ANALYSIS OF CLINICO-PATHOLOGICAL CORRELATIONS OF CONGENITAL AND INFANTILE NEPHROTIC SYNDROME

Introduction: Nephrotic syndrome is characterized by gross proteinuria, hypo albuminemia, hyperlipidemia, and peripheral edema. The etiology of nephrotic syndrome in adults is complex and ranges from primary glomerulonephritis to secondary forms.Objectives of the study: The basic aim of the study is to analyze the clinico-pathological correlations of congenital and infantile nephrotic syndrome in Pakistan.Material and Methods:This cross sectional study was done at Jinnah Hospital Lahore during 2018 to 2019. This was done with the permission of ethical committee of hospital. The data was collected from 100 children who presented within the first year of life with nephrotic syndrome who had a histological diagnosis obtained by renal biopsy in the hospital. The clinical diagnosis of NS was made on the basis of nephrotic range proteinuria, hypo albuminaemia and oedema.Results: Seventy children who presented at 0.1–11.6 (median 1.6) months were included with 31 presenting within the first three months of life. Histopathological review diagnostic categories were; 13 Mesangial proliferative glomerulopathy (MesGN), 12 Focal and segmental glomerulosclerosis (FSGS), 11 Finnish type changes, eight Diffuse Mesangial Sclerosis (DMS), three Minimal change disease (MCD) and one each of Dense Deposit Disease (DDD) and Membranous nephropathy. Conclusion: Nephrotic syndrome can increase your child’s risk of infection and blood clots. Children with identified causative mutations generally presented earlier and had poor renal outcome. The time taken to obtain genetic testing results is decreasing with improving technology. The availability of genetic testing varies from centre to centre and country to country