Arb

School of Nursing

Pediatrics and child health nursing

Renal disorders

By: Sayih M. (MSc, Lecturer)

03/28/2025 1
 Anatomy and physiologic overview
 The renal system consists of the kidneys, ureters, and
the urethra

 Kidneys are paired retroperitoneal structures that are

normally located between the transverse processes of
T12-L3 vertebrae, with the left kidney typically somewhat
more superior in position than the right

 The upper poles are normally oriented more medially and

posteriorly than the lower poles

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 Anatomy and physiologic overview…

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 Anatomy and physiologic overview…
Functions of kidney

 Filtration of metabolic waste products (urea and

ammonium)

Regulation of electrolytes, fluid, and acid-base balance

Stimulation of red blood cell production

Regulate blood pressure via the renin-angiotensin-

aldosterone system, controlling reabsorption of water and
maintaining intravascular volume

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 Anatomy and physiologic overview…
 Excretion of metabolic waste products (urea and
ammonium)

 Reabsorb glucose and amino acids

 Have hormonal functions via erythropoietin & calcitriol

production

 Vitamin D activation

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03/28/2025 6
 Definition
 Nephrotic syndrome is the clinical manifestation of
glomerular diseases associated with heavy (Nephrotic-
range) proteinuria

 Nephrotic-range proteinuria is defined as proteinuria > 3.5

g/24 hr. or a urine protein: creatinine ratio > 2

 Affects 1-3 per 100,000 children < 16 Yrs. of age

 Without treatment, in children it is associated with a high

risk of death, most commonly from infections

03/28/2025 7
 Etiology
 Can be primary or secondary
A. Primary (Idiopathic) Nephrotic syndrome
Minimal change disease
Focal segmental glomerulosclerosis
Membranous nephropathy
Glomerulonephritis associated with Nephrotic syndrome
B. Secondary Nephrotic syndrome
Systemic diseases like SLE
Infections (hepatitis, HIV, and malaria)
Genetic disorders
Drugs like captopril, NSAIDs…

03/28/2025 8
 Etiology…
Immunologic or Allergic Disorders
• Snake Biting
• Vasculities syndrome
• Food allergen
Glomerular hyper filtration
• Morbid obesity
• Adaptation to Nephrotic reduction
Associated with malignant disease
• Wilms tumor
• Leukemia
• Lymphoma

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 Pathophysiology
 The underlying abnormality in Nephrotic syndrome is an
increased permeability of the glomerular capillary wall,
which leads massive proteinuria and hypoalbuminemia

 In NS Pathogenesis basically is based on the role of:

I. Immune system

II. Podocyte related factors (like ANGPTL-4)

III. Systemic circulating factors( e.g. suPAR)

IV. Genetic variants

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 Pathophysiology…

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 Clinical manifestation
Tetrads of Nephrotic syndrome
1. Edema
The most common presenting symptom of children
 Despite its almost universal presence, there is uncertainty
as to the exact mechanism of edema formation
There are two opposing theories, the underfill hypothesis
and the overfill hypothesis, proposed as a mechanisms of
Nephrotic edema formation

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 Cont,d…
 Underfill hypothesis is based on Nephrotic-range
proteinuria that leads to a fall in the plasma protein level
with a corresponding decrease in intravascular oncotic
pressure

This leads to leakage of plasma water into the interstitium,

generating edema

Overfill hypothesis postulates that NS is associated with

primary sodium retention, with subsequent volume
expansion and leakage of excess fluid into the interstitium
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 Cont,d…

2. Hyperlipidemia

There are several alterations in the lipid profile in children with

Nephrotic syndrome, including an increase in cholesterol, triglycerides,
low-density lipoproteins, and very-low-density lipoproteins

It is thought to be the result of increased synthesis as well as

decreased catabolism of lipids

In adults, this results in an increase in the adverse cardiovascular risk

ratio, although the implications for children are not as serious,
especially those with steroid-responsive Nephrotic syndrome
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 Cont,d…

3. Proteinuria: With a consequence of two mechanisms:

• The abnormal transglomerular passage of proteins due to

increased permeability of glomerular capillary wall, and

• Subsequent impaired reabsorption by the epithelial cells of

the proximal tubuli

4. Hypoalbuminemia

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 C/manifestation…
Other symptoms of NS includes:

Fatigue, Headache , Malaise, irritability

Haematuria( not common)

Anaemia

Dyspnoea

Foamy urine

lipiduria

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 Diagnosis
History

Physical examination

Urinalysis- 24Hr. urine

Serum chemistry

Serum creatinine

Needle kidney biopsy

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 DDx
• Hepatic: insufficiency, hepatocellular cirrhosis, Budd Chiari
syndrome
• Digestive: exudative enteropathy, lymphangiectasia,
malnutrition
• Cardiac: hereditary angioneurotic edema
• Immune: anaphylaxis
• Renal: Chronic glomerulonephritis
-Diabetic nephropathy
-Focal segmental glomerulosclerosis
-HIV-associated nephropathy
-IgA nephropathy, membranous glomerulonephritis

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 Medical management
General considerations:
 The management of nephrotic syndrome is a long process
with remissions and recurrence of symptoms common
 A detailed assessment is necessary before starting
corticosteroids
 The patient's height, weight, and blood pressure should be
monitored
 Regular weight record helps in monitoring the decrease or
increase of edema
 Physical examination is carried out to detect infections and
underlying systemic disorders
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 Cont,d…
 Corticosteroid therapy: daily induction steroid treatment for
6 weeks, followed by alternate-day maintenance therapy for
another 6 weeks

 Diuretic therapy: beneficial, particularly in children with

symptomatic edema; loop diuretics such
as furosemide (starting at 1-2 mg/kg/d) may improve edema

 Plasma protein: used to supplement diuresis in patients with

edema; it increases oncotic pressure and thereby promotes a
fluid shift from interstitial tissues
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 Cont,d…
 Immunosuppressive agents: Often necessary for children
with frequently relapsing or steroid-dependent Nephrotic
syndrome. Example: cyclophosphamide

 In cases of steroid-resistant nephrotic syndrome, the first-line

choice is calcineurin inhibitors, and if there is no response,
then agents such as mycophenolate mofetil (MMF) or
prolonged and/or intravenous pulse corticosteroids could be
used

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 Cont,d…
 Home monitoring: Home monitoring of urine protein and
fluid status

 Diet: A sodium-restricted diet should be maintained while

a patient is edematous and until proteinuria remits

 Activity: A normal activity plan is recommended

03/28/2025 22
 Nursing management
 Monitoring fluid intake and output

Monitor and document intake and output

Weigh the child at the same time every day, on the same
scale in the same clothing

 Improving nutritional intake

Offer a visually appealing and nutritious diet

 Promoting skin integrity

Inspect all skin surfaces regularly for breakdown

Turn and position the child every 2 hours

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 Cont,d…
 Protect skin surfaces from pressure by means of pillows and
padding

 Promoting energy conservation

Bed rest is common during the edema stage of the condition

Balance the activity with rest periods and encourage the child to
rest when fatigued

