NEPHROTIC SYNDROME

Disorder characterised by loss of protein in the urine due to damage of the kidney. It is common in children.

Causes

  • Idiopathic/unknown (majority of cases)
  • Congenital (rare)
  • Secondary: Due to post-streptococcal acute glomerulonephritis, malaria, allergy, UTI, hepatitis B, HIV

Clinical features

  • Generalised oedema
  • Severe loss of protein in urine (proteinuria)
  • Low protein (albumin) levels in the blood serum (hypoalbuminaemia)
  • Hyperlipidaemia (high blood cholesterol)

Investigations

  • As for Acute glomerulonephritis plus
  • 24 hour urine protein quantification or Albumin creatinine ratio (ACR)
  • Serum protein and cholesterol

Differential diagnosis

  • Cardiac failure, liver disease
  • Malnutrition with oedema e.g. kwashiorkor
  • Malabsorption syndrome
  • Allergic states causing generalised body swelling
  • Chronic glomerulonephritis

Management<

  • Restrict salt intake (<2 g daily, i.e. less than a half teaspoon/day)
  • Restrict water/fluid intake
    • Both salt and water/fluid intake should be moderated until diuresis is induced and swelling
      is subsiding, which can take several weeks
  • Furosemide 40-80 mg each morning to induce diuresis
    Child: 1-2 mg/kg per dose (but see notes below)
  • Prednisolone 2 mg/kg daily (max: 60 mg)
    • Continue until no further proteinuria (around 6 weeks)
    • Gradually reduce the dose after the first 4 weeks, e.g. reduce by 0.5 mg/kg per day each week

When oedema has subsided and if still hypertensive

  • Give appropriate treatment

If clinical signs of/suspected streptococcal infection:

  • Give antibiotic as in Acute glomerulonephritis

If patient from area of endemic schistosomiasis

  • Praziquantel 40 mg/kg single dose

If no improvement after 4 weeks or patient relapses

  • Refer for further management