc najorify oI fhc uInonary
lIoodIov concs Iron Lhc aLcnLducius arLcriosus
andnoffhcrighfvcnfricIc.
LDCD NDDE5fDfOD5
lconafcsvifhfhcscvcrcIorn oIfhcdiscascrcscnf
vifh cyanosis and congcsfivc hcari IaiIurc ir fhc
IrsfIcvdaysoIIiIc.JhccardiaccxaninafionrcvcaIs
a vidcIy Ixcd sIif >,, and a fricusid rcgurgifanf
nurnurishcardafLhcIclIovcrsfcrnaIlordcr.Jhc
FCC rcvcaIs a righf lundlcranchlIocIviLh righf
afriaI cnIargcncnf. IcIfa vavcs duc fo \P\ syn-
dronc and suravcnfricuIar Lachycardia nay nani
Icst fhcnscIvcs. Chcsf radiograh rcvcaIs cxfrcnc
cardioncgaIy vifh nofalIc righf afriaI cnIargcncnf
anddccrcascduInonaryvascuIarnarIings.
< not tor zaci > < nc rn njoa i >
< cran u cxanr-ronncjcun: nvcm o- jon-. JV >
IIgure 3-1 Lbs!e| n'snCm|y[v|!h |rgenCnres!r|c!|veOuc!us
r!er|Csus). 1yp|c| n!Cn|c nO hemCOynm|c hnO|ngs
|nc| uOe:[)| n!er|CrO|sp|cemen!C!Ihe!r|cusp|Ov|ve| n!C!he
r|gh!ven!r|c|e,vh|ch my|sCCusesubpu| nCnry Cbs!ruc
I|Cn, [b) O| n| nu!|ve muscu|r r|gh! ven!r|c|e, [c) mrkeO
en|rgemen! C! !he r|ghI!r|um OueIC"!r|| |zeO"pCrIlCn C!
r|gh!ven!r|c|esve||s!r|Cusp|Oregurg|!!|Cn,[O) r|gh!!C|e!|
shun!|ng ! Ihe !r|| |eve| [nC!e r!er|| Cxygen s!ur\|Cn C!
78'),[e)|e!!!Cr|gh!shunInOpu|mCnryhyperIens|Cnsec-
CnOry!C |rge p!en! Ouc!us rIer|Csus supp|y|ng Ihe pu|
mCnry b|CCO !'Cv, [!) |Cv crO|C Cu!puI [nC!e | Cv n|xeO
venCus Cxygen s!urI|Cn |n Ihesuper|CrvenCv).
LO|C|1y ![ b1fK F|. OU O COO L|C, '
!
CO. |OC|jD.
LQjOCO1|VCO, ''. V.
lEDfHEDf
PCF, nayhcIincrcasculnonarylIoodIov.Con-
gcsfivohoarfIailurcnayloLrcafcdviLhdigoxinand
diurcfics. ProranoIol nay lc uscd fo surcss
suravcnfricular fachycardia iI rcscnf. SurgicaI
Lhcray Lo rcair fhc alnornal fricusio valvc has
hadoorrcsulfs.
CY0NDT|C CDNGcN|T0L Hc 0RT
D|Sc 0Sc: LcS|DNS W|TH DUCT0L-
DcPcNDcNT SYSTcM|C LDDD LDW
m m m & m a . m m m m m m m m m m m m m m mm m m m m m m m m m m m m m
Hypop|ast|c Left Heart Syndrome
IyoIasficIcILhcarfsyndronc [IlIS) [FigurcsJ-
S and J-9) i sfhc sccond nosf connon congcnifal
C|p!er3 / CrU| C|OQy Zb
IIgure 3- HypCp|sI|c|e!!herI synOrCne |n 24hCur-C|d
p!|en!v|!h !| | | ng pu| mCnryvscu|rres|s!ncenO nCn
resIr|C!|ve OucIus r!er|Csus. Typ|C| nICm|c nO henCOy-
nm|C hnO|ngs | nc|uOe. [) !res| Cr hypCp|s| C! !he | e!\
ven!r|c|e, m|!r| nO CrI|c v|ves, [b) O| m| nuI|ve scenO|ng
CrI nO Irnsverse Cr!|C rCh, usu||y v|!h n ssCC| !eO
cCrc!!|Cn,[c) cCrCnryb|CCO !'Cv |s usu||yre!rCgrOe!rCn
!heOuc!usr!er|Csus!hrCugh!he!|nyscenO|ngCrI,[O)sys
!em|Cr!er||Cxygens!ur!|Cn[|n||OC!0.21 ) C!80V,re!'ecI
| ng re|!|ve|y b|nceO sys!em|c nO pu|mCnry b|CCd
!'Cvs-!he pu|mCnryrIery nO Cr!|CsIur!|Cnsre equ|
[see !ex!), |e) pu|nCnry hyper!ens|Cn seCCnOry!CIhenCn
resIr|cI|veOuc!usrIer|Csus,[!)n| n|m| |e!|Ir||hyper!ens|Cn,
[g) nCrm| sysIen|C crO|c CuIpuI [nCIe super|Crven Cv
Cxygen s!ur!|CnC!65V) nO b|CCO pressure[65/45).
L|ODC|1y J|, bfK F|. OU O COO| LfC, '
'
CO. |OCj|.
LjjOCO1l|VCO, ''. V.
cardiacIcsionrcscnLingir LhcIrsLvccIoIIiIc and
fhc nosf connon causc oI dcafh Iron congcniLaI
hcarf discasc in fhc Irsf nonfh oIliIc. Ir Lhis syn
dronc,LhcroishyoIasiaoIfhclcILvcnfriclc,aorLic
valvc sfcnosis or afrcsia, niLraI valvo sfcnosis or
afrcsia, and hyolasia oI fhc asccnding aorLa vifh
discrcfccoarcfafionoILhcaorfa.JhcscIcsionsrcducc
or clininaLc lIood llov Lhrough fhc IcIL sidc oILhc
hcarL, causingar olIigaLoryIcIL-fo-righLshunfaLfhc
aLriaI Icvcl ard a righL-fo-lcIt shunf af fhc ducfus
arLcriosus.SysfcnicllovisconIcLcIyducfaI dccn-
dcnf, and coronary crIusion is rcfrogradc vhcn
aortic afrcsia orcrifical aorfic sfcnosis is rcscnf.
< not tor zac i > < nc rn noa i >
Z |l ucr|n1O |eU| !r|C$
IIgure 3-9 Acu!ec|rcu|!CrycC||pse!C||Cv|ngcCnsIr|c!|CnC!
!he Ouc!ust!etCsusnhypCp|s!c|e!!her!synOtCme.These
neCn!es re !yp|c||y |n shCckv|!h pCCrper!us|Cn,!chycr-
O|,c|OCs|snO |n resp| r!CryO|s!ress.NC!e[)!he|CvcrO|c
Cu!pu![sev|OenceO by!he|Cvm|xeOvenCusCxygens!ur-
!|Cn |n !he super|Cr ven cv C! 55'), |b) nrrCv pu|se
pressurC,[c)e|ev!eO !r||nO ven!r|cu|renO-O|s!C||c pres-
sure-e|ev!eO |e!! !r|| pressure my cuse pu|mCnry
eOem [nC!e |e!! !r|| s!ur!|Cn C! 9JV), [O) s|gn|cn!|y
|ncreseO pu|mCnry b|CCO !|Cv, s re!lec!eO | n n r!er||
Cxygens!ur!|Cn [|n ||OC!0.21 ) C!86V.
LO|Cfly 1| b|K F|. OU| O COO1| LfC. 4

CO. ||lOC|Q|l.
LlQQlOCOI1VCO, I 998:426.
LlDCD NDDE5fDfOD5
As fho ducfus cIosos, noonaLos vifh I!IS havo
sovoroIydininishodsysfonicllood Iovandrosonf
|n shocI. Thoy naniIosf signs oI congosfivo hoarf
Iailuro vifh nodorafocyanosis, fachycardia, fachy-
noa, ulnonaryraIos [Iron uInonary odona), and
hoaLonogaIy. Poor or alsonf orihoraI uIsos and
vasoconsfr|cfod oxfronifios aro characforisfic. Jho
cardiac oxaninafion rovoals an S and a Ioud singlo
S,. Jhe FCC shovs docroasod R vavo rogrossion
across fhorocordiun.1ho chosfradiograh rovoaIs
uInonary odona.
lEDfHEDf
PCF, shouId lo sfarfod Lo nainfain ducfaI-
doondonf sysfonic llood Iov Mo corrocfivo
surgoryis avaiIalIo.Jhosfago I [or Norvood) alIia-
fion, vhich is orIornod in fho IrsL vooI oI IiIo,
alIovs fho na]orify oI noonafos fo survivo inIancy.
Jho sfago I rocoduro invoIvos analganafion oILhc
uInonaryartory andaorfaforovidounolsfrucfod
sysfoniclIoodIov, afriaI sofocfony, andnodiIod
BIaIocI-Jauss|g shunf fo rovido rosfricfivo uI-
nonary lIood Iov. Aor Lho sfago I rocoduro, a
cavouInonary anasfonosis is orIornod af 4 fo 6
nonfhs oIago and a nodiIod FonLan rocoduro is
gonoraIly orIornod af 2 fo 4 yoars oI ago. Sonc
confors do noL orIorn fho sfago I aIIiafion and
rocooddiroctly fo hoartfranslanfafion.
|nterrupted 0ort|c 0rch
Jhoro aro Lhroo fyos oI inLorrufod aorfic arch
[FiguroJ- I 0) . 1yoA isinforrufionloyondfhoIoIf
sulclavian arfory,fyo B isinforrufionlofvoonLho
Iol sulclavian and Iol connon caroLid artorios,
ard Lyo C u inLorrufion lofvoon fho Iol con
non carotid and Lho lrachiocohaIic artorios. In
fhis anonaIy, sysfonic lIood Ilov is doondonf on
aLoncyoIfhcducfusarforiosus,vhicIshunfslIood
Iron LhouInonary artory fofhoaorfa.Inforrufod
aorfic arch is olon associafod viLh IiCoorgo's
syndrono, duofofho22qI I nicrodoIofion.
LlDCD NDDE5fDfOD5
PuInonary odona occurs aInosf innodiafoIy. Jho
cIinicaI rosonfafion is siniIar Lo Lhaf oI crificaI
coarcfafion oIfho aorfa.
lEDfHEDf
PCF LhorayshouIdlogin innodiafoIyfo nainfain
sysfonic llood Iov via Lho righf-fo-Iol shunf
Lhrough fho afonf ducfus artoriosus. Fnorgonf
surgory is nocossary fo roanasfonoso fho aorfic
sognonfs.
0CY0NDT|C CDNGcN|T0L
Hc 0RT D|Sc 0Sc
cyanofic cardiac doIocfs fhaf rosuIf in incroasod
uInonary lIood Iov incIudo afriaI sofaI doIocL,
vonLricuIar sofaI doIocf, afonf ducfus arforiosus,
and connon afriovonfricuIar canaI. Acyanofic Io
sions fhaf rosuIf in uInonary vonous hyortorsior
incIudo coarcLaLion oI Lho aorLa and aorLic valvo
< not tor zac i > < nc rn njoa i >
< cran u cxanr-ronncjcun: o- jon-. >
!r3 r|C|Cy 21
7
T
IIgure 3-1 U |n!errup!eO Cr!|c rch v|!h res!r|c!|ve p!en!
Ouc!usr!er|Csus.1yp|C|nICm|cnO hemCOynm|cnO| ngs
|nc|uOe:[)Ires|C!segmen!C!!heCr!|crch beIveen !he
|e!| subc|v|n r!erynO Ihe |e!| cCmmCn CrC!|O [!he mCs!
CCnmCnIypeC!|nIerrup!eU CrI|crch-"!ype B");[b) pCs-
!er|Cr m|||gnmenI C! Ihe cCn| sep!um resu|!|ng |n |rge
ven!r| Cu|r sepI| Oe!eCI nO nrrCv subCrI|c re, [c)
b|Cusp|O Cr!|c v|ve Cccurs |n 60V C! p!|en!s, [O) sys!en|C
pressure| n!her|ghIven!r|c|enOpu|mCnryr!ery[Oue!C!he
| rge, nCnresIr|cI|ve venIr|cu|r sep!| Oe!eC!), [e) |ncreseO
Cxygen sIur!|Cn |n !he pu| mCnryr!ery OueIC|e!!!Cr|gh!
shunI|ngI!heven!r| Cu|r|eve|,[!)"O|!Ieren!|| CynCs|s"v|!h
|Cver Cxygen s!urI|Cn | n !he OesCenO|ng Cr! Oue IC
r|ghI!C|e!! shunI! !hepIen! Ouc!us.NCIeIhe|Cver b|CCO
pressure | n !he OesCenO|ng CrI Oue !C cCns!r|c!|Cn C! !he
Ouc!us,Cpen|ng !he OuCIus v|!h |GL, resu|!s | n equ| upper
nO |Cverex!rem|!yb|CCO pressures, bu! cCn!|nueO "O|!Ieren
!||cynCs|s."
L|ODCf1y 1| b1|K F|. OU| O COO LfC, 4'" CO. DOC|j|.
LjjOCO11|VCO, J 998:426.
sfcnosis. Jhc acyanoLic sfrucfuraI anonaIy fhaf
rcsuIfs in rcIafivcIy nornal uInonary lIood llov is
uInonaryvaIvcsfcnosis.
0tr|a| SeptaI Defects
AfriaI sctaI dcIccfs account Ior Sv oI congcniLaI
hcarf discaso. Jhcrc arc fhrcc fycs oI atriaI scLal
dcIccfs.
CsfiunsccundundcIccf, sccn infhc nidorLion
oIfhc afriaI scLun
Cstiun rinun dcIccf, IocaLcd in fhc Iov afriaI
scfun
Sinus vcnosus dcIccf,Ioundatthc]uncfionoIfhc
righfafriunandLhe suoriororinlcriorvcnacava
Jhc dogrcooIairiaIshuniingisdccndcnfonfhc
sizc oILhc ASI and Lhc rcIaLivc conliancc oILhc
vcnfricIcsin diastoIc. Sincc rihtvontricuIardiastoIic
conIianco is usuaIIy grcatcr Lhan IcIL vcnLricuIar
diasfoIic conliancc, lcIt-fo-righf shunLing occurs
aL fhc aLriaI IovcI, Lhus incrcasing llov across thc
fricusid anJ ulnonary vaIvcs and incrcasing
ulnonarylIoodllov.
LDCD NDDE5fDfOD5
AfriaI scfaI dcIccLs arc usuaIly roLassociaLcd vifh
synfons, aIthough Lhcrc nay lc a hisfory oIsIov
vcight gain and Ircqucnf Iovcr rcsirafory inIcc-
Lions. Cn hysicaI cxaninafion, fhc rccordiun is
hycrdynanic, and arighfvcnfricuIarhcavc is oILcr
rcscnf.A sysLoIicc]cctionnurnur ir thcuInonic
arca and a nid-diasfolic runlIc in Lhc Iovcr righL
sfcrnaI lordcr rcllccL Lhc incrcascd llov across Lhc
uInonaryanJ fricusidvaIvcs.S,isvidcIyandcon-
sfanfIysIiL.CnchcsLradiograh,fhchcartandnain
uInonary arLcry arc cnlargcd and uInonary vas-
cularifyisincroascd.JhcFCCoILcnshovsrighLvcn-
fricuIar hycrLrohy or righf vcntricuIar conducfion
dcIay. Righf-axis dcviafionisoILcnsccnin sccundun
dcIccfs, vhcrcas rinun dcIccLshavc characLcrisfic
cxfrcnc lcIL-axis dcviaLion. Jhc anounf oI righf
vcnfricIc and IcIt afriun cnIargcncnf is dircctly
oortionaI Lo Lhc sizc ol fhc IcIL-to-right shunt.
Cncchocardiogran,fhcdcIccfcanlcvisuaIizcd,and
IoIcrllovnaingdcnonsLraLcsLhcdirccLionoI
lov.
lEDfHEDf
SonLancous closurc oI snaII sccundun ASIs is
liIcIyfooccurinthcna]oriLyoIcascsirLhcIrsfycar
oIIiIc. Cstiun rinun and sinus vcnosus ASIs do
not closc sontancously and nust lc addrcsscd sur
gicaIly.Jhc synfonafic chiId viLh anASI shouId
havcfhcdcIccfcloscJassoonasossilIc.JhcLining
oIASI rcair inLhc asynfonaticinIant orchildis
norc controvcrsial. IngcncraI,Lhc dcIcct shouldlc
rcaircd vhcn circulaLory arrcsf is nof nccdcd and
vhcn tho liIoIihood oIncoding a lIood fransIusion
< not tor c > < no rn njoa i >
2 || ucgr|n1O t|c
< cran u cxanr-ronncjcun: HCA o- jon-. jy >
is Iov. Acr b nonLhs oIagc, lofh oILhcsc crifcria
arc gcncraIly ncL. Sulacufc lacfcriaI cndocardifis
rohyIaxisisnotrcconncndcdlorsccundunafrial
scfal deIccLs uLis indicaLcd in rinun andsinus
vcnosus afrial scfaI dcIccfs.
Ventr|cu|ar Septa| Defects
ThcvcnfricularscfaIdcIccfs arc fhcnosfconnon
congcnifaI hcarL dcIccf, accounfing Ior 25v oI aIl
congcnifaI cardiac Icsions. Jhc Ivc fycs oI vcnfri-
cular scfaI dcIccfs arc as IolIovs.
MuscuIar
InIcL
ConoscLaIhyolasia
ConovcnfricuIar
MalalignncnL
MuscuIar vcnfricular scfaI dcIccfs occur in fh:
nuscuIarortionoILhcscfunandnaylcsingIcor
nuIfiIcandIocafcdinLhcosfcrior, aicaI, oranfc-
riororfionoIfhcscfun.Jhcinlct\SI isancndo-
cardiaI cushiondclccfandoccurs ir fhc inlcforfion
oI fhc scfun lcncaLh fhc scfal IcaIcI oI Lhc
fricusid vaIvc. Conoscfal hyolasia \SIs arc
osiLioncd |nLhc oufIov Lracf oILhc righfvcnfricIc
lcncafh Lhc ulnonary valvc. Jhe conovcnfricular
\SI occurs in fhc ncnlranous orLion oI Lhc
vcnfricuIarscfur.MaIali gnncnf VSIs rcsuIflron
naIalignncnfoIfhc inIundiluIarscLun.
Vhcnfhc\SI is non-rcsfricfivc,ulnonaryvas-
cular rcsisfancc [P\R) and sysfcnic vascuIar rcsis-
fancc [S\R)dcfcrnincshunfIov.WhcnfhcP\Ris
IcssfhanfhcS\RfhcshunfIovis|clforighf.Iargc
dcIcctscvcnLuaIIy rcsuIfinuInonaryhycrLcnsion,
vhcrcas snaII dcIccfs do nof changc P\R. Jhc
anounfoIIclvcnLricuIarandlclafrialdiIafafionis
dirccflyroorfionaI Lo flc sizc oIfhc Icl-Lo-righf
shunf. Right vcnfricuIar hycrfrohy occurs vhcn
ulnonary vascuIar rcsisfancc incrcascs. II Icl
unfrcatcd,Lhc largc\SI nayrcsulfinclcvafcduI-
nonaryarfcrial rcssurcsandnayIcadfoulnonary
vascular olsfrucfivc discasc, and Fiscnncngcr's syn
dronc.InsonccascsolFiscnnengcr'ssyndronc,Lhc
\SI shunfnayrevcrsc righf fo IcIL.VhcnLhc\SI
istcsfricfivc,shunfIov islcIfforighf lron thchigh
rcssurc!\ fofhclovcrrcssurc R\
LDCD NDDE5fDfOD5
ClinicaI synfons arc rclafcd Lo fhc sizc ol fhc
shunf./snaIIshunfroduccsnosynLons,vhcrcas
a Iargc shunt vifhouf clcvafcd uInonary arfcriaI
rcssurcs givcs risc Lo congcsLivc hcarL Iaiurc and
grov:h Iailurc. Jhc aficnf viLh a Iargc \SI vifh
Fiscnncngcr hysioIogy rcscnfs vifh shorLncss oI
lrcafh, dysncaoncxcrLion, chcsfain,andcyanosis.
Jhc snaIIcr fhc dcIccf, fhe Ioudcr Lhc hoIosysfoIic
nurnur./suInonaryvascuIarrcsisfanccircrcascs,
LhcholosysfoIicnurnurshorfcnsandfhcuInonary
cononcnf oI S, incrcascs in infcnsify In fhc rcs
cncc oI uInonary vascuIar olsfrucfivc discasc, a
righf vcnfricuIar hcavc, c]ccfion cIicl, shorf sysfoIic
c]ccfion nurnur, diasfoIic nurnur oI uInonary
valvc |nsuIcicncy, and loud, singlc S, arc hcard.
ChcsLradiograhIorsnaIIdcIccfs naylcnornaIor
shovniIdcardioncgaIyand asIighf incrcasc inuI-
nonary vascularify, vhcrcas in largc lcn-fo-right
shunfs cardioncgaIy, incrcascd uInonary vascuIar-
iLy, andcnIargcncnfoILhc Iclafriun andIclven-
fricIc arc sccn. In snall dcIccfs fhc FCC is nornal,
vhcrcasviLhaIargc\SI,IcILafrial,lcILvcnfricular,
orlivcntricuIarhycrtrohy is sccn. RighLvcnfricu-
Iarhycrfrohy rcdoninafcs vhcn uInonary vas-
cular rcsisLancc is high. Cn cchocardiogran, Lhc
dcIccfcanlcvisuaIizcd, and IoIcr Iovnaing
dcnonsLrafcsLhc dirccfion oIIov.
lEDfHEDf
MosLsnaII\SIscIoscvifhoufinfcrvcnfion [40vly
Jycars, 75vlyI 0ycars), vhcrcasLhcfrcafncnfIor
Iargc\SIs is surgicaIcIosurclcIorcuInonaryvas-
cuIar changcs lcconc irrcvcrsilIc. Congcsfivc hcarf
Ia|Iurc is frcafcd vifh digoxin, diurcfics, and an
angiofcnsin-convcrfingcnzync [/CF) inh|litor.
Common 0tr|oventr|cu|ar Cana|
Jhc cornon aLriovcnLricuIar canal dcIccf [Figurc
J-! !), rcsulfsIrondcIcicncyoILhccndocardiaIcush-
ions andrcsuIfsinanosLiunrinun/SI andinIcf
\SIvith lacIoIscfafion oIfhc nifral andfricus-
idvaIvcs[connonafriovcnfricularvaIvc j C/\\] ).
I r an |ncompIeIe aIriovenIricuIar canaI dcIccf, fhc
C/\\IcaIcLsaffachcsdirccflyfoLhcfooIfhcnus-
cuIar orfior oIfhc vcnfricuIar scfun. /s a rcsuIf,
Lhcrcisno connunicaLionlcncafhfhcafriovcnfric
uIarvaIvcslcfvccnfhcright andlcILvcnfriclcs.Jhc
connunicafionatfhcafriaIIcvcIisanosfiunrinun
/SI.JhenifraIvaIvciscIcIL,andfhcrcnaylcsonc
dcgrccoInifraIrcgurgifafion. IncompIeIe common
aIriovenIricuIar canaI, Lhcrc is a CA\\ Lhaf is nof
aLfachcd fo fhc nuscuIar vcnfricuIar scfun. /s a
rcsulf,fhcrcisaIargcinlcf\SI locafcdlcfvccnLhc
< not tor zac i > < nc rn njoax i >
< cran u cxanrronncjcun: nvcm o- jon-. jy >
IIgure 3-1 I CCmp|e!ecCmmCn!r|Cven!r|cu|rcn|.1ypic|
ntCmc nO hemCOynmc nOngs nc|uOe: |) |rge !r|
nO ven!r|cu|rsep!|Oe!ec!sC!!heenOCcrO||cush|Cn!ype,
[b) s|ng|e, !r|Cven!r|cu|r v|ve, [c) pu| mCnry r!ery hyper
!ens|Cn[Oue!C|rgeven!ricu|rsep!|Oe!ec!), [O) b|O|rec!|Cn|
shun!| ng[v|!h m||O hypCxemi) ! !r|| nO ven!r|cu|r |eve|
vhen pu|mCnry vscu|r res|s!nce |s e|ev!eO in !he |n|!||
neCn!| per|CO.W|!h subsequen! !| | | n pu|mCnry vscu|r
res|s!nce,!heshun!becCmespreOCm|nn!|y|e!!!Cr|gh!v|!h
synp!CmsC!cCnges!|veher!!| | ure.
L|ODCf1y 1R b1fK F|. OU| O COO| L|C, 4

'
CO. |OCj
LjjOCO11|VCO, J 998:426.
CA\\ and fhc Lo oI fhc nuscuIar vcnfricular
scfun.InLhisdcIccf,fhcrcisaleIL-fo-righfshunfaf
LhcafriaI[osLiunrinun/SI) andvcnfricuIarIcvcI
[inIef\SI). Bccausc oI fhc increasc in ulnonary
llood lov, ulnonary hycrfcnsion andulnonary
vasculardiscascnaydcvcIooverLinc.
LDCD NDDE5fDfOD5
In conlcfc connon afriovcnfricuIar canaI, congcs-
fiveIeartIailurcissccncarIyininIancy,viLhfachy
nca, dysnca, ano oor Iccding. Cn cxaninafion,
a lIoving hoIosysLolic nurnur is hcard aI fhc IcIL
Iover sfcrnaI lordcr duc fo fhe \SI and sonc
dcgree oI connon aLriovcnLricuIar valvc rcgurgifa-
fion, andan S,vifh a vidclyIxed sIif is hcard duc
fo Lhe afriaI scfaI dclccf.Jhc |CCrcvcaIs lcIt-axis
dcviafion, righfafrial diIaLion, andIcILafriaIdilafion.
Jhe clinicaI naniIcsLafions oI fhc inconIcfc
C|p!er3 / CrU|O| CQy 29
connonafriovcnfricuIarcanaIarefhesancasLhosc
dcscnlcd Ior an osLiunrinunASI.
lEDfHEDf
Surgical rcairlor conlcfc connon afriovcnfricu-
IarcanaI is usualIy donc vifhin Lhe IrsL ycar oIliIc.
Prior fo surgicaI rcair, congcsfive hcarf laiIurc is
Lrcafcdvifhdigoxin,diurcfics, andan/CF irhiliLor.
Conlefc bcarL llocI occurs in 5v oI aLicrfs
undcrgoingrcair,andrcsiduaInifralinsullcicncyis
oILcnscen.
Patent Ductus 0rter|osus
Pafcncy olfhcducfusartcriosusaccounfsIor I 0v oI
congenifaI hcarfdiscasc.Jhcrcis ahigh incidcnccin
rcnafurcnconaLcsanda2. I IcnaIercdoninancc.
JhcducfusartcriosusconnccfsfheaorLaandLhclcIt
uInonary arfcry j usf disfaI Lo Lhe faIcoI oI Lhc
lcIL sulcIavian arfcry lron fhc aorfa. Jhc dirccfion
ol Iov fhrough a Iargc aLcnf ducfus artcriosus
dccndsonfhc rcIafivc rcsisLanccs infhcuInonary
and sysfcnic circuifs. In flc non-resfricLivc aLcnf
ductus artcriosus, as Iong as fhe sysfcnic vascuIar
rcsisLancc is grcaLcr fhan Lhc uInonary vascuIar
rcsisLancc, a IcIL-to-righf shuni |s rcscnf. II ul-
ronary vascuIar rcsisfancc riscs alovc sysfcnic
vascuIarrcsisfancc, a righf-fo-lcILshuntdcvcIos.
LlDCD NDDE5fDfOD5
SynfonsarcrclafcdioihcsizcolLlcdcIccfandfhc
dirccLion oI llov. / snalI afcnf ducfus arLcriosus
causes no synfons./Iargc oncvifha lcIL-fo-righf
slunf nay rcsuIf ir congcsfivc learf IaiIurc, sIovcd
grovfh, and rccaLcd Iovcr resirafory LracL
inlccfions. RcvcrsaI oI llov as a rcsulf oI high
uInonary vascuIar r:sisfancc causcs shorfncss oI
lrcafh, dysncaon cxcrfion, ard cyanosis. Ina Iargc
shunf, lounding pulscs, rcrcscniing an aorLic dia
sLoIicrunoI[arcaIalIc.Jhcnurrur,oILcnrcIcrrcd
Loasa"nachincrynurnur,"isconfiruouslcginning
aILcrS, , caIsaLS_,andfrailsoI|duringdiasfoIc.Jhc
cIcsf tadiograh oIa largc afcnf ducLus arfcriosus
viIl slovcardioncgaIy, ircrcascd uInonaryvascu-
IariLy,andIcIL afriaIandIcILvcrfricuIarcnIargcncnf.
JhcneonafcvifhasnaIlafcnfducLusarLcriosushas
a nornaI FCC, vhcrcas Lhc nconaLc viLh a Iargc
afcnt ducLus arLcriosus and a gcnerous IcIL-fo-righf
shunfshovsIcIL orlivcnfricuIar bycrLrohy. RighL
vcnfricular hycrfrohy rcdoninafcs on ICC in
fhc rescncc oIincrcascd uInorary vascuIar rcsis
fancc. Jhc aLcnL ducfus arLcriosus is lcst sccn on
cchocardiogranusingIoIcrlovnaing.
< not tor zac i > < nc rn njoa i >
dU ||ucr|nIO |eU|tr|C$
lEDfHEDf
IndoncLhacin isolcn oIloctivcincIosingLhcafcnf
ducfus arforiosus in fhc rcnaturc nconatc ly
dccrcasing PCF, IovoIs. A atcnf ductus arLcriosus
usuaIIy cIoscs in Lho Irsf nonth ol Iilc, lut Ior
thosc fhaf do nof, surgical ligatior ly Lhoracotony
or vidco-assisfod thoracoscoic surgcry, or coiI
cnloIization lycaLhctcrizatior is curafivc.
Coarctat|on of the 0orta
Coarcfafion oIfhc aorfa [Figure J- I 2) accounfs Ior
Sv oI congonifaI hcart dcIccfs and has a naIo-fo-
lonaIc rcdoninancc oI 2.I . Vhor coarcfafion oI
||gure3-1 2 CCrc!!|CnC!!heCr! | nCr|!|C| | y||| neCn!e
v|!h ner|y C|CseO Ouc!us r!er|Csus.1yp|c| nICm|C nO
hemCOynm|c nO|ngs |nC|uOe. [) "ux!OuC!|" s|!e C! !he
CCrc!!|Cn, [b) b|cCmm|ssur| Cr!|C v|ve [see | n 80V C!
p!|en!s v|Ih cCrc!!|Cn), [C) nrrCv pu|se pressure | n !he
OescenO|ngCr!nO |CverbCOy,[O) b|O|reC!|Cn| shun!I
!heOuc!usr!er|Csus.As|n Cr|!|c| Cr!|cs!enCs|s[see||g.J1 J)
there|sne|ev!eO|e!!!r|| pressure,pu|mCnryeOem,|e!\-
!C-r|gh! shun! I !he !r|| |eve|, pu|mCnry r!ery hyper!en-
s|Cn,nOCn|y mCOerIe [J0-mm HQ) QrO|en!crCss!herch
Cbs!ruC!|Cn. The |Cv mesureO QrO|en! [Oesp|!e severe
n!Cm|c Cbs!ruc!|Cn) CrCss !he CrI|c rCh |s Oue !C |Cv
CrO|CCu!pu!.
L|O|C|1y J| bfK F. OU O COO| LfC, `` CO. D|OCQD.
LjjOCO1VCO, I 998.426.
fhcaorLaoccursinaIcnalc,Jurnor'ssyndronenust
lo considorod.Jhc olstrucfion is usualIy Iocatcd in
Lho dosconding aorLa, at tho |nscrLion sifo oI thc
ducfus arLcriosus. Jhc aorLic vaIvo is licusid in
S0v oIcasos, and nitraI vaIvc anonaIios nay aIso
lc rcscnf. Jhc coarctation rcsuIfs in nochanicaI
olsfruction lctvccr tho roxinaI and distaI aorLa
andinincrcascdIolvontricuIaralcrIoad.Congcsfivo
hoarfIaiIurc dcvcIos ir I 0v oIcascs ininlancy.
LDCD NDDE5fDfOD5
Cn oxanination, Lhc lcnoraI uIscs arc olcn voaI
and dclaycd rcIativc to ucr cxfronificsor arc
alsonfand fhcrc is olon ucr cxtrcnity hycr-
tonsion. loonafcs vifh criticaI coarcfation havo
ducfaI-dcondont sysfcnic lIood Iov and nay
rcscnt vith circuIafory coIIasc. Flov across fhc
coarctatior nayroducc a sysfoIic ojcctionnurnur
noard af fhc acx. Cn chcst radiograh, tho aorLic
Inol is cnIargcd, on FCC, right vcntricuIar hyor-
frohy is sccn in Lho noonafo, and lcl vontricuIar
hyorLrohyis sccn in Lho oldor chiId.Jho cchocar-
diogran is uscd fo visuaIizc thodoIocfand to chocI
IoralnornaIifiosoIfhoaorLicvaIvo,nitralvaIvc,and
lclvcntricuIarorIornanco.
lEDfHEDf
PaIIiatior naylo acconIishcd via laIIoon diIafion
angioIasty, stont IaconcnL, or ly surgicaI cnd-Lo-
cnd anastonosis, sulcIaviar Ia rcair, afch rcair,
or graIt Iaconont.
0ort|c Stenos|s
Ir aorficsfonosis[FiguroJ-! J), LhcvaIvuIartissuc is
LhicIcncdandolonrigid.MosfconnonIy, fhcvaIvc
is licusid, vith a singIc Iuscd connissurc and ar
occontricoriIco.JhosfcnoficvaIvoroduccs a rcs-
surcgradicnflcfvconthcIclvcnLricIcandthcaorfa
Lhaf rcsuIfs in Icl vontricuIar hycrLrohy and,
ovcr tinc, dccrcascd conIiancc and vontricuIar
crIornancc.
LDCD NDDE5fDfOD5
Jho noonato viLh aorfic sfonosis nay roscnt vith
cardiovascuIar coIIasc or vith a sol nurnur. Jho
IovcI oIsyntonatoIogyisrolatodLo ihc scvcrify oI
fhc sLcnosis and fho vcntricular Iuncfion. Jhc
nconafc viLh criticaI aorLic stonosis has ducfaI-
dccndcntsystoniclloodIovandnayrcscnfvitl
circuIafory coIIasc alor fhc ducfus cIosos. II von-
< not tor zaci > < nc rn oa i >
< cran u cxanr-ronncjcun: nvcm o- on-. jy >
Igure d-1 d Lrl!|c| v|vu|r Cr!|c s!enCsls v|!h c|CseO
Ouc!usr!er|Csus.1yp|c|n!Cm|cnOhemCOynm|cnOlnQs
|nc|uOe:[)mCrphC|CQ|c||ybnCrm|, s!enC!|cv|ve,[b)pCs!
s!enC!|c O| |!!|Cn C! !he scenO|nQ Cr!, [c) e| ev!eO |e!!
ven!r|cu|renO Ols!C||c pressure nO |e!!!r|| pressures cCn
!rlbu\|nQ !C pu|mCnry eOem [m|| O pu|mCnry venCus nO
r\er|| Oes!ur!|Cn),[O) |e!!-!C-rlQh!shun!!!he!r|| |eve|
[nC!e| ncrese|nCxyQen s!ur!|Cn!rCmsuper|Crvencv!C
r|Qh! !r|um), [e) pu|mCnry r!ery hyper!ens|Cn [|sC sec-
CnOry !C !he e|ev!eO |e!! !r|| pressure), [!) Cn|y mCOes!
[25-mmHQ) QrO|en!crCssv|ve.1he |CvmesureO QrO|en!
[Oespl\eseveren!Cm|cCbs!ruc!|Cn)crCss!heCr!|cv|ve|s
Oue!C severe|y | | m|!eO crO|c Cu!pu!,s ev|OenceO by!he
|CvmlxeOvenCusCxyQens!ur!|Cn[45V)| n!hesuper|Crven
cv.
LO|C|1y 1| b1|K F|. OU| O |COO1| LfC,

CO. |||OCQDl.
LQQOCO11|VCO, ''. V.
fricular Iuncfionisnaintaincd, a harsh systoIic cjcc-
fion nurnur is hcard af fhc righf ucr sfcnaI
lordcrandisrcccdcdlyancjccfionclicIhcardlcsf
at tIc IcIt IovcrsfcrnaI lordcr. lIvcnfricuIar Iunc-
tion is conroniscd, fhcrc nay lc signiIcanfsfcno-
sis vifh onIy a soIt nurnur arcciafcd. Cn chcsf
radiograh, osfstcnoiic dilatafion ol the asccnding
aorta |s rcscnf, and in scvcrc cases, uInonary
cdcna can lc secn. Jhc FCC nay shov IcIt vcn
fricuIar hyerfrohy, and a sfrain paftcrn oI S1
dcrcssions and invcrtcd1 vavcs naylc sccn. Jhc
vaIvuIar lcsion, fhc dcgrcc oIsfcnosis, and IcIt vcn-
tricuIarIunction are aIl sccn oncchocardiogran.
L|QtCr 3 / LrO|o| o_y 31
lEDfHEHf
IIintcrvcntion is rcquircd, cIicloI thc aorficvalvc
gradicnf nay bc acconIishcd ly ocn surgicaI
vaIvofonyorlylaIloonvaIvuIolasty. BotI surgicaI
vaIvotony and laIIoon vaIvuIolasty nay rcsuIf in
rogrcssivc aortic rcgurgifation fhat na, rcquirc
aortic vaIvc rcIaccncnf vifh a ncchanicaI, hono-
graIt, or aufograItvaIvc [Ross roccdurc) .
PuImon|c Stenos|s
Pulnonic vaIvc sfcnosis accounfs lor 5v fo Sv oI
congcnifaI hcarf dcIccfs. Jhc uInonary connis-
surcs arc Iuscd, fhc vaIvc is doncd and has a snaII
ccntraI ocning, and fhcrc is osfsfcnofic diIafaiion
oIfhc nain uInonary arfcry.JhevaIvc islicusid
ordysIasficir I 0voIcascs.Righfvcnfricularhycr-
trohyoccurs ovcrfinc asfhcvcnfricIcattcnfsfo
naintain cardiac outuf. In crificaI uInonic sfcno-
sis, a dccrcasc ir fhc conliancc oIfhc righfvcnfri-
cIc viIl incrcasc rigIt atriaI rcssurc and nay oen
fhe Iorancn ovalc, roducing a snaII righf-fo-IcIt
shunf.
LHCD NDHE5fDfOH5
Mosf aticnis are asyntonatic. Scvcrc fo crifical
ulnonary sfcnosis nay causc dysnca on cxcrfion
and angina. Right-sidcd congcstive hcart IaiIurc is
rarc, cxccpt in inlanfs vifh crifical uInonic sfcno-
sis vho nay Iavc ducfaI-dccndcnf ulnonary
llood lov. CharacfcristicaIIy, fhc ejccfion cIicI ol
uInonicsfcnosisvaricsvifhinsirafion,andaharsh
sysfoIic cjccfion nurnur is hcard at fhc IcIt ucr
sfcnaI lordcr. In scvcrc sfcnosis, a fhrilI and righi
vcntricuIar hcavc arcaIalIc. Cn chcsf radiograh,
hcarf size and uInonary vascuIarity arc nornal,
luf fhc puInonary arfcry scgncnf is cnIargcd. Cn
|CC,fhcdcgrccoIrigItvcntricuIarhycrfrohyand
righf-axis dcviation corrcIatcs vifh fhc dcgrcc oI
sfcnosis.JhctransvaIvuIargradicnfandfhcdcgrccoI
righ! vcnfricuIar hycrfrohy can lc ncasurcd ly
cchocardiogran.
lEDfHEHf
IcIniiivc trcatncnf is acconIishcd ly lalIoon
vaIvuIolasty ol thc sfcnoiic vaIvc. lndications Ior
uInonary vaIvofony includc a righf vcntricuIar
rcssurc grcafcr fhan 50nn Ig or synfons oI
righf-sidcdcongcstivchcarf lailurc.
Jhus Iar, fhis chafcr has Iocuscd on fhe cvalua-
tion oIfhc cyanotic nconafe andthc nosf connon
< nor tor zaci > < nc rn oa i >
dZ |lucr|n1O |eU!rCs
< cran u cxanr-ronncjcun: nvcm o- on-. jy >
l PLL 55
LSSc n0ngS Of the 1 NOSt LOmmOn LOngent Heft LeSOnS
LeSon |eSentaton ySCa LLL X-ray
Lxamnaton
A!r|| sep!| OeeC! Nurmur |XeO$p| | !5 N| | OKVH JLL,1|b|
Ven!rCu| rsep!| OeeC! Nurmur, LH| HC|Csys!C||Cmurmur LVH, KVH +LL,T|b|
|!en!OuC!u$ Nurmur, LH| LOn!|nuOu$murmur LVH, JKVH LL,T|b|
AV Cn|OeeC! Nurmur, JLH| HC|Osys!C| | Cmurmur "5uperOr"X|s J LL,T|b|
|u|mOnC$!enCs|s Nurmur, JCynO$s L|Ck,5LN KVH LL,NL,Or
]
|b|
1e!r|OQyO|||C! Nurmur, CynCs|s 5LN KVH LL,
]
|b|
AOr!|C s!enO$|s Nurmur, JLH| L|Ck, 5LN LVH JLL,NL, |b|
LCrC!!On O!Or! Hyper!en$|On
]
|emOr| pu|$e$ LVH LL, NL, |b|
Trn$pO$|!|OnO!!he LynOs|$ NrkeO CynOs|$ KVH JLL,NLOrT|b|
Qre!r!er|e$
5nQ|even!r|C|C [Vr|b|e) [Vr|b|e) [Vr|b|e) [Vr|b|e)
C|, card|ac en|argemen\, CH| CongesI|ve earI la|Iure, LVH, IelI venIr|Cu|ar hyperIrophy; N|norma|; PBF, pu|monary b|ood !|ow, FVH, r|ghI ventr|cu|a|
hypertrophy, SLM,sysIo|c ejecI|on murmur
cyanofic and acyanofic congcnifaI hcari dclccfs.
BcIorc noving fo acquired sfrucfural heari discasc,
IuncfionaI hcari discasc, and arrhyfhnias, scc JalIc
J-J, vhicI Iisfs fhc cIassic lndings Ior fhc I 0nost
connon congcnifaI hcarf Icsions.
0CQU|RD STRUCTUR0L
H0R D|S 0S
Rheumat|c Heart D|sease
Rhcunafic hcarf discasc rcsuIfslonsingIc or nuI
fiIecisodcsolacufcrheunaficlcvcr. MifraIrcgur
gifafion is fhc nosf connon Icsion Iound. ortic
insullcicncyisalso connonIyIoundvifhorvifhouf
nifraI rcgurgifafion. MifraI sfcnosis is Icss connor
and usuaIIy is thc cnd result olnuItiIc affacIs ol
acutcrIcunaficlever. !casfconnonisaorficsfcno-
sis. Jhc fricusid and uInonary vaIvcs arc aInosf
nevcr aIlccfcd. Synfons are rooriionaI fo fhc
degrcc olvalvular danagc. Rhcunafic lcvcr is dis-
cusscdin Chafcr | 2.
Kawasak|'s D|sease
CardiaccIlccfsnayincIudccricarditis,nyocardifis,
and fransienf rhy:hn disfurlanccs. Hovcvcr, if is
fhc dcvcIoncnf ol coronary aricry ancurysns,
vifh thcir ofcnfiaI lor occIusion or rufurc, fhat
naIcs fhc discasc Iilc-fhrcafcning. Coronary arfcry
ancurysns dcvcIo during fhc sulacufchasc [I1fI
fo 25fh day) in alouf J0' ol cascs luf rcgrcss
in nosf aiicnfs. FarIy fhcray vifh infravcnous
innunogIoluIindccrcascsfhcincidcnccolcoronary
aricryancurysnsfoIcssfhan l 0v. HigI-doscasirin
thcrayIcsscnsfhcIiIclihoodolIafcancurysns.1hc
cchocardiogranisuscdfo asscssvcnfricularIuncfion
and visuaIizc cricardiaI lluid and coronary arfcry
ancurysns. / fhorough discussion ol KavasaIi's
discasc is lound ir Chatcr I I .
cndocard|t|s
DfDO@EHE55
BacfcriaI cndocardifis is a nicroliaI inlccfion oIfhc
cndocardiun. /lfhough if nay occur on nornaI
vaIvcs, lacfcriaI cndocarditis is nuch norc IiIcIy
fo occur on congenifaIly alnornaI vaIvcs, vaIvcs
danagcd ly rhcunatic lcvcr, acquircd vaIvuIar
Icsions [nifraI vaIvc roIasc), and rosfhcfic
rclaccncnf vaIvcs as a conscqucncc ol furluIcnf
lIood llov. Facfors fhaf nay rcciifafc lacfcriaI
cndocardifis includc a rcvious cisodc ol cndo
cardifis, dcnfaI naniuIafion or inlccfion, insfru-
< not tor zaci > < nc rn oa i > < cx v cxanr-rnncjcn:


>
c` | dd ar ar | C CQy
ncnfafion oI fhc gasfroinfcstinaI or gcnifourinary
fracf, infravcnous drug alusc, an indvcIIing ccnfraI
vcnous cafhcfcr, andrior cardiac surgcry.
Ir chiIdrcn, aIha hcnolyLic sfrcfococci [Streto-
coccus uir|dons) and Stoby/ococcus outeus arc fhc
nosf connon ctioIogic agcnfs. S. uir|Jns accounfs
lor aroxinafcIy 67' oI fhc cascs, vhcrcas S.
outeus isrcscnfinalouf20v olcascs.VhcninIcc-
iion conIicafcs cardiac surgcry, Stoby/ococcus ei-
dernidis, gran-ncgafivc laciIli, and Iungi shouIdlc
considcrcd. Cran-ncgativc organisns causc alout
5v olcascsolcndocardifisinchiIdrcnandarcnorc
IiIcIy in nconafcs, innunoconroniscd aficnfs,
and irfravcnous drug aluscrs. Anong fhc I/CFK
[Hoemobi/us, .ctino/oci//us, Cotdio/octer|um, li/e-
ne//o, Kinge//o) organisns, vhich arc a rarc causc ol
cndocardifis, Hoemobi/us in]|uenzoe is fhc nost
connon, IrcqucnfIy aIlccfing rcviousIy danagcd
vaIvcs.
LHCD NDHE5fDfOH5
Fcver is fhc nosf connon Inding in childrcn vifh
lacfcriaI cndocardifis. CIten, a ncv or changing
rurnur is auscuItafcd. ChiIdrcn vifh cndocardifis
usuaIIy dislay nonsccilc synfons such as chcst
ain, dysnca, arfhraIgia, nyaIgia, hcadachc, and
naIaisc.Fnlolichcnoncnasuchashcnafuriavifh
rcdccII casfs and fransicnt ischcnic afiacIor sfroIe
nayle rcscnf. CfIcrcnloIic hcnoncna, such as
Rofh sofs, sIinfcr hcnorrhagcs, cfcchiac, CsIcr
nodcs, and 1ancvay lcsions, arc rclafivcIy rarc in
childrcn vith lacfcrial cndocardifis.
LD@HO5fC LvDUDfOH
!aloratory siudics incIude a conletc lIood couni,
cryfhrocytc scdinenfation rafc [FSR), c-rcacfive
rofcin [CRP), and urinaIysis. MuItiIc lIood cul-
furcs incrcase ihc rolaliIify ol discovering fhe
afhogcn. Positivc lIood culfurcs, an cIcvafcd FSR,
eIcvafcdCRP, hcnafuria, and ancnia arcnosfoItcn
lound.TIccchocardiogranisuscdfo dcInc vcgcfa-
tionsorfhronliinfhehcari.
lEDfHEHf
McdicaI nanagcrcnf consisfs oI 6 vccIs oI infra
vcnous anfiliofics dirccfed againsf thc isoIafcd
afhogcn.SurgcryisindicafcdIorcndocarditisvher
ncdical frcafncnf is unsucccssluI, rcIracfory con-
gcsfivc hcarf laiIurc cxisfs, or fhcrc arc scrious
enloIic conlicafions, nyocardiaI alsccss Iorna-
tion, orrcIracfory rosfhcticvaIve discasc.
Prcvcnfion oI cndocardifis is ncccssary lor high-
risl aficnfs. /nfiliotic rcgincns fo revcnf cndo
carditis duringdcnfaI,rcsirafory, gasfroinfcsfinaI,or
gcnifourinary roccdurcs incIudc oraI anoxiciIlin or
arcnfcraI anicilIin and gcnfanicin rior fo ihc
roccdurc.
M
hN LW
W
1 . Pa!Cn!s wl!h COngen!a||yaOnOtma|va|ves,va|vCs
OamagCOOytCuma!lClCvCt,aCquttCOva|vu|at
|CslOns(mttra|va|vCprO|apsC},OtprOsthC!lC
rCp|aCCmCn!va|vCsatCatnCtCasCOrsklOr
CnOCCatOl!ls.
2. P|pa CmC|y!lCs!rCp!OCCCCl5.vlllOOO5) anO
5.OUlU5 atCtCmCs!COmmCn C!C|CglCagCnIs
ln CncOCarO!s.
Coronary 0rtery D|sease
Coronary arfery discasc |srarcin chiIdhood,luffhc
afheroscIeroticroccss acarsfolcgin carIyin Iile.
1hcrc is cvidcncc thafrogrcssionolafheroscIcrotic
Icsions is inIucnced ly genetic Iacfors [laniIiaI
hycrchoIcsfcroIcnia) and IilesfyIc [cigarcffc snoI
ing, bigh-cboIesfcrol dief, high-safurafcd-Iaf dict) .
Bccausc nany Iilcfinc halifs arc lorncd during
chiIdhood, fhe oorfunify exisfs Ior rcvenfion ol
coronaryarfcrydiseasc.
W UNC|DN0L H 0R D|Sc 0S
Myocard|t|s
Mosf cases ol nyocardifis in lorih Arerica re-
suIf Iron viraI inlecfion ol fhc oyocardiun, rc-
doninanfIy enfcroviruscs [coxsacIie B virus and
cchovirus) . lfisuncIcarvhefhcroyocardiaIdanage
lron viraInyocardifisrcsuIfsIron dirccfviraIinva-
sion or an aufoinnunc antilody resons:.
LHCD NDHE5fDfOH5
Icending on fhc dcgree ol danagc fo thc
nyocardiun,atienfs nay lc asynfonafic and the
diagnosis nay lc nadc onIy ly lnding SJ- andJ-
d4
w

o tor c > : nc rnoa : >


ucrrIO |atrCs
< cx v cxanr-onnccun: nvcm o- on-. y >
vavc changcs on an FCC donc lor an unrcIafcd
rcason, vhcrcas ofhcrs nay rcscnf vifh luIninanf
congcstivchcarflailurc. ConnonsynfonsincIudc
lcvcr,dysnca,latiguc,andchcsfair [usuaIIyducfo
a sccondary cricardifis) . Signs |ncIudc fachycardia,
cvidcncc ol congcsfivc hcari laiIurc, and
S_ vcnfricuIar gaIIo. Jhc FCC oltcn rcvcaIs SJ-
scgncnfdcrcssion andJ-vave invcrsion, as vcII as
arrhythnias and conduction dclccfs.Jhc chcsfradi-
ograh varics lron niId fo narIcd cardioncgaIy.
Fchocardiogran dcnotcs diIafcd or hyoconfracfiIc
vcnfricIcs, or bofh. PcricardiaI cIusion nay lc
rcscnf.FndonyocardiaIliosynaylcindicafcdin
scIccf cascs fo conIrn diagnosis. \iraI cfiology
shouId lc cvaIuafcd ly viraI cuIfurc and PCR lron
fhc throaf, sfooI, lIood, and cricardiaI Iuid, il
rcscnf.
lEDfHEHf
Jhcray lor aficnfs vifh viral oyocarditis is su-
ortivcfonainfaincrlusionandoxygcnation.Jrcaf
vcnfricuIar arrhyfhnias, conducfion alnornaIitics,
andcongcsfivchcarilaiIurcasindicafcd.Infravcnous
innunogIoluIir is givcn fo oininizc lurihcr
danagc fo fhc nyocardiun. Jhc rognosis lor
aticnfs vifh nyocardifis dccnds on the cxfcnf ol
nyocardiaI danagc.
D||ated Card|omyopathy
Iilafcd or congcstive cardionyoafhy is charactcr-
izcd ly nyocardiaI dysluncfion and vcnfricuIar
diIafation./Ifhough usualIyanidioafhicdisordcr,if
can lc causcdlyncuronuscuIardiscasc [Iuchcnnc
nuscuIar dysfrohy) or drug foxicify [anfhracy-
cIincs) . IiIation ol fhc lclt vcnfricIc rcsuIfs in
congcsfivc hcari laiIurc. 1 incrcasc in Iclt afriaI
rcssurc,uInonaryvcnousrcssurc,anduInonary
caiIIary vcdgc rcssure rcsuIfs in uInonary
cdcna.
LHCD NDHE5fDfOH5
Synfons incIudc dysnca, orfhonca, and arox
ysnaI nocfunaI dysnca. FvcnfuaIIy, righf hcarf
laiIurc vifh dccndcnf cdcna occurs, and a pulsus
aIfcnans nay lc nofcd. Cn cardiac cxanination, a
righf vcnfricuIar hcavc and an S_ galIo arc lound.
JheFCCrcvcaIsrhythndisfurlanccs,IcItvcnfricu
Iar hycrfrohy, and nonscciIc SJ- and J-vavc
ischcnic changcs. \cnfricuIar luncfion is cvaIuafcd
lycchocardiogran.
lEDfHEHf
McdicaI fhcray incIudcs inofroic agcnfs and
vasodilaforsfo inrovcnyocardiaIconfracfiIifyand
fo dccrcascthcaltcrloadonfhcvcaIcncdvcnfriclc.
Iiurctics dccrcasc rcIoad and hocluIIy inrovc
cardiac oufufly noving fhc diIafcd vcnfricIc fo a
rorclavoralIc osifion orfhc FranI-SfarIingcurvc,
and antiarrhyfhnic ncdicafions arc uscd to confroI
pofcnfiaIIy lafaI vcnfricuIar arrhyfhnias. llncdicaI
fhcraylails,hcarifransIanfationnaylcncccssary.
Hypertroph| c Card|omyopathy
/lso knovn as idioaihic hycrtrohic sul aoriic
stcnosis, hycrfrohic cardionyoafhy is an aufoso
oaIdoninanfgcnctic disordcrinvhichfhcvcnfric
uIarscfunisfhicIcncd,rcsuItinginIcItvcnfricular
oufIov fracf olsfruction. In fhc fhicIcncd sfillIcIt
vcnfricIc,diasfoIicluncfionisvcIIrcscrvcd,lufsys-
tolic lunction is conroniscd. /lnornaI oofion ol
thc nifraI vaIvcrcsuIfs innifraI insulIcicncy.
LHCD NDHE5fDfOH5
Synfons incIudc dysnca on cxcrfion, chcsf ain,
andsyncoc.JhcrcisoltcnalislcriousuIsc[doulIc
caIcd) lccausc cjcction is hindcrcd ly scfal
olsfrucfion, a vcnfricuIar gaIIo [S_), and ournurs
indicativcolnifraIrcgurgifafionandIcltvcnfricuIar
oufIov tracf olstrucfion. FCC ilIusfrafcs Iclt-axis
dcviation, Iclt vcnfricuIar hycrfrohy, and ossilIe
SJ- andJ-vavc changcs consisfcnfvifI ischcnia or
sfrain.Jhc cchocardiogran is diagnostic.
lEDfHEHf
JhcrayisccnfcrcdaroundrcvcntingIafaIvcnfric
uIar arrhyfhnias and dccrcasing fhc stillcss ol
fhc IcIt venfricIc vifh ncgafivc inofroic ncdi-
cafions, such as caIciun channcI llocIcrs, and
lcfa-adrcncrgic lIocIing agcnfs. Jhc avoidancc ol
concfifivcsorisiscsscnfiaIlccauscsuddcndcafl
duringcxcrfionis a signiIcanfrisI.
W 0RRHYHM|0S
/rrhyfhniasi nchiIdrcnarcnuchIcssconnonfhar
in aduIfslufcanlcjusfaslilcfhrcafcning./rrhyfh-
nias rcsuIf lron disordcrs ol inuIsc lornation,
inuIscconducfion,orlofhandarcgcncraIIycIassi
Icdas loIIovs.
< not tor zac i > < nc pLn popa i >
< cx v cxanr-rnncjcua: o
/`C
>
O | db a16r ar | C OQy
W
hN LW
M
J . NOs! CasCs CI myOCatOl!s n NOtthPmCttCatCsuI!
IrCm vltaI lnICC!CnCItC myCCatOlum.
2. LIatCOCtCCngCstlvCCatOlCmyCpa!ylsChataC-
!CrZCOOymyCCarOa|OysIunCtlCnanOvCn!tCu|ar
OtIa!a!tOn,!ts usua|IytOtCpathlC.
3. CtapyICtOtIa!CO CarOOmyOpaty nC|uOCs
lnO!tCplCagCn!stC tmptCvC |CI!vCn!rCu|arCOn-
!taCtt|l!yanOvasCOl|atCtstCOCCtCasCaI!Cr|CaO.
LtutC!tCsOCCtCasC ptCICaO,anOan!tatry!mtC
mCOtCa!lCnsatCusCOtC CCnttC| pOtCn!ta||yIataI
vCnttCu|atarrhytmtas.
4. |nhypCt!tOpCCarOOmyOpaty,!C vCntttCuIat
sCp!um s !CkCnCO,tCsu|ttngln |CI!vCnttlCuIat
OutIIOw !taC!OOs!tuC!tOn.
b. CtapyICtypCtttCplCCatOtCmyOpa!ytsCCn-
!CrCO arOunOprCvCn!ngIa!aIvCnttlCu|atattyI-
mtas anO OCCtCaslng !CstlI!nCssCItC|CI!
vCnttC|Cw!nCga!lvCnCttOptCmCOtCattCns.
Lrudyorrbytbmios
Sinus nodc dysIuncfion
Conducfior lIocI
7ocbyorrbytbmios
larrov RS
Vidc RS
|temotute Leots
/friaI
\cnfricuIar
Bradyarrhyfhnias arc fhc rcsuIf oI cifhcr dcrcs-
scd aufonaficify or llocI oI an inuIsc, vhcrcas
fachyarrhythnias or prcnafurc lcafs arise Iron
alnornal inuIse Iornafion causcd ly cnhanccd
aufonaficify, arccnfranfcircuif,orfriggcrcdacfivify.
/rrhythniasnayrcsuIfIroncongcnifaI,IuncfionaI,
or acquircd sfrucfural hcart discasc, clccfrolytc
disfurlanccs [ofassiun, calciun, and nagncsiun),
drug foxicify, oisoning, or an acquircd sysfcnic
disordcr. JalIc J-4 Iisfs cfioIogics rcdisosing
chiIdrcnfo arrhyfhnias.
8radya rrhyth m |as
/salrcadysfafcd,lradyarrhyfhniasrcsuItIronsinus
nodc dysIuncfion or conducfion lIocI. Bradycar-
dias due fo sinus nodc dysIuncfion includc sinus
lradycardia, j uncfionaI lradycardia, ccfoic afriaI
lradycardia, anJ sinus auscs. Bradycardias duc
fo conducfion llocI incIudc lrsf-dcgrcc bcari
lIocI, sccond-dcgrcc hcarf lIocI, and third-dcgrcc
[conIcfc) hcari lIocI. Sccond-dcgrcc bcarf
lIocI is Iurfhcr dividcd info Molifz fyc l lIocI
[VcncIclach),Molifzfyc ll lIocI, andlxcd-rafio
afriovcnfricuIar [/\) lIocI.
LElEHfD LD@HO55
Figurc J-I4 shovs Hc rhythn sfris oI various
lradycardias. 5inus bradycardia is associafcJ vifh
incrcascdvagaI fonc,hypoxia,ccnfralncrvoussysfcn
disordcrs vifh incrcascd infracraniaI prcssurc,
hyofhyroidisn, hycrIaIcnia, hyofhcrnia, drug
infoxicafion [digoxin,lcfa-lIocIcrs,caIciun channcI
lIocIcrs), andrior afriaIsurgcry. Ifis also anornaI
Inding in hcaIfhy afhIcfic fccnagcrs. Jhc FCC
rcvcaIsanornaIPvavcvifhnornaI/\ conducfion
afrafcsIcssfhan I 00lninthcnconafcand60ln
in iheoIdcrchiId.Whcnsinus lradycardialcconcs
foo sIov,sinusauscsorcscac rhythos nayoccur.
JhccscacrhyfhnsnosfoItcr sccnincIudcccfoic
afriaIlradycardiaorccfoicafriaIrhythn,j uncfionaI
lradycardia orj uncfionaIrhythn, ora sIovidiovcn-
fricuIarvcnfricuIarrhyfhn.
Firsf-dcgrce hcari bIocI usuaIIy rcsuIfs Iron
sIovingoIafriovcnfricuIarconducfionaffhcIcvcIoI
fhc/\ nodc.IfisassociafcdvifhincrcascdvagaIfonc,
digoxin and lcfa-lIocIcr adninisfrafion, inIccfious
cfioIogics[viraInyocardifis,!yncdiscasc),hyofhcr-
nia, cIccfroIytc alnornalifics [hyo/hycrIaIcnia,
hyo/hypcrcaIccnia, hyonagncscnia), congcnifal
hcarf discasc [/SI, afriovcnfricuIar canaI dcIccf,
Flsfcin's anonaly, J/P\C, and -fransosifion oI
fhe grcaf aricrics or "corrccfcd fransosifion),
rhcunafic Icvcr, and cardionyoafhy. Firsf-dcgrec
/\ lIocI is characfcrizcd on FCC ly PR infcrvaI
rolongafionIoragcandrafc.Jhc rhyfhn isrcguIar,
originafcs in fhc sinus nodc, and has a noroaI RS
norhoIogy.
Sccond-dcgrcehcarflIocIrcIcrsfocisodicinfcr-
rufion oI/\ nodaI conducfion.
Nobitz type [enckebach} dcnofcs rogrcssivc
proIongafion oIfhe PRinfcrvaI ovcr scvcraI lcafs
unfiI a RS is drocd. Jhis cycIc rccafs ifscII
oItcn,aIfhoughfhcnunlcroIlcafsiracycIcnay
noflc consfanf.JhcRSconlgurafionisnornal.
FfioIogicsIorfhisrhythnarcfhcsancasfhoscIor
lrsf-dcgrcchcarilIocI.
Nobitz type is causcdlyalruf IaiIurc oIafri-
ovcnfricularconducfion lcIovfhc/\ nodcinfhc
< not tor a > .< nc ) oram i >
llucr| n1O eU|!$
< cran u cxanr-ronncjcun: HCA o- on-. jy>
1PLL 5
ctOfS lfeOSOSng tO OSfhthmS
LCnQen|!| hCr!O|$ese
5uprven!r|Cu|rOySrhy!hm|$.Lb$!e|n'$ nOm|y|my|sCpresen!w|!|W|W$ynOrOmC),!r||sep!|Oe!eC!$,!r||
$u|QCry, |-!rnspOs|!|Cn C!!hCQ|e!r!e||e$,!!er |OnInOpCr!|On
VCn!r|Cu|rOysrhy\hm|S:Or!|Cv|vCO|SC$e,pu| mOnryv|vCO|$C$e,!!Cr!C!r|OQyO! |||O! rep|r,nOm|Ous|e!!
COrOn|yr!ery, KVOySp|S|
Her!b|OCk|vry|nQOCQrCCs).!|erOpen-her!SurQery|Lb$!e|n'$ nOm|y,|-!rnSpOS|!|On O!!|CQrC!r!C||CS,
COmmOn !r|OvCn!r|Cu|rCn|,V5L rep|r),CCnQCn|!|COmp|C!C hC|| b|OCk||O|Op!||C,$$OC|!eO w|!| m!ern|
sys!em| C |upu$ Cry!hem!O$us,|-!rnspOs|!|OnO!!heQre!r!er|es)
|sO|!eO COnOuC!|On sy$!emO|$OrOers
W|WsynOrOme
|rO|OnQeOQ1|n!erv| synOrOmCs
As$CC|!eOw|!|Sys!Cm|C||| neSs
| n!eC!|Ou$ myCCrO|!|S
KwSk|'sO|se$e
| O|Op!h| CO||!eO Orhyper!rOph|CC|O|OmyOp!|y
|r|eOre|Ch's !X| |!r|| !ChyCrO| Orbr|| |!|On)
NuSCu|rOyS!rOph|e$|LuChenne,per|OO|Cpr|ys|s)
G|yCOQens!OrQeO|$e$e$||Ompe'$ O|se$e)
LO||Qen vsCu|rO|seSC$ |rhCUm!|CCrO|!|s,$y$!em|C| upu$ery!hem!O$us,pCr|r!er|!|s nOOOs,Oe|m!OmyO$|!|s)
LnOOCr|neO| sOrOer$ ||ypCr!hyrO|O|$m,Oren|Oy$unC!|On)
Ne!bO||CnO e| eC!rO|y!e O|$!urbnCes|hypOmQnC$Cm|,hyperk|Cm|,hypOC|Cem|,|ypOX|)
LymeO|$ese
LruQ!OX|C|!y
LhemO!|erpeu!|CQen!$|n!hrCyC| | ne$)
1r|CyC||C n!|Oepressn!s
LOC|nC
L|Q|!||$,be!-OrenCrQ|C b|OCker$,C| C| umChnne| b|OCkers
As!hm meO|C!|Ons|$ymp!hCm| me!|C$)
L!hCr Cuse$
b|un! Che$!!rum |myOCrO|| COn!u$|On)
| nCrCseO |n!rCrn|| prC$surC
lundIc olIis-PurIinje llcr sysfcn. If is a norc
scriouslradycardiafIan Irsf-dcgrcchcarflIocIor
VcncIclach lccausc if can rogrcss fo conIctc
hcarfllocI.CnFCC,fhercissuddcn/\ conduc
fion lailurcvifb a droed RSaItcra nornaI P
vavc. lo reccding PR infcrvaI roIongafion is
sccn |n oornaI conducfcdinuIscs.
ied-ratio A bIock isanarrhyfhniainvhichfhc
RS conIcx loIIovs onIy aItcr cvcry sccond
[fhird or |ourfh) P vavc, causing 2. [J. or 4.I)
/\ llocI. Jhcrc i s nornaI PR infcrval i ncon-
ducfedlcafs.Thcrc is usuaIIy a nornaI orsIighily
roIongcd RS. Fixcd-rafio llocI rcsuIfs Iron
cifhcrA\ node orIislundIc in]ury, andinfracar-
diacrccordings arcrcquircdfodisfinguishthesifc
olinjury. Paticnfsrayrogrcss fo conIcfc hcarf
lIocI.
Jhird-dcgrcc hcarf lIocI occurs vhen no afriaI
inuIscs arc conducfcd fo fhcvcnfricIcs.JIe afriaI
rhythnandrafcarenornaIlorfhcaficnf'sagc,and
thcvcnfricuIarrafcisslovcdnarIcdIy[4055ln) .
llan cscae rhythn ariscs Iron fhc/\ nodc [junc-
fionaIrhythn),fIc RS infcrvaI is olnornaI dura-
fion, luf ilan cscac rhythn ariscs |ron thc disfaI
HislundIcorPurIinjcIlcrs,thcRSinfcrvalisro-
Iongcd [idiovcnfricuIar rhyihn). CongcnifaI con-
lcfc/\ llocIcanlcanisoIafcdalnornaIifyorcan
< not tor zaci > < nc pLn ppa i >
t|lU5 O|3OyC3Ol3
ll|5I-OCQ|OOlVOlOC|


| OIZ I |
W ww W
O | yjO

[VC|CkOO3C| j|O|O|O|O|J
|OO|IZIyjO

Z 1 lV OlOCk
LOjlCIO [I|l|O-OOQOO)
lV O|OCk


l |

||gured-1 4 brOyrrhy!hms.
lcassociafcd vifh i-fransosiiion olfhcgrcafarfcr-
ics, afriovcnfricuIar canaI dclccf, or nafcrral Iuus
cryflenafosus. Cfhcr causcs includc ocr-hcari
surgery [cscciaIIy aItcr Iargc vcrfricuIar scfaI
dclccf cIosurc), cardionyoafhy, or lync discasc.
lcvlonsvifhcongcnifaIconIefchcarflIocInay
rcsenfvifh hydrosIcfaIis.
lEDfHEDf
Noinfcrvcnfionis ncccssaryIor sinus lradycardia il
cardiac oufuf is nairfaincd. / nanagcncnf aIgo
rifhnlorsinuslradycardia isshovnin Figurc J- I 5.
lo frcaincnf is ncccssary lor lrsf- or sccond-
dcgrec hcarflIocI [Molifz fyc I) . Molifz fyc II,
Ixcd-rafio/\ lIocI, andfhird-degrcchcarflIocIaIl
rcquireaccnaIcrlaccncnf.InMolifzfycIIand
lxcd-rafio/\ lIocI, rohyIacfic accnaIcr inscr-
fior is csscnfiaI to rofccf fIc aficrf shouId hc or
shcrogrcssfoconlcfchcarflIoclvifIiradcquafc
cardiacoufufavayIronncdicaIcarc.
IIfhc chiId vifh conIcfc heari lIocI is hcno-
dynanicalIyunsfalIc,franscufaneous orfransvcnous
acing can lc crlorncd acufeIy, and crnancnf
fransvcnous or cicardiaI accnaIer laccncnf can
lc crIorncd Iafcr. Jhird-dcgrec hcarf llocI is
nanagcd vifh cifhcr vcnfricuIar dcnand acing or
/\ sequcnfial acing. Figurc J- | 6is a nanagcncnf
aIgorifhnIor/\ lIocI.
achyarrhythm|as
Narrov-conlcxfachycardiashavea PS norhoI
ogy siniIar or idcnfical fo fhaf ol nornal sinus
rhythn.1hcy includc nosf,IufrofaII, S\1s [sonc
S\1shavcavidcncdRS) .Narrov-conIcxfachy-
cardiasnaylcducfoincrcascdaufonaficifyorlron
a rccnfranf circuif. larrov-conIcx tachycardias
duc fo incrcascd aufonaficify ircludc sinus fachy
cardia, ecfoic afrial fachycardia,juncfional ccfoic
fachycardia, ard afriaI IlriIIafior. Narrov-conIcx
fachycardias causcd ly rccnfrarf ncchanisns arc
catcgorizcd as orfhodronic rcentranf tachycardia
[CRT) oranfidronicrccnfranffachycardia [ARJ) .In
CR1the S\1roagafcsdovnfhe/\ nodcandu
fhc lyass fracf. Sincc fhcvcnfricIesarcdcoIarizcd
infhc nornaI lashion, dovn thc A\ nodc, fhc RS
conIexisnarrov.In/R1fhcS\Troagafcsdovn
fhc lyassfracf and u fhc/\ nodc. Sinccfhc vcr-
fricIcs arc dcoIarizcd dovn fhc lyass fracf, fhc
RS is vidcncd. larrov-conlex/\ rccitocafing
fachycardias incIudc/\ nodcrccntranffachycardia,
VPVsyndroncorihodronictachycardia [acccssory
afhvayrofconccaIcdon FCC-eIfavavc),orf!o
dronic afriovcnfricuIar rccirocafing fachycardia
[acccssory afhvay conccalcd on FCCno dcIfa
vavc), sinoafriaI rccnfranf fachycardia, and afrial
Iuffcr. larrov-conlcx fachycardias arc rcIafivcIy
vclI folcrafcd acufcIy.
Converscly,vidc-conIcxfachycardias,dclncdas
fachycardiasvifI aPSnorcthan0. I 2scconds, arc
a nedical cncrgcncy Vidc-conIex fachycardias
include vcnfricular fachycardia, venfricular IlriIla-
tion,VPVsyndroneanfidronicreentrantfachycar-
dia, and orfhodronic S\1vifh alerrancy.
LDElEDfD LD@DO55
Figure J- I 7 shovs fhc rhyfhn sfris oIfhc various
iachycardias. 1hc causcs oI tachyarrhyfhnia arc as
loIlovs.
lotrou-Com/ex 7ocbycotdios
Sinus tocbycotdio. Fcvcr, sfrcss, dehydrafion, and
arenia
CRT [most common non-sinus tocbycotdio S\:
Mosf cascs rcsuIf Iron a conceaIcd lyass fracf
causing CR1 A\ nodc rccnfrarf fachycardia,
VPVsyndronc CR1, Flsfcin's aronaly [associ
afcdvifhVPVsyndrorc), |-fransposifionoIfhc
grcaf aricrics
.ttio/ ]|uttet. /frialsurgcry [-1C/s/Musfard/
Scnningroccdurc,/SI s/ rcair Icni-Fonfan,
Fortan), nyocardifis,sfrucfuraI hearidiscascvifh
I >
< cx v pexaax~xouaejcvn: HYCA sT jouT. jy >
l |U5 O|3OyC3|Ol 3

lI|Ojl|C
. Z|Q}kQ
|j|Cj|l|C
1 |JQ}kQ
|5Oj|OIC|C|Ol
l|U5lO| OOQl| 2I
. b. 1
l
)Q}kQ}|l|
|3Cl|Q
[I|3|5VO|OU5 O|
I|3|5CUI3|OOU5J
XyQC0
LOIO|l|O
C3U5O
Ilgure d-1 b NnQement|QCr|!hm!Ors|nusbrOyCrO|.
diIafcd afria [Flsfcin's anonaIy, fricusid afrcsia,
rhcunatichcarfdiscascoIfhcnifraIvaIvc),scvcrc
fricusidrcgurgifation
.tt|o/ ]/r|//otion. Mosf oItcn sccn vifh IcIt afriaI
cnlargcncnf[rhcunatichcarfdiscascolfhcnifraI
vaIvc,\SI,sysfcnicfouInonaryarfcryaIIiafivc
shunfIaccncnf)ofhcrcauscs ihafrcsuIf |nrighf
afriaI or liafriaI cnIargcncnf incIudc Flsfcin's
anonaIy,VPVsyndronc, andnyocarditis
| |C|C35CO
l|I|3C|3|l3l j|C55u|O:
1|C3I l L|
myjC|VC|Ill3IlO|,
|3||lIOl
cOOCId/O!b
J|O3I
|yjC|IC|5lVCC|l3l3,
lyO|3l3Zl|O
|3OOIOlOl
|lI|Oj|U55lOC
\ide-Com/ex 7ocbycotdio
\entr|cu/ot tocbycotdio. Congcnifal or acquircd
hcarf discasc rcsuIting in vcnfricular diIation or
hycrfrohyor vcnfricuIar sufurcIinc, drug ingcs-
tion, orVPV syndronc/RJ
\entr|cu/ot ]/r|//otion. JcrninaIrhyfhnfhafdcvcI-
os aItcr hyoxia, |schcnia, or high-voIfagc cIcc-
fricaI injury rcdisosing Iacfors incIudc VPV
syndronc andIong J syndronc
< not foz saJe I > < ue pLn popa I >
lV OlOCk
> ||OlO|QOO || l |IO|V3l

I C I C

[5OO /OX[
|OOlI7 | [VO|CkOO3C|J
|| l |IO|V3| j|OQ|C55lVCly
j|OlO|QOO u|Il l L| O|OjjOO
L|, |OOlIZ ll - O|OjjOO
vlI|OUI j|OCOOl |Q j|OlO|Q3IlO|
O '| l |IO|V3l. L| |O||3l
L|OllOv5 OVO|y
Z|OO||O lv3VO
lO| O! LOjlOIOOl 55OCl3I
3I|l3l 3|OVO|I|lCUl3
VO|I|lCUl3||3IC|UC
I|3| 3I|l3l|3IO [4
|3CLlVlIy,
| 5lOvO|
bb OO3L5}l |J
IIgure d1 NnQemen!|QCr|!hm!C|AV b|Cck.
l|U5 I3C|yC3|Ol3
L|I|OO|O|lC
|OO|I|3|I
I3C|yC3|Ol3
lI||3'luIIO|
lI|l3l lO|ll l3IlO|
VO|I|lCul3|
I3C|yC3|Ol3
VO|I|lCUl3|
lOlll |3IlO|
Ilgure d-1 J TchyOysrhy\hm|s.
lEDfHEHf
|attCw-LC|p| Cx1aCyCatOla
JrcaLncnLoIsinusLachycardiainvoIvcscorrccLingLhc
undcrIyingcauscoILhcfachycardia.FigurcJ-I S ouL-
Iincs a nanagcncnt algorifhn lor suravcnLricuIar
Lachycardia. JrcatncnL lor sLalIc narrov-conIcx
l|OnOOy|3|lC3lly
U|5L3OlC, QlVO
Cl|Oj||l|C 1 )Q}|Q,
3I|Ojl|OU. Z|Q}kQ
| |O|3CIO|y