 Preventing infection

Protect the child from anyone with an infection

Hand washing and strict medical asepsis are essential

Observe for any early signs of infection

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 Staging

• Remission: Urine albumin nil or trace for three consecutive

early morning specimens

• Relapse: Urine albumin 3+ or 4+ for three consecutive

early morning specimens, having been in remission
previously

• Frequent relapses: Two or more relapses in the initial six

months or more than four relapses in any 12 months

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 Staging…
• Steroid dependence: Two consecutive relapses when on
alternate day steroids or within 14 days of its discontinuation

• Steroid resistance: Absence of remission despite therapy

with daily prednisolone at a dose of 2 mg/kg per day for four
weeks

• Congenital: presenting within the first three months of life,

and in these children, there is usually a genetic mutation

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 Complication
Infection: main causes of death in children with NS

Pneumococcal vaccines against capsular antigens is

recommended for all children with NS

Thromboembolism: Well-known risk factor for arterial or

venous thromboembolism
From loss of proteins involved in the inhibition of
systemic hemostasis, increased synthesis of
prothrombotic factors or by local activation of the
glomerular hemostasis system
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 Complication…
Cardiovascular complications: An increased risk of
cardiovascular disease exists in patients with NS because
of hyperlipidemia, increased thrombogenesis, and
endothelial dysfunction
little or no risk of cardiovascular disease in children with
MCNS who are responsive to CS because hyperlipidemia is
intermittent and of short duration

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 Complication…
 Hypovolemic crisis: Hypovolemic shock is one of the
attentive presentations in NS

Risk factors for hypovolemic crisis include severely

depressed albumin levels, high dose diuretics, and
vomiting

Anemia: Mild anemia is observed on occasion in patients with

NS

Usually microcytic and hypochromic, typical of iron deficiency,

but is resistant to therapy with iron because of large loss of
03/28/2025
serum transferrin in the urine of some nephrotic patients 29
 Complication…
Acute renal failure: is an uncommon but alarming

 When massive proteinuria develops and the levels of

albumin are profoundly decreased, the circulating
volume in the plasma is reduced to produce circulatory
collapse or pre-renal uremia, usually of mild degree

Ascites and pleural effusions: Frequently occurs

 Pericardial effusion is rare unless cardiac function is

abnormal

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 Hemolytic Uremic Syndrome (HUS)

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 Introduction
 Hemolytic uremic syndrome (HUS): Is thrombotic
microangiopathy characterized by the presence of a triad of
symptoms: thrombocytopenia, acute renal impairment, and
microangiopathic hemolytic anemia

 Thrombotic microangiopathy: Is the formation of platelet

microthrombi in walls of small blood vessels (arterioles and
capillaries), causing platelet consumption leading to
thrombocytopenia, nonimmune hemolytic anemia (Coombs
negative), and acute renal injury
03/28/2025 32
 Introduction…
 Although traditionally thought of as a triad, HUS
presentation may vary based on different etiology

 HUS can be a life-threatening condition requiring prompt

diagnosis and treatment

 It is one of the leading causes of acute renal injury in the

pediatric population

03/28/2025 33
 Etiology
 Hemolytic uremic syndrome is part of a spectrum of
diseases associated with thrombotic microangiopathy

 Specifically, HUS has two variants termed typical and

atypical HUS

 The typical variant is caused by Shiga-like toxin (verotoxin)

produced by Escherichia coli (O157: H7) and Shiga toxin
by Shigella dysenteriae

 The atypical form is linked to bacteria, medication, or

immune processes capable of endothelial damage
03/28/2025 34
 Etiology…
 Common drugs associated with HUS include

 Mitomycin C

 Cyclosporine

 Cisplatin

 Cocaine

 Quinine

 Rarely FK506 (tacrolimus) and interferon-alpha

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 Epidemiology
 Most often associated with children under ten years old,
with the majority occurring in those less than 5 years old

 More than 90% of the typical HUS is caused by Shiga toxin-

producing Escherichia coli

 The yearly incidence of typical HUS is 3 cases per 100,000

 About 40% of the atypical HUS is associated

with Streptococcus pneumoniae

 The estimated fatality rate for both atypical and typical HUS
is less than 5%
03/28/2025 36
 Pathophysiology
 Hemolytic uremic syndrome is typically associated with
bacterial infection resulting from the consumption of
undercooked beef or unpasteurized milk

 The Shiga toxin formed by the E. coli in typical HUS is then

absorbed by the villi in the intestines, allowing the toxin to
enter the bloodstream

 The toxin then binds to glycosphingolipid found on multiple

different cells throughout the body

03/28/2025 37
 Pathophysiology…

 The damage that ensues leads to an increase in thrombin

and fibrin levels resulting in microthrombi being laid down

 These microthrombi lead to platelet consumption, causing

thrombocytopenia

 Microthrombi present in the blood vessel also leads to the

mechanical breakdown of red blood cells, causing hemolytic
anemia

 As the red blood cells are hemolyzed, there is a further

increase in platelet use, causing thrombocytopenia
03/28/2025 38
 Pathophysiology…
 The Shiga toxin has a high affinity
for globotriaosylceramide (Gb3) membrane receptor
present in glomerular endothelium and tubular cells, causing
widespread damage resulting in:
 Glomerular necrosis

 Cellular apoptosis, and microangiopathic thrombosis

leading to acute renal injury

03/28/2025 39
 Pathophysiology…
 Atypical HUS is a broad term used for those patients who
develop HUS not associated with a Shiga-toxin illness

 A common etiology is associated with S. pneumoniae that

produces a neuraminidase enzyme

 This enzyme targets N-acetylneuraminic acid found on the

surface of red blood cells

 When the enzyme cleaves N-acetylneuraminic acid, a T-

antigen is exposed, initiating an immune response

03/28/2025 40
 Pathophysiology…
 Another proposed mechanism in atypical HUS :

 Increased expression and binding of pneumococcal surface

proteins with human plasminogen

 leading to bacterial surface plasmin activation and

widespread endothelial damage

 Exposing sub endothelial matrix and subsequent thrombotic

microangiopathy

03/28/2025 41
 Clinical manifestation
 Typical HUS is often associated with gastrointestinal illness

 Thus, patients presenting early in the course of the disease will

complain;
 Fever, Abdominal pain, Nausea, Vomiting, and diarrhea

 Diarrhea is often bloody and is within 3 days of diarrhea onset

 Bloody diarrhea that ensues can be secondary to colitis from

the invasion of the gastrointestinal cells or ischemia related

to a vascular lesion

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 Clinical manifestation…
 After a week of gastrointestinal symptoms, patients may
start to develop symptoms more closely related to the triad
that defines HUS; specifically Symptoms related to:

I. Anemia: (syncope, shortness of breath, and jaundice)

II. Kidney impairment: (oliguria/anuria, hematuria)

 Skin exam may show small ecchymosis and mucosal

bleeding

03/28/2025 43
 Clinical manifestation…
 Late in the course of the infection, patients may develop
seizures and encephalopathy related to ongoing uremia
and other electrolyte derangements

 In rare cases, the colitis can be severe enough to cause

intestinal necrosis and perforation.

