CONGENITAL NEPHROTIC SYNDROME
CONGENITAL NEPHROTIC SYNDROME It is a rare kidney disease which manifests in infants during the first 3 months of life and it characterized by high level of proteins in urine - NPHS1 gene - Nephrin decreased zipper mechanism block defect - NPHS2 gene - Podocin decreased (bad prognosis) CLINICAL FEATURES: Non immune hydrops fetalis Anasara Foamy urine WORKUP: 24 hrs urinary protein = increased = >40mg/m2 BSA Serum albumin - decreased TREATMENT: Daily albumin infusion Steroids