IO|OOlC3IlO|5
1O|jO|3|y |O||3|C|I
j3CO|3kO| I|3|5VC|Ou5 O|
I|3|5CUI3|OOU5 CjlC3|Ol3l
O|I|3|5VO|Ou5 j3CO3|O|
LachycardiarogrcsscsIronvagaInancuvcrsLohar-
nacoLhcray Lo cardiovcrsion. VagaI nancuvers
cnIarccvagaItoncfoslovconducLioninLhc/\nodc
and oItcn rcsuIf in fcrninafion ol Lhc arrhyLhnia.
\agal Lonc is incrcascd ir inlanLs ly aIying icc to
Lhc Iacc, and in oIdcr chiIdrcn Lhrough caroLiJ
nassagc,naIcsurcfoIccfhcinlanL'sairvayunol-
sLrucLcJ vhcn aIying icc Lo Lhe Iacc. Il vagaI
nancuvcrs arc incIlccLive in sLalIc narrov-conlcx
fachycardia, adcnosinc isgivcrLolIocILhc/\ node
Lo lrcaIarccnfranLS\vhosc circuiLinvoIvcsLhc
/\ nodc [/\ nodc rccnLranL Lachycardia, VPV
syndronc viLh CR} conccaIcd lyass LracL CRJ) .
/dcnosinc vilI lc incIlccLivc on a narrov-conIcx
Lachycardia thaL rcsuILs lron incrcascd auLonaiiciLy
or a rccnLranL occhanisn Lhat docs noL invoIvc Lhc
/\ nodc [sinus Lachycardia, ccLoic aLriaI fachycar
dia, j uncLionaI ccLoic Lachycardia, aLriaI lluLfcr, or
sinoaLriaIrccnLranLLachycardia) .IladcnosincreLurns
fhcchiIdfonornaIsinusrhythnandVPVisnoLsus-
ccfcd [nodcIfavavcsccn aItcrconvcrsionol Lachy-
cardia), thcchiId is sLartcdon digoxinLorcduccLhc
risIoIIuLurc cvcnLs.Iladcnosinc rcvcaIsVPVsyn
dronc [dcILa vavc noLcd aItcr convcrsion olLachy
cardia),uscalcLa-lIocIcr,lccauscfhcuscoldigoxin
can sIovLhcA\nodcandscedu conducLionovcr
fhc acccssory aLhvay in an anLidronic lasbion and
4
I >
< cx v pexaex~xouaejcvn: HYCA sT jouT. jy >
tUj|3VO|I|lCU|3|I3C|yC3|O|3
O|5I3OlC
3llU|O. |yjOIC|5lO|
L3|OlOVO|5lO|
.ZbZ. J}kQ
O|
LVO|O||VO j3Cl|Q
|OCO|VC|5lO|
U|SIBD|C
lOO|O5l|O bU|IQ | VOOlU5
LOUOlO OO5O UjIO 4U Q
l . O. , 1 , Z, 4
|OCO|VC|5|O|
SIC|C
LpI|O|S
Ll Ql I3llz3IlO| O|
||Oj|3|OlOl[.b|Q}kQ} l VO|
L5OlOl b|Q}|Q}|l| OVO|
1 l |. I|C| b|Q}kQ}|l| lV
[IlI|3IOIOOlOCIJO|
lnlOO3|O|CZ.b
l V3|O}O|i\/tnr|
Igure 3-1 MnQemen!|QCr|!hm!Cr$uprven!r|cu|r!chycrO|.
||yI||
CO|VO|5lO|
L||O|lC |3|3QCC|I
Ol QOXl |
[355U|||QV|V
5y|O|O|O |OLj|O5O|IJ
O|-OlOCkO|
[l V|V5y|O|OO
*t<cr)\
rcsuILinvcnLricularllriIIaLionscconJaryLoaLrialll-
riIIationorsoncoLhcrIasLaLriaIarrhyLhnia.ForLhis
rcason,digoxinshouIJlcavoiJcJtoLrcaLCRJasso-
ciaLcJviLhVPVsynJronc.ProranoIoIisancIlcc-
LivcanJsalcaILcnaLivcLoJigoxininCRJassociaLcJ
viLh VPV synJronc. Vhcn unsLalIc narrov-
conlex LachycarJia is rcscnL and Lhc aLicnL has
congcsLivcbcarLIaiIurcorhyoLcnsion,carJiovcrsion
orLransesohagcaIovcrdrivcacingisindicaLcJ.Syn-
chronizeJcardiovcrsionisrcquircJLoavoiJLhcinaJ-
vcrLcnLdcvcIoncnLolvcnLricuIarllriIIaLion.
!n unsLalIc aLriaI lluLLcr, synchronizcJ carJiovcr-
sion or ovcrJrivc acing is uscJ vhcn raiJ inLcr-
vcnLion is ncccssary lccausc ol congcsLivc hcarL
< nct fcz sa1e| * ue pLn pa |
||gure -TV N|QCC|I |QC:|I| C|
VC|I:|CU|:IC|yC|U|.
* cau n pexaenxouaeycun: HYC s you. y >
L|QIC| 3 / L|O|O|OQy " 4T
Ven|||Cu|a| |aCyCa|Cia
T. /HCs
. cyOC|C.eC Ca|C |Cv|s|CO
[U.b. U J!|Q)
(|jea|)
||CCCa|O T uQ!|Q| VX 2
lC||CweC Uy
||CCCa|OeU-bU Q!kQ!u|O
cjeCi||CH|C|CruQ-iOCuCeC V1
L|QU|OC |C|C|Q||a'|s
PC Cl0DI Da|S
Hb USUBlly WO0O
PvOSSDClBllD0
|B!` T-C|0l0U!
Us|C0 Oa|s
||CCCa|Oe T uQ!|Q X |V |CaC
!U-tU Q!|Q/u|O
j|CCa|OauCeT uQ!kQ|V 'CaC
Cve| u|Ou|s
auICCa|COe.b uQ!kQ|V
jLCOC|us au|CCa|CO
aOC |CCa|OaulC|CQ|e|
UC| j|C|CnQ!eC1 in||va|
aOC UC|0ayCaus yC|eOsiCO]
|a|CC
_
|C| |(|CyC||C aO||C|essaO|s
|eOy|C|O |C|CCCa|Oe. C|QC|O. C|Uuj|vaCa|Oe
[iU|CC|e(sC|uaQOes|uusu||a|e[bU uQ!kQ|V)
|C| |C|saCe Ce jC|O|es
IaiIure. Cncc cardiovcrsion las occurrcd, digoxin,
Iera-IIocIcrs, procainanidc, aniodaronc, soraIol, or
a quinidinc/digoxin conIinarion nay Ie givcn ro
lcIp prcvcnr rccurrcnccs. II rle cliId is lcnody-
nanicalIy sraIIc, lc or slc slould Ic Ioadcd vitl
digoxin and rlcn givcn procainanidc in an atrcnpf
ro convcrr tlc arrlyrlnia. |r is crirical ro load
virl digoxin IcIorc giving procainanidc, Iccausc
procainanidc las vagoIyric activity rlat couId
inadverrcnrIy incrcasc rlc vcnrricuIarratc and causc
acufe lcnodynanic dcrcrioration.
a|cvdays,anticoaguIarionis ncededIc|orcconvcrr-
ingflerlyrlnrodccrcasetlerisIoIcnIo|izarionoI
possiIleinrra-atriaIcIots.naIrernarivefoanricoagu-
Iation is rranscsoplagcaI cclocardiograply ro asscss
|or cIots. I| no cIots arc sccn, cardiovcrsion nay
proceed, aIflougl vitl a sIigltIy incrcascd risI oI
tlronIocnIolisnrcIativcroanricoaguIarion.uini-
dinc,procainanidc,oraniodaronecanIccIccrivcin
plarnacologic convcrsior oI atriaI IIrilIation, and
quinidincandprocainanidcarcgoodIong-fcrnnain-
rcnancc drugs. Synclronizcd cardioversion convcrrs
nosrcascstosinusrlyrln. |larriaIIIriIlarionlasIcen presentIornorctlan
< nor foz sa1e| > < ue pLn nyopa | >
||uc
[
r|nJs |CO|I||CS
< cxau n pexaax~xouaeycun: HYC s you. |V >
4Z
VeO|||Cu|a|l|U||||a||CO
VeO|||Cu|3||aCyCa|C|a
CaC||C
vei|||Cu|a| ja||e|O
||gure -ZU N|QCC|\ |QO||\| O| VC|I||CU||
O||| |I|O|.
W|de-Lomplex1achycarO|a
Trcar vidc-conpIcx vcnrricuIar racIycardia duc ro
\F\ syndronc virI anfidronic conducrion or
ortIodronic S\ virI aIcrrancy as tIougI rIc
paticnt Ias vcntricuIar racIycardia. Iyporcnsivc
or unrcsponsivc paricnts sIouId Ic rrcatcd in-
ncdiatcIy vitI cardiopuInonary rcsuscitation and
syncIronizcd cardiovcrsion. Itcr cardiovcrsion,
sinus rIyiIn can Ic naintaincd virI intravcnous
lidocainc or aniodaronc. lornorcnsivc paticnrs
vitI acutc-onsct ventricuIar racIycardia can Ic
trcatcd vitI intravcnous Iidocainc oraniodaronc in
an atrcnpt to lrcaI tIc arrIyrInia vitIout
cardiovcrsion.
CIiIdrcn vitI vcntricuIar IIrilIation sIouId
rcccivc CIR andnusf IcdcIIriIIatcdvirI nonsyn-
cIronizcd cardiovcrsion. Civing cpincpIrinc nay
turnIncIIriIIationinro coarscIIriIIarionandaIIov
succcssIuIdcIIriIlarion.TIcnanagcncnraIgorirIns
Ior vcntricular tacIycardia and vcntricuIar IIriIIa-
rion/puIscIcss vcntricular tacIycardia arc outIincd in
!igurc J- IJ and Figurc J-20,rcspcctivcIy.
M
LN LW
1
1. 6radyarrhythm|asw|thw|denedQRScomplexes
are | | ke|ytobeescape rhy\hmsfrom the H|s
bund|eorPurk|njesystem(|d|oventr|cular
rhythm)andareath| ghr|skforprogress|onto
completeheartblock.
Z. Symptomatics|nusbradycard|a,second-degree
heart block (Mob|tztype || andhxed-rat|oAV
b|ock),andth|rd-degree heartb|ocka|lneed
pac|ng.
d. Narrow-comp|extachycard|astendtobewe||tol
eratedacute|y,whereasw|de-complextachycar-
d|asarecons|dereda med|calemergency.
4. 1reatwide-comp|extachycard|a duetoSVT(WPW
syndromew|thAR1orSVTw|thaberrancy)as
though thepat|enthasventr|culartachycard|a.
b. WhentreatingSVT,ru|eoutWPWsyndrome,
becausethetreatmentforWPWassoc|atedSVT|s
d|fIerentfrom thatfor non-WPWSVT.
DEVELOPMENTAL M|LESTONES
lcuroIogic, intcIIcctuaI, and pIysicaI dcvcIopncnr
in inIanrs and cIiIdrcn cacI occur in an ordcrIy
andscqucntiaInanncr.TaIIc4-IIistsrIcnornaIpro-
grcssion oIdcvcIopncnraI niIcsroncs. 1Ic inIorna-
tion is suIdividcd inro gross notor, visuaInoror [or
Incnotoradaptivc),Ianguagc,andsocialnilcstoncs.
TIe Lvo dcvcIopncntaI scrccns nosf connonly
uscdIypcdiatricians arctIc LcnvcrIIdcvclopncnral
scrccning rcst and tIc CIinical /daptivc 1cst [C/T)/
CIinical Iinguistic and /udirory Milcstonc ScaIc
[CIMS).TIc Lcnvcr II dividcs strcans oIdevcIop-
ncnt inLo gross notoj Inc noLor-adaptivc, Ianguagc,
andpcrsonaI-social.1IcC/TrarcsproIIcn-soIvingand
visual notoraIili_andtIcCI/MSasscsscsIanguagc
dcvcIopncnt Iron IiriI ro J6 nontIs oIagc.
Sonctincs tIc dcvclopncntaI proccss docs not
progrcss appropriatcIy, and dcvcIopncntal disaIiIi-
tics nay Ic suspccrcd. /InornaI dcvcIopncnt can
Ic suIdividcdinto dcvcIopnentaIdcIay,dissociation,
and dcviancy. LeveIopmentaI deIay reIcrs to a pcr-
Iornancc signiIcanLIyIcIov avcragc in a givcn sIiIl
arca. / deveIopmentaI quotient [L) Iclov 70
constitutcsdcvcIopncntaIdcIay.TIcLrcIccrsrIe
cIiId's rarc oIdcvcIopncnt. L " [dcvclopncnraI
agc cIronological agc) I 00.
LeveIopmentaI dissociation rclcrstoasuIstantiaI
diIIcrcnce in tIc ratc oIdevcIopncnr Icrvccn tvo
sIiII arcas. /n cxanpIc oIa dcvcIopncnraI discrcp-
ancy IcLvccn gross notor and Ianguagc dcvcIop-
ncnr is a cIiId vitI isoIatcd ncnraI rcrardarion
vIosc grossnotor dcvcIopncnris nornal.LeveIop-
mentaI deviancy rcIcrs ro nonscqucntiaI dcvcIop-
ncnt vitIin a givcn arca oIsliIl . !or cxanpIc, tIc
dcvcIopncnt oIIand prcIcrcnce at I 2 nontIs is a
dcparturcIron nornaI scqucncc and nayIcrcIatcd
to anaInornaIityoItIc otIcrcxtrcnity.
Ianguagc is tIe Icsr indicator oIIuturc intcIIcc-
ruaI acIicvcncnt. Ianguagc dcvcIopncnr isdividcd
into tvo strcans, rcccprivc and cxprcssivc, cach
assigncda scpararc L.
FrcnarurcinIantsrcquircagc-ad]ustcdparanctcrs
vIcn asscssing rIcir dcvcIopncntal acIicvcncnt.
!ntiI 2 ycars oI agc, a cIiId's agc sIould ralc inro
account LIc gcsrarionaI agc aL IiriI. IorcxanpIc,ar
Iis or Icr 9-nonrI cIcclup, a Iorncr prcnature
inIantIornar 28 vccls' gcsrarion sIouIdIc aIIc to
pcrIornsliIIsappropriarcIor 6-nontIoId.
VAR| AT| ONS |N DEVELOPMENTAL
PATTERNS
Attention Decit Hyperactivty Disorder
Attention dehci| hyperactivity disorder [ALlL} is
a syndronc conposcd oIinattcnrion, Iypcractiviry,
and inpuIsivity to tIc cxtcnt tIat tIc IcIavior is
naIadaptivc and inconsisrcnrvirI rIc dcvcIopncn-
ral stagc oItIccIild./L!L nayIcIoundin 57 oI
girIs and I 07 oI Ioys in cIcncntary scIooI. Ip ro
707 oI tIosc aIccrcd vitI /L!L as a cIiId vilI
Iavc pcrsistcntsynprons inro aduItIood.
LDC0 N0Dl5l0lDD5
Ior a diagnosis oI/L!L to Ic nadc, a cIiId nusr
nccr tIc critcria outIincd in tIc LSM-!\ [1aIIc 4-
2) .TIcdiagnosisoI/LILrcquircs tIc prcscnccoI
inarrcnLion, Iypcracriviry, and inpuIsivcncssinnul-
tipIc cnvironncnts[c.g.,inscIooIandarIonc) .TIc
synptonsnustIc prcscnrIorar Icast6nontIsand
arc usuaIIy prcscnf Iy agc 7. Hovcvcr, rIc signs oI
/L!L nay Ic nininizcd in scrtings rIat arc aIlc
ro providc inncdiarc rcinIorccncnt, arcncvrotIc
cIiId, or are IigIIy supcrviscd.\iLI tIis in nind,.a
< not foz sa1e | > < ue pLn nyopa | >
C|uOprlrlO |CO|I||CS
< cxou n )eaan~xouaeycun: HCA s pu. @ >
44 "
1RbLb 4-1
LODDODl UZZ0O L0V0lOQD0Dtl Nl0StOD0S
P@6 LfOSS NOlOf DC [VSUl) NOtOf LDUC bOC
1 O|I| H|SCS |COS| |Q|Iy O||OwS w|I| CyCSIO PC|IS/SI|I|CSIO |CS O| CC
|O Q|O|C |O| |CO|y, I|Q|I SOU|O
Q|SQ
Z O|I|S bI|6S |CSQO|S|VC|y HCCOQ||ZCS Q|C|I
3 O|IDS MO|OSDCO UQ, M|OS OQC| I |CSI LOOS HCC|CSO|| | |f
SICOy OO|CCISO| QCOQ|C
4b O|I|S HO||S|O|IIOOCK, L|SQS w|IDOOID L||C|ISIO VO|CC OjOyS OOSC|VOQ
OCKIOfOOI,SIS |OOSIOQCI|C| 6OV|ODCOl
wC|| SUQQO|ICO
O|I|S b|ISwC| | ||SC|S D|O IO bOO|CS HCCOQ|ZCSSI||QC|S
UOSUQQO|ICO D|O, |CCDCS w|ID
C|IDC| D|O
9 O|I|S L|w|S,C|U|SCS,QU| | S OSCS Q||CC|Q|SQ, bCQ||SIO USCOO/ ||yS QICKC
IO SIOO |QC|CCOS ,U|OC|SIOOS
OO
12 O|IDS W|KS|O|C D|OwS, |C|CSCS 1 wO|OS OIDC|ID| | |IICS,COCSwDC|
OOjCCIS OO/,O|OwS C||CO;COOQC|ICS
OO6SICQCOOOS wIDO:CSS OQ
1bO|I|S W|KSOCKw|O, bU||OS2~O|OCK
C|CCQSUQSI||S IOwC|,SC||OO|CS
1 O|IDS HUOS CCOSSC|[CSS||y) |O||ISIO OOOy Q|IS ||yS |OUOO [OOIw|I|)
w|I| UIC|S||S w|COSKCO OI|C|C||O|CO
2J O|I|S bQUIS |O |CCOVC|S bU||OSbO|OCKIOwC| wOwO|O COO||I|OOS
2 OOIDS W|KSwC|| UQ OO HCOVCSC|OI||Q O|OC|SI|OS2SICQ ||||C| Q|y
OOwO SI||S CO|OS,SI||QC|
U|OC|SI|OS '/ O
SQCCC|
3OOIDS DfOWSO\ PQQ|OQ|IC QfOOOUO KOOWSDfSI,\SI
OVC|D|O US6 |CS
3yC|S |CO|SI||CyC|C L|wS C||C|C 5wO|OSC|ICOCCS, USCS L|OUQ Q|y,SD|CS
Q| U|S,QSIIC|SC,
SI||QCf U|OC|SI|OS
_OSQCCCD
4 yC|S P|IC||ICSCCI QOOQ LICDCSO| |,O|CSSCS KOOwS COO|S | Q||I|VC Q|y
OOw| SI|S,SK|QS |O|C
b yC|S |CS SDOCS
cIild nay not display any signs oIL!L vIcn in
rIc pcdiarrician'soIIcc.
F5555HDl
To assess a cIild virI possiIIc LIL a pIysician
nusrrelyoninIornationoIrainedIronparcnrs and
||||ISfSI OC ||ySCOOQC|IVC
QCS,UOOC|SI|OS
|U|CS|O O|OCSOy
I|C
tcacIcrs. Tvo connonIy uscdraring scaIcs arc tIc
LL-! ConprcIcnsivc 1cacIcr's Rating ScaIc
and rIe reviscd Conncr's Farcnr and 1cacIcr ScaIc.
conpIcrcpIysicaIcxansIouIdIepcrIorncd,Iur
nornaIIyrIcscnsory, pIysicaI,andneurologiccxans
arc nornal.
< 1O1 IOI sa1e| > < ue pLn nyopa | >
< cxau n pexaax~xouaeycun: HYC s you. y >
LgDOStC Llt0l Ol Ptt0DtOD L0Ct
HQ0lCtVt LSOlO0l
bDQtODSOlDt!CDtOD
| | | |QIO Q|VCIIC|I|OO IO OCI||
L|JCUIyCOQ|CI|OQISKS
L|JCU|Iy O|Q||2||Q CI|V|I|CS
PVO|OS CIV|I|CS IDI |CQU| |C SUSI|OCO C|I CUO|I
S||yU|SI|CICO OyCIC|||O|CCS
O|QCIJU| ||O||yCI|V|IICS
bDQtODSOlHQCfClVt
|UQCIS |O SQU|S
O|O|CIO|C||| QOSI|O|
CC| ||QSO|CSI|CSS|CSS
O|O|C IO C||Oy CI|V|I|CSQU|CI|y
|KSCCCSS|VC|y
bDQtODSOlDQUSVl
L|JCU|Iyw|I||QIU||
| |IC||UQISOIDC|S
JDESE SyDODS SDOUlO OE tESED lD WO Ot DOfE SEIlD_S DO tESUlI
lD lDltEO UDCIODlD_, D OOllOD, DE SyDODS DUSI OE lESEDI
llOt O DE IlEDI lEClD_ J yEtS O _E.
POEO tOD PDEtlCD SyCDlllC PSSOCllOD, Ll_DOSlC DO
SIlSIlC DDUl O DEDl OlSOlOEtS, 4I EO. VSDlD_IOD, LL. PDEll
CD SyCDlllC PSSOCllOD, 14.
N0D0gHDl
TIcrrcarncnr progranIorLILrcquircs anuIri-
disciplinaryapproacI.InotionaI supporrs sIouldIc
nadcavaiIaIIc IorrIcparicnf andparcnrs.IcIav-
iornanagcncnrprogrannustIcdcvcIopcdtoassist
IotI rIc parcnts and rcacIcrs vitI discipIinc. TIc
paticnr's acadcnic nccds sIouIdIc ncr, up ro 257
oIcIiIdrenvirILILviIIaIsoIavcaIcarningdis-
aIiIity. ConorIid condirions arc connon and nay
incIudcaggrcssionproIIcns,opposiLionaIdcIanrdis-
ordcr, conducrproIlcns, and nooddisordcrs.
CccasionalIy,pIarnacoIogictrcarncntsarcncccs-
sary.Iirst-IinctIcrapyconsistsoIpsycIo-stinuIants,
incIuding methyIphenidate and dex-troampheta-
mine. TIcy vorI Iy incrcasing rIc avaiIaIiIity oI
dopaninc and norcpincpIrinc in tIc ClS. Sidc
cIIcctsincIudcinsonniaandanorcxia,sonctincstics
anddysIincsiasnaydcvcIop.Scvcnrypcrccnrro807
oIpaticnrs viIl rcspond to rIc initial dose oItIcsc
ncdicarions. Iovcvcr, nonstinulanf ncdicarions,
sucI as sclccrivc scroronin rcupraIe inIiIirors and
clonidinc, arc soncrincs ncccssaryvIcnrIc paticnt
docs nor rcspond orcannorroIcrarc srinulanr ncd-
icarion. FIarnacoIogic trcarnenr sIould ncver Ie
LDQIC|4 / LCVC|OQDC1I 4b
N 1RbLb 4-
LgDOStC Llt0l Ol 0lVSV0 L0V0lOQD0Dtl
LSOlO0l
DQfDCDtSD bOCl DlCfCtODS
LCKOOO|VC|OOC|V|O|S
LCKOQCC||C|I|O|SD|QS
LCKOS|Ow||Q ||IC|CSI
LCKOCOI|O| |CC|Q|OCIy
DQfDCDtSD LODDUDClOD
LCVC|OQC|I| ||QUQCOC|y
O|O|CIO SUSI|| CO|VC|SI|O| w|\D OI|C|S
OSC O|CQCIIVC ||QUQC
LCKOSOC|| Q|y
lrCSCDCCOlbtCfCOlQC CHVOfS
||C|D|CO|C|C|CCIO ||IU|S
bIC|COIyQ|C| OIO||OC||SS
||COCCUQI|O|w|I|OO|CCIS
POEO tOD PDEllCD SyCDltlC PSSOCllOD. Ll_DOSlC DO
SIlSIlCl DDU O DEDI OlSOlOEtS, 4I EO, VSDlD_OD, LL!
PDEtlCD SyCDlItlC PSSOCllOD, 14.
givcninisoIarion,andatIcasronccaycarrIcparicnr
dcscrvcs a triaI oIncdicarions.
Pervasve Developmental Disorder
lervasive deveIopmentaI disorder [lLL} rcprcscnrs
aspccrrunoIcIronicnonprogrcssivcdcvcIopncntal
disaIiIitics involving inpairncnts in sociaI inrcrac-
tion,connunication,andIcIavior.utisnisaIorn
oI FLL. FLL is sccn ir 2 to 6 cIiIdrcn pcr I 000
cIiIdrcn and is |our tincs norc connon in naIcs.
losingIcundcrIyingcauscIasIccn idcnriIcd. Mosr
cIiIdrcn prcscnt Icrvccn | 8 nontIs and J ycars oI
agc, Iut synprons can Ic prcscnr Iron inIancy
[inpaircd attacIncnt) .
LhDC0 N0Dl5l0lDD5
CIiIdrcnvirI ILL IavesigniIcanrspcccI andIan-
guagc dcIays and proIIcns virI social inrcracrions.
TIcy Iavc Iinircd cyc conracr, do nor givc rccipro-
cal connunicafion, and do nor cngagc in prctcnd
pIay.Inaddition,nany cIiIdrcnIavc an attacIncnt
andlascinarionvirhunusuaIoI]ccrs andnaydispIay
srcrcorypicaI IcIavior. TIc LSM-I\ crircria arc
IistcdinJaIIc4-J.
N0D0gHDl
TIcrc is no pIarnacoIogic trcarnent availaIIc Ior
FLL. Sonc cIildrcn viII IcncIt Iron ncdication
< nor foz sa1e| > < ue pLn popa | >
Rb ||ucr|!s |CO|I||CS
1RbLb 4-4
b0CODOl b0X LDlC0lStCS. DD0l
r0St L0V0OQDCDt
bIQC| ||COO6SC6|I,6| CVI|O| OQdQ|| OOy
< cau n pexaanxouaeycun: HYC s you. y >
bIQC | | b|CSIOUO,CCVI|OOO O|CSI|O QQ|||SS|| | |Ou|O,C||gC|C|IO|6O||U||CIC|(11.1 b11.1 0)
bIQC | | | U|IDC|C|||QC|6OI|UC|CVIO|O O|CS\OUf6O,OOSCQ|IO|OIDC||CO|IOU|S (1Z.1 I1.9)
bIg6 |V ||O|CCI|O| O |CO |U QQ||IOO|| SCCOOO|y|OUOODOVC |CVC| OO|CSI (13.11 I1.1 b)
bIgC V NIU|C SIgC,Q|O|6CIO| OQQ||| O||yOUC IO |CCCSSO| O |CO IOQC|C|| CO|IOU| OO|CSI [1.d3 I1.74)
NOIC.bIg6S |VOO V|y|OIOCO|SI||CI || SO|C QIC|IS
L0Dtl L0VClOQDEDl[N0)
bIQC | |COO|CSC6|I,I6SICS,SC|OIU|,OO QC|SOOUI S|C S|2C |O Q|OQO|I|O| S || C||y C|||ODOOO
bIgC | | O||gC|C|IOSC|OIU| OOICSICS,SK| OSC|OIU| |COOC|S |O CD|gCS| OICIU|C, |II|CO| |OCO||QC|C|I
O QC|S [11.4 I1.7)
bIQC| | | O||gC|COIOQCO|S,|SI |||y| 6|QI|,U|I|6|Q|OwI| OI6SICS OO SC|OIU| (1Z.b 11.4)
bIQC |V | |CfCSCO S|2C OQC|Sw|ID g|Ow\D ||Of6OID |O O6VC|OQ|C|IOgOS,U|IDC|CO||QC|C|IOICSI6S |O
SC|OIU| OO ||C|CS6O OfKCO||QOSC|OI| SK|| [13.77 I1.Z)
bIgCV LC|I| OU|I| SZC OO SDQC [14.9Z I1.1 0)
lUDC Hf [N0 DU 0Dl0)
bIQC | |fCOO|6SCCOI,VC||USOV6| QUOCS |OUfIDCfOCVC|OQCO IDO IDIOVCfOOO|O|w|| [|.6.,OOQUO|C||f)
bIQ6 | | bQ|SC Q|OwIDO|O|g,S||QDI|yQIg|C|ICO OOw|y D||,SI||g|IO| O||yS|QDIy CU||CO,CDCyIDSC OQCO|S
O||O|g |O [N|C.15.44 I1.9. 6|C:11.9 I1.21)
bIgC| LO|SOC|D|yO|KC|,CO|SC||O |O|C CU||CO,D||SQ|COSSQfS6|yOV6||UOCI|O| OQUOCS [N|C.15.9 I1.4.
6||C.1Z.3 I1.1 )
bIQC |V M||fCS6|O|CS OU|I |OIyQC,OSIf|OUI|O| SI|| COOS|OCfO|yS||C|I|| | OU|I.NO SQ|CO IO |CO|| SU|CC
OIDgDS[N6.14.3 I1.b. C||C.12.9 I1.)
bIQCV POU|IOQUOIIy OO IyQC wID O|SI||OUI|OO O IDC DO|2O|I| QIIC|| [NC.1.1b I1.7. C||C.14.41 I
1.12)
bIQCV bQ|CO UQ|C |O.||CCSCUICDCO|
designcdto targetspeciIc synptonssucI asinpuI-
sivityandIypcractiviry.TIc nainsrays oItrcatncnr
arc IcIavioraI tIcrapy, inproving connunication,
and providing parcntaI supporr.JIc Icst prognostic
indicator oIIuturc succcss is tIc cxrcnt oIIanguagc
dcvcIopncnf prcscnt during tIc prcscIooI ycars.
SEXUAL DEVELOPMENT
AdoIescence rcIcrsrorIc passagcIroncIiIdIoodro
aduItIood, vIcrcas puber rcIersto rIosc IioIogic
cIangcs tIar Icad to rcproducrive capaIiIity. TIc
evcnts oIpuIcrty occur in a prcdictaIIc scqucncc,
Iut tIc tining oItIc initiation and tIc vcIocity oI
rIc cIangcs arc IigIIy variaIIc anong individu-
aIs. TIc intcgration oI tIc puIcrraI cIangcs into
tIc adoIcsccnt's scII-conccpt is cruciaI ro nonnaI
adoIcsccncc.
L
L
U 1 U
PQe[yea|s)
1 1 1 2 1c J A 1t
|1e|Q|jun
1es|| Cu|3|vC|u0e(CC]
U
^\O\1O \1O' 1'1O'
CeO||3||a s|2e Z^'
[!aOOe|s|aQe)
|uU|C al | Z^'
(1aOOe| s|aQe)
1 U J 1 1 1c 14
/Qe(yea|s)
1t
1
1
1 7
1 7
In naIcs, tIc initiation scqucncc oIscxuaI dcvcI-
opncntisrcsticuIarcnIargcncnt,IoIIovcdIypcniIc
guf6 4-1* bCQUC|CC O UOC|I| CVC|IS || \|C VC|QC
P|C||C| ||C.
not foz sa1e| > < ue pLn nyopa | >
< cxeu n peaan-xouaeycun: HYC s you. y
6
L
PQe(yea|s)
9 1 1 1 1Z 1c 1A
|e|Q|(jur|
|eOa|C|
Lleas| z_ 5
(IaOte| slaQ)
|uU.C a|| _o
(IaOO|s|aQe)
9 J U 1 1 1 1c
/Q[yea|s)
14
1U 1 1T
Jt 1 1 7
||gure 4-Z Sequence of puberta| events |n the average
Amercanfema|e.
cnIargencnt, IcigIr grovrI spurr, and puIic Iair.
TIis progrcssion is sIovn in Figure 4-I .
In Ienales, rIc ordcr oIpuIcrraI cvcnts H scxuaI
dcveIopncnris rIcIarcIc [lrcastIuds), IoIlovcdIy
IcigIt grovrl spurt, puIic Iair, and ncnarcIc.
Iigure 4-2 iIIustrarcstIcsc cIanges.
Chapter4 / Oeve|opment " 41
TIe Tanner staging system is used to detcrninc
vlcreacIiIdisintIepubcrtaIprocess.Tannerstages
|or tIe naIc gcnitaIia, |enaIe Ircasts, andnaIc and
|cnaIe puIic Iair arc sIovn in JaIIe 4-4. IuIcrraI
aInornaIirics arc addrcsscd in CIaprcr 6.
M
LN L W
M
1. Twoseparatedeve|opmenta| assessments are
morepred|ct|ve than as|ng|eassessment,and
test|ngshou|dbeperformed|n a|| areasofdeve|
opmenttoassessforde|ay,d|ssoc|at|on,or
dev|ancy.
Z. The treatmentprogramforADHDrequ| resamu|-
t|d|sc|p|| nary approach.
d. Pervas|vedeve|opmenta|d|sorder(PDD)repre-
sentsa spectrumofchron|cnonprogress|ve
deve|opmenta|d|sab|| |t|es|nvo|v| ng|mpa|rments
|nsoc|a||nteract|on,commun|cat|on,andbehav-
|or.Aut|sm|saformofPDD.
4. Theeventsofpubertyoccur|napred|ctab|e
sequence,butthet|m|ngofthe|n|t|at|onandthe
ve|oc|tyofthechangesareh|gh|yvar|ab|eamong
|nd|v|dua|s.
. The|n| t|at|onsequenceofsexua|deve|opmentfor
ma|es|stest|cu|aren|argement,pen||een|arge-
ment,he|ghtgrowth spurt,and pub|cha|r,
whereasthe sequenceforfema|es|sthe|arche
(breast buds),he|ghtgrowth spurt,pub|cha|r,and
menarche.
V|RAL EXANTHEMS
Clinical Manifestations
Ccrrain viraI cxantIcns arc cIaractcrisric Ior parLi-
cuIarviraIiIIncsscs.
IrIougI measIes is unconnon in dcvcIopcd
countriesvIerevaccinesareused, itcontinuestoIe
a najor IeaItI proIIcn vorIdvide. TIc incuIation
period is 8 to I Z days aIcr initiaI cxposurc to
rIc paranyxovirus, tIcrc arc no signs or synptons
during tIis stagc. prodronc IoIIovs, consisting oI
naIaise, IigIIevcr, cougI, coryza, andcon]unctivitis.
\itIinZtoJdaysoIrIconsctoIsynptons,KopIiI's
spots [snaII irrcguIar rcd spots vitI ccntraI gray or
IIuisI vIitc spccIs) appear on tIe IuccaI nucosa.
Iout 5 days aIter tIc onser oI synptons, an cry-
rIcnatous nacuIopapuIar rasI erupts on tIc Icad
and spreads caudaIIy, Iasting aIout 4 to 5 days.
Liagnosis is nadc Iy tIc distinctivc Iisrory and
cIaracteristic cIinicaI hndings Iovcvcr, it nay Ic
conIrncdIyscroIogicrcsLing.Scverc conpIications
incIudeacurecnccpIaIitis,rcsuItinginIraindanagc,
and suIacurc sclcrosing pancnccpIalitis.
RubeIIa isgcncraIIyinnocuousvIcnacquircdpost-
naralIy, IutvIcnaIctus isinIcctedduringgcstation,
tIeresultscanIcdcvasraring.!ordetaiIsoncongcni-
taIruIeIIa,sccCIaptcrI J. RuIeIIaiscauscdIyruIcIIa
virus, an Rl rogavirus. CIinicaI naniIcsrations in
postnataIIyacquircdruIcIIa arcaIsenrinnanycascs.
TIcreisnoprodroncduringtIcincuIationpcriodoI
I 4to 2I days. \Icn synptons do occur, ruIcIIa is
cIaractcrizcdIyancryrIcnatous,nacuIopapuIar,dis-
crctc rasI, vitI gencraIizcd IynpIadcnoparIy and
sIigIr Icver. JIc rasI rarcIy Iasts Iongcr tIan 5 days.
FcvcrnayacconpanytIconscroIrasI.JransicntpoI-
yarLhraIgia and poIyarrIritis are connon in adoIcs-
cents. EnccpIaIitis and rIronIocyropcnia arc rarc
conpIications. FostnataIIy acquircd ruIcIIa is con-
IrncdIyscroIogictcsting.TIcdiagnosisoIruIcIIais
oItcndihcuItIccausctIcsynptonsarcniIdandnay
Ic conIuscd virI tIosc oI cntcroviraI inIcction,
roscoIa,toxopIasnosis,inIcctiousnononucIcosis,nild
ncasIes,andscarIcfIcvcr.
RoseoIa inlantum is a connon, acutc discase
oIinIants and young cIiIdrcn caused Iy IunanIcr-
pcsvirus6[II\-6).JIciIIncssIcginsvitIanaIrupL
IcvcrcIaractcrizcdIytcnpcraturcsoII0J!toI 06F
[J9. 4-4I .0C)tIatpcrsistIor l toD days.LuringtIc
Icvcr,tIecIiIdgcncraIIyappcarsvcIIandIasnopIys-
icaIIndingstocxpIaintIcIcvcr.roundtIctIirdor
IourtIdayoIilIncss,anacuIopapuIarrasIappcarson
rIetrunIandsprcadspcripIcraIIy.TIcIcvcrtypicalIy
rcsoIves as tIcrash appcars. InitiaIIy, IeuIocyrosis up
roZ0,000vitIaIcItsIiItnayexisr,IurIytIcsecond
day oI iIIness, IeuIopenia and neutropenia nay Ie
norcd.ConpIicarionsarcunconnon,aIrIougIIcIrilc
scizurcsnayoccurductorIcrapid incrcascintcn-
pcratureduringrIconscroIinIcction.
Lrythema inlectiosum [IItI diseasc) is a niId,
scII-Iinitcd, systcnic iIIncss causcd Iy rIc Ll-
containing parvovirus BI 9. IL prinarily occurs in
cpidcnics. IsuaIIy tIcrc is no prodrone, and Icvcr
nay Ie aIscnf or Iov grade. TIe rasI progrcsscs
tIrougIrIrccstagcs. ItIcginsasanarIcdcryrIcna
oILIecIeeIs,vIicIgivcsa"sIapped cheek" appcar-
ancc.n eryrIcnatous, pruritic, nacuIopapuIarrasI
tIcnstarrsonrIcarnsandsprcadstorIctrunIand
Icgs.TIe tIird stagc is cIaractcrizcd Iy Iuctuations
intIcseverityoItIcnacuIopapuIarrasIandusuaIIy
Iasts Z to J vccIs. !Iucruations occur virI rcn-
pcrarure cIangcs and cxposurc to sunIigIt. Con-
pIicarions i ncIudc arrIriris, IcnoIyric ancnia, and
cnccpIaIoparIy. Farvovirus BI 9 inIection during
pregnancyisassociarcdvirIletaI hydrops anddcarI
oIrIe Ietus.
< not foz ca1o| > < no pLn nyopa | >
land-loot-and-mouth disease is a connon acutc
discasc oI young cIiIdrcn during tIe spring and
sunncr causcd Iy coxsacIic viruscs. TIerc is
usualIy a prodronc oIIcvcr, anorcxia, andoraI pain,
IoIIovcd Iy crops oIulccrs on rIc tonguc and oraI
nucosa and a nacuIopapuIar vcsicuIar rasI on tlc
Iands, Icct, and occasionaIIy tIc IurrocIs. Liagno-
sis is nadc Iy tIc Iistory and tIc consrcIlation oI
synprons.
'ariceIIa [cIicIcnpox) is a IigIIy conragious
discasc causcd Iy prinary inIcction vitI variccIIa-
zostcr virus. Ir is usualIy a nild, scII-Iinircd discasc
innornal cIiIdrcn. Its scvcritycanrangcIronaIcv
Icsions and a Iov-grade Icvcrto Iundrcds oIIcsions
and a rcnpcraturc up to I 05`I [40.6`C) . IaraI dis-
scninarcd discasc nay occur in innunoconpro-
niscd cIiIdrcn.Itcr an incuIation pcriod oI I 0ro
2I days,tIcrcisaprodroncconsisringoIniIdIcvcr,
nalaisc, anorcxia, and occasionalIy a scarIatiniIorn
ornorIiIliIorn rasI.TIc cIaractcristic pruriric rasI
occurstIcIoIIovingday, appcaringIrstonrIctrunI
and tIcn sprcading pcripIcraIIy. TIc rasI Icgins as
rcd papuIcs and dcvcIops rapidIy into cIcar vcsicIcs
rIar are aIour I ro Znn indiancrcr.JIc vcsiclcs
rIcn Icconc cIoudy, IrcaI, and Iorn scaIs. JIc
IcsionsoccurinvidcIyscarrcrcdcrops,"sotIcrcarc
usuaIIy scvcraI stagcs oIIcsionsprcscnf attIc sanc
rinc. \csiclcs oItcn occur on nucous ncnIrancs.
Faticnrs arc inIcctious Iron Z4 Iours IcIore tIc
appcarancc oI tIe rasI unril alI tIe Icsions arc
crusrcd, vIicIusuaIlyoccurs I vccIaItcrrIconscr
oIrIc rasI.
CIicIcnpoxisacIinicaIdiagnosis.InuncIcarcascs,
aTzancIprcp,looIingIornuItinucIcarcdgiantccIIs,
can Ic pcrIorncd on a vcsicIc, ora pIaryngcaIsvaI
orsvaIoIvcsicuIarIuidcanIc scntIorviraIcuIrurc.
IrcrnarivcIy, acutc and convaIcsccnt scra can Ic
tcstcd Ior a IourIoId incrcasc in antiIody titcr. Fro-
grcssivcvariccIlavitIncningocnccpIaIitis,Icpatitis,
andpncunonitisnayoccurininnunoconproniscd
cIiIdrcnandis associatcdvitl a Z07norraIityratc.
InnunizationvitIvariccIIavaccincIasrcduccdtlc
IrcqucncyoItIis inIcctionintIcInitcdStarcs.
Zoster [sIingIcs) rcprcscnrs arcacrivationoIvari-
ccIla-zostcrvirusinIcctionandoccursprcdoninantIy
inadulrsvIoprcviousIyIavcIadvariccIIaandIavc
circuIating anriIodics. Itcr cIicIcnpox, variccIIa-
zosrcr virus rcrrcatsro rIc dorsal roor gangIion, as a
rcsult, ir IoIIovs a dermatomaI distriIution vIcn
rcactivatcd.ItIougIIcrpcsoccursincIiIdrcn, ir is
unconnon in tlosc youngcrtIan I0ycars./attacI
oIzosrcrIcginsvitIpainaIongtIcaIcctcdscnsory
ncrvc and is acconpanicd Iy Icvcr and naIaisc.
vcsicuIar cruption tIcn appcars in crops conIncd
rotIc dcrnaronaldistriIutionandcIcarsin 7to I 4
days.TIerasInayIasrasIong as 4 vccIs, Iovcvcr,
vitI pain pcrsisting Ior vccIs ornontIs.
CtIcr conpIications Iron zosrcr incIudc cn-
ccpIaloparIy, ascptic ncningitis, CuiIIain-Barrsyn-
dronc, pncunoniris, tIronIocyropcnic purpura,
ccIIuIitis, and arrIritis.
Treatment
In unconplicarcd cascs, trcatncnf is nainIy sup-
porrivc. Fcvcr is trcatcd virI acctaninopIcn or
iIuproIcn and Iuids. [IIuproIcn is contraindicarcd
vIcnvariccIlaissuspcctcd, Iccausc oIincrcascdrisI
oI strcptococcaI cclIulircs.) spirin sIouId Ic
avoidcd, Iccausc aspirin tIcrapy Ior Icvcr in rIc
scrting oIa viraI inIccrion is associatcd vitl Rcyc's
syndronc. TIc ircIing associarcd virI IItI discasc,
variccIIa, and Icrpcs zostcr is rrcarcd vitI an anti-
Iistaninc ncdicarion. Luring cIicIcnpox, daily
IatIing in IuIcvarn varcr rcduccs tlc risI oIscc-
ondaryIactcriaIinIccrion.IcrpcszosrcrcanIequitc
painIul, and narcotics arc soncrincs nccdcd.
Innunoconproniscd cIiIdrcnvIo arc cxposcdro
sonconc vitI variccIIa-zostcr virus inIcction arc
givcn variccIIazostcr innunc gIoIuIin vitIin 96
loursoIrIccxposurcandoIscrvcdcIoscIy.cycIovir
is cIccrivc in tlc trcatncnt oI IorI variccIla and
zosrcr its usc is indicarcd in innunoconproniscd
paticnts. CraIacyclovirnayIc considcrcdIoruscin
paricnts oIdcr tIan IZ ycars, cIiIdrcn vitI cIronic
discasc,andtIoscvIoIavc rcccivcdsrcroidsIorany
rcason.dninistrationoItIcvariccIlavaccincvitIin
7Z Iours oIcxposurc nay prcvcnt or lcsscn discasc.
Innunizations are avaiIaIIe Ior tlc prcvcnrion oI
ncasIcs, ruIclIa, and variccIIa [scc CIaptcr I Z) .
M
LN L W
1. Vira|exanthemsaregenera|lyben|gnand are
treatedsymptomat|cally.
Z. 1heexanthemsared|fIerent|ated by h|storyand
rashappearance.
. Ch|ldrenw|thch|ckenpoxarecontag|ousfromZ4
hoursbeforethe onsetofrash unt|lall|es|ons
havecrustedover.
< cx n roeano-ronnopnn: uvcm os ons. |V >
BACTER|AL RASHES
BacteriaI rasIcs oItIc sIin arc connon and arc in
nost cases tIc rcsuIt oI group Icra-IcnoIyric
strcptococcaI or5lnhy/ococcus nureu: inIcction.
Clinical Manifestations
uIIous impetigo vIicI is causcd Iy 5. nureus,
IcginsasrcdnacuIcstIatprogrcsstoIuIIous [Iuid-
IIIcd) cruptions on an cryrIcnarous Iasc. TIcsc
Icsions rangc Iron a Icv niIIincrcrs to a Icv ccn-
tincrcrsindianctcr.ItcrtIcIuIIacruprurc,acIcar,
tIin, vanisI-IiIc coating |orns ovcr tIc dcnudcd
arca. 5. nureus can Ic cuIturcd Iron rIc vcsicuIar
Iuid. BuIIous inpctigo lcsions can Ic nisraIcn Ior
cigarcrrcIurs, raisingtIc suspicion oIaIusc.
honbuIIous impetigo, vIicI is causcd Iy IotI
group Icta-IcnoIytic srrcprococci and 5. nureus,
Icgins as papuIcs tIat progrcss to vcsicIcs and tIcn
to painIcss pustuIcs ncasuring aIour 5nn in
dianctcrvirIa tlincrytIcnatousrin.TIcpusruIcs
rupturc, rcvcaIing a Ioncy-coIorcd tIin cxudarc
rIartIcnIornsacrustovcrasIaIIovuIccrarcdIasc.
IocaI IynpIadcnopatly is connon vitI strcpto-
coccaI inpctigo. Ievcr is unconnon. TIc causativc
organisncanusuaIIy IcisoIatcdIronrIc Icsions.
StaphyIococcaI scaIded skin syndrome, vIicI is
causcd Iy cxIoIiarivc isoIatcs oI 5. nureus, is nosr
connonin inIancy andrarcIy occurs Icyond agc 5.
Cnscti s aIrupr,vitI dihusc cryrIcna,narIcdsIin
rcndcrncss,andIcvcr.\irIinI Z to 24IoursoIonscr,
supcrIcial Iaccid IuIIac dcvcIop and rIcn rupture
aInosr inncdiatcIy, Icaving a Icc[ rcd, vccping
surIacc. LxIoIiation is causcd Iy a toxin and nay
aIIcct nosr oItIc Iody, and tIcrc is usuaIIy a posi-
tivc hikoIs|'s sign [scparation oItIc cpidcrnis on
IigIr ruIIing) . JIc iniriaI Iocus oI stapIyIococcaI
inIcctionnayIcninorornotapparcnt.Inrupturcd
IuIIac contain stcriIc Iuid.
loIIicuIitis is an inIcction oItIc sIaIt oItlc Iair
IolIicIc. SupcrIciaI IoIIicuIitis is connon and casiIy
trcatcd.Lccp Iorns oItIis inIcctionincIudc |urun-
cIes[loiIs) and carIunclcs. luruncIes Icginassupcr-
IciaI IolIicuIitis and arc nosr IrcqucntIy Iound in
arcasoIIair-Icaring sIinrIararc suI] cctto Iriction
and naceration, cspcciaIIy tIc scaIp, IuttocIs, and
axiIIae.CarbuncIes arcanaccunuIationoIIuruncIcs.
CeIIuIiIis is aIocaIizcd, acutcinIannationoItIc
sIir cIaracrcrizcd Iy cryrIcna, pain, and varnrI.
CcIIuIitis in cIiIdrcn is nosroItcn causcdIy group
Icta-IcnoIyric strcprococcaI or 5. nureus inIcc-
rion.JIcscIacrcriaarenornaIIoraoItIc sIin, and
a IrcaI in tlc intcguncnr aIIovs cntry into tIc
dcrnis and cpidcrnis.TIc Iocation oItIc inIcction
isinporrant,Iccausc inrarc cascstIcccIIuIirisnay
ariscIronanundcrIyingostconycIitis, scpricartlri-
tis,sinusitis,ordccpvoundinIcction.BeIorcrIcusc
oItnemoh/us njluenzne typc I [IiI) vaccinc, .
njluenzne typcIvasasigniIcantpatIogcnrcsulting
innany cascs oIccIIuIitis Iy Icnatogcnous sprcad.
. njluenzne typc I ccIIuIitis is nov rarcIy sccn.
Currcntly,5lrelococcus neumonoe isrIcnostcon-
non causc oI IcnatogcnousIy sprcad ccIluIitis.
HcnarogcnousIy sprcad 5. neumonne oItcn aIccts
rIc Iacc and pcriorIiraI arca. CcIIuIitis oI tIc Iacc,
dcpcnding on vIcrIcr ir rcsuIts Iron trauna or
hcnatogcnous sprcad, can rcsuIr Iron all tlc
parIogcnsncntioncd.groupIcta-IcnoIyricstrcp-
tococci, 5. nureus, 5. neumonne, or . njluenzne
typcI.
Treatment
IinircdnonIulIousinpctigocanIctrcatcdtopicaIIy
vitI nupirocin ointncnt. BuIIous inpctigo and
nonIuIIousinpctigo,iItIc Icsions arc nuncrous,arc
trcatcdvitI aIrst-gcncrationccpIaIosporin sucl as
ccpIaIcxin, an oraI drug tIat is cIcctivc against
IorI srapIyIococci and group strcptococci. TIe
carctaIcrcanrcnovc anyIoncy-coIorcdcrusrsvirh
tvice-daiIycool conprcsscs.
MiIdtonodcrarccascs oIstapIyIococcaIscaIdcd
sIin arc frcarcd vitl an oraI antistapIyIococcaI
ncdication. CIiIdrcn vitI sevcrc cascs sIouId Ic
trcarcd as tIougI tIcy Iad a sccond-dcgrcc Iur,
vitl ncricuIous Iuid nanagcncnt and intravcnous
oxaciIIin orcIindanycin.
SupcrIciaI IoIIicuIitis rcsponds to aggrcssivc Iy-
gicnc and ropicaI nupirocin, vIcrcas |oIIicuIitis oI
rIcnaIcIcardisunusuaIIyrccaIcitraniandnccdsan
oraI antisrapIyIococcaI drug. SinpIc IuruncuIosis is
trcatcdvitInoistIcat. |argcranddccpcrIuruncIcs
nay nccd to Ic incised and draincd.Itcrdrainage,
rIcy nccdonIy topicaI nupirocin trcatncnt.
CIiIdrcn vitI niId ccIIuIiris can Ic rrcarcd
virh an oraIanriIiotic,sucIas ccpIaIcxin oranoxi-
ciIIin-cIavuIanic acid. TIosc vitI scvcrc inIcction
vIo havc IynpIangitic strcaIing orIynpIadcnopa-
tIy nay Ic IospitaIizcd and givcn a parcntcraI
anriIioric.IacialorpcriorIitalccIIuIitis [sccCIaptcr
8) usuaIIy is trcatcd virI intravcnous anpiciIIin-
suIIactanandadnissionrorIcIospitaIIoroIscrva-
rion.\lcnorIiraI orpcriorIitaI ccIIuIitis is prcscnr
< not foz ca1o | > < no pLn popa | >
or a pcripIcraI sIin ccIIuIiris rcsuIrs in IynpIad-
cnoparIyor IynpIangiric strcaIing, a IIood cuIrurc
sIouId Ic scnr ro dcrcrninc vIctlcr Iacrcrcnia is
prcscnr.
LN L W
W
1. b.UulPu5 and groupAbetahemo|yt|cstrepto-
coccicausemost bacterialsk|ni nfections.
Z. 6ecauseoftheHibvacci ne,b.QDPu0DDUP has
replacedH.DhuPDZUP asthemostcommon
pathogen|nhematogenous|yspreadce||u|itis.
d. 1hech||dw|th peripheralce|lul|t|swith |ym-
phadenopathyorlymphang|ticstreakingandthe
ch|ldwithorb|ta|orper|orb|ta| cel|ulitis shou|d
haveab|oodcu|turesenttodeterminewhether
bacterem|aispresent.
SUPERF|C| AL FUNGAL RASHES

LsscnriaIIy tlrcc IungaI organisns causc supcrhciaI


rinca inIccrions. 1ricopylon, icrosporum, and
Ipidermopylon. Tinca inIcctions and tlcir trcar-
ncnrs arc discusscdinTaIIc 5-I .
Jinea versicoIor, anorIcr rypc oIycasL inIccLion
causcd Iy n/n:sezn ]ur]ur, is cIaracrcrizcd Iy
N 1RbLb b-1
LODDOD D0 D0CtODS DO D0l l0tD0DtS
DECtOD
||Cd CdQ|I|S [SCdQ)
||C COfQOf|S [OOOy)
|
'
QwO||
||Cd C|U|S [QC||IOC|U|d|)
|OCK |IC|
||Cd QCO|S [OOI)
dI||CIC SOOI
1fCtDCDt
L|d|Q||SCOUV||,4-
wCCKS
bCC||U| SU|DOC S|d|QOO
IOOCCfCdSC | |CCI|VIy,
OOCS OOIC|dOCdIC
| OCCI|OO
OQ|Cd| d|I|UOQd|S [C.Q.,
COI||d2O|C) fordI
|CdSI4 wCCKS,Ofd|
Q|SCOU|V| ICfdCIO|y
bd|C dS I||C COfQOf|S
bd|CdSI|CdCOIQO||S,
QUS QfOQC|OOI
|yQ|C|C
supcrIciaI ran or IypopigncnrcdovaI scaly Icsions
on tlc nccI, uppcr parr oI rIc IacI, cIcsr, and
proxinaI arns in a Clrisrnas rrcc distriIution.
LarI-sIinncd individuals rcnd ro Iavc lypopig-
ncnrcdIcsions duringrIcsunncr,vIcnuninIcctcd
sIin darIcns Iron sunligIr cxposurc. Trcarncnr is
virIscIcniunsuIhdc sIanpoo.
Liaper rash nay rcsuIt Iron aropic dcrnariris,
prinary irritant dcrnariris, or prinary or sccondary
Lnnddn n/bcnn: inIccrion. LigIry pcrccnr oIdiapcr
rasIcs Iasting norc rIan 4 days arc colonizcd vitl
Lnnddn. Iicry rcd papuIar Icsions virI pcripIcral
scaIcsintlcsIin|oIdsandsarcIIireIcsionsarcrypical
Ior candidaI diapcrrasI.Topicalnystatin istlcIst-
Iinc rrcarncnroIcIoicc.
ACNE
Pathogeness
Acne vuIgaris iscauscdIycnIargcncnroIscIaccous
glands, incrcascd scIun producrion, proIiIcrarion oI
roonbnler|um ncnes, andsccondaryinIannarory
cIangcs. TIcrc is a prcdiIccrion Ior Iacc cIcsr and
IacI. Icsions progrcss Iron concdocs [Iirc-
Icads),toopcnconcdoncs [IIacIIcads),topusruIcs,
topapuIcs, ronoduIcs [cysts), andInalIyroaLropIic
and IypcrtropIic scars. ndrogcns arc rIe srinuIus
Ior scIaccous gIand dcvcIopncnL and sccrcrion.
r puIcrry, Iornonal srinuIi lcad ro incrcascd
grovrI and dcvcIopncnr oI scIaccous IoIIicIcs.
IcnaIc paricnts vitl scvcrc acnc oItcn lavc lIgl
Icvcls oIcircuIaringandrogcns.
Epidemiology
cnc is a vcry connon, scII-Iinircd, nuItiIacroriaI
disordcr oItIc scIaccous IolIicIcs, norcd duringtlc
rccnagc ycars. Icsions nay Icgin as carIy as 8 ro I 0
ycars oI agc. FrcvaIcncc incrcascs stcadily tlrougI-
our adoIcsccncc and tlcn dccrcascs in aduIrIood.
IrlougI girIs oItcn dcvcIop acnc ar a youngcr agc
rIan Ioys do, scvcrc discasc aI|ccrs Ioys I 0 rincs
norc Ircqucnrly Iccausc oI IigIcr androgcn lcvcIs.
In Iacr, | 57 oIaII rccnagc Ioys Iavc scvcrc acnc.
Risk Factors
RisIIacrors incIudc nalc gcndcr, puIcrry oiIy con-
pIcxion, CusIing's syndronc, or any otIcr proccss
tlarrcsuIrsin incrcascd androgcns.
bZ

not no Jna nyoa | >

I'e0Iaic
Clncal Manfestatons
5lDly
It is inporranr to dcrcrninc vIcn rIc acnc srartcd
andvIctlcrrIcrc is a |aniIyIisrory oIacnc. IuIl
ncnstruaI Iisrory sIould Ic raIcn ro dcrcrninc
vIctlcr rIcrc is a corrcIation Ictvccn tlconsct oI
ncnscs andrIcpaticnr'sacnecxaccrIarions.hisalso
inporranr ro discuss rIc paricnr's sIin carc, incIud-
ing Iov tlc paticnt's acnc Ias Iccn rrcarcd in rlc
past. Manydrugscauscacnc. Corticosrcroids, andro-
gcns,danazoI,iodidcs,andIronidcsoItcncxaccrIarc
acnc. Ctlcr possiIIc srinuIi includc isoniazid,
Iitliun, IalotIanc, viranin B,, and IypcraIincnra-
tion. TIcsc drugs arc nor dirccrly concdogcnic Iut
"princ"rIcIoIIicuIarcpitIcIiunrotIcconcdogcnic
cI|ccrs oIscIun.
Dy5C0 LX0HD0lDD
ListriIurion, norpIology, and scvcrity oI Icsions
sIouId Ic rccordcd. Ir is inporrant ro diIIcrcntiarc
connon acnc IronnoduIocystic acnc.
Dfferental Dagnosis
TIc diI|crcnriaI diagnosis Ior acnciIorn rasIcs
incIudcs acncvuIgaris,drug-induccd acnc, CusIing's
syndroncororIcrparIoIogicstIaiincrcasccndogc-
nous srcroid sccrction, and pcrioral dcrnaritis.
Rosacca, an acnciIorn cruption oItlc ccntraI Iacc
andnccI, is sonctincs conIuscd vitl acnc,Iuriris
prinariIysccnin aduIrs.
Treatment
Trcatncnr sIouId Ic individuaIizcd dcpcnding on
rIc paticnr's gcndcr and rIc scvcrity, typc, and dis-
triIutionoIIcsions.
enzoyI peroxide vorIs Iy dccrcasing tlc colo-
nizationoII LLML and dccrcasingrIcdcvcIopncnt
oInicroconcdoncs Iy Icsscning rIe conccnrrarion
oI surIacc Ircc Iarty acids. TopicaI retinoids [c.g.,
Rcrin-) Iavc strong anriconcdogcnic acriviry,
Iovcvcr, sidc cI|ccrs nay Iinit usc and incIudc
dryncss, Iurning, and, nosr inporranr, pIoroscnsi-
tivity. TIc usc oIsunscrccn vitl a prorcctivc Iactor
[SFI) oIarIcasr I 5 isncccssary.TopicaIandsystcnic
antiIiorics arc uscd ro prcvcnr and dccrcasc coIo-
nizarionoII LLML. TopicaIantiIioricsarcaIso avaiI-
aIIc in conIinarion virI IcnzoyI pcroxidc. TIc
sysrcnic anriIiorics uscd incIudctcrracycIinc, doxy-
cycIinc, ninocycIinc, and cryrlronycin. In sonc
cascs, oraI conrraccprivcs vitl Iov IcvcIs oI andro-
gcns nay aIso Ic IclpIuI Iy supprcssing androgcn
producrion.
To naxinizc tIc tlcrapcuric Icnchts, conIina-
tiontIcrapyisusuaIIyprcscriIcd.MildacncvirIIcv
concdoncs is trcarcd virl IcnzoyI pcroxidc and
ropical antiIiotics. MiId acnc gcncraIly rcsponds to
rIcrapyvitlour scarring.
Manyconcdoncs and sone papulcs andpusruIcs
arccIaracrcrisricoInodcrarcacnc.TIcrapyincIudcs
IcnzoyI pcroxidc, rrcrinoin, and topical or oraI
antiIiotics.TIcrcisavariaIlc rcsponserorrcarncnt,
andscarringis apossiIiIityvitIrIisscvcrityoIacnc.
Scvcrc acnc is cIaracrcrizcd Iy inIannatory
papuIcs, pusruIcs, cysrs, aIsccsscs, and scarring.
TrcatncnL consists oI ropicaI tlcrapy and scIa-
ccous gIandsupprcssivc agcnrs, incIuding csrrogcns,
srcroids, andretinoic acid [ccutanc).BccauscoIirs
rcratogcnicity, a ncgarivc prcgnancy rcsr nusr Ic
oIraincd virIin 2 vccIs oIinitiaring rcrinoic ucid
rIcrapy, and contraccption nusL Ic uscd Iron
nontl IcIorc ro 1 nontl aItcr rIcrapy. ccuranc
rIcrapyusuaIIy lasrs 4 to 5 nontIs.
8
LN PC|WT
d
1. There|snoonewaytotreatacne,comb|nat|on
therapyworksbest.
PSOR| AS|S
Pathogeness
Tlepatlogcncsis oIpsoriasis is unInovn. nuIri-
IacroriaI inIcritancc parrcrn Ias Iccn proposcd.
CIiIdrcn virI II rypc C6 arc cIcarIy norc
liIcIy ro dcvcIop tlc discasc. IistologicalIy, rIcrc is
IypcrproliIcration oI rIc cpidcrnis, and cpidcrnal
turnovcr tinc is norcd to Ic disrincrly acccIcrarcd
intIoscaI|ccrcd.TIcrasIusuaIIyappcars arsitcsoI
pIysical, tlcrnaI, or nccIanical rrauna. TIis is
Inovn as tIc Kbner phenomenon, a diagnosric
IcaturcoItlc discasc.
Epidemiology
FsoriasisisconsidcrcdIysonetoIcanaduIrdiscasc,
Iur | 07oIcascsIcginIcIorctIcagcoII 0,andJ57
< not Ioz ca1o | > < no rn nom | >
< cran n oeano-ronnocnn: uv s n
.
q >
| ater ermatoogy b
IcIorc rlc agc oI20. IiItypcrccnr oI cIiIdrcn vitl
psoriasisIavcaposirivcIanilylistoryIortIcdiscasc.
IIpsoriasis is prcscntduringadoIcsccncc, ir is IiIcIy
a IiIcIong discasc.
Rsk Factors
!I inlcriranccisparroIrIcnodcoItransnission
tIcrcIorc,aposirivcIaniIyIistoryisasignihcantrisI
Iactor.
C!inca! Manfestations
5lDly 0D0 Dy5C0 LX0HD0lDD
TIc nonpruritic rasl consisrs oI crytIcnarous
papuIcs tIar coaIcscc ro Iorn pIaqucs vitI sIarpIy
dcnarcarcd Iordcrs and a siIvcry or ycIIov-vIirc
scalc.TIcscalcsrcndtoIuildupinroIaycrs,andtIcir
rcnovaI nay rcsuIr in pinpoinr IIccding [Auspitz's
signj. TIe rasI is usually synnctric, virI pIaqucs
appcaring ovcr tIc Inccs, clIovs, scaIp, and gcnitaI
arca. 1Icsc arc sitcs oI rcpcarcd trauna. TIc scaIp
Ircqucnrly Ias a rIicI, adIcrcnt scaIc vitI alopccia
atsitcs oIinvolvcncnt.TIenails oItcn dcnonsrratc
puncrarc srippIingorpitring, dcracIncnroItIc nail
platc [onycIoIysis), and accunulation oI suIunguaI
dcIris. Lxaninarion oI tlc palns and soIcs rcvcals
scaIing and hssuring. Fsoriatic artIritis nay also Ic
prcscnt.
Dfferentia! Dagnoss
Tlcdihcrcnrial diagnosis Ior psoriaticrasI in cliI-
drcn incIudcs unconnon disordcrs sucI as Rcitcr's
syndronc, pityriasis ruIra piIaris, and licIcn pIanus.
Reiter's syndrome incontrastrosinpIcpsoriasis,Ias
a psoriaricliIe rasI tIatinvolvcstIc nucous ncn-
Irancs. !n sonc scvcrc cascs in vIicI rIc rasI is
also acconpanicd Iy arrIriris, rlc Icsions oI tIc
nucousncnIrancarctlcnaindiIIcrcntiatingpoinr
Ictvccn psoriasis and Pcitcr's syndronc. Cccasion-
aIIy, aropic dcrnatiris nayIc conIuscdvitI psoria-
sis lovcvcr, cczcna is pruritic and psoriasis is not.
ScaIp Icsions nay Ic conIuscd virl scIorrIcic
dcrnariris orrinca capiris.
Diagnostic Eva!uation
JIe diagnosis is a cIinicaI onc. SIinIiopsyrcvcaIs a
IypcrpIasric cpidcrnis.
Treatment
Fsoriasis,liIecczcna, is cIaractcrizcdIyrcnissions
and cxaccrIarions. TIc nost inportanr aspccr oI
rrcaringpsoriasisistocducaterIcparicntand|aniIy
tIaL tIc discasc is a rccurrcnr onc rIai cannot Ic
curcd Iur can lc conrroIIcd virl conscicnrious
rIcrapy. lo nattcr vIcrc tIc rasl is or irs scvcrity,
rIc goaI oIpsoriasis tIcrapyisto IccptIcsIin vcIl
Iydratcd.TarprcparationsnayIc addcdtotIcdaiIy
IatI or uscd as an oinrncnr. Ior noic scvcrc cascs,
naruraIsunIigIrorulrraviolct B [I\B) IigItnayIc
uscdincon]unction vitItIcrarluIricant. IorsnaIl
arcas oI involvcncnt, Iluorinarcd srcroids nay Ic
succcssIuI,tIcIcastpotcnrIurchccrivcdoscsIouId
Ic uscd,Iccausc adrcnaI supprcssion can occur.
LN PC|WT5
M
Psoriasiscannotbecuredand ischaracterized by
remiss|onsandexacerbationsthatcan becon-
tro|ledw|thconscientioustherapy.
Psor|asisoccursatskin points ofrepeatedtrauma,
andtherash |snonpruritic.
d. Treatmentconsistsofkeepingtheskinwel|
hydratedw|thtarpreparationsthathe|pho|d
moisture intheskin.
ALLERG|C RASHES
Atopc Dermatts Eczema)
Atopic dermatiIIs [cczema} is aconnonsIindisor-
dcr oIinIancyand cIiIdIood and aIccts 5% oIclil-
drcnIcIorcrIcagcoI5. ScvcnrypcrccnioIaIIccrcd
cIiIdrcn Iavc Irsr-dcgrcc rclativcs cxliIiting sonc
Iorn oIaIlcrgic discasc, and J07 to 50% oIcIildrcn
vitIatojicdcrnaritisgoonrodcvcIopalIcrgicrIini-
tis or astIna. pproxinarcIy 607 oI aIIccrcd cIiI-
drcn dcvclop atopic dcrnatiris virIin rIe Irsr ycar
oIIiIc, and 907virIinrIcIrsr 5 ycars oIIiIc.
LDC0 N0Dl5l0lDD5
H|storyandPhysical |xami nation
1Ic rasI is cIaractcrizcd Iy crytlcna, cdcna,
papuIcs, and vccpingin tIc activcpIasc. ScaIcs and
Iiclcnihcarion nay dcvclop Iatcr. Scvcrc pruritus is
rIc IaIInarI oI cczcna. TIe ircIing is a consranL
Icarurc tIar crcatcs an "itcI-scrarcI-ircI cycIc." II
b4

not r ue na nyoDa | >



tlcic is no piuritus, it is unIiIcIy tlat rlc iasl is
atopic dcrnatitis. CcIluIitis can oIrcn Ic supciin-
poscd on a Iasc oIcczcna. 5. oureus and 5/ohj/o
coccus oenes aictlcusuaIIactciiaI agcnts. Icrpcs
sinplcx inIcction can aIso conplicatc atopic dcr-
natitis, lcading to a dil|use ciuption as cczcna
lcipcticun.
1lctlicccIinicaI plascs arc as IoIlovs.
Flasc |-inIantilc cczcna [2 nontls2 ycars).
Pasl appcais on rlc Iacc, nccI, scaIp, trunI, and
cxtcnsoi suiIaccs oIcxtrcn|tics and progicsscs to
plasc II inonc-tliid olpaticnts.
FlascII-lildlood cczcna [2I0ycars) . Pasl is
prcscntonllexoi suiIaccs picdoninantly [antccu-
IitaI,popliteaI,nccI,viists, sonctincs lands and
Icct),andone-tliidprogiessroadoIcsccntcczcna.
Flasc |II-adoIcsccnt cczcna. Iands [nostIy),
cycIids, nccI, Icct, andllcxoi aicas lavc iasl.
topic dcinatitis tcnds to rcnit and cxacciIatc.
1ypicaIly, tlceiuptionsIcconcniIdcivitlagc,and
Iongci icnissions occui.1iiggcis nayincIudc cxccs-
sivc Iatling and land vasling, occIusive clorling
[cspcciaIIy vooI), svcating, strcss, and possiIlylood
aIlcigy [cggs,niII,scaIood, nuts, vlcat,oi soy).
LllDl0 L0gDD55
Sonc ol tlc norc connon raslcs tlaf nusr Ie
dillcicntiatcd lron cczcna includc scIoiilcic dci-
natitis,diapci dcrnatiris,contactdcinaritis,scaIics,
psoiiasis, diug rcactions, IungaI inIcctions, and
icltlyosisvuIgaiis.
Lczcnatous Icsions aic not cxclusivcly duc to
aropic dcinatitis, |ccausc a varicty oI innunodcI-
cicncics can causc sinilai iaslcs. 1lcse incIudc
\isIort-ldiicl syndionc, agannagIoluIincnia,
Icinci's discasc [C5 dcIcicncy), andlistiocytosisX.
l0lHDl
1lc nosr inpoitant aspcct oIticating cczena is to
intcirupttlcitcl-sciatclcycIc.1lcIaniIicsnccdto
Iccducatcdtlattlc discase is aiccuricntonc tlat
cannot Ic cuicd Iut can Ie controIIcd vitl consci-
entious tlciapy. 1lcrapy is dircctcd at controIIing
dryncss, inllannation, and pruiitus. Ccneral nca-
suics incIudc avoiding cxriencs oltcnpcratuic and
lunidity, clenicaIs, strong soaps, ccrtain loods,
vooI, and syntlctic natciiaIs.
Scvcic atopic dcinatitis is ticatcd vitl vct
conprcsscs soaIcd H aluninun acctate soIution
[Buriov's solution), oatncaI Iatls [vceno Iatl),
antipiuiitics [lydioxyzinc), cnolIicnts [Lucciin
cican), andtopicaIstcioidsoiotlciinnunonodu-
Iatois [topical taciolinus) .
Urticara
lrticaria [livcs) is tlc nost connon typc ol
lypciscnsitivityicactionintlesIinand aIIcctsupto
207 oI cliIdicn at sonc tine. It is IgL ncdiatcd
andicsultsIionrcinrroductionoIan agcnttovlicl
tlc innunc systcn las Iccn picviousIy sensitizcd.
Connon causcs oI innunc-ncdiatcd urticaiia
incIudc diugs [pcniciIIin), Iood [Isl, cggs, pcanuts,
clocolatc), plysicaI lactois [coId, Iiglt, lcat), llood
and hlood products, and inIections [Lpstein-Bair
viius, lcpatitis, sticprococcaI plaiyngitis). lonin-
nunoIogicuiticaiiacanoccuiaIrcrIistcxposuicto
suclagcntsasaspiiin,opiatcs, oi contiast ncdia.
LDC0 N0Dl5l0lDD5
n urricaiiaI iasl consists oI vlcaIs, vlicl aic
iaiscd, palc, pinI pruriric aicas oI cdcna oI tlc
uppci dcinis. 1lc rasl cvoIvcs ovci scvcial louis
oi pcrlaps in a singIc day.1lc diagnosis is cIinicaI
and |ascd on claractcristic appcarancc and, vlcn
possiIIc, a histoiyoIcxposuie.1lcpicscnce oIcon-
cuiicnt aitliitis and lcvcr suggcsts tlc diagnosis ol
seiun sicIncss.
l0lHDl
voidingtlcpiecipitatingcxposurcistlckeytopic-
vention. Coldconpicsscs canIcapplicdto pruiitic
aicas, and tlc cliId nay Ie givcn antilistanincs
Iy noutl. ntipruritic ncdication nay Ic uscd ro
relicvc itcling, and aitlraIgias or aitliitis can Ic
tieatcdvitliIuproIcn.
Erythema Mu|tforme
Lrythema muItilorme is an acutc, sclI-linitcd,
lypciscnsitivity icaction tlat is unconnon in
cliIdrcn. Connon ctioIogic agcnrs incIudc viiaI
inIcction [hcipcsvirus, adenoviius, Lpstcin-Bair
viius),jco/usmo neumonioe inIcction,diugingcs-
tion [cspccialIysuIIadrugs),innunizations,andIood
reactions.
LDC0 N0Dl5l0lDD5
In ciyrlcna nuItiIoinc, tlcrc is a synnctiic
distri|ution oI Icsions cvoIving tliougl nuItipIc
noiplologic stagcs. ciyrlcnatousnacuIcs, papulcs,
< not Ioz sa1e | > < ue pLn nycpa | >
< cxau n pexaax~ouaeycun: s

>
ater ermatoogy bb
plaqucs, vcsicIcs, and taigct lcsions. 1lc Icsions
clangc ovci days, not louis. Liyilcna nulriIoinc
tcndstooccuiovcitlcdorsunoItlclandsandIcct,
paInsandsoIcs,andcxtcnsoisuiIaccsoIcxticnitics,
IutnayspicadtorlctrunI.Buininganditclingaic
connon. Systcnic naniIcstations incIudc lcvcr,
naIaisc, and nyalgias.
Stevens-Johnson syndrome is tlc nost scvcic
Ioin oIciytlcna nuItiIoinc.1lcic is a piodionc
Ior l to I 4days oI Icvci, nalaisc, nyaIgias, airliaI-
gias, aitliitis, lcadaclc, cncsis, and diaiilca.1lis is
IoIlovcd Iy suddcn onsct ol ligl lcvci, ciytlcna
nuItiIoinc sIin Icsions, andinllannatoiyIuIIac oI
tvo oinoic nucous ncnIiancs [oial nucosa, Iips,
IuIIarcon]uncriva, and anogcnitaIarca) . Intlcnost
scvcic cascs, invoIvcncnt ol nost oI tlc gastioin-
tcsrinaI, icspiiatoiy, oi gcnitouiinaiy tiacts nay |c
sccn. Inticatcd, rlis syndronc las a norraIiry iarc
olappioxinatcIy I 07.
Toxic epidermaI necroIysis i stlcnostscvcicIoin
olcutancous lypciscnsitivity, considcicdIysoncto
Ic a vaiiantoIStcvcns-1olnsonsyndionc.Itlougl
irs occuircncc in clildrcn is raic, it is associatcd
vitl a J07 noitality ratc. 1lc patlogcncsis is not
vclI undcrstood, Iut nost cascs aic sccondaiy to
ncdications, cspcciaIly suIIa diugs, anticonvuIsants,
and nonstcioidal anti-inllannatoiy agcnts. Cnsct is
acutc, vitl Iigl lcvci, a Iuining scnsation oI tlc
nucous ncnIiancs, and/oi oiaI and con]unctivaI
ciyrlcna and ciosions.Tlc picscntation oItlc sIin
icscnIIcs rlat oIstaplylococcaI scaIdcd sIin, virl
vidcsprcad ciytlcna, tcndcincss, IIistci |oination,
and dctaclncnt oI tlc cpidcinis causing dcnuda-
tion [positivc liIoIsIy's sign). Mucous ncnIianc
invoIvcncntisscvcicandtlcnaiIsnayIc slcd.Sys-
tcnic conpIications incIudc cIcvatcdlivci cnzyncs,
icnaI lailuic, and lluid and cIcctroIyrc inIaIancc.
Scpsis and slocIaiclicqucnt causcs oIdcatl.
l0lHDl
Ioi unconpIicatcd crytlcna nuItiloinc, synpro-
natic trcatncnt and icassuiancc aic aII tlat is
ncccssaiy.CiaIantilistanincs,noistconpicsscs,and
oatncaIIatls aiclclpluI.1lcIcsionsrcsoIvcovci a
I - to J-vccI pciiod, vitl sonclypcipigncntation.
Tlcusc oIcorticosrcroidsis contiovcisiaI.
Ticatncnt ol tlc paticnt vitl Stcvcns-1olnson
syndioncincludcslospitaIizationvirlIaiiici isoIa-
tion, lluid and cIcctrolytc suppoit, tlc rrcatncnt
oI connon sccondaiy inIcction oI tlc sIin, noist
conpicsscs on IulIac, and coIloidaI Iatls. Ioi oiaI
nucosaI Icsions, noutlvaslcs virl viscous Iido-
cainc, diplcnlydraninc, and MaaIox [aIuninun
lydioxidc, nagncsiun lydioxidc) aic conIoiting.
Bccausc coincal uIcciation, Iciatitis, uvcitis, and
panopltlaInitis arc possiIlc, an oplrlaInoIogy
consultationis rcconncndcd.
Clildicn vitl toxic cpidcinal ncciolysis aic
trcatcdastlougltlcylad IuIl-Iodysccond-dcgicc
Iuin. IIuidtlciapy and icvciscIaiiici isoIation arc
ciiticaIto suivivaI.
M
LN Ll W

1. A||erg|crashesareaspectrumofhypersens|t|v|ty
react|onsworsen|ng|nsever|tyfrom urt|car|ato
ery\hemamult|formetoStevensJohnsonsyn-
drometotox|cep|dermal necrolys|s.
Z. Lczema|sachron|cd|sease thatcannotbecured
but| nwh|chrem|ss|onsandexacerbat|onscanbe
control|edw|thconsc|ent|oustherapyd| rectedat
stopp|ngthe|tchscratchcyc|e.
. Urt|car|a|sthemostcommontypeofhypersens|-
t|v|tyreact|on |nthe sk|n andafIectsone| nhve
ch||dren.
4. Stevens-Johnsonsyndrome|sery\hemamult|-
formew|thoralmucosalbu||ae,whereastox|c
ep|derma|necro|ys|s|ss|m||artostaphy|ococcal
sca|dedsk|n|n thatboth resu|t|n s|ough|ngof
theep|derma|layer.
HYPERP| GMENTED LES| ONS
\irlrlcincidcnccoIncIanonaincicasing,itisvciy
inpoitant to idcnti[ suspicious lcsions and undcr-
srand iisI |actois. Clildicn vitl Iaii sIin, cxccssivc
suncxposuic,andnuItiplcncviaicatincicascdiisI
Ioi sIin cancci.
Congental Nev
CongenitaI nevi arc cIassiIcd Iascd on tlcii sizc.
Iaigcoigiantncviaicgrcatcrtlan 20cn,snaIlncvi
arcIcsstlan2cn,andintcrncdiatcncviaicIctvccn
2 and20cn. Pcscarcllasslovntlatrlcicisanill-
dcIncd Iur incicascd isI oI ncIanona in aricnts
virl congcnital ncvi. CongcnitaI ncvi nust Ic Iol -
Iovcd annuaIIy Ioi clangcs and nay icquiic con-
pIctc cxcision. Cianr ncvi lavc an incrcascdiisIoI
nclanona [|ctvccn 57 and I 57). 1lcic is also an
associarion vitl ncuiocutancous ncIanosis, so
< not Ioz sa1e | > < ue pLn nycpa | >
||uOprl n!3 |CO|I||CS
< cxau n pexaax~ouaeycun: HYC s you. |V >
b
#
paticntsvitllcsionsovcitlcIcad andspincicquiic
an MPIto cvaluatclor ClS |nvolvcncnt.
Common Acqured Nevi
ManycliIdicnviIIgoontodcvclopncvi,icaclinga
naxinun nunIci in caiIy aduItlood. Faticnts vitl
noic tlan I 5 connon acquiicd nolcs lavc an
incicascd iisI loi ncIanona in tlc Iutuic. MoIcs
nccdtoIcasscsscd|yusingtlcBCIs.MoIcsvitl
asymmetry iiicguIaiborders, vaiiationsincolor, and
diameter Iaigcrrlan6nnhavcatypicaIIcatuicsand
nayicquiic cxcision.
Spitz nevus is a snootlpinI to Irovn donc-
slapcdpapulc.1lcscncviaicIcnign,Iutnaynccd
toIcicnovcd iItlcygiov iapidly. haIo nevus is
a noIc vitl a dcpigncntcd ring aiound it. 1lcsc
Icsions aicIcnign, Iut aic associatcd virl tlc pics-
cnccoIvitiIigo oincIanona at anotlci sitc.
Preventon
Iargc anount oI cliIdlood sun cxposuic and
IrcqucntsunIuinsaic associatcd vitl incicascdiisl
Ioi tlc dcvcIopncnt oI noIcs and sIin cancci. Sun
piotcction virl a sunIIocI laving an SFI oI I 5 oi
noic againstI\B andI\ Iigltis icconncndcd.
LN Ll W

1. Mo|esneedtobeassessedforasymmetry,irregu-
|arborders,color,ands|ze.
Z. Sunb|ockagai nstUV6andUVA |ight isrequi redto
decreasether|skofmelanoma.
D| ABETES ME
||
|

S
m @ g g g @ g ~ ~ ~ *
|nsuln-Dependent Diabetes
Mellitus Type 1 )
0lDDgD55
LiaIctcs ncIIitus is a clionic nctaIoIic disoidci
claiactciizcd Iy lypciglyccnia and aInoinaI
cncigy nctaIoIisn duc to aIscnt oi dininislcd
insuIin scciction. InsuIin-dependent diabetes meI-
Iitus [LLN type I icsuIts Iron IacI oI insulin
pioduction in tlc B ccIIs oItlc pancicas. Itlougl
tlc piccisc ctioIogy oI ILLM is unInovn, gcnctic,
autoinnunc, and cnviionncntaI Iactois lavc aIl
Iccn inpIicatcd.
Irci 907 oIB-ccII IunctionlasIccn dcstioycd,
IossoIinsuIinsccictionIcconcscIinicalIysigniIcant.
\itltlcIossoIinsulin,rlcna]oranaIoIicloinonc,
a cataIoIic statc dcvcIops, vlicl dccicascs gIucosc
utiIization and incicascs gIucosc pioduction Iy
gIuconcogcncsis and gIycogcnoIysis. 1lc IacI oI
insuIin picvcnts gIucosc Iion cntciing tlc ccII, and
lypcigIyccnia icsults. 1lc pioduction oI Ictoacids
is Iiouglt aIout Iy an incicasc in tlc cataIolic
ncdiatois gIucagon, cpincpliinc, giovtl loinonc
[CI), and coitisol.1Icsc ncsscngcisrriggcilipoly-
sis, Iatty acid rcIcasc, and Ictoacid syntlcsis. \lcn
tlcIIoodgIucoscconccntrationcxcccds I 80ng/dI,
tlc icsuIrant gIycosuiia causcs an osnotic diuicsis
vitl incicascd uiinc output [poIyuiia). II insuIin
dcIcicncy is scvcic, Ictoncs aic produccd in
signiIcant quantitics, tlc Ilood's nativc IuIIciing
capacity is ovcivlcIncd, and diabetic ketoacidosis
[LK) rcsults.
LK is claiactciizcd Iy lypcigIyccnia, ncta-
IoIic acidosis [Ictoacidosis), dclydiation, and lc-
tlaigy.ItisancdicaIcncigcncyrlat,inscvciccascs,
naypiogicssrocona and dcatl.1lcnost connon
causc oI LK in tlc Inovn diaIcric is inadcquatc
insulin dosing. 1lc condition can aIso Ic triggcrcd
Iy insuIin icsistancc, vlicI is cxaccrIatcd Iy
an intcicuircnt iIlncss oi cxtrcnc plysiologic
strcss. Iicqucntly, ncv-onsct diaIctics picscnr
in LK. 1lc nosr scvcic conplication oI LK
nanagcncntisccrcIiaI cdcna.
InadditiontoLK,rlcotlcina]oiconplication
sccn in ILLM is lypogIyccnia Iron insuIin ovcr-
dosc, dccicascd caIoiic intaIc, oi incrcascd cxcrcisc
virlouta conconitant incicasc incaIoiics.
LQ0HDDgy 0D0 h5K 0ClDl5
ILLM is tlc nost connon cndocrinc discasc in
cliIdlood, occuiring in I in 500 clildicn and
adoIcsccnts. 1Ic nain iisI Iactoi Ioi |LLM is a
IaniIyhistoiy.1lc prcscncc oILPJ and LP4 na]oi
listoconpatiIiliry antigcns incicascs tlc liIctinc
iisI Ioi an individual dcvcIoping |LLM, as docs
Iaving a Ist-dcgicc icIativc vitl ILLM. 1Icicis a
507 concoidancc anong idcnticaI tvins. 1lc prcs
cncc oIantiisIct ccIlantiIodicsin 857 oIindividu-
aIs vitl rcccnt-onsct |LLM and tlc incrcascd
appcaianccoIotlciautoinnuncdiscascsinclildrcn
vitl ILLM naIc tlc casc Ioi an auroinnunc
ctioIogy. 1lc cnviionncntaI ioIc in discasc pa-
tlogcncsis rcnains uncIcai. lo particuIar viius las
Iccn dctcinincdtoIcdiicctIyicsponsiIlc.
LDC0 N0Dl5l0lDD5
Hi storyandPhysical Lxaminat|on
listoiy oI ncv-onsct vciglt Ioss, polydipsia,
poIyplagia, and poIyuiia is consistcnt vitl rypc
bb

o o

> < ue Ln nyoDa | >


t
I diaIctcs neIIitus. 1lc plysicaI cxanination is
gcnciaIIy noinaI in typc I diaIctcs ncIIitus unIcss
LK/is picscnt.
VIcn LK is suspcctcd in a cliId vitI Inovn
ILLM, inportant listoric inIorna\ion includcs tlc
usuaI insuIin dosc, tlc Iast insuIin dosc, tIc cliId's
dict ovci tlc picvious day, and vlctIci tIc cliId
las Iccn iII and cnotionaIly oi plysicaIIy sticsscd.
1lc cliIdvitl LK/ appcais acutcIy iII and sullcis
Iion modciate toproIound dclydiation. Synptons
includc poIyuria, poIydipsia, Iatiguc, lcadaclc,
nausca, cncsis, and aIdoninaI pain. 1lc cliId's
ncntal status nayvaiy |ion conIuscd to conatosc.
CnpIysicaIcxanination,taclycaidiaandlypcrpnca
[KussnauI icspirations) are gcncialIy noted. 1lcic
nay Ic a Iruity odor to tlc IicatI Iecausc oI
tlcIctosis.IntiavascuIaivoIuncdcpIctionnayIcso
naiIcdtlathypotcnsionnayIc dctcctcd.ltlougl
ccicIiaI cdcna is iaic, it oItcn is Iatal. CIanging
ncntal status, uncqualpupiIs, dccoiticatc ordcccrc-
Iiatc posutiing, and/oi scizuics indicate ccicIiaI
cdcna. EaiIy idcntiIcation and aggicssive nanagc-
ncntolincicascdintiacianiaIpicssurcaicpivotaIto
inpiovc outconc.
Synptons oI lypoglycenia aic due to catc-
clolaninc icIeasc [ticnIIing, diaploicsis, lusling,
and taclycaidia) and to ccieIial gIucopcnia [slccpi-
ncss,conIusion,noodcIangcs,scizuics, and cona) .
LlPlPDl0 L0gDD55
SccondaiydiaIctcs nayoccuivlcn tlcieisinsuIin
antagonisn Iion cxccss gIucocoiticoids [Cusling's
syndioncoiiatrogcnic),lypertlyioidisn,plcoclio-
nocytona, CI cxccss, oi vitlncdications sucl as
tliazidc diuietics.
L0gDD5lC Lv0U0lDD
1vo iandon IIood gIucosc IcvcIs gieatci tlan
200ng/dI are consistcnt vitl adiagnosisoI ILLM.
II caiIy ILLM is suspcctcd, a 2-loui postpiandiaI
IIood gIucose conccntiation is tlc Iisf valuc to
IcconcaInoinal.IastingIIoodgIucoscconccntra-
tiongicatci tlan I 26ng/dL anda 2-lour postpran-
dial Ilood gIucosc concentration greatcr tlan
200ng/dI aic suggcstivc oI ILLM. IsIct ccII
antiIodics H tlc sciun nay Ic Iound in tlc
ncv-onsct insuIin-dcpcndcnt diaIctic poorIy
contioIIcddiaIctics lavc ligl IcvcIs oIgIycosyIatcd
IcnogIoIin.
In cliIdicn vitl suspccted LK/, tIe sciun
gIucosc conccntration is giossIy clcvatcd, and tlc
venous pI and sciun lCC, aic Iov. MctaIoIic
acidosis Iion Ietosis icsuIts in dininislcd pI, and
tlercsponsctonctaIolicacidosisisaconpensatory
iespiiatoiy aIIaIosis and a diop in sciun ICC,.
BccauscoItIcosnoticdiuicsis,IIooduicanitrogcn
is clcvatcd and tlcic is loss oI plosplatc, caIciun,
andpotassiun.ltIougltlcieis atotaIIodyIossoI
potassiun, scrun potassiun nay Ic Iov, nornaI, or
cvcn ligl dcpending on tIc levcl oIacidosis.Vlcn
acidosis is picsent,piotonsnovc Iion tlccxtiaccl-
luIai spacc to tlc intracclIulai spacc and potassiun
movcslron tleintraccIIuIai spacctotlccxtiacclIu-
lai space to naintain clcctroncutraIity. Intil tIc
cataIoIic statc is icvciscd vitl insuIin, tIe uiinc is
positivcIoiIctoncs,untiItlcscrunconccntiation oI
gIucosc Ialls Iclov I 80ng/dI, tlc uiinc is positivc
|or gIucosc.
lP0lHPDl
1lc inncdiate goaIs oI treatncnt oI nev-onsct
|LLM and |K aic to rcveisc tlc cataIolic statc
tliougl cxogcnous insulin tIciapy and to icstoic
Huid and cIcctroIyrcIosscs.
1lc cIiId vitl ILLM is ticatcdtliougl insuIin
ieplaccncnt, dict, cxcicisc, psycloIogical support,
andicguIaincdicaIloIIov-up.Faticntcducationlas
a vitaIiolc. CuiicnttIciapyiequiics IicquentIIood
gIucosc nonitoiing and caiIolydratecounting.1lc
paticnt Icarns lovto taiIoiinsulin dosingIascd on
tlc gIucosc Icvcl and tIc cuiicnt ncaI. 1lc ncvIy
diagnoscd diaIetic icquiics 0. 5 to I .0 unit/Ig ol
insulin pci day. Most diaIctics viII taIc insulin tvo
to tlicc tincs a day. It is custonaiy to givc
tvo-tliids oI tlc totaI daiIy dosc IcIoic IicaIIast
and onc-tliid IcIoic dinnci, and tlc lunan
insuIin is dividcd Ictvccn sloit-acting rcguIar
insuIin and inteincdiatc-acting lFI insulin. /
insuIin punp las nov Ieconc availaIlc. 1lis
dcIivcis a Iasal anount oI insuIin tlrouglout
tlcday,vitlIoIusdoscsoIsloit-actinginsuIingivcn
at ncal tincs. t tincs oI ncdicaI, surgicaI, or
enotionaI stress, additional insuIin nay Ic
necdcd. Clycosylatcd lcnogloIin l cvcls slould Ic
nonitoicdcvciyJnontlstoasscssavciagegIyccnic
controI.
II IypogIycenia occuis, a cIild nay ingcst a
caiIolydratc snacI to incicasc tlc scrun gIucosc
conccntiation. II tIc clild is voniting, Monogcl
nct fcz ca1o | > < no pLn pcpa | >
instant gIucosc or caIc icing nay Ic apjIicd to tIc
IuccaI nucosa to ptovidc gIucose. II tlc cliId is
stuporousorlavingascizurc,intravenousglucoscor
intranuscuIargIucagonnayIc given.
LKis a ncdicaI cncrgcncy. InitiaI lluid rcsusci-
tationisacconplislcdIygivinganornaIsaIincorIac-
tatcd Ringcr's soIution, I 0 nI/Ig intravcnous IoIus.
VliIe tlc lluid Iolus is running in, tIc tofaI lluid
dcIcitiscaIcuIarcdIascdontIc anountcldcIydra-
tion.TlclluiddcIcitslouIdIc repIaccd ovcr a 48-
lour pcriod.1leIcvcl ollypcrgIyccnia is asscsscd
andaninsulindripisstartcdat0. Iunit/kg/lrJlcgoaI
istodecrcasctIcscrungIucosc50 to l 0ng/d!/r.
glucosclcvcl tIatlaIIstooquicIlycouIdprccipitatc
ccrclraIcdcna.VIcnscrunglucoscapproaclcs250
toJ00ng/dI, dcxtrosc slouId Ie addcd tonornaI
saIine and tIc cIcctroIytc solution to avoid lyjo-
gIycenia. cidosis and Ictonc production corrccts
vitl insulin tlcrapy. IntiltIcre is adcquatc insuIin,
tlcIodyviIlcontinuctoproduceIetoacids.Ircqucnt
nonitoring ol Ilood glucosc lcveI, clcctrolytcs, and
acid-Iasc statusiscrucial.
lDgDD55
TIo LiaIctcs ControI and ConpIicaLions Trial las
dcnonstratcd tlat intcnsivc nanagcncnt and tigli
gIyccnic control vilI rcduce tle risI ol conpIica-
tions Iy 50% to 75%. ConpIications Iron |IL
incIude nicrovascuIar discasc oI tlc cyc [rctinopa-
tly), kidnoy [ncjlropatly), and ncrvcs [ncuropa-
tly).MicrovascuIardiscascisgcnerallynotsccnuntil
tlecliIdlasIecninsuIindcpcndentIoranininun
ol | 0 ycars. cccIcratcd largc vesscl atlcroscIcrotic
disease can lcad to nyocardial inIarction or stroIc.
LiaIetic clildrcn sIouId lavc annual urinc coIlcc-
tions to scrccn |or nicroaIIuninuria, annuaI opl-
tlalnologic cxaninations, and annual scrccning lor
lyperIipidcnia.
Noninsulin-Dependent Diabetes
Melltus Type 2)
0lDDgD55
PoninsuIin-dependcnt diabetes mcIIitus [PlLLM}
type Z is a polygcnic condition tlat rcsults |ron a
rcIativc insulin rcsistancc. Tlis insulin rcsistancc
initialIy causcs a conpcnsatory incrcasc in insulin
sccrction, lovcvcr, vitl tine tlere is iogrcssivc
dccIinein tIc gIucosc-stinuIatedinsuIinsccrction.
LQ0DDDgy
NILLM accounts |or Z7 to J% oI aIl diaIctcs in
cIiIdren. Hovcvcr, tlc incidence is incrcasing
Iccauseo|tlc Iigl prcvalcncco|oIesify.Mostcascs
occur during carIy adolcsccncc around flc onsct ol
puIcrty. Ircvalcncc is IigIcst in lativcncricans,
Irican ncricans, andIispanics. Gcnctic susccpti-
Iility is inportant, Iovcvcr, cnvironncntaI Iactors,
includingoIcsiry plysicaIinactivity, and dict,play a
najorroIc.
5lDly 0D0 Dy5C0 LX0D
Many paticntsvilI Icasynptonaticatprcscntation.
Ctlers naylavc synptons siniIar to tloscoItypc
I diaIetics.TlcrcisusuaIly apositivcIaniIyIistory
Cn plysical cxanination, oIcsity is notcd, vitI a
Iody nass indcx [BMI) usually greatcr tIan 85%.
CItcn associatcd vitl lILLM is acantIosis nigri-
cans, a sIin condition involving lypcrpigncntation
and tIicIcning olrlc sIin IoIds, Iound prinariIy on
thc IacI oItIonccI
l0lDDl
Currently, tIc nainstay o| rrearncnt is insulin
tIcrapy.ManyoraIIypoglyccnicagenrslavcnotyct
Iccn testcd in clildrcn, tlcir usc is jrinariIy
anccdotal Morc rcscarcl is nccdcd in tIis arca. In
addition to ncdicaI tlcrapy, IiIcstylc clangcs
dictandcxcrcisc arcparticularlyinportant.
M
LN L W
1. D|abetes me|||tusisachronicmetabolicd|sorder
characterized by hyperglycen|aandabnorma|
energynetabo|ismcausedbyabsentor
di m| nishedinsul|nsecret|onoraction atthe
ce|lularlevel.
Z. lnsu|i ndependentdiabetesmel|itus(|DDM)type
1 resultsfromalackof|nsulinproduction|n the 6
ce||softhe pancreas.
d. Ah|storyofnewonsetwe|ght |oss,po|ydipsia,
po|yphag|a,and polyur|a|sconsistentw|thtype 1
d|abetesme|||tus.
4. Long-termcomplicat|onsfron |DDMinclude
n|crovascu| ard|sease(ret|nopathy,nephropathy,
and neuropathy)and acce|eratedlargevessel
atheroscleroticd|sease.
b. 1hepercentageofNl DDMcasesinch|ldren|s
r|s|ng.
< cx V pexaax~ouaeycvn: HC s pou. y >
D| ABETE5 l bl l LLb
g , , , g , g , , , g , g , , g , g , g , . g , g , , , , , , g , g @ , g , , g
In diabetes insipidus tlcic is Ioss oI antidiuictic
loinoncsccictionIron tlcostciioiituitaiygIand
and an inaIiIity to conccntratc tle uiinc. LiaIctcs
insiiduscanoccuiaItcileadtraunaoivitlaIiain
tunoi or ccntialncivoussystcn inlcction. SurgicaI
intciiution oI tlc ituitaiy staII duiing cianio-
laiyngiona ienovaI olfcn icsuIts in diaIctcs
insiidus.CnlyiaiclyisdiaIctcsinsiidusanisoIatcd
idioatlic disoidci.
Clinical Manifestations
Tlc clild vitl diaIctcs insiidus las aliut-onsct
olydisia and olyuria. IItlc cause oItlc diaIctcs
insiidus is aIiaintunoiiningingontlcituitaiy
gIand,IocalncuioIogicsignsandvisuaIaInoinalitics
nayIe notcd.
Tlc incicascd uiinc outut nay rcacl 5 to
I 0I/day,vitlauiincsccihcgiavityanduiincosno-
IalitytlataicquitcIov.Cvcitinc,seiunsodiunand
sciun osnoIaIity incicasc, as lcnoconccntration
occurslionlrccvatcrloss.InuncIcaicascs tlevatcr
dciivation tcst is uscd to docuncn diaIctcs
insiidus. Lcnonstration oI antidiuictic loinonc
[LI) scciction is ciiticaI in dillcicntiatingLI-
dchcicnt diaIctes insiidus Iron ncliogcnic dia-
Ictcs insiidus, a raic X-IinIcd icccssivc discasc in
vlicltlecoIIcctingductsdo notrcsondtoLI.
Treatment
LL\F, an LI anaIoguc, is givcn intranasaIIy,
intravcnousIy, oi oialIy to stinulatc tlc Iidncys to
ictainvatciandrcvcrsctlc olyuiia,olydisia,and
lycinaticnia.
LN Ll W
\
1. | nd|abetesinsipidus,thereislossof ADHsecre-
t|on andan inab||itytoconcentratethe urine.
Z. Diabetes |nsipiduscanoccurafterheadtraumaor
w|tha braintumororcentralnervoussysten
| nfection.