03/28/2025 44
 Clinical manifestation…
 In atypical HUS, a gastrointestinal illness is not usually the
initial insult, and a thorough history and physical exam is
required to find the offending agent

 If S. pneumoniae is the source, the patient may site a recent

respiratory illness

 A review of any new medications or chronic autoimmune

diseases is necessary.

 These patients typically have decreased urine output,

pallor, and scattered ecchymosis
03/28/2025 45
 Diagnostic evaluation
 History (symptoms, dietary, travel)

 Physical examination

 CBC

 Urinalysis

 Comprehensive metabolic panel (CMP)- reveals elevated

creatinine consistent with acute kidney injury

03/28/2025 46
 Medical management
 Management of hemolytic uremic syndrome begins with
good supportive care

 Should be admitted to the hospital for further monitoring

 The patients are often fluid depleted and thus benefit from
several fluid boluses (intravenous nutrition)
 It is important to have a careful assessment of the
patient’s overall kidney function so as to avoid giving
too much fluid in the case of kidney failure

03/28/2025 47
 Cont,d…
 Patients should have their anemia corrected with packed
RBCs when hemoglobin reaches 7 to 8 g/dl or hematocrit
less than 18%

 The treatment of thrombocytopenia is often unnecessary,

given the continued consumption during the disease
 Platelet transfusion leads to worsening thrombosis

 Exceptions to this are those with active bleeding or before

a surgical procedure

03/28/2025 48
 Cont,d…
 Some patients (half) will go on to develop oligouric renal
failure (<0.5 ml/kg/hr. x 72 hr.) and require dialysis

 Most experts do not recommend antibiotics and

antiperistaltic agents to treat diarrheal illness
 They believe that this will increase the complications
associated with E. coli infection

 Those with encephalopathy or need for dialysis should be

admitted to an intensive care unit.

03/28/2025 49
 Nursing management???

(Reading assignment)

03/28/2025 50
 DDx
 Thrombotic thrombocytopenic purpura: thrombotic
microangiopathy characterized by a pentad of hemolytic
anemia, thrombocytopenia, renal dysfunction, fever,
and neurological dysfunction

 Disseminated intravascular coagulation (DIC)

 Systemic Vasculities: These patients typically present

with inflammatory signs like fever, rash, and arthralgia, and
lack prodromal diarrhea.

03/28/2025 51
 Prognosis
 Prognosis typically depends on the prompt initiation of
treatment

 Acute complications like acute renal injury, coma, and

death, as well as progression to chronic renal failure, can
be prevented with timely intervention

 Overall mortality from HUS is less than 5%, while long-

term renal complications occur in 5 to 25% of the children
with HUS

03/28/2025 52
 Complications
 long-term chronic kidney disease- Patients who require
dialysis are at high risk

 Abdominal strictures- Those who develops colitis as

evidenced by fever, leukocytosis, and severe abdominal pain

 End-stage renal disease- From atypical HUS

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 Acute kidney injury

03/28/2025 54
 Introduction
AKI is defined as an abrupt loss of kidney function leading
to:
 A rapid decline in the glomerular filtration rate (GFR)

 Accumulation of waste products such as blood urea

nitrogen (BUN) and creatinine
 Dysregulation of extracellular volume and electrolyte
homeostasis

03/28/2025 55
 Introduction
 AKI has largely replaced acute renal failure (ARF),
because the latter designation overemphasizes the discrete
event of a failed kidney

 AKI embodies a continuum of renal dysfunction that ranges

from a small increase in serum creatinine to complete
anuric renal failure

03/28/2025 56
 Epidemiology
 AKI is a common problem afflicting all ages

 leading reason to seek inpatient nephrology consultation,

and associated with serious consequences and
unsatisfactory therapeutic options

The incidence of AKI varies from 2–5% of all

hospitalizations to > 25% in critically ill infants and children

03/28/2025 57
 Etiology
 The etiology of AKI varies widely according to age,
geographic region, and clinical setting

 Etiologically AKI can be classified as:

A. Prerenal AKI( prerenal azotemia)

Characterized by a diminished effective circulating arterial

volume, which leads to inadequate renal perfusion and a
decreased GFR

Evidence of structural kidney damage is absent

03/28/2025 58
 Etiology…
Common causes of prerenal AKI include dehydration,
sepsis, hemorrhage, severe hypoalbuminemia, and
cardiac failure

 If the underlying cause of the renal hypoperfusion is

reversed promptly, renal function returns to normal.

 If hypoperfusion is sustained, intrinsic renal parenchymal

damage can develop

03/28/2025 59
 Etiology…
B. Intrinsic renal AKI

Characterized by renal parenchymal damage

By sustained hypoperfusion and ischemia

Ischemic/hypoxic injury and nephrotoxic insults (most

common)

Glomerulonephritis, Hemolytic-uremic syndrome

Acute tubular necrosis, Renal vein thrombosis

Acute interstitial nephritis , toxin, and drugs

03/28/2025 60
 Etiology…
C. Post renal AKI

Encompasses a variety of disorders characterized by

obstruction of the urinary tract

In neonates and infants, congenital conditions, like

posterior urethral valves and bilateral ureteropelvic
junction obstruction, accounts for the majority of cases

In older children and adolescents, urolithiasis, tumor

(intraabdominal lesion or within the urinary tract),
hemorrhagic cystitis, and neurogenic bladder
03/28/2025 61
 Etiology…
 In a patient with two functioning kidneys, obstruction
must be bilateral to result in AKI

 Relief of the obstruction usually results in recovery of

renal function

03/28/2025 62
 Staging
Three methods to define and stage AKI,

I. Acute Kidney Injury Network ( AKIN) ( Reading

Assignment???)

II. Risk of renal dysfunction, Injury to kidney, Failure or

Loss of kidney function, and End-stage kidney disease
(RIFLE) (Reading Assignment???)

III. Kidney Disease Improving Global Outcomes (KDIGO)

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 Staging…
 Kidney Disease Improving Global Outcomes (KDIGO)
classification system takes both serum creatinine and urine
output criteria to define and stage AKI
Stage Serum creatinine Urine output

1 1.5-1.9 times baseline, OR ≥0.3 mg/dL <0.5 mL/kg/hr. for 6-12

increase hr.

2 2.0-2.9 times baseline <0.5 mL/kg/hr. for ≥ 12

hr.

3 3.0 times baseline, OR SCr ≥ 4.0 mg/dL, <0.3 mL/kg/hr. for ≥ 24

OR Initiation of renal replacement therapy, hr. OR
OR eGFR < 35 mL/min per 1.73 m2 (< 18 Anuria for ≥ 12 hr.
yrs.)

03/28/2025 64
 KDIGO…
 Increase in serum creatinine by ≥ 0.3 mg/dL from
baseline within 48 hr. or

 Increase in serum creatinine to ≥ 1.5 times baseline within

the prior 7 days; or

 Urine volume ≤ 0.5 mL/kg/hr. for 6 hr.

