Reversed Clinical and Morphologic Characteristics of Idiopathic Childhood Nephrotic Syndrome

Background and aims: minimal change disease (mcd) is uncommon in nigeria; in the sixties and eighties quartan malaria nephropathy accounted for more than 80.0% of all cases of childhood nephrotic syndrome (cns). there is paucity of current clinical and morphologic data on cns. this study therefore determined the incidence and prevalence of cns, pre-treatment glomerular pathology of idiopathic cns (icns), renal and patient outcome in steroid sensitive icns. methods: a non-randomized prospective study of consecutive cases of nigerian children with idiopathic nephrotic syndrome was conducted over a 9-year period. results: cns accounted for 1.26% of pediatric admissions. fifty-four of 78 (69.2%) nephrotic children had icns (incidence, 0.44/100000/year). median ns onset age was 7.1 (2.5-14.0) years. male: female ratio was 1.7. the histopathologic lesions were membranoproliferative glomerulonephritis (mpgn, 44.4%), focal segmental glomerulosclerosis (fsgs, 25.9%), mcd (18.5%), mesangial proliferative glomerulonephritis (7.4%) and membranous nephropathy (3.7%). overall cumulative complete remission (cr) rate 4 to 8 weeks post prednisolone treatment was 49.6%. twenty-two of 25 with cr were early steroid responders while 3 were late responders. median time to cr was 12.0 (3.0 – 46.0) days. thirty relapses occurred; median time to first relapse was 11.0 months. cumulative five-year relapse-free rate was 26.6%. five-year renal survival was 16.1%. all patients with cr were followed-up for 6-93 (median, 22.0) months. conclusions: prevalence of non-mcd was very high with significant resistance to prednisolone; poor renal survival was due to high frequency of mpgn and fsgs. pre-treatment renal biopsy is advocated in our kind of patients so that steroid-sparing agents can be started early.