Neonatal
Jaundice
(Hyperbilirubinemia of The Newborn)
Neonatal Jaundice
• A yellow discoloration of sclera & skin due to
an increase in serum bilirubin
• Jaundice usually appears in the newborn infant
when serum bilirubin concentration exceeds
85 mol/L
• Neonatal jaundice occurs in the majority of
infants (>50% of the term and 80% of the preterm infants)
Pathogenesis
• Increased bilirubin load
• Extravascular blood collection
• Polycythemia
• Dehydration
• Increased enterohepatic circulation
▪ Paralytic ileus
▪ Hirschsprung’s disease
▪ Intestinal stenosis or atresia
Pathogenesis
• Uncertain mechanisms
• Breast milk jaundice
• Breast feeding jaundice
• Some ethnic groups
Pathogenesis
• Conjugated hyperbilirubinemia
• Decreased excretion rate
▪ Obstructive lesions
▪ Congenital biliary atresia
▪ Tumors or cyst
▪ Inspissated bile
▪ Metabolic and endocrine abnormalities
▪ Hypothyroidism
▪ Galactosemia
Pathogenesis
• Mixed hyperbilirubinemia
• Hepatocyte abnormalities
▪ Sepsis
▪ Intrauterine infections
▪ Neonatal hepatitis
▪ Hepatitis viruses
▪ TORCH
▪ Idiopathic hepatitis
▪ Hypoxic insult
Causes by the age of onset
At Birth Or Within 24h Within The 1st Week
• Hemolysis (Blood group • Physiological jaundice
incompatibility) • Congenital infection
• Congenital infection (TORCH)
(TORCH) • Sepsis
• Sepsis • Concealed hemorrhage
• Crigler-Najjar syndrome
Causes by the age of onset
After The First Week Persistent After The 2nd Week
• Sepsis • Neonatal hepatitis
• Hepatitis • Biliary atresia
• Biliary atresia • Inspissated bile syndrome
• Breast milk jaundice
• Hypothyroidism
• Galactosemia
• Inherited hemolytic anemia
Kernicterus
Bilirubin neurotoxicity
Acute Encephalopathy Chronic Encephalopathy
• Three phases • Athetosis
• Hypotonia, lethargy, high • Sensorineural hearing
pitched cry and poor sucking deficit
• Extensors hypertonia,
• Limited upward gaze
opithotonus, fever and seizures
• Dental dysplasia
• Hypotonia replaces hypertonia
in a week • Intellectual deficit
• Death may occur up the
second phase
Kernicterus
Physiological jaundice
• Due to:
1. Increased synthesis of
bilirubin caused by the
excessive RBCs load after
delivery
2. Relative immaturity of the
liver glucoronyl transferase
• Onset usually on 2nd-3rd day
• Disappear around day 7-10
• Infant is usually healthy
Physiological jaundice
• Exacerbating Factors
• Prematurity
• Polcythaemia
• Dehydration
• Breast feeding (usually higher by 50-65 mol/L than formula
fed)
• Laboratory
• Rate of accumulation is less than 85 mol/L/day
Physiological jaundice
• Treatment:
• Maintain sufficient feeding
• Serial measurements of serum bilirubin
• Look for pathological causes if bilirubin level
exceeds the expected for age
• Phototherapy if needed
Phototherapy
• Indicated if bilirubin level exceeds a set point
• Exposure of infant to light with wave length
around 450nm
• Isomerization of bilirubin to nontoxic water
soluble form
• Eyes should be protected
• Control for temperature and fluid balance is
important
Management
Phototherapy
Physiological vs. Pathological
Physiological Pathological
2nd-3rd day of life in term
Onset At any time
infants
Level of S. Bilirubin Usually lower Usually higher
Type of Bilirubin Unconjugated Any
Slow increase May be faster
Rate of increase
(usually <85 mol/L/24h) (usually >85 mol/L/24h)
Shorter
Duration (usually 7-10 days in the term and May be longer
14days in Preterm)
Physical Exam and
Normal, healthy infant Abnormal
Lab. investigations
Breast MILK Jaundice
• Conjugated hyperbilirubineamia beyound 2nd