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 Clinical presentation
 Thirst, decreased urine output, dizziness, and orthostatic
hypotension (Prerenal)

 Hematuria, edema, and hypertension (PSGN)

 Rhabdomyolysis (muscular pain, recent coma, seizure,

intoxication, excessive exercise, limb ischemia) or
hemolysis (recent blood transfusion)(intrinsic renal)

 Allergic interstitial nephritis should be suspected with

(fevers, rash, arthralgias)(intrinsic renal)

 Urgency, frequency, and hesitancy (Post renal)

03/28/2025 66
 Clinical presentation…

N.B. Clinical presentation of AKI depends on its cause,

therefore a carefully history taking and a thorough physical
exam are critical in defining the cause of AKI

03/28/2025 67
 Diagnosis
History

P/E

Complete blood count (CBC)

Serum biochemistries

Urine analysis with microscopy

Urine electrolyte

Doppler ultrasonography

Renal Biopsy
03/28/2025 68
 DDx
Some differentials • Heart failure

• Abdominal aneurysm • Metabolic acidosis

• Alcohol toxicity • Gastrointestinal bleeding

• Alcoholic ketoacidosis

• Chronic kidney disease

• Dehydration

• Diabetic ketoacidosis

03/28/2025 69
 Management
Medical

Ensure adequate drainage of the urinary tract through

bladder catheter when urinary tract obstruction and
posterior urethral valve

 Volume resuscitation, if there is no evidence of volume

overload or cardiac failure, the intravascular volume
should be given by intravenous administration of isotonic
saline, 20 mL/kg over 30 min.

03/28/2025 70
 Management…
After volume resuscitation, hypovolemic patients generally
void within 2 hr. failure to do so suggests intrinsic or
postrenal AKI

Diuretic therapy should be considered only after the

adequacy of the circulating blood volume has been
established

Electrolyte correction ( sodium ,potassium, calcium)

Nutrition

Surgery
03/28/2025 71
 Management…
Dialysis

Indications for dialysis in AKI include the following:

 Anuria/oliguria

 Volume overload with evidence of hypertension and/or pulmonary

edema refractory to diuretic therapy

 Persistent hyperkalemia

 Severe metabolic acidosis unresponsive to medical management

 Uremia (encephalopathy, pericarditis, neuropathy)

03/28/2025 72
 Nursing management???

(Classroom group discussion)

03/28/2025 73
 Prognosis
 The mortality rate in children with AKI is variable and
depends entirely on the nature of the underlying disease
process rather than on the renal failure itself

 Children with AKI caused by a renal-limited condition

such as post infectious glomerulonephritis have a very low
mortality rate (5%)

The prognosis for recovery of renal function depends on

the disorder that precipitated AKI

03/28/2025 74
 Prognosis…
 Recovery of renal function is likely after AKI resulting from
prerenal causes, ATN, acute interstitial nephritis, or
tumor lysis syndrome

 Complete recovery of renal function is unusual when AKI is

from:
 Rapidly progressive glomerulonephritis

 Bilateral renal vein thrombosis

 Bilateral cortical necrosis

03/28/2025 75
 Complication
 Most common complications include metabolic
derangements:
 Hyperkalemia

 Metabolic acidosis

 Hypophosphatemia

 Pulmonary edema from volume overload

 Peripheral edema from an inability to excrete body

water

03/28/2025 76
 Complication…
 Cardiovascular - Heart failure secondary to fluid overload

- Arrhythmias secondary to acidotic state

 Gastrointestinal (GI) - Nausea, vomiting, GI bleeding, and

anorexia
 A mildly raised level of amylase is commonly found in
patients suffering from AKI

 Neurologic - CNS-related signs of uremic burden are

common in AKI, and they include lethargy, somnolence,
disturbed sleep-wake cycle, and cognitive impairment
03/28/2025 77
 Renal Tubular Acidosis (RTA)

03/28/2025 78
 Introduction
 lungs and the kidneys are responsible for the
maintenance of acid-base balance within the body

 Alveolar ventilation removes carbon dioxide, while the

kidneys reclaim filtered bicarbonate and excrete
hydrogen ions produced by the metabolism of dietary
protein

03/28/2025 79
 Introduction…
 Renal tubular acidosis: a group of disorders in which
metabolic acidosis develops because of defects in the
ability of the renal tubules to perform the normal functions
required to maintain acid-base balance, despite a
relatively well-preserved glomerular filtration rate

 It is a non-anion gap hyperchloremic metabolic acidosis

03/28/2025 80
 Classification
 There are four subtypes of RTA

I. Type 1: Distal RTA

II. Type 2: Proximal RTA

III. Type 3: Mixed RTA

IV. Type 4: Hyporeninemic hypoaldosteronism RTA

N.B. Type 1 and type 2. are the most common RTA in

neonates and children.
03/28/2025 81
 Distal RTA
Etiology: can be genetic and acquired disorders

Genetic causes — Genetic primary causes of distal RTA

include mutations of genes that directly affect
membrane transport proteins such as the chloride-
bicarbonate exchanger (AE1) or subunits of the H-
ATPase pump and others that indirectly affect intracellular
trafficking

Acquired causes: Medications (lithium), Autoimmune

disorders (SLE), Obstructive uropathy
03/28/2025 82
 Distal RTA…
Pathogenesis

Type 1 (distal) RTA is due to impaired distal acid secretion

that results in an inability to excrete the daily acid load

In the absence of alkali therapy, progressive hydrogen ion

retention leads to a fall in plasma bicarbonate
concentration that is accompanied by an abnormally high
urine pH (greater than 5.5)

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 Distal RTA…
Clinical manifestations
Severe hyperchloremic metabolic acidosis (serum
bicarbonate levels may decrease below 10 mEq/L)
Moderate to severe hypokalemia (serum potassium ≤
3.0 mEq/L)
Nephrocalcinosis

Vomiting

Dehydration, Poor growth, and Rickets

03/28/2025 84
 Distal RTA…

Management

 Alkali therapy is required in patients with distal (type 1) RTA

 Correction of the metabolic acidosis has a number of benefits

Effects on bone – Correction of the acidosis restores normal

growth rates in children

Effects on urinary citrate excretion, nephrolithiasis, and

Nephrocalcinosis – Alkali therapy can also reverse
hypercalciuria, reduce the rate of kidney stone formation, and
prevent or ameliorate Nephrocalcinosis in many patients

03/28/2025 85
 Distal RTA…
Some alkali therapy
 Sodium bicarbonate

 Liquid sodium or potassium citrate

 Sodium bicarbonate tablets

03/28/2025 86
 Distal RTA…
Management

Effects on potassium wasting – Correction of the

metabolic acidosis with alkali therapy reduces inappropriate
urinary potassium losses, which often corrects the
associated hypokalemia

Effects on kidney function – Chronic kidney disease

(CKD) occurs frequently in patients with inherited forms of
distal RTA, and successful correction of the acidosis may
prevent loss of kidney function
03/28/2025 87
 Distal RTA…
Complication

Chronic kidney disease: with a glomerular filtration rate

of <90 cc/min per 1.73 m2, has been reported as a
complication of hereditary distal RTA
 CKD presents after the pubertal growth spurt and is
thought to be due to the combination of
Nephrocalcinosis, persistent hypokalemia, and
repeated episodes of hypovolemia that results in
progressive tubulointerstitial injury
03/28/2025 88
 Proximal (type 2) RTA
Pathogenesis

