J Korean Pediatr Soc.  1998 Mar;41(3):415-419.

A Case of Congenital Nephrotic Syndrome due to Diffuse Mesangial Sclerosis

Affiliations
  • 1Department of Pediatrics, Seoul National University College of Medicine, Seoul, Korea.
  • 2Department of Pathology, Seoul National University College of Medicine, Seoul, Korea.
  • 3Department of Pediatrics, Sung Kyun Kwan University, College of Medicine, Seoul, Korea.

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.

Keyword

Infantile nephrotic syndrome; Diffuse mesangial sclerosis; Denys-Drash syndrome; WT1 gene

MeSH Terms

Denys-Drash Syndrome
Diagnosis
Humans
Infant
Kidney Failure, Chronic
Male
Molecular Biology
Nephrotic Syndrome*
Pathology
Sclerosis*
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