Clinical presentations and outcomes of congenital and infantile nephrotic syndrome.

Abstract

Objective: To describe the characteristics and outcomes of congenital nephrotic syndrome (CNS) and infantile nephrotic syndrome (INS). Study Design: Cross-sectional study. Setting: Department of Pediatric Nephrology, National Institute of Child Health, Karachi, Pakistan. Period: July 2023 to January 2024. Methods: All cases of CNS or INS visiting the outpatient, emergency department, or admitted in Nephrology ward were analyzed. Data including demographic characteristic and anthropometric measurements, clinical history and presentation were noted. Short-term outcomes in terms of cases who were discharged from hospital or those who died were recorded. Results: In a total of 33 infants, 17 (51.5%) were male. The mean age was 5.8±3.2 months. The mean days from onset of edema to the time of enrollment was 33.27±22.63 days. The most common presenting features were ascites noted in 4 (12.1%). Associated infections were reported in 21 (63.6%) infants. INS and CNS were noted in 25 (75.8%) and 8 (24.2%) infants respectively. Mortality was reported in 11 (33.3%) infants. Conclusion: Infections were the most common associated conditions in congenital and infantile nephrotic syndrome. Short term outcomes exhibiting high mortality rates warrants early identification and treatment of congenital and infantile nephrotic syndrome.