_5JORT 5TATURE
- , . ~ + . . . . , . . g . . , .
~
, , g , . , . , . , g g , . . , , ,
Shor staturL is a connon conccin oI aicnts.
loinal causcs incIudc laniIial [gcnctic) slort
statuic and constitutionaI dcIay. Eiglty cicent oI
cascs oI sloit statuic aie attriIutaIlc to tlcsc
tvo causcs. FatloIogic causcs nay rcsult in eitlci
disproportionate or proportionate short stature.
EtioIogics tlat icsuIt in iooitionate sloit staturc
arc nucl noic icvalcnt tlan tlose oI disioor-
tionatc sloitstatuic.
Lisordcrs tlat icsult in disiooitionatc sloit
staturc allcct tle Iong Ioncs icdoninantly and
incIudciicIcts,vliclis causcdIyactivatcdvitanin
L dchcicncy, and aclondroIasia, an autosonal
doninantdisoidci.
Liscascs tlat causc rooitionate sloit statuic
nay icsuIt Iron citlci a icnataI oi ostnataI
insuIt to tlc giovtl ioccss. FicnataI ctiologics
includc intiautciinc grovth ictaidation, IaccntaI
dysIunction, intiautciinc inIcctions, tciatogcns, and
clionosonaI aInornaIities. 1lc nost connon
clionosonaI aInoinaIities tlat iesuIt in slort
statuic aic trisony 2 I and Tuinei's syndionc.
Fostnatal causcs includc naInutrition, clronic sys-
tcnic discascs, syclosocial dcrivation, diugs, and
cndociinedisoidcis.ConnoncndociincdcIcctstlat
icsuIt in sloit statuic incIudc lyotlyioidisn,
giovtl loinone [CI) dchcicncy, glucocoiticoid
cxccss, and iccocious uIerty.
Differental Dagnosis
CliIdrcnvitllamiIiaI short stature estaIIislgrovtl
cuivcs atoi IcIov tlchItlciccntiIcIytlc agc oI
2. Tlcy aic otlcivisc conIctcIy lcaItly, vitl a
noinaI lysicaI cxanination.Tlcsc cliIdrcn lavc a
noinaIIoncagc,anduIcityoccuisattlccxcctcd
rinc. Sloit statuic is usuaIly Iound in ai Icast onc
arcnt, Iui lciglt inlciitancc is conIcx and thc
dininutivc anccstoi nayIe norc distant.
CliIdion vitl constitutionaI deIay giov and
dcvcIo at oi IcIov tlc hItl pcrcentiIc at noinaI
giovth vclocitics. Jlis icsults in a cuivc aiallcl to
tlc hItl ciccntiIc. FuIcity is signihcantIy deIaycd,
vlicl iesuIts in a dcIay in tlc Ionc agc. Becausc
tlcsc cliIdrcn IaiI to cntei uIcity at tlc usuaI
agc, tlcii sloit staturc and scxual innatuiity aic
acccntuatcd vlcn tlcii ccrs cntci puIoity. IaniIy
ncnIcis aic usuaIIy oI avciagc leiglt, Iuf tlcic
is oItcn a listoiy ol sloit statuic in chiIdlood
and dcIaycd uIcrty. Tlc arcnts oI cliIdrcn
vitl constitutionaI dcIay slould Ic counsclcd tlat
tlcir cliId's giovtl is a noinaIvariantand tlat tlc
cliId viII IiIcly natuic to tlc lciglt cxccted Ioi
tlcii Ianily.
< nct fcz sa1e| > < ue pLn nycpa | >
< cxau n pexaax~ouaeycnn: HYC s u.
V
>
L|QIC| 1OOC||1O| OQy b1
Cl dehciency accounLslorapproxinaLcIy 57 oI
cascs oI slorL staturc rcIcrrcd to cndocrinoIogists.
Clildrcn viLl cIassic CI dcIcicncy grov ai a
dininislcd grovrl vcIocity, Icss tlan 5cn/yr, and
lavc dcIaycd sIcIctaI naiuration. lisiory o|Iirrl
asplyxia or nconaLal lypoglyccnia or plysicaI
Indings oI nicroplalIus or nidIinc dc|ccts arc
suggcsLivc oI idioparlic CI dcIcicncy. CI dcI-
cicncysccondaryLolyporlalanicorpiiuirarytunor
usuaIly is associaLcd virl orlcr ncurologic or visuaI
inpairncnLs. |n an oIdcr cliId vitl nore rcccnt
onsct oI suInornaI grovrl, tlc indcx oI suspicion
lor LunorslouIdIcligl.
lrimary hypothyroidism causes narIcd grovrl
Iailure Iccausc oIa dininislcd grovtl vcIociiy and
sIclctaI naLuration. 1lyroxinc [), triiodotlyro-
ninc rcsin uptaIc [1,R!), LlyroLropir [1SI), and
Llyroid aniiIodics slouId Ic ncasurcd, cvcn in
Llc alscncc ol synpLons, to ruIc out any dcgrcc
oI lypotlyroidisn vlcn cvaluaiing slorr sLaturc.
Irinary lypoLlyroidisn is rrcaicd vitl Icvoilyrox-
inc [Synrlroid).
Cushings disease IS a rarc causcoIslort staturc.
IypcrcortisoIisn, Iron cirlcr cxogcnous stcroid
Llcrapy or cndogcnous ovcrsccrcLion, nay lavc a
proIound grovil-supprcssion chcct. IsualIy, orlcr
sLignataoICusling'ssyndroncarcprcscnt iIgrovtl
supprcssion las occurrcd.
Clronic systcnic discascs car rcsuIt ir slort
staturc Iron IacI oI caIoric aIsorption or incrcased
nctaloIic dcnands. Cyanotic lcarr discasc, cysiic
IIrosis, poorIy controlIcd diaIctcs nclIitus, clronic
rcnaI Iailurc, II\ inIccLion, and scvcrc rlcuna-
Loid arilritis arc disordcrs tlat i ncrcasc ncta-
Iolic dcnands and dininisl grovrl. ItcrnativcIy,
inIlannatory IovcI discasc, ccliac spruc, andcystic
IIrosis can rcducc caIoric aIsorption and producc
slortstaturc.
Sonc cliIdrcn vlo livc in cnotionaIly or plysi-
caIIy aIusivc or ncglccLIul cnvironncnts dcvcIop
Iunciional CI dcIcicncy. CliIdrcn vitl psychoso-
cia| dcprivation nay lavc Iizarrc Iclaviors Llat
incIudc lood loarding, pica, and cncoprcsis, as vclI
asinnaturcspcccl,disturIcdsIccp-vaIccycIcs,and
an incrcascd paintoIcrancc. ClinicaIly,tlcyrcscnIIc
clildrcn vitl CI dcIcicncy, viil narIcd rctarda-
iion oIlonc agc andpuIerraldclay. IICItcstingis
donc vlilcLne clild rcnains inrlc lostiIccnviron-
ncnt, ilcrc is a IIuntcd CI rcsponsc vlcn Llc
cliId is rcnovcd lron tlc dcprivcd cnvi ronncnL,
CI tcsting rcvcrts Lo nornaI and catcl-up grovtl
is noLcd.
Cnc olrlc naniIcsLaiions olTurner's syndromc
vlicl is discusscd in dcLail in ClapLcr 9, is slorr
staturc. 1lc clinicaI naniIcsiations ol 1urner's
syndronc can sonctincs lc sulLIe. Civcn tlai rlc
incidcnceoI1urncr'ssyndroncis I in Z500 |cnaIcs,
gonadotropins and Iaryotypc LcsLing are indicaLcd
in tlc IcnaIc adoIcsccnL virl slorr staLurc and
dcIaycd pulcrry. ElcvaLcd gonadotropins, indicating
prinary ovarian IaiIurc, and a 45,XC Iaryotypc arc
diagnosiic.
Clronic adninistraLioo oI ccrrain medications
nay rcsuIt in poor grovtl. Such drugs includc
sLcroids, dcxtroanplctaninc [Lcxcdrinc), and nc-
tlyIplcnidatc [RiLalin).
C!inca! Manfestatons
5lDly
InportantlistoricaIinIornation incIudcstlcclild's
prcnaralandIirtllisiory, tlcpaLtcrro|grovtl,rlc
prcscncc oI clronic discasc, Iong-Lcrn ncdication
usc, ilc aclicvcncni oIdcvcIopncntal niIcsioncs,
andilcgrovtlarduIerraIpaiicrnsoIilcpaiicnt's
parcnts and siIIings. CItaining and cvaluaiing
rlc cliId's grovtl clarts are viLaIIy inporrant.
tlorougl Iccding listory, |ncIuding vlaL, lov, and
lyvlontlc clildisIcd,isaIsorcquircd.
Dy5C0 LX0HD0lDD
1lcna]orityoIplysicaI cxaninaiions donconcliI-
drcn viLl slort siaiurc arc nornal. IL is criiicaI Lo
plot rlc cliId's lciglL and vciglL on rle appropri-
atc grovtl curvc Ior agc. In addirion iolciglL, arn
span anduppcr-to-Iovcr-Iodyscgncntrario is nca-
surcdtoclccIIorparloIogicdisproportionatccauscs
oIslorr staturc. Inyoung clildrcr,rlc lcad circun-
|crcncc slouId aIsolccvaIuarcdio clccIIorIaiIurc
to tlrivc. In cliIdrcr vith laiIurc to tlrivc, vciglL
and lciglt arc dininislcd and tlc lcad circunIcr-
cnccisoItcnsparcd.\lcncxaniningrlc cliIdviLl
slorLsLaLurc,LlcplysiciannayInddysnorplicIca-
iurcsinapattcrnsuggcsrivco|aparticu
_
rsyndronc.
Tlc intcguncnt slouId Ic cxanincd Ior cyanosis
indicating poLcntiaI congcnitaI lcari discasc, aInor-
nal pigncntation notcd in Cuslirg'ssyndronc,tlc
stignaia oI lypoilyroidisn, and Iruiscs and poor
lygicne indicarivc ol psyclosocial dcprivation.
1lc tlyroid is palpaLcd Lo dctcrnirc its sizc, its
consistcncy, and rlc prcscncc oI rlyroid nodulcs.
1lcIungsandlcartarccxanincdtoidcnriIyclronic
cardiopulnonary discasc. ldoninaI icndcrncss or
< not Ioz sa1e | > < ue pLn nycpa | >
|lucgrl|lO Ped|atr|cs
< cxau n pexaax~ouaeycnn: HYC s you. |V >
bZ
IIoatingnayi ndicatcinIannaforyIovcl discasc or
ccIiac sruc. 1anncr staging Ior Iotl Ioys and girIs
nusf Ie docuncntcd to lcI diIlcrcntiatc anong
IaniIiaI slort staturc, constiturionaI dcIay, and
rccocious uIcrfy. rlorougl ncuroIogic and
Iunduscoic cxaninafion nay rcvcaI undcrIying
ccntraI ncrvous sysfcn discasc rlat nay rcsuIf in
CIdcIcicncy.
Dagnostc Eva!uaton
Bccausc nost cascs oI slort staturc rcsuIt Iron
citlcr IaniIiaI slorL sLaLurc or constiturionaI dcIay,
diagnostic sfudics arc gcncraIIy nof ncccssary
unIcssaInornaIitics arcIoundon cxan.loncagc
[antcroostcrior x-ray oIrlc lcIt vrisL) asscssncnt
lclsfo dcIincafc IaniIiaI slortstaturcIron consfi-
fufionaIdcIay.nadvanccdIonc agcliIcIyindicafcs
rccociousuIcrty,anornaIIoncagc,IaniliaIslorr
staturc, anda dcIaycd Ionc agc, consriturionaldcIay.
1lyroid IuncLion LcsLs nusr Ic donc to ruIc out
lyorlyroidisn. IrinaIysis and rcnaI Iunction tcsts
arc rccdcd to rulc out clronic rcnal discasc.
conIcfc IIood counf vitl dihcrcntiaI and an
crytlrocytc scdincnfation ratc nay rcvcal cvidcncc
oIclronicsystcnicinIccfion.1lc clild's nutritionaI
sLatus canIc cxanincdrlrougltlc scrun alIunin
and totaI rofcin counts. scrccn Ior insuIin-IiIc
grovrl IacLor-I [ICI- I ) and insuIin-IiIc grovrl
Iacfor Iindingrotcin-J [ICI-BFJ) nay Ic ordcrcd
toIooIIorCIdcIcicncy.IlaclronosonaIanonaIy
is considcrcd, oItaining aIaryotycnayIclcIIul
nagncticrcsonanccinagco|rlclcadnayidcnri[
alyotlaIanicorituifaryroccssrlatisrcsuItingin
dccrcascdCIsecrctionIronflcituifary.
Treatment
1lc cliIdvirlIaniIiaI slorrstaturc las Icv tlcra-
cuticotions.Iornosf cliIdrcnvitlconsfifutional
dcIay, rcassurancc rlat tlc cliId's slorr staturc is a
nornaI variant suIIccs. In sonc scIcct aricnts vith
nosignsoIpuIcrryIy agc I 4ycars, a4- to6-nonth
frcatncnt vitl flc aroriatc scx lornonc nay
lcI fo nodcstIy incrcasc sLaLurc and puIcrral
dcvcIoncnt Ior syclological suort unfil fruc
uIcrtaIdcvcIoncnf Icgins.
CliIdrcn vifl CI dcIcicncy arc nanagcd vith
Iiosynflcfic lunan CI Iy suIcufancous in] cction
cvcrydayorIy dcotIornoIgrovrllornoncrlat
is givcn 2 fincs cr nontl. Accclcrafcd grovrl
vcIocityon CI trcatncntrcsults in catcl-ugrovrl
in nost cliIdrcn. n MRI oI flc Irain slouId lc
ordcredriortoinitiafing CItlcray. CItlcray
is nccdcd info aduIflood Iccause oI its cIlccfs or
Ionc nass and Iiid nctaIoIisn. II uIcrfy is
dcIaycd Icyond agc I4 ycars, rlc addifion o| scx
stcroids nay Ic considcrcd, Iorl to augncnf fle
grovtl rcsonscfo CI and to stinulatc sccondary
scxuaIdcvcIoncnf.
Frinarylyotlyroidisn istrcatcd virl Icvotly-
roxirc [Syntlroid) . Itcr scvcraI vccIs oIflcray,
rlcgrovtlvclocify gcncraIIyrcturns fonornaI, and
ovcr tinc tlcrc nay Ic sonc cafclu grovtl.
InIiIc CI tlcray, Icvotlyroxirc tlcray docs noL
ronotc catcl-u grovtl.
1o nanagc rlc slorr sfafurc associatcd vitl
Cusling's discasc, tlc lysician nusf idcntiIy and
trcaf flc cfioIogy CirIs vitl slorr staturc causcd
Iy Turner's syndronc nay rcccivc CI to incrcasc
flcirInaI aduIflciglt. SlortstaLurc causcdIysy-
closociaI dcrivation is trcatcd Iy rcnoving rlc
cliIdIrontlecnvironncnt. Slortstafurc causcdIy
ncdicationsisrcvcrscdIydiscontinuingrlc ohcnd-
ing ncdication.

LN L W
N
1 . LighIy percenIofcasesofshortsIaIure resu|t
fromnorma|growthanddeve|opmenIandare
dueIo e|therfam||ia|(geneIic) shorIsIaIureor
consI|tut|onalde|ay.
Z. Patholog|ccausesmayresu|Iin e|therdispropor-
I|onateorproporI|onaIeshortstaIure;propor-
tionate shortstature ismorepreva|enIIhan
d|sproport|onateshortsfafure.
d. Themostcommon paIho|og|ceI|olog|esofpro-
porI|onaIe shortstature |nc|udeCH dehc|ency,
pr|maryhypothyro|dism,Cush|ng'sd|sease,
chron|c systemicdiseases,psychosoc|aldepr|va-
t|on,Turnerssyndrome,and medicaIions.
THYRO| D DYSFUNCT| ON
Hyperthyrodism
MostcascsoIlycrrlyroidisninclildrcnarecauscd
Iy Craves' disease. Ctlcr causcs i ncIudc a lycr-
Iunctioning "lot" tlyroid nodulc or acutc sup-
urafivcflyroiditis. Cravcs' discasc, anauLoinnunc
disordcr, is causcdIy circuIating rlyroid-srinulafing
innunogIoIulins Iinding fo tlyrotroin rccctors
ontlyroid ccIIs, vlicl rcsuIrs indiIusc lycrIasia
< nct fcz sa1e | > < ue pLn njcpa | >
< cau n pexaax~cuaejcun: HYC s jcu. j >
and incrcased IevcIs oI Irec 1,. lconafaI Cravcs'
discase IoIIovs franspIacenfaI passagc ol nafcrnal
flyroid-srinuIaring innunogIoIuIins.
LDC0 N0Dl5l0lDD5
Synpfons incIudc a voracious appcrife [viilouf
vcigli gain or virl vciglf Ioss), lcaf infoIcrancc,
cnofionaI IaIilify, rcsilcssncss, cxccssive svcafing,
IrcqucnfIooscsfooIs, and poor sIccp. ExoplflaInos
is unconnon in cliIdren. CIdcr cliIdrcn nay
conpIain oI paIpifarions. 1lcrc is oIen a clangc
in Iclavior and sclool pcrIornancc. Cn plysical
cxaninafion, flc cliId nay IcIusled, ldgcry, and
varn, vifh propfosis, a lypcracrive prccordiun,
rcsfing faclycardia, and a vidcncd puIse prcssurc.
1lcflyroidglandisgcneralIycnIargcd,snoofl,Irn
[luf noi lard), and nonfendcr. CItcn a Inc frcnor
is nofcd, and proxinaI nuscIc vcalncss is prcscnf.
cufc-onsct faclycardia, lypcrrlcrnia, diaplorcsis,
Icvcr, nausea, and voniring indicaic rlyroid srorn
[naIignanf lypcrrlyroidisn), vlicl can Ic liIc-
flrcaicningIufis rarc incliIdrcn.
InIanisviflnconafalCravcs'discascfcndrosfarc,
arc ]iffcry and lypcracfive, and lavc an incrcascd
appcfifcandpoorvciglfgain.1aclycardiaisusuaIly
prcscnf, andrlyroncgaIy nayIepaIpaIlc.
Inlypcrrlyroidisn,1, IevcIsarcclcvafcd,1,RIis
cIcvafcd, and1SIis supprcsscd.
l0lHDl
Medical flerapy Ior congcnifaI lyperflyroidisn
is rlc adninisfrafion oI propyIfliouraciI [l1\) .
lconafaI Cravcs' discasc gcncraIIy rcsoIvcs ovcr flc
lrsf scvcraI nonfls oI liIc. In flc inIanf lcnody-
nanicalIy conpronised |y lypcrflyroidisn, par-
cnfcral !luids, digoxin, and propranoIol nay Ic
ncccssary.
F1I, ncilinazoIc,orradioiodincnayIc us:dfo
ircaf Cravcs' discasc and nusf Ic fifrafed carcluIIy
lccausefooligladosc canrcsuIfinlyporlyroidisn.
IiIyperccnfoIcliIdrcnviflCravcs' discasclavca
sponfancous rcnission andnayIciaIcnoIlanfifly-
roid ncdicarion aItcr I 2 fo Z4nonfls olfrcafncnr.
1lose clildrcn vlo do noi lave rcnission ol rlcir
discasc viII conrinue on flc anfiflyroid drug and
Synflroid is addcdfoprevenflypoflyroidisn.
Hypothyrodsm
CongcniiaI lyporlyroidisn is discusscd in Clapfcr
1J. 1lc nosfconnon causcoIjuvcniIeoracquircd
lypoflyroidisn is lashimoto's thyroiditis vlicl is
L|Qie| OdoC|| OoloQy b
a clronic lynplocyric flyroidifis flai rcsuIrs in
auioinnunc dcsfrucfion oI rlc rlyroid gIand.
CrlcrcauscsoIlypoflyroidisn includepanlypopi-
fuifarisn, ccfopic flyroiddysgcncsis, adninisfrarion
oI aniiilyroid ncdicaions, and sutgical or radioac-
rivc iodinc aIIarion Ior rrcafncnf oI lypcrrly-
roidisn.1lc incidcncc oIlypoilyroidisn in girIs is
Iour fincs greafcr flan in Ioys. 1lcrc is oItcn a
IaniIylisforyoICravcs' diseascorIaslinofo' sily-
roidiiis. Mosf cliIdrcn prcseni af adoIcscencc, ii is
unusuaIfo devcIop flyroidifisIcIorc 5 years oIage.
LDC0 N0Dl5l0lDD5
SynpronsgcncraIIyappcarahcrileIrsfycaroIIilc
and incIudc coId infoIcrance, dininislcd appcfifc,
Icflargy, and consfiparion. Flysical Indings incIudc
sIov lincar grovfl, dcIaycd pulcrfy, innarurc
lody proporrions, coarsc puBy lacics, dry ilin lair,
dry skn, and dccp iendon rcIcxcs viil a dcIaycd
rcIaxafion rine.
1lyroid luncfion fcsis revcaI a dcprcssedfofal1,
scrun conccnfrafion and a dcprcsscd 1,RI IcvcI.
II prinary lyporlyroidisn is prcscnf, an cIcvaicd
scrun JSI conccnfrarion is nofcd. II sccondary
lypoilyroidisn is prcscnf, ilc 1SI IcveI nay lc
dcprcsscd, nornaI, or eIcvafcd. 1lc deiccfion ol
rlyroid auroanrilodics indicafcs an auroinnunc
|asis Ior diseasc, vlcrcas palpafion ol a rlyroid
nodulc slould pronpf cvaluafion vifl a rlyroid
scan.
l0lHDl
1lyroid rcpIaccncni vifl synilcric Icvoflyroxinc
[Synilroid) is providcd and ad]usfcd fo nainfain
nornaI scrun Irce 1, Icvels, nornaI 1SI IcvcIs,
grovil, and dcvcIopncnf. 1lyroid Iuncfion rcsfs
slouId lc noniiorcdIrcquenfIy.
B
LN L W
M
1 . Mostcasesofhyperthyro|disminch|ldren
arecausedbyCravesd|sease,wh|ch|san
autoimmune-inducedthyroid hyperplasia.
Z. NeonatalCravesdiseaseresu|tsfrom
transplacenta|passageofmaterna|thyro|d-
st|mulatingi mmunoglobu|ins.
. l nprimaryhyperthyro|dism,1leve|sareel evated,
TRU |se|evaIed,andTSH |ssuppressed.
Continmd
< nct fcz sa1e | > < ue pLn nycpa | >
b4 |iuOprlnlO Ped|atr|cs
AUtCna| cCnCx LVBIy 1CSIiS
LhO|C5!CtO|
\, L
1 !LHSC 11,\L
/` ||CgnCnC|CnC
cjc
||CgC5!CmnC
Z1 LHSC
1 1 -|CCxyCC|I|CCSIC|CnC
J 7O-|yC|Cxyj|COCOC|COC
jc
J ! LHSC 1 !,\-
1 7O|yCCxyj|C_CSIC|COC 1CSICSIC|COC
1 1 L|HSC
CC|I|CCSlCCOC
1L|HSC
1 6-|yCCxyCC|I|CCSlCCOC
|
A|UO5lCtOnC
|lnCta|OcO|C!|cClU5
Z1L|HSC
1 J -|CCxyCC|I|SC|

1 1 LHSC
LCn|5C|
L|ucOcOn|cC|U5
||gureb-1 A sc|CmaI|c C sIC|C|CCQCnCs|s | n I|C aC|Cn| cC|ICx.
4. Medica|therapyforCraves'd|seaseconsistsof
propylthiouraci|adm|n|sIraI|on.
b. 1hemostcommon causeofjuven| l eoracquired
hypothyroidismisHash|moIo'sIhyro|diI|s,which
isachron|clymphocyticthyroid|tisthatresulIsin
auto| mmunedesIructionofIhethyroidgland.
. 1hyro|dfuncIiontesIsi nhypoIhyroid|smreveal
a decreased1serumconcentration,decreased
TRU,andel evated serumTSHconcenIraI|on.
7. Hypothyroidism|sIreatedwiIhsyntheI|c
|evothyroxine.
ADRENAL DYSFUNCT| ON
Congenita! Adrena! Hyperp!asia
1lc clinical claractcristics ol congcnitaI adrcnaI
lycrIasiadccndonvliclcnzyneinrlcarlvay
oI stcroidogcncsis is dcIcicnt. Scc Iigurc 6-I Ioi a
sclcnatic oIstcroidogcncsis in tle adrenal corrcx.
Z -lydroyIase dehciency accounisIor90%oItlc
cascsoIcongenitaIadrcnallycrIasia.Tlcdiscascis
LSI|OuC LS!|BO|Cl
AnUtOgCn5
inlcritcdas an autosonaIrcccssivetraitandtendsto
occur as eitler cIassic saIt-vasring ZI -lydroxyIasc
dcIcicncy or as viiiIizing ZI -lydroxyIasc dehcicncy.
Z l -Iydioxylaseisnccdedtoioducealdosteioncand
corrisol.ZI -IydioxyIasedcIcicncyrcsultsinaIuiId-
u oI rle iccuisois oI aIdosterone and corrisoI.
ScciIcaIIy, I 7 -lydroxyrogesterone incieascs,
vlicl is ilcn nctaIoIizcd to dclydroeiandro-
stcronc and andiostcncdione. Botl Iorns oI
ZI -lydroxyIascdcIcicncyiesuItindccicasedcoirisol
and aIdostcionc secierion, incicased corricotroin
[CTI),and incrcascd I 7-lydioxyrogestcioncand
I 7 -lydioxyrcgncnoIone.
l -lydroyIase dehcien accountsIor 5% oIile
cascs oI congenitaI adrcnaI lycrIasia and is aIso
inlcrited as an autosonaI ieccssive tiait. SiniIar to
ZI -lydroxyIascdchcicncy, I I -lydroxyIasedcIcicncy
inairs tlc roduction oIaIdosrcione and coirisoI.
I | -IydroxyIaseconvcirs I I -deoxycorrisoItocorrisoI
and deoxycoiricostcronc to corricostcione in rlc
aIdosteronc atlvay.\irl rcduction oi alsencc oI
I |-lydroxyIasc, corrisoI and aIdosteronc rccursors
IuiIdu and arc sluntcdto androgen synrlcsis.
< nct fcz sa1e | > < ue pLn njcpa | >
< cx n pexaax~cuaejcun: HC s jcu. j >
LDC0 N0Dl5l0lDD5
In congcniLal ZI -lydroxyIasc dcIcicncy, IcnaIc
inIants aic Ioin vitl anIiguous gcnitaIia. CIi-
toroncgaIy and IaIioscrotaI Iusion nay rcsult in
crroncous naIc scx assignncnt. Tlcrc is noinaI
ovarian dcvcIoncnt, and |ntcrnal gcnital structurcs
arc lcnaIc. Mal c inIants Iorn vitl Llc dcIcct lavc
no gcnitaI aInornalirics. Syntons oI cncsis, saIf
vasting, dclydration, and slocI dcvcIo |n tlc Irsf
Zfo4 vccIsoIliIc.!yonatrcniaandlycrIaIcnia
rcsult hon IacI oI aIdostcronc, and lyogIyccnia
icsuIts |ron dccicascdIcvcIs oI coirisol. \orscning
lyonaticnic dclydration culninatcs | n slocI
and acidosis in scvcic cascs. Jlc diagnosis oI ZI -
lydroxyIasc dcIcicncy is nadc Iy docuncnting
cIcvatcdscrunlcvcIs oI I 7 -lydroxyrogcstcronc.
In I I -lydioxyIascdcIcicncy, rlcrcisovcrroduc-
rion oI dcoxycoiricosLcronc, vlicl las nincraIo-
coiricoid acr|vity and rcsuIts in lycrnatrcnia,
lyoIalcnia, and lycrtcnsion. Liagnosis is Iascd
ontlcncasurcncntoIincrcascdIcvcIsoII -dcoxy-
corrisoI and dcoxycorticostcronc in tlc scrun or
tlcii tctralydronctaIoIitcs in flc uiinc. Scrun
androstcncdionc and fcsfostcionc arc aIso cIcvatcd,
andrcnin and aIdostcroncIcvcls aic dcrcsscd.
l0lHDl
Tlcray Ioi ZI -lydroxyIasc dcIcicncy includcs
corrisol and nincraIocorricoid tlcray. Coirisol
tlcrayicduccs^CTIsccictionandovcrroduction
oIandiogcns,andnincralocorricoidadnini sfrarionis
ad]ustcd fo nornaIizc sciun rcnin Icvcls. SurgicaI
corrccrionoIIcnaIc gcnitaI a|nornalirics | saccon-
Iislcdcarly.Tlc lincar grovrl andscxualdcvcIo-
ncnL oI cliIdrcn vitl ZI -lydroxyIasc dcIcicncy
nust Ic nonitorcd cIoscIy. Indcrtrcatncnt, as
indicatcd Iy cIcvatcd I 7 -lydroxyrogcstcronc,
androsLcncdionc, and renin IcvcIs andIyacccIcraLcd
advanccncnf oIsIcIctaInaturity, Icads to cxccssivc
grovrl, rcnatuic scxual lair giovLl, and viriIiza-
tion oI tlc cliId. IIrinatcIy, undcrricatncnt nay
Icad ro renaturc cilyscaI Iusion and adult
slort statuic. Cvcrtrcatncnt vitl cortisoI su-
rcsscs giovrl and nay causc syntons oI
lycicoitisoIisn.
Precocous Puberty
True precocious puberty is dcIncd as sccondaiyscx
claractciistics rcscnring in girIs IcIorc tlc agc oI
7. 5ycars and inIoys lcIorcrlc agc oI9 ycais and
L|QIC| LOOOC|lOO|OQy bb
M
LN L W
M
\ . 2\ -Hydroxy|asedehciencyaccountsfor90%of
the casesofcongen|ta|adrena| hyperplasia.
2. |ncongen|Ia|2\hydroxy|asedehc|ency,fema|e
|nfantsarebornwiIhamb|guousgenita||a,
whereasma|e|nfantsbornwithIhedefecthave
nogen|tal abnorma|it|es.
d. l nsalt-wast|ng 2\ -hydroxylasedehciency,symp-
Iomsofemes|s,saltwasting,dehydrat|on,and
shockdevelopi nIhehrsI2Io4 weeksof| |fe.
4. Thed|agnos|sofcongen|ta|adrena|hyperp|asia
|smadeby documenI|ng e|evated levelsof
\ 7 hydroxyprogesterone|ntheserum.
b. 1herapyfor2\ -hydroxy|asedehc|ency|nc|udes
cort|so|andm| nera|ocorI|co|dtherapy.
naylccitlcrgonadofroindccndcntorgonadotro-
in indccndcnt. Truc ccntral [gonadotroin-
dccndcnr) iccocious uIcrty isnorc connon i n
girIstlaninIoys.FrccociousuIcrLyingiiIsisusualIy
|dioatlic,vlcrcasinloystlcrcisagrcatcr|ncidcncc
oI ClS atloIogy. Junors causing gonadoLroin-
dccndcnt rccocious uIcrry [CLIF) incIudc
gIionas, incalonas, and lanaironas. Ctlcr causcs
oI CLFF incIudc lydrocclalus, lcad in]ury, and
ccntral ncrvous sysLcn inlcction or congcnitaI
naIlornaLion.
Conadotroin-indccndcnr rccocious ulcrty
[CIFF) is cxtrcncIy rarc and is sccn in McCunc-
IIrigltsyndronc [oIyosforic Ilrous dysIasia oI
lonc), IaniIial rccocious ulcrty in Ioys [IaniIiaI
tcstitoxicosis) , |cydig cclI funors, and ccLoic ICC
roducrionIyncoIasns sucl aslcaLic and incal
tunors.
Irecocious theIarche rclcrsto|soIafcdcarIyIrcasr
dcvcloncnt. Tlc usuaI agc ol onsct is I Z to Z4
nontls. Frcnaturc tlclarclc is IiIcIy duc to snalI
transicnt Iursts oI cstrogcn Iron flc rcuIcrLaI
ovary or Iron incrcascd scnsitivity to lov Icvcls ol
cstrogcn in rlc rculcifaI Icnalc. lremature
adrenarche rclcrs to tlc carIy acarance oIscxuaI
lair IcIorc rlc agc ol8 in girIs andrle agc oI9 in
|oys. Tlis Icnign condition is duc to carly natura-
rion oIadrcnal androgcn sccrcrion.
LDC0 N0Dl5l0lDD5
In rccocious rlcIarclc, gonadotroin and scrun
cstrogcn lcvcIs arc in Llc rculcrtaI rangc, and
Iincar grovtl acccIcration and advancing sIcIctaI
< nct fcz sa1e | > < ue pLn njcpa | >
C|ucprlnlO Pediatrics
< cau n pexaex~ouaejcun: HYC s jou. j >
Ub "
naturation arc noi prcscnt. 1lis nonprogrcssivc,
lcnign condirion is disringuislcd lron truc prcco-
cious pulcrry ly tlc nornal grovtl ratc and lonc
agc notedvitlprcnaturctleIarcle.
In prenaLure adrcnarclc, rle IevcIs oI adrcnaI
androgensarcnornalIorpulcrralstagclutcIcvatcd
Ior clronoIogic age. 1lc cliId's lonc agc is usuaIIy
sIigltlyadvanccd. CliIdrcnvitl prenaturc adrcnar-
clc nust le evaIuatcd |or otlcr causes oIincrcascd
androgen producrion, sucl as congcnital adrcnaI
lypcrpIasia, poIycystic ovariansyndronc,or adrcnaI
iunor.IncliIdrcnviilcvidcnceoIsignihcanLandro-
gen elIeci [advanccd lonc age, gtovtl acccIcration,
and acne), ncasurcncnt oI adrenaI stcroids and
androgens lcIorc and aItcr C1I adninistration is
uscd to idcnti| tlosc vitl congenitaI adrcnaI
lypcrplasia.
1le cIinicaInaniIcstations olCLFF incIudcprc-
naiure dcvcIoncnt o| sccondary scxuaI clarac-
tcristics and an acconpanying grovrl sputr. IItlc
CLFF is secondary Lo patloIogy oI ile ccnrraI
ncrvoussysicn, rlcn|ocaIncuroIogic signs arcoItcn
present. I|agnos|s |s lased on advanced lone age
and pulertaI IcvcIs oI gonadotropins and csirogcn
or testostcronc. pulcrtaI pattern oI cIevaicd
gonadotropins aItcr inIusion oI gonadotropin-
rcIeasinglornonc [CnRI)isindicarivcoICLFF.In
CIFF, gonadotropins are Iov and CnRI las no
cIlccton gonadoiropin lcvcIs.
l0lHDl
Frcnaturc tleIarclc is aIcnign conditionrlat docs
noi requite any ireatncnt. Ftenature adrenarcle
ilaiisnotcauscdlycongcniraI adrenaI lypcrpIasia,
Lunor, or poIycysric ovarian syndronc is also a
lcnign condirion.
CLFF is trcaicd vitl in] cctions oI Iong-acring
prcpararions oI CnRI. CnRI anaIogues supprcss
gonadotropinrcIeascandtlcrclydecreascsccondary
scxclaractcrisrics,slovsIcIcialgrovrl, andprcvcnt
rlc Iusion oI long lonc epiplyseaI pIatcs. CIFF is
nanagedly trcaringilcundcrIyingdiscascproccss.
Puberta! De!ay
IuberaI deIayis claractcrizcdlyadeIayin tleonsct
oI pulerry or in tlc ratc ol progrcssion rlrougl
nornal scxual dcvcIopncnt. InIenaIes,tlisrcIcrsto
tle alscncc oI sccondary scx claractcrisrics at rlc
agc oI J or tlc aIscnce o|ncnarclc 5 ycars Iron
tlc onset oIscxuaIdcvcIopneni. InnaIcs,pulcrtaI
dcIay denotcs rlc alscncc olsccondary scx clarac-
tcristics ai tlc agc oI I 4or tlc Iailure to conpIctc
gcnitaI grovrl 5 ycars Iron tlc onset oI pulcrry.
ConstirutionaI dcIay is rlc cause Ior 90% io 95%oI
cases. In tlcsc cliIdrcn tlc lone age is nornal,
grovLl is sIov, andpulcriyviII sinpIy appcar Iatc.
1lcrc isusualIy a posirivc IaniIy listory.
LlDl0 L0gDD55
Systenic discasc can dcIay pulerty in lotl scxcs.
FulcrtaIdcIaynaylcductoprinarygonadaIIaiIure
or lypergonadotropic lypogonadisn. ExanpIcs oI
ilis incIudc 1urner's syndrone or autoinnunc
ovarian laiIurc [ingirIs) and KIineleIier's syndronc
[inloys) .Iypogonadotropiclypogonadisnisdueto
lypotlalanic/pituitary axis dysIuncrion. ExanpIcs
incIudc KaIlnann's syndrone, isoIated gonadotropic
dcIcicncy, lypotlalanic and pituitary tunors,
lypopiiuitarisn and anorcxia ncrvosa. Ctler
cndocrine disordcrs incIuding lypotlyroidisn nay
aIso dcIay pulcrLy.
LDC0 N0Dl5l0lDD5
Tle l|stoty and plysicaI exan slouId incIude an
cxaninarion oIgrovrl trcnds,tle tining oIpulcrty
in orler IaniIy ncnlcrs, and an assessncnt oItlc
paticnt's currcntTanncr siaging. |aloratory cvalua-
iion is lcIpIul, i ncIuding a lonc age, tcsLosLcronc
and cstradiol IcvcIs, gonadotropins, ISI and |I,
proIactin, andtlyroid Iunction tcsting. Scrccning to
IooI Iorsystcnic discasc is aIso indicaicd.
l0lHDl
|n tle case oIconstiut|onal delay, a slort coursc ol
scx steroids nay lc necdcd io iniLiaLc pulcrLaI
dcvcIopnent.FsyclosociaIsupporiisaIsoinportant.
IIpcrnanentlypogonadisnis dcternincdto lc tlc
crioIogy, scx stcroid rcpIaccncnt is iniiiatcd at rlc
nornaItincoIpulcrryandcontinuedIoraIiIctine.

9
LN Ll W
\ . Trueprecoc|ous puberIyisdehnedassecondary
sexcharacIer|st|cs presenting in gir|sbeforethe
ageof7.5yearsand inboysbeforeIheageof9
years and maybeeithergonadotrop|ndependenI
orgonadotropin|ndependenI.
2. Truecentra| (gonadoIropin-dependenI)
precoc|ouspuberIy is more common in girls
thani nboys.Precoc|ouspuberIyi ngi rlsisusua||y
|diopathic,whereasprecociouspuberIyi nboys
< nct fcz sa1e | > < ue pLn njcpa | >
< cau n pexaex~ouaejcun: HYCI s >
Cnapter ocrnolo * b1
isoften duetoIumorsofthecenIral nervous
sysIem.
d. Theclinica|manifesIaI|onsofgonadoIropin-
dependentprecociouspuberty(CDPP) inc|ude
premaIuredeve|opmentofsecondarysexual
character|sI|csandanaccompany|nggrowth
spurI.
4. CDPP|streatedw|th|nject|onsoflong-act|ng
preparat|onsofgonadotrop|nreleasinghormone.
5. ThemosIcommoncauseofpuberta|delay|s
const|tuI|onaldel ay.
Cushng's Syndrome
Cushing's syndrome is a corstcllatior oIsynptons
and signs rlat rcsult Iron ligl cottisol lcvcls. Ii is
ducto citlcrendogenous overproduction o cortisoI
or ecessive eogenous treatment vitl platnaco-
logic doscs oI cortisol. Endogcnous causcs includc
Cusling's discasc and adrcnal tunors. Cusling's
discasc, also Inovn as Iilatcral adrcnal lypcrplasia,
istlcnostconnonctiologyoICusling'ssyndronc
in clildrcn oldcrtlan 7 ycars.Innosiirstanccs, itis
causcd Iy a nicroadcnona oI tlc pituitary gland
tcsuliing in C1I ovcrsccrction. Rarcly, in tlc
youngclild or inIant, analignantcarcinona oItlc
adrcnalglandis sccn.Mostadrcnaltunorstlaicausc
Cusling's syndronc arc adcnonas. Ectopic C1I
sccrctionnayoccurvirlsonctunors,lovcvcr,tlis
IS cxcccdingly rarc inclildrcn.
LDC0 N0Dl5l0lDD5
1lc classic signs and synprons ol Cusling's
syrdtonc includc slov grovtl vitl puIcrtal
arrcst, "noon" Iacics, IulIalo lunp, iruncal oIcsity,
aIdoniral siriac, acnc, lypcrpigncntarion, lypcr-
tcnsion,Iatiguc,nusclcvcaIncss,andcnotionaland
ncrial clangcs. Most adrcnal runors atevirilizing.
Iriiial laIoratoty siudics includc docuncntation
oIan clcvarcd scrun cortisol lcvcl ard an incrcascd
Z4-loururinclrcccoriisoltcst.IIlypcrcorrisolisnis
dcnonstratcd,tlcdcxanctlasoncsupprcssiontcstis
pcrIotncd to docuncnt tlc prcscnce oI Cusling's
syndtonc. Lcxanctlasonc is givcn in tlc latc
cvcnirg, and a cortisol lcvcl is ncasurcd rlc ncxt
noning. Iailurc oI ilc dcxancilasorc to suprcss
ilc norning coriisol levcl is consistcnt virl
Cusling's syndronc. prolongcd dcxancilasonc
supprcssion tcst is uscd to diIlctcntiatc Cusling's
discasc Iron an adrcnal tunor. Vlcn cvaluating a
clildvirl Cusling'ssyndronc,oItainingMRI scans
oItlc pituiiatyand CT scans oIilc adrcnal glands
islclpIul to dctcrninciIadditionalparlologycxists.
l0lHDl
drcnal iunors rcquire sutgical rcnoval. Sinilarly,
Iilatcral adrcnal lypcrplasia is trcatcd vitl surgical
cxcision oItlc pituitaty adcnona.Trans-splcnoidal
nictosurgcty is tle nosi cIlcctivc ncilod oI
nicroadcnona rcnoval. Fcriopctativc strcss dosing
ol glucocotticoids is nccdcd to avoid adrcnal
insuIlcicncy. Fostopcrativcly, rlc paticnt nay
dcvclop nincralocorticoid dcIcicncy ir addition io
tlc glucocoriicoiddchciency.

LN Ll W
W
\ . Cush| ng'ssyndromeisaconstellationof
sympIoms and s|gnsIhatresu|tfrom h|gh
corI|so| |evelsand|sduetoe|Iherendogenous
overproductionofcorIiso|orexcess|veexogenous
treaImentw|thpharmacolog|cdosesofcortisol.
Cushing'sdisease |sthemosIcommon noniatro-
geniccauseofCushi ng'ssyndrome.
2. 1heclassics|gnsandsymptomsofCush| ng's
syndromeinc|ude"moon"fac|es,buffa|ohump,
trunca|obes|ty,abdomina|sIriae,acne,s|ow
growIh,hypertens|on,and musc|eweakness.
Addson's Disease
ddison's discasc, or ptinary adrcnal insuIlcicncy,
nay Ic congcnital or acquircd and rcsults in
dccrcascd cortisol sccrction. Lcpcnding on ilc
discascproccss,rlcrcnaylcaconconitantdccrcasc
ir al dostctonc rclcasc. In rlc ncvIorn, prinaty
adrcnalinsuIlcicncynaylcductoadrcnallypopla-
sia,C1I unrcsponsivcncss, adrenallcnotrlagc,or
isclenic inIarciion vitl scpsis [Vatcrlouse-
Iridcriclscn syndronc) . In oldcr clildrcn and ado-
lcsccnis, autoinnunc adrcnal insuIlcicncy is nost
connon. |t nay occur alonc or in association vith
anotlcr autoinnunc cndocrinopatly sucl as tly
roidiiis or I||M. TuIcrculosis, lcnorrlagc, Iungal
inIcction, rcoplastic inIltrarior, and II\ inIccrior
nay also causc dcstruction oI ile adrcnal gland.
drcnolcuIodysiroply is an X-linIcd rcccssivc
disordct ol long-clain Iatty acid nctaIolisn tlai
rcsulis in adrcnal insuucicncy and progrcssivc
ncurologic dyslunction.
In contrast to prinary adrcnal insuIlcicncy, sec-
ondary adrenaI insuhcicncy is due to ACl dch-
Ub *
< nct fcz sa1e | > < ue pLn ncpa | >
|lucr||lO Ped|atr|cs
ciency.TlcnostconnoncauscoICTIdcIciency
is clron|cstcroidtlcray tlatrcsuIts |n surcss|on
oI ituitary C1!. Fituitary tunors and cranio-
laryngiona aIso rcsuIt in dcrcsscd pituitary
CTI sccrction Iron cirlcr dcstruction oI tlc
ituitaryorituirary conrcssion.
LDC0 N0Dl5l0lDD5
SyntonsIronrinaryadrcnalinsuIIcicncyincIudc
vcaIrcss, nausca, voniting, vciglt Ioss, lcadaclc,
cnorionaI labiIify, and saItcraving. FlysicaI Indings
incIude ostural lyofcns|on andincrcascd|gcn-
tationovcr]oinrsandonscartissuc,|is,niIcs,and
tlc|uccaInucosa.TlcosturaIlyotcnsionandsaIt
craving arc duc to IacI oI aIdostcronc, vlcrcas tlc
incrcascd igncntation is duc to incrcascd CTI
sccrction. McIanocyrc-srinuIating lornonc is a |y-
roductoIrlcCTI|iosyntlcticatlvay.drcnaI
crisisisclaractcrizcd|yIcvcr,voniring,dclydrarion,
and slocI. It nay |c rcciitatcd |y intcrcurrcnt
iIlncss,trauna,orsurgcry
EIccfroIytc a|nornaIirics incIudc lyonatrcnia,
lycrIalcnia, lyogIyccnia, and niId ncta|oIic
acidosis Iron dclydrarion. n cIcvatcd |ascIinc
CTI vitl a concurrcnt Iov cortisol IcvcI is con-
sistcntvitlrinaryadrcnaIinsuIIcicncyTlcscrun
cortiso|IcvcI|ydcInitionisIovandisunrcsonsivc
to in]cctior oI CTI [corticotroin srinuIarion
tcst) . IIrlc corticotroin stinuIafion tcst is a|nor-
naI, arolongcdCTIstinulationtcstisncccssary
toruIcoutsccondaryadrcnaI insu|lcicncy.
l0lHDl
drcnaI crisis, aIso Inovn as addisonian crisis, is a
IiIc-tlrcatcning condirion rlat slouId Ic trcatcd
vitlout dcIay. Corrccrion oIcIcctroIyrc a|nornaIi-
tics and dclydration is rcquircd inncdiatcIy virl
57 dcxtrosc in nornal saIinc and strcss dosc
intravcnous gIucocorticoids.
|ong-tcrn nanagcncnt consists oInaintcnancc
doscs oIoraIgIucocorricoids andnincraIocorticoids.
Tlc gIucocorricoiddosc is incrcascd duringtincs oI
acutcncta|oIic strcss to avoid adrcnaI insuhcicncy.
&
LN Ll W
z
\ . Primaryadrenalinsumciencymaybecongen|Ia|
oracqu|red andresulIs| ndecreasedcort|sol
secret|on,whereassecondaryadrena| |nsum-
c|ency|sdueIoACTHdehc|ency.
Z. Symptomsfrom pr|maryadrenal insumc|ency
|nc|udeweakness,nausea,vom|Iing,we|ghI|oss,
salIcrav|ng,postura| hypoIens|on,and|ncreased
p|gmentat|on.
d. Anadrena|crisis|scharacIerized byfever,vomit-
ing,dehydraI|on,and shock|Imaybeprecipi-
taIed by|nIercurrenI| | |ness,Irauma,orsurgery.
4. L|ecIro|yteabnormal|tiesfoundi nadrena|
cr|s|sinc|ude hyponaIrem|a,hyperka|em|a,
hypoglycem|a,andmeIabo|icac|dosisfrom
dehydrat|on.
AlunanisIornvitl907oIlisor hcrIodyvciglL
as vaicr. Body conposiiion clangcs dranaticalIy
ovcrileIrsLycaro|IilcasnuscIcnassincrcascs. By
I ycar oIagc, a cliId's total lodyvatcr approaclcs
tlc aduIt lcvcI oI 607 Iody veiglL. LIectoIyic
homeostasis, lluid distribution and pl baIance arc
criticaItothcnaintcnanccoInornalplysiology.Tlc
youngertlcpat|cnt,tlcnorc|ntoIerantlcL slc|s
to chalIcngcs to tlcsc systcns.
M MA|NTENANCE FLU| DS
JlcanountoIluidnccdcdtonainiainnornaIIody
lunciion is dircctIy rcIatcd io caIoric cxpcnditurc,
vhicl in Lurn is rcIatcd to a chiId's vciglt. Tlc
Io|Iiday-Scgcr nctlod |s useIuI Ior caIcuIai|ng
naintenancc luids. I 00nL/Ig/day |or tlc Irsf l
Ig, pIus 50nL/Ig/day Ior ile next l 0Ig, plus
25nL/Ig/day |or cacl additionaI Ig tlcrcaltcr. !or
pracricaI purposcs, ii is oItcn norc uscIuI io
caIcuIaic lourIy ratc using 4 nI/Ig/lr [Irsi I Ig
Iody vciglt) 2nL/Ig/hr [second I 0 Ig Iody
vciglt InL/Ig/lr [cacl additionaI IiIogran) .
/n exanplc ol caIcuIating nai ntcnancc luid
rcquirencnts Ior a I 6-Ig cli|dloIIovs.
Ln/y rnle: [ I nL/Ig/dayX I 0Ig) [50 nL/Ig/day
X 6 Ig) " I J00nL/day
our/y role. I J00nL/day divided ly Z4 lr/day
54nL/lr
5horlcul melhod. [4nL/lr X U Ig) + [ZnL/lr
6Ig) " 52nL/lr
!or cacl I00cc oI naintcnance luids, a clild
nccds Jn|qoIsodiun and Zn|q oIpotassiun, as
vcIl as acarlolydratc sourcc. lngencraI, onc-Iouril
Lo onc

lalI nornaI saIinc vitl 57 dcxirosc [ I %


in inlants) and 20n|q/L KCI neets naintcnancc
glucose andclectroIyrcnecds.
W DEHYDRAT| ON
Lclydraiion in ilc pcdiairic patient is usuaIIy scc-
ondarytovomiting or diarrhea. InIants andtoddIcrs
arc particuIarIy susccpiilIc |ccause ol tlc Iinitcd
aIiIityoIilcinnaLurcIidncytoconscrvcvaLcrand
clcctrolyics and Iccausc oI tlc cliId's dcpcndcncc
on careraIcrsLo ncci lisorlcrneeds.
C!nca! Manfestatons
H5lDly
/ careIu| listory cIariIcs tlc dillerential and pro-
vidcs inIornation concorning tle acuity, sourcc,
and quanLiLy o| luid Iosi. PcccnL vcight Ioss and
decreascd urinc output arc inporiant indicators oI
tlc dcgrcc o| dcIcicncy. Tlc coIor, consistcncy,
Ircquency, and volunc oI stooI and/or cncsis nay
inIuenceinitialdiagnosticandtlerapeuticncasurcs.
Many clronic ncdicaI iIIncsses nay prcscnt
acutc|y vitl dclydration, inc|uding diactcs, ncta-
IoIicdisordcrs, cysiic Ilrosis, andcongcnital adrcnal
lypcrpIasia. FoIyuria in ilc prcsencc oI plysicaI
signs oldclydration nay indicatc dialctcs nclIiius,
dialcicsinsipidus, orrcnaltuIularacidosis.Clildrcn
vlo are ncgIccicd or rcIusc to drinI Iccausc oI
scvcre oroplaryngcal painnay also dcvclop signil-
canLdelydration.
< cxau n pexaex~ouaeycun: HYC s you. y >
1RbLb 1-1
LDC LS\D!OD O L0gl00 O L0DOl!OD
NlU
WC|QDI |OSS b7
V|Id| S|QOS
MCd|I|dIe |OC|EdSCO
HC5Q||IO|y|dIC OO|H|
b|OOU Q|eSSu|C OO||d|
bK|O
LdQ|||d|y |CH|| 25CCO|OS
NuCOuSHC|D|dOCS OO||d|O|y
POICfO|OOIOC||C OOf|d|
yCS
Cd||OQ OO||/dDSe|I
PQQCd|dOCC OO|Hd
NCOId| SIIuS |O|Hd|
LDVd|UCS
O||OCOSHO|d||Iy |LS|/L
O||OeSQCCHCQ|dV|Iy 1.2
b|OOO U|CO|I|OQCO 2
b|OOO QM OO||d|
bIdQCOS|OCK OOIlOS|OCK
Dy5C0 LX0HD0lDD
1lcrcisnosingIcplysicaIcxanorlaIoratoryInding
rlai vill accuratcIy asscss apaLicnt'sdcgrcc oIdcly-
dration[scc1aIIc 7- I ) . ILis inportanito rcncnIcr
rlat a cliId's prinary ncclanisn oI conpcnsaiing
IordccrcascdpIasnavoluncistachycardia, hypoten-
siOO iS a vcryIaic and oninous Inding.
Dagnostc Eva!uaton
Scrun cIcctroIyrc IcvcIs lclp guidc rlc cloicc ol
IluidconposirionandraLcoIrcpIaccncnt.Lclydra-
tion nay Ic isotonic, lypotonic [lyponatrcnic),
or lypcrtonic [lypcrnatrcnic), dcpcnding on rlc
naturc ol tlc Iluid Iost and tlc rcpIaccncnt Iluids
providcdIy rlc carctaIcr.
Isotonic dehydration is tle nost connon Iorn
and suggcsts ilai citlcr conpcnsation las occurrcd
or tlat vatcr Iosscs rouglIy paraIlcI sodiun Iosscs.
lypotonic [hyponatremic| dehydration isdcIncdIy
ascrunsodiunIcsstlan l J0nEq/L.CliIdrcnvlo
Iosc cIcctroIyrcs in tlcirstooI andarc suppIcncntcd
virlIrccvaicr orvcry diIutc]uiccs nay prcscntin
ilis nanncr. lyperonic [lypcrnaLrcnic) dehydra-
tion [la< I 50nEq/L)isunconnoninclildrcn,Iut
inpIicsancxccssivcIossoIIrccvatcrconparcdvitl
cIcctroIytcIoss [c.g., diaIctcsinsipidus).
NOU0ft0 b0V0f0
b17 >17
|OC|CdSCO Q|CdIy|OC|CdSCO
|O|Hd |OC|CdSCO
OO|Hd| [O|IDOSIdS|S) OCC|CdSeU
23 SCCOOUS >3 SCCOOUS
U|y O|y
OCQ|CSSCO UCQ|CSSCO
dDSCOI DSCOI
SUOKC| SUOKCO
|Ie|CO UCQ|CSSCO
|LS|/L HX||d
1.2b dX|d|
e|CVdICO |Q|
|||O| ydC|UOI|C HOOC|IC/Q|OOuOU COOS|S
LOHQCOSdIEU S|OK UOCOHQeOSdICO S|OCK
IsuaIIy, tlc scrun IicarIonatc conccntrarion is
dccrcasedsccondarytonctaIoIic acidosis.Iovcvcr,
protractcdvonitingnayrcsuIiin aIIalosisandaligl
IicarIonatc Icvcl as atcsuItoIacid loss Iron gastric
sccrcrions.Virl signiIcant dclydration, pcrIusion oI
rlcIidncys nay Icinpaircd.1lis viIIIc rcIlcctcd
inclcvationsoItlcscrunIIoodurcanitrogcn[BIl)
andcrcatininc[Cr)IcvcIsasgIoncruIarhItraLionrac
IaIIs A BIl/Cr rario grcatcr rlan 20 is consisrcnL
virl prcrcnal IaiIutc.
Treatment
CraI rehydration therapy [CR1) is tlc prcIcrrcd
trcatncnt Ior nild to nodcratc dclydration. 1lc
VorId IcaIrl Crganization rcconncnds rlat
solurions coniain 90nEq/L sodiun, 20nEq/L
potassiun, and20g/LgIucosc.ConncrciaIprcparu-
tions tlat approxinatc rlcsc conccnirarions [c.g.,
IcdiaIytc) arc avaiIaIIc. Ircc vatcr nay prccipitatc
lyponatrcnia and is contraindicatcd. CR1 is par-
ticuIarIy IaIor intcnsivc, rcquiringsnalI voIuncs ol
Iluid givcn vcry lrcqucntly. dninistcrcd corrcctIy,
iLis vcry cIlccLivc.
Scvcrc dclydraiion Icads to IiIc-ilrcatcning
hypovoIemic shock. CliIdrcn inlypovoIcnic slocI
slouId rcccivc 20nI/Ig intravcnous IoIuscs oI
isoionicIuid[nornaIsaIincorRingcr'sIactatc)unril
nct fcz ea1o| > < no pLn nycpa | >
rlcir condirion staIiIizcs [scc Claptcr I ) . ClinicaI
cstination oI dcgrcc oI dclydration and scrun
cIcctroIytc studics taiIor suIscqucni nanagcncnt.
MostdchcitsarcrcpIaccdovcr24lours,virllaII
givcn in rlc hrst8 lours andtlc rcstovcr tlc ncxt
I 6 lours. Cnc inporrant cxccption is tle clild
vitllypcrnatrcnicdclydrarion,invlontlcdchcir
slouId Ic rcplaccd ovcr 48 to 72 lours to prcvcnt
cxccssivcIuidsliItsandIraincdcna.CngoingIosscs
[usualIy in stooI) arc rcplaccdnilIiIitcr Ior nilIiIitcr
witl intravcnous Iuid conparaIIc in clcctroIytc
contcnt viil tlatIcing Iost.
IorcxanpIc,an I 8-IginIanrwitlanornaIscrun
sodiun ] udgcd to Ic I 0% dclydratcd las Iost an
cstinatcd 2000nL oI Iuid [ I nL " IIg) . IaII
tlcdchcitis rcpIaccdovcrtlchrst8lours,vitltlc
Ialancc givcn ovcr ilc ncxt I 6lours. Maintcnancc
tlcrapy nusraIsoIcincIudcd.1lc cliIdrcccivcd a
20-nL/IgIoIus initiaIIy.
I . 2000nL " 2 " l nL [one-laII rlc LotaI
dchcit), J60nL [20nL/lg) las aIrcady Iccn
rcpIaccd.JlcrcIorc,640nI|sg|vcnovcrtlchrst
8 loursat80nL/lr.1lisslouIdIcaddcdtotlc
56nL/lrtlccliIdrcquircsro ncctnaintcnancc
nccds. Ratc 80nL/lr 56nL/lr" I J6nL/lr.
2. Jlc sccond lall [ I nL) is rcpIaccd ovcr
tlc ncxt I 6 lours [6J nL/lr) aIong vitl rlc
nainicnancc rarc [56nL/lr) . Ratc " 6JnL/lr
56nL/lr" I 9nL/lr.
1lc conposition ol tlc rcpIaccncnt Iuid varics
dcpcnd|ng on tlc in|t|aI Iaboratory vaIucs. RcpIacc-
ncnt [and naintcnancc) Iuid slouIdIc potassium
lree untiItloparioni urinatcs. BicarIonatc oracctarc
tlcrapy nay Ic indicatcd iI tlc pI and scrun
IicarIonatc IcvcIs rcnain dangcrously Iov aItcrtlc
initiaI IoIuscs.
M
LN L W
M
\ . Ch| |drenaremoresuscept| b|etoseveredehydra-
t|onthanadu|ts.
Z. The h|storyand phys|ca|examinationarethe best
determ|nantsofthedegreeofdehydration.
d. Dehydrat|onmaybe|soton|c,hypoton|c,or
hyperton|c.
R. When calcu|at|ngflu|d needs,rememberto
replace previous |osses,keep upw|thongo|ng
output,andprov|demai ntenancetherapy.
Faticnts vitl proIound lypcrgIyccnia or clcc-
troIytcdisturIanccsducroanundcrlyingparloIogic
proccss[c.g. ,diaIcricIctoacidosis)nayrcquircnorc
spcciaIizcd nanagcncnt discusscd cIscvlcrc in tlis
rcvicv.
HYPONATREM| A
Iyponatremia [scrun sodiun IcvcI Icss tlan
I J0nLq/L) nay occur in tlc lacc ol dccrcascd,
nornaI, or incrcasod totaI lody sod|un contcnt. !n
cliIdrcn, tlc nost connon sctting is dehydration.
Crlcr causcs includc syndronc oI inappropriarc
sccrction oI antidiuctic lornonc [SlLI), vaicr
intoxicauon, rcnal or congcstivc lcart IaiIurc, and
adrcnaI insuIlcicncy.
Clncal Manfestations
5lDly 0D0 Dy5C0 LX0HD0lDD
1lc scvcrity oIcIinicaI naniIcstations dcpcnds on
Iotl tlc IeveI ot sodium in tlc cxtraccIIular spacc
and tlc ratc ot change lron nornaI. IalIing IcvcIs
tlat occur ovcr scvcraI days are Icticr toIcratcd
tlan rapid losscs. norcxia and nausca arc carIy,
nonspccihc conpIaints. lcuroIogic hndings incIudc
conIusion, lotlargy, and dccrcased dccp rcndon
rcIcxcs. Seizures and respiratory arrest arc Iilc-
rlrcatcning conpIications.
Diagnostc Evaluation
1lc IaIorarory vorIup oI lyponatrcnia includcs
scrun cIectroIytcs, glucoso, Ilood urea nitrogcn and
crcatininc, scrun osnoIaIity, livcr Iunction rcsts,
prorcin,andlipidIcvcIs.Jlcncasucdscrunsodiun
nccdstoIccorrcctcdintlcscttingoIlypcrgIyccnia.
Iorcvcry l 00ng/dLrise in gIucosc [aIovcnornaI"
I00ng/dL),addI . 6nEqla'rotlcncasurcdvaluc.
Irinc sodiun [I_) and spccilc gravity [ISC) aIso
assist u diagnosis.
Treatment
Lclydration is trcatcd vitl Iuid rcsusciration as
discusscd prcviousIy. Iyponatrcnia duc to orlcr
causcsrcquircsIuidrcstricrion and trcatncntoltlc
undcrlying disordcr.1lc caurious usc oI J7 lypcr-
tonic saIinc is Iinitcd to IiIc-rlrcatcning situarions
[i.c., intractaIIc scizurcs) .
HYPERNATREM| A
Iypcrnatrcnia is unconnon in cliIdren in rlc
aIscrcc oI delydration [discusscd carIicr) . Signs
and synptons |ncludc nusclc vcaIncss, |rritaI|l|ty,
ard Icrlargy. Scizurcs and cona arc tlc na]or
conplicatiors.
HYPERKALEM| A
ornaI scrun potassiun valucs rangc Iron J. 5 to
5. nLq/L, a ncasurcncntoI5. 5 nIq/L or grcatcr
is considcrcd hyperkaIemia. In cliIdrcn, tlc nost
connon cause oI an aInornally l|gl potass|un
IcvcI is arriIacfual, duc to rlc lcnoIysis oI rcd
ccIls during sanpIc coIlcction. JransccIluIar sliIts
in lydrogcn ions incrcasc serun potassiun virlout
clangingtotaIIodyconrcnt,Iorcvcryunitrcducrion
in artcriaI pI, pIasna potass|un incrcases 0. 2 to
0. 4nIq/I Lisordcrs andnedicationsrlat irtcrIcrc
vitl rcnal cxcrction oI tle cIcctrolytc prccipitatc
truc lypcrIaIenia.
Differential Diagnosis
Connon causcs oI lyperIaIcnia incIudc tlc
IoIIoving.
Ac|dosis
Scvcrcdclydration
Iotassiun-sparingdiurcrics
Lxccssivcparcntcral inIusior
PcraI IaiIurc
Cflcr Icss connon Iut inportant conditions to
considcr incIudc tlc IoIloving.
Adrcnal corticoid dchcicncy [i.c., Addison's
discasc)
PcnaIfuIuIaracidosis
Massivccruslin]ury
Bcta-IIocIerortlcoplylIinc intoxicarion
C|inical Manifestations
IarcstlcsiasandvcaIncssaretlccarIicstsynpions,
H accid paraIysis and tcfany occur Iare. Cardiac
involvcncnr corrcIarcs virl spccihc progrcssive
LCC changes, J-vavc cIcvation ["pcaIing") is IoI-
IovcdIylossoIIvavcs, vidcning PS conpIcxcs,
SL|LM |
<2. b|/|
|Cj|CSSCC S1 SCC(I
|| j'aS|C 1 wuvC
||CinCnI L wuvC
NC|a|
>6.Om|q}j
>7. b|(/|
>9. U|/| .
1al | 1 wavC
|Cn || |(IC|va|
\| CC L|S Cu|aI|Cn
!a| | 1 wavC
/CSCnI | wuvC
S|nuSC|Ca| wavC
||gure11 LLL hnCnQs C |yC|klC|.
and S1 scgnent dcprcssion [scc Iigurc 7-I).
\entricuIar hIriIIarion and cardiac arrcst occur at
serun lcvcIsgrearcrrlan 9nIq/I
Treatment
CaIcium gIuconate protccrs tlc lcari Iy staIilizing
tlccardiacccIIncnIranc.InIusionoIsodiunIicar-
IonatcorinsuIin [andgIucose)drivcspotassiuninto
tlc ccIIs. Cation cxclangc resins [c.g., Kaycxalatc)
andlcnodiaIysisarctlconIyncasurcsrlatactuaIIy
rcnovc potassiun Ion tlc Iody
M
LN Ll W
M
1. Progress|veLCCchangesassoc|aIedw|Ih hyper
kalem|a|ncludepeakedTwaves,d|sappear|ngP
waves,and w|den|ngoftheQRScomp|ex.
2. TreaImenIopI|ons|ncludecalc|umgl uconaIe,
sod|umb|carbonaIeor |nsu||nlg| ucose,caI|on
exchangeres| ns,andhemod|a|ys|s.
HYPOKALEM| A
IypoIaIcnia in tlc pcdiatric popuIation i s usuaIIy
encountcrcd in cascs oI aIIaIosis secordary fo
< nct fcz ca1o | > < no rn nom | >
< c~n n ozann-onnocnn: ucm os ons


L|QIC| 1 / | U|O,LCCI|O|yIC, O Q| OCCOI 1
voniting,adninistrationoIIoopdiurctics,ordiaIctic
Ictoacidos|s.SignsandsynptonsincIudc vcaIncss,
tctany, constipation,poIyuria, andpoIydipsia.Musclc
IrcaIdovn Icading to nyogIoIinuria nay conpro-
niserenaIIunction.LCCclangesarcnotcdafIcvcls
Icss tlanorcquaIto 2. 5nIq/L cardiac arrlytlnias
canoccur andarcnorcIiIcIyiItle paticntisIcing
trcated vitl digitaIis. BIood pressurc clangcs and
urine eIcctroIytc contcnt assist in diagnosis [Iigurc
7-2). 1rcatncnt consists oI correcting pI [vlcn
increased) and rcpIcnisling potassiun storcs.
METABOL|C AC| DOS|S
1lcextraccIIuIarluidpI [lydrogenionconccntra-
tion) isIcptinavcrynarrovrange,largclyasarcsuIt
oI tle bicarhonate buher system. Iydrogcn ions
[H') conIincvitlICC, toIorn ICC,,vliclin
turnIreaIs dovn to vatcr and CC [vlicl can Ic
cxpiredtlrouglflcIungs) .JlcadditionoIcxccssivc
I' , tle Ioss oI ICC
-
, or aInornaI puInonary
Iunction can aII alIcct tlis IulIering systcn and
lcad to acid|asc disturIanccs.
MetaboIic acidosis rcsuItsIrontlcIossoI!CC,
or tle addition oII' in tlc cxtraceIIuIar Iuid. It is
tlc nost connon acidIasc disordcr cncountcrcd
in tle pcdiatric popuIation. In tlc prcscncc oI a
nctaIoIic acidosis, tlc IoIloving lornuIa prcdicts
tlc expcctcdIacC. IacC," I . 5 X ICC, 8 [2).
Il tle ncasurcd IacC is ligler tlan cxpcctcd,
tlcn tlcrc is a prinary rcspiratory acidosis. Ilit is
Iovcr tlar cxpcctcd, tlcrc is prinary rcspiratory
aIIaIos|s.
Cl |n|ca! Man|festat|ons
Iypcrpnca is tlc nost consistcnt cIinicaI hnding in
nctaIoIic acidosis otlcr signs and synptons arc
rcIatcd to tlc urdcrIyingdisordcr. |nportantIaIora-
tory studics incIudc scrun cIcctroIytcs, IIood urca
nitrogen, crcatinc, glucosc, vcnous or artcriaIIIood
gas, and urincdipsticI lor pI and glucosc.1lc diI-
Icrcnce Ictvccn tlc suns oI tle neasurcd cations
[a' K') and anions [CI ICC, ), tcrn1cd Ilc
anion gap is nornaIIy I 2 4,1aIIe 7-2 Iists condi-
tionsassociatcdvitl clangcs intlcaniongap.
Treatment
1lc intravcnous adninistration oIsodiunIicarIon-
atc slouIdIcrcscrvcdIorcascs in vlicl tlc scrun
pI is Iess tlan 7. 0 and tlc causc is unInovn
or diIhcuIt to rcvcrsc. Iaficnts recciving alIali
tlcrapy requirclrcqucntpI,sodium,potassiun,and
calciun nonitoring, conplications incIudc aIlaIosis,
lypoIaIenia, lypcrnatrcnia, andlypocaIccnia.
METABOL|C ALKALOS|S
MctaIolic alIaIosis is iucl lcss connon tlan
acidosis in cliIdrcn. "Contraction" aIIaIosis rcsults
|yQOKOl|| S
LlOOO Q|SS|
.
|CVBICO O|OB
||gure 1-Z LV|U\|OO O
|yOkC.
>
'L|| | QOISSS| U0
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CXCSS |R|0
LCRg0|!B| BO|0B| |yQ|QlBS|B
LUS0| 0g S SyRO|C0
CCSS 0|0|OlOCC|l|CC|O
>
JL||G QOIOSS|G
|RS| IUDU|B| BC|OCS|S
|B0CC0| ` S SyRO|C0
B|I|| S Sy0O|C0
P0!|D|C!|CS
L| U|!|CS
PlHBlCS|S
l 0C|BSO | 0SUl | 0
4L||G QO!OSS|U0
CH|0 |CSSS
Ll lCSSS
l| g| CB|DC|yO|SI O|!
L00B||BXBl|V BDUS
P0C|X|B 0|VCSB
14
< nct fcz sa1e | > < ue pLn nycpa | >
||uOprlnl5 |CO|If|CS
M 1RbLb 1-Z
LDDg0S D D0 PDOD Q
DCrCSCU PDOD LQ NOrDlPDOD L@ LCCrCSCU PDOD L@
MyOK|C|
MyOC|CC|
MyO|gOCSC
MyC||OS|IC|
L||||C| UC|yU|I|O|
LCICC|UOS|S
L|DCI|C KCIOCOOS|S
b| |Cy|lC OSOO|OQ
HC||||U|e
NCI|OO| OSOO|OQ
O|C|
MyCf|I|C|CUC|yU|I|OO
HCO| IUDU||CUOSS
MyC|| |COII|O|
MyC|K|eH|
MyCfC|CC|
MyC||QOCSC
MyO|DU|OCH|
L|I||U O|SOO|OQ
IronrlcIossoIHuidliglinI'orCl

,asnayoccur
vitl protractcdgastricvoniting orclronic rliazidc
or Ioop diurctic adninistrarion.Faticnrs vitl cystic
hlrosis nay dcvcIop ncraIoIic aIIaIosis duc to
cxccssivc cIcctroIytc Iosscs in tlc svcat. VoIunc
cxpansion and clIoridc rcpIaccncnt corrccf rlc
aIIaIosis unIcss if is associatcd vitl disordcrs oI
nincraIocorticoid cxccss [c.g., rcnaI artcry stcnosis),
potassiun suppIcncnts arc ncccssary in tlcsc
cascs.
M
LN L W
M
1. Metabo||cacidos|s|sa relaI|velyconnond|sorder
|n ped|aIr|cpaIienIs.
2. Theequat|onPaCC" 1.b X HCO o () can
he|pd|sI|ngu|shbeIweenprimaryand secondary
neIabol|cac|dos|s.
d. NaHCO (sod| umb|carbonate) should beused
on|ywhenac|dos|s|ssevereordimcu|ItocorrecI.
ABDOM|NAL PA|N
AbdominaI pain is onc oItlcnosf connon synp-
tonsrlc pediarriciansccs, and it las a conpIcx dil-
Icrcnt|al d|agnosis.IdoninaI painnay Ic acutc or
clronic/rccurrcnt [at lcast tlrcc cpisodes virlin J
nonrls), anditnay rcprescnt a surgicaI orncdical
condirion.Clronic/rccurrentaIdoninaIpain occurs
in approxinatcIy I 0% oIcliIdrcn 5 io I 5 ycars old,
and Icss rlan I 0% ol tlesc cascs rcsult lron an
organic cause.
Differential Diagnosis
InIcctious conditions [incIuding Iactcrial and viraI
gastrocntcritis) arc tlc nost connon causc oI
aIdoninaI pain. Mcscnteric Iynpladenitis nay
causc pcrsistcnr pain lolIoving an inIcction. Croup
strcprococcaI inIcctions, urinary rract inIccrions,
and lovcr IoIc pncunonias nay aIso prcscnt vitl
aIdon|naIpain.FcIv|c |nIannatoryd|seasc[FIL) |s
an inportant considcration in adoIcscent lcnaIcs.
\irallcpatitis,inIcctiousnononucIcosis,andlcrpcs
zostcrarcnorcunconnoninIcctionstlatnaynccd
to Icconsidercd.
lon|nIcct|ous ncd|caI d|scascs arc Iess connon
and include Iorlprinary gastroinrcstinaI and gcni-
tourinarypatloIogyandsystcnicdiscases.CloIccys-
titis, pancrearitis, gastritis, and pcptic uIccr discasc
arc unconnon in clildren, Iut varrant considcra-
t|on.Idoninalpa|n|saprinaryIcatureinHcnocl-
SclnIcin purpura, Iut also nay Ic seen in orlcr
vasculiridcs, incIuding KavasaI's discase, polyartcri-
tis nodosa, and Iupus crytlcnatosus. Iltlc pain is
rccurrcnt, tlc diIcrcnriaI diagnosis nusr Ic
cxpandcd. Constipation and |unctional aIdoninaI
pain arc Irequcnt conplaints cvaIuatcd Iy a pcdia-
trician. Lacrasc dchcicncy rcsults in rccurrcnt pain
virlcxposuretodairylood.SicIlcccIIdiseasc,ulccr-
arivc coIitis, and Croln's discasc arc clronic condi-
tions in vlicl pair is a na]or synpron. Morc
rarc causcs incIudc aIdoninaI nigraincs, scizurcs,
Hirsclsprung's discasc, and naIignancy, incIuding
lcuIcnia as vclI as soIid runors.
ppcndicitis is tlc nost connon surgicaI causc
ol aIdoninaI ain. Intussusccptioo is ar inporianf
pcdiatric diseasc tlatprcscnts vitl intcrnittcntIut
scvcrcpain andstriIngIctlargy.Incarccratedlcrnia,
voIvuIus, IoveI oIstruction, and tcsticuIar rorsior
rcprcscnt surgical cncrgcncics. 1rauna can Icad to
signihcanr intra-aIdoninaI in]ury andpain.
Lrologic oIstruction at anyIcvcI is an inporrant
considcration. LrctcropcIvic oIstruction, lydro-
ncplrosis, andrcnaIsroncs car causcsigniI cantpain
Cynccologic causcs arc an inporiantpari oIrlc
diIcrcntiaI diagnosis in adoIcsccnt girls. Frcgnancy
slould aIvays Ic considcrcd, cspcciaIIy ilsynprons
arc consistcnt vitl an cctopic prcgnancy. Lysncn-
orrlca, ovarian cysts, nitteIsclncrz, FIL, ccrvicit|s,
endonctriosis, andovarian or adncxaltorsion arc aIl
porcntial proIlcns intlispopuIation.
Isycliatric causcs oI aldoninal pain arc un-
connon in cliIdrcn.1ruc nalingcrirgisunusual, as
arc convcrsion disordcrs. Hovcvcr, nany cliIdrcn
do cxpcricnce aIdoninaI pain in tlc sctting ol
strcss, cspcciaIIy in tlc contcxt oI sclooI, and nild
intcrnirrcnt pain aIso can Ic sccn in cliIdrcn vitl
dcprcssion.
Clinical Manifestations
5lOly
1lc lisrory slould Iocalize thc pain and dctcrninc
lts quaIity and tcnporaI claractcristics and its cxac-
crIatingandaIIcviaringIactors.\itl"inIannatory"
1b

not foz > ; no n ncm | >


< cran n ozann-ronnocnn: ucm os ons. y >
ucrnlO -'u. f
pain, tle cliId tcnds to Iic stiIl, vlcrcas vith
"coIicIy" pain, thc cliId cannot rcnain stiII. Colicky
pain usualIy rcsults lron oIstruction, vlcrcas
inI annatorypain is causcdIy an inIectcdorpcrlo-
ratcd organ or viscus. It is inportant to asccrtain
vlctler thc cliId las any drug or Iood aIIergics or
las lad prcvious aIdoninaI surgerics. ltcr Iapa-
rotony, snaII Iovcl oIstruction Icconcs norc
liIcly. Fain nay Ic acconpanied Iy anorcxia,
nausca, encsis, diarrlca, or constipation. BiIious
cncsis indicatcs oIstruction [or Iess connonIy,
iIcus), vlcrcas Iloody cncsis points to an uppcr
CI sourcc [csoplagitis, gastritis, or duodcnitis} .
BIoody or nucinous diarrlca suggcsts Iactcrial
cntcrocoIitis.
Sfooling claractcristics arc inportant, Iccausc
constipationisaconnonctiologyoIclronicaIdon-
inaI pain. Lysuriaand aIdoninaI pain arc indicativc
oIa urinarytract inIcction, vlcreas sorc flroat and
aIdoniraI pain inpIicafc plaryngifis.1lcrc nay Ic
alistoryoltrauna. CItaining a goodscxuaIlistory
in the adoIcsccnt is criticaI. II tlere is a listory ol
vaginal disclargc and Icvcr, IIL slould Ic con-
sidcred. InquiringaIoufiIl contacts can givclclpluI
clucsto tlc diagnosis,Iccauseviral gastrocntcritis is
quitc contagious andvcryconnon.laniIylistory
oI lactosc intoIcrancc, Croln's diseasc, ulccrativc
colitis,orirritaIIcIovclsyndronc incrcascsthclikc-
IiloodolthcscdiagnoscslccausctleyarcgcncticalIy
Iased. Clangcs in thc cliId's cnvironncnf [lonc,
Iricnds, sclool) or Iclavior [poor sclooI pcrlor-
nance, incrcasingIyarguncntativc) naysuggcstthat
tlc aIdoninal pain is not tlc resuIt oI organic
discase.
Dy5C0 LX0HD0lOD
Tlc goaI oItlc aIdoninal cxanination is to asccr-
tainvlctlcrthc cliIdlasanaIdoninaIproccsstlaf
rcquires surgicaI intcrvcntion. \atcling tlc cliId
vaII,cI|nIontotlccxaninationfaIlc,andintcracf
vitl Iotl parcnts andstal!IcIore lornaIIy cxanin-
ingtle cliId's aIdoncr hclpsone fogain an apprc-
ciation Iortlc dcgrccolincapacitationor cnotional
ovcrlaytlatnayIcprcscnt.1lcaIdoncnslouIdIc
inspccted, auscuItatcd,and palpatedFeritoncaIsigns
incIudercIoundtcndcrncss,guarding,psoas oroItu-
ratorsigns,andrigidityoltlcaIdoninal vaIl. LnIcss
tlc diagnosis is tlouglt to Ic unconplicatcd viraI
gastroentcritis, a rcctal cxanination should Ic
pcrIorned to dctcct tcndcrncss L lard stooI and
to oItair stooIlorguaiac tcsting. lltlc paticnf is an
adoIcscent lcnalc, a pcIvic cxanination slouId Ic
pcrIorned. CcrvicaI notion tcndcrness is consistcnt
vitl FIL.
Diagnostic Evaluation
Jlc diagnosticteststratcgyis dictatedIytlclistory
andhndingsoItlcplysicaIcxanination.IItlccausc
oltlc pain is tlouglt to Ic a surgicaI onc, tlcn a
surgicaI consultation slouId Ie oItaincd. CI tlc
connon causcs oI acutc or clronic/rccurrcnt
aIdoninaIpain,surgicaIcauscsarcthenostliIcIyto
rcquire inncdiatc intcrvcntion.
conpIctcIIoodcountvithnanuaIdihcrcntial,
scrun eIectroIytcs and clcnistrics, anylasc, Iipasc,
stooIguaiaccxanination,urinaIysis,andradiograplic
studics slouId Ic pcrIorncd il therc las Iccn
aIdoninal trauna or an acutc surgical condition is
suspccted. Blood slouId aIso Ic typed Ior possiIIc
transIusion. IariunsvaIIovvitl uppcr gastroin-
tcstinaI exanination, a pH proIc, or an cndoscopic
cxanination nay Ic uscd to cvaIuatc Ior rclux.
\lcnunconpIicatcdviralgastrocnteritisistlcnost
IiIcIy cause, no studics nccd Ic perIorncd, Iut il
IactcriaI entcrocoIitis is Icing considcrcd, stool
slouIdIeoItaincd |or cuIturc. Croupstrcptococ-
caIplaryngitisandIILrcquircappropriatccuIturcs.
In soncscvcrc cascs oIconsiipation, aIdoninaIradi-
ograplsnayIcindicatcd.1odiagnoseaurinarytract
inIcction, a urinaIysis and urinc cuIturc should c
pcrlorned.
Treatment
Jrcatnent is dircctcd at tlc underIying causc ol
tlc pain. SurgicaI proIIcns arc trcated accordingIy.
Croup sfrcptococcaI plaryngitis, urinary tract
inlcctions, and IIL rcquirc appropriatc anfiIiotics.
IndividuaIs vith Iactasc dchcicncy Iencht lron a
Iactosc-Iree dict or cxogcnous lactase rcplaccncnt.
Iaticnts vitl rcIux csoplagitis Icneht lion snalI,
lrcquent ncaIs [rathcr tlan inIrcquent Iargc oncs),
sitting upriglt Ior JU ninutcs altcr a ncaI or sIccp-
ingata45-dcgrccanglcaltcrcating,avoidanccolIafc
cvcningneaIs, aproIincticagcnt,andan H,-IIocIcr
and/or proton punp antagonist. Clildrcn vitl
aIdoninal pain cxaccrIafcd |y strcss rcquirc
paticnce, rcassurancc, and in rarc cases prolcssionaI
psycliatric assistancc. Constipation can Ic trcafcd
vith prunc ]uicc, scnna, Colacc, nincral oil, l
Iacfulose. In sonc cascs, disinpaction, cathartics, or
cncnas nayIcrcquircd.
< not Ioz ca1o | > < no rn nom | >
< cron n ozann-ronnocnn: ucm os ons. y >
LN Ll W
M
\ . DeIern|newheIherIhe pa|n |sacuteorchron|cl
recurrentandwhethera med|cal,surg|cal,or
nonorgan|c d|sorder|snosIl|kely.
2. lfthepaI|enI|s anadol escenIfemale,gen|tour|
narypatho|ogynustbecons|dered,anda pelv|c
examshouldbeperformed.
Appendicitis
Appcndicitis is thc nosr connon ind|carion Ior
aIdoninaIsurgcry in cliIdlood.ppcndicirisrcsuIts
Iron IacrcriaI invasion ol rlc appcndix, vlicl is
nore liIcIy vlcn rlc luncn is oIsrructcd Iy a
Iccalirl,parasirc,orlynplnodc.ppcndicitisoccurs
nostIrequcntIyin cliIdrcnIctveen I 0and l D ycars
oI agc. Lcss tlan I 07 oIpaticnts arc youngcr tlan
D ycars olagc.
LDC0 N0Dl5l0lOD5
ClassicalIy, Icvcr, cncsis, anorcxia, and diIluse pcri-
unIiIical pain dcvclop. SuIsequcnrIy, pain and
aIdoninaI tcndcrncss Iocalizc ro rlc riglt Iovcr
quadranr as tlc parictaI pcrironcun Icconcs
inI ancd.Cuarding,rcIoundtcnderncss,andoItura-
torandpsoas signs arc connonlyIound. 1lcappcn-
dix tends to pcrlorarc aIout Jb lours aItcr pain
Icgins. 1lc incidcncc ol pcrIorarion and dillusc
pcritonirisisliglcrincliIdrcnyoungcrrlan2ycars,
vlen diagnosis nay Ic dcIaycd. ArypicaI prcscnta-
tions arc connon in clildlood, cspcciaIly vith
rctrocecaIappcndiciris,vliclnayprcscnrvitlpcri-
unIiIicaI pain and diarrlca. RctrocccaI appcndicitis
usuaIIy docs not inducc riglr lovcr quadrant pain
unriI alrcrpcrloration. BactcriaI cntcrocoliriscauscd
Iy Lnm/obnclcr and Tcrsnn nay ninic appcn
diciris Iccausc Iotl can rcsuIt in rigltlovcr quad-
rant aIdoninaI pain and rcnderness. Liagnosis oI
appendicirisiscstaIIislcdcIinicaIIyIylistoryandIy
plysicaIcxaninarion,vlicl slouIdincIudca rcctaI
cxaninarionrodctccttcndcrncss or a nass. nod-
crarcly c|cvarcd vlitc IIood ceII counr virl a lclt
sliIis oltcnsccn in appcndiciris. pIain IIn oltlc
aIdoncn nay dcnonstratc a |ecalirl. IdoninaI
ulrrasoundnaydcnonstrarctlcinIancdappcndix,
Iurconpurcdronograplyscanslavcaliglcryicld.
l0lHDl
Laparorony and appcndcctony slouId Ic pcr-
IornedIcIorcpcrIorarion.WlcnappcndicitisrcsuIts
CnapterB Gastrcenterclcgy * 11
inpcrIorarion,rlcpaticnrslouIdIegivcnanpiciIlin,
gcnranicin,andnctronidazolc [IIagyl) ro rrcarpcri-
ronitis |ron inrcsrinaI Iora.Tlc norialiry rarcriscs
signiIcanrIy virl pcrIoration.
M
LN L W
M
1. Appendic|t|sisIhe mostcommon| nd|caI|onfor
abdom|nalsurgery |nch|ldhood.
2. Fever,enesis,anorex|a,and d|fluseper|umb|l|cal
pa| ndeve|op|n|t|ally,thepa|nandabdom|nal
IendernesslocalzeIoIher|ghIlowerquadranI
whenthepar|etal per|Ioneumbecomes|nIlamed.
Cuard|ng,reboundtenderness,and obturatorand
psoass|gnsarecommonlyfound.
|ntussusception
Intussusception rcsuIts lrontcIcscoping oIonc parr
oltle inrcstinc into anorlcr. Intussusccprion causcs
inpaired vcnoustcrur,Iovcl cdena andischcnia,
nccrosis, and pcrIoration. lr is one oI rlc nost
connon causcs olintcstinal oIstrucrion in inIancy.
Most inrussusccptionsarciIcocoIictlc iIcun invagi-
natcs inro tlc colon at tlc iIcocccal vaIvc. A prcvi-
ous viraI inIccrion nay causc lypcrrroply oI tlc
Icycr's patclcs or ncscntcric nodcs, vlicl arc
lypotlesizcdro actasrlc lcadpoinrinintussusccp-
tion.spcciIc Icadpoint isidcntiIedinonIyaIout
57 o|cascs Iur slould Ic souglt in nconarcs or H
chiIdren oIdcr rlan 5 ycars. A Icad point is virrually
ncvcr denonsrrarcd in clildrcn oIder rlan nconatcs
Iur youngcr rlan 2 ycars. RccognizaIIc Icad poinrs
in inrussusccption incIudc MecIcI'sdivcrriculun,an
intcsrinaIpolyp, lynplona, oraloreignIody. lnius-
susccprion las also Iccn associared vitl Hcnocl-
SclonIein purpura [ISI), Iut in rlis sctting is
usualIy iIcaI-iIcal. |t can Ie vcry diIIcult to distin
guisl tlis surgicaI causc Iron rlc nonsurgicaI
inI|annarory aIdoninaI pain sccn in ISI.
LDC0 N0Dl5l0lOD5
VioIcnt cpisodcs oI irritaIiIity, coIicIy pain, and
cncsis arc intcrspcrscd virl relarivcIy nornaI
pcriods. RcctaI IIccding occurs in 807 ol paticnts
lut only rarcIy in tlc |orn ol rlc cIassic currant
]cIIy" srooIs [stools containing Iriglt rcd IIood and
nucus .1lcdcgrccoIIctlargydenonsrratcdIytlc
cliId nayIc srriIing. tuIuIar nass is palpaIIc H
aIout 80% olparicnts. plain aIdoninaI IIn nay
< not Ioz ca1o | > < no rn nom | >
1b * |l ucrln1O |ediatrics
< cran n ozanx-ronnocnn: uvcm os ons. |V >
slov apaucityoIgas intlcriglrIovcr quadrant or
cvidcncc oI oIstrucfion vitl air-Iuid IcvcIs.
Iariun cncna or air cncna dcnonstratcs a coiIcd-
spring appcaranccto rlc IovcI, vlicl is diagnosric.
Stool slouIdIctcstcdIor occuItIIood.
l0lHDl
FIuid rcsuscitation virl nornaI saIinc or Iactatcd
Pingcr's soIution is usuaIIy ncccssary. Hydrostatic
rcduction virl Iariun encna or pncunaric rcduc-
tion vitl air cncna is succcssIuI in 757 oIcascs iI
pcrIorncd in tlc hrst 48 lours, and is succcssIuI
in 507 oI cascs iIpcrIorncd in tlc hrst 48 lours.
IcritoncaI signs arc an aIsoIute contraindication to
rlis procedurc. Laparotony and dirccf (cducrion
is indicatcd vlcn rcduction Iy cncna is cirlcr
unsucccssIuI or contraindicarcd. Tlc inncdiarc
rccurrcncc ratc is aIout I 57. \lcn a spccihc Icad
pointis idcntihcd, rlcrccurrcnccratcisliglcr.
M
LN Ll W