 Proximal RTA is caused by a reduction in proximal

bicarbonate reabsorptive capacity resulting in a fall in
the plasma bicarbonate

 Depending on the renal bicarbonate excretion threshold

and serum bicarbonate level, urine pH may be
inappropriately high or normal

03/28/2025 89
 Proximal (type 2) RTA…
Etiology

 Proximal RTA may present either as an isolated tubular

defect or as a component of a generalized proximal
tubular disorder called the Fanconi syndrome

 Isolated proximal renal tubular acidosis — In children,

isolated proximal RTA is less common
 It is usually due to a transient or inherited

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 Proximal (type 2) RTA…
 Fanconi syndrome — Generalized proximal tubular
dysfunction, referred to as Fanconi syndrome, is
characterized by proximal RTA, phosphaturia, renal
glycosuria (with a normal plasma glucose concentration),
aminoaciduria, and tubular proteinuria

Etiology
 Cystinosis

 Tyrosinemia type 1

 Galactosemia
03/28/2025 91
 Proximal (type 2) RTA…
Clinical manifestation

 Growth failure, and episodes of hypovolemia due to

polyuria caused by impaired concentrating ability
Poor growth may be due to hypophosphatemia,
persistent acidosis, and chronic hypokalemia

 Rickets and osteomalacia due to hypophosphatemia and

low levels of calcitriol (1,25 dihydroxy vitamin D)

 Constipation and muscle weakness caused by significant

hypokalemia
03/28/2025 92
 Proximal (type 2) RTA…
Management

Patients with Fanconi syndrome

 Alkali therapy

 Treatment of the metabolic acidosis is more difficult in

proximal RTA than in distal RTA

 Alkali doses in proximal RTA are higher

 Correcting vitamin D deficiency and hypophosphatemia

03/28/2025 93
 Proximal (type 2) RTA…
Patients with isolated proximal RTA

 Alkali therapy is directed to correcting metabolic acidosis

and hypokalemia

Isolated proximal RTA is not associated with

hypophosphatemia and vitamin D deficiency, and therefore
there is no need to administer phosphate or vitamin D
supplement

03/28/2025 94
 Reading Assignment

1. Mixed RTA???

2. Hypoaldosteronism ???

03/28/2025 95
 Urinary Tract Infection (UTI)

03/28/2025 96
 Introduction
 Urinary tract infection (UTI) is a common and important
clinical problem in childhood

 Upper UTIs (i.e. acute pyelonephritis) may lead to renal

scarring, hypertension, and end-stage kidney disease

 Although children with pyelonephritis tend to present with

fever, it is often difficult on clinical grounds to distinguish
cystitis from pyelonephritis, particularly in young children
(those younger than two years)

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 Epidemiology
 Prevalence — Awareness of the prevalence of UTI in
various subgroups of children enables the clinician to
grossly estimate the probability of infection in the patient

 In young children with fever — The prevalence of UTI in

children <2 years presenting with fever has been the subject
of several large prospective studies and a meta-analysis

 The overall prevalence of UTI is approximately 7 percent in

febrile infants and young children but varies by age, sex,
and circumcision status
03/28/2025 98
 Epidemiology…
 The prevalence is highest among uncircumcised males,
particularly those who are younger than three months

 Females have a two- to- fourfold higher prevalence of UTI

than do circumcised males

In older children — In pooled analysis of four studies that

included children <19 years (most of whom were older than
two years) and had urinary symptoms and/or fever, the
prevalence of UTI was 7.8 percent (95% CI 6.6-8.9)

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 Etiology
A. Escherichia coli is the most common bacterial cause of
UTI; it accounts for approximately 80 % of UTI in children

B. Gram-negative bacterial pathogens include

 Klebsiella, Proteus

 Enterobacter, and Citrobacter

C. Gram-positive bacterial pathogens include

 Staphylococcus saprophyticus

 Enterococcus, and

 Rarely, Staphylococcus aureus

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 Etiology…
D. Viruses: are usually limited to the lower urinary tract.
 Adenovirus, Enteroviruses, Coxsackievirus, and
Echoviruses

E. Fungi: Risk factors for fungal UTI include

immunosuppression, long-term use of broad-spectrum
antibiotic therapy, and indwelling urinary catheter
 Candida spp., Aspergillus spp., Cryptococcus
neoformans
 Endemic mycoses

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 Pathogenesis
 Bacteriology of UTI, along with the observation that a
minority (4 to 9 % of children with UTI are bacteremic, is
consistent with the hypothesis that most UTI beyond the
newborn period are the result of ascending infection

 Colonization of the periurethral area by uropathogenic

enteric pathogens is the first step in the development of a
UTI

 The presence of pathogens on the periurethral mucosa,

however, is not sufficient to cause UTI
03/28/2025 102
 Pathogenesis…
 Pathogens attach to the uroepithelial cells via an active
process mediated by glycosphingolipid receptors on the
surface of epithelial cells

 Bacterial attachment recruits toll-like receptors (TLR), a

family of trans membrane coreceptors involved in the
recognition of pathogen-associated protein patterns

 TLR binding triggers a cytokine response, which

generates a local inflammatory response

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 Host risk factor
 A variety of host factors influence the predisposition to
UTI in children

 Age: The prevalence of UTI is highest in males younger

than one year and females younger than four year

 Lack of circumcision: Uncircumcised male infants with

fever have a four- to eightfold higher prevalence of UTI
than circumcised male infants

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 Host risk factor…
 Female infants: Female infants have a two- fourfold higher
prevalence of UTI than male infants
It is presumed to be the result of the shorter female urethra

 Genetic factors: First-degree relatives of children with UTI are

more likely to have UTI than individuals without such a history
 Adherence of bacteria may, in part, be genetically
determined

Example: females uroepithelial cells are nonsecretors of blood

group antigens that have enhanced adherence of
uropathogenic E. coli
03/28/2025 105
 Host risk factor…
 Urinary obstruction — Children with obstructive urologic
abnormalities are at increased risk of developing UTI
Stagnant urine is an excellent culture medium for most
uropathogens

E.g.- Anatomic conditions (like posterior urethral valves)

• Neurologic conditions ( myelomeningocele with
neurogenic bladder
• Functional conditions (like bladder and bowel
dysfunction)
03/28/2025 106
 Host risk factor…
 Bladder catheterization: The risk of UTI increases with
increasing duration of bladder catheterization

 Vesicoureteral reflux: VUR is the retrograde passage of

urine from the bladder into the upper urinary tract
 It is the most common urologic anomaly in children

 Children with VUR are at increased risk for recurrent

UTI

03/28/2025 107
 Clinical manifestations
In neonate

 The symptoms and signs are nonspecific

 A neonate might present with signs of sepsis, like

temperature instability, peripheral circulatory failure,
lethargy, irritability, apnea, seizure, or metabolic
acidosis

 Alternatively, a neonate might present with anorexia,

poor sucking, vomiting, suboptimal weight gain, or
prolonged jaundice
03/28/2025 108
 Clinical manifestation…
In neonate

 Foul-smelling urine is an uncommon, but more specific

symptom of UTI

 Septic shock is unusual unless the patient is compromised

or obstruction is present

 In neonates with UTI, there is a high probability of

bacteremia, suggesting hematogenous spread of the
bacteria

03/28/2025 109
 Clinical manifestation…
In infancy: symptoms of UTI usually remain nonspecific

 Unexplained fever is the most common during the 1st two

Yrs.

