Najmul Hasan, Asma Razzaq.
Management of steroid resistant nephrotic syndrome: an experience with cyclosporine-a.
Pak Paed J Jan ;36(1):12-8.

Objective: To evaluate the effectiveness and safety of Cyclosporine-A as the mainstay of treatment for induction of complete and sustained remission in steroid resistant nephrotic syndrome in children. Design: A hospital based descriptive study (case series). Place and Duration: Renal Clinic of Department of Pediatrics Combined Military Hospital Lahore, Pakistan. From December 1999 to December 2003. Patients & Methods: Twenty nine patients of steroid resistant nephrotic syndrome (focal segmental glomerulosclerosis in 13 cases, minimal change disease in 8, mesangioproliferative glomerulonephritis in 4, diffuse membranoproliferative glomerulonephritis in 3 and crescentic glomerulonephritis in 1) with normal renal function and glomerular filtration rate, were treated with Cyclosporine-A and Enalapril for 12 months along with low dose alternate day Prednisolone tapered over six to nine months. Results: Mean age of the 29 patients (20 boys and 9 girls) was 8.3 years (range: 2.7-14). Mean age at the onset of nephrotic syndrome and commencement of Cyclosporine-A trial was 4.3 years (range 1.7-12) and 8 years (range: 2.5-14) respectively. \'Early steroid resistance\' was observed in 7 (24.1%) cases and \'Delayed steroid resistance\' in 22 (75.8%) cases. At the completion of 12 months of treatment, 17 (58.6%) patients were in a complete and sustained remission having predominance of focal segmental glomerulosclerosis (9 out 13 cases) and minimal change disease (6 out of 8 cases). Eight (27.5%) patients persisted with nephrotic range proteinuria. This group included 6 cases who initially achieved only a partial remission and 2 cases that later turned Cyclosporine-A unresponsive after staying in complete remission for some time. Four (13.7%) cases never went into remission right from the very beginning. The mean duration to achieve \'complete\' and \'partial remission\' after the commencement of Cyclosporine-A trial was 2.2 months (range 1-3.5) and 3 months (range 2-5) respectively. The correlation of an early remission with a longer duration of complete and sustained remission was significant. Recurrent pyuria was found in 4 (13.7%) and culture proven bacterial peritonitis in 2 cases. Hirsutism was reported in 22 (75.8%) patients. Three (10.3%) patients progressed to End Stage Renal Disease. Conclusion: Cyclosporine-A is reasonably effective and safe in inducing complete and at least 12 months long sustained remission in about 60% cases of steroid resistant nephrotic syndrome in children, particularly when it leads to an early remission.

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