| Juvenile Nephronophyjisis in An Infant. |
|
Young Seo Park1, Chang Youn Lee1, Hae Il Cheong1, Yong Choi1, Kwang Wook Ko1, Je Geun Chi2, Chang Bin Im3 |
1Department of Pediatrics, College of Medicine, Seoul National University, Seoul, Korea
2Department of Pathology, College of Medicine, Seoul National University, Seoul, Korea
3Department of Pediatrics, Dae Han Hospital, Seoul, Korea |
| 영아에서의 Juvenile Nephronophthisis |
|
박영서1, 이창연1, 정해일1, 최용1, 고광욱1, 지제근2, 임창빈3 |
1서울대학교 의과대학 소아과학교실
2서울대학교 의과대학 병리학교실
3대한병원 소아과 |
|
Received: 20 December 1988 • Accepted: 28 February 1989 |
|
|
| Abstract |
|
Juvenile nephronophthisis is a chronic tubulo-interstitial renal disease characterized by an early
onset of polyuria and polydipsia, decreased urinary concentrating ability, anemia, growth retardation
and progressive renal disease. It is recognized as an important cause of chronic renal failure in the
childhood. However only one case was reported in the Korean literatures.
In this paper we have reported the second documented case of juvenile nephronophthisis in Korea
in an 8 months old girl. This case was presented as chronic renal failure which probably had begun
since 5 months of age, and died of Pseudomonas sepsis. Characteristic features of nephronophthisis
with a marked tubulointerstitial reaction, microcystic change, tubular basement membrane abnor-
malities and congenital hepatic fibrosis were found at postmortem examination.
Juvenile nephronophthisis should be considered as one of the causative diseases of chronic renal
failure not only in childhood but also in infancy. |
| Key Words:
Juvenile Nephronophthisis, Chronic Renal Failure |
|
PDF Links
Download Citation