1. MosIinIussuscepI|onsare| leoco|ic,in wh|ch Ihe


|leum| nvag|naIes|ntoIhecolonaIIhe| l eocecal
valve.
2. V|olenIep|sodesofirr|Iab|l |ty,col|ckypa|n,and
emesisare| nIerspersedw|threlativelynorma|
per|ods.RecIa| bleedingmayoccur,buIon|yrare|y
|nIheformofthec|ass|ccurrantjelly"stoo|s.
d. HydrosIat|c reducI|on w|Ih bar|umenemaor
pneumaI|creduct|onw|Ih a|renema|ssuccessfu|
|n75%ofcases.
EMES|S
omiting is onc oIrle nost connon prcscnring
synptons in pcdiatrics and can Ic causcd Iy Iotl
gastrointcsrinaI and nongastrointcstinal parloIogics.
ConpIicarions oI scvcrc, pcrsistcnt cncsis incIudc
dclydration and hypoclIorenic, lypoIaIcnic
nctaIoIic alIaIosis. IorccIuI cnesis can (csuIf in a
MaIlory-\ciss rcar oIrlc csoplagus at tlc gastroc-
soplagcaI ]unction or crosion ol tlc gastric cardia,
clronic encsis canrcsuIrin distaI csoplagiris.
Differential Diagnosis
1aIIc 8- ! IiststlcnostconnoncauscsoIvoniring
inrlc pcdiatric popuIation.
Clinical Manifestations
5lOly
In inIanrs rlc listory slouId diIcrentiate Icrvccn
truc voniring and "spitring up" [gastrocsoplagcaI
rcIux) and vlctlcr tlc cncsis is acute orclronic.
Frcqucncy, appcarancc [IIoody or IiIious), anount,
and tining oI tlc cncsis arc inporrant. Lncsis
slorrIy aItcr Ieeding in rlc inIant is proIaIIy gas-
troesoplagcal rcIux. IIrlc cncsis is pro] cctilc and
fIccliIdis to J nonrlsold,pyIoricsfcnosisnust
Icconsidcrcd.Ioorvcigltgainandcncsisnayindi-
catcpyIoricstcnosisornctaIoIicdisordcr. MacroIidc
anriIiorics arc Inovn to cause encsis and diarrlca,
clcnorlcrapcuric agcnts and sonc toxic ingcstions
connonIy causc cncsis. IIrlc cliIdlas a ventricu-
Iar-pcritoncaI slunt,vonitingnayIc asignoIslunr
oIstruction and incrcascd intracraniaI prcssurc.
|ncsis virl scizurc or lcadaclc or |otl nay indi-
catc an intracraniaI proccss. Liarrlca, cncsis, and
Icvcr arc sccn vitl gastrocnrcritis. Icvcr, aIdoninal
pain,andcncsisarctypicalIorappcndiciris,vlcrcas
Iilious cncsis and aIdoninaI pain arc sccn virl
intcsrinaIoIstruction.|ncsisandsyncopcnayrcsuIf
lron prcgnancy.
Dy5C0 LX0HD0lOD
Cn plysical cxaninarion, tlc initial asscssncnf
slouIdIocus ontlc cliId's vital signs and lydration
status. Signs and synptons oI dclydrarion arc
discusscd in Claptcr 7. IuIging IontancIIc or
papilIcdcna inpIicates incrcascd intracraniaI prcs-
surcasrlccauscoltlccncsis.Lncsisisconnonin
inIcctiousplaryngiris.1lcIunghcIds slouIdIc aus-
cuItatcd Ior cracIlcs or an asynnctric cxaninarion
toruIcoutpncunonia. Lnesis andvaginaldisclargc
in rlc IcnaIe adoIcsccnf varrant a pcIvic cxanina-
rionrocvaIuatcIor IIL.TlcaIdoninaIcxanination
slouId Iocus on IoveI sounds and rlc prcscncc oI
distcnrion, tcndcrncss, or nasses. Hypoactivc IovcI
sounds nay indicatc iIcus or oIstruction, vlcrcas
lypcractivc IovcI sounds suggcst gastrocntcritis.
Idoninal nass vitl cncsis nay indicatc intussus-
ccption or naIignancy. 1cndcrncss on cxan is
suggcstivc oIappcndiciris, pancrcariris, cloIccystitis,
pcritonitis, or IIL.
Diagnostic Evaluation
Spccihc IaIoratory studics dcpcnd on tle suspcctcd
causc. ppropriarc culrurcs and a conpIctc IIood
countvitl nanuaI diIcrcntial slouId Ic scnt iIan
inIcctiouscauseis dccncdIiIclyandtlcvonitingis
< not Ioz ca1o | > < no rn om | >
< c~n n ozanx-onnocnn: uvcm os ons. y >
L|QIC|b LSI|OCOIC|O|OQy 1V
M 1RbLb-1
L0l0Dl LgDOSS O VODDg D LDlOl0D
DECtOUS
V||| QSI|OCOIC||IS [eSjCC|| |y
|OIdV||US dOU NO|wd| KV| |US)
bdCIC||| COIC|OCO||I|S/SCS|S
MCI|I|S
OOU OSOO|OQ
blGyOCOCCuS GUlCUS
LOSlHOlUD ClllOQCOS
bGDOOCG
|C|V|C |OHHdIO|yU|SCSC
|C||IOO|I|S
|||y|Q|I|S
|OCUHOO|
LI|I|SHCO|
OOS||||I|S
O||O|yI|dCI|OCCI|OO
NCtDOlC
L|dDCI|CKCIOC|UOS|S
| |DO||C||O|SO HCIDO||SH
PU|CO| |OSUhC|COCy
HCO| d||U|C
MCI|C||U|C
LCDtr NCrVOUS byStCD
| OC|CSCU |Ol|C|O|| |CSSU|e
VeOI||CU||C|IOOC| S|UOI
H|UOCI|O|
NC||OQ|I|S
OCC|||I|S
LDy||OI||I|S
N|Q|d|OC
HCyCS SyOU|OHC
bC|2U|C
UHO|
LyDCCOOgC
||CQOOCy
LStfODtCStDl.DDt
LdSI|OCSO|QC| |C U
LOw O|SOy H||K |OICO OIO|C|OCC
bOwC|ODSI|UCI|OO"
LUOUCO| l|CS|
y|O||C SICOOS|S
N||OII|O| w|I| O| w|I|OUI VO|VU|US
|OC|CC|ICO |e|||
|OIUSSUSCCjI|OO
NeCKe|S U|VC|ICU| UHw|I| IO|S|OO
M||SC|SUOQS O|SCSC
LStfODtCStD.LHlU
PC|OC|I|S
bOwC| ODSI|UCI|OO
N||OII|OO
|OC|CC|IeU |C|O|
|OIUSSUSCCI|OO
NCCKC|'S U|VC|I|CU|UHw|I| IO|S|OO
PU|CS|OOS
|OSII|dUHI|CODSI|UCI|OO
||C|CI|I|S
MCI|I|S
L|O|CCySI|I|S
hCSQftOfy
HCCI|VCd||wy U|SCSC
LDCOlOgy
L|CHOI|CfjCUI|CQCOIS
OXC DgCStOD
b| |Cy|ICS
|CO|y| | ||C
LUSI|CQCOIS
L|QO|O
LCO
LDOtODl
|SyC|OQC||C
bU||H|
NtOIIOD WlI Ot WIOUI VOlVUlUS lS DUC DOIE KEy D D DDI ID lD ClO.
fOD OUOOED EDIOD, fUIUtEO VSCUS, Of SUEtOt DESEDIEtC tIEty SyDOtODE.
signihcant. clcst radiograpl viIl ruIc our pncu-
nonia. Il a surgicaI proccss vitlin rlc aIdoncn is
considcrcd, upriglr and supine a|doninaI hlns
slouId Ic oItaincd, aIong vitl a conpIctc IIood
countandcIcctroIyte andclcnistrypancIs.nylasc
and lipasc slouId Ic scnr to dcrcct pancrcatitis. II
voniting is proIongcd or tlc paticnr is signihcantly
dclydratcd, cIcctroIytcsviII lcIp guidcrcpIaccncnt
tlcrapy.n annonia Icvcl, scrun anino acids, and
urinc organic acids slouId Ic scnt iI nctaIoIic
discasc is suspcctcd. Lrinalysis and urinc cuIturc
slouIdIcoItaincdtoruIcouturinarytractinIccrion
and asscss dcgrcc oIdclydration.
Treatment
IItlc causc appcars fo |c a scII-IinircdnonsurgicaI
inIcctious proccss [viral gastrocntcritis or IactcriaI
cntcrocoIitis) and rlc paricnf is nof signihcantly
dclydratcd, outpaticnt tlcrapy is indicatcd. CraI
rclydrationtlcrapy,vliclisdiscusscd in Claptcr7,
is rcconncndcd lor dclydratcd inIants. For oIdci
< not Ioz ca1o | > < no rn noax | >
bU Llucgrl nlO |ed|atr|cs
< cran n ozann-ronnocnn: uvcm os ons. y >
cliIdren, HuidsslouIdIccncouraged, vitl cautious
advancencnt to asoIt,IIand dict as toIcratcd. CliI
drcnvloarcscvcrcIydclydratcdorunaIlctocIcc-
tivcIy oraIIy lydratc tlcnscIvcs slouId Ic adnittcd
to tle lospitaI.
surgicaI consuItation nustIe oItaincd ilindi-
catcd. IlvcntricuIar-pcritoncal slunt naIIunction is
IcIicved to Ic causing cnesis, oItair a conputcd
tonograplyoltlclcad,asluntseries, andancuro-
surgicaI consuItation.
&
LN Ll W
M
1. Mostcasesofemes|sare caused bygasIroe
sophagealrefl ux,acutegasIroenIeriI|s,orsystem|c
disorderssuch asIons|ll |I|s,oIiI|smed|a,orurinary
tracI| nfect|on.
2. MosIch|ldrenw|th uncompl|catedv|ralgastroen-
teriI|sand mild dehydrat|oncanbetreaIedas
outpat|enIsw|Ihoralrehydrationtherapy.
Pylor|c Stenos|s
lyIoric stenosis is an inportant causc oI gastric
outlctoIstructionandvonitingintlehrst2nontls
oIliIe. IeaI incidcncc occurs at2 to 4 vccIs oIIiIc,
vitl an incidcncc ol I in bUU inIants. MaIc inlanis
arc aIlectcd 4 . I ovcr IcnaIc inIants, and pyIoric
stcnosis occurs norc IrcqucntIy in inIants vitl a
IaniIy listory oI tlc condition. Reccnt cvidcncc
suggcsts tlat crytlronycin tlcrapy nay prccipitatc
pyIoricstcnosis.
LDC0 N0D5l0lOD5
Iro]cctiIenonIiliousvonitingistlecardinaIlcaturc
oItledisordcr. IlysicaI Lndings varyvitltlcscvcr-
ityoltle oIstruction. Lclydrationandpoorvciglt
gain are connor vlcn tlc diagnosis is dcIaycd.
HypoIaIenic, lypoclIorcnic netaIoIic aIkaIosis
vitl delydration is sccn sccondary to pcrsistcnt
cncsisintlcnostscvcrccascs.TlecIassichndingoI
an olive-sizcd, nuscular, noIilc, nontcndcr nass in
tlc epigastric arca occurs in nost cascs. VisiIlc
gastric peristaItic vavcsnay Ic seen. IItrasonogra-
plyreveaIs tlc lypcrtroplic pyIorus.
l0lHDl
InitiaItrcatncntinvolvcsnasogastrictuIcpIaccncnt
and corrcction oI dclydration, aIkaIosis, and cIcc-
troIyte aInornaIitics. Iyloronyotony slouId taIc
pIacc as soon as tlc nctaIoIic anonaIics lavc Iccn
satisIactoriIy corrcctcd.
M
LN Ll W
M
1. |ylor|c sIenos|sisani mporIantcause ofgasIric
ouIleIobsIrucI|on and emes|s|nIhehrsI2
monIhsofl|fe,w|thapeakinc|denceat2IoR
weeksof|ife.
2. |roject||enonbil|ousvom|I|ng is Ihecard|na|
featureofth|sd|sorder.
d. |yloromyoIomyshouldIake placeassoonasIhe
meIabol|canomal|eshave been saI|sfacIor|ly
corrected.
Mal rotat|on and Volvulus
MaIrotation occurs vlcntle snaIl intestincs aInor-
naIIy rotatc in utcro, rcsuIting in naIposition in
tlc aIdoncn and aInornaI postcriorhxation oItle
ncscntery. \lcn tlc intcstinc attacles inropcrIy
to tlc nescntcry, it is at risI Ior tvisting on its
vascuIar suppIy, tlc tvisting plcnonenon is caIIcd
voIvuIus. Tlc nost connon agc ol prcscntation is
undcr nontl.
LDC0 N0D5l0lOD5
1lc listory aInostalvaysincIudcsIiIiouscncsis.In
oIdcrcliIdrcn,apastlistoryoIattacIsisoccasionaIIy
cIicitcd. IlysicaI cxanination nayrevcal aIdoninaI
distcntion, IIood-staincd cncsis or stool, and slocI.
Idoninal radiograpls typicaIIy slov gas in tlc
stonacl vitl a paucity oI air in tle intcstinc. n
uppcrgastrointcstinaIscricsvitlsnaIIIovcIIoIIov-
tlrougl conhrns tlc diagnosis Iy iIlustrating tlc
aInorna| position oItlc Iigancnt oITrcitz and tlc
cccun.positivcstooIguaiac cxaninationisapoor
prognosticsign,indicatingsignihcantIovclisclcnia.
l0lHDl
Cpcrative corrcction oI tlc naIrotation and tlc
voIvulus slouId Ic undcrtaIcn as soon as possiIIc,
M
LN L W
M
1. MalrotaIionoccurswhenthe | ntestinesabnor-
ma|lyrotate |n uIero,resulI|ng |n malposiI|on|n
theabdomenandabnormal posIer|orhxaI|onof
themesentery.WhenIhe inIesI| neaIIaches
| mproperly,|IisaIriskforvolvulus.
2. AnuppergasIro|nIesIina|ser|esw|Ih smal |bowe|
fo| low-IhroughconhrmsIhediagnos|sbycon
hrm|ng Iheabnormalpos|I|onofthe||gamentof
Tre|IzandIhececum.
< not Ioz ca1o | > < no rn oax | >
< cran n roxannronnoc _`

! ! V
| t I|OCIC|Ooqy b1
lccause IovcI isclcnia, nctaIoIic acidosis, and
scpsiscan progrcss quicIlyto deatI.
GASTROESOPHAGEAL REFLUX
CastroesophageaI reux [CLR} |s tIe rcgurgitarion
ol stonacl conrcnrs inro tIc csoplagus duc ro an
inconpctcnt Iovcr csoplagcaI splinctcr. snaII
dcgree olrcllux is connon in aII inlants, and it is
onIy inlants vIo lave nodcrate to scvcrc clronic
rcHux rlat tcndro conc to tlc pediatrician'sattcn-
tion. In tlis group, conpIications inc|udc IaiIurc to
rlrive, aspirarion pncunonia, csoplagiris, cloIingor
apreic cpisodcs, lcnatcncsis, anenia, and clronic
Iussiness.
Differentia! Diagnosis
InconpctcnccoltIcIovcrcsoplageaIsplinctcrnay
Ic rle rcsuIt oI prcnarurity, csoplagcaI discasc,
oIsrructivc lung discasc, overdistcntion ol tlc
stonacl causcdIyovcrcating, or ncdication [tlco-
pIyIIine) . II rlc inIant is laving IorccIuI cncsis or
pro]ectiIc voniring, rcllux is not rlc nosr l|IcIy
cause, and tIc dillcrcntiaI diagnosis lor cncsis ]usr
discusscd slouIdIcconsidcrcd.
Tle diIcrcntiaI diagnosisIor CLR in tIc adoIcs-
ccnt nay incIudc pncunonia, costoclondritis, pcri-
carditis,puInonarycnIoIisn, arrlyrlnias,isclcnia
duc to an anonaIous coronary arrcry, pancrcatiris,
cIoIecystiris, pcptic ulccr discase, and anxicry.
C!inica! Manifestations
5lDly
Ir is inporranr ro dctcrninc iItIe inlanrisspirting
up" or laving projccriIc cncsis and iItIc cncsis is
IIoody or IiIious. Cnc oltlc nost connon causcs
oI CLR is ovcrlccding, so a carcIuI lisrory slouId
incIudevlarIornulatlcinlanteats,lovitisnixcd,
lov nucl rlc inIanr cats during eacl Iccding, and
lov oItcn tlc cliId is Icd. |Itle encsis is indcpcn-
dcnt oIncaIs, itis proIaIIy not rellux. Iistory oI
cougling, gagging, and arcling ol tlc IacI vitI
cxrensor posturing during lccding nay rcsuIt lron
dircct aspiration, vlcrcas tIc prcscncc ol tIcsc
synprons soon aIrcr lccding nay suggcsr CLR. In
scverercllux,rlc inIanr naylavepoorvcigItgain.
In rlc oIdcr cliId, CLR is oIten nanilcstcd as
cpigastricaIdoninaIorclcstpain.LcIncrlcpain's
|ocation and scvcrity andvIctlcrit radiatcs andis
constantor intcrnittcnt. Butningepigastric orclcst
pain is proIaIIy rclux in tlc adoIesccnt, cspcciaIIy
ilit occursaItcrncaIs vlcntIc paticntIics dovn.
Dy5C0 LX0HD0lDD
In nost cascs, tlc plysicaI cxanination oltlc cIiId
vitl gasrroesoplagcaI rcllux is nornaI. In scvcrc
cascs, inIantsviII prcscntvirl laiIurerorlrivc.
Diagnostic Eva!uation
Tle diagnosis oIniIdrcHux isnadeIy tlc clarac-
rcrisriclisrory. |nnodcratcorscverercllux,tlcdiag-
nosis oICLRnayIc conIrncd Iy Iariun svaIIov
vitI upper gastrointcstinaI cxaninarion, pI proIc
pIaccnent in rIc csoplagus, or uppcr gasirointcsri-
nal cndoscopy.Ilscvcrc rcllux orpro]cctiIc cncsis is
prcscnt in tlc snaII inlant, gastric [pyIoric stcnosis)
or inrestinaI [duodcnal stcnosis or arrcsia, naIrota-
tion vitI voIvuIus) oIsrruction sIou|d Ic consid-
crcd. n aIdoninal uItrasound and Iariun svaIlov
atc useIuI to conhrn nornal anatcny and nornaI
gasrric entying.
1Ic cliId vitI niId ro nodcrare rcllux gcncraIIy
las an urrcnarIaIIc conpIcrc IIood counf and
cIcctroIyrc pancI. In scvcrc rcllux, a lypocllorcnic,
lypoIalenic nctaIoIic aIIaIosis nay cxist tlcsc
cliIdren laiI torlrivc and naylave pyIoric stcnosis
ratIcrrlan CLR.
Iltle clcsr cxanination isanorna|inrlcprcs-
cnccolrcHux,aclcstradiograpIslouIdIcoItaincd
to IooI Ioraspiration pncunonia or clangcs duc ro
rccurrenr aspirarion.
Treatment
Inlanrs vitl CLR sIouId rcccive snalI, Ircqucnt
lccdings in tIc upriglt posirion and Ic naintaincd
intIcpronclcad-uppositionIorarIcast20ninutcs
aIrcr a Iccding Iccds slouId Ie tlicIcncd vitI
ccrcalIIrlcsc ncasurcsIail,nctocIopranidcnayIc
uscdtoinprovcgasrricnotiIityand incrcasc tIcratc
oIgastriccnptying.IIcsoplagitisissuspccrcd,anI,
IlocIer [c.g.,ranitidinc) or a proton punpinliIitor
[c.g. , oncprazolc) nayIcuscIuI .
Ir casesvlcrcncdicaInanagcnentlails,aIisscn
IundopIication nayc ncccssary. |n tIis proccdurc,
tlc Iundus ol rlc stonacI is vrappcd around tlc
distaIesoplagustoincrcascIovcresopIagcaIspIinc-
tcrpressurc.
CIdercliIdrcn or adolcsccnts vitl rcllux slouId
bZ
nct , ,| < no nn oax | >
< cran n ozann-ronnocnn: ucm os ons. y >
ed|
aIsolavesnalI,IrcqucntncaIs,catsIovIy, andnain-
tain tle upriglt position aItcr neaIs. McaIs aItcr 7
l. N. slouId Ic discouragcd, and antacids nay Ic
uscIuI.
M
LN Ll W
M
1. Mostcasesofgastroesophagea|reI|uxoccuri n
thei nfantandado|escentpopu|ationsandwi||
noIrequiremedica|i ntervenIion.
2. MosIi nfantswith moderateCLRrespondto sma||,
frequentfeedingsinIheuprighIposition,Ihick-
ened feedswith ricecerea|,and maintenanceof
the prone head-uppositionforat |easI 20
mi nutesafterfeeding.
d. 1hemostcommonsymptomsofCLRi nIhe
ado|escentareburningepigastricpainand
chestpain.
D| ARRHEA
Liarrhea is dchncd as an incrcase in tlc Ircqucncy
andtle vatcr content oIstooIs.\iraI gastrocntcritis
accountslor 707 to 807 oIacutc JiarrlcainIortl
ncrica. Jlc conpIications oI acutc diarrlca
incIude dclydration, cIcctroIyte and acidIasc dis-
turIance, Iactcrcnia and scpsis, and naInutrition
in clronic cascs. LnIeritis reIcrs to snaII IovcI
inIannation, vlcrcas coIitis rcIers to Iargc IovcI
inIannation.
Diherentia| Diagnosis
TaIIc 8-2 Iists tlc nost connon causcs oIdiarrlca
in tlcpediatric population oItlc\estcrnvorId.
C|inica| Manifestations
5lDly
Tlc listory slouId asccrtainvlctlertlc diarrlca is
acute or clronic/rccurrcnt and estaIIisl tlc Irc-
qucncy, appcarancc [lIoody, nucosal, currantj cIIy),
anount, consistcncy, and color ol tlc diarrlca.
Lictary indiscrctions and nanipuIations nay resuIt
in diarrlca. SnaII inIants viII lave diarrlca vlcn
tlcy are |cd concentratcd Iornula. IItlc cliId las
travcled out oI tle country, consider a parasitic or
IactcriaI cntcrocoIitis.\ciglt Ioss or IacIoIvciglt
gain in association vitl diarrlea indicatcs norc
scvcrc diseasc. Certain ncdications, espcciaIIy anti-
Iioticsandclcnotlcrapcuticagcnts,naycause diar-
rlca.\iraI gastrocntcritisisliglIycontagious,sosicI
contactsare IiIcly. IIa cIosccontactoItlc cliIdlas
contact vitl rav pouItry, saInoneIIa slouId Ic
considcred. IouI-sneIIing diarrlca tlat Hoatsin tlc
toiIctisIiIcIysteatorrlcaandnayresuItIroncystic
hIrosisorIat naIaIsorption Iron otler causcs.
Dy5C0 LX0HD0lDD
Signs and synptons oIdclydration are discusscdin
Claptcr 7 and arc criticaI in tle evaIuation oI a
paticntvitldiatrlca.^nattenptslou|dIcnadcto
dctcnnine tlc dcgrce oI dclydration in ordcr to
guidc tlerapy. Tlc aIdoninaI exanination Iocuscs
onloveIsoundsandtlcprcscnccoIdistcntion,tcn-
dcrncss, or nasscs. Hypoactivc IoveI sounds point
to intcstinal oIstruction. Iypcractivc sounds arc
consistent vitl gastrocntcritis. IdoninaI nass
vitl diarrlca could indicatc intussusccption or
naIignancy .
Diagnostic Evaluation
\lcnevaIuatingacliIdvitldiarrlea,inspcctingtlc
stooIis ctiticaltocvaIuationandtletreatncntpIan.
Iltlcrc is a listory oIIIood ornucous orIotl in
tlc stool, IactcriaI cultures slouId lc oItaincd.
RapidtestsIorrotavirusandadcnovirusarcavailaIIc.
Rotavirus causes 657 oIinlant diarrlea during tlc
vintcr nontls.
IIaIactcriaIpatlogcn isIcing considcrcdandtlc
cliId is youngcr tlan 3 nontls, a IIood cuIturc
slouId Ie pcrIorncd Iccausc tlc incidencc olscc-
ondary Iactcrcnia Iron saInoncIIa entcrocoIitis is
ligl in tlis agc group.\hcn tlcre is a listory oI
Iong-tcrn or nuItipIc antiIiotic use, a L/uslr:dium
di]ci/e toxin assay slouId Ic scnt. StooI ova and
parasites slouIdIctcstcd Ior cliIdren vitl clronic
diarrlca,Ior tlosc vitl a listoryoIIorcigntravclor
rcccnt canping, and Ior innunoconproniscd
cliIdrcn vitl diarrlca. IItlc cliId appcars toxic, or
nodcrateto scvcrc dclydration is noted,a conpIctc
IIood count vitl nanuaI dihcrentiaI, c|cctrolytc
pancl, and urinc anaIysis is indicated. Lrinary tract
inIection is cvaIuatcd Iy urinc dipsticI, urinc
nicroscopy, andurinc cuIturc.
Treatment
IorunconpIicatcd viraI gastroenteritis vitlout sig-
nihcant delydration, tlc currcnt reconncndations
< not Ioz ca1o | > < no rn om | >
< CYc V ono

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L0f0Dt LgDOSS O LllD0 D LDOl0D
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HOIV||US
OIC|OV||US
POCOOV||US
NO|w|KQCOI
bCIC|||COIC|OCO| |I|S
blQCG
bGDOOCG
7Cl5lOlG
LGDyOOGClCl
LHfODCh0CUff0Dt LffH0
h0Dl
MC|O|y\|CU|C||CSyOO|O|C
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MCHOC|bC|O|C|O QU|QU|
D0CtOUS
||S|ICS
P|OCD|S|S
L||O|S|S
LQlO5OlOUD
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Oh[COIC|O|OVS|VCCOIC|OQI|OQCO|C)
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LVC|CCO|OQ
O| CC|I|VC CO||I|S
N.QOOOllOCGC
LlIOCODGl5
LXtf~Dt0StDl D0CtODS
LI|I|S |CO|
O||O|yI|CI|OCCI|OO
LStfODl0SlDl
|OIUSSUSCCQI|OD
PQQCOO|C|I|S
MyQCfCOOCCOI|ICO |OOIO||U|
LySI|CD|OS|S
OXC Dg0StOD
||OO,|C|CU|y, |CO,!UO||OC |OQCSI|OO
N0OCtOD DOUC0U
L|ODO`S O|SCSC
M||SC|SQ|UOQ'S O|SCSC
LCISCOCC|COCy
|||ID|C DOwC| O|SCSC
OCOQ|CS|S
CCSS|VC |UCIOSC |HIKC
LySICD|OSS
LC||C SQ|UC
Pll0fg
OOO | |C|Q|CS
POyOI|D|OI|C,C|C|OI|C|QCUI|C QCOIS
arctoIcedtlrougltlcdiarrlca.lccontinuationoI
nornal |ccdingsrcsuItsinlcssintcstinaldcnudcncnt,
inprovcdnutritionaI aIsorption, and a Iastcrrcturn
toanornalstooIingpatrcrn.IItlcinIantisaIsovon
iting, rcplacconcIccdvitl Picc-Iytc orIcdialytc to
caIn tlc stonacl and tlcn rcturn to nornaI Iccds.
CI:cn,tlcparcntsnccdtogivcsnaIIcrIccdingsnorc
Ircqucntly to acconnodatc tlc intcstinaI irritation
Iron tIc gasrrocntcritis and to nininizc cncsis.
InIantsvlodonottoIcratctlcirrcguIarIornuIaIut
arc not signihcantly dclydratcd or toxic appcaring
nayIeoraIIyrclydratcd atlonc. Scc Claptcr7Ior
dctaiIs on oral rclydrationtlcrapy.
Ior tlc inIant 0 to I 2 nontls oIdvitI diarrlca
Iornorctlan 5days,vitlsuspcctcdcntcrocoIitisor
cxposurctosaInoncIIa,astoolcuIturcslouldIcpcr-
Iorncd. lIood culturc slouldIcpcrIorncdiItlc
inIantis youngcrtlan 3 nontls. lItlc stooI cuIturc
is positivc and tlc inIant is aIcIriIc and docs not
appcar toxic, tlc inIant can Ie rccxanincd and
oIscrvcdat lonc. lItlcstool cuIturcispositivc and
tlc inIant is IcIriIc, tlc inIant's agc dctcrnincs
tlcrapy.
Tlc inIant youngcrtlan 3 nontls is adnittcdto
tlclospitaI,aIloodcuIturcisoItaincd,andintra
vcnous antiIiotics arc startcd. A IunIarpuncturc
andurinalysis slouId alsoIc considcrcdintlisagc
group.
TlcinIant oIdcrtlan 3 nontls IS adnittcdtotlc
lospitaI a IIoodcuIturc slouId Icscnt, Iut anti-
IioticsnayIcvitllcIdpcndingtlcrcsuItsoltlc
IloodcuIturc.
ny inIant vitl a positivc stooI cuIturc vlo
looIs toxic or las a positivc Ilood culturc is
adnittcdIorintravcnousantiIioticsandcvaIuaiion
Ior pycIoncplritis, ncningitis, pncunonia, and
ostconycIitis.
b4

not q > < no nn cam | >


< cran n ozann-ronnocnn: ucm os ons. y >
edacs
CIdercliIdrcnvitlviraIgasrroenteritisslouIdIc
encouragedtodrinIisoronic Iuids.nyIuid vitl a
liglcarIolydrarcIoadslouIdIediIutcdvitlvatcr.
dnission is indicatcd lor tlc cIild vlo is norc
tlan 57 dclydrarcd and cannot eIlcctivcIy oralIy
rclydrate linscll Scc Claptcr 7 Ior dcraiIs on
inrravenousrclydrarion.
\iraI gastroentcritis rcquires no plarnacoIogic
tlcrapy. ntidiarrlcal ncdicarions arc contraindi-
catcd Iecausc tlcy nay causc roxic ncgacoIon. In
gcncraI, antiIiotics arc nor indicated Ior Iactcrial
cntcrocoIitis. Lxccptions includc coIiris causcd Iy
on/mone//n (h, ohe/n, and L d]ci/c. sunnary
oI tle IactcriaI patlogcns and tleir treatncnt is
givcn in Claptcr Z. Farasitic gastrointcstinal inlcc-
tions slould Ic trearcdvitlrlc appropriarc antini-
croIiaI. /ntiIiotic-rcIatcd diarrlca renits vlcn rle
oIlcnding antiIioric is discontinucd. Intussusccption
is rrcated Iy hydrostatic rcduction vitl Iariun
cncna, air cncna, and/orsurgcry.
LN LW @
1. 1hemostcommoncauseofdiarrhea inchildren is
viralgastroenteritis.
2. 8acteremiaismore l i kelyininfantsyoungerthan
d monthswith bacterialenteroco|itis.
d. Mostchi|drenwith uncompl icatedvira|gastroen
teritis or bacteria| enterocolitiscan be rehydrated
ora| |y.
4. Do not use antidiarrhea|medications in chi|dren
with acutediarrhea.
5. Feedthroughdiarrheai ninfants.Pecoveryis
fasterbecausethereis|esss|oughi ngofthe
intestinal mucosa.
CONST|PAT| ON
Constipation isdcIncdasinIrcquenfpassagcoIlard,
dry stooIs. Constipatcd inIants IaiI to cnpty tlc
coIon conpIcrc|y vitl Iovc| novencnts and ovcr
rincsrrerclrlc snootlnuscIcoItlc co|on, rcsuIt-
ing a Iunctional iIcus. In contrast to constiparion,
obstipation is tle aIscncc ol Iovc| novcncnts.
BcyondtlcnconataIpcriod,tlcnostconnoncausc
[907-957)oIconstipationisducrovoIunraryvitl-
lo|ding orIuncrionaI consripation. IntcntionaIvitl-
loIding is oItcn norcd Iron rlc very Icginning ol
toiIcr rraining. laniIy listory oI siniIar proIIcns
is oIren oIraincd. StooI rctcntion nay Ic duc to
conIicts in roiIct training Iur is usua|Iy causcd Iy
painon deIccation,vliclcrcatcsaIearoIdcIccarion
andIurtlerrctcntion.\oIunraryvitlloIdingoIstooI
incrcases disrcntion oIrlc rcctun, vlicl dccrcascs
rccraI sensarion, ncccssitating an cven grcatcr lccal
nass to initiatc tlc urgc to dclccate. ConpIications
oIstoolrercntionincludcinpaction,aIdoninalpain,
ovcrllov diarrlcarcsuItinglronIcaIagcaroundtlc
Icca| nass, anaI Issurc, rcctaI IIccding, and urinary
tract inIection causcd Iy cxtrinsic pressurc on tlc
urcrlra.
|ncopresis vlicl is daytinc ornighrtinc soiIing
Iy Iorned stooIs |n cl|Idren Ieyond tle age oI
cxpccted toiIct rraining [45 years), is anotlcr
conpIicarion olconstipation. In oIder cliIdrcn, itis
inportant to asI spcciIcalIy aIour soiIing, Iccausc
sucl inIornarion nay not Ic expresscd duc to
cnIarrassncnr. Tlcsc cliIdrcn arc unalc to scnsc
rlc nced to dcIccarc Iccausc oI stretcling ol rlc
inrcnaIsplincrcrIytlc rctaincd leca|nass.
Crganic causcs oI laiIurc ro deIecatc includc
dccrcased jcristaIsis, dccrcascd expuIsion, and
anatonicnaIIornation.Crgan|cet|ologiesarcdel|n-
catcdintlc IoIlovingscction.
Differentia| Diagnosis
onornnic
Iunctional constipation [intcntionaI vitlloIding)
LysIunctional toilcttraining
Lrnnic
Le/ng. Lov-IIcr dict, inadcquare luid inraIc
Lns/roit/es/nn/. IuncrionaI iIeus, I|rsclsprung's
discase,anaIsrcnosis,rccraIaIsccssorIssurc,srric-
turc IoIIoving occrotizing cnterocoIitis [ILC),
coIIagenvascuIar discascs
Lrus or /oxn:. Lcad, narcotics, plcnotliazincs,
vincristinc, anticloIincrgics
euromuscu/or. McningonycIocele,tctlcrcdspinal
cord, inlant IoruIisn, aIsert aIdoninaI nuscIcs
[pruneIc|Iysyndronc)
e/nbo/c. Cystic IIrosis, lypothyroidisn, lypo-
Ialenia, lypcrcaIcen|a
!itdocrne. IyporIyroidisn
C|inical Manifestations
5lDly 0D0 Dy5C0 t0HD0lDD
AIdoninaI pain causcd Iy constipation is oI:cn
diIlusc and constant.1lc painnayIe acconpanicd
Iynausea, Iutvonitingis unusuaI. StooIs arc hard,
diucuItto pass, andinlrcqucnf. |atticuIar Ioods can
< nct fcz ca1o | > < no rn om | >
< crn n oxn-onnocnn: uvcm os ons. y
Chapter Gastroenterology bb
exacerIate constiparion. Liscussion ol tle psyclo-
IogicalstateoItlecliIdviI|heIpdetenninevletler
voIuntaryvitlloIdingistlenostliIeIydiagnosis.
nedicatior listoryisesscntiaI. IIalistoryoIdiarrlea
or IecaI spotringaIternatingvitl periods oIconsti-
pationexisrs, adiagnosisoIHirsclsprung'sdiseaseor
encopresis slouIdIe enterrained.
Cnexanination,tleaIdonen|s dihuseIyuncon-
IortaIIeratlertlantender, andtleIeItcoIonnayIe
easily paIpaIIe and IuI|olleces. RectaI exanination
osuaIly reveaIs a rectaIvauIt IuI| oIstooI. Iissure or
any otler recral processes can naIe deIecation
painIuI, so direct exanination is varranted.
Diagnostic Eva| uation
IItlediagnosisisuncIear, apIainaIdoninaIIIncan
Ie leIpIuI, Iecause a coIon |uIl oIstooI naIes tle
diagnosis ol constipation. Il lyporlyroidisn is con-
sidered, Iree1,,1SI,andJRLIeveIs are indicated.
IIlypoIaIeniaorlypocaIceniais apotentiaIcause,
an eIectroIyreandclenistrypaneInayIeoIrained.
Vlen !irsclsptung' s disease is suspected, a recta|
nucosal Iiopsyis required ro naIetle diagnosis./
IeadIevelassistsin diagnosingpIunIisnas tlecause
olcorstipation. Cenetic testing or a sveat test can
conIrn suspected cysric IIrosis.
Treatment
Mosr clildren virl |unctionaI consriparion can Ie
rreated tlrougl dietary clanges. 1le cliId's lluid
intaIeslouIdIeincreased,tleanountoIsinplecar-
Iolydrates[junIIood)decreased,andtleanountoI
IIer and IuII in tle dier [Iea[ vegetaI|es, cerea|s)
increased, tle cliId slould Iegir daiIy ingestion oI
undiIuredprune]uiceorappIe]uice.SennaorCoIace
slouId Ie reserved Ior cliIdren in vlon dietary
neasuresareinsuIlcient.1leroutineuseollaxatives
orenenas is discouraged.
Tle constipated clild vitl inpaction nay Ie
nanuaIIy disinpactedornayreceive aIIeetenena
vith asrooIsoIterer [Co|ace),osnoric agent [lacru-
Iose, ninera| oil, Mira|ax), or peristalsis inducer
[serra). nal Issures are trcared Iy soI:ening rle
stooIs, avoidingrle insertion oIoI]ecrs in tle anus
[tlernonerer), Ieepingrle rectun as clean as pos-
siIIe, andapplyingpetroIeun]c|IyIocalIyvitl eacl
diaper clange. Hirsclsprung's disease slouId Ie
nanagedir consuItationvitlapediatric surgeon or
gasrroenterologisrorIotl.
IncliIdrenvith cysticIIrosisandtlosevlolave
receivedvincristine,constipation canIesopersistert
and intracraIle tlat CoIYTLIY cIeanouts are
needed. CoLY1LIY [poIyerlyIene glycoI-eIecrrolyre
soIution) is a povetIuI osnotic catlartic. In sone
severecases,constiparionduetopsyclologicaIcauses
requires counseIingorpsyclotlcrapy.
M
LN Ll W
B
1. Const|pat|on |sdehnedas|nfrequentpassageof
hard,drystools.Const|patedpat|entsfa|l tocom-
pletelyemptythecolonw|thbowel movements
andovert|mestretchthesmooth muscleofthe
colon,result|ng|nafunct|onal|leus.
2. |a| l uretodefecateresult|nghomorgan|ccauses
maybeduetodecreasedper|stals|s,decreased
expuls|on,and anatom|c malformat|on.
d. |n|nfancy,const|pat|on |scommon|yassoc|ated
w|th anal hssure.
4. 6eyondtheneonatalper|od,themostcommon
cause(90%-95%)ofconst|pat|on|svo|untary
w|thhold|ngorfunct|onalconst|pat|on.
5. Mostcasescan betreatedw|thad|etora m|ld
stool softenerforashortt|me.
H|RSCHSPRUNG'S D|SEASE
lirschsprung's disease or congenitaI aganglionic
negaco|on, occurs ir 1 in 5000 cliIdren andresuIts
lron rle lai|ure oltle gangIion celIs oltle nyen-
reric pIexuses ronigratedovnrledeveIopingcoIon.
s a resuIt, tle aInoua|Iy innervated dista| coIon
renains tonicaIly contracred ard oIsrructs rle llov
oIIeces. Iirsclsprung's disease is tlree tines nore
connon anongIoys andaccounts lor 20% oIcases
ol neonatal intestinaI olstruction. In 757 oI cases,
tle agangIionic segnent is Iinited to rle rectosig-
noidcoIon,vlereas I 57extendIeyondrle spIenic
llexure.
C|inical Manifestations
TlediagnosisslouldIesuspecied in anyinIanr vlo
laiIs to pass neconiun vitlin tle hrst 24 lours ol
IiIe ard vlo requires repeated rectaI sinuIation to
induce Iovcl novenents. In tle Irst nontl olIile,
tle neonate develops evidence ol oIstruction virl
poor leeding, Iilious voniting, and aIdoninal dis-
tention. In sone cases, parricuIarIy tlose vitl slorr
segnent [Iess rlan 5cn involvenent, tle diagnosis
goes undetecred inro cliIdlood. In rle older cliId,
IaiIuretotltive nayIeseen,asvelI asinternittent
* nct fcz ca1o | > < no pLn pcpa | >
bb |l ugrinlO Ped|atr|cs
Iouts oI intcstinaI oIstruction, enterocoIitis vitl
IIoodydiarrlca,and,occasionaIIy,IovcI pcrIoration,
scpsis, andslocI.
StooI tlat is paIpaIIc rlrouglour tlc aIdoncn
and < enpty rcctun on digiraI exanination are
nostsuggcsriveoItlcdiscasc.IdoninaIradiograpl
slovs distenrion oItlc proxinal IoveI and no gas
oi Icces in tlc rcctun. Bariun cncna nay dcnon-
strareatransirionzoncIetveentlenarrovcdaInor-
naI disraIscgncnt and tlc diIated nornaIproxinaI
IovcI.nal nanonctry dcnonstrates IaiIurc oItlc
intcrnalsplinctcrto rcIaxvitlIaIIoon distcntionoI
rlc rectun. PccralIiopsyrcvcaIingno gangIionccIIs
and lypertroplicd ncrvctrunIs is neccssary lor tlc
diagnosis.
Treatment
Iirsclsprung's discasc is trcatcd surgicaIIy in tvo
stagcs.1lcIrststagcinvolvcstlccrearionoladivcrt-
ingcolosronyvitltlclovcI tlat containsgangIion
ccIIs,tluspcnittingdcconprcssion oItlcgangIion-
containing IovcI scgncnt. In tle second srage, rle
agangIionic scgncnt is rcnovcd Iy puIling tlc gan-
gIionicsegncnttlrougltlc rectun.Jlis proccdurc
is postponcd unriI rlc inIant is | 2 nontls oId oi
dcIayedIorJto6nontlsvlcntlcdiscasclasIccn
diagnosed in an oIder clild. 1lc norrality ratc Ior
rlis disordcr is Iov in rlc aIscnce oI cntcrocoliris,
najorconpIicarionsincIudc anal stenosis [57! 07)
andinconrincncc [7J7) .
LN Ll W
2
\ . Hi rschsprung'sdisease resu|tsfrom thefai l ureof
thegang|ioncellsofthemyentericp|exusesto
migratedown thedeve|oping co|on.Asa resu|t,
theabnorma||yinnervated dista| colon remains
tonical |ycontractedandobstructstheflowof
feces.
2. Thediagnosisshou|dbesuspected inanyinmnt
whofai | stopassmeconi umwithinthehrst24
hoursof|ifeandwhorequiresrepeated recIa|
simu|ationto i nduce bowe| movements.
d. l nthehrst month oflife,evidenceofobstruction
includespoorfeeding,bi|iousvomiting,and
abdomina|distention.
4. Recta|biopsyrevealingno gang|ion ce||sand
hypertrophiednervetrunksisnecessaryforthe
diagnosis.
GASTRO|NTEST|NAL BLEED|NG
CastrointestinaI bIeeding nay Ic acutc or clronic,
gross or nicroscopic, and nay nanilcst itscII as
lcnatcnesis, lcnatoclczia, or neIena. 1lcrc arc a
pIctlora oI disordcrs in cliIdlood rlat causc gas-
trointcsrinaIIIccding.
lematemesis rcIcrs to tlc cnesis olIrcsl oroId
IIood Ion tlc gastrointcstinaI rract. Ircsl IIood
Iccones clcnicaIIy aItcrcd ro a "ground cohcc"
appcarance vitlin 5 ninutcs oI cxposurc to gastric
acid. lematothezia is tle passagc oI Ircsl [Irigt
rcd) or darI naroon IIood Iron tle rcctun. Jlc
sourcc isusuaIIytle colon,aItlougluppcrgastroin-
tcstinaI tract IIccding rlat las a rapid transit rinc
can aIso resultin lcnatoclezia. MeIena is sliny, jet
IIacI, tarry stooIs rlatarcguaiac positivc. ItrcsuIts
usualIy Iron uppcr gastroinrcstinaI IIccding, tlc
Ilood las Iccn clcnicaIly altcrcd during passagc
rlrougl tlegut.
Diherentia| Diagnosis
1lc diherentiaI diagnosis Ior gastrointesrinaI IIccd-
ing is generaIIy dividcd into uppcr and Iovcr gas-
trointcstinal rracr crioIogics. \ppcr gastroinrcsrinaI
Ilccdingoccurs atasitc proxinal totlcIigancntoI
1rcitz,vlercasIovcrgastrointcsrinaIIIccdingoccurs
ata site distal to tlcIigancnt.Itlougllcnarcnc-
sis Iron upper gastrointestinaI Ilccding can Icsccn
in criticaIIy iIIcliIdrcnIron csoplagitis orgasrritis,
or in cliIdrcn virl porraI lyperrcnsion Iron
csoplageaI variccs, nost gastrointcstinaI IIccdingin
cliIdrcn is Iron tlc Iovcr tract and nanilcsts as
rcctaI IIeeding. 1aIIc 8-J Iists tle nost connon
causcsoIrectaIIIccdingIyagc. MinorIIccdingpre-
scntsassrooIsrrcaIcdvitlIIoodaIrerstooIispassed
andisusuaIIyducto an anaIIssureorpoIyp.InIan-
narory discascs, sucl as inIannatoryIovcl discasc
or inIecrious cntcrocoIiris, rcsuIr in diarrleal srooI
nixcd vitl IIood. Causes ollcnatoclczia incIudc
inlannatory Iovcl discasc, MecIel' s divcrricuIun,
lcnoIytic urcnic syndronc, Henocl-SclonIcin
purpura, and inIcctious cntcrocoIiris. JaIIe S-4 Iists
tlc associarcd signs and synptons oI tlc najor
causcs oIgasrroinrcstinaIIIccding.
Clinical Manifestations
5lDly
Ir is inporrant to dcInc tlc onsct and duration oI
IIccding,coIor [IriglrrcdvcrsusdarInaroonversus
< nct fcz sa1e | > < ue pLn pcpa | >
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O|VC|I|CU|U|
| bL
bCIC||| CO|I|S
Mb|
MOb
|HIUSSUSCCI|OO
bgDS DU bDQtODS
| HQCSI|OH OPbP,OI|C| NbP|LS
bQ|COO|CQ|yO|CV|OCOCCO||VC|OSCSC
LySQ|Q|,VO||I|OQ,OySCS|, ||||ID|| |Iy |O |OOIS
|QSIf|C |O, |CfC|ICO, |y DC |OC|CSCO IO|Q|I,||| y||SIO|y
b|Q|I|CO D|OOO OOSU|CC O SIOO, O,COHSI|I|OO,DSSU|COICOV|S|D|COH
O| CVC|S|OO
b||Q|I |CO D|OOO OO SU|CC OSIOO|,O|CSS
b|OOO ||CO wI| SIOO,O||||C, I|CHI |y|VC |yOQ|OIC|HC|,COC|
||H|CSS D|CCO|OQ,||CO wI| SIOO|,O\CO |OI O D|OOO
L||||C,CVC|, DOO||H| |H, OO|Q|OwI|,SSOCICO SySIC||C S|QOS HO Sy|IO|S
PDOO||O| O,O||||C,CVC|,OI|D|OI|CS
JO|HIQ|O,U|QU|,DOO||O| Q|O, OCQ|||I|S[CSIS,HbLS |OU||HC)
L|f||C,|CO|| | U|C,I||O|DOCy\OCO,||C|OOQ|OQI|C |C|O|yI|CHC||
| OIC|||IICOI DOO||H| Q|O,VO||I|OQ,|| O|,|CO CU||OI|C| |ySIOO|,||Q|IS|OCO |SS
P5P, CCIySllCylC ClO, m5[ EDOCbCDDElD UlUl, Lb, DCDOyIlC UlEDlC SyDOlODE, L, lDDDIOly OOWC O SESE, N5PL, DODSIElOOl
DIlDDDIOly OlU_, LL, EIlC UlCEt OlSESE, HL, tEO OOOO CE.
rarryIIacI), ratc [lrisIvcrsusgradual), and rypc ol
Ilccding [lcnatoclezia, lcnatcncsis, ncIcna,
IIood-strcaIcd srooI). Sonc clronic ncdical condi-
rions rcsuIt in gastioinrcstinaI IIccding, incIuding
prcvious gastrointcstinaI surgcry, Iivcr discasc,
csoplagitis, pcpticuIccrdiscasc,inIannaroryIovcI
discasc, history oI nilI coIitis, lisrory oI coIonic
poIyps, orcoaguIopatly.
aIout lorccIul voniting, ingcstion ol uIccrogcnic
drugs [saIicyIatcs, nonsrcroidal anti-inIannatory
drugs, stcioids), and a IaniIylistory oIIivcr discasc
or pcptic uIcci discasc. For lovcr gasrrointcstinal
tractllccding,inquircaIoutdiarrlca,inIcctiouscon-
tacrs, Iorcign travcI, antiIioric or clcnotlcrapcutic
usc, and constipation vitl Iargc or lard srools and
diIlcuIt orpainIuI dcIccation.
IoruppcrgastrointcstinaIIIccding,asIspcciIcaIIy 24- ro 48-lour Iood listory is inportant,
< o ;oz > < ue /TR poJam | >
bb r
< cxau n pexaex~xouaeycwn: HC s ou. y >
Iccause nuItipIc cpisodes oIrcd" vonitus or diar-
rlca could rcsuItIron tIc ingcsrion oIrcdIuids or
Ioods [KooI-/id, Iccrs, rcd 1cIlo, JyIcnoI cIixir) .
McIcnaisnotaIvaysducroIIoodin tIcstooI,itcan
occur in cIiIdrcn vlo lavc ingcsred iron, Iisnutl,
IIacIIerrics, orspinacI.
Dy5C0 LX0HD0lDD
TlcinncdiatcpriorityvIcncxaniningacliIdvitl
gastrointesrinaI lIccding is to deterninc il lypo
voIcniaexistslronanacutcIIced.\itaIsignsslouId
Iecxanincd Ior ortlosratic clanges or Ior cvidcncc
oIslocI [Laclycardia, taclypnca, lypotcnsion) .Jlc
carlicsrsign oIsigniIcantgasrrointesrinaI Ilccdingis
a raisedrcsringIcartrarc. drop in IIoodprcssure
is noi seen untiI at least 407 ol tle intravascuIar
voluneisdcplctcd.LcrnatologicaInornaliticssucl
as pctecliac and purpura indicatc coagulopatIy,
vlcreas cooI or cIanny sIin vitl palIor is suggcs-
tivcoIslocIorancnia. CnaIdoninaIexanination,
cvaIuate Ior cvidcnce oI nasses [a riglt Iovcr
quadrant nass nay Ie duc ro Croln's discasc or
intussusception), tcnderncss [cpigastric tcndcrncss
suggesrs pcpric ulccr discasc, riglr Iovcr quadrant
tcndcrness nay Ie duc to Croln's discasc or inIcc-
tious enrcrocoIitis), and lcpatosplenoncgaIy and
caput oI ncdusa [cvidcncc oI portaI lypc)tcnsion
andrisIoIvariccs) .CapiIIaryrcIII [tlcnarcnincncc
in nconarcs and inIanrs) sIouId Ie asscsscd on tle
cxtrcnirycxanination. CnrectaI exanination, IooI
IoranaI Issurc, vlicl is Icst seen Iy sprcading tle
IutrocIs and cverting tIc anaI canaI [nost Issures
arc Iocated ar tIc 6 and ! 2 L

cIocI positions),
pcrIorn a stooI guaiac cxanination, IccI Ior lard
stooI, and IooIIora diIatcdrcctun in cliIdrcnvitl
clronicconstiparionoranaI Issure.
Diagnostic Eva|uation
InIcss tle sourcc oI Ilecding is clcarIy Iron tlc
nasoplarynx, an anaI Issurc, orlenorrloids, a con-
pIcteIIoodcountvitlnanuaIdihercntiaI, coagula-
tionstudics,and atypc andcrossslouIdIcscnr.
IItleIIccdingsourccisuncIcarandtlcpaticntis
unstaIIe, rlc c|inician slouId use gastric Iavage to
dcrcrninc vlctlcrrlcIIcedingisIron tIcupperor
Iovcr gasrrointcstinaI rract. veII-luIricatcd naso-
gastricororogastricruIc oIrlcIargestIorcpossiIIe
slouId Ic pIaccd, and tle stonacl Iavaged vitI
roon-tcnpcraturc nornaI saIinc untiI Iavagc Iuidis
cIcar.IcedsaIincnaycausclypotlerniaandslouId
Ic avoidcd. LsoplageaI variccs are nor a contraindi-
cationto tlcplaccncnro|a nasogastricororogastric
ruIc. ReturnolcIcarIavagcIuidnaIcstIcdiagnosis
oIupper gasrrointcstinaI Ilccding unIiIcly, aItIougl
occasionaIIyduodcnaIuIccrs nayIIeed onIydistaIIy.
Rcturn oIguaiac-positiveIrigltredIIood or"cohcc
grounds" tIat eventuaIIy cIear indicates uppcr gas-
trointcsrinal IIccding tIat las rcnittcd. Fcrsistcnt
rcrun oIIriglt rcdIIood indicatcs activc IIecding
and nandatcs aggrcssivc intravcnous Iuid nanage-
ncnr.
In tIe staIIe patient, a tIorougl Iistory and
plysicaI exaninaLion vitI considcration oItIc agc-
rcIatcd causcs viII usuaIIy Icad to diagnosis. Castric
IavagcisunncccssaryincliIdrcnvitlninorornona-
cufc gasrroinrcstinaI IIccding.Tlc precisc diagnosis
is usualIynadcIyuppcrorIovcrendoscopy.
IIrlereisIIoodydiarrlca,stooIslouIdIcscntIor
nctlyleneIIuc stainingto IooIIor\BCs andstooI
cuIture. In tIe neonatc vitl IIoody stool, nccro-
tizing entcrocolitis nust lc considcrcd, and an
aIdoninaI IIn and cvaIuation Ior sepsis slouId Ic
pcrIorned.\Icn svaIIovcd natcrnal IIood is sus-
pcctcd as tlc causc oIgastroinrcstinaI IIeeding, tIe
pr test is pcrIorncd on tIc cliId's stooI to
diherentiate natcnal Ilood Iron rle Ilood oItle
nconate. IIoraI IIood is notcd and rlcrc is a vors-
cning puInonary cxanination, a clest radiograpl
nay denonsrratc puInonary lenorrlagc.
MccIcl' s scan can Ic pcrIorncd vIcn MccIcl's
diverricuIun is suspcctcd.
Treatment
In tIc unstaIIc cliId vitl severe IIeeding or lypo-
voIcnia,IoIIovtlcprinaryandsccondarysurvcysas
outlinedin Claptcr ! . RcncnIcr, nornaI lenoglo-
Iin or lenatocrir docs not ruIe out scvcrc acurc
IIccding, IulI IenodiIution taIes up to ! 2 Ioursin
tIc acuteIy IIccding paticnr. Inrravenous nornaI
saIinc orRingcr's Iactarc ar 20nL/IgIoIuscsslouId
IcgivcnuntiI tIc patienr isstaIIe.Jype C-ncgative
vloIe IIood slouId Ic rcscrvcd Ior tlc unstaIIe
patienr vitI acutc IIccding tIat cannor quicIly Ic
Irouglt undcr conrroI.Tlc nost connon crror ir
nanagenenr oI tlc cliId vitI scvere gasrrointcsti-
naI IIeeding is inadcquarc voIune rcpIaccncnt.
Iypotension is a Iatc Inding, Iluid rcsuscitation
slouIdIe govcrncd Iyrlc IcvcI oItaclycardia.
Tlc staIIc cliId vitIout lcavyIIecding or signs
oI IypovoIcnia slould Ie cvaluared and rrcatcd
accordingto tIc particuIar diagnosis.
Iigure S- iIIustrates a useIuI aIgoritIn Ior tIe
cvaIuation and nanagcncnt oI gasrrointestinaI
IIccding. Tlrce connon causcs ol gastrointcstinaI
IIccdingMccIcI's divcrticuIun, uIcerativc coIitis,
nor Ioz ca1o | > < no rn om | >
< c~n n ononnoc

ucm os
]
g:ons y
QtCr I|OCOIC|O|OQ bV
/HCs |uCCyOan|C slaC|||za||CO
||u|C rsUsCilaIiCO
||slC|yatC jys|Ca| (|c |)
Ljj|lraCl C| C|OQ

|Cw|laClC C|OQ
Casl|C | avaQ ~~~&&&&& &&
Casl|CCCu|ljCs|l|v
ns|s C|
OasCQasl|C asj||al

HCCnlnj|alu| sa||O | avaQ


C|ar
/OIaC|Cs
|_C|CC||s
||ClCOjuujC|CC||s
|
|ClC|a|
CCOs|C| |V|nlus|Cn
Cl|_C|CC||s aOC
vasCaCl|vaQOls
|

|Qal|v
|
Casl|CCCu|l-OQal|v
OasCQasl|iC asj|ral
_clCC| Qua|aC _
||CCCu|ljCs|l|v |nCCCu|lOQal|v
slCC| slCC|
Ca| | u|CCyls
'

H|snul
| rCO
HClCCCsaOC
lCCCCC|C|OQ
|Cs|l|v
|
Ojj|OCCsCCjy LjOCCsCCjy
Ljj|C| sr|s
|| sC|l sCu|C L|Ius sCu|C
CCOs|C|CasCCO|c |
/CCCn|Oa| l|'n
Ljj|C| w|lsna|'
CCw|lC||Cwl|CuQ
Har|uu Ona
CC|CtCsCCy
MC||'s sCaO
clCC| Cu|lu|!Cva aOC ja|aS|ls
l
|Cs|l|v lQal|v
| |
1aQQCHHC sCaO
1|nCCCaQu|al|CO
cC|rCl|ajy
|_C|CC||s
|rClCO juujC|CC||s
/OlaC| Cs
1|al
aCCC|C|OQ'y
CCOs|C|
CC|COCsCCQy
|igureb-1 P|QO||I|| OI CVl Ul|O| |U |QC|C|lOQS\|O|\CS\|||\|C\L|CCU|OQ.
B
LN Ll W
W
1. Uppergastrointestinalbleedingoccursata site
proximalto the ligamentof1reitz,whereas lower
gastrointestinalbleedingoccursdi staltothe
ligament.
2. Mostgastrointestinalbleedingi nchi l drenisfrom
thelowerL tractandmanifestsasrectalbleeding.
d. Theearliests|gnofsignihcantgastrointestinal
bleeding isaraisedresting heartrate.P drop in
bloodpressureisnotseenuntilatleast4%of
thei ntravascularvol umeisdepleted.
and Croln's discasc-arc discusscd in rlc loIloving
sccrions.
Mecke!'s D|verticulum
MeckeI's diverticuIum tlc vcstigiaI rcnnant ol
tlc onplaIoncscntcric duct, is tle nosr connon
anonaIyoltlc gastrointcsrinaI tract. Ir is prcscnt in
27 to J% ol tle popuIation and is Iocatcd vitlin
l 00cn oltlc ilcocccaI vaIvc in tle snalI inrcstinc.
1lc peaI incidcncc olIlccdinglronrlc divcrricu-
Iun is at Z ycars olagc. Ictcrotopic tissuc, usualIy
gastric, is I 0 rincs norc connon in synptonatic
cascs Iecausc olacid sccrction and uIccrarion.
9U
c )cz e > ue /TR QH | >
< CYo V pexaan~ouaeycvn: HC s ycu. y >

LDC0 N0D5l0lDD5
Tlc nost connon prcscntation oI MccIcI' s divcr-
ticuIun is painIcss rcctal IIccding. Liglty-Ivc
pcrccnt oIpaticnts vitl MccIcI'sdivcrticuIunlavc
ncIcna, I 7viIIdcvcIopintcstina|oIstructionIron
intussusccption or voIvuIus, and 57 suhcr Iron
painIl divcrticuIitis ninicIing appcndicitis. 1lc
diagnosisisnadcIypcrIorningaMccIcI'sscan.1lc
tcclnctiun-99 pcrtcclnctatcscan,prcccdcdIyprc-
pcntagastrin stinuIation or a listaninc I-rcccptor
antagonist [cinctidinc), idcntiIcs tlc cctopic
acid-sccrcting cclls crcating tlc lcnorrlagc in tlc
divcrticulun.
l0lHDl
Lcnitivc trcatncnt is surgicaIrcscction.
K
LX Ll W

1. Mecke|'sdiverticu|un,thevestigia|rennantofthe
onpha|onesentericduct,isthenostconnon
anona|yofthegastrointestinaltract.
2. Thenost connon presentation ofMecke|'sdiver-
ticu|un ispain|ess recta| b|eeding.
| nfammatory Bowe| Disease
lnllammatory boweI disease [VL} is a gcncrictcnn
Ior Crohn's disease anduIcerative coIitis vlicl arc
clronic inIannatory disordcrs oItlc intcstincs.
Ilccrativc coIitis produccs dilusc supcrIcial
coIonicuIccrationandcryptaIsccsscs.ItinvoIvcstlc
rcctunin 957oIpaticnts,vitl orvitloutcontigu-
ous cxtcnsior liglcr in tlc coIon. llccrative coIitis
docsnot aIlcct tlc snaII intcstinc.
Tlc patloIogy oI Croln's discasc invoIvcs trans-
nural inllannation in a discontinuous pattcrn,
vlicl rcsuIts in sIip Icsions. Croln's discasc nay
invoIvc anypanoItlc gastrointcstinaI tract [noutl
to anus). 1lc proccss is ilcocoIic in 607 ol cascs,
involvcs tlc snaII intcstinc in J07 oI cascs, and
inpairs tlc coIon in on|y I 07 oI cascs. IiIrosis is
transnuraI,and stricturcs arc connon. Cranulonas
arc olscrvcd in up to J07 oI paticnts. IntcrnaI or
cxtcrnaI IstuIa Iornation occurs in up to 407 oI
paticnts.
/Itlougl tlc cxact ctioIogy oItlcsc disordcrs is
notInovn,aconIinationoIgcnctic,cnvironncntaI,
psycloIogicaI, inIcctious, and innunoIogic nccla-
nisnslavcIccninpIicatcd. IBL isnostconnonin
vlitcs and 1cvs and occurs cqua|Iy in naIcs and
Icnalcs. Mostpcdiatricpaticnts arc adoIcsccnts, Iut
lotl discascs lavcIccnrcportcd in inIancy.
LDC0 N0D5l0lDD5
CranpyaIdoninaIpain,rccurrcntIcvcr,vcigltIoss,
and diarrlca arc connon naniIcstations in Crolr's
discasc./ltlougl diarrlca is connon, it is notuni-
vcrsaI in Croln'sdiscasc. RcctaIIIccdingisnotcdin
onlyJ57oIcascsoICroln'sdiscasc./IdoninaIpain
tcnds to Ic norc scvcrc in Croln's discasc tlan in
uIccrativc coIitis, nay Ic diIlusc, and is IrcqucntIy
vorsc in tlc riglt l ovcr quadrant. Fcrianal discasc
nay producc sIin tags,Issurcs,IstuIas,or aIsccsscs.
/orcxia, poor vciglt gain, and dcIaycd grovtl
occur in 407 oIpaticnts.
Most cliIdrcn vitl uIccrativc coIitis cxliIit
IIoodynucinous diarrlcal stooI [ I 007), aIdoninaI
pain [957),and tcncsnus [757) . lincty pcrccnt oI
paticnts cxliIit niId to nodcratc discasc. MiId
discasc is dcIncd as Icss tlan six stooIs pcr day, no
Icvcr,noancnia, andno lypoaIIunincnia,vlcrcas
nodcratcdiscasc lasgrcatcrtlansixstooIspcrday,
lcvcr, ancnia, and lypoaIlunincnia. Scvcrc discasc
nay Ic Iulninant vitl ligl Icvcr, aIdoninaI tcn-
dcrncss,distcntion,taclycardia,IcuIocytosis,lcnor-
rlagc,scvcrcancnia, andnorctlan cigltstooIspcr
day. Toxic ncgacoIon and intcstinal pcrIoration are
rarc conpIications./I:cr ! 0ycars oIdiscasc, tlcrc is
acunuIativcrisIolI7to 27 pcrycarIortlcdcvcl-
opncnt oIcarcinona. 1aIlc 8-5 conparcs Croln's
discasc anduIccrativc coIitis.
LxtraintcstinaI scqucIac, siniIar in Iotl discascs,
nay prcccdc or acconpany gastrointcstinaI synp-
tons and incIudc polyarticuIar artlritis, anIyIosing
spondyIitis, prinary scIcrosing cloIangitis, clronic
activc lcpatitis, sacroiliitis, pyodcrna gangrcnosun,
crytlcna nodosun, ncplroIitliasis, apltlous ston
atitis, cpisc|critis, rccurrcntiritis, anduvcitis.
Bccausc uIccrativc coIitis invoIvcs tlc rcctun in
957oIpaticnts,proctosignoidoscopyandIiopsyare
indicatcd. \isuaIization oI tlc nucosa in uIccrative
coIitis rcvcaIs dihusc supcrIciaI uIccration and casy
IIccding. In Croln's discasc, dircctvisualization and
Iiopsy oItlc iIcocccaI arca arc notalvays possiIIc.
Radiograplic cxanination vitl a douI|c air-
contrast Iariun cncna dcnonstratcs dihusc coIonic
lcsions and pscudopolyp Ionnation in ulccrativc
coIitis. 1lis cxanination slouId Ic dclaycd in
paticntsvitlscvcrcIyactivcdiscascto avoidprccip-
itatingtoxicncgacoIon.Croln'sdiscascoI:cnrcvcaIs
iIcal and/or coIonic invoIvcncnt vitl sIip Icsions,
< DO! IOJ sale! > < ue )7H 1]O)a ! >
LomtlSo ol Ltoh`S LlSeSe 0 UCeftlve
LoltlS
Lf0hn`S Ulcer1ve
e1ufe OSeSe L01S
Ma|a|se,fever, Common Common
we|ght|oss
Recta| b|eed|ng Somet| mes Usua|
Abdomina| mass Common Ra|e
Abdom|na| pa|n Common Common
Per|ana|d|sease Common Rare
||ea||nvo|vement Common None backwash
||e|t|s)
Str|ctures Common Unusua|
||stu|a Common Unusua|
Sk|p|es|ons Common Notpresent
Transmura| Usua| Notpresent
| nvo|vement
Cryptabscesses Unusua| Usua|
G|anu|omas Common Notp|esent
R|skofcancer S| | ght|y Great|y
|ncreased | ncreased
recfal saring, segnenfaI narroving oI fhe ileun
[sirirgsign),andl ongifudinal ulcors.
IneniaisconnonandusuaIIyis associafedvifh
iron delciency. MegaIoLIasfic anenia secondary fo
IoIafeandvifaninB,dehciencynayaIsoLeresenf
InelevafionoIfhe eryfhrocytesedinenfafionrafcis
seen in aLout 50% oI cases oIuIcerafive coIiiis and
in S0% oICrohn's disease cases. IyoaILuninenia,
cao-ed Ly oor rotein infake, is connoo io indi-
viduals vifh scvere synfons. Serun aninoirans-
Ierase levels are increased iIheafic inIlannafion is
a conIicafing Ieafure. SfooI exaninafion reveaIs
Llood and Iecal Ieukocyfes vifh a negat.ve sfool
cuIiure.
LHPfPOl0 L0gODSS
Tle diI!erenfial diagnosis oI 11 incIudes chronic
LacferiaIorarasii|ccauses oIdiarrhea, aendicifis,
henoIyfic urenic syndrone, Ienoch-SchonIoin
urura, and radiafion enferocoIifis. Lnferic inIec-
tions include L Jici|e, Con|o/octer jejuni,
Yersinio enterco|itico, aneLiasis, ard giard.asis.
fP0lPOl
Treainenf oI inIlannafory Lovel disease is ained
af confroI oI inIlannafion and suression oI fhe
innune sysfen. The variefy oI agenfs availaLIe is
quickIyincreasing.5-Ininosalicylicconoundshavc
IongLeenanainsfayoIanfi-inIlannaforyfreaineni.
InfiLiofics have a role as snfi-inIlannafory agenfs
in Crohn's disease. Iggressive nufrifional suorf
[incIudirg fulc Ieeding) is inorfanf Ior grovfh,
LufaIso seensfohaveanfi-inlannaforyeI!ecfsard
synfon confrol inCrohn' sdisease. Coriicosferoids
haveLoflanfi-inIlannaforyandinnunosuressive
eI!ecfs and renain anainsfayoInanagenenf.Iure
innunosuressivesinclude6-nercafourine, aza-
fliorine,cyclosorinI,andneflofrexafe.
P ageneral rule,ilorayischosen ir ar eIlorffo
achieve naxinunsynfonconfrol vifl nininun
side eI!ecfs. s a result, innunosuressive ageofs
are reserved Ior nore severc iIIness, Luf nay Le
necessary fo decrease Iong-fern sferoid use. lev
LioIogic agenfs arc Leing develoed and evaluafed
fhaf are ained at very secilc cononenfs oI fle
inIlannafory cascade. InIixinaois ar exanle oIa
geneficaIly eogineored anf|lody direcfed agai osf
funor necrosisIacforalha, and ifshovsronisein
fhc confroI oIsignilcanfCrohn'sdisease.

KEY LW
M
1. U|cerat|veco||t|sproducesd|ffusesuperhc|a|
co|on|cu| cerat|onandcryptabscesses.|t| nvo|ves
therectum|n9% ofpat|ents,w|th orw|thout
cont|guousextens|onh|gher |nthe co|on.Ulcera-
t|veco||t|sdoesnotaffectthesma| ||ntest|ne.
2. Rad|ograph|cexam|nat|onw|thadoub|ea|r
contrastbar|umenemademonstratesd|ffuse
co|on|c|es|onsand pseudopo|ypformat|on|n
u| cerat|veco|it|s.
J. U|cerat|ve co||t|s p|acesthe ch||d ath| gh r|skfor
thedeve|opmentof co|oncancer.
. Thepatho|ogyofCrohn's d|sease|nvo|vestrans
mura|| nf|ammat|on|nad|scont|nuouspattern,
wh|chresu|ts|nsk|p|es|ons.Crohn`sdiseasemay
| nvo|veanypartofthegastro|ntest|na|tract
mouthtoanus).
S. Rad|ograph| cexam|nat|onw|thadoub|ea|r-
contrastbar|umenema|nCrohn`sd|seasedemon
strates ||ea| and/orco|onic|nvo|vementwith sk|p
|es|ons,recta| spar|ng,segmenta| narrow|ngofthe
||eum (str|ngs|gn),and |ong|tud|na| u|cers.
. Therapyfor| nf|ammatorybowe|d|sease|sa|med
atach|ev|ngmax|mum symptomcontro|w|th
m|n|mum sideeffects.
< CBm M peaex~xueep: HCA o pou. |
>
Because anorexia and increased nufrienf |osses in
fhesfooIareconnoninchiIdrervifhIBI,adeqoafe
caIories and rofeir areessenfiaI. CraI sulenenis,
nasogasfr|c fuoe Ieed|ngs, and, in sone severe cases,
cenfraI venous hyeraIinenfafioo are necessary.
Vifanin and nineraI suIenenfafion, esecialIy
iror, nayLe required.
Iafienfs vifh uIceraiive coIiiis Ior nore fhan
I0 years need annuaI coIonoscoy and rectaI
Liosy Lecausc o| fhe high risk oI colon cancer
deveIonenf.
Surgery is evenfuaIIy reeded in 25% oIaiienfs
viih uIcerafive colifis and 70% oI chiIdren vith
Crohn's disease. Surgery is indicafed in ulcerafive
coIifis vhen fhere is IoIninanf coIifis vifh severe
Llood Ioss or foxic negacolon, infracfaLIe disease
vifh a high-dose sferoidrequirenenf, sferoidfoxic-
ity, grovfh IaiIure, or coIonic dysIasia. Because
uIcerafivecoIifisisresiricfedfofhecoIon,coIecfony
is curafive. Surgery is erlorned in Crohn's disease
vher fhere is henorrhage, oLsfrucfion, erIorafion,
severe hstuIa Iornaf|on, or ureferaI oLsirucfion. In
general , conservaiive nanagenenf is varranfed
Lecause renovaI oIfhe diseased LoveI is nof cura-
five in Crohn's disease. Recurrence rafes oI u fo
50% have Leen reorfed alier segnenfaI resecfion.
Sfrucfural Lirih doIecfs are cafegorized as ninor or
na]or. Minor Lirfh deIecfs such as skin fags, inner
eicanfhaI loIds, andrudinenfaryolydacfyIy are ol
Iiffehysiologicsignilcance.IroxinafeIy I5% ol
nevLorn inlanfs have af leasf one ninor anonaIy,
0. 5%olinIanfshavefhreeornoreninor anonaIies.
Ir confrasf, oajor Liril delecfs such as cIeIt aIaie,
nyeIoneningocele,andcongenifaIhearidiseasehave
an adverse eI!ecfor fhe inIanf. Major lirfh deIecfs
occur in 2% fo J% oI aIlnevLorns. The roLaLiIify
ol having a na]or lirfh delecf increases as fhe
nonLeroIninoranonaliesresenfincreases [1alIe
9-I). Birfh deIecfs can Le caused Lyenvironnenfal
orgeneficIacfors.SoradicdisordersareLirfhdeIecfs
causedLy unknovn Iacfors.
LPNHLPLPPL PLLHb
LnvironnenfaI lacfors are knovn fo cause af Ieast
I0% ol aII lirfh deIecfs. cralOgcn8 are environ-
nenfaI agenfs fhaf cause congenifaI deveIonenfaI
anonaIies Ly inferlering vifh enLryonic or IefaI
organogenesis or grovfh. Lxosure fo a ferafoger
LeIore inIanfafion [days 7 fo I0 osfconcefior)
caneitherhavc no eIlecf orcanresuItin loss oIihe
enLryo.To disruf organogenesis, a ferafogen nust
Le resenf LeIore | 2 veeks' gesfafion. Any ferafo-
genic exosure aIter I2 veeks' gesfation redoni-
nanfly allecfs grovfh and cenfraI rervous sysfen
deveIonenf
Terafogens include inirauierine inlecfions
[Chafer lJ), high-dose radiafion, naferraI nefa-
LoIic disorders [Chafer IJ), nechanicaI Iorces, and
drugs.The nosi connon nafernaI nefaLoIic disor
der fhaf has ierafogenic ofenfiaI is diaLefes neIli-
fus I0% olinlanfs oIdiaLefic noflers have aLirih
delecf.ALnornal infrauferine lorces such as oferine
hLroids or oIigohydrannios nay cause lefal con-
sirainf, resuIfing in cIuL Ioof or hi dysIasia.TaLle
9-2 Iisfs fhe nosf connon ferafogenic drugs and
ileir eI!ecfs.

KEY LW
M
1 . Env|ronmenta|factorscause1 V of b|rth defects.
2. |nfect|ousagents,h|ghdoserad|at|on,materna|
metabo||cd|sorders,mechan|ca|forces,anddrugs
cana| | serveasteratogens.
J. Ateratogen|c exposure before 12 weeks`gesta-
t|onaflectsorganogenes|sandt|ssue morpho
genes|s,whereasanexposurethereafterretards
feta|growthand centra| nervoussystem
deve|opment.
LLPLL PLLHb
Cenefic disorders can Le cIassiled as disorders oI
singIe genes, chronosones, arenfaI inrinfing,
and noIecular cyiogenics Advances in noIecuIar
geneiics lave Llurred fle disfincfion anong fhese
cafegories.
bPLLLLLPL LbLHLLHb
lornaIhunancelIs have46chronosones [22airs
oIaufosonesand l airoIsex chronosones) .Chro-
nosones confair geres, vhich occur ir airs af a
singIe Iocus or sife on secihc chronosones.These
aired genes, caIIed allclcs, defernine fhe genofye
nor tor sale ! > < ue pn n]opa ! >
V ||uopr|nlO |ed|atr|cs
oIar individuaI affhafIocus. IIfhe genes af a se-
ciIc Iocus are identicaI, the individuaI is homozy
gou8; iI fhey are dinerenf, fhe individuaI is
hclcrozygOu8. Morefhan J000 diIlerenfsingIe-gene
disorders have Leer descriLed and are cIassiled oy
fheir node oI inherifance [aufosonaI doninanf,
aufosonaI tecessive, orX-linked).
PU!O5ODB LODDBD! L5OOO5
IufosonaI doninanf disorders are exressed aIter
aIterafionoIonIyone gene infheair[usualIycoding
Ior a sfrucfuraI rofein) . Ionozygous disease sfafes
1HLL ~1
Cl0eCe ol Njot PomleS l the teSeCe ol
Nlof PomleS
NumDef 0 Nn0f
Pn0maeS

1
/
3
t 1HLL ~Z
lnc0ence 0 Naj0r Pn0maeS
|%I
<1
1
3
2
Lommo 1ettogelC LtugS
Oru@ HeSu1S
oI aufosonaI doninanf disorders are rare and are
usuaIIy severe or IethaI. nutant gene usualIy is
inherifed Iron one arenfviih ihe sane condifion.
TheriskIortheaIlecfedarenfs'oIlsringis50%Ior
each regnancy. Sonefines an individuaI is fhe Irsf
ersor in a laniIy fo dislay a fraif due fo sonfa-
neous nufafion.Vhen a sonfaneousnufaiion has
occurredinaIefus, fheriskoIrecurrenceinasuLse-
quenf regnancy is fhe sane as ihe chance ol fhe
sonfaneousnufafionoccurringdenovo.IufosonaI
doninanfgeneso|ter causecondifionsfhafnaniIesf
fhenseIves vith varying degrees oIseverify anong
allecfed individuaIs, a henoneror knovn as var-
aDlc cXrc88vly or varaDlc LelaLe+ TaLIe 9-J
Iisfs sone olfhenosi inorfanf aufosonaI doni-
nanf diseases. Cfher chafers discuss sone oIfhese
diseasesindefaiI.
PU!O5ODB HOCO55VO L5OOO5
IufosonaI recessive disorders are exressed aIier
aIferafionoILofhfhe nafernaIandafernaIgenesol
a geneair[usualIycodingIoranenzyne) . Because
haIIolfhenornalerzyneaciivifyisadequafeunder
nosf circunsfances, a erson vifh onIyone nufanf
gere is nof allecfed, vhereas individuaIs vho are
Warfar|nCoumad|n)
|thano|
ypop|ast|cnasa| br|dge,chondrodysp|as|apunctata
|sotret|no|nAccutane)
||th|um
Pen|c| | | am|ne
Phenyto|nD| |ant|n)
Rad|oact|ve|od|ne
D|ethy|st||bestro|
Streptomyc|n
Testosterone| | kedrugs
Tetracyc||ne
Tha||dom|de
Tr|methad|one
Va|proate
LIL, CDgCtt Cft C8C8C.
|eta|a|coho|syndrome,m|crocepha|y,CD septa|defects,patentductusarter|osus)
Fac|a|andearanoma||es,congen|ta| heartd|sease
CDEbste|nsanoma|y,atr|a|septa|defect)
Cut|s |axa syndrome
ypop|ast|cna||s,|ntrauter|negrowth retardat|on,c| eft | | pand pa|ate
Congen|ta|go|ter,hypothyro|d|sm
Vag|na|adenocarc|nomadur|ngado|escence
Deafness
V|r|||zat|onoffema|e
Denta| ename| hypop|as|a,a| tered bonegrowth
Phocome||a,CD tetra|ogyof|a||ot,septa| defects)
Typ|ca|fac|es,CDtetra|ogyof|a| |ot,transpos|t|onofthegreatarter|eshypop|ast|c
|ehheart)
Sp|nab|hda
not tor sale ! > < ue pn n]opa ! >
< c v pexanx~ouee]cvn: HYCA o ]ou. ]
L|Q\O| / LODO\|C L|SO|OO|S V
M 1PLL d
bXmeS ol PutoSom Lomt LSeSeS
Pu10S0m O0mnn1 OSeSe fe@uenc_ LDf0m0S0me L0mmen1S
PC|ODOlOQ|S| 1 ./b, Q
POU|\ QOyCyS\|C K|OHOy O|SOSO 1 . 1 / 1 OQ
MOlOO|\lyDg|OOOOH 1 . 1 , 1 1 Q
MOlOO|\ly SQ|OlOCy\OS|S 1 . b bQ, 1Q
MUHI|Hg\ODS O|SOSO 1 ./b 4Q
NlH`S SyDOlOO 1 . /, 1`Q
NyO\OH|COyS\|OQ|y 1 :2b, 1 VQ
NOU|OUDlO\OS|S 1 . 3 1 1Q
|lO\ODL OODCODCy 1 . 1 b, /Q
hOI|HOD|S\O 1 . 1 b, 1 3Q
UDOlOUS SC|OlOS|S 1 . 3, VQ, 1 OQ
VODW||ODlDO`S O|SOSO 1 ` 1 1 /Q
Q,5Dft It D CIDmD8DmC, Q,|Dg fn D CIDnD8DnC.
bXmleS ol PutoSoml HeCeSSve LSeSeS
Pu10S0m HeceSSve OSeSe
LODgOD|\| OlOH|
|yQO|QS|
LyS\|CHDlOS|S
L|C\OSOH|
LUC|OlS O|SOSO
|DD\||OQO|yCyS\|C K|OHOy
O|SOSO
||OHy|KO\OHU||
b|CK|OCO|| O|SOSO
ybC|S O|SOSO
W|| SOD`S O|SOSO
feguenc_
1 .b~1 .1 b,,
1 ;1 |HYUQ|KLSK|OS
1 ./ [LUCS|DS)
1 . O,
1 ./b [PS|KOHZ|JOwS)
1 . 1 4,
1 . 1 4,
1 .O/b [Pl|CD POl|CHS)
1 .3 [PS|KOHZ|JOwS)
!!/,
g,$Dft In D CIDmD8DnC, Q,|Dg fn D CIDnD8DnC.
honozygous lor a delective gene have the disorder.
Bofh arenfs ol a chiId vifh an autosonaI recessive
disorder arc usuaIIy heterozygous lorfhafgene, and
each child olsuch a couIe has a 25%riskolinher-
iting ihe disorder.TaLIe 9-4 Iisfs the nore connon
aufosonaIrecessive disorders.
MosfinLornerrorsolnefaLo|isn,viththeexce-
fionolorniihinctranscarlanylase[CTC)delciency,
are aufosonaI recessive disorders. InLorn errors ol
netaLolisn are discussedIaferinfhis chaier.
bV DOw U\\|OHS,QlOX|| |DS|O|\OH|Dg
hOH| CyS\S,|H\|C|H|| HOU|yS
LOHC|OHCyO L1 OS\O|SO |H||D|\Ol,OQ|SOO|COOO
bOO L|Q\O| 1 ,SOOVl|H\SU\OSO| lOCOSS|VO
LOOH\|,C|O|O
POl\|C |OO\ O||\\|OD,\| S\IU|O
NUSCU||wOKDOSS, C|O|C llyI|H|S
bV HOw U\\|ODS,CC U |\SQO\S
MyQOlCOgU|D|OS\IO
bOO L|Q\Ol 1 b
PS||OSQO\S,SO|ZU|OS
bOO L|Q\Ol 1
LDf0m0S0me L0mmen1S
OQ ||OD\| O|gHOS|SQOSS|D|O
1Q bOOL|Q\Ol /
VQ LlDO|yOl\O O\DO||S O|SO|OO|
1Q LySOSO| S\O|gOO|SOlOO|
OQ hOD HO |OQ\|CCyS\S,|yQOl\OHS|OH
1 /Q P|HOC|O OIDO||SO|SO|OO|
1 1 Q bOO L|Q\Ol !
1 `Q LySOSO| S\O|gO O|SO|OOl
1 3Q LOOC\|VO COQQOlOXClO\|OH
A~LDKOO L5OOO5
X-Iinkeddisorders,vhichareusuaIlyrecessive,occur
vhen a nale inherits a nutanfgene onihe Xchro-
nosone lronhisnother.Jhcallecfednale,ferned
hcmzygou8 lor ihe gere, has only a singIe X chro-
nosone and, therelore, a singIe set ol X-Iinked
genes The nother ol the ahecfed individoal |s
heferozygous lor that gene, Lecause she has Loih a
nornaI X chronosone and a nufanf one. She nay
< not tor sale! < ue pn n]opa !
V |lucr|nlO |OO|\l|CS
< c v pexanx~ouee]cvn: HYCA o ]ou. ]
le asynfonafic or denonsfrafemiIdsynfons oI
fhe disorder due fo lyonizafion, rn vhich only one
X chronosone is franscriiionaIIy acfive in each
cell Recurrence risk Ior X-Iinked disorders diIIers
deending on vhich arenf has fhe alnornal gene.
In aIIecfed Iafher viII ass fhe deIeciive X chro-
nosoneonfohisdaughfers,creafingcarriersIorfhe
disorder,hissonsviIInofleaIIecfed.1 nofhervifh
an alnornaI Xchronosoneis acarrier, andfhereis
a 507 chance she viII ass ihe alnornaI chrono-
sone fo her rogeny Iaughfers vho receive fhe
aonornaI X chronosone viII le carriers Ior fhe
disease, andsonsviIIhavefhedisease.TalIe 9-5 Iisfs
fhenosf connonX-Iinked disorders.
KEY LW @
1 . ODQO~QODOOOOC\S lO C|SSHOO Dy \DOl HOOO
O|DDOl\DCO D\OU\OSOH|OOHDD\,U\OSO~
H lOCOSSVO,DO 7~|IDKOO OlSOlOOlS.
2. D U\OSOH OOHDD\O| SOlOOlS,\DO QDODOHO
DODO|DCOHQO1O QODO\lDCO lOSU|\S |D VlBO|O
OXQlOSS|OD O\|OOOOC\VO QODO.
J. LODOS OOOC\|VO DU\OSOHB OOHDD\ OSOlOOlS
\yQCy COOOOlS\lUC\Ul| QlO\ODS,wDOlOS
\DOSO D U\OSOH lOCOSSVOO| SOlOOlS COOOOl
ODZyHOS.
. NOS\ DDOlDOllOlS O HO\OO|SH,w\D \DO DO\OO
OXCOQ\OD OOlD\DDO\lDSClOHySO OOH~
ClODCy,lO U\OSOH lOCOSSlVO O SOlOOfS.
1PLL b
XLlke0 LlSeSeS
ALnKe0 OSeSe
blU\ODgg| ODU| |DO|
L|lOH|CglHU|O\OUS O|SOSO
LO|OlD| |HOHOSS
LUC|PDDO USCU|lOyS\lOQ|y
L| UCOSOOQ|OSQ|\O OO|yOlOgOHSO
OOHC|ODCy
MOOQD|||S PHO b
LOSC|Ny|H SyDOlOO
L|H|\||HO\|HSClDy|SOOOHC|OHCy
fgUPDC_
1 . 1 ,
1 . 1 ,,
1 . 1 ,
1 ` 3O
1 .1 [Pl|CH
PO||CDS)
1 ` 1 ,
1 . 1 ,
ChronosonaI disorders are resonsilIe Ior reg-
nancy Ioss, congenitaI naIIornafion, and nenfaI
refardafion. Ilfhough nore fhan 50% oI hrsf-
irinesfer regnancy Iosses are due fo chronosonaI
inlalances,onIy0. 6%oInevlorninIanfshavechro-
nosonaI alnornaIifies. Mosf chronosonaI deIecfs
arise denovo during ganetogenesis, so fhaf an inIanf
can le conceived vith a chronosonaI alnornaIify
viihouf any rior IaniIy hisfory. ChronosonaI
aonornaIifies can aIso oe assed lron arenffo oII-
sring. !n such cases, fhere is ohen a IaniIy hisfory
oInulfiIe sonfaneous alorfions or a higher fhan
chance Irequency oI chiIdren vifh chronosonal
rollens. Iisorders oI chronosone nunler nay
invoIveaufosonesorsexchronosones.Birfh deIecfs
caused ly autosonaI alnornaIifies are generaIIy
nore severe fhan ihose caused ly sex chronosone
aonornaIifies. luneric deIecfs oI fhe aufosones
incIude frisony oI chronosones Z! , IS, and IJ.
!xanIes oI sex chronosone nunericaI alnor-
naIities are Jurner's syndrone and KIineIeIfer's
syndrone.
lndicafions Ior olfaining chronosonaI sfudies
include conlrnafion oI a susecfed chronosonaI
syndrone,nuIfiIeorgansysfennaIIornafions,sig-
nilcanf deveIonenfaI deIay or nenfaI refardafion
viihouf an aIfernafe exIanafion, shorf sfafure or
exfreneIy delayed nenarche in girIs, inIerfilify or a
hisfory oInuIfiIe sonfaneous alorfions, anligu-
ousgenifaIia, or advancednafernaI age. IefaIkaryo-
tying nay be acconIished throughanniocenfesis
or chorionic viIIus sanIing.
L0mmen1S
PDSPDCO O |HUDOg|ODU|DS, lOCUllPD\ |DOC\|ODS
LOPC\|VO K|||Dg Dy Q|gOCy\OS,lOCUl|OD\ DOC\|OHS
|lOX|| USC|OwOKDOSS,LOwOlS S|gD
LX|OD\|DOUCOO |OO|y\|CDO|
bOO L|Q\Ol 1
|Ul|HOO\DO||SO|SOlOOl,SOU\||\|OD
UlOCyCOO|SO|OOl,|yQOlODO|
< not tor sale ! > < ue pn popa ! >
< c v peaen~ouee]cvn: HYCA o u | >
L|Q\O|V OO\C L|SO|OO|S V7
PU!O5ODB 5ODO5
fSDy 2!
Trisony2! , orIovnsyndrone,isthenosfconnon
aufosonaI chronosonaI alnornaIity in hunans,
vith an incidence oI 1 er 700 Iive lirihs.The risk
oIIovn syndrone increases vith advancing nater-
nalage,itis I u J65Iornothers J5 yearsoIageand
I in50Ior fhose45oroIder.CIchiIdrenvifhIovn
syndrone,95%havefhreenunler2 I chronosones
[47 totaI chronosones), which resuIfs Iron chro-
nosonaI nondisjunction during maternaI neiosis.
IourercenthaveiransIocafionoIafhirdnunler2 I
chronosome to anofher chronosone [46 total
chronosones) . Cne-third oI uansIocafion cases are
IaniIiaI, neaning fhaf one oIfhe arenis has a laI-
anced iransIocafion invoIving one nonler 2! chro-
mosone and anofher chronosone. Cne ercenf oI
chiIdren vith Iovn syndrone have chronosone
nosaicisn, vith some ceIIs having fvo nonler 2 I
chronosones [46 tofaI chronosones) and sone
ceIlshavingihreenunler2 I chronosones [47 total
chronosones).JhenosaicisnresuIfsIronanifotic
division error thaf occurred during enlryonic
develonent.
Comnon dysnorhic IaciaI Ieatures incIude
lrachycehaIy [Ilat occiuf), Ilaf Iacial roIle,
osIanted aIelraI hssores, snaIl ears, Ilat rasaI
lridgevifheicanfhaIIoIds, andasnaIInouih vifh
aroiruding iongue. nonaIies oIihe hand incIude
singIe aInar creases [sinian creases), shorf, lroad
1HLL ~
Key etuteS ol 1|lSomy 130 1|lSomy 18
fSOm 13
hands [|rachydactyly) vifh ar incurved lIth Inger
[cIinodactyIy) and hyoIastic niddle haIanx, and
an excessive ga letveen fhe lrst and second toes
["sandaI sigr") . Cfher Ieatures incIude shoristature,
generalized hyofonia, cardiac deIecfs [endocardiaI
cushiondeIecisandsetaIdeIectsareseenin50% oI
cases), gasiroinfesfinaI anonalies [doodenaI airesia
and Iirschsrung's disease), hyoihyroidisn, and
nenfaI retardation [l range J565) . Leokenia is
20 tines more connon in chiIdren viih frisony
2I than ir the generaI ouIation. Iuring the third
and Iourfh decades, an Izheiner-like denenfia
deveIos. Vifh inroved nedicaI, educafional, and
vocationalnanagenenf, IiIe oxecfancyIorafienfs
vith Iovn syndrone nov exfends veII lnfo
adoIthood.
fSDy I8
Trisony I S occurs in I er c Iive lirfhs. !ighfy
jercenf oIcases resuIt |ron neiofic nondisj orctior
andare associated vithadvanced naternaI age.The
renaining 20% nay |e arfial [invoIving only a
oriion oI fhe chronosone) or nosaic, caosed ly
nitofic nondis] oncfion infhe zygofe. Chronosone
transIocafionasfhecaoseoItrisony I S isexfrenely
rare, and ifs resence shooIdromf karyoiying oI
fhe arenfs to excIude an inherifed deIecf. CIinicaI
naniIestationsoItrisony ISareshovninTalIe9-6.
The rognosis Ior atierts vifh irisony I S is
exirenelyoor.J0%die leIorereaching I nonfhoI
age, and 90% dicly I year oIage.
fSOmy 1B
MOO DO HOCK N|ClOCOQ|yw|\| SOQ|Dg OlO|OO
LU\|S QS| OSCQ
N|ClOQ|\||
||OH|DOD\ OCC|QU\
Nl|Ow D|lOD\ O|O\O|OO|O|OO
LOwSO\,O|OO O|S
L|OS\ HO DOOOH
LX\lO\|OS
L\|Ol
LO\ |Q DO Q\O
LODgOH|\ |O|\ O|SOSO [VbL,PbL, |LP)
LQ|OCO|O
LODC|OO |DOS w\| OVO|QQ|Hg HHgOlS
|OyOC\yy
|OyCyS\|C K|OHOy OlO\|Ol lOH OOOC\S
L|yQ\OlC||O|S
PgODOS|SOCO|QUSC|OSU
PbL, tI| 8Ct| CCCCt, LP, tCt CuCtu8 ItCf|O8u8, VbL, vCtfCuf 8Ct CCCCt.
N|ClOgD\||
LOHgOH|\ |O|\ O|SOSO [VbL,PbL,|LP)
b|OI\ S\OlHU
LODC|OO |HOSw|\| OVO|QQ|Hg HHgOlS
hOCKOlDO\\O OO\
MOlSOSO6 K|ODOy
LCKOSUDCU\HOOUS\
9B
nottor ! >

