Steroid resistant nephrotic syndrome in children: Clinical presentation, renal histology, complications, treatment and outcome at Bangabandhu Sheikh Mujib Medical University, Dhaka, Bangladesh

Abstract

Steroid resistant nephrotic syndrome (SRNS) remains a challenge for pediatric nephrologists. The underlying histopathology usually affects the course of the disease and the response to treatment. This study was designed to determine clinical presentation, renal histology, complications, treatment and outcome in children presenting with SRNS. A prospective analysis was carried out among 32 steroid resistant nephrotic syndrome patients aged 1-18 year in the department of pediatric nephrology, Bangabandhu Sheikh Mujib Medical University, Dhaka, Bangladesh during the period of January 2011 to June 2014. Percutaneous renal biopsy were done in all patients. The histopathology slides were reviewed by competent pathologists. Patients with congenital nephrotic syndrome and nephrotic syndrome secondary to systemic diseases were excluded from the study. Thirty two children fulfilled the inclusion criteria, and included 19 boys and 13 girls, male to female ratio was 1.4:1. Their mean age of presentation was 9.2 year (range 16 month to 16 year). Nine patient(28.22) presented with typical presentation and 23 (71.88%) presented with atypical presentation which included hematuria (62.5%), very high cholesterol (>500mg/dl), persistent hypertension (40.63%), unfavorable age (28.13%), hypocomplementemia (21.88%) and azotemia. None had a positive family history or hepatitis B surface antigen. The renal histopathology was compatible with mesengioproliferative glomerulonephritis (MesPGN) in 40.63%% (n=17), membranoproliferative glomerulonephritis (MPGN) 21.88% (n=07), minimal change disease (MCD) 18.75% (n=06), focal and segmental glomerulosclerosis (FSGS) in 12.5% (n=4) and inadequate tissue was found in two cases. All patients were treated by intravenous methylprednisolone four to six pulses along with intravenous cyclophosphamide followed by oral prednisolone. Cyclosporine was added in patients who failed to achieve remission The outcome with steroid and cyclophosphamide-based treatment for iSRNS was further enhanced with addition of ACE inhibitor. Regarding outcome 21(65.63%) patient responded, five (15.63%) patients died, four (12.5%) reached end stage renal disease and two refused to take any treatment. This study revealed that MesPGN was the commonest histopathology in children presented with SRNS, IV methylprednisolone and IV cyclophosphamide are still agood option for treatment of SRNS with a response rate of sixty five percent. KEY WORD: Management, Nephrotic syndrome, Outcome