week of life
• Disappears within 2 days if breast feeding is
discontinued
• May take up to 3 months to resolve completely
• Due to (?) Postulated substance in human milk that
inhibits enzymatic activity of glucoronyl
transferase
• Other pathologies need to be excluded
• Treatment:
Breast FEEDING Jaundice
• May be related to decreased amount of milk
consumed by the infant (breastfeeding failure )
• More effective nursing may prevent early
“starvation” in breastfed newborns and reduce
the incidence of this type of jaundice
Hemolytic disease of the newborn
• Is the hemolysis resulting from Rh or ABO
incompatibility between the mother and the
infant
Hemolytic disease of the newborn
• Rh isoimmunization
• Mother is Rh–ve and the fetus is Rh+ve
• First pregnancy usually goes with no fetal problems
• Mother is sensitized by fetal Rh+ve RBCs
• Antibodies to Rh+ve RBCs is formed and it is
transmitted to the fetus during second pregnancy
• ~ 1/100 pregnancies and not in every Rh-ve mother
• May be prevented by AntiD administred to the
mother
Hemolytic disease of the newborn
Hemolytic disease of the newborn
• ABO incompatibility (milder disease)
• The anti-A or Anti-B hemolysins (antibodies)
present in a mother with blood group O is
transferred to the fetus of A or B blood group
• This results in hemolysis (most transferred
antibodies can be neutralized by the baby)
• 1 in every 200 births
• The first baby can be affected
Hemolytic disease of the newborn
• Severe hemolysis manifestations
• Hydrops fetalis
▪ The most severe form
▪ Often still birth with severe anemia and
massive edema and ascitis
• Icterus gravis neonatorum
▪ Jaundice in the first 24hr, not at birth
▪ Variable degree of hemolytic anemia
▪ Hepatosplenomegaly
• Gradual onset of anemia and slight
Hemolytic disease of the newborn
• Prevention
• Anti-D within 72 hrs after delivery of Rh+ve child
or abortion.
• Management:
• Life support
• Establish diagnosis
• Early phototherapy
• Transfusion to restore normal hemoglobin level
• Prevent dangerous rise of bilirubin hoping to
Exchange transfusion
• Indicated in
• Kernictrus
• Cord bilirubin >85 mol/L or
• Cord hemoglobin <100 g/L
• Rate of bilirubin rise > 8.5 mol/L/hour
• Unconjugated bilirubin level > 340 mol/L at any
age (at a lower level in presence of risk factor as
prematurity, sepsis)
• Double volume exchange transfusion
Exchange transfusion
Conjugated hyperbilirubinemia
• Always pathological
• Direct bilirubin >34 mol/L
• Evaluation
• Serum bilirubin
• Liver function tests
• Cultures (blood, urine,….)
• Abdominal ultra sound
• Isotopic scan (scintegraphy)
• Liver biopsy
Conjugated hyperbilirubinemia
Neonatal hepatitis Biliary atresia
Stool color Intermittent pale Persistent pale
Serum Bilirubin Biphasic Mainly conjugated
ALT, AST Marked increase Mild increase
Alk. Phosphatase Mild increase Marked increase
Liver Sluggish uptake, Uptake is intact but excretion
scintegraphy Eventual excretion of isotope into intestine is absent
Inflammatory Infiltration, Intact lobular architecture, bile
Liver biopsy distorted lobular architecture ductular proliferation or paucity,
and focal necrosis perilobular edema and fibrosis
Conjugated hyperbilirubinemia
• Treatment of chronic cholestasis
• Treatment of the cause
• Supportive
▪ Fat soluble vitamins
▪ Use of medium chain triglycerides-containing formula
▪ Attention to micronutrients as Ca, Ph, Zinc
• Surgical interference
▪ Billiary atresia: Kasai operation´, Liver transplantation
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