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NEPHROTIC AND

NEPHRITIC
SYNDROME IN
PAEDS
NEPHROTIC SYNDROME
WHAT IS NEPHROTIC SYNDROME?
■ Increased permeability of the glomerulus leading to loss of proteins into the tubules
■ It is a clinical syndrome of massive proteinuria defined by
– Oedema
– Proteinuria > 40mg/m2/hour (>1g/m2/day)of an early morning urine protein
creatinine index of >200mg/mmol (3.5 mg/mg).
– Hypoalbuminaemia of <25g/l
– Hypercholesterolaemia
AETIOLOGY

■ Primary or idiopathic (of unknown cause) - the commonest type in children


■ Secondary causes – post streptococcal glomerulonephritis and systemic lupus
erythematosus (SLE)
SIGN AND SYMPTOMS
■ Pitting edema is the presenting symptom in about 95% of children with nephrotic
syndrome.
■ It is typically found in the lower extremities, face and periorbital regions, scrotum or labia,
and abdomen (ascites).
■ Other signs and symptoms of nephrotic syndrome may include the following:
• Respiratory tract infection - A history of a respiratory tract infection immediately preceding the
onset of nephrotic syndrome is frequent
• Allergy - Approximately 30% of children with nephrotic syndrome have a history of allergy [1]
• Macrohematuria
• Symptoms of infection - Such as fever, lethargy, irritability, or abdominal pain due to sepsis or
peritonitis
• Hypotension and signs of shock - Can be present in children presenting with sepsis
• Respiratory distress - Due to either massive ascites and thoracic compression or frank
pulmonary edema, effusions, or bot
• Tachypnea - To compensate for mechanical restriction to breathing
• Seizure - Due to cerebral thrombosis
• Anorexia
• Irritability
• Fatigue
• Abdominal discomfort
• Diarrhea
• Hypertension
Investigation
■ Full blood count
■ Renal profile – urea, electrolyte, creatinine
■ Serum cholestrol
■ Liver function test – particularly serum albumin
■ Urinalysis, urine culture
■ Quantitative urinary protein excretion (spot urine protein: creatinine ratio or 24 hour
urine protein)
Other investigations would depend on the age of patient, associated renal impairment,
hematuria, hypertension or features to suggest an underlying secondary causes for the
nephrotic syndrome
These tests include:
■ Anti-nuclear factor/ anti dsDNA to exclude SLE
■ Serum complement (C3,C4) levels to exclude SLE, post- infectiuous
glomerulonephritis
■ ASOT titres to exclude Post-streptococcal glomerulonephritis
Renal biopsy
MANAGEMENT
■ Confirm that patient has nephrotic syndrome by ensuring that the patient fulfills the
criteria above
■ Exclude other causes of nephrotic syndrome. If none, than the child probably has
idiopathic nephrotic syndrome.
General management
■ A normal protein diet with adequate calories reommended
■ No added salt to the diet when child has oedema
■ Penicillin V 125mg BD(1-5 years age), 250mg BD (6-12 years), 500mg BD(>12
years) is recommended at diagnosis and during relapses , particularly in the
presence of gross oedema.
■ Careful assesment of the hemodynamic status
check for sign and symptoms which may indicate
- Hypovolaemia: abdominal pain, cold peripheries, poor capillary refill, poor pulse volume
with or without low blood pressure
- Hypervolaemia: Basal lung crepitation, rhonchi, hepatomegaly, hypertension
Fluid restriction – not recommended except in chronic oedematous state.
■ Diuretics (e.g Furosemide) is not necessary in steroid responsive nephrotic syndrome but
if required, use with caution as will precipitate hypovolaemia.
■ Human Albumin (20-25%) at 0.5-1.0 g/kg can be used in symptomatic grossly
oedematous states together with IV Furosemide at 1-2 mg/kg to produce diuresis.
ALGORITHM
MANAGEMENT COMPLICATION OF
NEPHROTIC SYNDROME
HYPOVOLAEMIA
-
NEPHRITIC SYNDROME

■ NEPHRITIC SYNDROME