 Non specific symptoms: Irritability, poor feeding, anorexia,

vomiting, recurrent abdominal pain, and failure to thrive

 Specific symptoms and signs: increased or decreased

number of wet diapers, malodorous urine, and discomfort
with urination

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 Clinical manifestation…
After the second year of life

 Symptoms and signs of UTI are more specific

 Pyelonephritis symptoms and signs: fever, chills, rigor,

vomiting, malaise, flank pain, back pain, and costovertebral
angle tenderness

 Lower tract symptoms and signs: suprapubic pain,

abdominal pain, dysuria, urinary frequency, urgency, cloudy
urine, malodorous urine, daytime wetting, nocturnal
enuresis of recent onset, and suprapubic tenderness
03/28/2025 111
 Diagnosis
 History & Physical examination

 Laboratory investigation

A urinalysis and urine culture

Quantitative urine culture is the gold standard

 Imaging

Renal and bladder ultrasonography : to visualize the

urinary tract
Cystoscopy: VUR, Urethral obstruction

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 Diagnostic criteria
 According to the American Academy of Pediatrics (AAP)
clinical practice guidelines

Example

 The diagnosis of UTI in children 2 to 24 months requires:

Positive dipstick test (leukocyte esterase and/or

nitrite test)
Microscopy positive for pyuria or bacteriuria, and

The presence of ≥ 50,000cfu/ml of a uropathogen in

a catheterized or suprapubic aspiration specimen
03/28/2025 113
 Diagnostic criteria…
 Clinical judgment is important:

 As UTI can occur, though rarely, in the absence of

pyuria and that urine culture can be negative in
children with UTI, if there is ureteric obstruction
preventing discharge of bacteria from the kidney to the
bladder

03/28/2025 114
 DDx
 Viral infection  Appendicitis

 Post-vaccination fever  Group A streptococcal

 Urinary calculi infection

 Vaginal foreign body  In the adolescent female,

pelvic infection
 Orchitis

 Kawasaki disease

03/28/2025 115
 Treatment
Goals:

 Prompt diagnosis of other systemic infections

 Prevention of progressive renal disease

 Identification of urologic abnormalities

 Resolution of acute symptoms

03/28/2025 116
 Treatment
 Prompt antibiotic therapy is indicated for symptomatic
UTI based on clinical findings and positive urinalysis while
waiting for the culture results to eradicate the infection
and improve clinical outcome
The empirical antibiotic chosen should provide
adequate coverage for Gram-negative rods notably E.
coli and Gram-positive Cocci

03/28/2025 117
 Treatment…
The ideal antibiotic should be:
• Easy to administer

• Achieve a high concentration in the urine

• Have minimal or no effect on the fecal or vaginal flora

• Have a low incidence of bacterial resistance

• Have minimal or no toxicity, and have a low cost

03/28/2025 118
 Treatment…
 Empiric antimicrobials that have been used in the
treatment of acute uncomplicated UTI in children
Cephalosporins: cefixime, cefuroxime, and cefprozil

Fluoroquinolones: ciprofloxacin, Norfloxacin

Penicillin: Ampicillin , Amoxicillin , and amoxicillin-

clavulanate

 The choice of antibiotics should take into consideration

local data of antibiotic resistance patterns

03/28/2025 119
 Treatment…
 Adjunctive therapies

 Phenazopyridine hydrochloride (Pyridium) : for

symptomatic treatment of severe dysuria in adolescents

 Recommended dose is 4mg/kg TID for up to 2 days

 Phenazopyridine when excreted into the urine, has a

local analgesic effect

 It is often used to alleviate the pain, irritation, discomfort,

or urgency caused by UTI

Turns the urine dark orange to bright red

03/28/2025 120
 Complications
 Permanent renal damage from recurrent UTI

 Risk factor for recurrent abdominal pain in infancy

 Renal insufficiency

 Electrolyte and acid-base disturbance

 Renal scarring (5% of girls and 13% of boys after their

first symptomatic episode of pyelonephritis)

 Very rare complications in post antibiotic era

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 Acute nephritis

03/28/2025 122
 Introduction

 Acute nephritis occurs when kidneys suddenly

become inflamed
 Has several causes, and it can ultimately lead to
kidney failure if it’s left untreated
 Previously, used to be known as Bright’s
disease

03/28/2025 123
 Definition

 It is acute inflammation of the kidney

 Involves the:
 Glomeruli

 Interstitium, and

 Renal parenchyma

03/28/2025 124
 Risk factors
 The risk factors for acute nephritis include:

 Family history of kidney disease and infection

 Having an immune system disease, such as lupus

 Taking too many antibiotics or pain medications

 Recent surgery of the urinary tract

03/28/2025 125
 Classification
 Based on the anatomical part involvement of the kidney:

A. Acute Interstitial nephritis

B. Acute Pyelonephritis

C. Acute Glomerulonephritis

03/28/2025 126
 A. Interstitial nephritis
 It is a diffuse or focal inflammation and edema of the renal
interstitium and secondary involvement of the tubules

Epidemiology

 Among the biopsies performed specifically for AKI

evaluation, the incidence of AIN was higher, ranging from
6.5% to 35%

 Drug-induced AIN accounts two-thirds of all cases of AIN

03/28/2025 127
 Interstitial nephritis…
Causes

 Acute interstitial nephritis is associated with medications,

infections, systemic diseases, and idiopathic

Drugs - are the most common

Antibiotics( e.g. penicillin , cephalosporin

Nonsteroidal anti-inflammatory drugs

Proton pump inhibitors (PPIs)

5-aminosalicylates, rifampin, allopurinol, and acyclovir are

other common drugs that can cause AIN
03/28/2025 128
 Interstitial nephritis…
Infections
Bacteria- E. coli

Viruses- Human immunodeficiency virus (HIV)

Spirochetes- Leptospirosis, syphilis

Systemic disorders
Sarcoidosis

Sjogren’s syndrome

IgG-4 related systemic disease, and systemic lupus

erythematous (SLE)
03/28/2025 129
 Interstitial nephritis…
Pathophysiology

Depends on cause

 The immune mechanism mediates the pathogenesis of

drug-induced AIN

 Drugs act as haptens that bind to the cytoplasmic or

extracellular components of tubular cells during secretion
and generate a host immune response

 In some patients' serum, IgE levels are elevated,

suggesting a type -1 hypersensitivity reaction
03/28/2025 130
 Interstitial nephritis…
 While in other cases, the latent period between drug
exposure and development of a rash, eosinophilia, and
presence of positive skin tests to drugs suggests a T-cell
mediated type-IV hypersensitivity reaction

