A Case of Congenital Nephrotic Syndrome due to Diffuse Mesangial Sclerosis.
- Author:
Jung Jin YU
1
;
Hae Il CHEONG
;
Hyun Soon LEE
;
Yong CHOI
;
Dong Kyu JIN
Author Information
1. Department of Pediatrics, Seoul National University College of Medicine, Seoul, Korea.
- Publication Type:Case Report
- Keywords:
Infantile nephrotic syndrome;
Diffuse mesangial sclerosis;
Denys-Drash syndrome;
WT1 gene
- MeSH:
Denys-Drash Syndrome;
Diagnosis;
Humans;
Infant;
Kidney Failure, Chronic;
Male;
Molecular Biology;
Nephrotic Syndrome*;
Pathology;
Sclerosis*
- From:Journal of the Korean Pediatric Society
1998;41(3):415-419
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
Diffuse mesangial sclerosis (DMS) is one of the underlying pathology of congenital and infantile nephrotic syndrome. Infants with DMS develop nephrotic syndrome before 2 years of age and progress to end stage renal disease within 3 years of age. The authors experienced a case of isolated DMS in a 4-month-old male infant who had nephrotic syndrome for 1 month. The diagnosis was confirmed on the basis of clinical, laboratory, pathological and molecular genetic findings. This is the 3rd case report of DMS in our country and the 1st case report of isolated DMS confirmed by molecular genetic study.
