Nephrotic syndrome in African children: lack of evidence for ‘tropical nephrotic syndrome’?

Abstract

Infections such as malaria, schistosomiasis, hepatitis B and HIV have been suggested as major causes of the nephrotic syndrome (NS) in African children. We retrospectively analysed the course of the NS in 32 children from Ghana and reviewed the literature on NS from 18 different African countries for the presence of 'the tropical nephrotic syndrome'. Thirty-two children (22 boys, 10 girls, median age 12 years, range 1-18 years) with NS were treated from 2000-2003 at Battor Hospital, Ghana. Thirteen out of 32 children underwent a renal biopsy which was investigated by light, immune and electron microscopy. All 32 patients were initially treated with oral prednisone (PRED) therapy (29 with standard therapy for 8 weeks and three individually tailored), and steroid-resistant children received also intravenous methylprednisolone pulses (three children) or oral cyclophosphamide (two children). All patients fulfilled the clinical and laboratory criteria of a NS. The initial median serum creatinine was 65 micromol/l (range 44-133 micromol/l). Renal biopsy was performed in 13/32 children and revealed focal and segmental glomerulosclerosis (FSGS) in 10 patients, minimal change disease (MCNs) in two and no conclusive result in one patient. Glomerular immune complex deposition was absent in all biopsies. After treatment with PRED, oedema disappeared in 24/32 patients; however, proteinuria normalized in 16/32 patients only. The NS relapsed in 9/16 steroid-sensitive patients after cessation of PRED therapy, and two children were frequent relapsers. The steroid-resistant NS did not respond to an intensified immunosuppression in 5/16 children receiving methylprednisolone or cyclophosphamide. Five out of 32 children died, all were steroid resistant. There was no evidence for a dominating role of steroid-resistant 'tropical glomerulopathies' in children with a NS in Ghana. Similar to South Africa, focal and segmental glomerulosclerosis (FSGS) and minimal change disease were the most frequent findings on histology. Contrary to Nigeria, membrano-proliferative glomerulonephritis was not found in these patients. We conclude from this data and from the literature that the histological pattern of NS may vary between different African countries. Concerning therapy of NS under tropical conditions, we emphasize that despite the limited therapeutic facilities half of these patients may benefit from corticosteroids; however, steroid resistance and FSGS resulted in a high mortality.