< ue JYH n]o) ! >


< cm v pexanx~ouee]cvn: HYCA o pou. ]
l OI
fSDy !3
Trisony IJ occurs H er I 0,000 Iive lirtls lut
constitutes I % oI aII sortaneous aloriions. -
roxinateIy 75%oIsurvivingcases are tle resuItoI
neiotic nondisj unction and are associated vitl
advanced naternaI age. Tle risk vitl advanced
naterraIageisnucl IesstlatIortrisony2 I . Tventy
ercent oIcliIdren vitl trisony I J lave 46clro-
nosonesvitlairansIocationoIatlirdclronosone
I J to anotler clronosone. Cne-Iourtl ol trarsIo-
cation cases are IaniIiaI, neaning tlat onc ol tle
arents las a laIanced translocation involving one
clronosone I 3 and anotler clronosone. Tle
renaining 5% oI clildren vitl trisony I 3 lave
nosaicisn, sone ceIIs lave 46 clronosones vitl
tvo nunler I 3 clronosones, and sone ceIls lave
47 clronosones vitl tlree nunler I 3 clrono-
sones.Tlenosaicisnresultslronanitoiicdivision
errortlat occurred durin enlryonic deveIonert.
CIinicaI nanilestations oltrisony I 3 are slovn in
TalIe 9-6. Prognosis Ior atienis vitl ir|sony ! 3 is
extrene|yoor. 50%die leIore reacling ! nortl oI
age, and 907 die ly yesroIage.
bOx LDODO5ODO PODODBO5
SexclronosoneanonaIiesinvoIveaonornaIitiesir
tlenunlerorstructureoItleX orYclronosones
orlotl
UHPf! byO0fOP
Turner's syndrone occurs ir I er 5000 live lirtls.
roxinateIy 9S% ol Ietuses vitl Turner's syn-
drone exire ir utero, onIy 2% are lorn.dvancing
naternsI age does not increase tle risk, lecause
Turner'ssyndrone is causedlydeIective enlryonic
ceIldivisiontlatoccursaIterIeriiIization.TlereIore,
tle recurrenceriskIorarenis vlo haveacliIdvitl
Turner's syndrone is ro ligler tlan tlat oI ile
general ouIation.
SeveraI genotyes car cause tle Turner's leno-
tye In 60% oI cases, tle karyotye is 45, XC, in
vlicl tle IenaIe Iacks an ` clronosone.notler
I 5% ol individuals arc nosaics viil a genotye
oI 45, XC/46,XX 45, XC/46, XX/47,XXX or
45,XC/46,XY. Mosaic individuaIs nay lave Iever
lysicaI stignata oI Turner's syndrone. In ile
renaining 25% oI cases ilere are tvo X clrono-
sones luttle slort [) arn oIore oltle X clro-
nosonesis nissing.
C| | n| ca| Man|festat|ons
Iysnorlic Ieatures incIude lynledena oI tle
lands andIeet, aslield-slaed clest,videIysaced
lyolastic niIes, a veloed neck, Iov lairlire,
culitus valgus [increased carrying angIe), slort
stature, and nuItiIe pignented nevi. dditioraI
alnornaIities incIude gonadaI dysgenesis, gonado-
llasiona, renaI anonaIies, congenitaI leart disease,
autoinnune tlyroiditis, and Iearning disaliIities.
ConadaI dysgenesis, resent in ! 00% ol atients,
is associated viil rinary anenorrlea and lack
olulertaI deveIonentdueto loss oIovarianlor-
nones. Tle gonads are aroriately inIantiIe at
lirtl lut regress durirg clildlood and lecone
"streak"ovarieslyulerty.InnosaicsvitlaYclro-
nosoneinonc olileirceIIIines,gonadolIastonais
connon. TlereIore, rolyIactic gonadectony |s
necessaryin tlese atients. Renal aronaIies, usuaIIy
duIicated colIecting systen or lorsesloe kidney,
occur ir 40% oltlosevitlTurner'ssyndrone. Con-
genitaI leart disease occurs in 20% oIatients, and
connon deIects incIude coarctation oI tle aorta,
aorticstenosis, sndlicusid sortic vaIve.s sconse-
quence oI laving onIy one IunctionaI X clrono-
sone, IenaIes vitl Jurner's syndrone dislay tle
saneIrequencyoIsex-Iinkeddisordersas naIes.Tle
diagnosis is nade ly karyotye. Because oI tleir
nosaicisn, sone girIs susected oIlaving Turrer's
syndronelavea46,XXkaryotye intleerileraI
lIood, ard a skin liosy viII le necessary to nake
tle diagnosis.
Slort stature las leen successIuIly treated using
lunan grovtl lornone. Secondary sexuaI clarac-
teristics deveIo aIter esirogen and rogesterone
adniristration.P nentionedearIiergonadectonyis
indicated in nosaics vitl aY clronosone ceII |ine.
Vitl tle rare excetion oI a lev nosaics, vonen
vitl Turner'ssyndronecannotlecone regnant.
hOPPlPfS byO0fOP
KIineIeIter's syndrone, caused ly an exira X clro-
nosone, aIlects in I 000 nevlorr nales, 20% oI
asenc sduIt nen, and I in 250nenover 6Ieet
taIl . Tle karyotye is XXY ir S0% oI cases and
nosaic [XY/XXY in 20%. Recurrence risk is tle
sane as tle initiaI riskintlegeneral ouIatior.
C||n|ca| Man|festat|ons
Tle lysicaI stignata oIKIineIeIter's syrdrone are
not olvious untiI ulerty, at vlicl tine naIes are
inconIeteIynasculinized.Tley lave aIenaIelody
lalitusvitldecreasedoodylair, gyneconastia, and
< not tor sale! > < ue pn n]opa ! >
< c v pexanx~ouee]cvn: HYCA o ]ou. ]
L|Q\O| V / LODO\|C L|SO|OOlS 99
snalI laIIus and testes. InIertiIiiy results Iron
lyoserniaorasernia.IlectednaIesare usuaIIy
talIertlanaveragerelativetot heirlaniIies,and tleir
arn sjan can le greater tlan tleir leiglt.Tlere is
anincreasedincidence oIIearningdiIlcuIties,luttle
average Iis9S. ConadotroinIeveIsareusuaIIyeIe
vated because oIinadequate testosterone IeveIs.
Testosterone tleray durirg adoIescence nay
inrovesecordarysexuaIclaracteristicsandrevent
gyneconastia.
PHLPPL HPPL
LbLHLLHb
mrnlng relers to diIlerent lenotyes resulting
Iron tle sane genotye, deending on vletler a
nutation-narkedclronosoneis inleritedIrontle
notler or Iatler. 1narcnlal U8ouy is tle tern
used vlen lotl clronosones oI a air lave oeen
inlerited Iron only one arent. Prader-ViIIi and
ngeInan's syndrones are exanIes oI inrinting,
and sone cases are aIso exanIes oI uniarental
disony.
BOOV byDOODO
Prader-ViIIisyndrone occurs |r I er I 5,000 rev-
lorrs and is associated vitl an rnterstitiaI deIetion
oI tle Iong arn oI clronosone I 5 [deIetior oI
I 5qI I I J). roxinately 70% ol t|ose aI|ected
lave a clronosone deIetion in tle aternalIy
derivedclronosone I 5 andanornaInaternaIclro-
nosone I 5. Tle renaining J07 lavc a nornal-
aearingclronosoneconIenentvit|tvocoies
oInaternal clronosone I 5. Tlis is krovn as un-
artnlal malcrnal U8omy andthesyndroncresuIts
Irontle IackolaaternaI coyolclronosone I 5.
Recurrence risk ls I lr I 00, unless tle clronosone
! 5 deIetion resuIts Iron a arental transIocatior,
vlicl is extreneIy rare.
LOC0 %0OPSl0lDOS
Iysnorlisns incIude narrov bilrontal dianeter,
aInond-slaed eyes, a dovn-turned noutl, ard
snall lands and Ieet. Slort stature and lyo-
gonadotroiclyogonadisnvitlsnalIgenitaliaand
inconlete ulerty are seen. Tlese clildren suIler
Iron severe lyotonia, vlicl is associated vitl
IeedingdiIlcuItiesandIaiIuretotlriveinirIancy.By
severaIyearsoIage,ilesecliIdrendeveIoar uncon-
troIlabIeaetitetlatIeadstosevere central olesity.
1lese clildren eat constantIy unIess Iood is Iocked
avay Cbesity-related oostructive sIee anea and
cardioresiratory conIications [ickvickian syn-
drone) nay develo. Tlere is miId nentaI retarda-
tionvitl claracteristic inulse control robIens.
Ior tle average atient, strict dietary control ls
attented lut diIlcult to enIorce ltlougl tlose
allected car Iive nornal hle sans, conIications ol
olesity sucl as olstructive sIee anea and diaoetes
neIIitusoIter Iead to earlier deatl.
PD@ODBD5 byDOODO
roxinateIy 60% oI atierts vitl ngelnan's
syndrone lave a nicrodeIetion on tle naterral
clronosone I 5 [deletioo oI I 5qI I 1J) and a
norna| aternal clronosone I 5. 1le otler 40%
lave tvonornaI coies oIaternaIIy derived clro-
nosone I 5, a lenonenon knovn as unarcnlal
alcrnal U8omy. Tle syndroneresuIts Iron lackol
anaternaI coyoIclronosone I 5.
LOC0 %0OPSl0lDOS
Iysnorlisnsseeninngelnan'ssyndroneincIude
naxilIary lyoIasia, Iarge noutl, rognat|isn, and
slortstature.PatientsaresevereIynertalIyretarded,
vitl inaired or alsent seecl and iraroriate
aroxysns oIlauglter.1erky arn movenents, ataxic
gait, ardtitoevaIkresuItin narionette-Iike nove-
nents, Ieadirg to its designation as tle "lay
uet"syndrone. Maoy atients lave seizures.
LLLLLLPH LXLLLPL
LbLHLLHb
B@O A byDOODO
FragiIe X, an X-Iinked lorn oI nentaI retardation
tlat occurs ir I in I 000 naIes, is tle lrst exanIe
oIatrinucIeotide reeat disorder.Tlegere invoIved,
caIled FMR- I , isactivein orainandsern. InrornaI
individuaIs, t|e INtrinucleotide CCC isreeated
alout J0 tines at tle start oI tlis gene. Tlose
allected vitl lragiIc X lavc over 200 CCC reeats.
Tle disorder receiveditsnane lecause acytogenet-
icaIIy detectalIe lreakae occurs at a secilc IragiIe
site ort|eX clronosone. Currerty, Sout|ernllot
anaIysis and oIynerase chain reaction [PCR) are
usedtodeterninetlenunlerolCCCreeats.C|in-
icaInariIestationsnayincIudenacrosoniaatlirtl,
< not toz sale ! > < ue pn n]opa !
< c v peaen~rueepn: HCA o Qpu. ] >
100 llucpr|r1O |OO|\||CS
nacroorclidisndoetotesticuIaredena,dysnorlic
lacial Ieatures [Iarge ]av and large ears), ersevera-
tiveseecl, andnental retardation [90% oIaIIected
naIes have an I letveen 20 and49) . Sone naIes
vitl IragiIe X syndronc lave nentaI retardation as
tle solc nanilestation. FenaIe carriers oltle Iragile
X clronosone nay lave a sulnornal I. utisn
occors nore connonIy in cliIdren vitl tle lragiIe
XclronosonetlanintlegeneraIoulation.Jlere
isnoueatnentIor tlesyndrone.
LDODO5ODO 22Q LOO!OD
byDOODO5
MicrodeIetion oI22qI I . 2lasleen Iound in 90% oI
cliIdrenvitl IiCeorge'ssyndrone, in 70% oIcliI-
dren vitl veIocardiolacial syndrone, and in I5% ol
cliIdren vitl isoIated conotroncaI cardiac deIects.
Itlougl tle nanes oI tle alove-nentioned syn-
drones are stilI in use, tle nore general tern
ZZQ 11.Z UclclOn 8ynUrOmc norc aroriateIy
enconassestlesectronoIalnornalitiesIoundin
tlese cliIdren. Its revaIcnce intle generaI ouIa-
tion is I er 4000 Iive lirtls. Tle deletion can le
inlerited [S% to 2S% oI cases), lut nore tyicaIIy
occurs as a de novo event. Iovever, iIa arent las
tle deIetion, tle risk to eacl cliId is 50%. Tle
nicrodeIetion can le detected using Iloorescent in
situlylridization [FISI)ro|es.ClassiccardiacIea-
turesoItlisspectrunoIdisordersincIudeconotrun-
caI deIects sucl as tetraIogy oI IaIlot, interruted
aortic arcl, andvascuIar rings. Ctler connon lnd-
ingsare alsenttlynus, lyocaIcenic lyoaratly-
roidisn, T-ceII nediated innune delciency, and
alate alnornaIities. Tlese cliIdren usuaIIy lave
Ieeding diIlcuIties, cognitive disaliIities, andlelav-
ioraI and seecl disorders.
LmHLLbLPL~LL LbLHLLHb
Sonesyndronesvitlout adetectalIeclronosonaI
alnornaIity lave cIinical Ieatores tlat suggest a
clronosonal disorder. TlesesyndronesoIten enter
intotle diIIerentialdiagnosisoIasusected genetic
disorder. CIRC! is an acronyn Ior a nonrandon
association oI Ieatures incIuding cololona oI tle
retina or iris, heart alnornaIities, atresia oI tle
cloanae, retarded grovtl, genitaI lyoIasia in
nales and ear aonornalities tlat can incIude deaI-
ness.\TFR reIers to tle nonrandon association oI
vertelraI and anal anonaIies, uacleoesolageaI
lstuIa vitl esolageaI atresia, and radiaI or renaI
alnornaIities.!xosuretosignilcantIeveIsolserun
aIcoloI results in a constelIation oIcIinicaI leatores
reIerred to as clal alCOhOl 8ynUrOmc. TyicaI md-
ings incIude slort alelraI lssures, snootl
|iItrun, and tlin uer Ii. Ilected inIants nay
aIso lave lyotonia, oor grovtl, develonentaI
deIay, congenitaI leart disease, andrenal anonaIies.
@ KEY LW
1 . PQQlOXH\Oy bU% OHlS\ \lHOSOlSQOD\DOOUS
DOl\|ODS DVO CDlOHOSOH ODOlH|\|OS.
2. Ul\D OOOC\S CUSOO DyU\OSOH DOH|OSlO
QODOl| | yHOlOSOVOlO\DBD\DOSO CUSOO Oy SOX
CDlOHOSOHO ODOlH|\|OS.
3. |DOC\ODSOlOO\|D|DQ CDlOHOSOH S\UOOS
DC| UOOCODHlH\OD O SUSQOC\OO CDlOHOSOH
SyDOlOO,HU\QOOlQDSyS\OH HOlH\|ODS,
S|QD|HCD\ OOVO|OQHOD\| OOy Ol HOD\| lO\l~
O\OD DO\O\DOlwSO OXQ|DOO,SDOl\ S\\UlO Ol
OX\lOHO|yOO| yOO HODlCDO DQ|l|S,|DOl\||\yOl
D|S\OlyOHU|\Q| OSQOD\DOOUS OOl\ODS,
HDQUOUSQOD\| |,Ol OVDCOO H\OlD| QO.
LPLLL LbLHLLHb
PQQOBCD O NO!BOOlC L5OOO5
Itlouglindividualnetalolicdisordersarerare,coI-
IectiveIytleyare resonsilIe Iorsignilcantnorlid-
ity and nortaIity. !nlorn errors oI netaboIisn are
genetic diseases tlat occur vlen a deIectiverotein
disruts a netaloIic atlvay at a secilc ste. Ire-
cursors and toxic netaloIites oI excess recursors
accunuIate, androducts neededIornornal netal-
oIisn are dehcient. Certain etlnic grous are at
increasedriskIorsecilc netaloIic errors.
CIinicaI resentation and age at onset vary. !rea
cycIe deIects and organic acidenias resent earIy in
liIevitl acutenetaloIicdeconensation.Fattyacid
oxidation and carlolydrate netaloIisn disorders
usuaIIy resent vitl Ietlargy, encelaloatly, and
lyogIycenia aIter Iov carlolydrate intake or
lasting.LysosonaIstoragedisordersareclaracterized
ly rogressive leatonegaIy, sIenonegaIy, and,
occasionalIy, neurologic deterioration. Iindings tlat
slouId increase susicion Ior an inlorn error oI
< not tor sale! > < ue pn ppa ! >
< c v pexanx~ouee]cvn: HYCA o ]ou. ]
neiaoIisn ircIude enesis and acidosis aIterinitia-
iion oI Ieeding, unusual odor oI urine or sveat,
HeaioslcnonegaIy, hyerannonenia, earIy inIani
death, IaiIurc io ihrive, deveIoneniaI regression,
neniaI reiardaiion, ard seizures. SeveraI inoriani
disorders are discussed here.
LBlOODyOlBO NOBOO5D L5OlOOl5
00ClDSP0
CaIaciosenia, ihe nost connon error oIcarlohy-
drat: neia lolisn, P caused ly a dehciency oIthe
enzync gaIaciose-I -hoshaie uridyliranslerase,
resuIiing in inaired conversion oI gaIactose- I -
hoshaic io gIucose-I -hoshaie [vhich can
undergoglycoIysis) .CaIaciosc-1-hoshaieaccunu-
Iaies in ihe Iiver, Iidneys, and lrain. The disorder
occurs in I oI Iive lirihs, and |nheritance is
auiosonal recessive.
Clinical Manifestat|ons
CIinicaI naniIestations are noied viihin aIev days
io veeks aIfer liriH. InitiaI syntons include
eviderce ol liver IaiIure [heaionegaIy, direci
hyerliIiruinenia, disordered coagulaiior), reraI
dysIunction [acidosis, gIycosuria, aniroaciduria),
enesis, anorexia, and oor grovih. Caiaracis nay
dcveIo y 2 noniHs oI age in unireaied chiIdren.
Inlarts viih galacioscnia are ai increased risk ol
:cbercbio co/i sesis. CIder chiIdren have severe
IcarringdisaiIiiies,vhetherorrotiheyveretreaied
ir irIancy. /|ecied IenaIes have a higH inciderce
oI renaiure ovarian Iailure. Ietecting reduced
IeveIs oI eryihrocyie galactose-I -hosHaie uridyI-
irarsIerase is diagnostic. Laoraiory lrdirgs include
a direci HyerliIiruinenia, e|evaied serun anino-
iransIerases, rolonged rothronlin and pariial
ihronlolastir iines, hyoglycenia, and anino-
aciduria. CaIaciose in iHe urine is detected ly a
osiiive reaction |or reducirg sulsiances ard ro
reaction viih glucose oxidasc on urine iesi siris.
1reatment
LIininaiealIIornuIasanJ|oodsconiaininggaIaciose
[incIuding laciose-coniainirg lornuIas and lreasi
nilI).
yCDgPO blDHgP LSP0SPS
Clycoger is a higH|y lranched poIyner ol gIucose
fhat is stored in liver and nuscIe. Clycogen storage
diseases [CSIs) are agrouoIcondiiionsthatresuIt
Iron delciency oI enzynes invoIved in gIycogen
L|QferV / Geretic D|sorders 101
syrthesis or reaIdovn. |ecause nany diI|erent
enzynes arc invoIved in glycogen netaloIisn, ihe
cIinicaI naniIestations ol ihe CSIs arc varialIe.
TyicaI naniIesiaiiors lncIude grovih Iailure,
hepaionegaIy, ard Iasiing hyogIycenia. The nosi
connorCSIsarefyeI, vonCierIe' sdisease,type
II,Ione'sdisease,ardiye\,McIrdIe'sdisease.IlI
are aufosonal recessive disorders. Treatneni is
desigred io revent hyogIycenia vhiIc avoiding
siorage olever nore gIycogen in ihe Iiver.
PDDO PCO NOBOO5D L5OlOOl5
POykPlDOUf0
IherylIeioruria [IKI), ihe nostconnon oIthese
disorders, occurs in I in I 0,000 Iive lirihs. IKI
resuIts hon a delciency oIhenyIalanine hydroxy-
lase, the enzyne ihat converis henyIalanine io
iyrosine.Vith nornaIhenyIaIanine intaIe, atieris
deveIo highserunconcenirationsolioxicneialo-
liiessuch ashenyIaceiic acidand henylIaciic acid.
CI|n|ca|Man|festat|Ons
InIiIe nost anino acid disorders, synions oI
unireaiedIKIdeveIoinchiIdhoodratherihanearIy
inlancy. leuroIogic naniIesiaiiorsincIudenoderaie
toseverenertaIretardaiion,hyerionia,irenors,ard
lehavioral rolIens.Tyrosine is needed IoriHe ro-
ductionolnelanin,soihclIocIin tHe conversion oI
henyIaIanineioiyrosincresuIisinIighiconIexion.
The atieni'surinesneIIsnouse-Iike.
1reaIment
Ireveniion oIneniaIretardationinIKI isachieved
lyearIyard liIeIongdieiaryresiriciionoIhenyIaIa-
nine. Most siaies include IKL deiection on iheir
nandaiory neoraiaI screens. Iregnani vonen viih
IKI vho do noi restrici herylaIanine intaIe dra-
naticaIIy ircrease ihe risI oI having a chiId viih
nicrocehaly, nerial reiardaiior, and corgeniiaI
heart disease.
HDDCySlOUf0
|onocysiinuria is caused ly a deIeci in ihe anmo
acidneialoIicaihvayihaiconverfsneiHionineio
cysieine anJ serire. The inciderce oIihc cystaihio-
nine synihase delciency is I in I 00,000 livclirihs.
The neonaiaI screen used ly nosi siaies deiecis
increased neihionircIeveIs ir ihe lIood.
Clin|cal Man|festat|ons
There are rosynions ininIarcy. CIinicaInaniIes-
iaiions olservedduringchiIdhoodinclude aMarIan's
< not tor sale ! > < ue pn ppa ! >
< cau v pexann~oueepn: HYCM o ]ou. g >
1U2 |luopr| nlO OO\|CS
lody haliius [Iong ihin linls and digiis, scoIiosis,
sfeoaI deIonnities, andosteoorosis),disIocatedeye
Ienses, niIdtonoderatenenialretardation, andvas-
cuIar ihronloses ihat resuIf in chiIdhood stroIe or
nyocardial inIarction.
TreatmCnt
Iieiary nanagenent is exirenely dilLcuIi lecause
restriction oIsuIIhydryI grous Ieads to averyIov-
rotein, IouI-tasting diet. IroxinaieIy 50% oI
atienis resondto Iarge doses oIyridoxine.
LHlOP f0OSC0fD0y0SP LP0CPOCy
CniihinetranscarlanyIase[CTC)delciency, aurea
cycIedeIect,isoneoliheIevinlonerrorsoInetal-
oIisnvith'-IinIedinheritance.Ininoacidcaialo-
IisnroducesIieeannoniathatisdetoxilediourea
throughaseriesoIreactionsInovnastheurca CyClc.
In ihe urea cycIe, oniihine joins vith car-
lanoyIhoshaie ihrough the action oI CJC to
Iorn citrulIineviihinihe niiochondria.VhenCJC
IeveIs are less than 20% oI nonnaI, ihc nitrogen-
coniaining noiety in ooiihine cannot le quicIly
converted to urea Ior excretior and, instead, Ions
annonia, vhich resuIts in severe hyerannonenia
vheniheatientconsunes roiein. MiIderIornsoI
ihe condition are seen in heierozygous IenaIes and
in sone aI|ectednaIes,
Cl | n|cal Man|festat|ons
Vithin21to4ShoursaIeriheinitiation oIrotein-
containing Ieedings, the nevlon lecones rogres-
siveIyIethargicandnaydeveIo cona orseizures as
ihe serun annonia IeveI rises. IenaIecarriers nay
deveIo headaches and enesis aher roiein neals
and naniIest nenial retardation and Iearning dis-
aliIities Iiagnosis isnadelyneasuringiheIeveIoI
orotic acid, a ly-roducf oI carlanoyIhoshaie
neialoIisn, inihe urine.
Treatment
Treatnent centers onanextrenely Iov-rotein diet
and ihe exIoiiation oI aliernative aihvays Ior
nitrogenexcretion using lenzoic acid and henylac-
etate. EarIy intervention nay nininize deleterious
eI|ecis, lut nanagenent is conIex and extreneIy
diILcuIt lor arenis to nainiain
Ly5O5ODB bOlB@O L5OlOOl5
IelciencyoIaIysosonaIenzynecausesitssulstrate
to accunuIaie in Iysosones oItissues ihat degrade
ii, creating a characteristic cIinical icture. These
"storagediseases are cIassiled as nucooIysacchari-
doses [e.g. , IurIer's, Iunter's, and SanhIio's syn-
drones), Iiidoses [e. g., lienann-IicI, Kralle's,
Caucher's, andTay-Sachs diseases), ornucoIiidoses
[e.g., Iucosidosis and nannosidosis), deending on
thenatureoIihestorednaieriaI.
HUfPf! byO0fDP
IelciencyoIo-iduronidascIeadstoaccunuIationoI
ihe dennatan and hearan suIIates in tissues ard
their excretion in urine. JyicaI Ieatures include
coarseIacies,corneaIclouding,exaggeratedIyhosis,
heatosIenonegaIy, unliIicaI henia, and congeni-
taIheari disease. IeveIoneniaIregressionleginsin
ihelrsiyearoIIiIe.MosichiIdrenviihIurIer'ssyn-
drone die inearIyadoIescence.
0UCPf! LSP0SP
Caucher's disease is caused ly deIciency oI the
enzynep-gIucosidase,IeadingtotheaccunuIationoI
gIucocerelroside.The cIassic Iorn does not invoIve
ihecentraInervoussysten.IatientscharacteristicaIIy
haveheaionegaIyandsIenonegaIy.SiorageoIgIu-
cocerelroside in ihe lone narrov Ieads to anenia,
IeuIoenia, ihronlocyioenia, and recurreni
eisodes oI lone ain. RadioIogic changes incIude
iheLrlenneyerIlasIshaeoIthedistaIIenur.IIov
enzyne leveI in ihe vhite lIood ceIls conlrns ihe
diagnosis. Reconlinani enzyne iheray inroves
nostsyntons.
PPLP
. . . . . . , . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
Inenia, deIned as a henogIolir concentraiion [or
henaiocrit) tvo ornore standard deviations leIov
thc ncanvaIucIoragc and sex, is not a diseasc lut
rathcrasyntonoIanothcrdisordcr.ThehenogIo-
lin concentration is reIatively high in thc ncvlon
luf thcn declines, rcaching a nadir Inovn as the
hysologC anCma oIinIancy. This nadir occurs ai
aroxinaieIy 6veeIs ir the rcnaiure inIani and
2 to J norihs in ihc iern inIant. ThereaItcr, the
henogIolir conceniration riscs graduaIIy during
chiIdhood, reaching aduItvaIues aItcruleriy.
LOlODB LB@DO55
Inenia results Iron decreased rcd ceIl roducfion,
incrcascd red ceII destruction, or lIood |oss. Ic-
creascdrcdceIIroductionisduciociiherdeIcicncy
oIhcnatooietic recursors orloncnanovIaiIure,
and increascd red ceII destruction rcsuIis lron
hcnoIyiic diseasc, vhich nay lc duc to extracor-
uscuIar orintracoruscuIar deIccts. BIood Ioss nay
le acuic or chronic. TalIe 0- outIines ihe nost
connon causcs oIanenia.
ThcadjusiedreticuIocyiccounf [IRC) isusedto
detcrnine vheihcr thcrc has lccn an adequatc
eryfhrooietic resonsc to the givcn ancnia. Jhc
IRC is caIcuIaied as IoIIovs.
McasuredIenatocrif
IRC

xcctedIenatocrit
? ReticuIocyieCount
In IRC Icss ihan 2 signiIes incIlcctive eryihro-
oicsis. In IRC grcater ihan 2 signiIes cIcctivc
eryihrooiesis,suggesiinghenoIysisorchroniclIood
Ioss.
LDCB BDO5BOD5
HSlDfy
In fIc young inIani, erinaial history nay reveal
ivir-io-tvinorIeionaieraIiransIsion.IniheoIder
child, thcdietaryhistorynaysuggcstrisk|aciors Ior
iron, vitanin B,,, or IoIatc dcIcicncy anenia. Boih
iron dcIcicncy ancnia andIeadoisoningcannan-
iIestasica.SignsoIoverioroccuItlleedingircIude
neIena, henaiochezia, henaturia, henaienesis,
anornaInenses,oreistaxis.Theatienis' race/cth-
nicity and IaniIy history oI sIencctony or cholc-
cystecfony suggesf an inheriied hcnoIyiic ancnia.
Medicationscan causc eitherLoncnarrovsurcs-
sionorhcnoIysis.CthcrqucstionsshouIdattentto
eIicit a history oI Iever, veight |oss, Iafigue, rash,
lruising,jaundice, andcough.
ySC0 LX0O0lDO
LxanincthcaticnttoasscssthescvcrityoIanenia.
!noriantIndings include alIor [sIin, conjunctiva,
nucosa) anJ Ioss oI aInar creasc igneniation.
Conaring ihe conIexion oI thc afient and
arents is aIso useIul. Tachycardia and osturaI
changcs in heart rate and lIood ressurc arc
sccn vith acute lIood Ioss. Cthcr Indings nay
rovidc cvidence oI congcstivc hcart IaiIure
[heaiosIenonegaIy,Ioverexircnitycdena,tachy-
cardia), ancyioenia [etechiae, urura), Iood
Ioss [osifive stooI guaiac or gasirocculi, gross
henaiuria), henoIysis [scIeraI ictcrus, jaundice,
urolilinogen in ihe urine), or inIItrative disorders
1HLL 1 1
Letetl LlgoSlS ol Lommo PemlS
LeHe0 by Ne LotuSCut Voume
Pnem
Ncf0ctc PnemS
Ncf0ctc PnemS
NOg|OD|S\|C
NOHOg|OD|S\|C
N0fm0ctc PnemS
| H|Ol|\OO |OO|y\|C
HO|S
PCQU|lOO |OO|y\|C
HOS
L|fOHC H\\OH'
PCU\OD|OOO|OSS
bQ|OH|CSOQUOS\l\|OH
L|lOH|C lOH| O|SOSO
Oefent Ogn0SS
| lOH OODC|OHCy
bOVOfO |OOQO|SOH|Hg
||SSOSyHOlOOS
bOOfODS\CHO
V|\|Hb_OODC|OHCy
|O|\OOODC|OHCy
LfO\|CC|OUl|
PQ|S\|CHO|
LOHOb|CKH HO|
bOHOffOw HD\f\OH
MyQO\|ylO|O|S
L|VOlO|SOS6
PDHOf| |OOg|OD|HS
b|CK|OCO||O|SOSO
hOO D|OOOCO||OHZyO
OSOlOO|
LO|LOOUC|OHCy
|ylUV\O K|HSO
OODC|OHCy
hO DOOO CO ODfHO
O|SOlOElS
MOlOO|\ly SQ|OlOCy\OS|S,
O| | |Q\OCy\OS|S
|lOXyS| HOC\UlH
|OOg|OD|HUl|
PH\|DOOyOO|\OO
HOS
PU\O|UHO|OO|y\|C
HOS
|SO|UHO |OOy\|C
HO|S
N|ClOHg|OQ\|C OO|y\|C
HO|S
MOOy\|CUlO|C
SyHOfOO
L|SSO|H\OO |H\fVSCU|l
COgU|\|OH
Ol Cm8 O! ClOItC !|nnttO, 1DlC OfnOCyttC U ZD
lC ntClOCytC.
< c v peann~xounepmn: HCA o pou. p >
[Iynhadenoathy, heatosIcnoncgaIy) . lI thc
chiId has oor veight gain, consider ancnia
oI chronic diseasc. IhysicaI Indings that suggcst
a seciIc cause oI ancnia are Iound in TalIc
I 0-2.
LB@DO5C LVB UBOD
Tlc goaI oI tcsting is to dctcrnine vhethcr the
aneniarcsuItsIron dccreasedroduction,increased
destruction, or lIood Ioss. !nitial Ialoratory iesis
ncededtocvaIuatcanenia incIudc aconlctcllood
count vith nanuaI diIlercntiaI and red llood ccII
indiccs, reticuIocyic count, and criheraI lIood
sncar.
The ncan coruscular voIune [MC\) and ad-
justedrcticuIocyiccountcategorizethedisordcrinto
anicrocytic,nornocytic,ornacrocyiicancnia,viil
adcquatc or inadcquatc rcd lIood ceII roduction.
IeriheraI lIoodsnearis uscdto assessile rcd ard
vhitc lIood cclI norhoIogy, and the IatcIet
nunler and sizc. lIhenoIysis is susccted, consider
eIectroIyies, Iactatc dchydrogenase, liIirulin,
Coonls' test [indirect and direct), and scrun ha-
togIolin. IroliIinogen nayle detcctcd on urinaIy-
sis. I glucosc-6-loshatc dehydrogenasc [C6II)
assay shouId lc considercd in IIican-Incrican
and Mcditerrancan ouIations vho resent vith
henoIytic anenia. IerIorn hcnogIolin cIcc-
trohorcsis io diagnosc susected henogIo-
linoailics. lIirondcIciencyancnia is high on thc
diIlcrentiaI, scrun iron lcveI, totaI iron linding
caacity, and serun Ierritin leveI arc rcodcd Ior
anaIysis. I lcad IcveI is indicated iI Iead oisoning
is contcnIated. Iree eryihrocyie roioorhyrin
[ILI) IcvcIscanlcoltaincdquicklyandvitlasnaII
anounioIlIood.LIcvatcdILIIeveIssuggesttledis-
ordered hcne incororation seen vith iron deI-
ciency and Iead oisoning. In cIcvated eryihrocyte
sedincniation ratc is secn in ancnia oI cIronic
discase. Iositivc hene tests oIstooI or gastric con-
ients irdicaie gastrointestinaI lIceding. lI a nacro-
cyticancniaisIound,lothvitaninBj_ andredllood
ceIIIoIate IeveIs arenccded.
lOBDOD
Treatncnt varics deending on ihe causc oI
ile ancnia. Incnias that aear nost oIten on
ISMLL cxans arc discussed in ihe IoIloving
scctions.
not tor sale! > < ue pn popa ! >
1HLL 1 Z
< c v pexaex-oueepcvn: o p
LQ\O| 1 |O\OOgy

105
hySlC l0lgS l the bvutlo o Peml
b_S1em
bk|H
|OO
LyOS
LfS
NOU\|
L|OS\
PDOOOH
LX\|O|\|OS
hOC\|
NO|VPS
LDSefv10n
MyQO|Q|gOH\\|OH
LC U ||\SQO\S
V|\|||gO
||\|| OCU|OCU\HOOUS|D|H|S
JUHO|CO
|O\OC||O,QU|QU|
L|y\|O\OUS |S|
bU\IO|y|S|
||OH\| DOSS|Hg
N|C|OCOQ||y
N|C|OQ|\|||
hO\|HOQ\|y
LQ\|C \|OQ|y
b|OCkOO |C||| g|HO
|ySO|||O|SC|O|||Hg
bUOSC|Ol
LODOSS
L| OSS|\|S
PHgU| |S\O\|\|S
L|O\ | |Q
||gOH\\|OH
O|Hg|OC\S|
LOUkOQ|k|
b||O|O C|OS\ O|w|OOSQ|PO H|QQ|OS
NU|U|
MOQ\OOg|y
bQOHOOgy
NOQ||OOg|y
PDSOH\ k|OHOy
PDSOH\\|UDS
||Q||HgO|\|UD
bQOOHH||S
bOU||HO[H||S)
LyS\|OQ||CH||S
MOO|||O|OS
MOOQOS\VOS\OO
| |l|\D|O,Q\|y
|O||Q|O|| HOU|OQ\|y
LOOH\|
P\X|,QOS\O||O|CO|UH S|gHS
b\|OkO
SLL, 8y8tCnC |uu8 Cly!CmtO8u8.
b@nDcnce
|HCOH|`S HO|,OySkO|\OS|S COHgOH|\
|HCOH|`S HO|
V|\| Hb_OOUC|OHCy
L|CO|kM|gS| SyHO|OO
MOO|yS|S
bOHO||Ow |HU|\|\|OH,U\O|UHO|OO|yS|Sw|\| U\O|UHO
\|lODOCy\OQOH|,|OO|y\|C U|O|C SyHO|OO
||VOV||US,LQS\O|Hb||V||US
bLL
||SSO| jO|,SOVO|O ||OHOOUC|OHCy, C||OH|C SUDOU||
|O\O
|HCOH|`S HO|
|HCOH|`S HO|
bICk|OCO|| O|SOSO
LS\OOQO\US|S
LySkO|\OS|SCOHgPH|\
W|| SOH`S O|SOSO
| |OHOOUC|OHCy
LS\OO\fOSS
b_OOUC|OHCy, ||OHOOUC|OHCy
| |OHOOUC|OHCy
L|OHOb|Ck!H SyHO|OO
|OU\ZJOg|O|S SyHO|OO[|H\OS\|H| D|OOO |OSS)
LS|O|WODO|hOHOU SyHO|OO[DOOO |OSS)
LySkO|\OS|SCOHgPH|\
LOHOb|CkH SyHOlOO
LHOOC|O|\|S.Q|OS\|O\|CV|VO |OO|yS|S
MOO|yS|S,|HU\|\|VO\UO|,C||OH|C O|SOSO, |OHg|O,
C|O|OCyS\|\|S
MOO|yS|S,S|Ck|O CP|| O|SOSO,[O||y) \|SSO|, ||,
|yQ|O,LQS\P|Hb||V||US,QO|\| |yQO|\OHS|OH
|HCOH|`SHO|
|HCOH|SHO|
|HCOH|SHO|
L|OHOb|CkH SyHO|OO
||OH OOUC|OHCy
MOVy O\| |H\OX|C\|OH,SOVO|O || | HOSS
LySkOl\OS|S COHgPH|\
|O|\| |yQO|\OHS|OH
|D\OS\H |OOff|BgO
| |OHOOUC|OHCy
LOUC|OHCyOV|\|HSb b,HO L, |OO QO|SOH|Hg
LOUC|OHCyOV|\|HS b_HO L
V|\|H b_ OOUC|OHCy
b|Ck|O CO|| O| SOSO, Q|OXyS| HOC\U|H| |OOg|OD|HU||
< no toz sale! > < ue pn ppa ! >
I U ||uopr|nlO |OO|\||CS
| KEY lLW
1 . Anem|a|SnoIad|seasebuI raIherasymptOmOf
anotherd|sOrder.
2. Anem|aresu|tsfrOmdecreased red ce|| prOduc-
I|On,|ncreasedredce|l destruct|On,Orb|oOd |Oss.
J. The mean cOrpuscu|arvOl umeand adjusIed
reticu|Ocyte cOunt categOr|zethe d|sorderintOa
m|crOcyt|c,nOrmocyt|c,OrmacrOcyt|canem|a,
w|thadequaIeLf |nadequateredb|oodce||
prOduct|on.
NLHLLXL PPLPb Vm
LLLHLPbLL HLL LLLL LLLL
HLLLLLP
|yochronic nicrocytic red lIood ceIIs indicate
inaired syntlesis oI tle hene or gIolin cono-
nents olhenoglolin. IeIective hene synthesis nay
le ile resuIt oI iron deIciency, Iead oisoring,
chronicinIannatorydisease, yridoxinc deIciency,
or coer deIciency. IeIective gIolin synthesis is
characterisiic oI the rhalassenia syndrones. lron
deIciency anenia, ihe ihaIassenia syndrones, and
anenia oI chronic disease are ile nost connon
causes oI hyochronic, nicrocytic anenias. Lead
oisoning, vhich nay cause a niId hyochronic,
nicrocyLic anenia, is discussed ir detail M
Chater 2.
lOD LOCODCy PDODB
lron deIciency, the nost connon cause oIanenia
during childhood, is usuaIIy seer letveen 6 and 24
nonils oIage. |utritionaI iron deIciency deveIos
vhenraidgroviland an exandinglIoodvoIune
ui excessive denands on iron stores. Iietary risk
Iactors incIude extended excIusive lreast |eeding
[nore than 6 nonihs) vithout iron suIenenta-
tion, consuntion oIIov-iron IornuIarearations,
earIy institutior oI lov-iron iron-containing soIids,
excessive vhoIeniIkintake, andile alsence oIiron
suIenents.TheironresentinlreastniIkisnuch
norelioavaiIalIethaniheironincov'sniIk.Iscor-
lic acid enhances the alsortion olnon-hene iron,
vhereas tea decreases its alsortion.
lron deIciency anenia can occur as earIy as J
nonthsoIageintherenaturcinIantvhohasinad-
equateironstores atliril lt car occurir theinlani
ortoddIervho receives adiet excIusiveIyconrised
conosed oI niIkor Iov-iron IornuIa. lutritionaI
iron deIciency can aIso occur during adoIescence
vher araidgrovLhsuricoincidesvithadietviih
sulotinaIironcontent.Thisis aarLicuIa:rolIen
in adoIescent IenaIes lecause oI iron Ioss during
nenses.
lrondehciencycausedlylIoodIosscanaIsooccur
in youngchildren. IrenataI iror Ioss can occur |ron
IetonateuaI transhsionor Iron tvin-to-tvintrans-
Iusion. IerinataI lIeeding nay result Iron olstetric
conIications such as IacentaI alrution or Ia-
centa revia. IostnataI lIood loss nay occur Iron
olvioussourcessuchassurgeryortrauna ornayle
occuIi, asoccursvith idioathic uhonary heno-
siderosis, arasitic inIestations, and inllannatory
loveI disease.
LOC0 %0OCSl0lDOS
Mild iron delciency is usuaIIy asyntonatic. Viih
noderate irondeIciency[henogIolin6Sg/dL),ile
inIantdeveIos anorexia, irritaliIity, aathy, andeasy
IatigaliIity. Cn hysicaI exanination, the anenic
inIant nay have skin and nucous nenlrane aIIor,
gIossiiis, angular stonatitis, and koiIonychia [soon
naiIs). The child nay aIso have tachycardia and a
systoIic ejection nurnur at ile IeIt uer stenaI
lorder.The inIantvith severe anenia [henogIolin
Iess than Jg/d!) viII shovsigns oIcongestive heart
IaiIure, vhich incIude tachycardia, an S, car-
dionegaIy, heatonegaIy, distended neck veins, and
uInonaryraIes
Laloratory Indings tyicaI Ior the nicrocyiic
anenias are Iound inTalIe I 0-J. Bone narrov ex-
anination is noi cIinicaIIy indicated io conlrn ihe
diagnosis, lut vhen erIorned denonstrates
nicronornolIastic hyerIasia oIihe eryihroidIine.
fC0lCOl
MiId io noderate iron deIciency anenia, vithout
evidenceoIcongestiveheariIaiIure,istreatedvithJ
to 6nglgday oIeIeneniaIiron erday.The retic-
uIocyie count viII increase viihin 2 to J days, and
tle henogIolin viII increase at a rate oI aroxi-
naieIy 0. Jg/dL er day. Continue theray Ior S
veeksaIterthe henogIolin has retuned to nornaI
to reIenish tissue stores. lIthe henogIolin has not
increased sulstantiaIIyaIterone I nonthoItheray
and conIiance has leen estalIished, consider
other causes oI hyochronic nicrocyiic anenia.
IIthough inIants can toIerate renarkalIe degrees
oIanenia, esecialIyiIihe decIire in henogIolinis
< not toz sale ! > < ue )7H )B ! >
M 1HLL 1 d
Lbottoty 0gS ht the Lommo Nctocytc PemS
f0n 1DSSOm 1DSSem F umDSm LDf0nc
OeDcenc_ 1ft N_0f OSeSO
hLW T M| 1 1 M|
NLV 1 1 1 1 M| 1
hbL no. 1 M| 1 1 !
|L| T M| M| T! 1
gb A 1
|
-T
|
T M| M|
o-M| o-M|
| ron 1 M| 1 M| i
|bL M|T M| M|1 M| M| 1
V saturat|on 1 M| 1 M| 1
|err|t|n 1 M| T M| M|1
L !lCC Cly!fOCytC fCtOO|yf, gD, CmOgOD, |bL, tOt| fODIUIg CCty; T,tICfC8CU, 1,UCCfC8CU, L, Orm|.
graduaI, inIants vith severe anenia nust le trans-
Iused very sIovIy viil snaIl [J5nL/kg) aIiquots
oI acked red lIood ceIIs to avoid causing cardiac
deconensation.
M
KEY LW
M
1 . lron dehc|encyanem|a,thethalassem|a syn-
dromes,and anem|aofchron|cd|seasearethe
mostcommoncausesofhypochrom|c,m|crocyt|c
anem|as.
2. l rondehc|ency|sbyfarthemostcommoncause
ofanem|adur|ngch||dhood and |smostoften
seen beIween O and24monthsofage.
J. M|l dIomoderate|rondehc|encyanem|a,w|thout
ev|denceofcongest|vehearIfa||ure,|sIreated
w|th d IoOmg/kg/dayofe|emenIa| |ron perday.lf
the hemog|ob|n has noI|ncreased subsIant|ally
after 1 month ofIherapy,oIhercausesof
hypochrom|cm|crocyt|canem|a should be
cons|dered.
PQDB BDO OB DBB55ODB
0lDgCOCSS 0O0 LOC0 %0OCSl0lDOS
The ihaIassenias are hereditary henoIytic anenias
characierizedlydecreasedoralsentsynthesisoIone
ornoregIolinsulunitsoIilchenogIolinnoIecule.
IIlathaIassenia,causedlydeIetionoIoneornore
oItle Iouro-glolin genes, Ieads to reduced synthe-
sis oIo-glolin chains. Beta thaIassenia is caused ly
errors in the transcrition or transIation olp-glolin
nRl/ and Ieads to reduced synthesis oI p-gIolir
chains. Thalassenia syndrones are conared in
Talle I 0-4.
TlenunleroIdeIetedo-glolingenesdeternines
the henatoIogic consequences oIaIha thaIassenia.
Jhese deIetions can le ci: or tnn:. Cis deIetions
occurvhentvoo-gIolingenesaredeIetedlronone
clronosone,vhereastnn:deIetions signi[ asingle
o-glolir gene deIetion or eacl olthe tvo clrono-
sones. IiIlerent races and erhniciiies have varying
ratesoIloihci: andtnndeIetionsoIo-gIolingenes
intheirouIation.Jhis Iacior is discussedIater.
|onozygous aIha tha| assenia, or henogIolin
Bari's disease, occurs vhen alI Iour o-gIolin genes
are deleted. FaiIure io roduce any o-gIolin chains
results in -gIolin tetraner [lenogIolin |ari's).
|enogIolin Bart's has a ligh aIlnity Ior oxygen
and does not reIease it to ihe tissue. The resuIi
ls severe anenia, tissue anoxia, heart IaiIure,
heaiosIenonegaly, generalized edena, and deaih
in utero due to hydros IetaIis. Tle cis deIetion is
nostrevalent in SoutheastIsians.
|enogIolin | disease resulis Iion deIetion oI
three o-gIolin genes. -CIolin chains are onIy ro-
duced in utero. ln nornaI inIants, IetaI henogIolin
[vhich consists oI tvo o-gIolin clains and tvo -
glolin chains) usuaIIy redoninates at liith. In
nevlorn inlants vith henogIolin | disease, ile
dearth oIo-gIolin Ieads to the Iornation oIheno-
glolin |ari's, vhich accounts Ior I 0% to 40% oIthe
totallenogIolin.ViththecessationoI-gIolinsyn-
thesis and ihe orset oI p-gIolin synihesis at lirth,
1HLL 1 9
LOmfSO O the 1hSSem by0fOmeS
Lenetc PDn0fmlt Fefcent HemQ@0Dn LtDef
HD P HD P HD
Norma|g V~Vb /-3 /~3
8etathalassem|as
Thalassem|amajor
ptha|"p-thal" /-b b
pthalptha| Very|ow /~b /b
Tha|assem|a| ntermed|a Cver|apsw|ththa|assem|amajor
(var|ed genet|cg|ob|n
abnormal |t|es)
Tha|assem|am|nor
ppthal"orppthal ~b
A| phatha|assem|as
omozygous
o-tha|assem|a
/
emoglob|nd|sease O~1
- - / - o
A| phatha|assem|am|nor ~b
- o / -o
o ol - -
S|lentcarr|er ~b
o/oo
henogIolin |aris dininishcs and hcnogIolir I
[vhichconsistsoIap-gIolintetraner) redoninates
aIter the rsi Iev nonihs oI IiIe. Icnoglolin I
eventuaIIy accounts Ior J0% io 40% oI ihc totaI
henogIolin, andnornaIhcnogIolin1 accounisIor
aroxinateIy60%io 70%oIihe totaI henogloin.
This diagnosis is nost connon in chiIdrer viih
SoutheastIsiar ancesiry.
IIha thaIassenia trait, aIso krovn as alha iha-
Iasscnianinor,resuItsIrondeIctionoItvoo-gIolir
gencs. This deIect naniIests viih niId anenia,
hyochronia, ard nicrocytosis. /Iha thaIassenia
trait, present in J% oII.S.lIacks, is oItcn corIused
vith nild iron deIciency. Thc henogIolin eIec-
irohorcsisisnornaIinthesechiIdrcn,andihcdiag-
nosis isoncoIexclusionconIrnedly docuncnting
arcntaI nicrocyiosis.
ThoseviihdeIctionoIonlyoreo-gIolingcne are
considcred siIcnt carriers Ior aIha ihaIassenia, as
they have a nornal hcnoglolin conccntration and
nornaI red lIood ceII indiccs.The conditior car le
neasured onIy ly quantitativc neasurencnt oI
glolin chain synihesis orly gere analysis.I carrier
b-1 /1
b (p)
b 8art[j4)
/~b /-b b3-4
/-3 /~3
/-3 /-3
canroduceoIIsringvithalhathaIasscniairaiior
henogIoin I disease.
BciathaIassenia canlc suldividediniohonozy-
gous [eta thalassenia najor) and heterozygous
Iorns [eta thaIassenia ninor) . Beta ihaIassenia
najor results eithcr Iron conIeie alserce oI p-
gIolin synihesis [B0/B0 gcnoiyc) due to deIectivc
transcritior oInRlI orIron artial reduciion oI
gene roduct [B/B genotye) due to iransIatioral
errors. Thc child vith leta thaIassenia ninor, ihc
heterozygous Iorn, has one nornaI p-glolin gene
and one alnornaI p-gIolin genc.
ChiIdrenvithlcta thaIassenia najorhavcsevere
henolyiic arenia and sIononegaIy duringihcIrsi
year oIIiIe. lI urtreaied, lonc narrov hyerIasia
and cxtrancduIlary henatooiesis roduce charac-
ieristic Ieatures such as iover skulI, Irontal lossing,
naxilIary hyertrohy vith roninert cheeklores,
andanoverlite.FaiIuretoihriveisronineni.Ieaih
occurs vithin the IrstIev years oIliIe due to ro-
gressive congestive heartIaiIure iItheationt is noi
suoried vith lood transIusions. Iesiie scvere
anenia,ihoreisreiiculocyioenia, releciingineIIec-
< not tor sale ! > < ue pn popa ! >
tive henaiooiesis. IcrlheraI lIood snear reveaIs
narked nyochronia, nicrocyiosis, anisocyiosis,
and oiIiIocyiosis. Cn henogIolir eIectrohoresis,
henoglolir I is eithor narIedIy docroasod [B/B
orB/B0) orioiaIIyalseni [B0/B0). Cnquaniiiaiive
henogIolir eIecirohoresis, henoglolin F accounis
Ior95% ir ihe B0/B0 genoiye and 20% to S0% in
the B/B geroiype. lIihe diagnosis is in question
orthe chiId's henogIolin electrohoresls is equivo-
ca|, the parenial conIete llood couri, snears,
ard henogIolir eIectrohoresis nay cIari[ the
diagnosis.
Childror vitl leia ihalassenia ninor have onIy
a nild Ienolyiic arenia. Cr lIood snear, ihe
hyochronia, nicrocyiosis, ard anisocyiosis are dis-
roortionately severe given thc degree oI anenia.
|enog|olir eIecirohoresis shovs elevaiion oIihe
henogIolirI,leveI andsoneiinesaniId eIevaiior
olhenoglolir
LQ0PDDgy
Iha ihaIassenia is nosi connor in IIrican,
SouiheasiIsian, Mediterranean, andMiddle Lastern
ouIatiors. JIe nost severe Iorns oI aIha tha-
| assenia, three- and lour-gene deIeiions, are seer ir
the SouiheasiIsiarouIaiionlecauseoIihehigh
revaIence oltlS dclciiors. Beiaihalassenia is nost
oIten Iound in ouIaiions origirating Iron ihe
Mediierrarear, MiddIe Last, and lrdia.
lP0lPOl
Theray lor chiIdrer viih leia thalassenia na]or
consisisoIIrequentackedredlloodceIItransIusions
toanoIioraieihoareniaandrcvonicongesiivehcart
lailure. These chiIdrer require I 0 io 20nIkg oI
IeuIodeleted red l|oodcelIs everyJ to 5 veeIsio
naintainthehenogIolinaloveI 0 g/dL.Jhisreginer
eIininaiesanincreasederyihrooieticdrive,aIloving
nornal Iirear grovth and lore deveIonert. Su-
ression ol eryilrooiesis aIso | inits the stinuIus
Ior ircreased iron alsoriior, vhich heIs io nini-
nizoironovcrload. SIenccionyisconsidoredvhcn
iransIusioraIrequirenenisexceed250nI/Ig/yr.!ror
ovcrIoaddeveIosirchildrenviihleiaiIaIassenia,
vhethertheyarefransIusedor noi, duciohyperal-
sortlon oI dieiary i ror. Vhen ihe lone narrov
storagecaacityIorirorisexceeded,ironaccunulates
in theliver,heart,ancreas,gonads,andsIir,roduc-
irgsynionsolhenochronaiosis.IsaresuIt,nany
ihaIasseni c atienis develo cardionyoaihy and
congestive leart IaiIure lr their Iate teens.To nini-
nize ihe norlidity associaied vith iron overIoad,
aiienis are ireaied viih cIelafing agenis such as
deslerrioxanine. Because oI ihe constant siaie ol
increascderyihrooiesis,IolicacidsuIononiaiionis
reconnendedIoraiienisnofnaintainedonchronic
trarsIusion iheray ir order io reveni loIate del-
ciencyardnegalolIasiicarenia.Bonenarrovtrans-
Iartation is curaiive, luf lecause oliis associaied
norlidifyardnorialiiy, ihisrocedureiserIorned
in onIy a lev certers using |LI-natched silIing
donors.
IrirciIesoIihcrayIorhenogIolinIdisoascarc
iho sane as tIosc lor leta fhalassenia najor. The
need Ior translusior and cheIaiion fheray deends
or iheseverityoIihe arenia.
lo freatnent is necessary Ior aIha or leta
ihaIassenia oinor. Ceneiic courselIing is recon-
nerded.BecausethesncaroIirordeIciencyanenia
ard aIha and leia fhalassenia ninor are quiie
siniIar, thc child viih resuned iron delciercy
anenia vho does not resond to oraI iron iheray
andiseIievedtolcconIiani shouldhave aheno-
gIolir eIecirohoresis to ruIc ouf leta thaIassenia
ninor.Jhe chiId viih aIha ihalassenia traii has a
norna| henoglolin eIectrohoresis [nay have eIe-
vaied henoglolin Bart's as neonaie), vhereas the
eIecirohoresis oI ihe chiId vitl lcia ihaIassenia
niror nay shov an eIevated henogIolin I, and
hcnoglolin I
M
KEY LW
M
1 . Thesever|IyofsympIomsofal pha and betatha
|assem|adependsonthe|evelofO orpgl ob|n
cha|nsynthes|s.
2. Hemogl ob| nH d| seaseandbeIatha|assem|a
majoraretreatedw|thred b|oodce||transfus|ons,
|ron che|at|on,andfolatesupp|ementat|on.A|pha
andbetathalassem|a m|norusuallydo not
requ|retreatmentbuImaybem|stakenfor|ron
dehc|encyanem|a.
PDODB O LDlODC L5OB5O
Inenia oI chronic disease can resuIi Iron chronic
inllannatory diseases, such as inllannatory lovel
disease and juverile rheunatoid arthritis, chronic
inIectiors, such as iulercuIosis, ard naIignancy.
TyicalIy, anenia oI chronic disease is rornocyfic,
25% oI cases ol arenla oI chroric discase have
nicrocytosis.IroniaoIchronicdiseaseresuItsIron
< no tor e! > < ue pn popa ! >
1U |luopr.rlO e|atr|cs
< c v pexaex~ouee]cvn: HYCA o ]ou. ] >
an inaliIityto noliIize ironIronitssiorage sites in
nacrohagcs. The chronic lnllannatory state trig-
gers cyioIines that rcsuIt in reticuIoendothcIiaI
lIocIade viihin ihe narrov.I nodcst decreasc in
thcsurvivaI tine oIred lIood ceIIs and a reIaiiveIy
Iinited eryihrooietin resonse aIso coniriluie to
the anenia.
LOC0 %0OPSl0lDOS
The anenia ls niId in degree [henogIolin SI 0
g/d!) .The Ialoraiory Indings typicaI Ior anenia oI
chronic disease are noied in TalIe I 0-J. P in iron
deIciency ancnia, the seruniron leveI is reduced,
ir contrast to iron deIciency arenia, ihe totaIiron-
linding caacity is Iov, and ihe serun Ierriiin IeveI
is nornaI or increased. Bonc narrov exaniraiion
shovs nicronornolIasiic hyperIasia ard ar in-
creascin storage iron, lui a decreasc in ihe nunler
oIiron-containingeryihrolIasts.
lP0lPOl
Jhe anenia rcsolvcs vhen the underIyingcondition
istreatcdadcquateIy.TherayviihironsuIenenis
is unncccssary unless true iron deIciency is aIso
reseni.
B
KEY LW
M
1 . Anem|aofchron|cd|sease can resu|tfromchron|c
|nf|ammaIoryd|seases,chron|c|nfect|ons,and
mal|gnancy.
2. Typ|cal |y,anem|aofchron|cd|sease|snormocyt|c;
2b% ofcasesofanem|aofchron|cd| seasehave
m|crocytos|s.
3. Anem|aofchron|cd|sease results froman|nab|l |Iy
Io mob| | |ze |ronfrom |ts storages|Ies |n
macrophages.
PLHNLLXL PPLPb Vm
LLLHLPbLL HLL LLLL HLLLLLP
|ornocyiic anenias resuIi Iron ihe IaiIure oI ihe
lone narrov io roduce adequate nunlcrs oIred
lIood ceIIs duo to systenic iIlness. Bone narrov
Iunciion car le inaired ly Ilrosis, naIignant ir-
IItration, iransicni narrov IaiIure, or IaiIure to
synthesize eryihrooieiin [chronic renal disease) .
Jransient narrov Iailure siates incIude iransieni
eryihrolIastoenia ol chiIdhood, arvovirus BI 9
induccdaIasticcrisis,anddrugtoxicityIronnyeIo-
suressiveagents.InornocyiicaneniaaIsooccurs
viih acute lIood Ioss. Re-equiIilration oIihe totaI
lIood voIune lcIore eryihrooiesis resuIts ir ihe
anenia.ChronicinllannatorystatcsresuItinanenia
oIchronic diseasc, vhich can le nornocytic [75%)
ornicrocyiic [25%), as discussed earIicr.
lBD5OD LlyDlOOB5OQODB
O LDODOOO
Transient cryihrolIastoenia oIchildhood [JLC) is
anacquircdureredceIIaIasiacausedlytransient
lone narrov suression. Jhe rcsuIting anenia is
nornocyiic.IIthough aseciIcetioIogyhasnotleen
ldeniiIed,JLC is usuaIIy recededly a viraIinIec
fion.TLC occurslctveenihe ages oI6 nonihs and
5 years, vith a eak incidencc at 2 years oIage. lr
contrastto Iianond-BIacIIan syrdrone, vhich is a
congcnitaI nacrocyiic urc red ceII alasia, S5% oI
cases oITLC occur aIfer I ycar oIage, ihere arc no
otherassociatedanonaIies, andIctalhenogIolinand
l aniigen arc noiresent.
LOC0 %0OPSl0lDOS
The hisioryandhysicaIexaninaiionareunrenarI-
alIe excet Ior graduaI onset oI aIIor over thc
course oI veoIs. IeriheraI snear is nornaI other
than reticuIocytoenia. Bone narrov exanination
reveaIsIevcryihroidrecursors andnornaInyeloid
and IaieIeirecursors.
lP0lPOl
TIe henoglolinisusuaIlyatitsradiratihetine oI
diagnosis.Sonianeousrecoveryoccursviihin I to2
nonihs oIdiagnosis. Rcd lIood ceII transIusions arc
necessary onlyiIihe |aticni has signs or syntons
oIcorgcstive heariIaiIure .

KEY L W
1 . Trans|enIerythroblastopen|aofch||dhood,anor-
mocyt|c anem|acaused by bone marrowsuppres-
s|on,|sanacqu|red purered ce|l aplas|aw|th a
peak|nc|denceat2 yearsofage.
2. V|ral |nfect|onusual l yprecedesTEC,but nospe
c|hcet|o|ogyhasbeen |dent|hed.
3. RecoveryfromTEC|ssponIaneous.
< not tor sale ! > < ue pn popa ! >
< cau v pexaexouee]cn: HYCA o ]ou. | >
Chapter 1 l Henatology 1 1 1
PLHLLXL PPLPb Vm
PLHLPbLL HLL LLLL HLLLLLP
w w w w m + w
mODOlyC PDODB
oinocytic ancnias vitl incicascd icd ccll ioduc-
tion aic nost connonIy causcd Iy IcnoIytic ancnias.
1lc icd IIood ccIl dcsiiuction and ancnia aic scnscd
Iy tIc kidncys, vIicI icIcasc ciy\liooiciin to stinu-
Iatc Ionc naiiov ciy\liooicsis. HcnoIytic ancnias
aic causcd Iy Iactois cxiiinsic to tlc icd ccII [cxtia-
coiusculai) oi Iy dcIccts intiinsic to tlc icd ccII
[intiacoiusculai) . In gcnciaI, cxirinsic dcIccts aic ac-
quiicd and iniiinsic dcIccts aic Icicditaiy.
LxiiacoiuscuIai anonaIics aic dividcd into
isoinnunc, autoinnunc, and noninnunc Icnolytic
ancnias. Isoinnunc IcnoIytic ancnia icsuIts Iion
antiIodies ioduccd Iy onc individual against tIc
icd |lood cclIs oI anotlci individuaI oI tIc sanc
sccics. BC oi ninoi antigcn inconatiIiIity is an
cxanlc oI isoinnunc IcnoIytic ancnia [scc
CIatci I J) . In autoinnunc IcnoIyiic ancnia,
aInoinaI antiIodics diiccicd againsi icd IIood
ccIIs aic ioduccd Iy tIc aticnt. utoinnunc
IcnoIyiic ancnias can Ic idioatIic, ostinIcctious
[Ayco|n:nn neunonine, Istcin-Baii viius), diug-
induccd [cniciIIin, quinidinc, aIIa-nctlyIdoa),
oi nay icsuIt Iion a cIionic autoinnunc discasc
[sysicnic Iuus ciyiIcnatosus) oi naIignancy
[non-Hodgkin's IynIona) . JIciay Ioi autoin-
nunc Icnolytic ancnia vaiics dccnding on tIc cti-
oIogy oI tlc IcnoIysis and tIc cIinicaI condition oI
iIc aticnt. ln gcnciaI, ticatncnt is suoitivc, vitl
tle caicIuI usc oI ackcd icd IIood cclI tiansIusions
and coiticostcioids. utoantiIodics icact vitI
viituaIIy aII icd IIood ccIIs, making ciossnatcIing
diIl cuIt. In sonc scvcic cIionic cascs, intiavcnous
innunogloIuIin, innunosuicssivc plaina-
cotlciay, and sIcncctony nay Ic indicatcd.
1Ic antiIodics tlat causc isoinnunc and auto-
innunc IcnoIyiic ancnias nay Ic oI tlc IgC oi
IgM cIasscs. IgC antiIodics tcnd to Ic vain icac-
tivc [naxinaI activity at J7C) and aic consideicd
inconIctc aniiIodics. 1Icy coat tIc suiIacc oI tIc
icd Ilood ccIIs and Ix caiIy conIcncni cono-
ncnts Iut cannot aggIutinatc icd IIood ccIls oi acti-
vatc tlc conIcncnt cascadc tliougI tlc entiic
IcnoIyiic scqucncc. HcnoIysis occuis cxtiavascu-
laiIy Iccausc oI tiaing oI tIc osonizcd icd IIood
ccIls Iy nacio Iagcs in tIc icticuIocndotIcliaI
systen. IgC antiIodics aic associaicd vitl auioin-
nunc discascs, IynIonas, and viiaI inIcctions.
1Icsc aniiIodics aic idcntihcd Iy iIc diicct
CoonIs' fcst. lg antiIodics aic usuaIIy cold icac-
tivc [naxinaI aciivity at Iov tcnciatuics) and aic
dccncd conIctc antiIodics. Jlcy aggIutinatc icd
IIood cclIs and activatc tIc conlcncnt scqucncc
tliougI C9, causing Iysis oI icd IIood ccIls. HcnoI-
ysis occuis intiavascuIaiIy. IgM antiIodics aic associ-
atcd vitl A. neunonine, Istcin-Baii viius, and
tiansIusion icactions.
oninnunc IcnoIytic ancnias can Ic nicioan-
gioatIic [disscninatcd intiavascuIai coaguIation,
tIionIotic tIionIocyiocnic uiuia, IcnoIytic
uicnic syndionc, naIignant Iyciicnsion, giani
Ienangiona, iccclansia, icnaI giaIt ic]cciion) oi
can Ic duc to danagc Iion noncndotlcIiaIizcd sui-
Iaccs [aiiiIciaI Icaii vaIvc, aiiciiovcnous nalIoina-
tion, KasaIacI-Mciiitt syndionc), IycisIcnisn,
aIctaIioiotcincnia, toxins [snakc vcnon, coci,
aiscnic), naIaiia, oi Iuins.
Intiacoiusculai dcIccts incIudc intiinsic ncu-
Iianc dcIccts sucI as Icicditaiy sIciocytosis, Icicd-
itaiy cIlitocytosis, Icicditaiy stonatocyiosis, and
aioxysnaI noctuinaI lcnogIoIinuiia [IH) . IH
is tlc onIy intiacoiuscuIai disoidci iIat is not inIci-
itcd. HcnogIoIinoatIics [sickIc ccIl disoideis) and
cnzync disoidcis [C6II dcIcicncy, yiuvatc kinasc
dcIcicncy) aic aIso intiacoiuscuIai disoidcis. IoI-
loving aic discussions oI Icicditaiy sIciocyiosis,
sicklc ccII ancnia, and C6II dcIcicncy, tIicc oI iIc
nost connon intiacoiusculai dcIccts.
M
KEY LW
M
1 . Normocyt|canem|asw|th | ncreased red cell pro-
duct|onare mostcommonlycausedbyhemolyt|c
anem|as.
2. Hemolyt|canem|asarecausedbyfactorsextr|ns|c
totheredcellorbydefects|ntr|ns|ctothered
cell.|n genera|,extracorpuscul ardefectsare
acqu|red,and | ntracorpuscul ardefectsare
hered|tary.
3. Extracorpuscularanomal|esare d|v|ded|nto
|so|mmune,auto|mmune,andnon|mmune
hemo|yt|canem|as.
4. |ntracorpusculardefects|nc|ude|ntr|ns|c
membranedefects,hemog|ob|nopath|es,and
enzymopath|es.
MOlOOBly bQDOlOCyO55
Heicditaiy sIciocyiosis is causcd Iy a dcIcct in icd
IIood ccII ncnIianc suoiiing iotcins [scctiin,
ankyiin, oi Iand J ioicin). 1lc dcIcct Icads to a loss
< o tor e > < ue JYR n]o)az ! >
112 e|ar|cs
< cau v pexanx~ouee]cn: HCA o ]ou. ]
oI ncnIianc Iiagncnts vitIout a iooiiionaI loss
oI volune. JlcicIoic, niciosIciocytes [snalI sIcii-
caI icd IIood ccIIs vitI a IigI voIunc-to-suiIacc
iatio) Ioin MiciosIeiocyics aie Icss dcIoinaIlc
tlan noinal icd IIood ccIIs, so tlcy aic tiacd and
dcstioyed in tlc niciovascuIatuic oI tle sIccn. InIci-
itancc is usualIy autosonal doninant, Iut Z5% oI cascs
aic due to ncv nutaIions oi autosonaI icccssivc Ioins.
LOC0 %0OPSl0lDOS
Hcicditaiy sIciocytosis vaiics gieatly in cIinicaI
scvciity, ianging Iion an asyntonatic, vcIl-
conensatcd, niId IcnoIytic anenia discovcicd
incidcntaIIy to a scvcic IcnoIyiic ancnia vitI
giov:I IaiIuic, sIcnoncgaIy, and cIionic tiansIu-
sion iequiicncnts in inIancy neccssitating caiIy
sIcncctony. JIc ncvIoin vitI tlis disoidci nay
icscnt vitI scvcic uncon]ugatcd IyciIiIiiuIinc-
nia causcd Iy IcnoIysis. InIants and cIiIdicn nay
icscni vitI aIIoi and sIcnoncgaIy. CccasionaIIy,
aticnts nay icscnt vitI aIastic ciisis aIici ai-
voviius B ! 9 inIcction. Bccausc oI cIionic IcnoIysis,
tccnageis dcvcIo gaIIstoncs and cIoIecystitis. IIys-
icaI cxanination icvcaIs aIloi, scIeiaI ictcius, and
niId to nodciaic slcnoncgaIy. IaIoiatoiy studics
dcnonsiaic nild noinocyiic ancnia, icticuIocyio-
sis, and indiicct IyciIiIiiuIincnia. Iuiing an
alastic ciisis, tlc ancnia Icconcs scvcic and
icticuIocytocnia occuis. Iiagnosis is conIincd Iy a
ositive osnotic IiagiIity tcst.
fP0lPOl
Jicainent incIudcs IoIic acid suIenentation to
ncc tIe nccds oI incicascd icd IIood ccII tuinovci
and ied IIood ccII tiansIusions duiing an alastic
ciisis. SIencctony aIIcviatcs ancnia, icticulocyiosis,
and scIeial ictcius, aItlougI niciosIciocyics
cisist. SIcncctony sIouId Ic dcIeiicd untiI aIici 6
ycais oI agc Iccausc oI tlc IigIci tlc iisk oI scsis
Iion encasuIatcd oiganisns in young cIildicn.

L
KEY LW

1 . Hered|taryspherocytos|s| scaused byadefect| n


themajorsupport|ng prote|nsofthered b|ood
ce|l membrane.
2. Thedefect |eadstoa lossofmembranefragments
andtheformat|onofr|g|dm|crospherocytes,
wh|chare pronetohemo|ys|s.
3. D|agnos|s | sconhrmedbyapos|t|veosmot|c
frag|||tytest.
bCKlO LOll L5OB5O
0lDDgPOPSS
Sicklc ceII discasc is an autosonal iecessivc disoidci
tIat icsuIts Iion a vaIinc-Ioi-gIutanine suIstitution
in tlc sixtI anino acid osition oI tIe Icta -gIoIin
cIain. JIis suIstitution aItcis tlc stiuctuic oI tIc
IcnogIoIin noIccuIc, vIicI, undei conditions oI
dcoxygcnation, ionotcs aggicgation oI IcnogIoIin
into Iong olyncis tIat distoii tIc icd IIood ccII into
a sicklc sIac. Sickling sIoitcns icd IIood ccII sui-
vivaI tine and icsuIts in a cIionic IenoIyiic ancnia.
SickIcd ceIIs aIso causc niciovascuIai oIstiuction,
vIicI Ieads to tissuc iscIcnia and inIaiction. JIc
sickling Ienonenon is acccntuatcd Iy Iyoxia, aci-
dosis, incieascd oi dccicascd tcnciatuic, and dcIy-
diation. II onIy onc oI tIc tvo -gIoIin gcncs is
a!cctcd, tIc individuaI Ias 8:CKlC CCll !ra1, vIicI is
tIc Ictciozygous statc vitIout cIinicaI conscqucncc.
II IotI -gIoIin gcncs Iavc tlc gcnetic suIstitution,
tIc aticnt is Ionozygous Ioi IcnogIoIin S and
Ias 8CKlC CCll U8Ca8C. Sickling disoideis oI vaiying
scvciity aIso icsuIt Iion IcnogIoIin S cxisting H
conIinaiion viiI otlci aInoinaI IcnogIoIins
[IcnogIoIin C, I,,,,_,
,,, C__[) oi tlaIasscnias [B'
oi B tIaIasscnia).
LQ0PDDgy
Sicklc ceIl discasc a!ccts ! in 6Z5 Iiican nciicans,
nakIng it tlc nost connon autosonaI icccssivc dis-
oidci in tIat ouIation. It aIso occuis in iIosc oI
Cicck, ItaIian, and Saudi iaIian descent.
LOC0 %0OPSl0lDOS 0O0 %0O0gPPOl
CIiIdicn vitI sicklc ccII tiaii aic gcneiaIIy asynio-
natic. RaicIy, an individuaI viII exIiIit ainIcss
Icnatuiia and tIc inaIiIity to ioeiIy conccntiate
tIc uiine [isostlcnuiia). Paticnts vitI sickle ccII tiait
occasionaIIy lavc sicklc ccIIs on eiiIciaI IIood
sncai, Iut IcnogIoIin cIcctioIoicsis iovidcs
tIc dcInitivc diagnosis. JyicaIIy, IenogIoIin cIcc-
tioIoiesis icvcaIs 55% to 6|% IcnogIoIin , 4|%
to 45% IenogIoIin S, and 2% io J% IcnogIoIin
,. !t L inoitan to dctcc tIc tiait Ioi gcnctic
counscIling.
InIike sicklc ccII tiait, sicklc ccIl discasc causcs
scvcic noiIidity and noitaIity. uantitativc Icno-
gIoIin cIectioIoicsis sIovs |% IcnogIoIin , S5%
to 95% IenogloIin S, Z% to J% IcnogIoIin ,, and
u to ! 5% IcnogIoIin ! In nost cascs, diagnosis
is nadc Iion ncvIoin sciccning tests. JIc IigIIy
vaiiaIIc cIinicaI naniIcsiations oI sicklc ccII discasc
< not toz sale ! > < ue pn n]opa ! >
< cau v pexae~ouee]cn: HCA o ]ou. ] >
L|Q\O| 1 l |OH\O|Ogy 1 13
M 1HLL 1 b
LllC NlleSttloS ol blCke Le Peml"
NDESIIOD LOmmED1S
PHOH
PQ|S\|C C|S|S
bOQUOS\|\|OHC||S|S
MOHO|y\CC|S|S
LC\y\S
L||OH|C,OHSO\34HOOQO,|OQU||OSC|\O\|O|QyP|C|OHC |OHO|yS|S
||VOV| |US| HOC\|OH,CU\OHOSO||OSO|V|HQ
NSS|VOSQ|OHOHOg|y,SOCK,\|O\w|\|\|HSUS|OH
NyOOSSOC|\OO w|\| LO|L OOUCOHCy
MHOWO\SwODQ HO|y HHCy
||D ClS|S N|C|OVSCU||Q|HU| VSOOCC|US|VO D|C\S OHUSC|O,DOHO,|UHg,|D\OS\|HES
LO|OO||VSCU||CCOOH\S
PCU\OC|OS\SyHO|OHO
L||OH|C |UHg OSOSO
||QSH
L|QO HOSH||VOSSO| S|CK|Hg HO\||OHDOS|S[S\|OKO),|OQU||OSC||OD|C\|HSUS|OH
|DOC\|OHHOlO||H|C\|OD,SOVO|O |yQOXOH|,IHU|\|\O,OySQDO,||OS
|U| HOHlyUD|OS|S,|OS\l|C\|VO | UHg OSOSE,COlQU|OH|O
LUSOSOVOH\U| |HQO\OHCO,\|O\w|\|\|DSUS|OH,OXygOH,O|COlQO| CVO|HOS
\OSQODg|OS S|UH\
LCU|| hO\|HOQ\|y
L| | D|OOO|O|SOSO
hOD
L|OOHyOQ\|y
| HOC\|OHS
b||||UD|HS\ODOS,C|O|OCyS\|\|S
MOH\Uf,QQfy DOCfOSS,fOHCOHCOD\f\Hg OOC\, HOQfOQ\|y
MO|\ | U|O [UO|OSS)
|UHC\|OD| SQ|OH|,|HC|OSOO ||SKO|HVS|VO|HOC\|OH OUE \O OHCQSU|\OO
OC\O||, SUC|S5.tCuOttOC,M.tthuCt2OC,HON.CtttQtltOt5,5OCtChOHO
5lOhyOCOCCu5OuICu5OS\OOHyO|\S,SOVO|ONyCOO5O QHOUHOH|,\|DSUS|OH
CQUlEO DO\ODS
L|Ow\|m|| U|O,OO|yOO QUDO|\y Ny fOSQODO \O DU\||\|OH| SUQQ|OHOD\S
LttC| me$tttD8 wtt 8tCke Ce t|tt le uu8u Dut tCuUe le t|Iy eCID$t$ (emtul), $uUUe Uet D eXeItD, tlDCu|l yem
eXte8tD, U 8tCktg ule88ul2CU tIe$.
LNV, Cy!Dneg|Dvlu8, LbV, L8teblI vIu$ LL, g|uCD8eD8te CeyUIDge$e.
icsuIi Iion ancnia, inIcction, and vasooccIusion
[JaIIc ! |-5) .
t aroinatcIy 4 nontIs oI agc, vIcn tIc cr-
ccntagc oI IcnogIoIin I dininisIcs and tIc cr-
ccntagc oI IcnogIoIin S iiscs, tIc cIiId vitI sicllc
ccIl discasc dcvcIos a iogicssivc IcnoIytic ancnia.
JIc ancnia oI sicllc ccII discasc is a cIionic, vcII-
concnsatcd, scvcic ancnia tIat is iaicIy tiansIusion
dccndcni. Connon naniIcstations oI tIc ancnia
incIudc paIIoi, |aundicc, sIcnoncgaly in |nIancy, a
systoIic c]cction nuinui, and dclaycd scuaI dcvcI-
oncnt and giovtI. SIcnic scqucstiation, aIastic
ciisis, and IycrIcnoIytic ciisis aII suciinosc
acutc IiIc-tIicatcning dccIincs in hcnogIoIin con-
ccntration on tIc cIionic concnsatcd ancnia oI
sicllc ccII discasc. In sIcnic scqucstiation, iaid
sIcnic cngorgcncni causcd Iy tiaing oI icd IIood
ccIIs nay lcad to IyovoIcnic sIocI Scqucstiation
tyicaIIy occuis Ictvccn bnontIs and Z ycars oI agc.
ViiaI suicssion oI icd |lood ccIl piccuisois in tIc
Ionc nariov, nost oIicn Iy aivovirus B I 9, icci-
iiatcs aIasiic ciisis. Iosurc oI a atient vitI sicllc
ccII discasc and conconiiant LbII dcIcicncy to
an oidativc sticss icsults |n acuic IcnoIysis suci-
inoscd on a cIionic IcnoIytic ancnia [IyciIc-
moIy tic ciisis). Mcdications oi inIcction usualIy causc
tIc acutc IcnoIysis. SIcnic scqucstiation, aIastic
ciisis, and IyciIcnoIytic ciisis arc oIicn ticatcd
vitI icd IIood ccIl iiansIusion. Bccausc oI thc
icscncc oI cIronic IcnoIytic ancnia, galIstonc
loination and cIoIccystitis aic connon during
adoIcsccncc.
PtIc siclcd ccIIs iiavcisc tIc slccn, thcy causc
nicrovascuIai oIstiuction, inIaiction, and IIiosis oI
tIc sIccn. JIis ioccss, lnovn as aulouarClou,
causcs tIc sIccn to giaduaIly icgicss in sizc, Iy tIc
agc oI 4, tIc sIccn is no Iongci aIaIIc. Morc
inoitant, autoinIarction dininisIcs tIc caaIility
1 1 4
o or p]e > < ue JYR n]o)az ! >
< cau v pexanx~ouee]cn: HCA o ]ou. ]

eatcs
oI tIc sIccn to hItci cncasuIatcd IactciiaI oigan-
isns and Iaccs tIc inIant at gieat iisl Ioi ovci-
vIcIning inIcction Iion Stretococcs neunonioe oi
Hoenobi|us in]luenzoe. ny inIant oi cIiId vIo Ias
sicllc ccII discasc and Icvci [tcnciatuic gicatci
tIan JS. 5'C) nust Ic cvaIuatcd inncdiatcIy. l-
tIougI tIc cIiId IikcIy Ias a Icnign viiaI inIcction,
invasive IactciiaI inIcction nust Ic cxcIudcd. Jo
nininizc tIc iisk oI IiIc-tIicatcning inIcction, cIiI-
dicn vitI sicllc ccII discasc stait eniciIIin ioIy-
Iaxis at aioxinatcIy nontIs oI agc and icccivc
vaccinations. BotI tIc H. in]luenzoe tyc I [HiI) and
IctavaIent ncunococcaI con]ugate [Ircvnai} vac-
cincs aic givcn at tIc Z-, 4-, and 6-nontI visits and
tIcn again Ictvccn ! Z nontIs and ! 5 nontIs oI agc.
JIc ZJ-vaIcnt ncunococcaI oIysaccIaiidc vaccinc
[IIV] sIouId Ic adninistcicd at Z ycais oI agc and
tIcn again at 4 to 6 ycais oI agc. IeniciIIin ioIy-
Iaxis is continucd until at Icast 5 yeais oI agc.
VasooccIusivc ciiscs icsuIt Iion niciovascuIai
inIaicts, nay occui in any oigan oi tissuc oI tIc lody,
and aie connonIy icciitatcd Iy inIcction, coId
cxosurc, dcIydiation, vcnous stasis, and acidosis.
IactyIitis, oi Iand-Ioot syndionc, is synnctiicaI
ainIuI svcIIing oI tIc doisaI suiIace oI tIc Iands
and Icct causcd Iy avascuIai neciosis oI tIc
nctacaiaI and uctataisaI Ioncs. IactyIitis occuis at
4 to 6 nontIs oI agc and is tIc caiIicst clinicaI nan-
iIcstation oI vasooccIusivc discasc in tIc sicllc ccII
aticnt. In oldci cIiIdicn, ain ciiscs nost oItcn
IocaIize to tIc Iong Ioncs oI tIc ains, lcgs, vcitcIial
coIunn, and stcinun. Iain ciiscs Iast Iion Z to 7days
and aic ticatcd vitI nonstcioidaI anti-inhannatory
diugs and naicotics. Hydioxyuica naintcnancc
tIciay dccicascs tIc nunIci and scvciity oI vasooc-
cIusivc ciiscs. vascuIai ncciosis oI tIe IcnoiaI Icads,
anotIei vasooccIusivc naniIcstation in Ionc, tyi-
calIy occuis in tIc adoIcsccnt ouIation.
iciovascuIai oIstiuctivc discasc can aIso occui
in tIe Iungs, ccntiaI ncivous systcn, pcnis,
nyocaidiun, and intcstinc. cutc cIest syndionc, a
vasooccIusivc ciisis vitIin tIc Iungs, is oItcn causcd
Iy uInonaiy inIcction and inIaiction. Iaticnts
icscnt vitI Iyoxia, icsiiatoiy disticss, and uI-
nonaiy inIItiatcs. Cxygcn, anaIgcsia, antiIiotics, and
cxcIangc tiansIusion aic uscd to naxinizc icsiia-
toiy status and nininizc IuitIci uInonaiy danagc.
SiniIaiIy, occIusion oI tIc laigc ccicIiaI vcsscIs
icsuIts in stiokc. Iaticnts icscnt vitI ncntaI status
cIanges, scizuics, and IocaI aialysis. Bccausc oI tIc
IigI iisk oI iccuiicncc, cIiIdicn vIo Iavc Iad a
stiokc aic Iaccd on cIionic icd IIood ccII tiansIu-
sion iotocoIs to nininizc tIc iisl oI lutuic stiokc.
Iiiaisn nost tyicaIIy occuis in Ioys Ictvccn 6 and
Z| ycais oI agc. JIc cIild dcvcIos suddcn ainIoI
cngoigcnent oI tIc cnis tIat viII not suIsidc. cutc
cIcst syndionc, stiokc, and iiaisn aic ticatcd Iy
cxcIange tiansIusion to dccicasc tIe ciccntagc oI
IcnogIoIin S to IcIov J|% in an attcni to nini-
nizc vasooccIusion.
By adoIcsccncc, tIc c!ccts oI cIionic nyocaidiaI
niciovascuIai oIstiuction and inIaiction aic cvidcnt
Iy an enIaigcd IycitioIic Icait. any aduIts
cvcntualIy succunI to congcstivc Ieait IaiIuic Iion
rogrcssive nyocardiaI danagc. IdoninaI ciisis
icsuIts Iron niciovascuIai oIstiuction oI tIc intcsti-
naI ciicuIation. Iaticnts icscnt vitI iIcus and
icIound tcndcincss, niniclng an acutc aIdoncn.
JIc ain nay Ic IaniIiai to tIc atient and icadiIy
iccognized as "ciisis ain." Idoninal pain consistcnt
vitI tIe cIiId's noinaI ain consteIIation duiing
ciisis nay vaiiant a ciiod oI oIscivation vitI
Iydiation and anaIgcsic adninistiation. |I tIc aI-
doninaI ain is not tyicaI Ioi tIe aticnt duiing
vasooccIusivc ciisis, surgical consultation sIould Ic
oItaincd.
*

KEY LW
I
1 . S|ck|ecelld|sease| sanautosomalrecess|ved| sor
derthat resu|tsfrom an am| noac|dsubst|tut|on
onthep-glob|ncha|n.Th|ssubst|tut|onresu|ts|n
analterat|onofthestructureofthehemog|ob|n
mo|ecule,wh|ch,undercond|t|onsofdeoxygena
t|on,promotesaggregat|onofhemplob|n|nto
long polymersthatd|stortthered b|ood cell |nto
as|ckleshape.
2. S|ckl|ngshortensred b|oodcellsurv|valt|meand
resu|ts| nachron|chemolyt|canem|a.
3. Thec||n|calman|festat|onsofs|cklece||anem|a
resu|tfromanem|a,|nfect|on,andvasoocc| us|on.
LlUCO5O~~DO5QDBO
LODyOfO@ODB5O LOCODCy
C6II deIcicncy, tIc nost connon icd IIood ccll
cnzyne dcIcct, is tiansnittcd as an X-Iinkcd icccs-
sivc tiait. JIc Iack oI tIis cnzyne in tIc Icxosc
nono IosIatc sIunt atIvay icsuIts in dcIction
oI nicotinanidc adcninc dinucIcotidc IosIatc
[IIH) and tIc inaIiIity to icgenciatc icduccd
gIutatIione, vIicI is nccdcd to iotect tIc icd IIood
ccII Iion oxidativc sticss.
not tor sale ! > < ue pn popa ! >
< cau v peaexouee]cn: HC^d o


L|Q\O| / OH\|OQy 1 1 5
JIe nost connon Ioins oI C6II dcIcicncy aic
tIc
-
and Mcditcrrancan variants. JIc variant,
Iound in aroxinatcly ! 0% oI Irican ncricans in
tIc Iniicd Statcs, is associatcd vitI an isocnzync
tlat dctcrioiaics raidly, vitl a IaII-IiIc oI ! J days.
JIc Meditcriancan variant occurs rcdoninantly in
crsons oI Crcck and ItaIian dcscent its isocnzync
is cxtiencIy unstaIIc, vitI a IaII-IiIc oI scvcraI
Iours.
VIen tIcrc is an oxidativc stiess on tIc rcd IIood
ccII, exoscd suI!lydryI gious on tlc IcnogIoIin
arc oxidizcd, l cading to dissociation oI Icnc and
gloIin noictics, vitI tIc gIoIin rcciitating as
Hcinz Iodics. Ianagcd rcd celIs aic icnovcd
Iron circuIation Iy tIc rcticuIocndotlcIial systcn,
scvcreIy danagcd ccIIs nay Iyse intravascuIarIy.
Knovn oxidants incIudc suIIonanidcs, nitroIuran-
toin, rinaquinc, and dincrcarol . HcnoIysis nay
aIso Ie rcciitatcd Iy Iava Icans and inIcction.
LOC0 %0OPSl0lDOS
JIc cIassic coursc oI C6II dehcicncy is cisodic
strcss- oi druginduccd Icnolyiic ancnia. Iaticnts
vitl tIc / variant Iavc a Iinitcd IenoIysis conhncd
to tIe oIdcr rcd Ilood ccII ouIation. Rccovcry
occurs as young rcd IIood ccIIs vitl cnzync activ-
ity suIlcicnt to rcsist oxidativc strcss cncrgc Iion
tIc Ionc nariov. HcnoIysis is nost connon in
naIes vIo osscss a singIc aInornaI XcIionosonc.
Hctcrozygous IcnaIcs vIo Iavc randonIy inacti-
vatcd a ligIcr crccniagc oI tIe nornaI gcnc nay
Iccone syntonatic, as nay honozygous IcnaIcs
vitI tIc / variant. Cnc ercent oI !rican-
ncrican lcnaIcs arc variant Ionozygous.
Iatients vitl tlc Mcditcrrancan variant Iavc IcnoI-
ysis tIat dcstroys nost oI tIcir red ccIIs and nay
rcquire tiansIusions untiI tlc drug is cIininatcd Iion
tIcir Iodics. JIc ncutioIiIs oI aticnts vitl tlc
nost sevcrc dcgrccs oI C6II dchciency dcnonstiatc
dcIcctivc oxidativc kiIIing Iccause ol tIc dclction
oI IIH, vIicI scrvcs as an cIection donor to tIc
ncnIranc-Iound oxidasc tlaf roduccs IactcricidaI
oxygen sccics.
Cn criIcraI Ilood sncar, tle red cclIs acar to
Iave "Iitcs" takcn out oI tIcn [|Iistcr ccIIs) . JIc
Iittcn arcas rcsult Iion Iagocyiosis oI Hcinz Iodics
Iy slenic nacro Iagcs. Iuring IenoIytic cisodcs,
IysicaI cxanination rcvcaIs ]aundicc and dark
urinc [causcd Iy IcnogloIinuria and IigI lcvcls oI
uroIiIinogcn) . IaIoratory tcsts revcal clcvatcd in-
dircct IiIiruIin and Iactatc dcIydrogcnasc and lov
IatogIoIin. InitiaIIy, tlc IcnoIysis cxcccds tIc
aIility oI tlc Ionc narrov fo concnsatc, so tIc
rcticulocytc coun nay Ic lov Ior tIc hrst J to 4
days.
1Ie diagnosis oI C6II dcIciency is nadc Iy
Inding dcIcicni IIH Iornation on C6II assay.
C6II IevcIs nay Ic nornaI in iIe sctting oI acutc,
scvcrc lcnolysis Iccausc iIc nost dcIcicni ccIIs
Iavc Ieen dcstioycd. Rccating tIe tcst at a Iatcr
tinc vIen tIc aticnt is in a stcady-statc condition,
tcsting tIc notIci oI nalcs vitI suscctcd C6II
dcIciency, and crIorning cIcctroIorcsis to idcnti|
tIc recisc variant IaciIitatc diagnosis.
fP0lPOl
Iaticnts vitI C6II dcIcicncy associatcd vitl acutc
scvcrc IenoIysis nccd to avoid diugs tIai initiaic
Icnolysis. Jrcatncnt is suortivc, incIuding ackcd
rcd IIood ccIl tiansIusion during signiIcant cardio-
vascular conronisc and vigoious Iydration and
urinc alkaIinization to rotcct tIc kidncys against
danage Iron rcciitatcd lrcc IcnogIoIin.

