Nephrotic syndrome will affect 2-4 children per 100,000, usually at Primary school age and is more commonly seen in children of Asian descent.
It is characterised by oedema, low albumin and proteinuria and may present with lethargy, abdominal pain, shortness of breath or unexplained weight gain with swelling.
It can be broadly categorised into three groups:
- Minimal Change Disease - Accounts for 78%. Named for the very subtle changes to glomeruli that can only be seen on Scanning Electron Microscope. Usually responds to steroids alone and is the presumptive diagnosis when first line treatment is effective.
- Focal Segmental Glomerulosclerosis (FSGS) - Accounts for 8%. FSGS is not one but many different diseases. When someone has focal segmental glomerulosclerosis (FSGS), some of the glomeruli are damaged and become scarred.
- Membranoproliferative glomerulonephritis (MPGN) - Accounts for 6%. Glomerulonephritis is the term used when the glomeruli become inflamed. Membranoproliferative glomerulonephritis (MPGN, sometimes called mesangiocapillary glomerulonephritis) is one of several forms of glomerulonephritis and one that is rather uncommon.
Treatment is with high dose steroids but, until protein loss is controlled, it is important to closely monitor fluid balance, weight and blood pressure. Children should be started on prophylactic antibiotics whilst they remain on steroids.
Children who respond promptly to steroid therapy are presumed to have minimal change disease; if they do not improve on steroids or have multiple relapses then renal biopsy is indicated.