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A Case of Congenital Nephrotic Syndrome due to Diffuse Mesangial Sclerosis

Journal of the Korean Pediatric Society 1998;41(3):415-419.
Published online March 15, 1998.
A Case of Congenital Nephrotic Syndrome due to Diffuse Mesangial Sclerosis
Jung-Jin Yu1, Dong Kyu Jin1, Hae Il Cheong1, Hyun Soon Lee2, Yong Choi1
1Department of Pediatrics, Seoul National University, College of Medicine, Seoul, Korea
2Department of Pathology, Seoul National University, Seoul National University
Diffuse Mesangial Sclerosis에 의한 선천성 신증후군 1예
유정진1, 진동규1, 정해일1, 이현순2, 최용1
1서울대학교 의과대학 소아과학교실
2서울대학교 의과대학 병리학교실
Jung-Jin Yu, Email: 1
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.
Key Words: Infantile nephrotic syndrome, Diffuse mesangial sclerosis, Denys-Drash syndrome, WT1 gene

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