KEY LW
M
1 . L| UCOSOQDOSQD\O OODyOlOQODSO OOHCODCy,
\DO HOS\ COHHOD |OO OOOO CO ODZyHOOOOC\,
S \lDSHlOO S D 7~|DKOO lOCOS5VOl\.
2. JDO |CK O\DS ODZyHO D \DO DOXOSO HODOQDOS
QD\O SDUD\Q\Dwy|OSU|\S D OOQ|O\OD O
NPM DO D DO| | \yO lOQODOl\O lOOUCOO
QU\\DODO,wDCD SDOOOOO \O QlO\OC\\DO lOO
O|OOOCO| | lOH OX|O\VO S\lOSS.
PLHLLXL PPLPb Vm
LLLHLPbLL HLL LLLL HLLLLLP
Macrocytic ancnias arc suIdividcd according to tIc
rcscnce or aIscncc oI ncgaloIlasiosis, a narkcr oI
incIlectivc I syntIcsis vitlin a rcd IIood ccII
rccursor. Causcs oI ncgaloIlastic ancnia incIudc
vitanin B, , and IoIaic dcIcicncy, drugs tIat intcrIcrc
vitI Iolatc nctaIolisn [Icnyioin, nctIotrcxatc,
irinctlorin), and nctaIoIic disordcis [oioiic
aciduria nctlyInaIonic aciduria, IescI-lyIan syn-
drone) . Macrocyiic ancnias vitIout ncgaIoIIasio-
sis rcsuIt Iion Ionc narrov IaiIurc and incIudc Ionc
narrov IaiIurc syndroncs [Iianond-BIackIan syn-
dione, Ianconi's ancnia, idioatIic aIastic ancnia,
rcIcukenia), drug-induccd ancnias [azidotIyni-
< not tor sale ! > < ue pn n]opa ! >
||ucpr| n1O |OO|\||CS
< Co V peaen~ouee]cxn: HCA) o ]ou.
J
>
1 1 6
dinc, vaIioic acid, caiIanazcinc|, cIionic Iivci
discasc, and Iyothyioidisn.
NO@BOOB5C NBClOCyC PDODB5
Vl0O b_ LC0CCOCy
Vitanin B, _ cocnzync Ioi 5-nciIyI-tctiaIydioIo-
latc Ioination, is nccdcd Ioi I syntIcsis. li is
Iound in ncat, IsI, cIccsc, and cggs. Iiciaiy vitanin
B, , dcIcicncy is iaic in dcvclocd countiics cxcct
in thc Iicast-Icd inIant vIosc nothci is a vcgan vitl
ooi attcntion to dictary souiccs oI vitanin B, ,.
notIci causc oI vitanin B, , dcIcicncy is scIcctivc
oi gcncializcd naIaIsoition. Vitanin B, , conIincs
vitl intiinsic Iactoi, vIicI is pioduccd Iy gastiic
aiictal ccIIs, and is aIsoiIcd in tIc tcininaI iIcun.
1ianscoIaIanin II tlcn tiansoi:s viianin B, , to tIc
Iivci Ioi stoiagc. 1Ic avaiIaIiIity oI vitanin B, , is
rcduccd Iy any condition tIat aItcis intiinsic Iactoi
ioduction, intciIcics vitI intcstinaI aIsoition,
oi icduccs tianscoIaIanin II IcvcIs. Iisoidcis sucI
as congcnitaI cinicious ancnia [aIscnt lntiinsic
Iactoi), ]uvcniIc cinicious ancnia [autoinnunc
dcstiuction oI intiinsic Iactoi), and tianscoIaIanin lI
dcIcicncy icsuIt in vitanin B, , dcIcicncy. Ctlci
causcs incIudc iIcaI icscction, snaII |ovcI IactciiaI
ovcigiovtI, and inIcciion vitI tIc IsI tacvoin
Iij||o/otbrIun /otun.
L| D|C| NDIESIODS
JIc c!ccts oI vitanin B,, dcIcicncy incIudc gIossi-
tis, diaiiIca, and vcigIt Ioss. cuioIogic scqucIac
incIudc arcstIcsias, criIcraI ncuroatIics, and, in
tIc nost scvcic cascs, dcncntia, ataxia, and/oi os-
iciioi coIunn sinaI dcgcnciation. VitiIigo is tlc
nain dcinatoIogic naniIcstaiion.
cgaloIIastic cIangcs on ciilciaI IIood sncai
includc ovaIocytosis, ncutioIiIs vitl Iyciscg-
ncntcd nucIci [noic tIan Ioui ci ccII), nucIcatcd
rcd IIood ccIIs, IasoIiIic stiIing, and HovcII-1oIly
Iodics. 1Ic ncan coiuscuIai voIunc is usuaIIy
gicatci tIan ! 00I. Intianaiiov IcnoIysis, aIso
knovn as incIlcctivc crytliooicsis, icsuIts in cIc-
vatcd lcvcIs oI sciun Iactatc dcIydiogcnasc, indiicct
IiliiuIin, and sciun iion. In scvcic cascs, ncga-
IoIlastic ancnia nay Ic acconanicd Iy Icukocnia
and tlionIocyiocnia.
Iiagnosis is conIincd Iy a suInoinaI sciun lcvcl
ol vitanin B, ,. In nondiciary dchcicncy, tIc ScIilIing
tcst IcIs dclincatc cinicious ancnia Iion Iactciial
ovcigiovtI. In tlis tcst, an oial dosc oI iadio laIclcd
vitanin B, , is givcn, and its aIsoition is cIcckcd Iy
uiinaiy cxciction. lI uiinaiy cxciction is nininaI, an
oiaI dosc oI intiinsic Iacioi is givcn. oinaI uiinaiy
cxciction aItci intiinsic Iactoi conIins tIc diagnosis
oI cinicious ancnia. Inadcquatc uiinaiy cxciction
aItci intiinsic Iactoi suggcsts IactciiaI ovcigiovtI.
ntiIiotics aic givcn, and iI vitanin B, , uiinaiy
cxciction thcn incicascs, tIc aticnt Ias Iactciial
ovcigiovtI.
|EHED
1icatncnt Ioi nost Ioins oI vitanin B, , dchcicncy,
vith tIc cxcction oI IactciiaI ovcigrovtI and !sI
tacvoin, is nontlIy intianuscuIai vitanin B, ,. JIc
ciytliooictic icsonsc is iaid, viiI naiiov ncga-
IoIlastosis inioving viilin Iouis, rcticuIocytosis
acaiing Iy day J oI tlciay, and ancnia icsolving
vitIin ! to Z nontls.
D0lC LCDCCOCy
Iolatc is Iound in Iivci, giccn vcgctaIIcs, ccicaIs, and
cIccsc and is convcitcd to tctiaIydioIoIatc, vIicI
is icquiicd Ioi Il syntIcsis. Bccausc IoIaic stoics
aic icIaiivcly snaII, dcIcicncy nay dcvclo vitIin
nontI and ancnia vitIin 4 nontls oI dciiva-
tion. Iiiologics incIudc inadcquatc dictaiy intakc,
inaiicd aIsoition oI Iolatc, incicascd dcnand Ioi
IoIatc, and aInoinaI IoIaic nctaIoIisn. Iictaiy dcI-
cicncy oI IoIic acid is unusuaI in dcvclocd countiics.
CIiIdicn ai iisk aic inIants Icd goat's niIk, cvao-
iatcd niIk, oi Icat-stciilizcd niIk oi IoinuIa, cacI
Ias inadcquatc IoIatc contcnt. alaIsoitivc statcs
oI tlc jcjunun, sucI as inhannatory IovcI discasc
and ccIiac siuc, can causc IoIatc dc!cicncy.
Incicascd dcnand Ioi IoIaic occuis vitI an incicascd
iatc oI ied Ilood ccII tuinovci [Iycithyioidisn,
icgnancy, cIionic IcnoIysis, nalignancy) . Rclaiivc
IoIatc dcIcicncy nay dcvcIo iI tIc dict docs not
iovidc adcquatc Iolatc to ncct tIcsc nccds. Cci-
tain anticonvuIsant diugs [Icnytoin, IcnoIaiIitaI}
intciIcic vitI IoIatc nctaIolisn.
L| DIC| ND|OS| ODS
ScciIc syntons aic oItcn aIscnt, aItIougI aIloi,
gIossitis, nalaisc, anoicxia, ooi giov:I, and ic-
cuiicnt inIcction nay Ic sccn. InIikc vitanin B, ,
dcIcicncy, ncuioIogic discasc i s not associatcd vitI
IoIatc dcIcicncy. IaIoiatoiy Indings includc lov icd
IIood ccII IoIatc and noinal sciun vitanin B, , lcvcIs.
McgaIoIIastic cIangcs on ciiIciaI IIood sncar and
Ionc naiiov asiiatc aic thc sanc as tIosc notcd
vitI vitanin B, , dcIcicncy.
< not tor sale ! > < ue pn n]opa ! >
< cau v pexanx~ouee]cn: HCA o
ou
Chapter l eatolgy 1 1 7
Treatment
It is inciativc not to nisdiagnose Bj ,dcIcicncy as
IoIate dchcicncy, Iccausc icatnent vitI IoIatc nay
icsult in IcnatoIogic iniovcnent and aIIov Ioi
iogiessivc ncuiologic dctciioiation. Jicaincnt vitl
! ng oI IoIatc givcn oiaIIy cacI day Ioi ! to Z nontIs
vilI tieat tlc ancnia and icIcnisI Iody stoics. CIini-
caI iesonsc is iaid, IoIloving a tinc couisc siniIai
to tlat oI vitanin Bj ,icIaccncnt tlciay. CIiIdicn
vitl cIionic IcnoIyiic conditions icquiic continucd
IoIate suIcncntation.
KEYLW

1 . Megalob|ast|cmacrocyt|canem|asreflecti nef|ec-
t|ve DNAsynthes|sandcan resultfromv|tam|n
6,andfo|atedehc|ency,drugsthat|nterferew|th
fo|atemetabol|sm,and someraremetabo||c
d|sorders.
2. V|tam|n 6,| sacoenzymeneededforDNAsyn-
thes|s.D|etaryv|tam|n 6 dehc|ency| srare|n
deve|opedcountr|es,becausev|tam| n 6stores
are|arge.The usualcauseofv|tam|n 6,dehc|ency
| smalabsorpt|on.
J. Folate|sconvertedtotetrahydrofolate,wh|ch|s
requ| redforDNAsynthes|s.6ecausefolatestores
are relat|velysmall,dehc|encymaydevelop
w|th|n 1 monthandanem|a w|th|n4 monthsof
depr|vat|on.
4. Neuro|og|csequelaeofv|tam|n 6, ,dehc|ency
|ncludeparesthes|as,per|pheral neuropath|es,
and,| nthemostseverecases,dement|a,atax|a,
and poster|orcolumnsp|naldegenerat|on.
b. M|sd|agnos|sandtreatmentofv|tam|n 6,deh
c|encyasfo|atedehc|encymayresul t|n hemato-
log|c|mprovementwh|l eal low|ng progress|ve
neurolog|cdeter|orat|on.
PODDO@BOOB5C NBClOCyC PDODB5
L0DO00Ck0O byO0fDP
Iianond-BIaclIan syndionc is an autosonaI icccs-
sivc, uic icd ccII aIasia oI unlnovn ctioIogy.
Cl | n|cal Man|festat| ons
1Ic ancnia dcvcIos sIoiily aIiei Iiiil Iut is no:
usuaIIy dctcctcd untiI Iatci, vIcn syntons dcvcIo,
90%oI cascs aic oIscivcd vitlin ilc Iist ycai oI IiIc.
InIants icscnt vitI mild naciocyiosis and icticuIo-
cyioenia. Cn lcnogIoIin cIcctioIoicsis, tlcic is
an cIevarcd IcnogIoIin I, and IetaI i antigcn is
icscnt on tIc icd ccIIs. 1vcnty-hvc ciccnt oI
aticnts havc associatcd congcnitaI anonaIics tIat
incIude sIoii statuic, vcI ncck, cleIi Ii, sIicId cIcst,
and tiiIalangcaI tIunI. JIcsc cIildicn aic at ligI
iisk Ioi lcukcnia Iatci in IiIc.
Treatment
Scvcnty-hvc ciccnt oI aticnts icsond to ligI-dosc
coiiicostcioid tIciay Iut nust icccivc tIciay
indchnitcIy. JIosc vIo do not iesond to stcioid
ticatnent aic tiansIosion dccndent and aic at iisk
Ioi tIc conIications oI iion ovciIoad .
0DQ0lC PQ0SlC POP0
Idioatlic aIastic ancnia is an acquiicd IaiIuic oI
tIc Ienatooictic stcn ccIIs tIat icsuIts in an-
cytoenia. 1Ic disoidci nay icsuIt Ion cxosuic
to cIenicaIs [Icnzcnc, IcnylIutazonc), diugs
[cIloianIcnicoI, suIIonanidcs), inIcctious agcnts
[Icatitis viius), oi ionizing iadiation. CIicn an cti-
oIogic agcnt is not idcntiIcd, and tIc casc is cIassi-
hcd as idioatIic.
C| | n|cal Man|festat| ons
JIcse aticnts su!ci lion ancyiocnia, and Ionc
naiiov asiiatc rcvcaIs a IyoccIIuIai naiiov.
1reatment
ntitIynocyic oi antiIynIocyie gIoIuIin is tcn-
oiaiily cIlcctivc, Iut sciun sickncss is a ncaily
univcisaI sidc cIIcct and icIasc is connon. Higl-
dosc coiticostcioids aic oIicn used in conIination
vitI antitlynocyic gIoIuIin. CycIosoiin Ias Iccn
cHcctive in sonc cascs, Iut Ieatic dysIunction,
icnaI insuIlcicncy, and innunosuicssion Iinit
its useIuIncss. Bonc maiiov tiansIantation is tlc
solc eIIectivc ticatncnt vitlout tiansIantation,
S0% oI aticnts dic vitlin J nontIs oI diagnosis.
II tiansIantation is Icing considcied, it is inoiiant
to nininizc tiansIusions to icducc cxosuic io
otcntiaIIy scnsitizing IIood ioducts. Icutiocnic
aticnts aic at iisk Ioi sciious IactciiaI inIcction
and usuaIIy icquiic antiIiotics vIen ilcy dcvcIo
Icvcr.
0OCDOS POP0
Ianconi's ancnia is an autosonaI icccssivc disoidci
tIat iesuIis in ancy:ocnia. ConnonIy associatcd
conditions includc igncntaiy cIangcs and skcIctaI,
icnaI, and dcvcIoncniaI aInoinaIitics. JIc disoidci
icsuIts Iion dcIcctivc II icaii mccIanisns tlat
Icad to eccssivc cIionosonaI Iieals and icconIi-
< o tor e > < ue pn n]opa ! >
11 8 r|
< cau v pexae~ouee]cn: HCA o ]ou. | >
nations. JIcsc cIionosonaI anonaIics aic Iound in
aII ccIIs oI tIc Iody, not ]ust in Icnatooictic stcn
ccIls. 1Ic ncan agc at onsct oI ancytopenia is S
ycais, and it aInost aIvays occuis IcIoic agc ! |
Cl | n| ca| Man|fesIat| ons
Connon signs incIudc Iycipigncntation and caIe
au laii sots, nicioccIaIy, nicioItlaInia, sIoit
statuic, IoiscsIoc oi aIscnt kidncy, and aIscnt
tIunIs. HcnatoIogic maniIcstations incIudc io-
gicssivc pancytocnia. Maciocyiosis is univcisaI
cvcn IcIoic tlc onsct oI ancnia, and IcnogIoIin I
is sccn on hcnogIoIin cIcctioIoicsis. ioxi-
natcIy ! 0% oI cIiIdicn vitI Ianconi's ancnia vilI
dcvcIo |cukcnia duiing ado|csccncc.
Iiagnosis is conIincd Iy dcnonstiating incicascd
cIionosonaI Iicakagc vitI cxosuic to dicoxyIu-
tanc oi otlci agcnts tIat danagc II.
1reatment
Iaticnts Ircquenily icquiic icd IIood cclI tiansIusions
and antiIiotics to ticai ancnia and inIcctions. Sonc
aticnts tiansicnily icsond io andiogcns. Coitico-
stcioids aic oIicn givcn vitI tIc andiogcns to
countciIaIancc andiogcn-induccd giovtl acccIcia-
tion. Bonc naiiov tiansIantation is tIc ticatncnt
oI cIoicc iI an HI-natcIcd donoi can Ic Iound.
Bccausc oI cIionosonal scnsitivity, tIc icaiativc
iadiation and cIcnotIciapcutic icginen nusi |c
nodiIcd Iccausc noinaI iotocoIs rcsuIt in scvcic
noiIidity and noitaIity.
KEY LW
1 . Macrocyt|canem|asw|thoutmegalob|astos|s
resu|tfrom bonemarrowfa||ureand |nc|ude bone
marrowfa| | uresyndromesD|amond-8lackfan
syndrome,Fancon|'sanem|a,|d|opath|caplast|c
anem|a,pre|eukem|a),drug-|nducedanem|as,
chron|c l|verd| sease,andhypothyro|d|sm.
2. D|amond-8|ackfansyndrome| sanautosomal
recess|vepureredcellaplas|a.Assoc|atedanom-
a||es|nc|udeshortstature,webneck,cleft l|p,
sh|eldchest,andtr|phalangea| thumb.
3. ld|opath|caplast|canem|a | sanacqu|redfa| | ure
ofthehematopo|et|cstem ce||sthat resu|ts| n
pancytopen|a.
4. Fancon|'sanem|a|sanautosoma| recess|ved|sor-
derthatresults|npancytopen|a and p|gmentary,
skeletal,renal,anddevelopmentalabnorma||t|es.
LbLHLLHb L mLLbPbb
IoinaI Icnostasis icquiics tlc intcgiity oI tIc
IIood vcsscIs, pIatcIcts, and soIuIIc cIotting Iactois.
BIccding dciangcncnts can icsuIt Iion aInoinaI
Icnostatic Iug Ioination, vIicI occuis in pIatclct
disoidcis, aIciiant cIot Ioination, vIicI is notcd in
dcIccts oI tlc coaguIation cascadc, oi vitI vascuIai
aInoinalitics.
LxanIcs oI vascuIai anonaIics tlai icsuIi in
IIccding includc Icicditaiy dcIccts oI coIIagcn syn-
tIcsis [IIIcis-IanIos syndionc), acquiicd disoidcis
oI coIIagcn ioduction [vitanin C dcIcicncy,
scuivy), and vascuIitis [HcnocI-ScInIcin uipuia,
oi HSI) . HSI is associatcd vitI aIdoninaI ain,
aitliitis, ncIiitis, and uiuia, cIassicaIIy distiiI-
utcd ovci tlc Iutiocks and lovci cxticnitics.
lBOlO L5OlOOl5
IlaicIci disoidcis can Ic ciiIci quaniitativc oi quaI-
itativc and rcsuIt in aInoinaI Icnostatic Iug Ioi-
nation. uantitativc aInoinaIitics aic dctcctcd Iy
tIc IatcIct count oi IatcIct cstinatc on ciiIciaI
IIood sncai, vIcicas quaIitativc disoidcis aic dc-
tcctcd Iy tIc IIccding tinc oi pIatclct aggicgation
studics. hroDDoCyOcna dcIncd as a IatcIct
count IcIov I 5|, /nn', is tlc nost connon
causc oI aInoinaI IIccding. 1 Iov IatcIct count can
icsuIt lion inadcquatc ioduction oi incicascd
dcstiuction oI IatcIcts. IIatcIct ioduction is cvalu-
atcd Iy asscssing tIc nunIci oI ncgakaiyocytcs in
tIc Ionc naiiov asiiatc.
|ccicascd IatcIct ioduction can icsult Iion
IaiIuic oI tlc Ionc naiiov oi Ionc naiiov su-
icssion. Bonc naiiov IaiIuic statcs causing tlion-
Iocyiocnia incIudc disoidcis causing ancytocnia
[Ianconi's ancnia, idioatIic aIastic ancnia,
Icukcnia), tlionIocyiocnia-aIscnt iadius [JR)
syndionc, and Viskott-ldiicI syndionc. JR syn-
dionc, aIso lnovn as congcnitaI ncgakaiyocyiic
IypoIasia, is an autosonal icccssivc disoidci in
vIicI iIionIocytocnia dcvcIops in tIc Iist Icv
nontIs oI IiIc and tIcn icsoIvcs sontancousIy aIici
! ycai oI agc. 1iansicnt Icukocytosis is connon and
oIicn suggcsts Icukcnia. IcIoinity oI tlc iadii is
atIognononic. Viskott-IdiicI syndionc is an X-
inkcd disoidci cIaiactciizcd Iy IyogannagloIu-
|incnia, cczcna, and tIionIocyiopcnia. Bonc
maiiov tianslantation is cuiativc. ItioIogics oI
tlionIocyiocnia causcd Iy Ionc naiiov suics-
< not tor sale ! > < ue pn n]opa ! >
sior ircIudechenofheraeufic agerfs acquiredviraI
irIecfiors [II\, Lsfein-Barr virus, neasIes) cor-
genifaI inIecfions, incIuding foxopIasnosis, syhilis,
ruIeIIa, cytonegaIovirus, ard arvovirus |I 9, and
cerfair drugs [anficonvuIsanfs, suIIonanides, quiri-
dine,quirine,fhiazidediuretics) .IcquiredosfrataI
irIecfions,vifhfheexcefionoIII\ anddrugreac-
fions usualIy cause fransienf fhronIocytoenia,
vhereas corgerifal irIecfiors nay roduce ro-
Iorged suressior oIlore narrovIurcfior.
ThronIocytoeria caused Iy shortened IafeIef
survival is nuch nore connor fhar fhronIocy-
fopenia caused Iy inadequafe producfion. Ilafelef
desfruction ls nosf connonIy innure nediated.
ThronIocyfoenia in fhe nevIorr car resuIf Iron
isoinnune or aufoinnune anfiIodies. I soinnure
IgC anfiIodies are roduced agairsf fhe IefaI
IafeIefsvher fhe IefaI IafeIefcrossesfheIacerfa
ard reserfs ifseIIfo fhe naferraI innure sysfen.
II fhere is ar anfigen or fhe IefaI IafeIef fhaf does
rof exist on fhe nafernaIIateIef, if viII le recog-
rized as Ioreign and isoinnure antiIodies viII Ie
creafed againsf fhe anfiger. MafernaI arfiIafeIef
arfiIodies fhen cross fhe Iacerfa, causing desfruc-
fioo oIfhe Iefal Iafelef. This disorder is knovr as
nCona!al 8oDDunC !hroDDoCj\oCnC urura.
ThenaterraIarfiIafeIefartilodydoesrot roduce
naferraI fhronIocyfoeria. Iufoinnure IgC
anfiIodies are frarsIerred fo fhe Iefus fhrough fhe
Iacenta vhen fhe nofher has idioafhic fhronIo-
cytoenicurura,sysfenicIuuserythenafosus,or
drug-induced fhronIocytoenia. In aII fhree cases,
nafernal aufoanfiIodies cross fhe Iacenfa ard
affack fhe IefaI IafeIefs. In confrasf fo isoinnune
anfiIodies, aufoinnune anfiIodies aIso resuIf in
nafernaIfhronIocytoenia.IIterIirfh,lnIarfsvifh
severe isoinnune or aufoinnure fhronIocytoe-
nia nay Ie treated vifh corticosteroids or infra-
verous innunogIoIuIin unfiI fhe naferraI
anfiIafeIef anfiIodies dissiafe. I defaiIed discus-
sionoIchiIdhoodidioafhicfhronIocyfoeniaur-
ura [ITI) aearsIaferir fhis chafer.
Microangioafhic henoIyfic arenias aIso cause
fhronIocyfoenia Iy decreasing IafeIef survivaI.
Nlicroangioafhic disorders ircIude disseninafed
irfravascuIar coaguIafion [IIC), henoIytic-urenic
syndrone [IIS),andfhronIoficfhronIocyfoenic
urura [11I) . IICis discussed Iafer. IIS, charac-
ferized Iy a nicroangioafhic henoIytic anenia,
reral cortical irjury, ard fhronlocyfoeria, is H
najor cause oI acufe renal Iailure ir chiIdrer.
\erofoxir-roducirg gran-regafive organisns, such
asscbercbin co|i CI 57. I7, fhatllrdfo erdoflelial
ceIls cause IIS. |ecause oIerdofheliaI ceII injury,
fhere is IocaIized cIoffing and Iafelef acfivafion.
Microargioafhic henolyfic arenia resuIfs Iron
necharical lnjury fo red celIs as fhey ass fhrough
the irjured vascuIar erdofheIiun ard fhronIocy-
toeria resuIfs Iron IafeIef adhesior fo fhe
danaged erdofheIiun. Sixfy ercenf to S0% oI
afierfs vifh IIS fransiently requ|re diaIysis. Mosf
chiIdrensurvive fhe acufe hase ardrecoverrornal
renal Iurcfior. IrT1I,Iafelef consunfior reci-
ifafedIy aIasnaIacfor or fhe IackoIan irhiIifory
Iacfor appears fo Ie the prinary process. There is
noderafe deosifior oIlIrir, vhich causes red ceIl
desfrucfior.
Iinirished IafeIet survivaI canaIsoresulf Iron
lafeIef trairg, as seen vifh giarf henargionas
ard hypersIerisn. IyersIerisn nosf connorly
occurs secondary fo sickle ceII arenia, fhaIassenia
syrdrones, Caucher' s disease, and ortaI hyer-
fersior. TaIle I 0-6 lisis the connor causes oI
fhronIocytoenia durirg fhe neonafaI, inIarf, and
chiIdhooderiods.
M
KEY O| N15
W
1 . Abnorma| hemostat|cp|ugformat|onoccurs| n
pl ateletd|sorders.
2. P|ate|etd|sorderscanbee|therquant|tat|veor
qua||tat|ve,andthrombocytopen|a|sthemost
common causeofabnormalb|eed|ng.
J. Thrombocytopen|acausedbyshortened p|atelet
surv|val| smuch morecommonthan thrombocy
topen|acausedby|nadequate product|on and |s
dueto |so|mmuneant| bod|es,auto| mmuneant|
bod|es,andm|croang|opath|chemolyt|canem|as.
0DQ0lC fDDDCylDQPOC UfQUf0
ITI reIers io afhronIocytoeriaIorvhich a cause
is nof aarert. ITI resuIfs Iron fhe deveIonerf
oI anfilafeIef anfiIodies fhaf Iind fo fhe IateIef
nenIrare.These arfiIody-coafed IafeIefs arefher
desfroyed ir fhe reficuloerdofheIiaI sysfen. RareIy,
ITI nay Ie the reserfirg synfon oI ar aufoin-
nure disease, such as sysfenic Iuus eryfhenafosus
or II\ irIecfior.
Cl | n|cal Man| festat|ons
ChiIdrer fyicaIIy reserf 1 fo4 veeks alier a viraI
iIIress vifh aIruf orsef oI etechiae and ecchy-
< cau v pexanx~ouee]cn: HCA o ]ou. ]
1AbLL 1 -
LuSeS ol 1h|omb0ytoen
NOOH\O
N\O|H| ||" bLL,O|UQS, QlOOC|HQS|
|SO|HHUHO"
LOHgOD|\| HOQK|yOCy\|C |yQOQ|S|
[I|lOHDOCy\OQOD| DSOH\ |O|US)
L|D\ DOHDg|OH
bOS|S"
L|L
LOHgOH|\| |HOC\|OHS
| HH\
W|SKO\\P|O||C| SyDO|OHO
V||| |HOC\OHS"
NOO|C\|OHS
N||QHDCOS[|OUKOH|,DOU|OO|S\OH)
MOHO|y\|CU|OH|C SyDO|OHO
bOQS|S
||
L||| O|OOO
||"
NOO|C\|ODS"
P|S\CDOH
LOUKOH|"
MyQO|SQ|ODSH [\||SSOH|,LUC|Ol'S O|SOSO,
QO|\| |yQO|\OHS|OD)
bOQS|S
bLL
V||US|HOUCOO |OHOQ|gOCy\|C SyDO|OHO
||w|\| UO|HHUHO |OHO|y\|C HOH| [LVH`S
SyHOlOHO)
P|Lb
LDmmD.
hPtUtDttC t|DmDDCytDeC ufuf, 5LL, Sy$temC uu8
ery\emtD$u8, LL, Ut88emtteU ttIv8Cuf CDgutD, PlLb,
CqufeU mmuDUeDtteCy 8yUfDme.
nosesontheskIrardIleedirgoIthenucousnen-
Irares. SevereIIeedirgoccursaItertrauna. Sonta-
neous interral henorrhage, though rare, has Ieen
notedvith IatelotcountsIeIov I0, 000/nn'.
Cther thar thronIocytoenia, thc conIete
IIood count L nornaI. Large Iatelets are seen on
eriheral IIood snear, ard seroIogy reveaIs arti-
IateIetartiIodies.IiagrosisolITIdoesrotrequire
aIonc narrov asirate. Iovever, iIthere are aty-
icaI lndirgs on either the conIete IIood court or
eriheraIIIoodsnear,narrovexanirationisirdi-
cated to excIude Ioukenia and idioathic aIastic
arenia. In ITI, Ione oarrov asiratior reveaIs
rornaI nyeIoid ard erythroid elenerts ard ar
increased runIer oInegakaryocytes.
JlOHOD
IroxinateIyS07 oIthecasesoIacuteITIresoIve
sontaneousIy vithin 6 nonths. Sone cases, hov-
ever, Iecone reIasirgor chroric.
CIinicaIIy sigrilcanf IIeedirg orsevere thronIo-
cytoeria [IateIetcourtIessthar20, 000) istreated
vith high-dosc steroids, irtraverous innunogIoIu-
Iins [I\IC), or arti-I innure gloIuIir [in Rh-
ositive chiIdrer) . These neasures alI decrease the
duratior oI severe thronIocytoenia Iy decreasirg
the rate o|cIeararce oIartiIody-coated IateIetsir
the reticuIoendotheIiaI systen Iut do not dinirish
the roduction oI anti|latelet antiIodies. lone oI
theseneasuresaI!ectstheIong-ternoutconeoIITI.
Chronic ITI, dehned as thronIocytoenia con-
tiruingIor nore than I 2 norths aIter ar acute ITI
eisode,istreatedvithI\ICorsIenectonyorIoth.
Roeatedtreatnentsvith I\IChaveIeen eIIective
in deIaying sIenectony, vhereas sIerectony
irducesrenissionu 707 to S0% oIcases oIchroric
ITI. Ir reIractory casesinvhichsteroids, I\IC,arti-
innure gIoIuIin, and sIenectony have IaiIed,
innunosuressionvithazathiorineorcycIohos-
hanide and Iasnaheresis nay Ie irdicated.
Inicar, a drug thaf irhiIits lIriroIysis, ls used to
treat uncortroIIed eistaxis ardoraI IIeedirg.
KEY O| N15
1 . |OOQD|CDlOHOOCyOQODC QUlQUl lOSUS
lOH UO HHUDO D| OOOyOlHOD QDS\
DOSQ| OOS.
2. PQQlOX|HO|yU% OCSOS OCUO |J| lOSO|VO
SQODDOOUS|yw|DD HODDS.bOHO CSOS,
DOWOVOl,OOCOHO lOQS|DQ Ol CDlODC.
3. LDC| | ySQDHCD O|OOODQ OlSOVOlO |lOHOO~
CylOQOD [Q|\O|OCOUD |OSS DD 20,000J |S
lOOO w|D DQDOOSO SOlOOS,|DlVODOUS
| HHUDOQOOU|DS,DODLQOOU| |D.
4. LDlODC J SlOOOwDDlVODOUS
| HHUDOQOOU| DSOlSQ| ODOCOHyOl OOD.
bQ| ODOCOHy DOUCOS lOHSS|OD D U% O U% O
DO CSOS OCDlOD|C |J
LSSPO0lP0 Olf0v0SCU0f LD0gU0lDO
lornaI honeostasis is a IaIance Ietveer henor-
rhage ardthronIosis. Ir IIC, thisIaIarceisaItered
Iy severe iIIness so thaf the atiert has activatior
ol Ioth coaguIatior [thronIin) ard IIrinoIysis
[lasnin). LrdotheIiaI irjury, release oI thronIo-
< not tor sale ! > < ue pn n]opa ! >
< cau v pexae~ouee]cn: HYCA o ]ou. | >
Iasfic rocoaguIanfIacforsinfofhe circuIafion, and
inairnenfoIcIearanceoIacfivafedcIoffingIacfors
direcfIy acfivafefhe coaguIafion cascade. Irfravascu-
Iar acfivafion oI fhe coaguIafior cascade leads fo
IIrin deosifion in fhe snaII IIood vesseIs, fissue
ischenia,reIeaseoIfissuefhronIoIasfin,consun-
fior oI cIoffing Iacfors, and acfivafion oI fhe IIri-
noIyfic sysfen. CoaguIafior eIenenfs, eseciaIly
IafeIefs, IIrinoger, and cIoffirg Iacfors I!, \ and
\II!,areconsuned,asarefhearficoaguIanfrofeins,
eseciaIlyanfifhronIir !!I, rofeinC, andlasniro-
gen. Icufe and chronic condifions associafed vifh
I!C incIude sesis, Iurns, frauna, ashyxia, naIig-
nancy, and cirrhosis.
Cl | n|cal Man|fesIaI|ons
TheIIeedingdiafhesisisdilluse, vithIIeedingIron
veniuncfure sites and around indvelIing cafhefers.
CasfroinfesfinaI ard uInonary IIeeding can Ie
severe, and henafuria is connon. ThronIotic
lesions aIecf exfrenifies, skin, kidneys, ard Irain.
Bofh ischenic ardlenorrhagicsfrokecar occur.
ThediagnosisoI I!CisacIinicaIoneIoIsferedIy
laIorafory evidence. TlronIocytoenia is eviderf,
aIong vifh rolonged rofhronIin fine [IT) and
arfiaIfhronIolasfinfine [ITT) .!iIrinsIifrod-
ucfsandd-dinersareeIevafed.!iIrinogenandIacfor
\ard\!IIIeveIsareIov.TheerileraIIIoodsnear
reveaIs schisfocytes, vhich are classicaIIy seer vifh
nicroangioafhic disease.
TreatmenI
The freafnenf oI I!C is suorfive. The disorder
fhaf caused IIC nust Ie freafed, and hyoxia,
acidosis, and erIusion aInornaIifies need fo Ie
correcfed. !I IIeeding ersisfs, fhe chiId shouId Ie
freafedvifhIafelefsandIreshIrozenIasna,vhich
reIaces deIefed cIoffing Iacfors. Iearin nay Ie
useIuIinfheresenceoIsigniIcarfarteriaIorverous
fhronIofic disease unless sifes oI liIe-fhreafering
IIeeding coexisf.
$ KEY O|N15
1 . D|ssem|nated |ntravascularcoagul at|onresulIs
from severe| l |ness,caus|ngact|vat|onofboIh
coagulat|onthromb|n)andhbr|nolys|splasm|n).
2. l ntravascularact|vat|onofthecoagulat|on
cascadeleadstohbr|ndepos|t|on| nthesmal |
b|oodvessels,t|ssue|schem|a,releaseoft|ssue
thromboplast|n,consumpt|onofclott|ngfactors,
andact|vat|onofIhehbr|nolyt|csystem.
Chapter 1 Henatology 1 21
LOOC5 O DO LOB@UBOD LB5CBOO
CoaguIation disorders can Ie inherifed or acquired.
ThenosfconnoninheriteddeIects arelenohiIia
I and B and von ViIIeIrand's disease, vlereas
vifanin K deIciency is an inortanf acquired co-
agulafiondeIecf.
HPDQm0 P 0O0
IonoliIia1 is caused Iy deIcioncy oIlacfor\I!I
and occurs in ! in 5000 naIes, vlereas henohiIia
B resulfsIron IacforIX deIciercyandisIoundu I
ir 25, 000 naIes. Bofh are X-Iinked recessive disor-
ders. III ofher cIoffing |acfors are coded on aufoso-
naI chronosones and are fhereIy inherited in ar
autosonaI Iashion. Tle Iack ol lacfor \!!I or !X
causes H deIayirfleroducfionoIflronlin,vhich
cafaIyzesfheIornafior oIfhorinaryIIrincIotIy
fhe conversior oIIIrinogen fo IIrin and sfaIiIizes
fhe IIrinIyacfivafirg|acforX!II.
Cl | n|ca|Man|festaI|ons
CfherfhanfleirIacforrelacenerfreginens,heno-
hiIiaIandBareindistinguishaIIeclinicalIy,andthe
severifyoIeacldisorderisdeferninedIythedegree
oIIacfor deIciercy. CliIdrer vifl niId henolilia
[5% fo 49v oI nornaI Iacfor) require signiIcanf
trauna to induce Ileedirg, and sonfaneous IIeed
ing does not occur. Iatienfs vifh noderafe heno-
hiIia [I %to 5% oInornaIIacfor) requireooderate
frauna fo induce Ileedirg eisodes. Severe heno-
liIiacs [chiIdrerviflIessfhanI%oInornal|acfor)
nay have sonfaneous bIeeding and vilI IIeedvifh
very ninor trauna. MiId lenoliIia nay go undi-
agnosed Ior nanyyears, vhereas sevore henohilia
naniIesfs ifseIIdurirg inIancy. IenohiIia is char-
acierizedIysonfareous ortraunafichenorrhages,
vlich canIe suIcutaneous, intranuscuIar, orvifhin
joinfs[henartlroses) .LiIe-flreaferinginferraIhen-
orrhage nay IolIov frauna or surgery In revIorns
vifh henohiIia, fhere nayIe infracraniaIIleeding
secondary to fraunafic delivery or alter circunci-
sior, otlervise, Ileeding conpIicafions are uncon-
non u fhe Irst year oIliIe. Circuncision slouId Ie
avoided |nIoys vifh aIanilylistory oIhenolilia.
In Iofh |orns oI lenohiIia, fhe ITT is ro-
Ionged. IrhenohiIiaI,Iacfor\!!IcoaguIarfacfiv-
ify[\III. c)islov,vhereasinhenohiIiaB,IacforIX
activify is Iov. TaIle I 0-7 conares henoliIia A,
henohiliaB,andvorViIleIrand'sdisease.
TreaImenI
Thegoal oIflerayistorevenfIorg-ferncriling
orfhoedic injuries due to henarthroses. Mos:
< not tor sale ! > < ue pn nopa ! >
1 22 |lucpr|nlO |OO\|CS
< cau v pexanxouee]cn: HYCA o ]ou. ]
1AbLL 1 7
LomtSo ol Hemoh P, Hemoh ,0 vo Wlebt0S LSeSe
HOmO_h A HOm0@h V0n WeDfn0S OSOSO
| HO|\HCO Z|HKOO
|C\Ol OODCOHCy |C\O|V|||
b| OOHgS\O[S) NUSC|O,jOH\,
SUlgC
|1 NOlH
P| ||O|OHgOO
b|OOO Hg\HO NOlH
|C\O|V||| COgU|H\ C\V\y [V|||.L) LOw
VW|.Pg NOlH|
VW|.PC\ NOlH|
|C\Ol |Z NOlH|
hS\OCO\HHOUCOO Q\O|O\ gg| U\H\OH NOlH|
||\O|O\gglOg\OH NO|H|
atientsrequireeriodicorreguIarinIusionsoIIactor
\I!! or!X to raisetheirIactorIeveIshighenoughto
stotheIIeeding.VIiIeIasna-derivedIactorsvere
used in the ast, reconIinant Iactors \I!! and !
are nov avaiIaIIe. For niId to noderate IIeeding
eisodes, such as henarthroses, a 40% IactorIeveIis
aroriate. !or IiIe-threatening IIeeding, IeveIs oI
S0% to | 00%oInornaIIactors\IIIandIXarenec-
essary. Iesnoressin acetate [III\I), a synthetic
vasoressir anaIogue, reIeases Iactor \!!! Iron
endotheIiaI ceIIs. Vhen adninistered, it triIes or
quadrules the initiaI Iactor \III leveI oIa atient
vith henohiIia I Iut has no el!ect on Iactor !X
IeveIs.IIadequatehenostaticIeveIsoIIactorV!!Ican
Ieachievedvith III\I, it is the initial treatnert
oIIIeeding Ior those ahlicted vith nild to noder-
ate henohiIia I. Since |II\I is an antidiuretic
IornoneanaIoguo,henohiIiacsvhoIrequentIyuse
III\I shouId Ie nonitored Ior hyonatrenia
caused Iy vater retertion. MiId acute IIeeding
eisodescanIetreatedinthehoneoncetheatient
Iasattainedthearoriateageandthearentshave
learned hov to adninister reconIirant Iactor \III
or !X or II1\P. BIeeding associated vithsurgery
trauna, or dentaI extraction can Ie anticiated,
andexcessivelIeedingcanIerevertedvitharo-
riate reIacenent theray. Ininocaroic acid
[Inicar), ar inhiIitoroIIIrinoIysis, nay heItreat
oralIIeedingaIter a dentaI rocedure. h is generaIly
givenIeIore andaIter therocedure.
Z|HKOO PU\OSOH| OOHHH\
|C\O| |Z VOHW||OO|HO C\Ol HOV|||.L
NUSC|O,jO H\, NUCOUS HOHOlHOS,SKH,
SUlgC| SUlgC|,HOHS\lU|
NOlH NOlH
|lO|OHgOO |lO|OHgOO Ol HOlH|
NOlH| |lO|OHgOO OlHOlH|
NOlH| LOwOl HOlH|
NOlH| LOw
NOlH| LOw
LOw NOlH|
NOlH| NOlH| Ol|Ow
NO|H| NOlH|
Testing oIlIood roducts Ior I!\ and heatitis
viruses did not Iegin untiI the nid- I 9S0s, and as a
resuIt, nany henohiIiacs contracted the viruses.
Betveen I 979 and I 9S4, 90% oIhenohiIiacs vho
received lasna-derived Iactor roducts Iecane
II seroositive. Icquired innunodeIciency syn-
drone is the nost connon cause oIdeath in oIder
atients vith henohiIia. lever ooIed concen-
trates aresaIer, andaIIreconIinantrearatiorsare
conIeteIysaIeIronviraIagents.
InothersigniIcantconIicationoItherayisthe
Iornation oI irhiIitors, vhich are !gC artiIodies
directed against transIusion Iactors \!!I and !X.
!nhiIitors arise during theray u I 5% oI those
atients vith Iactor \!!! deIciency and in 1% oI
those vith Iactor !X deIciercy. The treatnent oI
IIeeding atients vith ar inhiIitor is diIlcuIt.
!or Iov-titer inhiIitors, otions incIude continuous
Iactor VIII irIusions or adninistration oI orcine
Iactor \!II. For high-titer inhiIitors, it usuaIIy is
necessaryto adninister a roduct thatIyasses the
inhiIitor, such as activated rothronIin conIex
concentratesorreconIinantIactorV!Ia.The use oI
Irequent high doses oIrothronIir conIex con-
centrates, ard eseciaIIy oIthe activated roducts,
aradoxicaIIyincreasestherisksoIthronIosis,vhich
hasresuItedir IataInyocardiaIinIarctionandstroke.
!nduction oI innune toIerance vith continuous
antigen exosure Ius innunosuression nay Ie
IeneIciaI.
< not toz sale ! > < ue )7H .]o)B ! >
P' KEY O| N15

1 . Hemoph|l|aA resultsfromadehc|encyoffactor
Vl l l ,andhemoph|||a 6from a |ack offactor lX.
6othd|sordersare| nher|ted| nanX-||nkedreces-
s|vefash|on.
2. Otherthanthe|rfactorrep|acementreg|mens,
hemoph|l|aAand 6are|nd|st|ngu|shab|ecl|n|
cal |y,andthesever|tyofeachd|sorder|sdeter-
m|nedbythedegreeoffactordehc|ency.
J. Hemoph|||a |s character|zed byspontaneous
ortraumat|chemorrhages,wh|chcanbe
subcutaneous,|ntramuscular,orw|th|njo|nts
hemarthroses).||fe-threaten|ng|nternalhemor-
rhagemayfollowtraumaorsurgery.
vDO WhPDf0O0! LSP0SP
von VilIelrard's disoase is caused Ly deIciency oI
von VilIeLrand |actor [vVF), ar adhesive rofeir
fhaf corrects suLendofheIiaI coIIagen fo acfivated
lateIefsandaIsoLindsfocircuIatingIactor\II!,ro-
fecfing if Iron raid cIearance. von VilIeLrand's
disease is cIassiIed on fhe Lasis o| vhefher vVF
is quanfifafiveIy reduced Luf nof aLsent [fye I ) ,
quaIitafiveIy aLrornaI [fye 2, dysrofeinenia), or
aLsenf [fye J).
Cl | n|cal Man|festat|ons
The cIiricaI nariIesfafions oI vor VilIeLrand's
diseasearesiniIartofhoseoIflronLocytoeniaand
include nucocufaneous LIeeding, eisfaxis, gingival
LIeeding, cutaneous Lruising, and nenorrhagia. Ir
severevonViIleLrand'sdisease,Iacfor\IIIdeIciency
nayLeroIoundandfheafienfnay aIsohavenan-
iIesfafions sinilarfo fhose oIlenohiIiaI. !Itlere
is IiffIe or no vVF in fhe lloodfo lind Iacfor\III,
Iacfor \I!! is cIeared quicky Iron tle circuIafion,
rosulfing in Iacfor \I!I deIciency. IroxinafeIy
S5% oIafients vifh von ViIIelrand' s disease have
classic fye I disease, vhicl resuIfs in niIdfo nod-
erate deIciencyoIvVF.
Lalorafory testirg ircIudes neasuronert oI
the anount oI profein, usuaIIy acconlished Ly
innunoIogic defecfion oI vV! anfigen [vV!.Ig)
and vV! activify [vVF. Icf) . vVF acfivity is nea-
sured IuncfionaIIy ly fhe risfocefin coIacfor assay
[vV!. RCoF),vhich uses flo arfiLioficrisfocefinfo
,
inducevV!folindfoIafeIefs.TheatierffyjicalIy
has roIonged LIeeding tine, due fo fhe eIIecf o|
vV! deIciencyorIafelef acfivity, andaroIorged
PJ,vlichresuIfsIronfheeIlecfoIvVFdeIciency
on Iacfor VIII acfivity IrTaLIe I 0-7, tle Indings in
cIassic von ViIIeLrand's disease are conared vith
those in henohiIiaI and B.
Treatment
Thefreafnent oIvonWilIelrard's disease deends
on the severify oILIeeding. III\I, vhicl sfinu-
lafesfhereIeaseoIvVFIronendotleIial ceIIs,isfhe
treafnenf oI choice Ior LIeeding eisodes in nosf
afienfs. Pafienfs vifh fye J disease [vho have
no vVF fo reIease) or vifl severe Lleeding rof re-
sondirg fo III\I car Le freafed vith a viraIly
afferuatedvV!-corfainingcorcenfrafe[IunateI) .
Cryoreciitate nay also le usod, Luf if cannof le
vlraIIy atfenuafed. Ieafifis B vaccine shouId Le
glven leIore exosure fo Iasna-derived producfs.
Is in aIl LIeeding disorders, nedicatiors thaf
aIter Iafelef |uncfion, such as asirin, shouId Le
avoided.
~
KEY O| N15
1 . vonW|llebrand'sd| sease|scaused bydehc|ency
ofvonW|l|ebrandfactor,anadhes|veprote|nthat
connectssubendothel|a| collagentoact|vated
plateletsand a|so b|ndstoc|rcu|at|ngfactorVlll,
protect|ng|tfrom rap|dc|earance.
2. Thecl|n|calman|festat|onsofm|ldtomoderate
vonW|||ebrand'sd|seaseare s|m||artothoseof
thrombocytopen|aand|nc|udemucocutaneous
b|eed|ng,ep|stax|s,g|ng|valb|eed|ng,cutaneous
bru|s|ng,and menorrhag|a.
J. l nseverevonW|l lebrand'sd|sease,factorV|ll deh-
c|ency maybeprofound andthepat|ent mayalso
haveman|festat|onss|m|lartohemoph|||aP.
4. DDAVP |sthetreatmentofcho|ceforthemajor|ty
ofb|eed| ngep|sodes|npat|ents.
Vl0O h LP0CPOCy
CoaguIafionIacfors [Iacfors II, \II, !X, ard X) ard
anfifhronloficIacfors [rofein Cand roteinS) are
synfhesizodinfheIiveranddeendonvifanin K Ior
their activify. Vher vifanin K is deIcienf, coagula-
tion is lnaired. \ifanir K deIciency oIen occurs
Lecause oI naIaLsorfion, eseciaIIy vifh cysfic
Ilrosis ard vifh anfiliotic-induced suression oI
intesfinaILacferiafhafroducevifanin K. Cverdose
oIcounadin, a drug fhaf interIeres vifl vifanin K
< Co V peaen~ouee]cxn: HCI o pou.
J
>
netaIolisn, causes deIciency oIvitanin Kdeen-
dent |actors. SiniIarIy, naterraI use oIcounad|nor
anticonvuIsant theray [henoIarIitaI, henytoin)
nay also resuIt in vitanin K deIciency in the
nevIon.ThenostconnondisorderresuItinglron
vitanin K deIciency is henorrhagic disease oIthe
nevIorn vhich occurs in neonates vho do not
receivei.tranuscuIar vitanin K atIirth.
C| |ni ca| Man|Iestat|ons
IIthough nost revIorn lnIants are Ion vith
reducedleveIs oIvitanin KdeendentIactors, onIy
a Iev deveIo henorrhagic conIications. Because
IreastniIkis aoorsourceoIvitaninK,Ireast-Ied
inIants vho do not receive rohyIactic vitanin K
ontheIrstdayoIIiIeareatthehlghestriskIorhen-
orrhagic disease. Peaklncidenceis at 2 to I 0 days ol
IiIe.Thereconnendedreventive doseoIvitaninK
is I . 0ng given intranuscuIarly. The disorder is
narked Iy generaIized ecchynoses, gastrointestinaI
henorrhage,andIIeedinglronthecircuncisionsite
ardunIilicaIstun.IIectedneonatesareatriskIor
intracraniaI henorrhage.
Both the! andIT are roIonged invitanir K
deIciency, Iecause Iactors oI Ioth extrinsic and
intrinsicathvays are aIlected. ProIongation oIthe
P a nore sensitlvetest Iorvitanin K deIclency
Iecause nost inIants have transient roIongation oI
the IT1 at Iirth.The coaguIoathy seen vith hen-
orrhagic disease nayIeconIused vlth Iiver disease
or I!C, Ioth oI vhich have a roIonged IT and
decreased Iactor\l! IeveI. TaIIe I 0-S diIlerentiates
vitanin KdeIciency, liver disease, and |!CIyIaIo-
ratorydata.
Treatment
lutritionaI disorders and naIaIsortive states
resond to arenteraI adninistratior oI vitanin K.
!resh lrozen Iasna orrothronIin conIex con-
centrate,vhichisanixtureoIcoaguIationIactorsI!,
\!I IX andX lsindicatedlor severeIIeeding.
7
,
AbLL 1 -b
L!etetto o! Vtm K LeCeCy, Lvet
LSeSe, 0 D|C
LD0ft0f Vtmn K LVef LL
1eS1 OecOn@ OSeSe
| 1 T T
|lte|ets H| 11O nl 1
|OlHOgOH H| 1 1
|C1OlV||| n| H\O1 1
|OlHOgOH
OOglO1OH H D 1OT T
QlOOUC\S
|C1OlV|| 1 ! 1ton|
n|, norma|.

KEY O| N15

1 . Coagu|at|onIactors,V, A, andA andant|throm-


bot|cIactorsprote|nCand prote|nSaresynthe
s|zed| nthe||verand dependon v|tam|n KIor
the|ract|v|ty.
2. The most commond|sorderresult|ngIrom
v|tam|nKdehc|ency|s hemorrhag|cd|seaseoIthe
newborn,wh|choccurs| nneonateswhodonot
rece|vev|tam|nKatb|rth.
J. |ecoagulopathyseenw|th hemorrhag|cd|sease
maybeconIusedw|th ||verd| seaseorDlC,bothoI
wh|ch havea pro|ongedPTanddecreasedIactor
V| leve|.
LPLLLLX
The innune systen, conosed oI seciaIized ceIIs
and noIecuIes, is resonsilIe Ior recogrizing and
neutraIizingIoreignantigens.SeciIcconIexinter-
actions roduce adative inIannatory resonses
and deIerd against inIection. mu1:tnOUCHCCnCy
8ynUrOmC8 increase suscetiliIity to inIection,
naIignancy, andautoinnunedisorders[TalIe I I -I ).
!nIortunately, evena"nornaI"innunereactionnay
resuIt in undesiralIe consequences, such as tissue-
danagirginIlannation,IiIe-threateninganahyIaxis,
or graIt rejection.
L5OlOOl5 O MUDOlB DDUDy
B ceIIs roduce antilodies, the rinary eI|ectors oI
hunoraIinnunity.IntilodiesareavitaIcononent
oItheinnunesysten,articuIarIyindeIenseagainst
extraceIIuIar athogens sucl as encasuIated lacte-
ria. I variety oI antilodies activate conIenent,
serve as osorins, inhilit nicrolial adherence to
nucous nenlranes, ard neutraIize various toxins
ard viruses. Is a grou, -CCll mmunOUCHCCncy
8ynUrOmC8 are the nost connonIy encountered in
ediatric ractice.
LOC0 %0OPSl0lDOS
H|sIOryandPhys|cal Lxam|naI|On
I history oI recurrent inIections vithCnCa8ula1CU
organisns such as Hoenobi/us in)uenzoe andStre-
tococcu: neunonioe ard IaiIuretoresondto aro-
riate antiliotic theray is suggestive oI a rinary
B-ceII deIciency. !n addition, there is sonetines a
historyoIIrequentuerresiratorytractinIections,
incIudingotitis nedia, sinusitis, andneunonia.
LPfPOl0 L0gODSS
`-lnKCU [ru1On8} agammaglODulnCma occurs in
naIes and aears aIter 6 nonths oIage as nater-
nalIy derived antilody IeveIs IalI. These atients do
rotroduceantilodies and have virtuaIIyro B ceIIs.
In addition to their suscetiliIity to encasulated
organisns,individuaIsviththisdisorderareroneto
severe, oIen IiIe-threateningenterovirus inIections.
LOmmOn varaDlC mmunOUCHCCnCy is H inher-
iteddisorderolhyogannagIoluIineniavithequaI
distrilution letveen the genders. InIections are
usuaIIy Iess severe, hovever, the incidences oIIyn-
hona and autoinnune disease are increased in
theseatients.
hClCC1vC gP UCHCenCy is the niIdest and mosi
connon innunodeIciencysyrdrone. Serun IeveIs
oI the other antilody cIasses are usuaIIy nornal.
Iatients react nornaIIy to viraI inIections lut are
nore suscetilIeto lacteriaI inIections oIthe resi-
ratory, gastrointestinaI, andurinarytracts.
L0gODSlC Lv0U0lDO
uartitative neasurenentoItotaI and Iractionated
seruninnunogIoluIinIeveIsisaninortantscreen-
ing test Ior seciIc deIciencies and Ior arhy-
ogannagIoluIinenia. Intilody titers generated
agairst tetanus, dihtheria, and neunococci aIter
innurization assess artilodyIunctior.
fP0lPOl
Thenairstays oItheray are aroriate antiliotic
use and eriodic gannagIoluIin adninistration.
not tor sale ! > < ue pn npopa ! >
1 2 |lucpr|nlO |OO\lCS
AbLb1 1 1
LllC LhtCtetlStlcS ol mmue Lomo0t LeHLleceS
L0n0t0nS be@uee LD0ft0_ 1eStS
LSOlOOlSOUHOl|
HHUH\y
|lOQUOH\ lOCUl|OH\, QyOgOHC HOCIODS w\
OXlCO||UlOHCQSU|\OO OlgHSHS
|lOQUOH\ OC\O|O\\SHOO,SHUS\S,HO
QHOUHOH
[UH\\\VOHO QU| \\VO
HHUDOg|OOU|H |OVO|S
|gOCy\C OSOlOOlS hOCUllOH\ SKH HOC\OHS,OSCOSSOSw\| C\SO
QOS\VO OC\Ol,I5CuOOOtO5,UHg
POSO| U\O HOU\|OQ | COUH\
[PNL)
POSCOSSOlH\OH DI|O |UHg,| VOl,HO |yHQ
HOOOS
LL|SSy
PO|OSOH HOC|OHOC\C
OC\Ol| SSyS LUCU|\\OC|OlOOHO, jOHI HOCIOHS
LSOlOOlSOCP||HOO\OO
HHUHIy
|lOQUPH\,lOCUllOD\HPC\OHSw\
OQQOl\UHS\C Ol |OwglOOO|gHSHSHO
VlUSOS
POSO|U\O |yHQ|OCyIO COUH\
[PLL)
N\OgOH S\HU|IOD
lOSQOHSO | HCfOSOO HCOOHCOOU\OHHUHOOSOlOOlS
HO H|gHHCOS LO|yOO yQOlSOHS\V\y
SKH \OS\Hg PDOlgy
LOHQ|OHOH\ OSOlOOlS hOCUllOH\ OC\Ol| HOCIODSw\| QyOgOHC,
OX\lCO||U|l,OHCQSU\OO OlgHSHS
O\| |OHO|yICCOHQOHOHI
[LM
so
)
|DClOSOO SUSCOQIO|\yIO HODHgOCOCC| OSOSO
|HClOSOO HCOOHCOOU\OHHUDOOtSOSO
PSSySO\|O C|SSC| HO
\OlH\VOQ\DwyS
n!ravCnou8 mmunCgoDuln [J) rovides anii-
lody reIacenenf and has revoIufionized fhe freat-
nenf oIIunoral innunodeIciency syndrones.
BD5OD MyQO@BDDB@OOUDODB
O DBDCy
IIfhoughnafernaIIgCisactiveIyfransorfedacross
fhe Iacenta and is rofeciive throughouf the Irsf
IevnonthsoIIiIe, neonafes areconsideredreIativeIy
innunoconronised hosfs. All serun innuno-
gIoluIincIassesareresentatlirih,lufnosfdonot
reach aduIf IeveIs unfiI earIy to niddIe chiIdhood.
CverfheIrsf6fo SveeksoIIiIe, nafernaIIyderived
innunogIoLulins decrease and are reIaced Ly the
chiId's groving roducfion. Thus, inIants are arti-
cuIarIysuscetilIefoinIecfionaf6fo ! Z veeks,fheir
innunologicnadir.
ran8Cn! hyogammaglCDulnCma o nanCy isa
recognized disorder |n vhich acquisifior oInornaI
inIant innunogIoluIin leveIs is deIayed. IIfhough
sone oI fhese atients are sulsequenfly diagnosed
wifh otherrinaryinnunodeIciencies,nosteven-
fuaIIydeveIonornaI innunify.
KEY O| N15
1 . Most| mmunodec|encysyndromesencountered
|n ped|atr|csarehumoral.
2. Humoral|mmunodehc|encypred|sposespat|ents
to|nfect|onw|thencapsulatedorgan|sms.
J. Qunttt|Ve|mmunog|obul|nstud|esandant|
bodyt|tersaga|nstvacc|netox| nsareabnormal | n
pat|entsw|th humoral| mmunedysfunct|on.
4. |V|Lprov|desant|bod|estoQa\|Cntsw|thhumoral
| mmunodehc|ency.
L5OOO5 O LO~NOOBOO DDUDy
T ceIIs noduIatenosf innune resonses, rinarily
fhrough the secrefion oI inferIeukIns. ln addifior,
fhey are major eIIecfors oIceII-nediated innunify,
inoriarf in fhe deIense againsf intracelIuIar and
ooriunistic inIections. Cerfain sulcIasses are
caalIe oI kIIIing tunor and viraI-inIecied ceIIs.
Patienfs vifh dysIunctionaI T ceIIs are af increased
riskIor autoinnunedisorders.T-ceIl diseasesgener-
aIIy inarf signiIcantIy greater norlidiiy and nor-
falify fo their victins fhan hunoraI disorders aIone,
< not tor sale ! > < ue pn npopa ! >
< cau v pexapx~ouee]cn: HYCA o pou. ] >
L|Q\O| 1 1 | HHUDO| Ogy,P| | Olgy,DO H|OUH\OOgy 1 27
survivaI leyond chiIdhood is rare. IiCeorge's syn-
drone, a congenifaI disorder, and hunan innuno-
deIciency virus, an acquired one, lofh reresenf
-CCl mDunoUCHCCnCC8. Pafienis wifh near-fofaI
thynic hyoIasia are highIy suscefilIe fo oor-
funisficinIecfions Iron organisns such as Iungi and
|neunostis cor|nii.
LOC0 %0OPSl0lDOS
H| sIOlyandPhys|cal Lxam|nat|on
InexanIeoIadisorderoIceII-nediafedinnuniiy
is LCOrgC8 8ynUrOmC [chronosone 22qI I deIe-
fion) . These afienfs resenf earIy ID inIancy vifh
disease unrelaied fo fhe innure sysien [e.g., con-
genifaI hearf disease, hyocaIcenic fefany Iron
fhymic hyoIasia) . Cfher sfrucfures and organs
derivedIronfhe branchiaI ouches duringenlryo-
genesisnaylenaIIornedasveIl,incIudingfheears
and Iace. The severiiy oI fhe innunodeIciercy is
exfreneIyvarialIe.
L0gODSlC Lv0U0lDO
IlsoIufe Iynhocyte counfs are nornaI or noder-
afeIydecreased.T-celI Iuncfion, measuredlyu vifro
nifogen sfinuIaiion andintradernaI deIayed hyer-
sensifiviiy fesfing, is alsenf or signiIcanfly conro-
nised. lo fhynic shadov is seen on chesf x-ray.
Fluorescenf in sifu hylridizafion [F!SI) fesfing oI
chronosone 22 defecfsfhe 22gIdeIefion.
fP0lPOl
The innunodeIciencyoIIiCeorge'ssyndronehas
leensuccessIuIIyfreafedvifhlothfhynicandlone
narrov fransIanfafion. !nifiaI fheray shouId le
ainedafreairingassociafedcongenifaIhearfdeIecis
and nairfainingnornocaIcenia.
`
KEY O| N15

8
1 . Pat|entsw|th cellmed|ated |mmunedysfunct|on
aresuscept|bletoauto|mmuned|sordersandto
OppOltun|st|C |nfect|onsfrom organ|smssuchas
nPunOy5l5 OID.
2. Pers|stenthypocalcem|ctetanyOlOlIC lCD
anomal|essuggestD|George's syndrome.
LODODOO DDUDOOOCODC
#
b
#
DOlODO5
Conlined hunoraI and ceII-nediafed innuno-
deIcienciesfendfoleinheriiedandnaniIesfavide
range oI cIinicaI severify. II|ecfed afienis disIay
increasedsuscefiliIifyio lofh fradifionaIlyviruIent
and ooriunisfic inIections.
hCvCrC COmDnCU mmunoUCHCCnCy U8Ca8C
[SCII) is aarficuIarIydevasfafing disordercharac-
terized ly sulsfarfiaI deIcifs in lofh hunoraI ard
ceII-nediafedinnunify.IafienfsaresuscefilIefoa
vide range oI inIecfions and usuaIIy resenf vifh
nuIfiIe iIlnesses ir fhe Irsf Iev nonfhs oI IiIe.
These afienfs viII have an alsoIufe Iymhocyfe
counf Iess fhan 2S00, which can le nofed on a
roufinc CBC. BonenarrovfransIanfafionhasleen
curafive, genc fheray is nov leing sfudied as a
ossilIe alfernafivefreafmenf.
P1aXa-\ClangCC1a8a is arare aufosonaIrecessive
disorder characferizedlyvarialIehunoral and celI-
nediafed innune deIcifs, cereleIIar afaxia, and
ocuIocufaneous feIangiecfasia [snaII diIafed vesseIs
easily visilIe aIong fhe luIlar conjuncfiva and skir
surIace) . The incidence oI naIigrancy, eseciaIIy
non-Iodgkin's Iynhona and gasfric carcinona, is
increased. |o seciIc fheray is avaiIalIe, nosf
atienfs are wheeIchairlound ly ulerfy and die
renafureIy
8Ko1\-PlUrCh 8ynUromC is ar X-Iinked reces-
sive disorder oI | and T-ceII innunify, afoic
dernafifis, and fhronlocyioenia. SurvivaI fo
aduIfhoodisrarelecauseoIlIeeding,inIecfions,and
associafed naIignancies.
L5OlOOl5 O DB@OC
#
C DDUD
#
Ihagocyfes are resonsilIeIor renoving arficuIafe
maffer Iron fhe lIood and fissues ly ingesfing and
desfroyingnicroorganisns.These ceIIsnusflealIe
fo adhere fo fhe endofheIiun, nove fhrough fhe
fissues fo fheir sife oI acfion, enguII fhe harnIuI
naffer, and knI if infraceIIuIarIy. LhrOnC granulO-
ma1Ou8 U8Ca8C [CCI) is fhc nost connor
inherifed disorderoIhagocyiic innunify.
LOC0 %0OPSl0lDOS
H|sIOlyandPhys|cal Lxam| nat|on
CCI is characferized ly chronic or recurrenf
yogenic inIecfions caused ly lacferiaI and IungaI
afhogens fhafroduce cafaIase. IIfhough fhenosf
connon Iorn oIfhis disorder is inherifed as an X-
lirked iraii, aufosonal inherifance Ias aIso leen
reorted.Ilscesses and granulona Iormafion occur
irfheIymhnodes, Iiver, sIeen,Iungs,skin,andgas-
froinfesfinaliracf.!aiIurefoihrive,chronicdiarrhea,
< not toz sale ! > < ue pn n]opa !
< cau v pexaer~ouee]cn: HCA o Qpou. ] >
1 28 llucpr|nlO Ped|atr|cs
andersistent candidiasis oIthe nouth and diaer
area are connon. IIIected individuaIs are aIso at
increased risk Ior ooriunistic inIections and dis-
seninatedviraI disease.
L0gODSlC Lv0U0lDO
White lIood ceIl count tyicalIy ranges leiveen
I 0,000 and 20,000/pL vith 60% to S0% oIynor-
honucIear ceIIs. Leukocyte chenotaxis is nornaI.
ThehaIInarkalnornaIityistheinaliIityoIaIIected
ceIIstoroduceanoxidativelurstresuItinginhydro-
gen eroxide.The1roDluC 1C1ra7Olum 1C81 [lBT)
and the 2, 7-dichIoroIluorescin assay [IC!) are Ial-
oratory studies erIorned to detect this reduction
reaction.
fP0lPOl
IIIatienis vith CCI shouId receivedaiIyrohy-
Iactic trinethorin-suIIanethoxazole. 1udicious
antiliotic theray during inIections is criticaI
` Lamma n1CrCrOn 1hCraj has leen shovn to
reduce the incidence oI serious inIection. Bone
narrov transIantation is not as successIuI as u
other innunodeIciencysyndrones.
KEY O| N15
G
1 . Chron|cgranulomatousd|sease|scharacter|zed
bychron|correcurrent| nfect|onsduetocatalase-
produc|ngbacter|aorfung|.ln part|cu|ar,pat|ents
candevelopfrequentsk|n and l|verabscesses.
2. Gamma | nterferon reducesthe|nc|denceof
scr|ous|nfect|on.
L5OlOOl5 O LODQODOD! DDUD!y
Ithough quantitative deIciencies oI viriuaIIy alI
conIenent cononents have leen descriled, they
are Iess connon than the cIasses oI disease nen-
tioned earIier. Patient vith ComCmCn! U8orUCr8
have increased suscetililiiy to lacteriaI irIections
and a higher incidence oIrheunatoIogic disease. In
pariicuIar, deIciencies oIthe terninaI conIenent
cononenis C5 to CS increase the IikeIihood oI
Ies:er nenngitiJs inIections.
PLLLHLX
In aCrgC rCaC1On is an undesiralIe innune-
nediated resonse to an environnentaI stinuIus.
IIIergies have leen inlicated as a contriluting
Iactor ir anahyIaxis, asthna, aIIergic rhinitis, and
atoic dernatitis.IIIergic reactions range Iron nild
to IiIe-threateningandareneuer consideredadative.
POl@C HDD!5
0lDgPOPSS
PlCrgC rhn18 is atye I hyersensitivityinnune
resonse to envirornentaI aIIergens, incIuding air-
lorne oIIens, aninaI dander, noId, house mites,
cocI:oaches, cigarette snoke and ceriain Ioods.The
oIending aIIergen linds to IgL on nast ceIIs in the
uer resiratory tract, vith sulsequent release oI
inIlannatory nediators.IIIergic rhinitis isthenost
Irequent cause oIchronic or recurrent cIear rhinor-
rheainthe ediatricouIation.
LQ0PDDgy
SeasonaI aIIergic rhinitis, or haj CVCr, is Iinited to
nonths oI oIIination and is unconnon leIore 5
years oI age. Tree oIIens are connon during earIy
sring,IoIIoved lygrassoIIens, vhicharedetected
untiltheearIysunner.Ragveedseasonstarts inthe
Iate sunner and ersists untiI the IrstIrost. Ieren-
nial disease ersists year rourd, usuaIIy in resonse
to househoIdalIergens,esecialIythedustnite.
HSK 0ClDfS
Itoy and geneticredisositionarethe najor risk
Iaciors. LarIy heavy aIIergen exosure aIso increases
theIikeIihoodoIsulsequentdisease.
LOC0 %0OPSl0lDOS
H| story
Iatienis vith alIergic rhinitis areIagued vith nasaI
congestion,roIusevateryrhinorrhea, andsneezing.
Issociated aIIergic conjunctivitis is connon. Lnre-
IentingosinasaIdriroducesIrequentcoughingor
throat cIearing. Iatientsnay aIso conIain oIleing
drovsy lecause oI recurrert lrieI avakenings ai
night.
Phys|cal Lxam|nat|on
Cn exanination, the nasaI nucosa aears loggy
and lIuish. Tvo characteristic Ieatures oI aIIergic
rhinitisareaCrgC 8hnCr8 [darkcircIesthatdeveIo
underthe eyes secondaryto venous congestion) and
the aCrgC 8au1C [a horizontaI crease across the
niddIe oIthenose due to aconstantuvardviing
notion vith the hand) . Because oIthe severe con-
gestion, atients nay lecone olIigate mouth
< not tor sale ! > < ue pn n]opa ! >
lreafhers,andagaingnoufhnayleseer onhys- lecone invoIved, olsfrucfive slee anea nay
icaI exan. ensue.
LHPfPOl0 L0gODSS
InIecfiousrhinifisisnuch nore connonfhanaIIer-
gic rhinifis in inIanfs and ioddlers and is oIten
nucouruIeni. Sinusifis causes chronic rhinorrhea
andosfnasaI dri associafed vifh IaciaI fenderness,
cough, and/or headache. When a nasaI Ioreign lody
is resenf, fhe discharge is usualIy uniIaferaI, fhick,
and Ioul-sneIIing. CccasionaIIy ofher diagnoses
should le considered, incIuding cysficIlrosis, ese-
ciaIIyiInasaIoIys are seen.
L0gODSlC Lv0U0lDO
!suaIIy, a careIuI hisfory conIrns fhe diagnosis.
Iafienis vho do noi resond Iavorally fo a friaI oI
second-generafion [nonsedafing) aniihisfaninesnay
require Iuriher vorku. LIevafed serun and
nasoharyngeaIeosinohiI|eveIs andinfranasal alIer-
gen chalIenges may suorifhe diagnosis hovever,
direcf skin fesfing is fhe reIerred nefhod Ior
seciIc aIIergy iesiing.
fP0lPOl
The nosf eIIecfive ireafnenf Ior any alIergic
condifion is aCrgCn avCUanCC. Svifching fo air-
condifioninginfhe sunner [rafher fhankeeingfhe
vindovs oen) aIIords sone rofecfion fo afienfs
vifholIenaIIergies.Liniiingfhe anounioIhunid-
ify in the hone can decrease fhe resence oI dusf
nifes and various Iungi. LIininafing aninal dander
and linifing exosure fo cigareiie snokc are aIso
helIuI.
IharnacofherayisaninorfanfadjuncfiIavoid-
ance is nof ossilIe. h8lamnC DCCKCr8 are ihe
nainsfay oI freafneni. They are nov avaiIalIe in
nonsedafing IornuIafions aroved Ior use in chiI-
drengreaier fhan2yearsoIage.InfranasaI cronoIyn
ishelIuIasareveniivenedicaiioniIfakenriorfo
iheonsefoIsynfons.lasaIfoicalsferoidsarevery
eIIecfive ireafnenfs viih nininaI side eIIecfs.
ToicaI and inhaIed synafhoninefics [ihe nosi
ouIarleingseudoehedrine) areuseIuIIorshorf-
fern fheray onIy and, iI faken inroerIy, nay
resuIfinseverereloundcongesiion.IIIergyshofsare
ainIuI, fine-consuning, risky, and exensive, they
are indicafed only Ior severe synfons nof con-
frolIedvifh convenfionaIharnacofheray.
CccasionaIIy, congesfionis so severe fhaf children
leconeexclusiveIynouih-lreafhers, vhich Ieadsfo
denfal naloccIusion. !I fhe fonsiIs and adenoids
B
KEY O| N15

1 . Allerg|crh|n|t|smaybeseasonalorperenn|al.
2. Allerg|crh|n|t|sshouldbecons|dered |nanych||d
w|thchron|correcurrentrh|norrhea andupper
resp|ratorytractsymptoms.
J. "A|lerg|csh|ners"andthe "a||erg|csal ute"are
character|st|c phys|calfeaturesofal |erg|crh|n|t|s.
4. Nonsedat| ngH,-h|stam|neblockersandnasal
top|calstero|dsarethema|nstaysoftreatment.
P5DDB
Asfhna is discussed |n defaiI in Chafer 20. I sig-
niIcant rooriion oI cases oI asfhna is alIergic in
nafure. IIIergens IrequenfIy associaied vifh asfhna
exacerlaiions incIude noIds, dusf mifes, ef dander,
cigarette snoke, oIIens, Ioods, and cock:oach
anfigens. IlIergen avoidance is fhe Irsf sfe |n
eIIecfivefreafmenf. Cfher fheraies arediscussed |n
Chafer 20.
POQC LOlDB5
ThealCrgC !raU consisfsoIaIIergicrhinifis,asfhna,
and atoic dernafifis [eczena) .P1oQC UCrma!!8 is
achronic,relasingand reniffinginIlannaforyskin
reacfionioseciIc aIlergens. Lczena usuaIIyaears
ininIancyandaIIecfsuvardoII 0%oIfheediairic
ouIafion. Cenefic rediIecfion is fhe highesi risk
Iacfor.
LOC0 %0OPSl0lDOS
Thefyicalrash consisfs oIaruriiic, eryihenafous,
veeing aulovesicuIar reaciion ihaf rogresses fo
scaling, hyerfrohy, and IicheniIcafion. Ir inIanfs
younger fhan 2 years, fhe erufion involves fhe
exiensorsurIacesoIihe arns and Iegs, fhe vrists, ihe
Iace, and the scaI, ihe diaer area is invarialIy
sared.!IexorareasredoninafeinoIderagegrous,
as veII as fheneck, vrisfs, and ankes.The diagnosis
oI afoic dernafifis is rinariIy cIinicaI, lased on
hisfory, hysicaI exaninafion, and resonse fo freaf-
nenf. The diIIereniiaI diagnosis incIudes confaci
dernafifis and soriasis, a chronic nonalIergic skin
disorder [seeChafer 5).
fP0lPOl
ThenainsfayoIireafnenfisfo ierninafefhe"ifch-
scrafch-iich" cycIe. Iafienfs shouId iry fo keefheir
1 U
>_< ue JYR n]oDa ! >
< cau v pexae~ouee]cn: HCA o ]ou. | >
HC5
skin veII hydrafedlyavoidinghotvaterandsfrong
or Iragranf soas.Tighf clofhing and heaf nay re-
ciifafeexacerlafions.Moisfurizersarefhenainsfay
oI freafnenf, IoIIoved ly fhe use oI 1oCal Cor\-
Co81CroU8 |or areas oI inIlannafion. !r fhe nosi
severe cases, ofher foicaI innunonoduIafors
have leen used, incIuding facroIinus. Severe
chronic eczena nay le conIicafed ly lacferial
suerinIecfion.
LlCBlB BDO PD@OOOODB
Irficaria and angioedena are cIassic fye I hyer-
sensifivify reacfions. 1r\Cara descriles tIe iyical
raisededenafoushiveson fIeskinornucousnen-
lranesresuIfingIronvascuIardiIafionandincreased
ernealiIify. The Iesions ifch, lIanch, and generalIy
resoIve vifhin aIev hours fo days.PngCCUCDa is a
siniIarrocessconlnedfotIeIoverdernisandsul-
cufaneous areas, fhe defh resuIfs ir a veII-
denarcafed area oIsveIIing devoid oIrurifus, ery-
fhena, or varnfh. Ilfhough acufe uriicaria and
angioedena occur IrequenfIy in fhe ediafric
ouIafion, chronic Iorns are rare.
LOC0 %0OPSl0lDOS
ThediagnosisislasedonadeiaiIedhisforyoIrecenf
exosures or changes in tIe afienf's environnenf.
The nuIiiIe aIIergens and condifions associafed
vifh uriicaria and angioedena incIude Ioods, ned-
icafions, inIecfions, and sone sysfenic iIInesses.
CIinicaInaniIesfafionsnayledeIayedas Iongas4S
hours aIter fhe inifiaI encounfer. Ieredifary Iorns
exisf, afienfs vifh heredifary angioedena have an
inherifed L e8!Cra8C inhilifordeIciency. CItenfhe
efioIogyrenairs anysfery.
fP0lPOl
Treafnenf deends on severiiy, vhich ranges Iron
niId fo IiIe-fhreafening [i.e., sveIIing around fhe
airvay) . Sulcufaneous einehrine is ihefreafnenf
oIchoiceinenergencysifuafions,IoI|ovedlyinfra-
venous dihenhydranine and sferoids. CraI anfihis-
fanines, synafhoninefics, and occasionaIIy oraI
sferoids are aroriafeir niIder cases.
OOO POl@OS
0lDgPOPSS
Steady advances have leen nade ir Iood aIIergy
researchover fIe asf decade. !fis inorianffo dis-
iinguish leiveen Iood intolerance [an undesiralIe
roninnunoIogic reacfion) and irue Iood aIIergy,
vhich is nediafed ly innune nechanisns. Lx-
anIes oI noninnunoIogic adverse Iood reacfion
incIude caIIeine-induced fachycardia and Iacfose
infoIerance.
LQ0PDDgy
LightyercentoIaIIIoodaIIergiesresentduringfhe
Irsfyear olIiIe.TheoveraIIrevaIenceoIIoodaIIer-
giesis aIsohigherinchiIdren[5%S%)fhaniraduIfs
[ I %2%) . ReIafiveIy Iev Ioods are reresenfed,
Canu\8, Cgg8, DlK rC1Cn8, 8Cy, vhCa!, and H8h
accounf Ior over 90% oI reoried cases. LxcIusive
lreasfIeeding nay deIay resenfafion unIess fhe
nofher is irgesfing fhe oIIendingrofeins reguIarIy.
Cnefhird oI afienis vifh afoic dernafifis also
haveaIoodaIIergy.
LOC0 %0OPSl0lDOS
H| storyandPhys|ca| |xam|nat|on
I defaiIed hisfory, ircIuding daiIy records oIintake
and synfons, is essenfiaI Ior fhe diagnosis. True
Iood aIIergies can resenf vifh isoIafed cufaneous
reacfions, gasfroinfesfinaI synfons, resirafory
synfons, and IiIe-fhreafening anahyIaxis. Syn-
fons fhaf deveIo during veaning are arficuIarIy
suggesfive oI|ood aIIergies.
L0gODSlC Lv0U0lDO
IIfhough skinfesfingnayleheIIuI insonecases,
fhe UouDlC-DlnU, aCCDo ChalCngC-ooU ChalcngC
is fhecurrenfgoIdsfandard. SeveraI Ioods are eIin-
inated |ron fhe afient's dief Ior a eriod leIore
fesfing.ThenfheIoodsaredisguisedandfesfed,aIfer-
nafing vifh Iacelos, over several days.I chaIIenge
is considered osifive iIsigns and synfons recur
aIteringesfion.
fP0lPOl
Treafnenf enfails eIininaiing fhe oIIending Iood
Iron fhe dief. !n chiIdren vifh severe, videsread
aIIergies,eIenerfaIhyoaIIergenicIornuIasareavaiI-
alIe. Cov's niIk, soy, egg, and vheaf aIIergies are
usuaIIy oufgrovn aIter avoidance oI fhe oIIending
Iood.CraIchaIIengescanleconducfedsaIeIyforein-
froduce fhe |ood. Iovever, nuf and Ish aIIergies
usuaIIyersisf.
< not tor sale! > < ue pn n]opa ! >
< u
^d

\C| I I HHUDOQy C|Q DC\O|OQy 1 31


@ KEYO| N15
1 . PeanuIs,eggs,m|l k,soy,wheaI,andshaccounI
fortheovCrwhCl H|ngmajor|tyoffood al lerg|es.
2. S|gnsand sympIonsoffood al lergy|n|nfanIs
|nclude|rr|Iab|l |ty,d|arrhea,andfa|l uretoIhr|ve.
J. Thedouble-bl|nd,placebochallenge-food chal
lenge|sIhegoldsIandardofd|agnos|s.
HmLLPLLLLX
hhcumalology involves ihe diagnosis and treatnenf
oI a varieiy oI loosely relaied chronic, recurrent,
arihritic and connective tissue disorders. Most are
thought to result Iron nisdirected host deIense
nechanisns, the innune systen IaiIs to recognize
"selI antigers and iritiates an inaroriate inIan-
natory resonse against the host. Isually, autoanti-
lodies are roduced and nay le recovered Iron
lasna ortissue sanles, assistingvith diagnosis.
JUVODlO HDOUDB!OO Pl!Dl!5
0lDgPOPSS
Juvcnlc rhCumaloU arlhrls [IRI) consists oI a
grou oI innurologic disorders characterized ly
chronicsynovitis.ThenericanCoIIegeoIRheuna-
ioIogy has estalIished the IolIoving criteria Ior the
diagnosis oI1R.
" Ige younger fhan 1 byears
" /rihritis ir at leasi ore joirt Ior 6 consecutive
veeks
" Irihritis as deIned ly the resence oIIinitafior
oIrange oInotion, tenderness or ain onnotion,
orincreasedvarnth
" LxcIusion oIother causes oIarihritis
LQ0PDDgy
1RI, as is true oI nost rheunatologic conditions,
occurs nore connonly in girls. Iatients nay le
aBicted earIyorlateirchildhoodorinadoIescence.
HSK 0ClDfS
ManyatientshaveaositiveIanilyhistoryIorother
rheunatologic disorders. Certain ILI tyes also
have Leen associated vith increased risk oIdisease
[e.g.,-Z1 andauciariicular1RI).
AbLL 1 1 -Z
bg5 0 bymtom5 Juvee
Hheumto0 Ptthft5
J0nt-Hete0 bmt0mS
NOfDHQ S\HCSS
Ng\H
HCUH\OO HOOU|CS
LU|OHg
HOUS|\ODO|wCg\
LOOfH\y
LOC0 %0OPSl0lDOS
b_Stemc b_m_t0mS
|\gUO
PHO|OX
||U|O\O \|VC
HS
| ||\D| \y
LyHOOHO\y
MO\OS|OHOHCg|y
H|sIoryandPhys|cal Lxam| naI|on
lotevorihyclinicaInaniIestationsareIistedin1alle
I I -2.
hyslCmC-onscl JVP constitutesaloutI 0%to 20%
oI aIl cases and occurs equally in loys and girls. lt
resents vith high siking Ievers ard a salnor-
colored, evanescent rash rior to the onset oIjoint
syntons.ThesechildrenviIlaearquiteillduring
a Ielrile eisode, Iynhadenoathy and heato-
sIenonegaly are oIter resent on exan. Flevated
vhite lIood ceIl and latelet counts, anenia, and a
high sedinentatior rate are characteristic. Tests Ior
antinuclear aniilody [IlI) and rheunatoid Iactor
aregeneraIlynegative.Theseatients do notdevelo
chronic iritis.
1auCarlCular JVP resenisinJ0%to 40% oIchil-
drenvith1RI.ltisdividedintoearly-onsetandIate-
onset disease. !nauciarticuIar1RI,the atienf nay
have involvenentin u to Iourjoinis and rinarily
has syntons in large joints such as knees and
ankles. Fighty ercent oIthese atierts vill have a
ositive /lI, vhich indicates an increased risk
Iactor Iorthe develonent oIuveitis.
In olyartCular JVP ihe involvenent oIIve or
norejoints is requiredIordiagnosis. Cirl: redoni-
nate, vith a ratio oI J . 1 . 1oint invoIvenent nay
irclude snalI and large ]oints as vell as the ten-
oronandilular joint and cervicaI verielrae.
RheunatoidIactornay le resent insone atients
vho go or to develo disease sinilar to adult
rheunatoid arihritis. Fever atients have a ositive
lI, lut those that do coniirue to le at risk Ior
uveitis.
132
<

o I e <, ue n n]oDa ! >


OafCS
LPfPOl0 L0gODSS
\iriuaIly any rheunatologic disorder can resent
initially vith isolated arihritis. Cther conditions to
considerincIudeseticarihritis,toxicsynovitis,Lyne
disease, and reactive arihritis. loninIannatory
causes oIlinl andjointain are discussed in detail
in Chater l 9.
L0gODSlC Lv0U0lDO
SynoviaIIuidanaIysistyicaIlyrevealsavhitelIood
ceIIcountoI I 0,000to 20, 000/jtL andhighrotein
vith decreased glucose and conlenent levels.
Radiograhs reveaI soIi iissue svellirg earIy, later,
lonyerosionardnarrovingoIfhejointsacesoccur.
!fP0lPOl
Treatnent corsists oI nedicaI nanagenent vith
inIannatory-suressive drugs [nonsteroidal anti-
inIannatory drugs, innunosuressive drugs,
steroids, efc.) and hysical fheray. Functional or
cosneticsurgery isgeneraIIydelayed until grovthis
conIete.
Most atients vith 1R exerience Iittle erna-
nent disalility and renain in renission Ior long
eriods. Severe invoIvenent oIien leads to ]oinf
destruction and deIornity and nay resuIi in a leg
Iengil discreancy. Children vifh auciariicular
diseasenaydeveloiridocycliiisandvisionloss. Sys-
tenic 1R is associated vith ulnonary, heatic,
certralnervous systen andcardiac disorders.
KEY O| N15
I
1. Juven|lerheumato|darIhr|I|s|scharacIer|zedby
chron|csynov|I|s and |sclass|edaccord|ngIo
degreeof|nvolvemenIsysIem|c,pauc|art|cular,
polyarI|cular).
2. AnI|-|nf|ammaIorydrugsandphys|calIherapyare
Ihemai nsIaysofIreatmenI.
by5!ODC LUQU5 Lly!DODB!O5U5
0lDgPOPSS

hy8lCmC luu8 CrylhCmaIo8u8 [SLL) ischaracterized
ly videsread connective tissue irIannation and
arieriolarvasculitis. S!Ldevelosvhentheinnune
systen sonehov legins to recognize selI' nuclear
roteins,cyiolasniccontents, andconnectivetissue
as "Ioreign" and atients to neutralize or renove
then./ntigen-antilodyinnuneconlexeslecone
deosited in thevaIIs oIsnaIl arieries, resulting in
inIannation and necrosis. This innure conlex
vasculitisisthelasicathologiclesionresonsilleIor
the extensive clinicalnaniIestations.
LQ0PDDgy
SLLusually aearsirIatechiIdhoodoradoIescence
and is Iar nore connon in Ienales. IIricar-
Inerican and Iisanic atients tend to have nore
severe disease.
LOC0 %0OPSl0lDOS
Hi sIoryand|hysicalLxami naIion
The diagnosis oI SLL is lased on clinicaI criferia
[Talle ! I J) . The onset nay le reciifous and
raidly rogressive, or insidious vith a slov, steady
course. Fever, nalaise, and veight loss are Irequert
constitutional conlainis. Irilritis oI ihe hands,
vrists, eIlovs, shoulders,knees, andankles roduces
ain out oIroortion io the hysical signs ir Iact,
thearthritis oISLLisneithererosivenordeIorning.
CentraInervoussysteninvoIvenentnay resent at
anytine over thecourse oIthe disease.
uu8 nChr18 isthenostconnoncIinicalnar-
iIestationandisoIienresentatdiagnosis.TheVorld
HeaIil Crganizaiion classiIes renal involvenent as
nornaI [tye l, 6%, renal Iailure extrenely rare),
mesangiaI[tyell,20%,renalIailurerare),Iocalro-
liIerative [tye lll, 2J%, renal IaiIure unconnon),
diI!use roliIerative [tye l\ 40%,rogressiverenaI
Iailure connon, high noriaIity), and nenlrarous
disease [tye\, renaI IaiIure unconnon) .
1AbLL1 1 d
Lftef lof the LS5HCto ol byStemC LuuS
btythemtoSuS
N||[DU\\Ofly) rash
L|SCOO |upusraS
|O\OSOHS\V\y
L|| O|DS| UCOCU\HOOUS U|CO|\OHS
NOHO|OSVO|\|\S
NOprif|S
LDCOQDOQ\y
||OU|\SO|QO|C|O\S
Ly\OQOH
|OS\VO UHOSOfO|Ogy
|OS\VOH\HUCOfH\DOOy\OS\
< not tor sale ! > < ne pn popa ! >
L
< rg_ srns = Q| J
>
apter l l nnunoogy lIeryanto| ogy 1 33
L0gODSlC Lv0U0lDO
Irenia, leukoeria [viih a redoninance oIneu-
frohiIs), and fhronlocyfoeria are characferisfic.
Conlenenf levels, including L3, C4, and LI50,
are gererally deressed or Iallirg, esecially during
aciivedisease.Iosifive anfinuclearanfilodyfesfis
verysersifivelufnoi necessarily seciIc. Flevafion
ir UouDlC-s1ranUCU P arfilodies |arallels disease
severify, esecialIy renal disease. Cfher aufoanii-
lodies, includirg anfihosholiid and anficardi-
oliinanfilodies,nayleresenf.Circulafirganfi-Ro
and anfi-La anfilodies ir an SLL-aIIecfed nofher
nay cause corgenifal hearfllocku herIefus.
fP0lPOl
Treafnenf is long fern ard nuliiIacforial. CareIul
affenfion nusfle aidfonufrifioralsfafusardluid
lalarce.Iinifirgsurexosureandusingaroriaie
sur llock inroves skin rollens. ggressive char-
acferizafiorandfreafnerfoIrenaldisease,ircluding
renalliosy ard Irequerfinaging, areinvaluallcin
nininizirg norlidify. Iyperfension is a relafively
connon conIicaiionfhaiisusuallyvellconfrolled
vifhconvenfionalfheray.
/rfi-irIannafory iheray renairs fhe nainsfay
oIpharnacologicireafnerf. Cral redrisoneis re-
scriled as reeded Ior nainferance fheray, high-
dose oraI or infravenous uIse fheray is reIeralle
during acufe exacerlafions. Cfher innunosures-
sarfs,suchascyclohoshanide,areheIIulinfreaf-
irg luus nehrifis. Iydroxychloroquine nay le
usedfo freafnucocuianeous synfons.
Cverall, rognosis and qualify oIliIe are inrov
ing.Renal diseaseroducesfhenosfsigniIcartnor-
lidify,renalIailureisfheleadingcauseoIdeafh aIter
inIecfion. Dman-haCKs CnUoCarU!s is a serious
cardiacconlicaiion.Mosfafienisvifh SL! donoi
achievenornaIliIeexecfarcy.
KEY O| N75
B
1 . SLEcons|stsofw|despreadconnect|vet|ssue
|nflammat|onandvascul |t|s.
2. Thed|agnos|sofSLE |scl|n|cal.
J. Lupusnephr|t|s|sthemostcommoncl|n|calman-
|festat|on,result|ng|ns|gn|cantmorb|d|ty.
4. Typ|call aboratorynd|ngs|nc| udefal | |ngcomple
mentlevelsand pos|t|veant| nuc|earant|bodyand
doub|estranded DNAant|bodyt|ters.
b. Thed|sease usual ly respondsto| mmunosuppres-
santtherapy.
LOlDB!ODyO5!5
0lDDgPOPSS
Crmalomyosls is an inIannaiory disease involv-
ingfhesnalI vossels oIfhe skin, sfriafednuscle, and
occasiorally fhe gasfroinfesfiral fracf. lnnure
conlexes are deosifed in fhe valls oI arferioles
caillaries, and venules, leading fo irIannaiion
uIcerafion,lleeding, and Ilrinousreair. Iolynyosi-
fis, a sinilar inlannafory nuscular condifion
vifhouf skir Indings, occurs Iess Irequenfly in ihe
ediafric oulafion.
LQ0PDDgy
Cnsef usuaIly occurs in ihe lafer childhood years.
Like nosf rheunafologic cordifiors, dernafonyo-
sifis is nore connor irIenales.
HSk 0ClDfS
Iredisosifion is herifalIe, ard fhc condiiion seens
fo le associafed vifhviral iIIresses insone cases.
LOC0 %0OPSl0lDOS
H| storyandPhys|cal Lxam|nat|on
Iafienfs reorf a hisiory oInalaise, lafigue, voiglf
loss, ard iriernifterf Ievors. Irogressive muscle
veakness oI fhe elvic and shoulder girdIe nuscle
grous acconariedlyile characferisficVolaCCous
dernafifis oIihe eyelids, hards, ellovs, knees, and
arkles viriually clinches fhe diagnosis. Coffron's
auIes are characierisfic |esions resenlling scaly
eryflenafous|aulesonfheexiensorsurIucesoIfhe
inferhalargeal]oinfs oIfhe Ingers, fheellovs, ard
fheknees.Theveaknossnayadvanccfoinvolvcfhe
anferior neck, frurk, ard muscle grous used Ior
svalloving, horaiion, ard resiraiior. Long-
sfanding inlannafior everiually resulfs u calciun
deosifs in fhe skir [caIcinosis cufis), cufaneous
siriafion, scarring, ardsigniIcanfnuscle afrohy.
L0gODSlC Lv0U0lDO
The nosf sfrikirg |alorafory alnornaIify is narked
elevafion oI sCrum CrCalnnC KnasC, an enzyne
released during nuscle lreakdovr. SeciIc elec
ironyograhyardhisfologicresuIisarccharacferisfic
oI fhe disorder. Serun acufe-|hase reacfarf leveIs
[eryilrocyfe sedinenfafion rafe, C-reacfive rofein)
correIaievifh disease severify.
fP0lPOl
Treafneni consisfs oI resf, aroriafe hysical
fheray,andinnurosuressanfs./sIongasnuscle
134
< no tor sale
d
| > < ue JT n])a | >
|luor|nlO Pe |atr|cs
enzyne levels enain high, activity is Iinited and
the rinary ain oItheray is to |revent contrac-
tures vith ositioning and slirts. Iigh-dose red-
nisone is rescriled ir ar attent fo control the
inlannatory resonse. Cnce evidence oI muscle
destructionleginstoalate,steroiddosesaretaered
and strengthening exercises are graduaIly added.
Iatients vhose disease does not resond to oraI
steroids nay require lntravenous ulse steroids or
second-Iine agents lncIuding nethotrexate, intra-
venous innunogloIuIin, cyclohoshanide, and
cyclosorine.
Croharyngeal,chesivaIl, ardresiratory muscle
veaknessredisosesatientsto asiration.Resira-
tory Iailure necessitatirg necharical ventiIatior is
rare.Mostchildrendiagrosedvitl dernatonyositis
recover vith no ernarent disalility vithir a Iev
years.IloutI 0%|rogresstovheelchairdeendence
andrenature death.

