Nephrotic Syndrome

• Urinary protein >3.5 g / 24 hour

• leads to hypoalbuminaemia (low blood albumin)→

  • oedema

  • salt and water retention

• massive dyslipidaemia

• increased concentration of clotting factors

Clinical Features

• child

  • not hypertensive

• oedema initially periorbital

• ascites and Pleural Effusion

Investigations

• urinalyis

  • protein

  • not gross haematuria

• Creatinine clearance

  • low due to decreased intravascular volume

• C3 concentration normal (cf glomerulonephritis)

Causes

• idiopathic nephrotic syndrome

  • may be familial

  • 2x common in boys

  • commonest in 2-6 years

Types

• Minimal change (85% of idiopathic)

  • normal histology of glomeruli with retracted foot processes on EM

  • >95% response to steroids

• Mesangioprolioferative (5% of idiopathic)

  • diffuse increase in mesangial cells and matrix

  • 55% response to stroids

• Focal glomerulosclerosis (10% idiopathic)

  • segmental scarring of juxtamedullary glomeruli

  • 20% response to steroids

Management

• 1mg/kg/day prednisolone

• diuretic therapy for massive oedema

• cyclophosphamide for 8 weeks if can't be weaned off steroids

Complications

• spontaneous bacterial peritonitis

  • Str. pneumoniae

• venous thrombosis

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