Histopathological diagnosis and outcome of paediatric nephrotic syndrome.

OBJECTIVE: To determine the histological picture and outcome of treatment in cases of childhood nephrotic syndrome who needed renal biopsy. DESIGN: An analytical study. PLACE AND DURATION OF STUDY: Department of Paediatrics, King Edward Medical College, and Mayo Hospital, Lahore from 1st June 1996 to 31st December, 2001 over a period of 5-1/2 years. SUBJECTS AND METHODS: Children suffering from nephortic syndrome who had atypical features at presentation were initially or late non-responders; frequent relapsers on < 1mg kg/day and were steroid dependent or frequently relapsed on < 1 mg kg/day but developed steroid toxicity were included. Renal biopsy was performed in these patients. Treatment was administered according to the histopathology reports. Prednisolone 60 mg /m2/day followed after response by 40 mg /m2 on alternate days (AD) which was later tapered off. In minimal change nephrotic syndrome (MCNS) with frequent relapses cyclophosphamide, cyclosporine and levimisole were used. For steroid resistant focal segmental glomerulosclerosis (FSGS) intravenous pulses of methyl-prednisolone and cyclosporine were also given. These patients were followed to see the response of the therapy. RESULTS: The commonest diagnosis was focal segmental glomerulosclerosis (FSGS) (42%) followed by minimal change disease (MCNS) (22%), membranoproliferative or mesangiocapillary glomerulonephritis (MPGN) (14%) and Mesangioproliferative glomerulonephritis (Mes PGN) (12%). There were 6% cases of membranous nephropathy and 4% of diffuse proliferative glomerulonephritis. On presentation, 40% had hematuria, 20% were found to be hypertensive, 12% patients had renal insufficiency and in 4% C3 level was low. Majority of the patients with MPGN and FSGS had atypical features whereas none of the patients with membranous nephropathy had any of these features. Thirty percent cases each of FSGS and MCNS were responders. Among non-responders there were 4 cases of FSGS and one of MPGN. CONCLUSION: FSGS was the commonest histology in cases of childhood nephrotic syndrome that needed renal biopsy. Highest frequency of atypical features was seen in MPGN and FSGS.