Clinico–pathological Spectrum and treatment response of Atypical Nephrotic syndrome

Pradeep Sivaraj; Subal Kumar Pradhan; Kedarnath Das; Anil Kumar Mohanty

Volume : III, Issue : V, May - 2014

Clinico–pathological Spectrum and treatment response of Atypical Nephrotic syndrome

Pradeep Sivaraj, Subal Kumar Pradhan, Kedarnath Das, Anil Kumar Mohanty

Abstract :

Introduction and objectives: nephrotic syndrome (NS) is one of the common problems in children. Aim of the study to find out the prevalence, clinical course and treatment response in atypical NS. Atypical nephrotic syndrome includes extremities of ages (< 1yr & >10yr), hypertension, low C3 levels, haematuria, impaired renal dysfunction. Materials and methods: Patients with nephrotic syndrome children from 1 year to 14 year age were evaluated for presence of atypical features (any one of the features) were included and studied for the clinical course and treatment response were followed–up, analyzed and compared with other NS patients. Results: Prevalence of atypical NS is 16.9% with male predominance and in mostly in older children. Most common clinical course of atypical NS was Infrequent Nephrotic Syndrome (IFRNS), but most of Steriod Resistant Nephrotic syndrome (SRNS) patients had atypical NS. Response to steroid was only 50% in Atypical NS, and the need for combination therapy was high in case of atypical NS (30%).Renal biopsy findings of atypical NS showed MCD (50%) followed by FSGS (30%). Conclusion: Incidence of the atypical nephrotic syndrome was alarmingly high. Most of the atypical cases presented after 10 years of age. Nearly half of atypical NS needs an alternative immunosuppressive therapy with or without steroids. Most of the SRNS patients had an atypical presentation.