03/28/2025 131
 B. Pyelonephritis
֎ Acute pyelonephritis (AP) is an infection involving the
renal parenchyma, which is generally associated with
systemic signs of inflammation

֎ In the majority of cases, the infection starts within the

bladder and then migrates up the ureters and into the
kidneys

֎ Ascending infection

03/28/2025 132
 Pyelonephritis…
Causes

 Usually due to a bacterial infection(e.g. E. coli)

 Urinary examinations that use a cystoscope

 Surgery of the bladder, kidneys, or ureters

 The formation of kidney stones, and other waste

material

03/28/2025 133
 Pyelonephritis…
Pathophysiology

 The kidneys and urinary tract are usually germ free

 Mostly result from the ascension of fecally derived

organisms through the urethra and periurethral tissues into
the bladder, with subsequent invasion of the kidney

 Usually, urine flow prevents infection, washing out the

bacteria penetrating into the urinary tract

03/28/2025 134
 Pyelonephritis…
 When bacteria colonize the urinary tract, some children

will develop asymptomatic bacteriuria or lower urinary

tract infections (LUTIs), while only a minority will

experience AP, with systemic symptoms secondary to

immune system activation

03/28/2025 135
 C. Glomerulonephritis
Acute nephritis in which an immunologic mechanism
triggers inflammation and proliferation of glomerular
tissue that can result in damage to the basement
membrane, mesangium, or capillary endothelium

Causes

 The causal factors that underlie acute GN can be

broadly divided into infectious and noninfectious
groups

03/28/2025 136
 Glomerulonephritis…
Infectious

 The most common infectious cause of acute GN is

infection by Streptococcus species (i.e. group A, beta-
hemolytic)

Noninfectious

 Noninfectious causes of acute GN may be divided into

primary renal diseases, systemic diseases, and
miscellaneous conditions or agents

03/28/2025 137
 Glomerulonephritis…
Pathophysiology

 Involves both structural and functional changes

 Structurally, cellular proliferation leads to an increase in

the number of cells in the glomerular tuft

 The proliferation may be

Endocapillary (i.e., within glomerular capillary tufts)

Extracapillary (i.e. in the Bowman space involving the

epithelial cells)

03/28/2025 138
 Glomerulonephritis…
 In extracapillary proliferation, proliferation of parietal
epithelial cells leads to the formation of crescents, a
feature characteristic of certain forms of rapidly
progressive GN

 Glomerular basement membrane thickening appears as

thickening of capillary walls on light microscopy

 Hyalinization or sclerosis indicates irreversible injury

 These structural changes can be focal, diffuse or

segmental, or global
03/28/2025 139
 Glomerulonephritis…
 Functionally, include proteinuria, hematuria,
reduction in GFR (i.e. oliguria or anuria), and active
urine sediment with RBCs and RBC casts
 The decreased GFR and avid distal nephron salt and
water retention result in expansion of intravascular
volume, edema, and, frequently, systemic
hypertension

03/28/2025 140
 Clinical manifestation
• Though, clinical manifestation depends on the type of
acute nephritis, the most common S/S are:

• Pelvic pain, Dysuria, Urgency, and frequency

• Cloudy urine, blood or pus in the urine

• Flank pain

• Edema , commonly in the face, legs, and feet

• Vomiting, Fever

• High blood pressure

03/28/2025 141
 Diagnosis
 History

 Physical Examination

 Laboratory Evaluation of Renal Function

Serum Analysis(e.g. Creatinine)

Urinalysis

 Radiographic Evaluation

 Renal Biopsy

03/28/2025 142
 Management
 Treatment for acute nephritis may require treating the
underlying conditions causing the problems

Medication

 If the infection is very serious, may require intravenous

(IV) antibiotics within the hospital inpatient setting

 pyelonephritis can cause severe pain, anti-pain may be

administered

 If kidneys are very inflamed, corticosteroids

03/28/2025 143
 Management…
Dialysis

 If kidney function is significantly impaired, may require

dialysis

 May be a temporary necessity

 If too much damage, may need dialysis permanently

Home care

 Bed rest, body needs time and energy to heal & Increase
fluid intake

03/28/2025 144
 Nursing management
 Monitor intake and output: Fluid intake and urinary
output should be carefully monitored and recorded

 Monitor BP: Blood pressure should be monitored

regularly using the same arm and a properly fitting cuff

 Monitor urine characteristics: The urine must be

tested regularly for protein and hematuria using dipstick
tests

 Bed rest should be maintained until acute symptoms

and gross hematuria disappear
03/28/2025 145
 Complication
 All three types of acute nephritis will improve with

immediate treatment

 However, if the condition goes untreated, will develop

kidney failure

03/28/2025 146
 Prevention
 Early treatment of a strep. infection with a sore throat or
impetigo

 Avoid too much use of drugs

 Have a proper dietary habit

 Early treatment of possible underlying causes

03/28/2025 147
 Renal stones
(Nephrolithiasis)

03/28/2025 148
 Definition

 Kidney stone is a solid, pebble-like piece of

material that can form in a child’s kidney when

minerals in the urine are too high

03/28/2025 149
 Epidemiology
 Kidney stones are increasingly recognized in children

 Frequency of urolithiasis in children has not been studied

in a systematic population-based fashion

 Institutional and case reports indicate regional variation

 A significant increase in the number of children

diagnosed with and treated for urolithiasis has occurred in
the past decade

03/28/2025 150
 Epidemiology…
 Stones are more common in certain areas

 In Europe, kidney stones occur in 1-2 children per

million population per year

 In underdeveloped countries, children more frequently

have endemic bladder stones than renal stones

 Endemic bladder calculi are common in developing

countries where dietary protein is mostly derived from
plant sources rather than meat

03/28/2025 151
 Epidemiology…
 Age: The incidence of kidney stones is lower in children
than in adults, and the incidence increases with age, with
adolescents having the highest risk of kidney stones

 Sex: Boys were more commonly affected in the first

decade of life and girls in the second decade

 Geography: Research Database reported that the risk of

kidney stones was greater in children who lived in urban
areas

03/28/2025 152
 Risk factors
A. Metabolic risk factors

B. Infection

C. Congenital/structural abnormalities

03/28/2025 153
 Risk factors
A. Metabolic risk factors

Pathogenesis: two mechanisms by which metabolic

factors enhance stone formation

1. Solute excess: High urinary concentrations of calcium,

oxalate, uric acid, and cystine due to increased renal
excretion and/or low urine volume cause solute excess
 This leads to solute supersaturation and
precipitation, and results in the formation of crystals,
which may aggregate into a stone
03/28/2025 154
 Risk factors…
2. Decreased levels of inhibitors of stone formation

 Natural inhibitors of urinary stone formation include

citrate, magnesium, and pyrophosphate
 Low levels of these inhibitors, particularly
hypocitraturia, are associated with kidney stones in
both adults and children

 Common metabolic risk factors include low urine

flow rate, hypercalciuria , and hypocitraturia

03/28/2025 155
03/28/2025 156
 Hypercalciuria
 Hypercalciuria is defined as urinary calcium excretion
rate that is greater than 4 mg/kg per 24 hours in a child
greater than two years of age who is ingesting a routine
diet
 The most common metabolic abnormality associated
with pediatric stone disease
 Identified as the major contributing factor in at least
half of the children with a metabolic cause for kidney
stones

