NEPHROTIC SYNDROME

Causes of nephritic syndrome

  • Idiopathic or unknown for the majority of children with nephritic syndrome.
  • Congenital nephritic syndrome, which may be to congenital syphilis.
  • Secondary nephritic syndrome, which is due to post acute glomerulonephritis, plasmodium malaria, other infection and infestations, allergy following bee stings, heavy metal poisoning (e.g., mercury and lead), urinary tract infection.

Clinical Features

The clinical features of nephritic syndrome include the following:

  • Oedema that is marked to massive and may be accompanied by ascites and/ or pleural effusion
  • Marked proteinuria
  • Hypoproteinaemia, mainly low serum albumin in blood
  • Hyperlipidaemia
  • Children with nephritic syndrome who have haematuria with hypertension are categorized as nephritic nephrosis.

Investigations

  • Urinalysis
  • 24-hour urine for protein
  • Serum protein
  • Urea and electrolytes
  • Serum cholesterol

Management

  • High protein if urea is normal, low salt diet (no salt added to food)
  • Frusemide administered carefully to induce diuresis
  • Prednisone 2mg/kg/day (maximum 60mg). The responses to prednisone are generally divided into steroid responders and non steroid responders:
    • Response usually occurs within 2 weeks demonstrated by no protein in urine. When urine is protein free start tapering of the dose over 6-–12 weeks.
    • Relapses are treated the same way
    • If there is continuing proteinuria after 1 month the child is steroid resistant.
    • If proteinuria returns after the steroids are stopped the child is steroid dependent and may require continuation
    • Repeated relapses or steroid dependants who develop steroid toxicity can be treated with cyclophosphamide. Cyclosporin or levimazole may be better alternatives in future.
    • Steroid unresponsive cases may benefit from ACE inhibitors even in the absences of hypertension. Cyclosporin can also be tried; diuretics are used to control oedema.
  • Antibiotics are used if there are clinical signs of/or suspected infections. Possibility of urinary tract infection should always be considered.
  • Refer to specialist patients:
    • With persistent haematuria
    • With hypertension
    • Who develop chronic renal failure
    • Who relapse or do not respond.