Childhood idiopathic steroid resistant nephrotic syndrome in Southwestern Nigeria

Abstract EN

Clinical charts of 23 Nigerian children diagnosed with Idiopathic Steroid Resistant Nephrotic Syndrome (iSRNS) between January 2001 and December 2007 were retrospectively reviewed to determine their clinicopathologic characteristics and outcome.

iSRNS (54.8%) was primary in 19 patients (83%) and secondary in four (17%).

The mean age at diagnosis was 8.3 ± 3.5 years (2.1-13 years).

Histopathology revealed membranoproliferative glomerulonephritis (MPGN) in 43.5%, focal and segmental glomerulosclerosis (FSGS) in 39.1% and mesangial proliferative glomerulonephritis in 8.7% of the patients while minimal change disease (MCD) and membranous nephropathy accounted for 4.35% each.

Routine treatment protocol comprised pulse intravenous (i.v.) cylophosphamide infusion and i.v.

dexamethasone ? lisinopril or spironolactone.

Cumulative Complete Remission (CR) rate was 57.12%.

The overall median time to CR from start of steroid sparing agents in 12/21 treated patients was 4.5 weeks.

CR was better achieved in MPGN than FSGS (P = 0.0186).

Five patients had eight relapses with the overall median relapse-free duration being four months.

Cumulative renal survival at 36 months was 41.8%.

The median follow-up duration was eight months.

Our study revealed that there was a high prevalence of iSRNS and preponderance of non-MCD lesions, with MPGN and FSGS being the major morphologic lesions.

The outcome with steroid and cyclophosphamide-based treatment for iSRNS was further enhanced with addition of either lisinopril or spironolactone.