Dr Nouman Shafique, Dr Muhammad Tehseen Ali,Dr Farhan Ghaffar Bhatti
Aim: To evaluate the efficacy and safety of cyclosporin A, which is the basis for the treatment of complete and sustained remission in steroid resistant nephrotic syndrome in children. Project: A hospital based descriptive study (case series). Place and duration: In the department of pediatric Nephrology, Children Hospital Lahore for one-year duration from January 2019 to January 2020. Patients and methods: Twenty-nine patients with steroid resistant nephrotic syndrome (focal segmental glomerulosclerosis in 13 cases, minimal change in disease in 8 cases, mesangoproliferative glomerulonephritis in 4 cases, 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: The average age of 29 patients (20 boys and 9 girls) was 8.3 (range: 2.7-14). The mean age at the beginning of the nephrotic syndrome and the beginning of the cyclosporin-A study was 4.3 years (range 1.7-12) and 8 years (range: 2.5-14), respectively. Seven (24.1%) cases had "early steroid resistance" and 22 (75.8%) had "late steroid resistance". At the end of the 12-month treatment, 17 (58.6%) patients were mostly in full and continuous remission with focal segmental glomerulosclerosis (9 of 13 cases) and minimal lesion disease (6 of 8 cases). Eight (27.5%) patients continued to have proteinuria in the nephrotic range. This group initially included only 6 cases in which partial remission was achieved, and then 2 cases that stopped responding to cyclosporin-A after a period of full remission. Four (13.7%) cases did not undergo remission from the very beginning. The median time to achieving "full remission" and "partial remission" after the start of the cyclosporin-A study was 2.2 months (range 1 to 3.5) and 3 months (range 2-5), respectively. The correlation of early remission with longer and total remission time was significant. Recurrence of pyuria was demonstrated in 4 (13.7%) and bacterial peritonitis in 2 cases. Hirsutism was reported in 22 (75.8%) patients. Three (10.3%) patients went on to end-stage renal disease. Conclusion: Cyclosporin-A is highly effective and safe to achieve full remission lasting at least 12 months in approximately 60% of cases of steroid-resistant nephrotic syndrome in children, especially when it leads to early remission. Keywords: Nephrotic syndrome, steroid resistance, cyclosporin-A.