Steroid-sensitive nephrotic syndrome from childhood to adulthood

This is the mainstay of treatment and should be commenced once the diagnosis is established.  If there are concerns of the possibility of secondary nephrotic syndrome please discuss with nephrology prior to commencing steroid therapy.
Many patients require the addition of gastroprotective medication whilst on high dose daily steroids.

Steroid therapy for first presentation:
The most recent systematic analysis (Cochrane library 2015) recommends 3 months of corticosteroid therapy. 
- Prednisone or Prednisolone - start at 60mg/m2/day (max 80mg) in a single daily dose to complete a total of 28 days.
- This is followed by alternate day therapy at 40mg/m2/day (max 60mg) for further 28 days.
-Then wean steroid dose gradually over the next 4-6 weeks and stop.
-Total treatment duration of first presentation should be approximately 3 months.

Steroid therapy for relapses:
Infrequent Relapses
Prednisone or prednisolone - start at 60mg/m2/day (max 80mg) until in remission. Then give alternate day prednisone or prednisolone at 40mg/m2/day (max 60mg) for total of 28 days, then stop.
Children having <2-3 relapses per year can be managed with repeated courses of prednisone, providing they have a complete response to treatment each time.

Frequently relapsing or Steroid Dependent
Consider alternate day prednisone -/kg for 4-6 months, at the lowest dose to avoid side effects.  After 4-6 months therapy, slow taper to attempt cessation of steroid therapy. Consider non-steroid immunosuppressive therapy earlier if the patient is already exhibiting significant steroid side effects.
For patients whose relapses are precipitated by URTI or other infections whilst on alternate day steroid, increasing the alternate day dose to daily for 5 days at the onset of the illness will reduce the risk of relapse by up to 60%. This strategy is supported by a number of randomised controlled trials.

If relapses continue despite alternate day steroids >/kg or steroid related adverse effects develop, referral to Nephrology is indicated for consideration of alternative immunosuppressive therapy.

Purpose of investigations in nephrotic syndrome (NS) is

  • (1) confirm the clinical diagnosis;
  • (2) seek a possible cause;
  • (3) assess renal function; and
  • (4) identify biochemical disorders related to the nephrotic state . hypercoagulability, hypovolaemia, hyperlipidaemia
Finding of heavy proteinuria (3-4+) on dipstick and oedema in a child in the developed world usually means a diagnosis of NS. Proteinuria needs to be quantified as the protein: creatinine ratio or per litre of urine

Steroid-sensitive nephrotic syndrome from childhood to adulthood

steroid-sensitive nephrotic syndrome from childhood to adulthood

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