Outcome of Idiopathic Steroid Resistant Nephrotic Syndrome of Children in Central Child Teaching Hospital


Background: Steroid resistant nephrotic syndrome accounts for 10%-20% of all cases of idiopathic nephrotic syndrome. These patients are at risk of developing end stage renal disease.Aim of the study: to determine the outcome in pediatric patients with steroid resistant nephrotic syndrome, demographic characteristics, renal biopsy findings, and response to immunosuppressive treatment.Materials and Methods: This retrospective study included 136 patients diagnosed as primary steroid resistant nephrotic syndrome followed by pediatric nephrology department in central child teaching hospital during the period from 2000 to December 2015 (by collecting files of patients). the study done from June 2016 to December 2016.Age at first episode, gender and family history of nephrotic syndrome were recorded. Demographic characteristics, clinical features at presentation, renal biopsy findings, response to immunosuppressive treatment and outcome were analyzed.Results: one hundred thirty-six patients involved in the study, mean age at first episodeof nephrotic syndrome was 7.18+_ 3.9 years (range: 1 - 16 years). renal biopsy was done for 83 patients and main histopathology was focal segmental glomerulosclerosis found in 54 patients represent (65.1%). Many items of drugs given to the patients and most commonly used and most effective drug is Cyclosporine A in which 34 from patients achieved complete remission. There were 21 patients (15.4%) get complete remission, 38 patients (27.9%) lost follow up, 6 patients (4.4%) died, 13 patients (9.6%) developed stage 3 chronic kidney disease 24 patients (17.6%) developed stage 4 chronic kidney disease and 34(25%) patients developed End stage renal disease. There is significant relationship between hypertension, hematuria, and impaired renal functions at presentation and response to immunotherapy and development of end stage renal disease, while there is no significant relationship between age of patient at presentation, gender, histopathology and development of end stage renal disease.Conclusion: we found near half of the patients developed chronic renal failure and 25% developed end stage renal disease. Patients with atypical presentation, resistance to immunosuppressive are liable to develop End stage renal disease significantly. Inaddition, we found Cyclosporine A is more effective than other immunotherapy as the initial therapy for many patients.