KEY O| N15
I
1 . Dermatomyos|t|s |san|nf|ammatoryd| seaseof
thesmallvesselsofthesk|n,str|ated musc|e,and
gastro| ntest| naltract.
2. Theweakness beg|ns|n theprox|malextrem|ty
musclegroupsand |saccompan|edbyacharac
ter|st|cv|olaceousdermat|t|s.
J. Serum creat|n|nek|naselevelsaremarkedly
elevated.
4. Theweaknessmay progressto| nvolvethe resp|
ratoryandoropharyngealmuscles.
VB5CU!OO5
InunleroIotherconnectivetissuediseases,incIud-
ing olyarieritis nodosa, Wegener's grarulonatosis,
and Ieroch-SchonIeir urura, resent vith vas-
culitis as the rinary naniIestation. bava8aK`8
U8Ca8C a vasculifis ostuIated lut rot roven fo
le inIectious in origin, is linited to the ediatric
oulation.
LOC0 %0OPSl0lDOS 0O0 fP0lPOl
Polyarter|t|sNodosa
lnsidious ir onset, varialle in syntonatology,
vaxingandvaning,olyarteritis rodosaoIterroves
diIlcult to diagnose. Signs ard syntons nay
include any oI the Iolloving. vague systenic
conlaints, ainIul erythenatous skin nodules,
urura, hyertension, henaturia, aldoninaI ain,
encehaloathy, and neuroathy. The lngers and
toes lecone gangrenous in extrene disease. The
erythrocyte sedinentationrateis invariallyelevated
during active disease. Iiagnosis rests on signature
vascuIar lesions on liosy. Corticosteroids and
innune suressants are the nainstays oItheray.
Irognosis is Iair, nortality is related to renal or
cardiac conlications.
Wegener'sGranu|omatos|s
Wegener's granulonatosis, a rare, necrotizing vas-
culitis,tyicalIyresentsvithatriadoIinvolvenenf
including the uer and Ioverresiratorytracts and
kidneys. This rare disease causes a recrotizing vas-
culitis. The diagnosis is Iurther suorted ly the
resence ol C-IlCI [antineutrohil antilodies
vith a cytolasnic staining attern) . Treatnent is
vith innunosuression vith corticosteroids or
cyclohoshanide.
Henoch-Schn|e|n |urpura
Ienoch-Schonlein urura is an innurologicaIly
nediated vascuIitis invoIving the gastrointestiral
tract, skin, joints, and kidreys. lt occurs in young
children, eaks in the vinter nonths, and nay le
receded ly a viral or grou I stretococcal uer
resiratory inIection
,
Castrointestinal invoIvenent
isusuaIIysigniIcant,includingaldoninalain,von-
iting, iIeus, and uer and lover tract lleeding.
CloneruIorehritis nay rogress to acute renal
Iailure.Thecharacteristicnonilronlocytoenicur-
uric or nacuIoaular rash overtheluttocks and
lover extrenities is alnost alvays olserved. Treat-
nent is suortive, corticosteroids have not leen
lourd to le articularly hel|IuI. Thc rognosis Ior
Iull recoveryvithin4 to 6veeksis exceIIenf. Long-
tern conlications arallel the severity oI renaI
invoIvenent.
Kawasak|'s L|sease
Kavasaki's disease is a systenic vascuIitis character-
ized ly high Iever, lynhadenoathy, and nucocu-
taneouslesions.!toccursaInostexclusiveIyininIants
and young children and is nore connon in nales.
Ir inIectious etiologyhas leensuggestedlutnever
conlrned. CurrentcriteriaIordiagnosis arenotedin
Talle ! I -4.
Jhe nost serious conlications are cardiac,
incIuding Coronary va8Cul18 and concurrent or
delayed anCury8m lormalon. Irognosis is tied to
severity oI cardiac irvolvenent, cardiac instalility
can roduce arrhythnias, inIarction, or congestive
not tor sale ! > < ue pn n]opa ! >
bLL 1 1 9
Lftetl ut UgOS1S o KwSklS UlSeSe
|OVO|lO|b OySOlHO|O,\OgO\DO|w|\| lOU|Ol\|O
lO||OwHgUVOS|gHSOD QyS|C| OXH (orDy D|S\O|y).
1 . b||\Ol| COHjUHC\|V|\|S
2. L|HgOS Ol ||QS HO O|| CV|\y [O|y, |OO,USSUlOO ||QS
OlS\lwDO||y\OHgUO)
3. L|DgOSOlQO||Q|O|OX\fOH\|OS [ery\eHaO|
| HOU|\|VOOOOH Ol |HOS HO lOO\)
4. |OyHOlQ|OUSrash [Q||H|||y OH \|UDk)
b. PCU\OHODQUlU|OH\SWO||HQOlCOfVCB| |yHQ| HOOO
\O 71 .bCH |H O|HO\O|
heartIailurevithindays oIresentatior./neurysns
ardcoronaryartery disease ersistardnayresultin
death nonths to years Iater.
Psrn isrescriledduringtheacutecourseasan
antilateIet agent. L !Hcray adninistered over
2 to J days resuIts in roIound in|rovenent. Boil
treatnents signihcantly reduce the risk oIcoronary
ariery aneurysns.

KEY O| N15
1 . Henoch-Schnle|npurpura |scharacter|zed by
abdom|nalpa|n,vom|t|ng,gastro|ntest|nalbl eed-
|ng,and nonthrombocy\open|cpurpuraoverthe
buttocksand lowerextrem|t|es.
2. Kawasak|'sd|seasepresentsw|th h|ghfever,|ym
phadenopathy,and mucocutaneousles|ons.
J. H|ghdoseasp|r|ntherapyand |VlGreducether|sk
ofcoronaryarteryaneurysms|nKawasak|'s
d|sease.
Renarkalleadvances inthe diagnosis,nanagenent,
and revention oIinIectious diseases have occurred
duringtheastcentury. levtechniquesIordiagro-
sis include Iuorescent antilody testing, olynerase
chain reaction [ICR), and inaging nodalities
such as nagneiic resonance inaging [MRI). Secilc
treatneni oI lacterial ilInesses legan viih the
introduction oI suIIonanides in the I 9J0s and
enicillin in the ! 940s. lever classes oI antiIac-
terial agents include senisynthetic enicilIins,
tetracyclires, nacroIides, HuoroquinoIones, anino-
glycosides, carlaenens, and Iour generations oI
cehalosorins. /ntiIungal, antiviral, ard antiara-
sitic agents have also leen deveIoed. Cther anti-
inIectives include secihc antilodies, |ntravenous
innunoglolulin, hagocyte stinulaiing Iactors, and
interIerons.\accines have led to a dranatic decline
ir ceriair inIectious diseases. Snallox vas
eradicated vorldvide in ! 977, and indigerous
oIionyeIitisvaselininatedIron the!nited States
in \ 979. The annual incidence oI neasles,
nuns, rulella, dihtheria, eriussis, tetarus, and
Hoenobi/u: in]luenzoe tye l neningitis has leen
decreasedlynorethan9S% intheInitedStatesly
vaccine use.
InIortunately, nev athogens continue to
energeIorexanle,hunarinnurodehciencyvirus
[II\) vas unheard oI 20 years ago. Fqually dis-
conceriing is the raid energence oI resistance to
knovnant|liotics[e.g. ,nethiciIlir- andvanconycin-
resistant Stoby/ococcu: ou reu: and enicillin-
resistant Stretococcu: neunonioe). Thus, aIier I 00
years oI rogress againsi irIectious diseases ihe
currert chaIlenges are every Iit as IornidalIe

as at
the leginning oIthe last century.
_ _ _ _ v JCCN LP b
~ . = . .
-
m m m ~ m m ~ m ~ ~ m ~ ~ m m ~ ~ ~ ~ m ~ ~ m
HOU!DO DDUDZB!OD5
Ictive innunization involves stinulaiing an indi-
vidual's innune systen to develo a raid rotec-
tive resonse during Iuture inIectious exosures. I
vaccine coniains aIl or ari oIeither aveakened or
nonvialle Iorn or ari oIthe organisn.Talle ! 2- !
contains a sinIihed versior oIthe current vaccina-
tion guidelines reconnended ly the /nericar
Acadeny oIIediatrics.
Iesite theirlonghistory oIsaIcuse andinres-
sive cost-to-lenehtratio, vaccines should leheId or
deIayed ir certain circunstances. Talle I 2-2 lists
alsolute and relative contraindications to vaccine
adninisfration and soneconnon nisconcetions.
POO!ODB NBCCDBOD5
Children vith congental, iatrogenic, or IunctionaI
[e.g., sicklc ceIl disease) asleria should receive the
neningococcal ard loth neunococcal [corjugate
and olysaccharide) vaccines. I yearly inIuenza
vaccineisreconnendedIorchildrenletveen6 and
24 nonths old and Ior atients vith chronic ul-
nonary disease [including asthna), cardiac disease,
or sickle ceII disease and Ior aiierts receiving
innunosuressivetheray.
lLNLH L LPPLVP LHLP
m m m = + = = ~ = = ~ ~ = ~ ~ = ~ ~ = ~ ~ ~ m ~ e ~ ~ ~ ~ ~ ~ ~ ~ ~ ~ ~ ~ ~ ~ ~ ~ ~ ~
Thehrase"IeveroIunknovnorigin"[IIC)inIies
Iever oIrolongedduration [>I 0 days),docunented
tenerature greaterthan JS. JC [I 0I) on nulti-
leoccasions, and unceriainefiology. FICusuallyis
< not tor sale ! > < ue Dn njopa ! > < CKo V peeueen

__u ]
\O| IO1C\lOS lSOSO

Lhl0hoo0 I mmul2tlo bChe0ue


P@O lmmunZI0nS
b|\ MbV[ 1 )
/ HO MbV [/) L1|[ 1 ) MD ( I ) I|V[ 1 ) |LV( I )
HO L1|2) MD2) I|V [Z) |LV [Z)
OHO" L1|[d) M| D[d) |LV(d)
O~1 bHO MbV [d) I|V [d)
1 /1 b HO MD [) |LV [4) NNh [1 )
>1 /HO
V||C6||
b1 b HO L1|[4)
4~Oy| L1| [b)' I|V [) NNh[/)
" |f|ueZ vccle l8D l8 |ecDnneUeU uy Dl clUfef geU nD!8 tD 7^nDf!8 fU DI ctUIe nDt8 wt! clDntc puImDnIy,
cfU|Dv8cu|r, ne!DD||c, DI 8tckIe ce U8e8e.
`1etU8U!el vccfe 8 gve ! ge 1 1 yel8 fU tC eve|y 1 yel8 teIeter.
1e DunDel8 D pfef!e8e8 lUc!e te funDel l !e 8euefce D tnnuflZttD8. L Uplef, !e!fu8, fU ceu| e|lu888 vccle, |bV,
eptl!8 b vfu8 vcctfe, |D, Hcemophi/u:inuenzceIye D vccle, IIV, tctvleU DlD vlIu8 vcce, NNH, ne8e8, nunp8, fuUe|| vccle, ILV
cDjug!eU 8evC-v|ef! eunD6Dcc vcc|e .
M 1AbLL 1 Z-Z
Lottl0lCtloS to 0 t0CutloS Hegf0lg VCCltlo
PDS0ute L0ntf n0ctOnS
bOV6lO | |OfQ|C|OC\|OH [6.g.,HQy|X|S)
!6| Q|OVOUS VCCHOOOS6
|HOwD SOVO|E |HHUHOOOUC|6HCy [NNh,
V||C6||)
LHC6Q|OQ\yw|\|H 1OySO
OH|H|S\l\|OH O\6QlOV|OUS
OOSO[L1|)
|lOgDHCy [NNh,V|C6|)
Ifecut0nS (HetVe L0ntf n0cI0nS)
bOCKlyQOlOSQOHS|VE 6Q|SOO64b OU|S
!OfQ|OVOUSOOS6OL1|
|6VOf > 4.b`Lw|\|H4b DOU|S OQ|OV|OUS
OOSOO L|
+
b6|ZU|6d OySI6|Q|OV|OUS OOS6O
L1|
NOOE|\O\O SOVOfO CU\6 | | | HOSSw|\ O|
w|\OU\ OV6|
N0t L0ntf n0ct0nS
N|O ||HOSSw|\ O|w|\OU\
|Owg|O6OV6|
LU||6H\H\|D|O\|C\6|Qy
|OS|\|V6 ||L
||OH\Ul|\y"
IlentUle !8 8DuU Ue vcclfIeU ccDlUlg tD c|DDDglc ge. IeDt!l8 b vcce 8Du|U Ue Ue|yeU utl| le cl|U welg8 nD|e l Zg |
!e nDleI 8 Mb8Pg eg!lve.
LH UteIl, tetU8, fU ce||ul e|tu88l8 vccDe, Mb8Pg, e!t8 b 8ulce tgef, NNH, ne8e8, nUm8, |uDe vccle, I|L, Ur|DeU
lDte UelvIv3.
1 dZ
caused ly a connon ediatric inIectior vith ar
atyicalIyroIonged UC course.
LOlOO!B LB@OO55
scratch disease, Rocky Mountair sotted |ever,
ehrlichiosis, Lyne disease, lruceIlosis, Ie-
tosirosis, tularenia, endocarditis, setic arihritis,
osteonyeIitis, intra-aldoninal alscess, enteric
fever, tulerculosis, II\ oortunistic inIection
F!Cintheediatricoulatiorisusuallyaconnon
disorderresentinginan unconnon nanrer, rather
thar a"zelra."Iiagnostic considerations include the
Iolloving.
" nCClon. Sirusiiis, heatitis, cytonegalovirus
[CM, Lstein-Barr virus [LB\, arasites, cat-
" LonnCClvC 188uC U8Ca8C. Systenic ]uvenile
rheunatoid arthritis, sysienic luus eryfhe-
natosus
" algnanCy. Leukenia, Iynhona, reurollas-
tona
" llhCr. IrIannatory lovel disease, Kavasaki's
syndrone, drug Iever, ilyrotoxicosis, sarcoidosis,
not tor sale ! > < ue pn n]opa ! >
\ 38 |luor|rl Pediatrics
Ianilial dysautononia [Riley-Iaysyndrone), and
!actitious !ever
LDCB BDO5!B!OD5
MSlDfy
The age and gender oI the atient narrov the diI-
Ierential diagnosis. !nIannatory lovel disease and
connective tissue disorders are unconnon in
youngerchildren.Iutoinnunedisordersoccurnore
Irequently in IenaIes. Sexual history, travel history,
current nedications, exosure to aninals, tickLites,
artecedent illness, trauna, and IaniIy history are
inortant areas oI inquiry. I thorough history
and hysical exan [usually aIter reeated encoun-
ters) vill reveaI the diagnosis ir nore thar hall
oI the chiIdren in vhon a cause oI the Iever is
Iound.
ySC0 LX0O0lDO
Conjunctivitis, the alsence oI tears, rashes, Iyn-
hadenoathy, joint tenderness, oral ulcers, thrush,
heart nurnurs, organonegaIy, nasses, aldoninal
tenderness, cutaneous naniIestations [rash, hyer-
ignentation, etc.), joint Indings, andnental status
changes nay suggest a seciIc cause and guide
Iuriher evaluation.
LB@DO5!C LVB UB!OD
Jhe initiaI evaIuation car leerIorned in theout-
atient setting in olde; vell-aearing children.
leonates and illaearirg childrer require
hositaIization.
Screeninglaloratorytestsincludeconletellood
countanddinerential,serunelectrolytes,lloodurea
nitrogen [B!l) and creatirine levels, |iver Iunctior
tests, aIkaIine hoshatase, ard urinalysis. Bacterial
cuItures should le oltained Iron secinens ol
llood, urine, stool, ard ossilIy cerelrosinaI Iuid
[CSF) .StoolnayaIsolesent Iorviralantigendetec-
tion and arasite exanination. IdditionaI tests to
consider include erythrocyte sedinentation rate
[LSR), C-reactive rotein, antinuclear artilodies,
and secihc serologic tests such as antilody studies
lor cat-scratch disease and LB\ I chest x-ray and
skintestingIortulerculosis aretyicaIlyerIorned.
More exensive and invasive studies nay le var-
ranted lased or screening results. Ir alout 25% ol
cases, no etiology is deternined and the children
recovervithoutsequelae.
KEY lLW
T . Thephrase"feverofunknownor|g|n"|mp||esfever
ofprolongeddurat|on(21 U days),documented
temperaturegreaterthanJ.J'L ( T U\ '|) on mult|-
pleoccas|ons,and uncerta| net|ology.
2. UL |s usua||ycaused bya common ped|atr|c
|nfect|onw|thanatyp|callyprolongedt|me
course.
J. H|story,phys|cal exam,and|n|t|all aboratory
stud|esgu|defurthereval uat|on.
PLLHLP PPL bLbb
Bacterenia is the resence oIlacteria in the lIood.
Bactereniais Iurther descriled as occult iIit occurs
inavelI-aearingchildvithoutanyolvioussource
oIinIection.TheriskoIoccuItlacterenia is highest
[ I . 5%2. 5%) inchildrer letveen 2 and 24 nonths
oI age vith a Iever greater than J9. 0C and Ieuko-
cytosis.Jhe na]ority oIeisodes are due to Streto-
coccus neunonioe andresoIvesontaneousIy. RareIy,
neningitis occurs.
In contrast, 8C88 inlies lacterenia vith evi-
dence oIa systenic resonse [tachynea, tachycar-
dia, etc.) and altered organ erIusion. IIIected
children aear quite iII and nay develo shock.
Thecause oIsesisvarieslyage. Inneonates,grou
B stretococci, enteric gran-negative laciIli, and
Iister|o nonotogenes are nost revalent. !n older
chiIdren, S. neunonioe redoninates, IoIloved ly
leisser|n neningitiJis. Less connon causes irclude
Stoby/ococcus oureus, So/none//o s., |seuJononos
oerginoso, and viridans stretococci.The evaIuation
oIthe child vith susected sesis includes cuItures
Ironthellood, urine, andoccasionally CSFI chest
x-rayisoltainediIresiratorysignsorsyntonsare
resent. Eniric treatnent vith a third-generatior
cehaIosorin and [occasionaIly) vanconycin is
couIedvith aroriatc suortive neasures.
Lb LLP
B!DO@ODO55
SusectedorconIrnedacuteinIectionoItheniddle
earaccountsIornorehysiciarvisitsthananyother
ediatric ilIness.JheniddleearisnornaIly sterile, a
atent lut coIlasille eustachian tule aLovs Iuid
drainage Iron the niddle ear into the nasoharyrx
< not toz sale ! > < ue )7H .]o)a ! >
lutnornaIlyrevenfsthe retrograde entry oluer
resiratory lora. In childrer, the angle oI entry,
short length, and decreased tone ol the tule nay
allov Ior retrograde Iov and increased suscetilil-
itytoinIection.
LQOODOO@y
Ctitisnedia [CM)isnostconnoninchiIdren6to
J6nonths oIage. ByJ years oIage, S0% oI aIl chiI-
drer ir the Inited States have had at least one
eisode oI otitis nedia, and 50% have had at Ieast
three eisodes.Ilout J0% oIcases oIacuteCMare
causedly viruses lut nayle conIicatedlylacte-
riaI suerinIection.The other 70% reresenf lacter-
ial inIections, nost connonly S. neunonioe [50%
oI aIl lacterial eisodes), nontyealle Hoenobi/u:
in]luenzoe [J0% oI all lacterial eisodes), ard
Aornxe//o cotorbo/is [ I 0% oIalllacterialeisodes).
Chronicsuurative CMis norelikelyto lecaused
lyStob/ococcus oureus, IseuJononos oeruginoso, or
nixedathogens.
H5K BCOl5
Caretaker snoIing, lotcle Ieeding, day-care atter-
dance, aIlergic disease, cranioIacial anonaIies,
innunodeIciency, and aciIer use aIl redisose
chlIdrento CM.
LDCB NBDO5BOD5
MSlDfy 0O0 DySC0 LX0O0lDO
Children nay have local or sysienic conlaints or
loth, including ear ain, Iever, and Iussiness.Icute
CM is Irequertly receded by syntons oI uer
resiratoryinIection[cough,rhinorrhea,congestion) .
Cn hysicaI exanination, the aIlected tynanic
nenlrane aears lulging, oaque, and eryrhe-
natous or yellov vith an alerrant Iighf rehex.
Ineunatic otoscoy reveaIs decreased tynanic
nenlranenoliIity.
LOlODB LB@DO55
Ctitis media vith eIlusion is diagnosed vhen there
is aarent uid lehind the tynanic nenlrane
[reduced nolili on neunaiic otoscoy) or the
tynanicnenlraneisinnolile,lutthereisnoevi-
dence oIinhannation [tynanic nonlrane is not
red or yeIlov, there is ro Iever, there is no evidence
o| ear air). Ctitis exierna [irl annation oI the
externalearcanal) also causes earain hovever, ihe
tynanic nenlranes should aear nornal on
hysical exanination. The |air oI otiiis exterra is
exacerlated ly naniuIation oIthe external ear.
tynanic nenlrane that is eryihenatous vlthouf
anyothersignsoIdiseasenaylecausedlyvigorous
cryirg ard shouId nof le considered CM.
lOBDOD
|roxinatelyS0%oIuntreatedchildrenvithacute
otitis nedia have cIirical resoIutiorly7 to I 4 days,
conared vith greater thar 95% ol those treated
vith antinicrolials. Most clinicians reconnend
antiliotics Ior acuie CM to revent rogression to
chronic otiiis nedia and reducc the risI oIrare lui
serious sequelae including nastoiditis ard nenirgi-
tis.The athogens resorsille lor acute CM gener-
ally resond to high-dosc oral anoxiciIlin [S090
ng/Ig/dayIor5 io I 0 days) , resistant orgarisnsnay
require anoxicillin-clavulanate or second- or third-
generatior cehalosorins.Tynanocentesis reIieves
severe air,nay identi[ theafhogen [in reonates,
innunoconronisod childrer, or childrer vho |ail
aniiliotic theray), and treats conIications such as
nastoiditis. Vhen nore than Iour inIectiors have
occurred vithin a year [or three in 6 nonths), the
child should le reIerredIor ossille tynanostony
tulelacenert.TheuseoIdailyrohylacticantili-
oiic theray is in disute. Intiliotic theray is rot
reconnended Ior treatnent oI otitis nedia vith
eIlusion.
Ir addition to conductivehearingloss, conIica-
rions oIlrequent eisodes oICM include tynanic
nenlrane |erIoration, excessive scarrirg [tyn-
-
KEY O| N75
1 . Thethreemostcommon bacter|a |mpl |cated |n
acuteOMareb.DCUDODOC, nontypeableM.
DhUCDZOC, and NOIOXCO COfOI1DOS.
2. ln acuteot|t|smed|a.thetympan|cmembrane| s
bul g|ng,opaque,andery\hematousoryellow.
Mob|l|ty|s d|m|n|shedasassessed bypneumat|c
otoscopy.
d. Tympanostomytubesshouldbecons|dered for
ch|ldrenw|th recurrentep|sodesofacute OM.
4. Chron|correcurrent|nfect|on pred|sposestoper
manentconduct|vehear|ng loss.
anosclerosis),cholesteatonaIornation,andchronic
suurative CM.MostsontaneouserIorationsdue
to CMresoIvevithir 24 to 72 hours vithout se-
ciIc treatnent. Lalyrinthitis and nastoiditis nay
resultIron direcfsreadoIorganisnsintotheinner
ear andnastoid air cells, resectively.
bPLbb
The naxillary and ethnoid sinusos are resent at
lirth fhe sphenoid and Irontalsinuses develo Iater
inchildhood.ThesectrunoIathogensresonsilIe
Ior sinusitis is viriually +denticaI to that lor CM.
SinusitisisoItendihculttodiagnoseinayoungchild
since tle cIassic syntons oIheadache, Iacial ain,
and sinus tenderness nay le alsent or dillcult to
articulate.1cutelacterial sinusitis hastvo connor
clinical resentatiors. [ I ) orsistent resiratory
syntons [> I 0-! 4days), incIudingeither nasal dis-
charge [clear or urulent) or a daytine cough, and
[2) severesyntons includinghighIeveranduru-
lentnasaIdischargeIor atleastJ days.ThediIleren-
tial diagnosis includes viraI uer resiratory tracf
inIections, allergic rhinitis, and nasal Ioreign lody.
Conuted tonograhy [CT) is quite relialle at
detecting nucosal thickening, air-Iuid Ievels, and
oacihcation lut is not routineIy requiredIor diag-
nosis./ntiliotic coverage is sinilar to that Ior CM,
although treatnent should continue Ior I 0 to 2I
days. Iersistent inIections nay require surgicaI
drainage. Conlications are unconnor lutincIude
lony erosion, otic neuritis, orlital cellulitis, and
intracranial extension. Children vith recurrent or
chronicsinusitis slould le evaIuated Iorcystic hlro-
sis,ciliarydyskinesia, or rinaryinnunedelciency.
mLHPPLPP
Ierangina is a synton conIex caused ly
enteroviruses, nost notally coxsackie I. lt is tyi-
cally diagnosed during the sring and sunner in
younger children. Initially, the atient deveIos a
high Iever and very sore throaf. Cn exanination,
characteristicvesicularlesionsthatrogresstoulcers
are scattered over the soIt aIate, tonsiIs, and
harynx. Irinary heretic gingivostonatitis [caused
lyheressinlexvirus)resentsinasinilarnanner,
aIthough thc lesions are generally nore videsread
overtheguns,lis, and nucosa. Ieranginais selI-
Iinited [57 days) and requires no secilc fheray.
Vlen sinilar Iesions are noted on the alns and
soles [and occasionally on the luttocks), the nore
incIusive nane hanU-ool-anU-mou\h U8ca8c is
used.
bHLLLLLLPL mPHXPLb
B!DO@ODO55
CrouI leta-henolytic stretococci [Stretococcu:
ogenes) are the nost inortant cause oIlacteriaI
haryngitis. /ntinicrolial theray Ior stretococcal
diseaseis reconnendedlecause oltheIrequencyoI
suuralvc [eritonsiIIar alscess, retroharyngeaI
alscess) and non8uura!Vc [rheunaticIeyer, ost-
stretococcaIglonerulorehritis) conIications.
LQOODOO@y
"Strethroat"a!hictsolderchildrenandadolescents
it israreleIoreage J.Theorganisnissreaderson
to ersonthrough inIected oral secretions.
LDCB NBDO5!B!OD5
HSlDfy 0O0 ySC0 LX0O0lDO
Classic syntons include sore throat, Iever,
headache, nalaise, nausea, and occasionally aldoni-
nal ain. Ihysicalexaninatior revealsenlarged, ery-
thenatous, exudative tonsils and tender cervical
lynhadenoatly. Ietechiae nayle resent on the
soIt palate. Rhinorrhea, hoarseness, and coughing,
the hallnarks oIviral uer resiratorytract inIec-
tions, are notally alsent. The diagnosis oI scarlet
!ever is nade vhen a characteristic erythenatous,
"sandaer-like" rash acconanies the Iever and
haryngitis.The rash aears or the neck or trunk,
sreads to the extronities, and nay desquanate I 0
to I 4days Iater.
LOlOD!B LB@DO55
IiI!erentiating viral haryngitis and inIectious
nononucIeosis Iron stretococcal haryngitis nay
le inossillelased oncInicalsyntons,de!nitivc
diagnosis requires either throat culture or antigen
detection testIor grou Istretococci.
LB@DO5!C LVB UB!OD
Theraeutic decisions should le lased on fhroat
culture or raid antigen detection test results. The
< DOL IOI Sa)e1 > < ue jYR DOjd 1 >
scciLciIy oI nos raid anIigcn IcsIs is grcaIcr
Ian 95% [conarcd viII lroaI cuIurc), so IaIsc-
osiIivc IcsI rcsuII: arc rarc. TIc scnsIIiviIy oI raid
anIigcn IcsIs rangcs I:on 80% io 90%, ncaning Ialsc-
ncgaIIvc rcsuII: occasionalIy occur.
1tCtmCnt
lutIcnIs vIl docuncnIcd grou sIrctococcal
IaryngiIis sIouId rcccIvc a 10-day coursc oI oral
tniciIlin [or a singIc dosc oI inI:anuscular Lcnz a-
ilInc cnicilIin C) Io rcvcnI acuIc rleunaic Icvcr.
ryIIronycin, azIlronycin, and cIindanycin arc
acccIaLIc alIcrnaIIvcs Ior cIiIdrcn aIIcrgic Io cni-
ciIIin. 1Ic IrcaIncnI oI scarIcI Icvcr is idcnIical Io
ilaI Ior sIrcIococcaI IaryngIIIs.
TtutC rhCumatt !CVCr [II] occurs J Io 4 vcck:
aIcr sI:cIococcaI IaryngiIis in a snaIl crccnIagc
oI unIrcaIcd aIicnIs. II Is an inLannaIory con-
diIIon InvoIvIng conntcIIvc IIssucs oI Ile IcarI
[cardIIis, vaIvuIar dcsIrucIion), joInIs [nigraIory oI-
yarIIriIis), and ccnIraI ncrvous sysIcn [IransicnI
clorca). IiagnosIs rcsIs on IulLIIing lc 1oncs criIc-
ria [1aLIc 12-3). lniIialIy, Icvcr, dysnca, clcsI ain,
1bLL 12-$
H0VSU JDDS LlLl Dl 1D0 LgDDSS D PCUt
HDUDLC V0l
NdOl ND05ttIOD5
LIOIS
|OyIIIIS
byOCDH`S COIC
LIyICH HI_OIUH
bUOCUIOCOUS OOOUCS
NIDOf ND05ttOD5
L|D|O/
|CVCI
PIII_
LODOl0lOly
LCVICO LICCIVE [IOICO OI CIyIIOCyIC
SCOHCOIIOD rdI3
|H OIClV [IOOO_IOD ODCCCIIOCIOO_IH
PUUtOD Lft0f
bU[[OIIO_ EVOCDCC O [ICCEOD_ SIIC[IOCOCC
DCCIOO
|OSIVCIIOICUIUICOI_IOU[ PSIIC[IOCOCCOl
|OSIVE I[O OI_CD I6SIOl
DCICSCO SIIC[IOCOCC DIOOOy IICI"
LQDOSS O CU1C lCUD\C CVCt tCQUlC5 IWO DjOl Ol ODC DjOl
DO \WO ODOl Cl1Ct QlU5 SUQQOt1HQ CVOCDCC O D1CCCOCD\ QlOUQ
P 51lCQ1OCOCCl DCC1OD.
PD1DOO 1CS15 DClUOC D1S1lCQ1Ol5DL |PbL|, D\LP5C ,
H1 lUlODOSC, Ol D1 5\tCQ\OKDSC.
cardiac nurnur, and arlriIis rcdonInaIc, long-
Icrn norLidIIy rcsulIs Iron vaIvuIar dcsI:ucIion
viII conscqucnI niraI or aorIic vaIvc InsuIcicncy
or sIcnosis. cuIc cIsodcs rcsond IavoraLIy Io
anIILioIics, anIi-inLannaIory drugs, and cardiac
nanagcncnI. I! nay tccur aIcr IIc initial cisodc.
1IcrcIorc, individuaIs diagnoscd viII II sIould
rcccivc roIylacIIc cnIcilIin lcray to rcvcnI
rccur:cnI II
TtutC [uststrC[tututta gumCruunC[hrts nay
IoIIov cilcr grou sIrcIococcaI laryngiIis or
scarIc Icvcr and is noI rcvcnIcd Ly anIiLioIic
Ilcray. Iinical naniIcsIaIions IoIlov InIccIion Ly
aLouI 10 days and incIudc IcnaIurIa, cdcna, oIig-
urIa, and IycrIcnsion. onIcncnI [J) lcvcls arc
Iov. TrcaIncnI consisIs oI cnIcIIlIn tlcray and
diurcIics, sIcroids arc rarcIy indicaIcd. In conI:asI Io
aIIccIcd aduIIs, IIe najoriIy oI aIIccIcd cliId:cn
rccovcr viIIouI rcnaI sequcIae.

KLY HLlTb

1. L|||Dl6G W|I| QDdlyGg|I|S S|OUD GOIO6Il6dI6D
W|I| dGI|O|OI|CS 6HQ|||Cd|y S|GC6HOSI 6Q|SOD6S
W||| O6 DU6 IO v||US6S.1D6ldQ6UI|C D6C|S|OGS
S|OUDO6OdS6D OG I|lOdI CUIUl6 Ol |dQ|D
dGl|gCG D6I6Cl|OH I6SI |6SU|IS.
Z. PCUI6 ||6UHdI|C 6v6l |GvOv6S lD6|6dlI,jO|GIS,
dHD Old|G.
d. PCUI6 QOSISIl6QIOCOCCdg|OH6lU|OG6Q|l|I|SHdy
O|OW6|ID6l SK|G O| Q|dlyGg6d |G6CI|OG dGD |S
GOIQ|6v6GICDOydGI|O|OI|L I|6|dQy.
NL1L1LL1LLblb
PthOgCnCs|s
lnIccIious nononucIcosIs is an acuIc sclI-IiniIcd
Illncss laI nay occur during rinary inIccIion vil
sIcin-Iarr vIrus or cy:oncgaIovirus.
L|dCm|O|Ogy
TransnIssIon occurs Ly cxcIangc oI inIccIcd saIiva
[Icncc IIc Icrn "kissing dIscasc"). losI inIccIIons
arc acquIrcd in carIy cIiIdlood, rcsulIIng u asyn-
IonaIic inIccIion or nIId IlIncss. |n conIrasI, lc
nononucIcosIs syndronc occurs nosI I:cqucnIIy
anong individuaIs infccIcd In IaIe cIiIdIood or
adoIcsccnce.
<O > <. ue pm DjOpa 1 >
14Z |
< cKau M pexaek~xcueejcxn: NCA os gQcus. |Y >
L||n|C| Nn|Cstt|Ons
Ht5loryun0hy5tCuLxumtnulton
1lc rcdonInanI synIon Is usualIy a scvcrc,
exudaIive laryngIIIs. Ievcr, roIound IaIIguc, and
gcncraIIzed IynladcnoaIly occur. CIler manIIcs-
IaIions Includc sIcnoncgaIy, aIaIaI eIcclIac, jaun-
dIcc, and rasl. lIlougI Ilc laryngIIis usuaIIy
rcsoIvcs vIIIIn a vcck, IIc naIaIsc nay lasI nucI
Iongcr.
O|CtCnt|| O|gnOs|s
lassIc mononucIcosIs causcd Ly I\ accounIs
Ior nosI cases. |n l\ inIecIion, IyIcaI sIgns oI
nononucIcosIs arc rcscnI In only IaII IIe aIicnIs.
CIIcr InIecIious agenIs IIaI causc sInIIar synIons
IncIude 10x0]/nsmn g0nd, lunan IcresvIrus 6, and
lIV !IaryngIIis causcd Ly g:ou sI:cIococcI or
adcnovirus Is dIncuII Io dIsIinguIsI I:on IlaI oI
nononucIcosIs vIIIouI IaLoraIory sIudIcs. lancy-
IoenIa In Ile rcscncc oI IIe cIInIcaI nanIIesIaIIons
IIsIcd reviously suggcsIs naIIgnancy.
O|gnOst|C Lv|ut|On
Lcukocy:osIs or IcukocnIa nay lc rcscnI, Iyn-
Iocyies accounI Ior norc Ilan 50% oI IcukocyIcs,
and aI IcasI 10% arc usuaIIy aty[ta ym[huty!Cs.
losI cIIIdrcn dcvcIo niId IIronLocy:ocnIa and
^U
c2U
1U
|I!|BIy !|CC!lO|

.
7

_ VLF[N
t

x
x
nIId Io nodcraIc cIcvaIions oI lcaIIc I:ansanInascs.
leIcrolIIc anIILody IcsI alIovs raId deIccIIon oI
I\-assocIaIed nononuclcosIs [LuI noI l\) In Ilc
ouIaIicnI scIIIng, lovcvcr, II las lInIIcd scnsIIIviIy
In clIIdrcn youngcr ilan 8 ycars. SccILc scroIogIc
anIILody IcsIing Is avaIIaLIc Ior I\ [!Igurc I2-1)
and lV
1tCtmCnt
1Ic disordcr Is IyIcaIly scII-IInIIcd, rcquirIng onIy
suoriivc carc. cIivIIy rcsI:IcIions [i.c., no conIacI
sorIs) are advIscd unIII any assocIaIcd slcnoncgaIy
rcsolves Lecause oI Ile ossiLIIiIy oI sIenIc ruIurc.
Iarc LuI scrIous conIIcaIIons IncIudc ucr
aIrvay oLsIrucIion, sIcnIc ruIurc, and ncnIngocn-
cclalIIis. oncurrcnI I:caIncnI vIIl anIcIlIIn
oIicn rccIIIaIcs a cIaracIcrIsIic [LuI IarnIcss)
rasI. JnnunoconronIscd indIvIduaIs arc at rIsk Ior
scvcrc dIsscnInaIcd dIscasc and IynlorolIIcraIIve
dIsordcrs.
KLY HLlTb
1. LdSS|CHOHOHUC|6OS|S |S DU6 lOLQSl6|Hdll v|lUS.
Z. L|G|Cd|HdG|6SIdI|OGS O HOGOGUC6OS|S |GCUD6
6KUDdl|v6 Q|dlyGg|l|S,g6H6ld|Z6DyHQ|dD6GO
QdI|y,6v6l,dHD dI|gU6.
LO|VBCSCC|! SCIOO[y
x
LBIy B|!I[C| VLF[L

C
x
x J
UU
L

l
tUC BI _Q!I[C|

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l
m
^U
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2U
.
.
.
.
.


U

2 ^ 2 ^
VCCKS NO|!|S
J!|C OlOW!|[ O|SC! O !|CSS
Itgur6T2-T P[[CdIdDCC OdDIOOOCSOUID_ L[SICDdII VIUSDCCIOD.
< nOt 1Oz Sa)e1 > < ue JH Qa 1 >
LHLLl
1Ie Iern CIOUj reIers Io vIrus-Induced InLannaIion
oI IIe suIgIoIIIc IIssues, resuIIing u a syrdrone oI
uer aIrvay oIsIrucIIon. rou usually is due Io
arain!uenza virus, LuI can also Le caused Iy oIIer
viruses, sucI as InLuenza and resiraIory syncyIIaI
vIrus [IS\).JI Is mosI ronounced In young cIIldren
Iecause o!IIe narrov caIILer oI IIe aIrvay IeIov
IIe vocaI cords [suLgIoIIic regIon), LuI aIso aIIcIs
adoIescenIs and aduII:. IncIdence eak: durIng IIe
sring and IaIe IaIl. I IIs nosI severe, IIe disease
rogresses Io arIial or IoIal airvay oLsI:ucIion.
L||n|C| Nn|Cstt|Ons
Ht5loryun0hy5tCuLxumtnulton
IiIdren yIcaIly deveIo a Ioarse voIce, Iarky
["seal-IIke' cougI, and sIridor, vIicI nay rogress
Io resIraIory dIsI:ess. Many cIIldren Iave a ro-
drone consisIIng oI Iov-grade Iever and rIInorrIea
12 Io 24 Iours rIor Io IIe onseI oI sIrIdor. IesIra-
Iory conronIse varIes I:on nInInal sI:Idor vIII
agIIaIion Io severe dIsIress vIII IacIynea, IyoxIa,
nasaI LarIng, reI:acIions, and InendIng aIrvay
oLsrucIion.
O|CtCnt|| O|gnOs|s
1Ie diIIerenIIaI diagnosis oI oer aIrvay oLsIruc-
IIon IncIudes eigIoIIIIIs, LacIerial I:acIeIIis, Ioreign
Lody asiraIion, anaIyIaxIs, and angIoneuroIIc
edena.
O|gnOst|C Lv|ut|On
1Ie dIagnosIs usuaIIy Is nade on IIe LasIs oI cIInicaI
LndIngs. leck radiograI reveaIs a Iaered, narrov
suIgIoIIIc aIrvay In IIe anIeroosIerIor vIev [sIeeIe
sIgn) [!Igure I2-2) or vIdenIng oI IIe IyoIarynx
In IIe IaIeraI vIev. 1Iese Lndings are resenI in 50%
oI cases and do noI cor:eIaIe viII dIsease severIIy.
IirecI Iaryngoscoy In IIe oeraIIng roon Is recon-
nended In cases vIen IIe diagnosIs Is uncIear and
IIe cIIId Is crIIicalIy iII [see "IgloIIiIIs").
1tCtmCnt
losI cIIIdren vIII crou never Iecone synIo-
naIIc enougI Io ronI a vIsII Io IIe edIaIrIcIan.
1Iey are usually I:eaIed aI Ione, cougI and sIridor
IgurC T2-2 L|OU[ O d dyCd|OO. NOIC IC SICC[C SgD
DOCdIvCOSUOgOII|C Dd||Ow|Og.
resond veIl Io cooI nIgII air or IunIdiIy. In IIe
energency roon, sIridorous inIanIs receIve oraI,
inIravenous, or inI:anuscuIar sIeroIds, occasIonaIly,
neLulized racenIc eineIrIne Is used Io sIrink
aIrvay nucosa. InendIng resIraIory IaIlure and
aIrvay oLsIrucIion consIIIuIe nedIcal energencIes
and are addressed accordingly [see IaIer !].
KLY HLlTb
t
1. L||D|6G W|ID C|OUQ D6v6OQ d |Od|S6 vO|C6,Od|Ky
[S6d||K6) COUgD dHD SI||DO|,W||CD Hdy Q|Og|6SS
IO |6SQ||dIO|y D|SI|6SS.
Z. GdGIS WDS6v6|6SI|DO| CdUS6DOy C|OUQd
I|6dI6DW|lD SI6|O|DS dHD G6OU|Z6D6Q|G6Q|||G6.
< nOt 1Oz Sa1e1 > < ue nn DjOa 1 >
1A |lucgr|rl5 COIICS
< cKau V exaek~xcueejcxn: NCA os gjOus. jy >
LllL1L11l1lb
PthOgCnCs|s
IgIoIIiIis consIsIs oI InLannaIion and edcna oI
IIc cIgIoIIis and arycIgIoIIIc IoIds. II Is consIdcrcd
a IIIc-IIrcaIcnIng cncrgcncy Lccausc oI ile rocn-
siIy oI Ilc svoIIcn IIssucs Io resuII In suddcn and Irrc-
versILIc aIrvay occIusion.
L|dCm|OIOgy
l. tnuenne Iye L lIL) vas IIe nosI connon
causc In IIe asI, LuI cascs duc Io 5. ]neum0nne
and grou sIrepIococcI IncrcasIngIy arc rcorIcd.
8ccause oI rouIinc adninIsIraIion oI IIc D VattnC
sInce IIc IaIe 980s, Ilc IncIdcnce oI cIgloIiIIIs Ias
dccreascd dranaIicalIy. MosI cases occur durIng Ile
vInIcr nonIIs in cIIIdrcn J to b years oId.
H|sk |CtOts
IaIIurc Io reccIve liL vaccinaIIon Is IIe greaIcsI rIsk
IacIor Ior cpIgIoIIIIIs.
L||n|C| Nn|Cstt|Ons
Ht5loryun0hy5tCuLxumtnulton
Icvcr sore IlroaI, Ioarscness, and sIrIdor dcveIo
ovcr I Io 2 days. Cn cxanInaIion, IIc cIIId las a
ioxIc acarance and Is in sevcrc rcspIraIory disIrcss.
1Ie cIIId vIII IncndIng aIrvay olsIrucIion drools
and leans Iorvard vII cIIn cxIcndcd Io naxInIzc
aIrvay aIcncy.
O|CtCnt|| O|gnOs|s
1Ie dIIIcrcnIial dIagnosIs Is sIniIar Io IIaI Ior crou.
O|gnOst|C Lv|ut|On
LaIcraI ncck radIograIs sIov "IIunl-rInIIng" oI
ilc cIgIoIIIs IIgurc !2-J). Jlougl radIograIs nay
aId In diagnosIs tIcy arc noI rcconncndcd sInce
IIey deIay arorIaIc carc.
1tCtmCnt
JIe cIIId vIII susecIcd cIgIoIIiIIs rcquIrcs IIncIy
IransoriaIIon Io Ilc ocraIing roon and encrgcnI
endoI:acIeaI inIuLaIIon. Fmergcncy cricoIIyroidoI-
ony nay Le crIorncd II an endoi:acIeaI aIrvay
IgUfCT23 L[_OlllS|n4yCIOOwlHSS|vCCOCHO
lCC[_OllS,lCKCDCO IyC[_OllC OOS,DOC!CCHCOlO
ICvCCUC.
cannoI Lc sccured In ilc Iace oI raIdIy rogrcssIve
oLsi:ucIion. Ini:avcnous anIcIIIIn-suILacIan ro-
vIdcs aroriaIe cnIrIc coveragc unIII lIood and
IIroaI culIurcs Iakcn In ilc ocraIIng roon rovidc
IdcnIiLcaIIon and anIILIoIc sensIIIviIy roLIe oI Ilc
InIccIIng organIsn.
KLY HLlTb
1. |Q|g|OlI|I|S |Sd ||6IDl6dI6G|Gg 6H6|g6GCy.
Z. 1|6 lyQ|Cd QdI|6GI |dS dIOK|C dQQ6dldGC6,W|lD
DlOO|GgdGDS6v6l6,QlOgl6SS|v6f6SQldIOly
D|Sll6SS.
d. W|6G6Q|g|OII|I|S |SSUSQ6CI6D,l|6 C||D S|OUDO6
IldGSQO|I6D lO I|6OQ6ldl|GglOOH Ol 6GDCI|d
C|6d||GIUOdI|OG dHD D|l6CIv|SUd||ZdI|OGUGD6l
g6G6|d| dG6SI|6S|d.
< nOt 1Oz saci > < uc nn DjOa i >
UHL1LHlL1l1lb
PthOgCnCs|s
IroncIIoIIIIs Is an acute vIral Iovcr rcsiraIory I:ac
inIccIion IIaI rcsulIs In an InLannaIory oLsIrucIion
oI IIe criIcraI aIrvays. 1Icrc is a rcdoninanIIy
IynIocyIIc InLIIraIc InIo Ilc crILroncIIaI and
crIlroncIioIar cIIlcIIun IlaI ronoIcs suLnu-
cosaI cdcna. InIraIunInaI nucous Iugs and ccIIular
dcLrIs accunulaIc duc Io inaIrcd nucocIIIary
cIcarancc.
L|dCm|OIOgy
IcsIraIory syncyIIaI vIrus causcs 65% oI cascs, vlIIe
araInLucnza, In!ucnza, and adcnovIrus arc rcson-
siIIc Ior IIc rcnaInIng J5%. IroncIIoIiIIs occurs
lcvccn lovcnLcr and rII. I IcasI lalI oI aII cIII-
drcn arc InIccIcd viII IS\ durIng lc LrsI ycar oI
IIIc, and rccurrcnI InIccIions arc connon. Icvccn
0.5% and 1 % oI aII cIildrcn vIIl LroncIIoIiIIs
rcquirc IosIIaIIzaIion. JIe dIscasc Is nosI scvcrc in
InIanIs youngcr IIan J nonIIs, Iorncr rcnaIurc
inIanIs, and cliIdrcn viII undcrIyIng IcarI or Iung
dIseasc.
H|sk |CtOts
IIIdrcn vIII cIronIc Iung dIscasc, congcniIaI IcarI
diseasc, and congcniIal or acquIrcd InnunodcLcIcn-
cIcs arc norc susccIILIc Io scvcrc discasc. lrcdIcIors
oI scvcre IIIncss IncIudc rcsIraIory raIc grcaIcr Ilan
70/nInuIc, IyoxIa, aIclccIasis on cIcsI :adIogral,
and IIsIory oI rcIcrn lirIl.
L||n|C| Nn|Cstt|Ons
Ht5lory
1Ic acuIc IIIncss IasIs Ior 5 Io 10 days, IoIIovcd Ly
gradual rccovery ovcr Ilc ncxI I Io Z vcck:. InIccIcd
nconaIcs nay dcvcIo IIIc-IlrcaIcning ajHCa. InIanIs
InIIIaIIy dcvcIo Icvcr, cougI, and rlinorrIca IoI-
Ioved Ly rogrcssIvc rcsIraIory disI:css. louscIoId
conIacIs usuaIIy Iavc ucr rcsiraIory synIons.
hy5tCuLxumtnulton
IIndings on cxan incIudc Icvcr, IacIynca, and nIId
Io scvcrc rcsIraIory dIsI:css. WncczIng, rIoncIi,
crackIcs, and accessory nusclc usc durIng rcsIraIIon
[Iugging) nay Lc noIcd. III InIanIs nay Lc rcsIIcss or
IcIIargIc. lyoxia Is connon In sevcrcIy anccIcd
aIicnIs.
O|CtCnt|| O|gnOs|s
1lc vIcczing assocIaIcd vIl lroncIIoIiIis nay le
dIIcuII Io dIsIinguisl Iron rcacIivc aIrvay dIscasc
or aIrvay IorcIgn Lody In oIdcr InIanIs. auscs oI
rccurrcnI cIsodcs oI vIcczIng IncIudc vascuIar
rIngs, cysIIc LLrosIs, and cIlIary dyskinesIa.
O|gnOst|C Lv|ut|On
1Ic vIrus nay Lc cuIIurcd I:on nasaI sccrcIons,
Iovcvcr, innunoLuorcsccncc oI nasoIaryngcaI
asIraIc Io dcIccI vIraI anIigcns Is a raId and racII-
caI aIIcrnaIIvc. IcsI x-ray slouId Lc oLIaincd Ior III
or Iyoxic aIicnIs and Ior Iosc viIl rccurrcnI or
uncxIaincd vIcczIng.
1tCtmCnt
HyoxIc or iII-acarIng cIIIdrcn rcquIrc losiIaI-
IzaIion. IIIdrcn vIIl oxygcn saIuraIIon grcaIcr lan
94%, nInInaI rcsIraIory disrcss, good LuId InIakc,
rcIiaLIc carcIakcrs, and good IolIov-u nay Lc
IrcaIcd as ouIaIIcnIs.
losI InIans rcquire onIy suorIivc carc Ior IcIr
scII-IinIIcd iIlncss. 1Ic LcncLI oI LronclodiIaIors
and corIicosIcroIds is conIrovcrsIaI. WIIIc LcIa-
agoniss nay I:ansIcnIIy Inrovc rcsIraIory syn-
Ions, IIcy do noI acar Io slorIcn le duraIion oI
iIIncss or IosiIaIIzaIIon. IIa-adrcncrgIc agcnI:
sucI as cIncIrIne, gIvcn Ly InIalaIIon, nay Lc
norc LcncLcIaI Ior IIc IosiIaIizcd InIanI. orIIco-
sIcroids Iavc n0/ Lctn cIcarIy sIovn Io aIIccI IIc
coursc oI IIc dIscasc, Iovcvcr, adnInIsI:aIion carIy
in Ic coursc oI iIIncss nay LcncL tIosc InIans vIII
a IaniliaI rcdIsosIIIon Io rcacIivc aIrvays discasc.
IIlavIrIn, an anIivIraI agcnI la surcsscs vIraI
Il oIyncrasc acIivIIy, nay slorIcn synIons,
and IIs usc sIouId Lc consIdcrcd In aIicnIs vIIl
undcrIyIng clronIc Iung discasc or Innunosurcs-
sIvc condiIIons.
IcsICan, an inIravcnous InnunogIoLuIIn vIII
IigI IS\ anIILody conccnIraIIon, and aIIvizunal,
an injccIaLIc IS\ nonocIonaI anIILody, rovidc
assIvc roIyIaxIs and arc rcconncndcd durIng Ilc
vinIcr nonIIs Ior aIicnIs aI rIsk Ior scvcrc discasc
[csccIaIIy Iorncr rcnaIurc InIanIs).
1Ic norIaIIIy raIc Ior IosIIalIzcd aIIcnIs is
aroxInaIcIy 1 %. IIIdrcn vIIl congcnIIal Icar
1A
O p,>
I
< :te J7R DjOHaw i >
< cKau v exaaK~Kcueejcxn: NCA os gjcus. jy >
lLb
dcIccIs, cIronic Iung dIscasc, and InnunodcLcIcncy
Iarc aricuIarIy oorIy PaIIcnIs vIII docuncnIcd
IS\ lroncIIoIIIIs lavc norc aIrvay lycrrcson-
sivcncss laIcr In IiIc IIan ilc gcncraI oulaIIon,
causc vcrsus cIIccI Ias noI lccn cIucIdaIcd.
KLY HLlTb
1. lOGCD|O|I|S |S d S6|H|I6D OUI QOI6GI|dy S6v6l6
|G6CI|OG |G |GdHIS,6SQ6C|dy I|OS6 W|I| UHD6ly
|Gg COGD|I|OHS.
Z. lOGCD|O|I|S |S DU6 IOOW6l d|lWdy OOSIlUCI|OH
dGD,I|6l6Ol6,HOSICD|Dl6G D6v6OQ W|66Z|Hg Ol
lDOGCD|.
d. PQG6d |S d l6QU6GI Ql6S6HIdI|OH |GH6OGdI6S.
PLH1Lbblb
InIccIion vIII 0rde/e//n ]er/ussts causcs ucr rcs-
IraIory IracI InIccIIon and crsisIcnI cougI in aduIIs
luI nay rcsulI In IIIc-IIrcaIcnIng rcsIraIory dIscasc
In nconaIcs and InIanIs.
L||n|C| Nn|Cstt|Ons
Ht5loryun0hy5tCuLxumtnulton
laIIcnIs vIII criussIs are alnosI InvarIaLIy aIcLrIIc.
Jlc cIassic rcscnIaIion In young cIIId:cn Is vIoo
Ing cougI." 1Ic tatarrhal [hasC consIsIs oI 1 Io 2
vccks oI Iov-gradc Icvcr, cougI, and coryza. 1Icn
concs a 2- Io 4-vcck [arC\sma [hasC cIaracIcr
Izcd ly aroxysns oI cougI IolIovcd Ly suddcn
InIaIaIion, vIIcI roduccs Ilc cIaracIcrIsIic vIoo.
PosI-IussIvc cncsIs Is connon. MosI synIons
rcnIi durIng Ilc tunVaCstCnI [hasC, luI Ilc cougI
nay IasI Ior 2 Io 8 vcck:. lcvLorns vIII scvcrc
dIscasc nay rcscnI vIIl anca or IIc IyicaI arox-
ysnal cougI IoIIovcd Ly cIokIng and rogrcssIvc
cyanosIs. 1Ic claracIcrIsIic "vIoo" Is alscnI in vcry
young InIanIs sIncc Ilcy cannoI gcncraIc suncicnI
ncgatIvc InsIraIory Iorcc. Iacial cIccIIac and scIcral
Icnorrlagcs oIicn dcvcIo as a conscqucncc oI
IorccIuI couglIng.
O|gnOst|C Lv|ut|On
LaLoraIory cvaIuaIion usuaIly rcvcals Icukocy:osIs
[>J0,000 vlIIc lIood ccIls cr jt!) vIil a rcdonI-
nancc oI IynIocy:cs. lasoIaryngcaI sccrcIions
conIaIn tlc organIsn, vIicl nay le dcIccIcd ly
LuorcsccnI anIilody sIaInIng, lI, or cuIIurc. JIc
cIcsI x-ray usuaIly Is nornaI, luI nonsccI!c InLI-
Iraics nay Lc sccn.
1tCtmCnt
Young InIanIs vIII scvcrc dIscasc sIould Lc IosI-
IaIIzcd Io nanagc anca, cyanosIs, IyoxIa, and
Iccding dIIculIIcs. ryIlronycIn slorIcns Ilc dura-
Iion oI IIIncss II gIvcn carIy In IIc caIarrIal Iasc.
Iicr Ilc cougIIng aroxysns lcgIn, anIIlIoIics do
noI afIccI IIc coursc oI IlIncss luI arc rcconncndcd
Io dccrcasc IIc crIod oI InIccIIvIIy. 14-day coursc
conlcIcly cradIcaIcs ilc organIsn Iron IIc
nasoIarynx and rcsIraIory I:acI. louscloId and
oIlcr cIosc conIacI: rcquirc cIcnorolyIaxIs vIIl
cryilronycIn.
1Ic accIIuIar crIussIs vaccInc Is 95% cIIccIIvc
agaInsI scvcrc IlIncss, aIIlougI aI IcasI onc-Ilird oI
InnunIzcd indIvIduals are susccIIllc Io nIld InIcc-
IIons laIcr In lIIc.
KLY HLlTb
1. 1D6WDOOQ|G Q6|IUSS|S |S I|6 OGg, SIl|DUOUS
|GSQ|ldI|OG dI6| I|6 QdlOKySHd COUg|.
Z. L6UKOCyIOS|S W|ID d Ql6DOH|GdGC6 O dIyQ|Cd
yHQ|OCyI6S |S IyQ|Cd| O Q6lIUSS|S.
d. 1|6DlUg O CDO|C6 |S 6lyIDlOHyC|G.
l1LLNL1lP
PthOgCnCs|s
nCumuna rcIcrs Io an acuIc InLannaIory roccss
occurrIng In ilc Iungs. II nay Lc InIccIious or
nonInIccIIous.
L|dCmO|Ogy
1Ic agc oI an InnunoconcIcnI cIIId suggcsIs an
cIioIogIc organIsn [TaLlc 12-4). \Iruscs arc Ilc nosI
connon causc oI ncunonIa In young cIIIdrcn.
Lh/nmydn /rnchomn/ts ncunonIa nanIIcsI: aI 2 Io
J nonIls oI agc in InIanIs lorn Io voncn vIII
unI:caIcd gcnIIaI L. /rnch0mn /ts InIccIion. 5.
]neum0nne sIouId lc consIdcrcd u any con-
nunIIy-acquIrcd Iovcr rcsIraIory IracI inIccIIon.
< nOt 1Oz Sa)e1 > < ue )7H DjO)a 1 >
| 1bLL 124
LUS0S D DmDDD D Pg0
<T NODtD
LIOU[ SIIE[\OCOCC
LIdH~DC_IVECDICIC OC
lOHy/OCOCCU5 OUtU5
LyIOHC_OV|U5
L|5lt|O OODOCy!OgD5
HbV, lCSQl1Ot SDCll VlUS.
T O NODtD5
H5V
|DUEDZ
|IdDUCDZ
ltlOCOCCU5 DUOOD|O
LH/OOy|O ltOCHOOO lt5
MOOOH|/U5 |DUDZO
lOHy/OCOCCU5 OUtU5
yc0]nsmn ]neum0nne neunonia is unconnon in
cIiIdrcn youngcr Ilan 5 years. !css connon IacIcr-
IaI causcs IncIudc nonIycaIlc l. njuenne 5.
nureus and grou sIrcIococcI.
H|sk |CtOts
ondIIions assocIaIcd vIII an incrcascd rIsk oI Iac-
Icrial ncunonIa includc IIe IolIovIng:
Ironic Iung dIsease, includIng cysIic !Irosis
lcurologIc inairncnI vIII svaIlovIng dysIunc-
Iion
CasIrocsoIagcaI rcLux vIIl asIraIIon oI gasIrIc
conIcnIs
\cr airvay anaIonic deIccIs [IracIeo-soIagcaI
LsIuIa, cIe aIaIc)
lcnogloIInoaIlics [IncIudIng sIckIc ceII dIscasc)
InnunodeLcIcncy or InnunosurcssIvc Ilcray
L||n|C| Nn|Cstt|Ons
Ht5lory
\IraI neunonIa dcvclos graduaIly ovcr 2 io 4 days.
II Is usualIy rcccdcd Iy ucr rcsIraIory synIons
sucI as cougI, rIinorrIea, osInasal drI, coryza, and
Iov-gradc Ievcr. InIanIs vIIl ncunonIa causcd Iy
C /rnch0mn/ts arc aIcIrIIe and Iave conjuncIIvIIIs
and a sIaccaIo cougI. InIanIs and young cIildrcn
vIIl IacIcraI ncunonIa nay rcscnI vIIl non-
sccihc consIiIuIIonaI conIainI-, incIuding Icvcr,
IrrIIaIiIIIy, oor Ieeding, vonIIIng, aIdonInal aIn,
NODtD5 tO Y0f5
H5V,[dIdDUCOZ,DUCDZd,
dOCDOVIUS
ltlOCOCCU5 DUOOD|O
MOOOH|/U5 tDhUDZO
lOHy/OCOCCU5 OUtU5
NyCODOClt|UO lUDtCU/O5|5
bCDOOl P@0PUOl05C0Dt
NyCO/O5OO DUOODtO
LH/OOytO D6UOODtO
lt6lOCOCCU5 DUOODtO
|DUEDZd
NyCODOClt|UO
lUDtCU/O5|5
LIOU[ P SIIE[IOCOCC
lOHy/OCOCCU5 OUtU5
and lcIIargy. IruI onseI oI Icvcr, cIiIls, dysnca,
and cIcsI ain Is IyIcaI. lroducIivc cougI is norc
connon In oIdcr aIIcnIs. . ]neum0nne and C
]neum0nne ncunonIa rcsenI InIIIally vIil Icvcr,
lcadacIc, and myalgIa. 1Iesc synIons graduaIIy
suIsIdc over 5 Io 7 days, vIilc cougIing increascs
and crsIsIs Ior 2 vecks or norc.
hy5tCuLxumtnulton
ny IndicaIion oI rcsIraIory dIsIrcss can sIgnaI ncu-
nonia, aIIIougI IacIynea and dysnea are nosI
connon. TacIynca ouI oI rooriion io Ievcr Is an
inorIanI cIue Io ncunonIa In IIc young clIld.
IIIIusc vlcczing and crackIcs suggcsI involvcncnI
oI nuIIiIc areas oI Ilc lung, cIaracIcrIsIIc oI vIral
or aIyical [. ]neum0nne C ]neum0nne C lrG
ch0mn/ts| neunonIa. IocaI LndIngs sucI as IocaI
crackIcs or decrcased IrcaII sounds, duIIness Io cr-
cussIon, cgoIony, and IroncIolony suggesI ncu-
nonia oI IacIcrIaI orIgIn. yanosIs Is unconnon
exccI H severc dIscasc. roxInaIcIy !0% oI
aIIcnIs vIII . ]neum0nne inIecIion deveIo a
rasI, usuaIIy eryiIcnaIous and nacuIoaular and
occasIonaIIy cryilcna nuIIiIornc.
O|CtCnt|| O|gnOs|s
lncunonIa is nucI norc connon In IIe cdIaIrIc
ouIaIion Ilan arc oIIcr condIIIons vIII sinIIar
rcscnIaIions, incIudIng congcsIivc IcarI IaIlurc,
cIcnical neunonIIis, uInonary cnIoIIsn, sar-
coIdosis, and rInary or neIasIaIIc naIIgnancy.
14b
<O 1qz e > <, ue )H DjOa i >
< cKau V exaek~xcueejcxn: NCA os gQOus.
|Y >
IC
O|gnOst|C Lv|ut|On
ilorougl IIsIory and IysIcaI cxanInaIion suggcsI
Ilc dIagnosIs. SuIun cuIIure Is noI IIkcIy Io Lc
IclIuI, sIncc cdIaIrIc aIIcnIs gcncralIy do noI
roducc suIun sanIcs. CIcsI x-ray renains an
exccIIcnI IcsI Ior dcLnIng Ile cxIcnI and aIIcrn oI
InvoIvcncnI and asscssIng reIaIcd conIIcaIIons [I.c.,
IeuraI cIIusIon, ncunaIoccIc). IacIcriaI ncuno-
nIa causcs Iolar consoIIdaIIon. IIIIusc InIcrsIiIiaI
InLIIraIcs suggcsI vIraI or aIyIcal neunonIa,
IIougI clIIdren vIII yc0/n?mn ncunonIa nay
Iavc Iolar consoIIdaIIon. sIraIIon ncunonIa is
IyIcaIIy IocaIed In IIc rIgII nIddIc or rIgII ocr
loLc. C. /rGch0mn /ts ncunonIa can lc dIagnoscd Ly
dirccI huorescenI anIibody IesIIng oI conjuncIIvaI or
nasoIaryngcaI sccIncns. . neum0nne InIccIion
nay Lc dIagnoscd Ly lCI oI sccIncns oLIaIncd ly
nasoIaryngcaI svaL or ly sccILc anIinycolasnaI
IgM anIilody dcIcrnInaIIon. CoId-aggIuIInin IIIcrs
arc cIcvaIcd noI onIy In . neum0nne InIecIIons LuI
aIso in nany cascs oI vIral neunonIa and sonc cascs
oI LacIcrIaI neunonIa.
1rCtmCnt
1Icray dccnds on IIc nosI likcIy aIlogen. In IIc
ouIaIIcnI scIIing, anoxicIlIIn is arorIaIc Ior
nosI cascs oI IacIcrIaI ncunonia vIcn anIilIoIIcs
arc IIougII io lc ncccssary. noxIcIIIin-cIavuIanIc
acId or a sccond- or IIIrd-gencraIion ccIaIosorIn
nay lc ncccssary vlcn l. njuenne or 5. nureus
are susccIed. ryIIronycIn, cIarIilronycIn, or
azIIlronycIn is rcconncnded Ior "vaIking ncuno-
nIa" causcd Ly . neum0nne or C. neum0nne.
ryiIronycIn Is uscd Io IrcaI InIanI: vII inIccIion
causcd Ly C. /rGch0mnl?.
ny cIIId vIII ersIsIen IyoxIa or nodcraIe Io
scvcre rcsIraIory dIsi:css requIrcs IosIIaIizaIIon.
InIravcnous anIcIlIIn is aprorIaIe InIIIaI IIcray
Ior IosIIaIized cIIIdrcn vIII susccIcd lacIcrIaI
ncunonIa, IIougl sccond- or IIird-gencraIion
ccIalosorIns arc oIicn uscd lccausc oI conccrn
rcgarding rcsIsIanI 5. neum0nne. MosI vIraI inIcc-
iIons arc scII-IInIIcd.
1Ic nosI rcqucnI conIIcatIon Is dcveIoncnI
oI a lcuraI eIIusIon Iargc enougI Io conronIsc
rcsIraIory cIIori. lIcuroccnIcsIs viII ossILIc cIcsI
IuLc IaccnenI rovIdcs raId reIIcI nycna
rcsuIIs vlcn uruIcnI LuId Iron an adjaccnI Iung
InIccIion draIns InIo IIc IcuraI sacc. Lung alsccsscs
nay conIIcaIc anacroLIc InIccIions.
KLY HLlTb
1. .QD0uDOD00 |S ID6 HOSl COHHOG CdUS6 O OdC
l6l|d| QG6UHOG|d. PHOK|Ct|G O| dnQ|C|||H |SID6
If6dIH6Gl O CDOC6.
Z. N. QD0uDODO0 SDOUDO6 COGS|D6|6D |G O|D6l C|||
Dl6G dGD dDO6SC6GIS.NdClO|D6 dGI|O|OI|CS dl6
I|6 Il6dlH6HI O CDO|C6.
d. 1|6 QdII6lG O |GP|IldI6 OG C|6Sl ldD|OgldQ| Hdy
SUgg6SIID6 6I|O|Og|Cdg6GI.
NL1l1Ll1lb
PthOgCnCs|s
InosI any aIIogcn can InIccI Ilc IcIoncnIngcs
and ccrclrosInaI LuId. \IraI ncnIngIIis Is IyIcaIIy
an acuIc, scII-linIIcd IIIncss, LacIcrIaI ncnIngIIIs Is a
IIIe-IlrcaIcnIng condIIIon associaIcd vIII suLsIanIiaI
norLIdIIy and morIaIIIy. TIc Icrn asC[tt mCnng
!S rcIcrs Io nenIngcaI InLannaIIon caused ly an
anIigtnIc sIinuIus oIIcr IIan yogcnic LacIerIa [c.g.,
cnIcrovirus or 0rre/n| .
L|UCm|O|Ogy
1Ic IIkcIy cIIoIogy oI ncnIngIIis dccnds on agc
[1aIlc !2-5). lconaIcs and cIIIdrcn youngcr IIan
J ycars arc aI IIgIcsI risk Ior LacIcrIaI nenIngIIis.
5. neum0nne and I metngt/td? are Ilc nosI
connon rcsonsIlIc organIsns. IIL vaccInc Ias
nearIy cIInInaIcd l. njuenne Iyc L neningIIIs
In Ilc \nIIcd SIaIcs. IoIl inIanIs and oldcr cIIIdrcn
are aI rIsk Ior ncnIngIIIs causcd ly cnIerovIruscs
[IIc nosI connon causc oI vIral ncnIngiIIs).
ncroviruscs circuIaic rinarIIy In IIc laic sunncr
and carIy IaII. Lync ncnIngiIIs, causcd ly 0rre/n
|urgd0Qert usuaIIy aIIccIs scIooI-agc cIiIdrcn and
adoIcsccnIs. Rare causcs oI ncnIngIIIs and mcnIn-
gocnccIaIIIis includc I\, nr/0ne//n heItse/ne
[caI-scraIcl dIseasc), . neum0nne . /u|ercu/0s?
and L/0c0ccus ne0j0rnnn?.
H|sk |CtOts
IIsk IacIors Ior LacIcriaI ncnIngIIis arc IIe sanc as
ilosc Ior sesIs, Lccausc nosI cascs IoIIov IenaIogc-
nous scedIng. |IrccI InvasIon [nonIcnaIogcnous)
occurs as a rcsulI oI Irauna, nasIoIdIIIs, sInusIIis, and
anaIonIc dcIccI: In Ilc scal or skuII. In Ilc nconaIc,
< nOt 1Oz Sa)e1 > < ue nn DjOa 1 >
< CKd V eeuee

n os __us |Y
>
.
d[ICI D1CI oS LlSCdSC TA9
* 1bLL 12
LU505 D N0DDgL5 D Pg0
<J NOHtD 1 tO Z NODtDS Z NODD5 tO YEfS bCDOOl PgEPUOlE5C0Dt
LIOU[ b SIIC[IOtOCt
5CHt|CH|O CO/|
5CHuCH|O CO/| ltlOCOCCU5 DUOOD|O ltlOCOCCU5 DUOOD|O
N|55t|O OD|Dg|l||5
LDICIOVIUSCS
ltlOCOCCU5 DUOOD|O N|55t|O OD|Dg|l||5
LICI_IdHDC_dIVC OdC
CI[CS SH[EX VIUS
LDICIOVIUSE5 LDICIOVIUSCS
MOOOH|/U5 |DUDZO bOtt/|O DUtgOt/t| bOtt/|O DUtgOt/0t|
Iy[C O"
L|5lt|O OODOQlOgD5 LIOU[ b SIIC[IOtOCC
ltlOCOCCU5 DUOOD|O
HtC D DDUDtZCO QOQUl1OD5.
Iov LIrII vcIglI, roIongcd ruIurc oI ncnLrancs,
and cIorIoannIonIIIs rcdisosc Io scIiccnia and
ncnIngIIIs nycIoncnIngocclc aIso incrcascs IIc rIsk.
L||n|C| Nn|Cstt|Ons
Ht5lory
\IraI ncnIngIIIs Is rcccdcd Ly a nonsccILc ro-
dronc IncIuding Icvcr naIaIsc, sorc IlroaI, and nyaI-
gIas. IIldrcn lcn dcvclo nausca, voniIIng,
IoIoIoLIa, irrIIaLIIIIy, IcIlargy Ieadaclc, and sIIII
ncck. \nIcss conlIcaIcd Ly cnccIaIIIIs, synIons
oI cnIcrovIraI ncningIIIs gcncraIIy rcsoIvc ovcr 2 io
4 days and nay Inrovc aIicr IunLar uncIurc.
Lync ncnIngIIIs Is cIaracIcrIzcd Ly Iov-gradc Icvcr,
lcadacIc, sIiII ncck, and IoIoIoLIa dcvcIoIng
ovcr IIc coursc oI 1 Io 2 vcck:. ranIaI ncrvc aIsIcs
nay occur. In LacIcrIaI ncnIngiIis, IIc rodronaI
Iasc Is aLscnI and IIc Icvcr Is gcncraIIy quIIc IIgI.
lcnIal sIaIus cIangcs, IocaI ncuroIogIc sIgns, aIaxIa,
scizurcs, and sIock arc noI unconnon.
hy5tCuLxumtnulton
lnIanIs nay rcscnI viI a LuIging IonIancIIc. In
oIdcr cIIIdrcn, signs oI incrcascd inIracranIaI rcssurc
IncIudc cranIal ncrvc aIsIcs and aIlIcdcna.
IucIaI rIgIdiIy and osIIIvc bCrngs [LcxIon oI IIc
Icg aI Ilc II vIil suLscqucnI aIn on Incc cxicn-
sion) and HruCznsks [InvoIuniary Icg HcxIon on
assivc nccI hcxIon) sgns arc narkcrs Ior ncningcaI
IrrIIaIIon. JIcsc LndIngs arc rarcIy rcscnI In cIII-
drcn youngcr IIan 1 ycar. |aIIcnI: vIl ncnIngIIIs
causcd Ly I menngi/tds nay rcscnI vIil cIccIIaI
or ururIc skIn IcsIons. ArIIraIgIas arc connon
vIII ncningococcaI ncnIngIIIs. n cryIIcna
nIgrans rasI nay acconany !ync ncningIIIs.
MOOQH|/U5 |DUDZO
Iy[C O"
O|CtCnt|| O|gnOs|s
JIc dIIIcrcnIIaI dIagnosIs incIudcs cncclaIIIis,
vIIcI nay dcvcIo concurrcniy or suLscqucnIIy
[scc IaIcr 15). CIIcr condIions IIaI nay rcscnI
vIl a sinIIar clInIcaI IcIurc IncIudc drug InIoxica-
IIon or sidc cIIccIs, rcccnI anoxIa or lyoxIa, rinary
or ncIasIaIIc ccnI:aI ncrvous sysIcn [IS) naIig-
nancy, LacIcrIal cndocardiIIs vIII scIic cnLolIsn,
|nIracranIaI IcnorrIagc/IcnaIona, naIIgnanI Iy-
cricnsIon, and dcnycIInaIIon dIsordcrs.
O|gnOst|C Lv|ut|On
!unLar uncIurc Is dIagnosIic. cIl counIs and diI-
IcrcnIIal, Cran siain, gIucosc, and roIcIn lcvcIs
slouId Lc dcIcrnIncd. IIood and SI cuIIurcs
sIouId Lc oLIaincd. IacIcrIa arc dcIccIcd on Cran
sIaIn In 80% oI cascs oI LacIcrIaI ncnIngIiIs. lI
assays Ior SI Icrcs sinIcx vIrus [I S\} ,
cnIcrovIruscs, and Lync dIscasc arc avaIIaLlc and arc
IigIIy scnsIIIvc and sccILc. SI LndIngs laI sug-
gcsI a sccILc cIIoIogy arc dcscriLcd |n 1aLIc 12-6.
LunLar uncIurc sIouId noI Lc aIicnIcd In a cIiId
viII IocaI ncuroIogic dc!ci\s un\iI an cxanding
nass IcsIon Is cxcIudcd Ly LJor nagncIIc rcsonancc
|nagIng, Lccausc oI ilc oIcnIIaI Ior LraIn sIcn Icr-
nIaIIon. CIIcr conI:aIndIcaIIons incIudc cardioul-
nonary InsIaLIIIIy and skIn InIccIIon ovcrIyIng IIc
IunLar uncIurc sIIc.
1tCtmCnt
WIcn IIc dIagnosIs oI unconIicaIcd viral ncningI-
Iis Is uncquIvocaI, IosIIaIIzaIIon Is gcncraIIy noI
ncccssary II LacIcrIaI ncnIngIIIs cannoI Lc cxcIudcd,
1
<O 1Oz e

<ue J7R DjOH


3d HCS
1bLL 12b
1 >
< cxon v exaek~xcueejcxn: NCA os gjcus. |Y >
L0l0DlD5QD UU DUDgS bUgg0StDg B bQ0LDC tDlDg l
N0DDgtS D LDUDDU
LbI lfD0t0f Ct0fl Vf L@D0
WD|I3O|CCU L3|S[6| nn`| >1Z <b <1
N3uIlCH||S >1% <b%" <d%
|rCI3|G TT NC|nd|C|T NC|nd| C|T
L|uLCS3 C| NC|nd NC|n
NCU1lOQlS D QtCOODD1E Ell D 1C COUt5C O Vll DCDDQ1S, DODODUClEt CEllS USUll
QlCOODD1C D LDC DCDDQ1S.
Lbl, CClCDll SQDl lUO, T,DlO DCtCSC, TT,DOOEt1C Ol SCVCtC DClESC, 1,DlO OCCtCSC, 11,
DOOCt1C Ot SCVClC OECtCSC.
Ilc aIIcnI slould Le IosIIaIIzed Ior Ini:avenous
aniILIoiic IIeray.
\anconycIn lus a IlIrd-gcncraIIon ccIalosorIn
[ccIoIaxInc or ccIt:Iaxonc) acIIcvc ilcracuIIc
leveIs In IIc SI and rovIdc Lroad-sccIrun cov-
cragc oI Ilc nosI IIIcIy aIlogcns In InIanI: and
oIdcr clIld:cn. lconaIcs sIouId Lc IrcaIcd vIIl
anicIlIIn Io I:caI grou L sI:cIococcI and . m0n0
/0genes ceIoIaxInc Is addcd Io IreaI gran-negaIIve
ailogens. Cncc an organisn and I\ susccIILIIIIy
aI:crn arc avaIIaLIc, anIILIoIIc covcragc nay Lc
adjusIcd. Jlc course oI IIcray Ior LacIcrIaI menIn-
gIIIs Is usualIy 10 days. |xccIIons Includc ncnIn-
gococcaI ncnIngIIIs [57 days), Lync neningIIIs
[1428 days), and neonaIcs [14Z1 days).
Jle currcnI norIaIIIy raIc Ior LacIcrIaI ncnIngI-
IIs Is J0% Ior nconaics and Iess IIan 5% Ior InIanis
and oIdcr cIiIdren. Iovcvcr, 15% Io J0% oI aIIcnIs
cxcrIcnce sonc crsIsIcnI ncuroIogIc dcLciI, nosI
connonIy IcarIng Ioss, dcvcIoncnIaI dclay, noIor
IncoordInaIIon, scIzures, and IydroccIaIus. lorLId-
IIy and norIaIIIy are ligIer aItcr InIccIIon vIil
gran-ncgaIIvc organIsns.

KLY HLlTb
1. N6H|Hg|l|S HdyO6 S6Ql|C [OdCl6l|d) Ol dS6Ql|C.
Z. HHUG|Zdl|OHW|lD |O vdCC|G6 |dS DldHdl|Cdy
O6Cl6dS6D ID6 |GC|D6GC6 O CD|D|OOD H6H|Gg|I|S,
COGjUgdI6 QG6UHOCOCCd vdCC|G6[|l6vGdl) |S
|K6y IOl6SUI |H D6Cl6dS6O l6QU6GCy O QG6U
HOCOCCd H6G|Gg|I|S dHOGg |GdGIS.
d. LUHOdl QUHClUl6 |S |GvdUdO6 |G l|6 D|dgGOS|S
dGD I|6dIH6HI SIldl6gy O H6G|Gg|I|S.
4. N6W LHOdS6D dSSdyS dC||IdI6 l|6 D|dgGOS|S O
bV,6HI6|Ov||d, dGDLyH6C6GIldH6lvOUS SySI6H
|G6CI|OG.
LPb1HLL11LHl1lb
laIlogcns causc dIarrIca Ly a varIeIy oI ncclanIsns.
Ior cxanlc, sonc lacIerIa Invadc InIcsIInal IIssue
dIrccIIy, vIereas oIlcrs sccrcic InjurIous Ioxins
lcIore or aIIcr IngesIIon. \Iruscs, arasIIcs, and ro-
Iozoa aIso arc caaLle oI InLIcIIng dIscasc. xccssIve
sIooIIng causcs dcIydraIIon, Inadcquaic nuIrIIIon,
and cIccIroIy:c aLnornaIIIIcs, alI oI vIIcI arc oorIy
IoIcraIed in inIanIs and snaII cIIIdrcn.
L||n|C| Nn|Cstt|Ons
Ht5lory
JIc IIsIory sIouId IncIudc InIornaIion aLout
syntons In oIlcr IanIIy mcnLcrs, recenI IravcI,
ncdIcaIIon usc, Innune sIaIus, day-carc aIIcndancc,
source oI drInIIng vaIcr, conIacI vIIl anInaIs, dura-
IIon oI synIons, Iever, and nunLcr, coIor, and cIar-
acIer oI sIooIs.
Jlc nosI connon LacIcrIaI causes oI gasIroen-
IcrIIIs Includc 5n/m0ne//n s., 5hge//n s., . c0/
Jersnn en/enc0//tcn and Lnmg/0|nc/er ]e]un,
V|r0 ch0/erGe nay Le acquIrcd durIng I:avel Io
IndIa, IrIca, or Ilc Middle asI and I:on caIIng
undcrcooIcd CulI oasI sIcIlLsI. laIIenIs vIIl Lac-
IcrIal dIarrIca rcscnI vIIl Iever, sIgnILcanI aldon-
InaI cranIng, nalaIsc, and Icncsnus, vonIIIng Is lcss
connon. JIc sIooIs conIaIn nucous and nuy lc
guaIac osIIIvc or nIxcd vIIl LIood. CccasionaIIy,
clIIdrcn vIIl sIIgcIIosIs rcscnI vIII nCuruugt
nanIIcsIaIIons [IeIIargy, scIzurcs, nenial siaius
cIangcs), ossILly due Io a neuroIoxIn cIaLoraIcd Ly
Ilc organIsn. 5n/m0ne//n s. arc caaLIe oI InvadIng
Ilc LIoodsI:can and causIng exI:aInIcsIInaI dIscase,
IncIuding neningIIIs and osIconycIIIIs [arIIcuIarIy
In cIIIdren vIIl sIcIlc ccII dIscasc). 5hge//n dysen/e
< IOI TOI SB1O1 ~ * HO IR IOB 1 ~
riae anU L. co|i \b . prCUucC an cn!crC!Cxin
[>iga Cr >iga-iKc !Cxin| assCciatCU Vit hCmOyIt
urCmt 8yuUrOmC a scrtCus cCnplica!tCn cCnsts!tng
C ncrCangiCpatic cnCytic anCnia ncprCpa!y
anU trCnCcytCpcnia. nCsI ZbV C! inUiviUuas
ncctCU Vit Y enterco|itica UCvcCp suscqucn!
CryIhCma uOUO8um. a!icn!s Vi! crCnic giarUiasis
arc at risR Cr aiurc !C !rivc rcsuting !rCn CngCing
naasCrp!iCn.
1n sCnc pa!cn!s par!icuary tCsc Vi! Yersinia,
scvCrC pain Ccai2cs !C !c rig! Cvcr quaUran!,
crca!ing a pscuUCappcnUici!s pic!urc.
1n cCcra !c sICC!s qucKy ccCnc cCCrCss
anU LCcRcU Vi! nucus !CrnCU ricc-VaIcr stCCs.
>CvCrC Uiarrca caUing !C ypCvCcnic sCcR nay
UcvCCp in Curs !C a cV Uays.
1C!avirus is !c naCr causc C nCnac!cria gas-
trCcntCri!s in inan!s anU !CUUCrs in !c Ycs!crn
VCrU. Incc!Cns pcaR ctccn January anU pr.
\Cnpaints incuUc prCsc U:arrCa vCni!ing anU
CV-graUc cvcr. >cvcrc Uiarrca nay caU tC scvcrc
UcyUra!Cn, aciUCsis anU ccc!rCytc U:s!uranccs.
\arUiasis s !C nCs! cCnnCny rCpCrtcU para-
si!c Uiscasc n !c \ntcU >!atCs. Crc va!cr-rca!cU
Cu!rCaKs C Uiarrca arc Uuc !C Ciardia |am||ia !an
any C!cr Crganisn. Jc i!ncss prcscn!s Vit rc-
qucnt !Cu-sncing va!cry s!CCs !a! rarcy cCn!ain
CCU Cr nucus, aUCnina pain nausca vCni!ng
anCrCxia anU a!ucncc C\cn accCnpany !C U:ar-
rca. >ynp!Cns gcncray rcsCvC Vi!in b !C Uays
a!Cug sCnc cascs ingcr Cr nCrC !an a nCn!.
hy5tcolLxomtnoItOn
JC nain gCas C !c pysica Cxanna!Cn arc cs!i-
na!ng !c Ucgrcc C UcyUra!iCn anU ruing Cu\ !c
nccU !Cr aUCnina surgcry.
LHOlOD!B LBQDO55
cu!C U:arrca in ciUCCU is usuay Uuc !C incc-
!Cn. L!cr cCnUitiCns assCciatCU Vi! Uiarrca
incuUC naasCrptiCn an!iC!ic usc cys!ic rCsis
anU tnanna!Cry Cwc UtsCasC.
LBQDO5!C LVBUB!OD
LcctrCytc anU rcna nc!iCn stuUics [a' ' \
\\ L\, crca!ninc| gHiUc rCpaccncn! !crapy
in signican!y UcyUra!cU ciUrCn. UCnina raUi-
Cgraps arc gcncray nCrna Cr nCnspccic. LCCU
nucus anU |cca cuRCcytcs suggCst a ac!cria Crigin
Cr !C ncss. LCCU cu!urc sCuU c pcrCrncU at
!c !inC C in!ia cvaua!Cn i ac!cria Uscasc s
suspcctCU Lac!cria s!CC cu!urC rCsuts taKc scvcra
Uays u! arc cpu H UC!Crntntng tc nccU Cr
antiC!ics. 1! !crc is a isICry C an!iC!c usc s!CC
sCuU C !cs!cU Cr C|ostridium dici|e !Cxins anU
. IaptU an!igcn !cs!ng is avaiaC Cr rCtavirus. 1
( |am||ia incctiCn is suspcc!CU nutipc stCC
sanpCs rCn Ui!crcn! !ncs sCuU c cxanincU Cr
cys!s. 1nnunCLuCrcsccnt an!iCUy UCtcc!iCn in s!CC
can asC c uscU !C UagnCsc C |am||ia ncctiCn.
|nUCscCpic iCpsy nay C inUicatCU i! tc Uarrca
ccCnCs crCnic anU nC c\CCgy as ccn |CunU.
lOB!DOD!
Jrca!ncnt incCrpCra!cs Cra rcyUraICn Vcncvcr
pCssiC, aggrcssivc parcntcra tCrapy nay c
rcquirCU n scvcrc cascs. n!iUiarrCa agcn!s sCuU
c avCiUCU.
\nCss !c pa!icn! is a cric inan! yCungcr !an
Z nCn!s an!iiC!ics sCuU gcnCray c Vi!cU
pcnUing cuturc rcsuts. ntiC!c tcrapy prCCngs
Sa|mone||a scUUtng anU sCu!U c rcscrvCU Cr
systCnic n!cc!iCns. 1n!iC!cs nay cnancc tc
iRciCCU C UcvcCpncnI C cnCy!ic urcnic syn-
UrCnC anCng pa!cn!s Vi! UiarrCa causcU y L. co|i
\b.. 1 synp!Cns pCrsist CncC cu!urc rcsu!s
arc RnCVn an!iiC!ic !crapy sCuU c cCnsiUcrcU.
JrinCtCprin-suanc!Cxa2Cc is usuay c!cc!vc
in trcatng sigcCsis. LrytrCnycin is !c trca!ncnt
C cCicc Cr | jejuni. a!icnts Vit | dici|e
cn!crCcCi!is usuay nprCvC Vit suspcnsCn C
an!iC!ic tcrapy uI u IrcatnCnt is varranICU
nCtrCniUa2Cc is tc trca!nCn! C cCcc. a!cn!s
Vi! giarU:asis nay asC c !rCa!cU Vi! Cra
nctrCniUa2Cc.
1s Cng as !c pa!cnt UCcs nC! UcvcCp ypC-
vCcnic sCcR prCgnCsis Cr rCcCvcry is cxcc-
cn!. vcn in ic-!rca!cning cascs apprCpra!c
nanagCncn! nay prcvcn! pcrnanCn! scqucac.

hLX LW
T. 111f11C1S 1B111B 1By 1 1Bf111B1, V11B!, C1
|B1BS111f.
Z. CB1111 l11 B1 !<11C1y1 1B1BQ11! 1S 11
1OS1 11|C11B11 11B1111 11 111f11O1S 1B111B.
d. C11111 v111 S11Q11CS1S 1By |1611 v111 111B1
S1B11S f1B1QS.
4. b.dyventer/0eB1 ccCl/ LT*?:H? 1BV 11 BSSC
c1B1 v111 11C1y11f 1111f Sy11C1.
* CPm M OBBkPOHBOQCMR* WYLP G9 OH9=
|Y ~
MLVP11b
VB!DOQODO55
cu!C cpa!c inamma!Cn in ciUcn can c Uuc
!C a argc numcr C incc!Cus anU nCnincc!iCus
causcs. `iruscs !a! arc primariy Cpa!Cpic
ncuUc cpa!i!s virus [` , cpa!!s L virUs
[L`, cpa!!is \ virus [\`, cpa!i!s vrUs
[1`, |CrmCry Uc!a cpa!!is| anU Cpa!!is
virUs [\. Ca!urcs C \, L\, anU \` arc
cCmparcU in Jac Z-.
LQOODOOQg
` anU ` arc acquirCU via cca-Cra !ansmis-
siCn. Ly \\, anU 1` arc !ansmi!!CU y pcr-
cu!ancCus Cr mucCsa cxpCsurC !C incc!Cus CUy
uiUs anU y vcr!ica !ransmissiCn Cm an incc!cU
mC!cr !C cr inan!. 1\, Cr Uc!a an!igCn, cCnsisIs
C singc-s!ranUcU 1. 1! is a UcCc!vC virus in
!a! t! rcquirCs !c prcscncC C an ac!vc L` incc-
IiCn !C rcpica!c. L` anU \` can pcrsis! Cr many
ycars CCVng acu!c ncc!iCn. Jis carricr s!a!c
is assCcia!cU V! UcvcCpmcn\ C cpa!Cccuar
carcinCma.
H5K BC!Ol5
1n!ravcnCus Uug uscrs, !CsC VC avc unprC!cc!cU
scx ViI muIpc par!ncrs, anU !Csc VC rcccivc
CCU !rans!siCns arc a! ncrcascU risR C cCn!rac!-
1bLL 12-7
ing L\ \`, anU 1\. 1isR |ac!Crs Cr ` anU
` incuUC Crcign !avc, pCCr sani!a\Cn, anU
cCn!ac! V! C!cr cUrcn n Uay carc.
LOCB NBDO5!B!OD5
H5IOry
crina!ay inCc!cU inan!s arc usuay asymp!Cma!c.
\inica sig;s C acu!c cpa!i!is incuUc anCrcxia,
nausca, maaisC, vCmi!ng, aunUicc, UarR urinc,
aUCmina pain, anU CV-graUC cvcr. \iUrcn Vi!
` anU ` may avc U:arrca. CVcvCr, a ViUc
rangc C scvcr!y cxis!s, anU as many as JV !C bJV
C! incc!cU ciUrcn arc asymp!Cma!c. L` anU
\` ncc!iCn arC usuay sCn!, in !a! !C pa!icn!
cCmpains C nC symp!Cms uncss crCnic incc!Cn
as causcU signican! cpa!c Uamagc.
hy5colLomnoIOn
>ccra c!crus anU aunUicc arc nC!cU in sCmc ci-
Urcn Vi! \, bJV C ciUrcn Vi! L\ anU ZJV
tC JV C ciUrcn Vi! \\. cpa!CmCgay anU
rig! uppcr quaUran! !cnUcrncss may c prcscn!.
cnign-appcaring ras may c prcscn! cary in !c
cCursc.
LOlOD!B LBQDO55
L`, \`, cn!CrCvirUs, anU C!cr vira incc!Cns can
asC causc cpa!!s, u! C!cr Crgan sysIcms arc
usuay invCvCU.
VlUS0S h0SQODSDl0 Ol M0QttS. LOHQlSOD OO bUDHl
lPlUfP
VIuS lyC
|DCuDlOD (UyS)
CIOU O DClVly
|uuDDl PllS
LlIODC lCllS
LgDOSlC VuIOD
HEQllS P
|PF
1b4b
LlC DCUDlOD lO CIy
SyulOulC SllC
<1V
PO
FDlMFV |gN
HPQllS
LPF
4b-1bU
WlCD MbSFg SPIOOSllVC
1V-5V
bV-1UVO Uul5, 2bV-bUV
O DDlS, 9UV O DODlCS
wOSC uOllPIS IC
MbCFg+
MbSFg, MbCFg, DllMbS,
DlMbC, Dll-MbC
HPQllS L
|PF
1-1bU
ODkDOwD
1V
bUV
FDlMLV DllDOUy, MLV
LH
lMC, lOl| HlDOUg lO HCllS COfC HlgCH, HlMC, lOl lDOUy lO CllS C HlgCH, HllS, 1Ol HlDOUy lO CllS SUrCC
HlgCH, MPV, HCllS P VtUS, MCPg, CllS C HlgCH, lSPg, HCllS 5UfCC OlgCO, MLV, CllIS CVIfUS.
< IOI TOI SB1O1 ~ * HO IR IOB 1 ~
LBQDO5!C LVBUB!OD
vcr CnZymcs arc uniCrmy ccva!CU in cpa!i!is.
LccausC !c cinica manics!a!Cns arC sC smar spc-
cic scCCgc !cs!s arc inUspcnsaC Cr sccurng an
accura!C UiagnCsis [acs Z- anU Z-c|. Jc prcs-
cncc C an!-V 1g an!CUy cCnhrms \ incC-
!Cn [igurc Z-+| . Jcs!s arc asC avaiac !C Uc!cc!
an!iCUtCs !C !c Uc!a an!igcn.
JrCC UiUcrcn! parIccs may C |CunU in !c
scrum C pa!cn!s incc!cU Vi! mL` Jc anc
par!icc is !c argcs! maUc up C a cCrc an!igcn
[Lcg| anU cnvcCpc an!gcn [)LCg| surrOunUcU
y a spCica sc C Lsg [sur!acc| parIccs.
bLL 12-
LOHHQlSOD O S0S0 btt0S D
M0QttS VlUS
PSl PCUlE HV HOSOlVO0 HV LrODC HV
MbSFg + +
FDlI-MbS
FDlMbL + + +
MbCFg 1 I
FDlMbC + I
DlMC, lOl HlDOUy lO CllS COrC HlgCH, Dl-MC, lOl
HlDOUy lO CllS C HlgCD, DlMS, lOl HlDOUy lO CllS
SUfCC HlgCH, lCPg, CllS C HlgCH, lSPg, CllS
SUICC DlgCH, lV CllS VfUS.
|QL-B|!IlV
~ /
|GCB
GXC|G!IOl
`
Z
^ #
U
|lCUOB!IO| QG|IOO

4 b U b U U U
VGGKS NOl!S
J|RG B!G! GXQOSU|G
Igure 124 1l6 COu|S6 O| Cul6 HClIS F.