03/28/2025 157
 Hypercalciuria…

 Hypercalciuria may also cause Nephrocalcinosis,

a condition in which calcium salts precipitate out

of solution within the kidney and urologic system

 Hematuria, dysuria, and urinary frequency

can be seen in children with hypercalciuria

03/28/2025 158
 Hyperoxaluria and oxalosis
 Hyperoxaluria is defined as a urinary oxalate

excretion rate greater than 0.7 mmol (62

mg)/1.73 m) per 24 hours

 In case series of pediatric kidney stone

disease, hyperoxaluria was detected in 10 to 20

% of children
03/28/2025 159
 Hyperoxaluria …
Etiology

Primary hyperoxaluria: By rare autosomal disorders

affecting genes that encode enzymes involved in
glyoxylate metabolism
 These disorders are characterized by enhanced
conversion of glyoxalate to poorly soluble oxalate,
resulting in increased serum oxalate and
hyperoxaluria, which can lead to kidney stones

03/28/2025 160
 Hyperoxaluria …
 Fat malabsorption (i. e, enteric hyperoxaluria) –
Children with fat malabsorption may have an enhanced
enteric absorption of oxalate known as enteric
hyperoxaluria
 Excess fatty acid binds calcium, leaving less available
calcium to combine with oxalate, and thus more free
oxalate is absorbed

03/28/2025 161
 Hyperuricosuria

 Hyperuricosuria (HU), defined as an increased

urinary acid excretion

Thresholds for Hyperuricosuria depend on age, sex

 Hyperuricosuria is detected in 2 to 8 % of children

with kidney stones

03/28/2025 162
 Hyperuricosuria…
Etiology

 Increased urinary excretion of uric acid can result

from either enhanced renal excretion or increased
production of uric acid

 Idiopathic Hyperuricosuria is thought to be due to a

defect in renal tubular uric acid excretion and is
often seen in conjunction with hypercalciuria

03/28/2025 163
 Cystinuria
 Cystinuria is characterized by excessive urinary
excretion of the basic amino acids cystine, ornithine,
lysine, and arginine

 Cystine stones account for 5% of pediatric kidney

stone disease and are caused by Cystinuria, an
autosomal recessive disorder of renal tubular
transport

03/28/2025 164
 Hypocitraturia
 Citrate is an inhibitor of calcium oxalate and
calcium phosphate crystallization

 Hypocitraturia has also been reported in up to 68

percent of children with kidney stones

 In children, hypocitraturia is defined as a urinary

citrate excretion rate that is less than 400 mg/g of
creatinine in a 24 hour urine collection

03/28/2025 165
 B. Infection

 In 20 to 25 % of children with kidney stones,

urinary tract infection (UTI) is detected or there is a

history of a UTI

 Infection may be the primary cause of a stone or

occur concomitantly with a underlying urinary

metabolic abnormality or structural abnormality

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 C. Congenital/structural abnormalities
 In case series of children with kidney stones, structural
abnormalities are reported in 10 to 25 % of patients

 Congenital and structural abnormalities that are

accompanied by urinary stasis are associated with kidney
stones

 Urinary stasis predisposes to crystal and stone formation

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 Congenital…
 Kidney and urinary tract abnormalities associated with
urinary stasis and kidney stones include:
Ureteropelvic junction (UPJ) obstruction

Autosomal dominant polycystic kidney disease

Medullary sponge disease

Horseshoe kidney

Bladder exstrophy ( rare)

Augmentation of the bladder, and Neurogenic bladder

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 Stone composition
 In general, stone composition varies based on age and
sex in both adults and children

 Based upon case series, the frequency of different

stone composition in children is:

● Calcium oxalate – 45 to 65 %

● Calcium phosphate – 14 to 30%

● Struvite – 13 %, Cystine – 5 %

● Uric acid – 4 %, and Mixed or miscellaneous – 4%

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 Clinical manifestation
 Pain: Abdominal or flank pain (referred to as renal colic)

In 50 to 75 percent of patients, pain was the

presenting complaint

 Hematuria: Hematuria can present as the sole symptom

of kidney stones or concomitantly with abdominal pain
In pediatric case series, gross hematuria as a
presenting symptom for nephrolithiasis varied from 30
to 55 percent

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 Clinical manifestation…
Dysuria and urgency — Approximately 10 percent of
children with nephrolithiasis present with symptoms of
dysuria and urgency suggestive of a urinary tract infection

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 Diagnosis
֎ History

֎ Physical examination
An abdominal examination for tenderness or mass

Blood pressure measurement and assessment for

edema
Growth measurements, as poor weight gain and/or
failure to thrive may be an indication of a congenital or
chronic condition that may be associated with
nephrolithiasis, like renal tubular acidosis
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 Diagnosis…
֎ Laboratory evaluation
 Urinalysis

 Urine culture

 Serum creatinine

֎ Imaging

• Non-contrast helical computed tomography (CT)

• Ultrasonography

• Plain abdominal radiography

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 Management
Medical
 Supportive care — Supportive management includes
symptomatic treatment and hydration

 Pain control — Nonsteroidal anti-inflammatory drugs

(NSAIDs) and opioid therapy

 Management of urinary tract infection — Because

urinary tract infection (UTI) may be a concomitant finding
in children with nephrolithiasis

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 Management …
 Monitor stone passage: Ultrasonography and a single
kidney-ureter-bladder (KUB) radiograph (if the stone is
radiopaque)
 Although computed tomography (CT) is the most
sensitive imaging modality in the detection of renal or
urinary tract stones, it is more costly and is
associated with higher radiation exposure

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 Management …
 Medical expulsive therapy: medical interventions like
antispasmodic agents, calcium channel blockers, and
alpha blockers have been used to increase the passage
rate of ureteral stones
 Collectively, these treatments are known as "medical
expulsive therapy
 Minimal effect in pediatric patient

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 Management …
 Urine alkalization: In patients with uric acid stones, it
increases the solubility of uric acid and may result in a
decrease in stone size with subsequent passage

 Stone retrieval: The family/patient should be instructed

to strain the child's urine for several days, in order to
retrieve the stone
If the stone or any fragment is recovered, it should be
sent for stone analysis.

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 Management …
Surgical/ urologic intervention

1. Indications for surgical intervention without a trial of

medical observation

 Urinary obstruction: Complete obstruction from renal

calculi can result in renal parenchymal injury and a
decrease in kidney function

 Urinary tract infection: Urinary tract infection in the

setting of a partial or completely obstructing ureteral
calculus is an indication for decompression
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 Management …
Symptomatic stones with unremitting severe pain

 Severe pain despite adequate analgesia is most often

due to uretero-pelvic junction (UPJ) stone, which is
accompanied by obstruction

2. Symptomatic stones that fail to pass after a trial of

conservative therapy
 Surgical intervention is performed if there is no
improvement after a trial of medical therapy for two to
four weeks
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 Reading Assignment

Polycystic kidney disease (PKD)???

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Thank
you!