Steroid resistant nephrotic syndrome in children: Clinical presentation, renal histology, complications, treatment and outcome at Bangabandhu Sheikh Mujib Medical University, Dhaka, Bangladesh
Journal Title: IOSR Journal of Pharmacy (IOSRPHR) - Year 2014, Vol 4, Issue 11
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 studywas designed to determine clinical presentation, renal histology, complications, treatment and outcome inchildren presenting with SRNS. A prospective analysis was carried out among 32 steroid resistant nephroticsyndrome patients aged 1-18 year in the department of pediatric nephrology, Bangabandhu Sheikh MujibMedical University, Dhaka, Bangladesh during the period of January 2011 to June 2014. Percutaneous renalbiopsy were done in all patients. The histopathology slides were reviewed by competent pathologists. Patientswith congenital nephrotic syndrome and nephrotic syndrome secondary to systemic diseases were excluded fromthe study. Thirty two children fulfilled the inclusion criteria, and included 19 boys and 13 girls, male to femaleratio 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 includedhematuria (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), minimalchange 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.
Authors and Affiliations
Roy RR1 , Haque SMS2 , MamunAA3 , MuinuddinG4 ,Rahman MH5
Keywords
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