!Z Z U
`GB|S
lOOEO fOH bHUHH b1, |Of J[bOHHCf5 |l. 1OE DOO_C DU LO
Cl D5S O |OEClOUS L5ESC5, H`` LU. |H!OClO V.D. bUOOCfS, 1992;
315,319.
* CPB M OBEKYOHBOQR* VLP G9 OH9= y ~
LllC| 12 / |D|CClOuS LSCSC 1d
igrc Z-b anU Jac lZ-c prcscn! !c cnica
cCursc anU scrCOgtc marRcrs tmpCr!an! tn UtagnOs-
ing mL\ UiscasC s!agc. n!-Ls craUs rcsCu!iCn
C !c inCss anU cCncrs icCng mmuni!y.
!LV an!CUy is prcscn! in C! acu!c anU
crCnic incc!Cn. LY 1 can C Uc!cc!cU y
FLI V!in 1 VccR C incc!Cn Vcrcas !c
VinUCV pCriCU rCm ncc!iCn !C an!tCUy rcspCnsc
Cr L\ may c as Cng as Z VccRs. JcrcCc !c
prcscncc C ILY ! in !c ascncc C an!iCUy
rcspCnsC inUica!cs acu!c incc!Cn. ICcCvcry is car-
ac!criZcU y Uisappcarancc C L\ II1 rCm !c
CCU.
lOB!DOD!
LC! ac!ivC anU passivc Crms C! mmuniZa!iCn arc
avaiaC UCpcnUing Cn !c sCurcc C incc!iCn. \
immuniZa!Cn is rccCmmcnUcU Cr a ciUrcn in
sCmc par!s C !c \ni!cU >!a!cs VCrC incc!iCn is
mCrc iRCy. \ immunCgCuin V prcvcn! cin-
ica UiscasC Vcn aUminis!crcU Vi!in + Uays C
cxpCsurC. Jc L\ vaccnc scrics is rCcCmmcnUcU
Cr a inan!s in !c \ni!cU >!a!cs. 1nan!s C incc!cU
mC!crs sCuU rcccivc C! !c vaccinc anU \c
L\ immunCgCuin a! Ucivcry !C prcvcn!
!c UscasC anU mCs! impCr!an! UcvCCpmcnt C !c
carricr s!a!C. 1pa-in!crcrCn as sCVn prCmisc n
|LSlQ
blQ
|LV1`F
Z U
1

PD-|S

;` CG!UR
!|B|SBRI|BSGS

.

\
/

l
/

4 b
/
/
_ l|!I-C
/
lDI-G

s
U !Z Z4
NOl!S BIG| GXQOSU|G
IgJte 12 1H6 COutS6 O| CuI6 H6IIS b.
lOOCO fOH bOUHH b1, |Of J[bOHHCf5 ml. 1OE DOlOgC OU LlH
C DSS O OEClOUS LSCSC5, H`` LO. |lOE[H! V.D. bUHOEfS, 1992:
315.319.
* tor SB1O1 ~ * HO /IR npoca l ~
1b4 COl:CS
* CPo M OBBkPOHBOQ* VLP G9 [OH9. Qg ~
!rca!ing pa!cn!s Vi! crCnc L` cpa!!s, s!uUics
n ciUrcn arc css cncCuraging. Jcrc is nC spccic
!rca!mcn! Cr 1. pa-in!crcrCn as ccn c!cC-
!ivc in prcvcn!ng cCnvcrsiCn rCm acu!c !C crCnc
\` cpa!!s. Lny suppCr!vc carc is avaiac !C
`-incc!cU inUiviUuas.
Jc prCgnCsis Cr pa!icn!s V! cpa!!s UcpcnUs
Cn !c virs rcspCnsic.
HAV `cry cV pa!icn!s UcvcCp minan! cpa!-
!is, u! !c mCr!ai!y ra!c amCng !Csc VC UC is
amCs! DJV.
H8V L` may pcrsisI as crCnic cpa!i!is, anU
!c cCursc nay c rca!ivcy cnign Cr mCrc
scvcrc. \rCnic pcrsis!cn! cpa!i!is L is carac!cr-
iZcU y i!!c ccuar inLamma!Cn anU usuay
rcsCvcs Vi!in a ycar. \rCnic ac!vc cpa!!is is
mCrc aggrcssivc prCgrcssing !C cirrCsis anU
incrcasing !c risR C cpa!Cccuar carcnCma.
\rCnic ncc!Cn s mCrc liRcy amCng ncc!cU
ciUrcn !an aUu!s.
HDV Ycn 1` anU L` arc acquircU simu-
!ancCusy !c rccipicn! s a! grca!cr risR Cr mCrc
scvcrc crCnic cpa!i!s L anU uminan! cpa!!s
assCcia!cU Vi! a igcr mCr!ai!y ra!c. Ycn an
inU:viUua is incc!cU Vi! 1` Cn !Cp C prccx-
s!ng L` acu!c cxaccra!Cn, anU an accccra!cU
cCursc rcsu!. Jc risR C! prCgrcssing IC cirrC!c
ivcr Uiscasc is incrcascU Vcn 1` s prcscn!.
HCV a C ICsc incc!cU Vi! \` UcvcCp
crCnc cpa!!s Vi! an incrcascU risR Cr
cirrCss.
HLV L` UCcs nC! appcar !C rcsu! in crCnic
cpa!!s.
hLX LW
1. HTV B1 H1V B1 S1B V1B 1fB1-O1B! 11B1S11S-
S1O1. HbV, HCV, B1 H1V B1 11B1S1111 111OUQ1
111f1 1O\1j 11U1S.
Z. C!111fB! S1Q1S O1 Bft1 1|B1111S 11f!t B1O1X1B,
1BUSB, 1B!B1S, VO11111Q, BU11f, B1 t111,
B1O111B! |B11, B1 1Ov-Q1B V1. HOv1, B
v1 1B1Q O1 SV111j X1S1S, B1 BS 1B1y BS d%
1O b%O1 111f1 f11111 B1 BSj11O1B11f.
d. L1V1 1Ij1S B1 U111O111y 1VB1 11 1|B1111S.
BfBUS 11 f1111fB1 1B111S1B11O1S B1 SO S1111B1,
S|f11f S1O1OQ1f 1S1S B1 111S|1SB11 1O1
SfU111Q B1 BffU1B1 1BQ1OS1S.
bXVM11b
VB!DOQODO55
>ypiis is prmary a scxuay !ransmi!!cU U:scasc
rcsu!ng rCm tncc!Cn Vi! !c spirCcc!c
7reponema pa||idum.
LQOODOOQg
>ypiis in !c pcUia!ric pCpua!Cn may c acquircU
!ranspaccn!ay [cCngcn!a sypiis| Cr !rCug
scxua cCn!ac!. Jc inciUcncc C sypiis as
incrcascU sarpy Cvcr !c as! scvcra ycars. \Cin-
cc!Cn Vi! C!cr scxuay !ransmi!!cU U:scascs is
cCmmOn.
H5K BC!Ol5
cCna!cs Crn !C a mC!cr V! un!rca!cU incc!iCn
arc a! risR Cr cCngcni!a sypiis. UCcsccn!s anU
aUu!s VC avc unprC!cc!cU scx Vi! an incc!cU
par!ncr arc a! risK Cr prmary sypiis.
LDCB NBDO5!B!OD5
Ht5IOryondhy5tcolLxomtnoItOn
pprCxima!cy +JV C inan!s Vi! cCngcni!a
sypiis UiC. JCsc V!C survvc arc Ccn asymp!C-
ma!c a! ir! u! UcvcCp symp!Cms V!n mCn!
i un!rca!cU. 1nan!s Vi! cCngcni!a sypiis may
aVc cpa!Cmcgay spcnCmcgay, mucCcuIancCus
csiCns aunUicc ympaUcnCpa!y anU !c car-
ac!cris!ic 8uuC8 a CCU, mucCpurucn! nasa
Uiscargc. L!cr nUings incuUc Ucancss anU
rc!arUa!Cn.
>ypiis acquircU !rCug scxua cCn!ac! prC-
grcsscs !rCug !rcc s!agcs. !cr a Z !C +-Vcc!
ncua!iCn pcriCU, incc!cU inU:viUuas cn!cr !c
Qrmary s!agc C sypiis carac!criZcU y !c cassic
cancrc a! !c nCcua!Cn si!c: a Vc-Ucmarca!cU
\rm sIrangcy aincss gcni!a uccr Vi! an
nUura!cU asc. Lccausc !c csiCn cas spCn!a-
ncCusy Vi!in !C 6 VccRs pa!cn!s Vi! primar
sypiis Ccn UC nC! sccR mcU:ca a!!cn!Cn.
becOnUary sypis s rcqucn!y manics!cU y
VUcsprcaU Ucrma!CCgic invCvcmcn! cCinciUing
Vi! Uisscmina!iCn C !c sprCcc!c !rCugCu!
!c CUy. Lnsc! CCVs Ic primary s!agc Uircc!y
Ccn Vc !c cancrc is s! prcscn!. c !ypica
ras cCnss!s C gcncraiZcU [incuU:ng !c sCcs anU
pams|, cry!cma!Cus macucs [-11mm| !a!
< not tor SB1O1 ~ * HO IR IOB 1 ~
prCyrcss !C papucs. >Cmc pa!icn!s asC UcvcCp
sys!cmtc symp!Cms tncluUtng cvct maaisc, paryn-
gi!is mucCsa uccra!Cns, anU gCncra2cU ym-
paUcnCpa!y pa!cy aCpccia anU !inning C !c
a!cra !irU C !c cycrCV avc asC ccn assCcia!cU
Vi! sccOnUary sypiis. >ymp!Cms C sccCnUary
sypiis rCsCvc n !C J mCn!s.
Craj sypiis UcvcCps ycars a\cr prinary
cxpCsurc anU is rarc in !c pcU:a!ric pCpua!iCn.
\ranuCma!Cus csiCns cacU guDa8 Ucs!rCy
surrCunUng !issucs cspcciay in !c sRn Cnc
car!, anU \>. nCr!una!cy !cr!iary syptis may
Cccur Vi!Cu! any prcviCus primary Cr sccCnUary
manics!a!Cns.
LOIOD!B LBQDO55
>ypis is Cnc C !c grca! masqucraUcrs a Uiscasc
Vi! a ViUc spcc!rum C prcscn!a!Cn. Jc prcscncc
C !c ras i carac!cris!ic grca!y aiUs n UiagnCss.
LBQDO5!C LVBlUB!OD
\ancrC scrapngs [anU mucCsa sccrc!Cns in
incc!cU ncCna!cs UcmCns!ra!c rapiUy mCic
Crgansms mCving in a cCrRscrcV-iRC mC!Cn unUcr
UarR-CU mcrCscCpy. spira!Cn C CnargcU ymp
nCUcs may asC yicU !c Crgansm. LC! !c `11
[UcvcCpCU y !c `cncrca iscasC 1cscarc aC-
ra!Ory C !c \>. uic ca! >CrVicc| anU !c
rapiU pasma rcagin [11 arc Cxcccn! OOU
scrccning !cs!s Cr ig-risR pCpua!iCns prCviUing
rapiU, inCxpcnsivc quan!i!a!ivc rcsu!s. LC! arc nCn-
!rcpCncma !cs!s Cr an!iCUics !C a ipCiUa mCccuc
ra!cr !an !c Crganism i!sc! LC! arc cCnsiUcrcU
igy sCnsi!ivc Vcn !!crs arc ig Cr vcn !c !cs!
is cCmpCmcn!cU y is!Crica Cr pysica cviUcncc
C !c Uiscasc. CVcvcr incc!Cus nCnCnuccCsis
cCnncc!ivC !issuc Uiscasc cnUCcarUi!is anU !ucrcu-
Csis may a rcsu! in asc-pCsi!vc `11 anU II
rcsu!s. Ly cCn!ras! !rcpCncma !cs!s suc as !c Lu-
Crcsccn! !rcpCncma an!CUy asCrp!iCn [-L>
anU micrCcmaggu!ina!iCn !cs! [-J] arc
muc Css Rcy !C prCUucc asc pCs!ivcs uncss
ymc UisCasc is prcscn!. pCsi!vc scrccnng `1I
Cr 11 cCupcU Vi! a Cs!ivC l-L> n a
ncVCrn Cr scxuay ac!ivc aUCcscCn! is vir!uay
UiagnCs!tc C un!rca!cU sypiis. Cn!rcpCncma !cs!s
may ccCmc ncga!ivc a!cr !rca!mCn!, Vcrcas !rc-
pCncma s!uUcs rcman pCsi!ivc Cr c.
cCna!cs vi! suspcc!cU cCngCn!a sypiis
rcquirC umar punc!urc. \> pcCcy!Csis anU cc-
* CPB M OBBYOHBOQR2 VLP G9 OH9= @
C11: 1 / l1<11O1S L1SS 1
va!cU prC!cin suggcs! ncurCsypis u! Cst!ivc
\> `11 ts UtagnCs!tc. 1nan!s may UcvcCp raUt-
Cgrapc anCrmai!ics C !c Cng Cncs. ncmia
anU !rCmCcy!Cpcnia may asC UcvcCp in
un!rca!cU inan!s.
IOB!DOD!
arcn!cra pcnicin \ rcmains !c !rca!mcn! C
cCicc Cr any s!agc C incc!Cn anU |uy craUca!cs
!c Crgansm rCm !c CUy. LCxycycnc may c
uscU Cr !Csc VC arC acrgic !C pCntcitn.

hLX LW

T. Sj|1111S 1Bj 1 11B1S1111 111S|!Bf11B11j O1


SXUB!!j.
Z. NO1B1S v111 fO1Q111B1 Sj|11!1S |1S11 v111
"S1111!S," 1|B1OS|!1O1QB!j, 11fOf11B1OUS
!S1O1S, B111f, B1 1j1|1B1O|B11j.
d. NOS1 |B1111S B1 1BQ1OS 11 11 SfO1B1j
S1BQ O1 Sj|1111S, v11 v1S|1B 11B1O1O1
1B111S1B11O1S B1 |1S11.
4. 11 VLHL B1 HH B1 Xf1111 Sf1111Q 1S1S
1t1 mBj |1O1f 1B1S |OS111VS.
b. |B1111B! 11f11!11 G 1S 11 11B1111 O1 f1O1f.
LLW1P MLHVLb b1NVL7
M1 HLb 1WLL1LW
\cni!a Crpcs usuay rcsu!s rCm tncc!iCn vi!
crpcs smpcx virus !ypc Z. >ma mucCsa !cars Cr
sRn cracRs arc nCcua!cU Vi! !c vrus usuay
Uurng sCxua ac!vi!y. \cn!a crpcs is Cnc C !c
mOs! cCmmCn scxuay acquircU UisCascs JV !C
ZJV C aUu!s avc a is!Cry suggCs!ivC C priCr
gcn!a Crpcs incc!iCn. JransmissCn C >` rCm
mC!cr !C tnan! a! !c !tmc C! t! may rcsu! n
Ucvas!a!in incc!Cn n !c ncVCrn.
LlDCB NBDO5!B!OD5
Ht5IOryondhy5tcolLxomtnoItOn
!cr a variac incua!Cn pcriCU [b-+ Uays|
gcn!a urning anU i!cing prCjrCss !C vcsicuar
C!cn pus!uar csiCns. Jcsc urs! !C Crm painu
saCV uccrs !a! ca Vi!Cu! scarring. cvcr
* Co M OBBPOHBOQR* VLP G9 OH9=
|Y
~
paryngi!s caUacc anU maaisc may accCmpany
!c primary cpisCUc. Icr acquisi!Cn !c virus
asccnUs pcripcra ncrvcs !C UCrsa rCCI gangia,
Vcrc iI may ic a!cn! Cr rccur pcriCUicay 1ccur-
rcnccs avc cVcr symp!Cms !an !c prmary
cpsCUc, anU asymp!Cma!tc scUUng may Cccur.
LBQDO5!C LVBUB!OD
\ian! mu!nucca!cU ccs Vi! inIanuccar incu-
siCns arc CunU in scrapings Cm tc uccrCus asc.
>` may c cu!urcU Cm !c ac!ivc csiCns Cr
asymp!Cma!c incc!cU inU:viUuas in !C Uays
rapiU an!igcn !cs!ng s asC avaiac.
lOB!DOD!
cycCvir [!Cpica Cr parcn!cra| Uiminscs !c
cngt C C! symp!Cms anU scUUng u! UCcs nC!
craUica!c !c Crganism. 1! as imi!cU ccacy in
rccurrcn! cpisCUcs. \Cn!nucU prCpyac!c usc C
Cra acycCvir as ccn sCVn !C prcvcn! Cr rcUucc
!c rcqucncy C rccurrcnccs.
VLV1L 1WPNNPLHX L1bLPbL
VB!DOQODO55
cviC inamma!Cry Uiscasc [1| s a cCnsIcaIiCn
C signs anU symp!Cms rca!cU !C !c asccnUing
sprcaU C pa!Cgcnc Crganisms Cm !c CVcr
cmac gcniIa! !rac! !C !c ccrVtx cnUCmc!ium anU
aCpian !ucs.
LQOODOOQg
Lvcr I miCn cascs C 1 Cccur annuay in !c
\n

cU >!a!cs. Jc c!iCCgy is gcncray pCymicrC-


ia Vi! Ch|apdia trachomatis anU ^eisseria
onorhoeae cing y ar !c mCs! cCmmCn isCa!cs.
Larricr cCn!raccp!vc mc!CUs arc prC!cc!vc. l on
orhoeae Cr gcni!a C trachomatis incc!iCn ID a prc-
pucr!a ciU s!rCngy suggcs!s scxua ausc.
H5K BC!Ol5
IisK ac!Crs incuUc agc [aUCcsccncc| scxua in!cr-
cCursc Vi! mu!pc par!ncrs unprC!cc!cU in!cr-
cCursc anU prccxis!ng mucCsa scxuay !ransmi!!cU
Uiscasc.
LDCB NBDO5!B!OD5, M5!Olg, VDg5CB
LXBDDB!OD, BDO LBQDO5!C LVBUB!OD
Jc cinca UiagnCsis C lI is ascU Cn !c prcs-
cncc C !rcc rCqurCU anU Cnc C scvcra sujjOrIug
symp!Cms.
lequired. UCmina pain anU !cnUcrncss, ccrvica
mC!Cn !cnUcrncss, aUncxa !cnUcrncss
Supportin. Jcmpcra!urc grca!cr !an c`\ cuKC-
cy!Csis ccva!cU |>1 grca!cr !an ZJ V!C CCU
ccs pcr ig-pCVcr cU Cr in!accuar gram-
ncga!ivc UtpCcCcct Cn cnUCccrvtca smcar Vt!c
CCU cc cCun! anU/Cr ac!cria Cn cuUCccnIcsis,
inLamma!Cry mass y u!rasCunU
LOlOD!B LBQDO55
L!cr gynccCCgic cCnU:!Cns anU inIra-aUCmina
pa!CCg arc incuUcU in !c UiUcrcn!ia UagnCsis.
Cneco|oic. c!CpiC prcgnancy rup!urcU Cvaran
cys! scp!c aCr!tCn
longneco|oic. ppcnUic!is, pycCncpri!s in-
amma!Cry CVc Uiscasc
lOB!DOD!
a!cn!s Vi! 1 sCuU c !rca!cU Cr C! /
onorrhoeae anU \. trachomatis. sngc UCsc C a
Cng-ac!ng !irU-gcncra!iCn ccpaCspCrin suc as
cc\raxCnc Cr ccximc is sucicnI IC craU:ca!c l
onorrhoeae, ciprCCxacin, ClCxactn, Cr spcc!inC-
mycin may c uscU as Vc. Zi!rCmycin craUica!cs
| trachomatis in Cnc argc UCsc, a!crna!vcy a `-Uay
cCursc C UCxycycinc is apprCpria!c. >ignican!
incc!Cns rcquirc mCrc cx!cnsivc cCurscs C !crapy.
a!cn!s VC arc aUmi!!cU !C !c Cspi!a Cr scvcrc
vCmi!ng Cr CCU prcssurc ins!aii!y sCuU rcccivc
!crapy Vt! | an!tC!tcs ncuUtng C! a !trU-
gcncra!iCn ccpaCspCrn anU UCxycycinc anU prc-
vcn!ivc cUuca!iCn.
JVcn!y pcrccn! C incc!cU VCmcn Vi ccCmc
incr!c a\cr a singc cpisCUc C 1. L!cr gnccC-
Cgic cCmpica!Cns incuUc incrcascU risKs Cr
cc!CpiC prcgnancy Uysparcunia, crCnic pcvic pain
anU aUcsiCns.
l onorhoeae is capac C nvaUing !c CCU-
s!cam anU !us any Crgan sys!cm. JCin! invCvcmcn\
is mCs! cCmmCn. Jc arIhrl8 may acc! Cny Cnc
Cin! Cr may c pCyar!icuar anU migra!Cry Vi!
assCcia!cU !cnCsynCvi!s anU sKn |csiCns. !Cug |
* IOI TOI SB1O1 ~ * HO IR IQOB 1
* CPB M OBBKYHBOQR2 VLP G9 QOH9= Qg ~
trochoma tis scUCm causcs sys!cmic ncss un!rca!cU
inUiviUuas may gC Cn !C UcvCCp VCICrs syuUrOmC
[a cCns!caIiCn C! urc!r!is, cCnunc!ivi!s anU
ar!r!is. lz-ugh-LurIs syuUrOmC, a Crm C pcr-
iCpa!i!s is a RnCVn cCmpca!Cn C ncc!Cn V!
Ci!cr Organism.

hLX LW

1. L.trochomot/vB1 N.gonorrhoeoeB1 11 1OS1


fO11O1 1SO1B1S 11 |1V1f 1111B11B1O1j 1SBS.
2. 11 1BQ1OS1S O1 L 1S f1111fB1, 1BS O1 11S1O1j,
|1jS1fB1 B111B11O1, B1 SU||O1111Q 1B1O1B1O1j
1SU11S.
d. F 1O1Q-B111Q 11\1-Q11B11O1 f|1B1OS|O111 |1O1
N.gonorrhoeoe)B1 BI1111O1jf11 |1O1 L.tro
chomot/v)fO1S111t1 B||1O|11B1 OU1|B1111
111B|j D 111 111f11O1S.
MLMLMPL1WP lWLL1LWb
JrcCnCnasis ac!cria vagnOsis anU Candida
vagini!is arc a C!crsCmC u! rca!ivcy cngn
vagina ncc!iCns cCcc!vCy mancs!cU y cangcs
in !c amCun! anU carac!cr C vagina sccrc!Cns.
LDCB NBDO5!B!OD5 BDO lOB!DOD!
rtchOmOnto5t5
JrcCmCniasis rcsu!s Cm scxuay Iransmi!!cU
7richomonas iaina|is, a mCic Lagca!cU prC!C2Can.
Cs! ncc!cU inUivUuas rcman asymp!Cma!ic
a!Cug urc!r!is is nC! uncCmmCn in mCn. Jypica
symp!Cms in VCmcn incuUC a maCUCrCus rC!y
gray Uiscargc anU vagna UiscCmCr!. >CmC pa!cn!s
asC UcvcCp Uysuria anU vaguc CVcr aUCmna pan.
Jc ccrvix anU vagina mucCsa may c ci!Cr nCrma
Cr visy rriIa!CU anU inLamCU. rcs VCI prcp C
!c vagina uiU rcvcas pCymCrpCnucCar cuRC-
cy!cs anU !c carac!cris!ic mC!ic !ricCmCnaUs.
c!rCnUaZCc in a singc Z-gram Cra UCsc is !c
!rca!mcn! C cCcc Cr pa!icn!s anU !cir par!ncrs.
bocICrtolVogtnO5t5
Lac!cria vaginCsis Cng !Cug! !C C armcss s
nCV RnCVn tC incrcasc Ic risRs C 1 cCrCam-
niCn!is anU prcma!urC ir!. Lac!cria vagnCsis s
causcU y Cardnere||a spp. Aycop|asma hominis, anU
variCus anacrCc Crganisms. Jc cpUcmiCCg C
!c Uiscasc suggcs!s scxua !ransmissCn a!Cug !c
LllC| 12 / |H| CLlOUS LSCSC 1
Ua!a rcmain unccar. 1ncc!iCn is usuay asymp!C-
ma!c cxccp! Cr a tin Vi!c Cu-smcing Uis-
cargc !a! cmi!s a sy CUCr Vcn mixCU V!
pC!assium yUrCxUc. Jc inca UiagnCsis is ascU
Cn pa!cn! is!Cry [muc mCrC cCmmCn n sCxuay
ac!vc cmacs !c appcarancC anU CUCr C Uis-
cargc a vagna pl grca!cr !an +. b anU carac!cr-
is!iC cuc ccs Cn !c VcI prcp [squamCus cpi!cta
ccs V! smuUgcU CrUCrs causcU y aUcrCn! ac-
!Cria). Lncc again a singc UCsc C mc!rCniUazCc
c!Cc!vcy curcs !c ncc!iCn. \Cncurrcn! an!iC!ic
!rCa!mcn! C mac par!ncrs scCms !C avc nC ccc!
Cn rccurrcncc ra!cs.
VogtnolLondtdto5t5
\uvCvagina canUUiasis s nC! a scxuay !ransm!!cU
Uiscasc. VCmcn arc cCCniZCU Vi! Candida,
CVcvcr ac!Crs suc as an!iC!c usc prCgnancy
Uiac!cs immunCsupprcssiCn anU Cra cCn!raccp!ivc
usC prcUispCsc VCmcn !C canUiUa CvCrgrCV\
[mCniiasis| . >gns anU sympICms incuUc a ticR
Vi!C vagina U:scargc Vi! vagina i!cng anU
urnng. Xcas! anU pscuUCypac arc apparcn! Cn
VC! prcp Irca!cU V! pC!assium yUrCxiUC. Lvcr-
!C-cCun!cr Cca an!iunga crCams arc sac anU gcn-
cray c!cc!ivc. LccasCnay Cra an!Unga agcn!s
arC rcuircU.

hLX LW
1. 111f1O1O11BS1S 1S 1BQ1OS 1j 1O1S11B111Q
1O111 111f1O1O1BS O1 11S1 v1 |1| B1 1S
11B1 v111 111O11BIO1.
2. BBf111B1 VBQ11OS1S, O111 fBUS 1j N.hom/n/v,
S1OU1 1 SUS|f1 v11 11 VBQ11B1 | H 1S
Q1B11 11B1 4.bB1 f1U f1!S B1 S1 O1 v1
|1|. N11O11BIO1 1S 1f11V 11B1111.
M1M PWL PL|L1HLL
1WNLWLLL1L1LWLX bXWLHLNL
VB!DOQODO55
1\ is a rCIrCvirus !at nCc!s anU Rs \+ ym-
pCcy!cs rcsu!ng in prCgrcssivc immunCUCCicncy.
CUia!rc cascs accCun! Cr V !C ZV C !c !C!a in
!c \ni!cU >!a!Cs. Cs! ncc!iCns in ciUrcn arc
acqurcU in u!CrC Cr pcrina!ay [cJV| smacr
numcrs rcsuI rCm CCU prCUuc! !ransusiOns anU
sCxua !ransmissiCn. l1\ prCUuccs a ViUc rangc C
IO1 TOI SB1O1 ~ * HO IR IOB 1 ~
1 |lucr|rL P11111S
cinica mani!cstatCns in ciUrcn, tc mCst scvcrc
C! Vtc ts acqutrcU tmmunCUchctcnc snUrOmc
[11>|.
LQOODOOQg
Jc risK C! 1` transmssiCn !Cm a scrCpCsitvc
mCtcr tC cr !ctus is apprCximatc ZJV tC JV.
Jrcatmcnt C! in!cctcU prcgnant VCmcn vit ant-
rctCviras Uuring tc tirU trimcstcr, !CCVcU
trcatmcnt C! tc in!ant !Cr tc hrst 6 VccKs C! i!c,
as ccn sCvn IC rcUucc Ic vcrttca transmtssCn
ratc tC aCut cV. 1smptCmatc 1`-pCsitvc
vCmcn ma nCt rcaiZc taI tc arc n!cctcU anU,
tcrc!Crc, CIcn UC nCt rcccivc tcrap. 1` Urg ausc
is rcpCrtcU n apprCximatc JV C! in!cctcU
VCmcn. Jc Uiscasc is mCrc cCmmCn in uran pCp-
uatCns, CVcr sCcCccCnCmic casscs, anU raca
minCritcs.
H5K BC!Ol5
1isK actCrs ncuUc irt tC an 1`-pCstivc mCtcr,
irt tC a vCman VC uscs 1` Uugs anU sarcs
nccUcs, anU irt tC a VCman vit mutpc scxua
partncrs VC UCcs nCI practcc sa!c scx. Ltcr
grCups at rIsK ncuUc paticnts VC rcccivcU muti-
pc units C! CCU prCUucts [c.g., cmCpiiacs|
c!Crc arc cD victms C! scxua ausc, anU aUC-
csccnts vC cngagc in ig-risK caviCr.
LDCB NBDO5!B!OD5
Ht5IOryondhy5tcolLomtnoItOn
1` ma prcscnt in in!anIs anU ciUrcn Vit an
Cnc Cr scvcra C! tc !CCving sgns anU smp-
tCms. gcncraiZcU mpaUcnCpat, cpatCmcga,
spcnCmcga, !aiurc tC trivc, rccurrcnt Cr crCnic
Uiarrca, Cra canUiUiasis, parCttis, anU UcvcCpmcn-
ta Uca. 1cspiratCr mani!cstatiCns incuUc m-
pCiU intcrstita pncumCna anU lneumostis carnii
pncunCnta [\|. 1cgrcsstCn tn UcvcCpmcnIa
micstCncs, prCgrcssivc cnccpaCpat, anU \I>
mpCma arc un!Crtunatc ncurCCgic cCmpica-
ICns. \arUiCmCpat anU ncprCpat asC Cccur.
Iccurrcnt, CIcn scvcrc, actcria anU CppCrtunistc
[!nga, UisscminatcU >` Cr \`, anU Ayco|ac-
terium aiium] in!cctCns arc tc amarK C! tc
acquircU cpcr Jcc mmunCUccicnc.
\, \I> mpCma, anU mpCctc intcrstta
pncumCnits [I| arc cCnsiUcrcU 11>-Uchning i-
ncsscs n tc pcUaInc pCpuatiCn. Ycn an C! tcsc
ctnU:tiCns Cccurs, tc cU is cCnsiUcrcU tC avc
11> rcgarUcss C! Ic asCutc \ cCunt.
LOlOD!B LBQDO55
1` as ccCmc a grcat masqucraUcr ccausc C! its
variac prcscntatCn; tc vrus can a!!cct an Crgan
sstcm, anU smptCms arc CIcn nCnspccihc.
LBQDO5!C LVBUB!OD
1n!ants Crn tC 1`-pCsitvc mCtcrs arc avas
scrCpCsitivc !Cr matcrna UcrvcU 1g\ antiCUics tC
tc virs, tus, tc cnZmc immunCassas uscU !Cr
scrccning CUcr pCpuatCns arc nCt cp! in ciUrcn
c!Crc c mCnts C! agc. |! tc mCtcr is 1` pCsi-
tvc, 1\ I1 \1 C! tc in!ants CCU sCuU c
pcr!CrmcU at irt, I mCnt, anU + mCnts C! agc, anU
CCU Cr 1` cuturc sCuU c scnt at I mCnt C!
agc. Jc cCminatCn C! 1` I1 \1 anU 1`
cuturc Vi Uctcct cV C! a pCsitvc in!ants l
mCnt C! agc anU mCrc tan V mCnts C! agc.
lOB!DOD!
Jc stanUarU C! carc cCnsists C! antrctrCvira Urugs
suc as 17J [ZiUCvuUinc| anU UU1 [UiUanCsnc|, prC-
tcasc iniitCrs, anU tmctCprim-su!amctCxaZCc
prCpaXs againsI \, tc mCst cCmmCn anU
scriCus CppCrtunistc in!cctiCn. IcV parmacCCgc
tcrapics avc UrasIica imprCvcU tc canccs C!
iving Cngcr vit 1` n!cctCn.
hLX LW
_
T. NOS1 H1V 11f11O1S 11 f11111 B1 Bft11 11
111O O1 |111B1B11j (8U%); S1B111 11111S 1S111
11O1 11OO |1O1f1 11B1S11S1O1S B1 SX1B1
11B1S11SS1O1.
2. 111B11S 1O11 1O H1V|OS111V 1O111S B1 B1vByS
S1O|OS111V 1O1 1B111B11j 11 1QG B1111O1S
1O 11 V111S; 111S, 11 1Iy1 11111OBSSByS 1S
1O1 Sf1111Q O11 |O|11B11O1S B1 1O1 11|111 11
f11111 11O1 T8 1O111S O1 BQ.
d. 11 1B111S1B11O1S O1 |1B111f H1V B1 VB11.
C11111 1By 1 BSy1|1O1B11f O1 |1S11 v111
B1y O1 O1 SV1B1 O1 11 1O11Ov11Q S1Q1S B1
Sy1|1O1S: B1O|B11y, 1|B1O1QB1j,
S|11O1QB1j, 1B1111 1O 1111V, 1f11111 O1
f11O11f 1B111B, O1B1 fB111BS1S, |B1O1111S, B1
V1O|111B1 1By.
* IOI TOI SB1O1 ~ * HO IR IOB 1 ~
M1HP 1WLL1LWb
L LM1LLMLLL
* CPB M OBBKYOHBOQR* VLP G9 OH9=
|Y ~
LllC| 12 / |HCClOuS LSCSC 159
ira ircc!iCns arc guI!c cCmmCn In !c inan!
anU yCurg ciU u! Uccrcasc Vi! agc ccausc C
ac_uircU immuni!y. cvcra vira incsscs !a! arc c-
gucn!y crcCun!crcU in !c QcUia!ric QCQua!iCn arc
nC! usuay sccn in aUu!s. any C !csc Qrcscn!
Vi! carac!cris!ic rascs !a! Qcrmit rcac cinica
UiagnCsis. ivc a!!crua!cU vaccircs arc rCu!incy aU-
minis!crcU !C Qrcvcn! mcascs, mumQs ruca arU
varicca [cicRcnQCx). 1CscCa anU cry!cma incc-
!iCsum arc gcrcray cnign in ciUrcn. ac Z-
Ucscrics !c !yica grcscr!aIICrs anU cCmQca!iCns
C !csc vira incsscs in ciUrcn Vic arc Uis-
cusscU r!cr in LaQ!cr D.
|n!rCUuccU in!C !c sRn y a !IcR i!c anU susc-
qucn!y sQrcaU via !c ymQa!ics anU CCU vcsscs.
cy rvaUc anU mu!IQy Vi!n !c cnUC!cia anU
smCC!h muscc ccs C CCU vcsscs causing gcncr-
aiZcU vascuar inury !a! caUs !C yCaumincmia
cUcma, yQCvCcmia anU yQC!cnsCn.
LQOODOOQg
1I Cccurs mCs! C!cn c!Vccn 1Qri arU
cQ!cmcr In !IcR-ncstcU arcas C !c sCu! 1!ar!ic
anU sCu!cas!crn s!a!cs. csQi!c !c namc nCnc C
!c !CQ !cn s!a!cs rcQCr!ing 1 is ncar !c 1CcRy
Cun!ains.
H5K BC!Ol5
HLLhX WLLWP1W
bVLLL LMLH
VB!DOQODO55
c mCs! 8igriLcan! risR ac!Cr is rcsiUcncc ir Cr
!avc !C an cnUcmic arca Uurirg !imcs C !c ycar
vcn !icRs arc ac!ivc.
1CcRy Cun!ain sQC!!cU cvcr [1) is a !IcR-
Crrc Uiscasc causcU y liclettsia tclettii, a gram-
rcga!ivc In!raCcuar ac!crium. 1cRc!!sac arc
LDCB NBDO5!B!OD5
Ht5IOryondhy5tcolLxomtnoItOn
ymQ!Cms UcvcCQ Z !C Uays a!cr a !IcR i!c.
!n!ia symQ!Cms C!cn arc nCnsQcciLc anU ircuUc
bLL 12-9

l0S0DttODS DO LOHQlCtODS O LDlODOOO Vll llD0SS0S
VrUS
NCSCS
NuuS
|uD
HOSCO (luuH
lC|SV|uS 6)
L|yllCu lHCClOSuu
(hII USSC,
IVOVIuS 819)
LlCkCHOX (VICC|
LXDlPD
LODUPHl, CIyIlCulOuS
uCuOuf |Sl
lll Sl|lS OD lCU HU
|OgfPSSCS CuUy
POH
bu| lO uCSS Dul
UOCS HOl COCSC
NCuOuf
|C |yIlCu gVHg
SCU ClCCkS
CIHCC OOwCU
Dy SfCU lO CXl|CulS
H IClCu| lIC|H
||u|lC, C|yllCulOuS
uCuS CVOVC lO
VSCS HU llCH C|uSl
OVCf, DCgHS OH CC HU
S|CUS lO CXlICullPS
LlDPr lPlUrOS
LO|yZ, COugl, COHjuHClVlS,
hOkS SOlS (OH DuCC
uuCOS |y H USS)
bwOH SV|y gHUS,
SCly rOlU gHUS
buDOCCl HU OSICtO|
u|Cu| yulUCHOlIy
Mgl CVf |CSOVCS S fSl
CIS
fHSHl SlC C|SS H
ClU wll
CuOgODHOlly
FS Hl SOHS |CSOVC, Hw
C|OS O|u SO lll CSODS
H U!CfCHl SlgCS fP
ODSfVU SuulHCOuSy
LOD@|ClODS
|HeuuOD, uyOCfUlS,
HCCllS, ffC. SuDCulC
SCPIOSHg HDCllS
L|CllS, HC|CllS, ||.
uHlHglS, CHCCllS
|Oy|lCu| IllIlS OI
|lI|gS, ||C: CHCllS
|CDIC SCZufCS, ||:
uCHHgOCHCCllS
Ffll|lS, ||C. CHCPllS
bCCOHU|y DCICI
DCClOH, ||. HCuuOH,
CC|D| lX,
CDCllS, lCllS
< IOI TOI SB1O1 ~ * HO IR IOB 1 ~
1 ||ucr|rL |COl|CS
* CPB V OBEKYOHBOQRt VLP G9 OH9= @
cvcr cis caUacc maaisc anU myagias. c ras
cgins Cn !c !irU Cr CurI Uay anU cCnsis!s C cry-
!cma!Cus macuCQaQuar csiCns !a! QrCgrcss !C
Crm QC!ccIac Cr QurQura [cCrrcsQCnUng !C a viUc-
sQrcaU sma-vcssc vascuI!is|. !! carac!cris!Icay
aQQcars ini!iay Cn Ic Vris!s anU anRcs anU sQrcaUs
QrCxImay !C invCvc !c IunR anU caU Cvcr scvcra
Curs. yQicay !c Qams anU sCCs arc InvCvcU as
vc. JC ras Is ascn! in 5% C ciUrcn. 1QQrCxi-
ma!cy 30% C ciUrcn avc sCmC ImQaIrmcnt C
mcn!a s!atus.
LOlOD!B LBQDO55
cningCcCcccmia anU mcascs [CsQcciay a!yQica
mcasCs| may c cCnscU Vi! 1. rIciCsIs
anC!Cr IicR-Crnc rIcRc!!sia InCc!ICn is usua
assOcia!CU Vi! ncuICQcria, ras is Qrcscn! in uQ !C
50% C ciUcn.
LBQDO5!C LVBUB!OD
1!Cug ImmunCHuCrcsccn! s!aining C sRIn iCQ-
sIcs !aRCn Cm ras si!cs may UcmCnsIa!c !c Crgan-
ism !Crc is nC rciac UagrCs!Ic !Cs! !a! ccCmcs
QCsi!IvC cary cnCug in !c cCursC C !c Uiscasc !C
gUiUc !craQy us !c cinicIan must maintair
ig susQiciCn Cr tc Uiscasc. 1n!iCUics !C cCnLrr
Ic cinIca UiagnCsis arc Uc!cc!aC aQQrCxima!cy
I 0 Uays a!cr symQ!Cm Cnsc!. cy aCra!Cry ca!urcs
IncuUC !rCmCcyICQcnia ccva!CU ivcr cnZymcs
yQCaumtncmta anU yQCna!rCma.
lOB!DOD!
rca!mCnt VI! UCxycycinc is cCc!Ivc. CIai!y is
igCr In !Csc rcccivIng Ica!mcn! mCrc !an 5 Uays
a!cr symQ!Cm Cnsc! anU !Csc Vi! gas!Cin!cs!Ina
symQ!Cms.
XWL L1bLPbL
VB!DOQODO55
ymC Uscasc Is a !IcR-Crnc InCss rcsu!ing |Cm
incc!ICn VI! !c sQirCccIc 8ore|ia |urgdoqeri.
c UisCasc Vas Lrs! UcscricU 20 yCars agC in ymc
LCnnCc!Icu! isCaIiCn C !c causa!ivc Crganism Cc-
currcU sCvcra ycars a!cr.
LQOODOOQg
1l!Cug cascs avc ccn rcQCr!CU acrCss tc
cCun!ry mCs! Cccur In sCu!crn cV nganU sCu!-
cas!crn Cv ^CrR |cV Jcrscy cas!Crn cnnsyvania
aryanU caVarc InncsC!a anU YiscCnsin. c
irciUcncC C ymc UIscasc is igcs! amCng ciUrcn
5 !C 0 ycars CU.
H5K BC!Ol5
!nUiviUuas vi! incrcascU CccuQa!ICna Cr rccrc-
a!iCna CxQCsurc !C !IcR-incs!cU vCCUanUs In
cnUcmic arcas arC a! Igcs! risR C ymc Uiscasc. 1n
Incc!cU !IcR mus! ccU Cr mCrc Ian +c Curs !C
IansmI! 8. |urdoqeri.
LDCB NBDO5!B!OD5
Ht5IOry
Cs! Qa!ICn!s UC nC! rCca a !IcR i!c. c cinica
manics!a!ICns UcQcnU Cn !c s!agC C !c Uiscasc-
cary CcaiZcU cary Uisscmina!cU Cr a!c. tylhCma
mgrau8, !C manics!a!iCn C Cary OtazCU Uiscasc
aQQcars a! !c sI!c C !c !IcR i!c !C I 0 Uays a!cr
Ic iIC. Jc ras cgins as a rcU macuc Cr QaQuc
anU QrCgrcsscs !C Crm a argc annuar cry\cma!Cus
csiCn Vi! ccnIa ccarirg !at is aCu! I 5 cm in
Uamc!Cr. c sRIr csiCn C!cn Is accCmQantcU y
CV-graUC cvcrs maaisc caUacC arIragias anU
myagias. aty U88CmualCU |ymC Uiscasc [3-5
vccR8 a!Cr !icR t!c| may manics! as mu!iQc cry-
!cma mIgrans csiCns [anyvcrC Cn !c CUy|
crania nCrvc Qasy mcnIngi!Is anU carUi!Is [carI
CcR|. c mCs! cCmmCn maniCs!a!ICn C alC
ymc UIscasc [>6 VccRs alcr !IcR I!c| is arIrI!is
usuay tnvCving !c Rncc.
hy5tcolxomtnoItOn
c ras UcscricU caricr may C QrCscnt. LiUcn
Vi! cary Uisscmina!cU ymc may avc mu!iQc
cryIcma migrans csiCns, acia ncrVC Qasy Cr signs
C mcningt!Is. LiUrcn vi! ymc ar!ri!is may avc
a sVCCn anU !cnUcr CIn!.
LOlOD!B LBQDO55
c UIHcrcn!Ia UiagnCsis UcQcnUs Cn !c Qrcscnta-
!iCn. YCn !c ras Is a!yQica i! may c cCnscU
Vi! cryIcma mu!ICrmc Cr crICma margina!um
[sccn in rcuma!Ic cvcr|. c Ui!CrCn!Ia UiagnCsis
IOI TOT SB1O1 * HO IR QOB 1
C! ar!ri!is asC incuUcs uVcnic rCuma!CU ar!ri-
lis, tcacltVc at!tt!ts, anU Ict!cts snUtCmc. hc
U:!!crcn!ia U:agnCsis C! mc mCningi!is incuUcs
C!cr causcs C! ascp!c mcningi!:s.
LBQDO5!C LVBUB!OD
Cr !c mCs! par!, car CcaZcU mc Uiscasc is
a cinica UagnCsis, ascU Cn suggcs!:Vc is!Cr anU
!c carac!cris!:c ras Cn psica cXamina!iCn. Jc
Crganism cannC! c rcia cu!urcU !rCm !c sKn
cstCns, CCU, anU C!hct CU utUs. mc Ig !i!ct
is ccVa!CU scVcra VccKs a!cr !c !icK !c. mc
\ C! \> [Cr Cin! uU rcia UiagnCscs mC
mcningi!is [Cr ar!ri!is. \arUiac inVCVcmCn!, in !C
!Crm C! cCnUuc!iCn anCrma!ics, is rarc u! can c
UagnCscU ccc!rCcarUCgram u cCnj unc!:Cn V!
suppCr!ing is!Cr anU an!CU s!uUics.
Jcs!ng !Cr mc Uiscasc in !c prCscncC C! Vagc
Cr nCnspccihc cCmpain!s is nC! cp!U, !asc-
pCsi!:Vc !cs! rcsu!s can Cccur. !CUcs !C 8
|urJqer| crc---rCac! Vi! C!cr incc!iCus agcn!s,
par!cuar C!cr spirCcc!cs, incuUing spis,
a!Cug `1I anU II rcmain ncga!iVc in pa!:cn!s
Vi! mc Uscasc.
* CPBH V OBBOPOHBOCHR* WYLP G9 [OH9=
|Y
C111 12 / l11<11O1S L1SS 11
lOB!DOD!
rca!mcn! C! car CcaiZCU mc UisCasc prcVcn!s
car UssCmina!cU anU a!c Uiscasc, incuUing mcnin-
gi!is anU ar!ri!:s. YCungcr ciUrcn can c !ca!cU
V! amCXiciin Cr ccurCXimc. Cnicin-acrgic
cUrcn can c !rca!cU Vi! cr!rCmcin. \iUrcn
CUcr !an c cars sCuU tccciVc Cra UCXccinc
!Cr + !C JU Uas. \Urcn Vi! scVcrc ar!ri!is Cr
carUiac Cr ncurCCgc inVCVcmcn! Varran! parcn!cra
!crap Vi! ig-UCsc pcnicin \ Cr ccriaXCnc.
mp!Cms !a! UC nC! rcmi! Vi!in JU Uas C!
!crap arc unlic !C C UuC !C rCsiUua in!cc!iCn,
anU anC!cr causc sCUU c sCug!.

hLX LW
1. 11 1y|1<! 1S1, 1y111 11Q11S, 1y 1
<O1|11 1y 1V1, 11, 1 11111Q1.
Z. Ly1 1SS 1S 111 v111 1OX1<1!!11 11 11!
11 yO11Q1 111 8 y1S, 1 v111 OXy<y<!11
11 O!1 11!11. Ly1 1111Q111S 1111S
<1111XO1.
d. Ly1 1SS, v11 111 |1O|1111y, OS
1O1 1S 11O11 Sy1|1O1S.
BI RTH
NeonatalNortal|ty
JlcIatc IctaI andcarIy nconataI criod is tlc tinc
oIIiIccxliIitingtlcliglcstnortaIityratcoIanyagc
intcrvaI. Jlc Qctnu1u mot!u1y tu1c rcIcrs to IctaI
dcatls occurring Iron tlc 20tl vccl oI gcstation
untiI tlc 7tl dayaItcrIirtl. !ntrautcrinc IctaIdcatl
[i.c.,stiIIIirtl) rcrcscnts40%to 50% oItlccrina-
taI rortaIity ratc.
Jlc nconu1u mot1u1y tu1c incIudcs inIants vlo
dicIctvccn Iirtl and2S days oIIiIc.Modcrrncona-
taI intcnsivccarc las dcIaycdtlcnortaIityoInany
ncvIorn inIants vlo lavc IiIc-tlrcatcning discascs,
sotlattlcysurvivcIcyondtlcnconataIcriodonIy
todicoItlciroriginaIdiscascsoroIconIicationsoI
tlcray sonctinc aItcr tlc 2Stl day oI IiIc. Jlis
dcIaycd nortaIity occurs during tlc Qo$!nconu!u
criod,vliclIcginsaItcr2S daysoIIiIcandcxtcnds
to tlc cnd oItlc IrstycaroIIiIc.
Jlcnun1 mot!u1y tu1c incIudcsIotltlcncona-
taI andtlcostnconataIcriods and is cxrcsscd as
tlcnunIcroIdcatlscr 000IivcIirtls.JlcinIant
nortaIity ratc intlc Lnitcd Statcs dccIincdin 999
to 7. cr 000 Iivc Iirtls. Jlc ratc Ior IIrican-
Incrican inIants in I 990vas a distrcssing 4. 6cr
000IivcIirtls.Jlcrcvcrc 27 couniricsvitlIovcr
inIantnortaIityratcs.
W
KEY PCI M
1. hC 1 tnn!mCtI||!yt!C|n!C Un|!Cd 5!!Cs
ws |QCt!n 21 C!Ct CCun!t|Cs,ndAlttCn
AmCttCn |nO!swCtC!wtCCs|tkC|y!C d|C
duttnQ!CDts!yCtC||C.
Apgar5cor| ng
JlcIgarcxanination,araidscoringsystcnIascd
on lysioIogic rcsonscs to tlc Iirtl roccss, is an
cxccIIcntnctlodIorasscssingtlc nccd IornconataI
rcsuscitation. lt isnot gcncraIIy uscIuI as a rognos-
tic tooI.JlcIgarscoringsystcnis slovr in JaIIc
J- I . It ! and 5 ninutcs aItcr Iirtl, cacl oI Ivc
lysioIogicaranctcrsiscvaIuatcd.FuII-tcrninIants
vitl a nornaI cardiouInonary transition viII lavc
a totaI scorc oIS to 9 at ! and 5 ninutcs.InIgar
scorc oI 0 to J indicatcs citlcr cardiorcsiratory
arrcst or a conditionrcsuItingIronscvcrc Iradycar-
dia, lyovcntiIation, and/or ccntraI ncrvous systcn
dcrcssion. Most IovIgar scorcs arc duc to diIl-
cuIty in cstaIIisling adcquatcvcntiIation and notto
rinary cardiac atloIogy.
BI RTHTRAUNA
Cephalohematoma
/ cclaIolcnatona is a traunatic suIcriostcaI
lcnorrlagc [usuaIIy invoIving tlc arictaI Ionc)
tlatdocsnotcrosssuturc Iincs.JlcscaIlcnatona
is claractcristicaIIy Irn vitlout discoIoration oI
ovcrIying sIIn and nay not Icconc aarcnt untiI
lours to days

aItcr dcIivcry. Frcdisosing Iactors


incIudc Iargc lcad sizc, roIongcd IaIor, vacuun
cxtraction, andIorccsdcIivcry. SontancousrcsoIu-
tion occurs ovcr scvcraI vccls. Jvo crccnt oItlc
lcnatonas viII organizc, caIci[, and Iorn a ccntraI
dcrcssion intlc caIvariun. CclaIolcnatona dis-
soIutionnayrcsuItinan indircctlycrIiIiruIincnia
rcquiring lototlcray, cscciaIIy in a rcnaturc
inIant.
< not 1oz sale I > < ue )Ln o)a I >
< cxau v )exaer~xoueepcvn: HYCA sT pouT. p ?
Cap!e| ! 3 / NeCOa!C|CQy 1 3
TAbLb 1 3- 1
PQgl bLOlDg bSt0D
hSlC OlntS 1 Olnt 2 OlntS
Lxam LvutU t
1 anU NlnuteS
NOpu|se <! 00 >! 00 Hea|!ta!e
Resp| |a!O|y ehO|!
CO|O|
NO|esp||a!|Ons
|a|e,cyanO!tC
AOseO!
|||eQu|a|,weakc|y
CyaOO!tce!|em|!|es
V|QO|Ous c|y
|| Ok!h|CuQOu!
Ac!|ve Nusc|e!One
Relex | |||!aO|||!y AOseO!
Weak,s||Q!|ylexeO ex!|em|!|es
G||mace Ac!|vec|yaOOavC|OaOce
Caput5uccedaneum
I caut succcdancun i s a diIlusc, cdcnatous, and
oItcndarlsvcIIingoItlcsoIt tissucoItlcscaItlat
ctcnds across tlc nidIinc and/or suturc Iincs and
is connonIy Iound in inIants vlo arc dcIivcrcd
vaginaIlyin tlc custonaryocciut-antcriorosition.
FrcssurcinduccdIronovcrridingarictaIandIrontaI
Ioncs against tlcir rcscctivc suturcs causcs tlc
noIdingassociatcdvitltlccaut.JIccautiscon-
nonIy sccn aItcr roIongcd IaIor in Iotl IuII-tcrn
andrcnaturc inIants.
Fractured C| av|c|e
I hacturcd cIavicIc is Iound in 2%toJ% oIvaginaI
dcIivcrics, and tlc riglt cIavicIc is tvo tincs norc
IilcIy to Iracturc ilan tlc IcIt. Jlis rcdiIcction
cistsIccausctlcrigltslouIdcrnustnovcIcncatl
tlcuIicsynlysisduringnornaIdcIivcryandnay
gctcntracd.FrcdisosingIactorsincIudclargcsizc,
slouIdcr dystocia, and traunatic dcIivcry. |indings
incIudc svcIIing and IuIIncss ovcr tlc hacturc sitc,
crcitus, anddccrcascdarnnovcncnt. CInconatcs
vitlcIavicuIarIracturc, S0% lavcnosyntons and
onIy nininaI lysicaI Indings. lt is oIcn diagnoscd
vlcnacaIlusisdctcctcdatJtoo vccIs.Radiogral
is noi indicatcd. o scciIc trcatrcni is ncccssary.
Jlc arcnts slouId Ic adviscd to avoid tcnsion CD
tlc aIIcctcd arn.
Erb's Pa| sy
lnjury to ncrvcs oItlc Iraclial Icxus rcsuIts lron
cxccssivc traction on tlc nccl, roducing arcsis.
FrI' s aIsy rcsuIts Iron strctcling oI tlc IItl and
sixtl ccrvicaI ncrvcs.Jlc inIant' sarn is lcId in tlc
"vaitcr`sti"osition,vlcrctlcarniscxtcndcdand
intcrnaIlyrotatcd,andtlcvristisIcxcd.Whentlcrc
is an aIscnt Moro rcIlcx in tlc riglt arn, and tlc
rigltland gras is intact, FrI's aIsy slouIdIc sus-
cctcd.inctycrccntoItlcscIcsionsrcsolvcson-
tancousIyIy4rontlsoIagc,IutiItlcncrvcdcIcit
crsists, ncrvc graIting nayIcIcncIciaI.
B
KEY PCI M5
M
1. A Cepa|Cema!Cma |sa!tauma!tC suOpe|tCs!ea|
emCttaQe!a!dCes OC! C|Css su!ute|tnes.
2. A Capu!suCCedaneum ts a dtluse,edema!Cus,and
CleO datkswe||tnQClIesCl !|ssueCl!esCa|p
!a!e!eOdsaCtCss!em|d| |neaOdlCtsu!ute
|tnes.
3. L|avtC|etaC!utes ea|wt!Cu!tO!etven!|CO,and
ate mCs! cCmmCO tn OaOteswt!maC|CsCmta
and/C|sCu|detdys!CCta.
4. LtO`s pa|sytesu|!stCm s!teIC|OQC!eDl! aOd
st! Ce|vtCa| Oetvesand sCu|d OesuspeC!ed
weO Ietets an aOseO!NCtC tel|eC!ettQ!
atm and antO!aC! ttQ! and Q|asp.
PRENATURITY
Iov-Iirtl-vciglt [IBV) inIants, dchncd as tlosc
inIantslavingIirtlvcigltsIcsstlan2500grans[g|,
rcrcscnt a disroortionatcIy Iargc crccntagc oI
nconataI and inIant dcatls. IItlougl tlcsc inIants
nalcu onIy 7% oIaIIIirtls, tlcy accountIortvo-
tlirds oIaII nconataI dcatls. \cry Iov-Iirtl-vciglt
[\LBV) inIants, vcigling Icss tlan 500g at Iirtl,
rcrcscntonIyaIout % oIalIIirtlsIuiaccountlor
50% olnconataI dcatls. ln conarison vitl inIants
vcigling 2500g or norc, IBV inIants arc 40 tincs
1 4
< o_ >
t
<.ue JTR o0 I >
< cxau v )exaex~xoueepcvn: HYCA oT pouT. DV >
| H C'
norclilcIytodicintlcnconaiaIcriod,and\IBV
inIantslavca200-IoIdliglcrrisloInconataIdcatI.
In contrast to tlc inrovcncnts in tlc ovcraII
inIant nortaIity ratc, tIcrc las not Iccn inrovc-
ncnt in tIc ratc oI IBV brhs. Jlis is onc rcason
tlattIcinIantnortaIityratc oItIc Lnitcd Statcs is
tIcvorstoItIcIargc,nodcrn,industriaIizcd coun-
trics. II Iirtl-vciglt nortaIity ratcs arc caIcuIatcd,
tlc Lnitcd Statcs las onc oI tlc liglcst survivaI
ratcs, Iut Iccausc oI tlc Iargc nunIcr oI IBV
inIants,tIctotaI inIant nortaIityratc rcnainsligl.
IBVis causcdIyrcnaturcIirtIorintrautcrinc
grovrl rctardation. MatcrnaI Iactors associatcdvitI
laving an IBV inIantincludc rcvious IBV Iirtl,
Iov socioccononic status, Iov IcvcI oI cducationaI
aclicvcncnt, |acloIrcnata! carc, natcrnaI agcIcss
tlan ! o ycars orgrcatcr ilan J5 ycars, a slort tinc
intcrvaIIctvccn rcgnancics, unnarricd status, Iov
rcrcgnancyvciglt [css tIan JJII) and/or oor
vcigltgain during rcgnancy [css tlan I 0iI), and
IIrican-Incrican racc. MatcrnaI usc oI cigarcttcs,
alcoloI, and/or ilIicit drugs is aIso associatcd vitI
Iaving an IBV inIant. ScciIc ncdicaI causcs oI
rctcrnIirtl arcIistcd inJaIIc I J-2.
KEY PCI M5
1. LOwD| |twC|Qt | tS mkC Up 1%O|| D|f\S
DU\CCOUtlOf\wO \| fUSO|| COt| UC\S.
2. VCfy|Ow D||twC|Q\ |ltSfCpfCSCt 1% O||
D|f\SDU\CCOUtOfb%OCO\| UC\S.
3. | COmf|SO w|t |tSwC|Q|Q 2Q Of
nOfC, LbW| ltSfC4\|nCSmOfC | | kC|y\O U|C
|tC CO\| Cf|OU,UVLbW |\S C
2U O|U |QCff|Sk OCO\| UCt.
4. LCfCSO\\\C | t mOft||\y ftC OtC
U|\CU b \\CS |S SO |Q |S\\\CftCOL1W
D|f\S |S |Q.|D|ftwC|Qt nOf\||\y ftCS fC
C|CU|\CU,\C U|tCU b \\CS SOCO\C
|QCS\ SU|V|V| f\CS,DUtDCCUSCOtC |fQC
UnDCfOLbW|\S,\C|\mOft||\yftC
fCn|S|Q.
b. LbW|SCUSCU DyfCm\UfCD||!Of |tfUtCf|C
QfOwt fCtfUt|O.
PC5TNATURITY
InIants vlosc gcstation cxcccds 42 vccls arc con-
sidcrcdostnaturc and arc at rislIortlcsyndronc
1AbLb 1 3-Z
N0OC LUS0S O l0t0fD ttD
C1 1
| Ct||StfCSS
N!|t|p|CgCStt|I
Lfyt1 |I1 |StIS|S1 Ct||S
NI |nn!C yUrOpSCt||S
LIQC|t| I n| |CS
F |<C1 1
| |CCt prC |
P1 |! pt|I p|CCt
U1 Cf1C
1 |CI |!tC!tC|!S
|CI npCtCtCC| |x
M1 C1|
| fCCC|npS|
L|I |CnC |C| |||CSS
|CCt|I (CI r|In|O|t|S)
L |!Q1 USC (CSpCC|||yCIC|C)
O1 Cr
| |CntU|C |!ptU|CI1 nCn1 rCS
|I |yy|n |OS
1 |! n
L |Cty|St! |1CSt|O| CxpOS! |C
oIostnaturiiy.Jlccausc oIproIongcdrcgnancyis
notInovn innostcascs.
C| | n|ca| Nan|lestat|ons
Jlc syndronc oIostnaturity is claractcrizcd Iy
nornaIIcngtI andlcadcircunIcrcnccIutdccrcascd
vciglt. !nIantsvitItIis syndronc arcdisiinciIion
snaII Ior gcstationaI agc inIants in tIat tIcy vcrc
doingvcI!untiItIcyvcntIcyond42 vccls' gcsta-
tionandIccancnutritionaIIydcrivcdIronIaccn-
taI |nsuIIcicncy. Connon syntons incIudc dry,
ciaclcd, ccling, loosc, and vrinIlcd sIin and a
naInourislcd acarancc vitI dccrcascd anounts
oIsuIcutancous tissucs. Conditions tlatoccuinorc
IrcqucniIy inosinaiurcinlants incIudc ncconiun
asiration and dcrcssion at Iirtl, crsistcnt uI-
nonarylycrtcnsionoItlcncvIorn[FFI),lyo-
glyccnia, lyocaIccnia, and poIycytlcnia.
Treatment
FctaI vcII-Icing slouId Ic nonitorcd c!oscIy Iy
uItrasound, IiolysicaI roIIc, and nonstrcss tcsts.
IntraartuntrcatncntinvolvcsrcarationIorcri-
< not 1oz sale I > < ue )Ln o)a I >
nataI dcrcssion and ncconiun asiration. LarIy
Iccdin to rcduce tIc risl ol IyoIyccnia and
cvaIuation Ior tIc conditions notcd aIovc cnconass
ostartun trcatncnt.
KEY PCI M5
W
! . |tSwOSC QCS\\|O CxCCCUS42wCCkS fC
COS|UCICU pOS\m\UfCUfCtf|SkOftC
SyU|OmC OlpOS\mtUf|\y.
2. LOU|t|OStt OCCUf nOfCfCQUCt|y|pOStn
tUfC |tS |C|UUCmCCO|UmSp||\|O U
UCpfCSS|O \ D|f\,pCfS|StC\ pU|nOfyypCf
tCS|O Ol\C CwDOf,ypOQ|yCCn|,ypOC|
CCn|, U pO|yCytCm|.
I NTRAUTER| NEPRCBLEN5
5ma| | lorOestat|ona| Age
0IDDg6D6SS 0D0 LDC0 N0D6SI0IDDS
InIants vIo arc snaII lor cstationa ac Iavc IirtI
vciIts IcIov iIc ! tI crccntiIc Ior cstationaI ac.
Tvo Iroad catcorics oI intrautcrinc rovtI rctarda-
tion Iave Iccn dcscriIcd. carIy onsct and Iatc onsct.
Cnc-iIird oI IovIiriI-vciIt nconatcsinIants
vciIin css tIan Z5 arc snaI Ior cstationaI
ac.
LarIy-onsct, or synnetricaI, inirautcrin rovtI
rciardation is iIouIt to resuIt Iron an insut tIat
Icins IcIorc ZS vccls' esiaiion. TIc carIy insuIi
rcsuIts in a nconaic vIosc Icad circunIercncc
and IciIt arc rooriionatcIy sizcd and vIosc
vciIi-Ior-IciIi ratio is nornaI. TIis aitcrn is
sccn in inlants vIosc noiIcrs Iavc scvcrc vascuar
discasc vitI Iycricnsion and rcna diseasc or
in inIants vitI concniiaI naIIornations or cIrono-
sonaI aInornaIiiics.
Latc-onsci, or asynnctricaI, intrautcrinc groviI
rctardation starts aItcr ZS vccls' csiaiion. TIcsc
inIants Iavc a norna, or cIosc to nornaI, Icad cir-
cunIccncc viiI a rcduccd hcit and vciIt. TIc
vciIt-Ior-IciIt raiio is Iov, and iIc inIant acars
Ion and cnaciatcd. In tIis tyc oI inirautcrinc
roviI rciardatin, tIc neonatc initiaIIy Ias a nornaI
rov:I tra]cciory and IoIIovs a norna ccnti c Iinc
and tIcn IaIs oI1 iIc curvc Iatc in cstaiion.
HSk 0CIDlS
CrovtI rciardation nay rcsuIi lron IctaI causcs sucI
as nuItiIc cstation, concnitaI vira inIcctions,
< cz V )exaer~xoueepcxn: NCA sT pouT.
DV
>
LhptCr \3 / MCO1 tO1 OQy 1 b
cIronosonaI aInornaIitcs [trisonics or Turner's
syndronc), and concniiaI [cscciaIIy C!S) naIIor-
naton syndrones. !IaccntaI causcs incIudc cIori-
onic viIIi tis, cIronic aIrutio Iaccntac, tvin-ivin
transIusion, IaccntaI iunor, and IaccntaI insull-
cicncy sccondary to natcrnaI vascuIar diseasc.
MatcrnaI causcs ol intrauicrinc roviI rctardation
|ncIudc sevcrc criIcraI vascuIar discascs tIai
rcducc utcrinc IIood Iov, sucI as cIroni. Iyertcn-
sion, diaIetic vascuIoatIy, rcccIansia, sicllc ccIl
ancnia, and cardiac and rcna discasc. CtIcr natcr-
naI causcs incIudc reduccd nutritionaI intalc, aIcoIoI
or dru aIusc, ciarct t snolin, and utcrinc anon-
aIics or uterinc constraini. Iterinc constraint is notcd
in noiIcrs oI snaI statur and rcduccd vciIt ain
durin rcnancy.
l60ID6DI
lnlants vIo arc snaI lor csiationaI ac Iavc a II
risl Ior intrautcrinc IctaI dcaiI. TIcrcIorc, rcnataI
nanacncnt incIudcs idcntifcation, cvaIuation, and
nonitorin. TIc standard intrauicrinc rovtI rciar-
dation vorlu incIudcs a rcvicv ol oIstctric causcs,
canination Ior idcniifallc syndroncs, and IaIora-
tory cvaIuation lor concnitaI inlcction. /ntcartun
IciaI nonitorin vitI scriaI uItrasound, IioIysicaI
rofIc, nonstrcss tcst, and oyiocin cIaIIcnc test is
oItcn uscd. locr canination oI accntaI lov is
uscd to dctcrninc iI utcroIaccntaI | nsulicncy
cists. Il carIy dcivcry is Icin contcnIaicd, dctcr-
nination oI uInonary naiurity is criticaI. Lary
dcIivcry is ncccssary vIcn it is dctcrnincd iIat iIc
risl to tc Ictus oI stayin in uicro is rcaicr tIan tIc
risl oI rcnaiurc deIivcry. lctaI Iun naturity can Ic
acccIcratcd, iI ncccssary, Iy stcroid adninistraiion. II
iIcrc is accntaI insuIlcicncy, iIc Ictus nay noi
tocratc IaIor and nay rcquirc dclivcry Iy ccsarcan
scction.
lcIivcry sIouId talc Iacc ai a ccnicr vitI a IiI-
risl nurscry, Iccausc inIants vIo arc vcry sua!I Ior
cstationa ac arc at risl Ior IiIc-tIreatcnin roI-
cns at tIc tinc oI dcivery. TIc dcIivcry tcan
sIouId Ie rcarcd Ior crinataI asIyxia and/or
dcrcssion, ncconiun asiration, and IyoiIcrnia.
Laninaiion oI tIc Iaccnia aI tcr dcIivcry Ior atIoI-
oy consistcnt viiI concnitaI inIcction or inIarction
nay Ic IcIIuI in dctcrninin iIc causc ol tIc
intrautcrinc roviI rctardation. TIc ncvIorn tIat is
snaI Ior cstationaI ac sIoud lc nonitorcd Ior
IyoiIcrnia, IyoIyccnia, IyocaIccnia, Iyona-
trcnia, oIycytIcnia, uInonary IcnorrIac, and
crsistcnt uInonary Iycrtcnsion. Lculocnia,
< not 1oz sale I > < ue )Ln o)a I >
|lLcgr|rl |CU|t||CS
< cxau v )exaex~xoueepcvn: HYCA oT pouT. p >
1
ncutrocnia, and tIronIocyiocnia nay Ic sccn in
inIanis Io to Iycricnsivc notIcrs. Conncncin
Iccdins as soon as ossilIc nininizcs IyoIyccnia.
Largelor Oestat| ona| Age
InIanis vIosc vciIt is rcatcr tIan Z standard dcvi-
ations aIovc tIc ncan or aIovc tIc 9tI crccntilc
arc dcfncd as Iarc Ior cstationaI ac. !conatcs at
risl Ior Icin arc Ior cstationaI ac arc tIosc oI
diaIctic notIcrs [cIass /, !, or C), ostnaturc
inIants, and nconatcs vitI transosition ol tIc
rcat vcsscIs, cryiIroIIastosis IctaIis, or !cclvitI-
Vicdcnann syndronc. lost inIants vIo arc Iarc
Ior cstationaI ac arc constitutionaIIy Iarc, Iron
Iarc arcnis or a IaniIy vitI a rcdiIcction Ior Iarc
inIants. /I1cr IiriI, tIc inIant sIouId Ic cvaIuatcd Ior
tIc disordcrs just dcscriIcd, as vcII as IiriI trauna,
vIicI occurs oI tcn in Iarc Ior cstationaI ac
nconatcs. TIc lIood suar oI tIc Iarc Ior cstationaI
ac inIant sIouId Ic nonitorcd and tIc cIiId Icd
carly, lccausc Iarc Ior cstational ac inIanis vIo
Iavc diaIctic notIcrs or vIo suIlcr Iron !cclvitl-
Vicdcnann syndronc or cryiIroIIastosis IctaIis
arc ronc to IyoIyccnia. CItainin a hcnatocrit
aI tcr IirtI is advisaIIc, lccausc Iarc Ior cstationaI
ac nconatcs Iavc an incrcascd incidcncc oI
oIycyiIcnia.
lacrosonic nconatcs havc IiriI vciIis rcatcr
tIan 4 . /II nacrosonic inIants arc Iarc Ior cs-
tationaI ac, Iut not aII Iarc Ior cstationaI ac
nconatcs arc nacrosonic. lacrosonic inIanis Iavc
an incrcascd risl oI sIouIdcr dystocia and otIcr Iiril
trauna. Conditions sucI as uatcrnaI diaIctcs ncIIi-
tus, oIcsity, and osinaturity arc associatcd vitI an
incrcascd incidcncc oI nacrosonia.

KEY PCI M5
1. |\ |S USClU| tOU|V|UC|!SwOfCSn||OfQCS
\\|O| QC|\OtwOCtCQOf|CS.SymmCtf|C|
(Cf|yOSCt) U SynnC\f|C| (|\COSC\OfCU
Spf|Q).
2. | tfU\Cf|CQfOwt fC\fUt|O myfCSU|\lfOm
C\|, p|CC\|,O| n\Cf| CUSCS.
3. | tSwOfCSn||lOfQCS\\|O|QCVC
|Q f|SkOf |t|U\Cf|ClC\| UC\,tCfCOfC,
pfCt| mQCmCt |C|UUCS |UCt|DC\|O,
CV|U\|O,U nO|\Of|Q.
4. NCO\CSt f|SkOfDC|Q |fQClOf QCStt|O|
QCfC\OSC OU|DCt|C mOtCf6 (C|SS P,b,O|L),
pOStmtUfC |tS,U COtCSw|\\fSpOS|
\|O O\CQfCtVCSSC|S,CfytfOD|StOS|SCt||S,Of
bCCkw|tW|CUCmSyUfOmC.
b. NOS\|tSwOfC |fQCOfQCS\\|O|QCfC
COSt|\Ut|O||y |fQC,lfOn |fQC pfC\S Of
n||yw1 t pfCU||CCt|OlOf |fQC | l\S.
. NCfOSOn|C CO\CSfC SUDC\CQOfyOl |fQC
lOf QCS\t|O| QC| tS U VC D|f\ wC|Q\S
QfC\Cft4Q.1CyfC tS|Q|DCtf|SkOf
SOU|UCfUyStOC|.
Po|yhydramn|os
!oIyIydrannios is dcfncd as an anniotic Iuid
voIunc rcatcr tIan Z itcrs, it occurs in in !
IiriIs. /cutc oIyIydrannios is associatcd vitI rc-
naturc IaIor, natcrnaI disconIori, and rcsiratory
conronisc. lorc oI tcn, oIyIydrannios is cIronic
and is sccn vitI cstationaI diaIctcs, innunc or non
innunc Iydros IctaIis, aIdoninal vaII dclccis
[onIaIoccIc and astroscIisis), nuItiIc cstations,
trisony ! S or Z ! , ncuraI tuIc dcIccts, and ccriain
concnitaI anonaIics oI tIc astroinicstina tract.
/ncnccIay and ncninonycloccIc arc ncural tulc
dcIccts tIat inair IctaI svaIIovin, vIcrcas
csoIacaI or duodcnal atrcsia, diaIranatic
Icrnia, and cIcI t aIatc intcrIcrc vitI svaIIovin and
astrointcstinaI Iuid dynanics.
C| | gohydramn|os
CIioIydrannios is associatcd vitI intrautcrinc
rovtI rctardation, anniotic luid Ical, ostnaturity,
and concnitaI anonalics oI iIc IctaI idncys.
!iIatcraI rcnaI acncsis rcsuIts in a sccifc dcIorna-
tion syndronc novn as !oticr's syndronc. TIc syn-
dronc is cIaractcrizcd Iy cIuI Icct, conrcsscd
Iacics, Iov-sct cars, scaIoid aIdoncn, and dinin-
isIcd cIcst vaII sizc tIat is acconanicd Iy uI-
nonary IyoIasia and ncunotIora. Itcrinc
conrcssion in tIc aIscncc ol anniotic Iuid rctards
Iun rov1 I, and aticnis vitI iIis condition circ
oI rcsiratory IaiIurc ratIcr tIan oI rcnaI insuIf-
cicncy. CIioIydrannios incrcascs tIc ris oI Icta
distrcss durin IaIor. TIis risl nay lc rcduccd Iy
nornaI saIinc annioinIusion durin IaIor.
< not 1oz sale I > < ue )Ln o)a I >
< cxau v )exaex~xoueepcvn: HYCA sTpouT. DV >
LtC| 1. |CCtC|Cgy 17

KEY PClM5
"
1. L|O|CpO|yyUfm|OS |S SCCw|\ QCStt|O|
U|DC\CS,| mnUC OfO|nnUCyUfOpSC\||S,
DUOn|| w|| UCCC\S (Omp|OCC|CU
QStfOSC|S|S),n1 |\|p|C QCS\\|OS,tf|SOmy !BO|
21, CUf|\UDCUCCCtS,U CCft| COQC|\|
Om||CSOtCQSt|O| \CS\||tfCt.
2. L| |QOyUfm|OS|SSSOC|\CU w|t |\fUtCf-
|C QfOwt fCtfU\|O,m|Ot|C|U|U |Ck,
pOStmtUf|ty,U COQC|\| On||CSO
tCCt| k|UCyS.
CCNOENITALI NFECTI CN5
InIcctions oI tIc Ictus durin tIc frst, sccond, or
carIy tIird trincstcr arc rcIcrrcd to as Congcn!u
ncC!on8. CIassicaIIy, tIcy arc rcIcrrcd to as
lVL1 1cc\o1h, an acronyn Ior tooIasnosis,
1reonemn n//dum inIcction, otIcr inlcctions,
ruIcIIa, cytoncaIovirus inIcction, Icrcs sinIc,
and |I' /!tIouI it is inoriant to Ic Ianiia: vitI
tIis acronyn, it Ias scvcra sIoriconins. Icrcs
sinIc and II\ arc nucI norc connonIy crina-
taI [ratIcr tIan concnitaI) inlcctions, and tIcrc is an
cvcr-candin Iist oI viruscs tIat couId Ic incIudcd
in tIc otIcr" rou. TIc nost inortant concnitaI
inIcctions and tIcir syndroncs arc discusscd in tIis
scction. TIcrc arc nany siniIaritics in tIc concnitaI
syndroncs, so Iocusin on tIc diIlcrcnccs can IcI
rcInc tIc cvaIuation. TaIIc ! J-J sunnarizcs discasc-
scci!c cIinicaI fndins and IaIoratory cvaIuation.
Toxop| asmos|s
TooIasnosis is causcd Iy 1oxo/nsmn gond|| an
intraccIIuIar rotozoa arasitc Iound in nannaIs
and Iirds. lcnIcrs oI tIc cat Ianiy arc tIc dcfni-
tivc Iost. InIcctcd cats ccrctc tooIasna oocytcs in
tIcir stooI, rcsuItin in |cca-ora transnission to
Iunans.
TIcrc arc aroinatcIy J cascs ol concnitaI
inIcction annuaIIy in tIc Initcd Statcs. CnIy rinary
inIcction ol tIc notIcr, vIic is usuaIIy asynto-
natic, rcsuIts in concnita inIcction. /non tIc
inIants oI voncn inIcctcd vitI tooIasnosis durin
tIc frst trincstcr, Icss tIan Z% viI Ic inIcctcd, Iut
tIcir discasc viII IilcIy Ic scvcrc. |I tIc notIcr's
inIcction is acquircd in tIc tIird trincstcr, as nany
as o5% viI ivc IirtI to an inIcctcd nconatc, Iut tIc
inIcction viI Ic niId or asyntonatic.
LDC0 N0D6SI0IDDS
InIants inlcctcd carIy in rcnancy suIlcr Iron
intrautcrinc ncninocnccIaIitis and rcscnt vit
nicroccIaIy, IydroccIaIus, nicroItIaInia, cIor-
iorctinitis, intracraniaI caIcfcations, and scizurcs.
TIcsc inlants nay aso acar sctic and Iavc j aun-
dicc, IcatoscnoncaIy, urura, ctccIiac, a nac-
uIoauIar rasI, and cncraIizcd IynIadcnoatIy.
CI inIants vIo arc asyntonatic at IirtI, 7% viII
suIIcr Ion-tcrn scqucIac, vIicI nay includc ncntaI
rctardation, Icarnin disaIiIitics, and cIoriorctinitis.
CcuIar discasc can Icconc rcactivatcd ycars aItcr tIc
initiaI inIcction, IotI in IcaItIy and innunocon-
roniscd individuaIs, rcsutin in inaircd vision or
IIindncss .
ScroIoic tcsts arc tIc rinary ncans oI dcfnitivc
dianosis. / lourloId risc in antiIody titcr or scro-
convcrsion Iron ncativc to ositivc indicatcs tIc
rcscncc oI inIcction. In concnitaI inIcction, dia-
nosis nay Ic conicatcd Iy tIc rcscncc ol natcr-
naIy dcrivcd transIaccnta antiIody. II tIc natcrnaI
antiIody status is ncativc, tIc dianosis oI concni-
ta tooIasnosis is ccIudcd. |l natcrnaI and
nconatc IcvcIs arc ositivc, scriaI studics oI antitoo-
Iasna !C Ior scvcra nontIs arc ncccssary to dis-
tinuisI transIaccntaI antiIody |ron concnitaI
inIcction. LcvcIs oI transIaccntaI antiIody IaII ovcr
tIc frst ycar oI Iilc, vIcrcas antiIody IcvcIs hon
concnitaI inIcction rcnain staIc or risc. / con-
utcd tonoraIy [CT) scan oI tIc Icad nay rcvcal
ccrcIraI caIcifcations in tIc ccntraI ncrvous systcn.
TIc arasitc nay Ic visuaIizcd in tIc ccrcIrosina
luid Iy cytoccntriluc rcarations or Iy roxI
in inocuIatcd inIant nicc. TyicaI IistoatIoloy or
cysts nay Ic | dcntifcd in Iiosy sccincns oI
invoIvcd un, Irain, Ionc narrov, or IynI
nodc.
l60ID6DI
Jrcatncnt includcs IotI yrinctIaninc and suIIadi-
azinc, vIicI act syncristicaIIy aainst 1oxo/nsmn.
TIcsc antiIiotics inIIit IoIic acid, so tIcy arc uscd
in conjunction vitI IoIic acid. Corticostcroids arc
rcscrvcd |or inIants vit scvcrc ccntraI ncrvous
systcn or ocuIar inIcction.
Incstion oI vcI-cookcd ucat and tIc avoidancc
oI cats and soiI in arcas vIcrc cats dclccatc
rcducc tIc ris oI tooIasnosis in rcnant or
innunoconroniscd aticnts. Cat Iittcr sIouId
Ic disoscd oI daiy, Iccausc tooasna oocytcs
arc not inIcctious Ior tIc frst 4S Iours aI tcr
assa.
I >
1AbLb 1 3- 3
L0l0DttDg DO LVUtDg bOH0 LODg0DI D0CtOD5
Pent
!OxO|05u0 gOn0tt
!tCOnCu0 0|t0uu
KU1C| |
Ly!OnCg|I ||US
|C|pCS S|np|Cx
b_ClC L nC tuleS
|yUfOCCp1|US |tgCC|||2CU
C| C|1Ct|OS, CO||O|Ct||t|S
LStCICIf|t|S pC||ISt|t|S,
C<CntI |U Sk| |S1,S!11CS
LyC.Lt|CtS,C|I!yCO|C,
p|gnCtC |Ct|
bk|1 |!C1C||yn!1"Sy |I nC
1IC.NC|t|C| St||t|I
|C|t | tCt!CtUS,pU1 nO|y
StCOS|S
N|CfICCp|y |t pC||Ct||CU||
C|C|1Ct|IS,
CptOSp|CI nCg|y,
CI ||O|Ct!|t|S|QU|| Cm|S
| n|CS,t|I n1ICy!OpC |
bk|CS|C|CSI | C!C Sk|,
kC|tOCI )!Ct| |t|S,C!tC
CCtf|C|I USSyStCn D|gS
SUCSSC|7 U! CS
LDOftOf_ LvutOn
1 OXOp|SnIS|S|g!t|1Oy
1I ||IC1y|gN,1 |C| S nO|C
SpCC|1C.
NI t|CpOCn|tCSt SUC S K| KI |
N1 K1,SUppOftCU1 y tfCpI Cn|
tCStSUCS |gN 1PPb.
NtC|| |!1C|||nnUCStt!S.|1
|nn!C,SCU |1 t S | g! tC
nI fC SpCC|1 C |gN.|1 |QN |S
Cgt|C,1!t | g! |S pOS|t|C, |||
C!1 tU|CS|In! ||C, CC|C1 |ISp|1
1! |, t|OtS1 S ny|SI |tC
tC ||!S.
U ||C1 O|Cy!I nCQ|O |fUSCU|t! |CI |
|p|ULNN C||yt|gC tCSt.
N ||| CU|t! |CS |I n CC|C1 |ISp||
1! |,Sk| |CS|IS,CO)!Ct|C,
U||C,1 |Of,|CCt! n,
SI p! yxSI!1 g! Ow w1 t|
2-3yS.|LKO1 Lb1 .
L ||CCt1!I |CSCCtt|1IySt||g
O1 SC|p|Q1 |I n Sk| |CS|O |S
SpCC|1 C1 UtItSCS|t|C.
LNV, CyIOUCgOvI|US, CS|CC|COf S[lH u|d,|JAA8S,UO|CSCCHI IfC[OHCU HIlOOCy ICSI, LH, [OyUC|SC CDH fCCIlOH ICSI, HH, fQC [SU
fCglH tCSI, VLHL, VHCfCl LlSCSC HPSCfCD LOOlIOfy IPSI
_ KEY PClM5
1. 1OXOp|SnOS|S |S CUSCU Dy !OxO|O5DO gOD0,
|tfCC||U|fpfOtO2O| pfS|tC wOSCUCD|t| C
OSt|StCCtln||y.
2. L|y pf|nfy |lCCt|O OtC nOtCf,wO|S
USU11 y SynptOnt|C,tCSU1 tS| COQC4 t1
|lCCt|O .
3. | ltS |CCtCU Cf|y |pfCQCySUl1 CflfOn
|tfUtCf|C nC|QOCCCp||t|S U pfCSCt
w|t n|CfOCCp|y,yUfOCCp|US,n|CfO
t|n|,COt|OtCt||t|S,1 ttCt|1 C1 C|DCt|OS,
U SC|2UfCS.
4. L|tSwOfCSynptOnt|CtD|ft,1%
SUl1 Cf|On |OQtCfn SCQUC|C,w|C ny
| C|UUCnCt| fCtfUt|O,|Cf|QU|SD|||t|CS,
U COf|OfCt||t|S.
5yph| | | s
SyIiIis rcsuIts Iron transIaccntaI transnission oI
1reonemn n//dum. SyIiIis in tIc untrcatcd rc-
nant vonan uay Ic transnit tcd to tIc lctus at any
tinc, Iut IctaI transIcr is nost connon durin tIc
hrst ycar oI natcrnaI inIcction.
LDC0 N0D6SI0IDDS
conatcs syntonatic at lirtI nay cIiIit nonin-
nunc Iydros vitI ancnia, tIronIocyiocnia,
Iculocnia, ncunonitis, Ieatitis, ostcocIondritis,
and rasI. Connon nanIcstations dcscriIcd u
tIc frst ycar oI IiIc incIudc intcrnittent |cvcr,
ostcitis and ostcocIondritis, IcatosIcnoncaIy,
lynIadcnoatIy, nucocutancous lcsions [nacu-
Ioauar rasI on tIc trunl, aIns, and soIcs),
crsistcnt rIinitis [snuHcs), ]aundicc, and IaiIurc
to tIrivc. LaIoratory tcsts nay rcvcaI IycrIiiru-
< not 1oz sale I > < ue )Ln o)a I >
Iincnia, a transaninitis, tIronIocyiocnia, lculocy-
tosis, and a Coonls' -ncaiive IcnoIyiic ancnia.
TIc Iaic scqucIac oI conenitaI syIils acar
nany ycars aItcr IiriI. TIcy ncudc nuItiIc Ionc
sins [IroniaI Iossin, saIcr sIins), IuicIinson tcctI,
nuIIcrry noIars, a saddIc-nosc dcIorniiy, rIaadcs,
]uvcnic arcsis, juvcnic taIcs, intcrstiiiaI kcratitis,
ciItI ncrvc dcaIncss, and CIution joints [ainIcss
]oint cIlusions) . TIcsc naniIesiations arc rarc in iIc
nodcrn cra in vIicI cniciIIin iIcray is uscd to
ireai concnitaI syIiIis.
L0gDDSIC Lv0U0IDD
LaIoratory tcsts | ncIudc nontrconcna tcsts sucI as
iIc vaid Iasna v cain tcst [ R!R) and tIc \cncvcaI
Liscasc RcscarcI LaIoratory icsi