Children with Steroid-Resistant Nephrotic Syndrome: A Single -Center Experience.

Background: Nephrotic syndrome (NS) is one of the most frequent glomerular diseases among children. While most of the children with primary NS respond to steroid treatment, 10 to 20% of the patients are steroid-resistant, and the best therapy for such cases has never been defined.

Objective: The present study aimed to evaluate steroid-resistant nephrotic syndrome (SRNS) patients.

Materials and methods: Our research included 50 children (56% female and 44% male) with NS. NS was defined as the presence of edema, massive proteinuria, hypoalbuminemia and hyperlipidemia. Patients with NS were treated according to international protocol. SRNS was diagnosed in patients with idiopathic NS based on lack of complete remission despite treatment with steroids. Renal biopsy was performed in 22 patients with SRNS at the Pediatric Clinic II of the University Clinical Center in Sarajevo (UCCS). Histopathologic analyzes of renal biopsy were performed at the Department of Pathology, University Clinical Center in Tuzla (UCCT). Patients with SRNS, after kidney biopsy were treated with nonsteroidal immunosuppressant's.

Results: Eight (36.4%) of the 22 patients who had undergone renal biopsies had minimal change disease (MCNS) and seven (31.8%) had focal segmental glomerulosclerosis (FSGS). The immunosuppressive drugs used in SRNS were Cyclosporine (CsA), Cyclophosphamide (CYC), Mycophenolat mofetil (MMF) and Rituximab (RTX). Three patients (13.6%) did not respond to any treatment and had developed end - stage renal disease (ESRD).

Conclusion: With current treatments, some children will ultimately achieve a sustained remission with one of the second line or third line of the proposed drugs. Patients with refractory NS will go to progression towards ESRD. The rapid development of molecular genetics will give a new contribution to the pathogenesis and treatment of this disease.