Relapse of Steroid-Dependent Nephrotic Syndrome Despite Long-Term Ciclosporin Therapy in an 11-Year-Old Child: A Case Report and Review on the Management of Nephrotic Syndrome in Children.

Background: Frequently relapsing nephrotic syndrome (FRNS) and steroid-dependent nephrotic syndrome (SDNS) are distinct phenotypes defined by relapse frequency and the temporal relationship of relapse to prednisolone therapy, respectively. Both remain therapeutic challenges despite the use of corticosteroid-sparing agents. Relapses may be triggered by intercurrent infections and can be complicated by significant edema, hypoalbuminemia, and infection risk, requiring prompt optimization of immunosuppressive and supportive therapy. Case Presentation: An 11-year-old boy with SDNS presented with a two-day history of bilateral periorbital swelling and facial puffiness following fever, rhinorrhea, and productive cough. He had experienced 11 previous relapses and was receiving ciclosporin 50 mg twice daily and enalapril 5 mg once daily with good adherence. Previous kidney biopsy showed minor glomerular change, and ciclosporin trough concentration was therapeutic. On admission, he was febrile (38.3°C) with leukocytosis, neutrophilia, thrombocytosis, marked hypoalbuminemia, significant proteinuria, and hematuria. Penicillin V was initiated for spontaneous bacterial peritonitis prophylaxis. Prednisolone was optimized from 40 mg once daily to 30 mg twice daily based on a body surface area of 1.17 m Conclusion: This case highlights several important therapeutic considerations in relapsing childhood nephrotic syndrome: recognition of SDNS as a subgroup of SSNS, accurate prednisolone dosing during relapse, careful assessment of edema and intravascular volume status before diuretic therapy, and individualized use of steroid-sparing agents and antimicrobial prophylaxis. In children receiving prolonged ciclosporin therapy, treatment should be regularly reviewed with blood pressure, renal function, and therapeutic drug monitoring in view of potential calcineurin inhibitor toxicity, and alternative steroid-sparing options such as levamisole may be considered where clinically appropriate.