Jeune syndrome and liver disease: Report of three cases treated with ursodeoxycholic acid

Citation
P. Labrune et al., Jeune syndrome and liver disease: Report of three cases treated with ursodeoxycholic acid, AM J MED G, 87(4), 1999, pp. 324-328
Citations number
12
Categorie Soggetti
Molecular Biology & Genetics
Journal title
AMERICAN JOURNAL OF MEDICAL GENETICS
ISSN journal
01487299 → ACNP
Volume
87
Issue
4
Year of publication
1999
Pages
324 - 328
Database
ISI
SICI code
0148-7299(199912)87:4<324:JSALDR>2.0.ZU;2-U
Abstract
Three children with Jeune syndrome (asphyxiating thoracic dystrophy) had cl inical and laboratory evidence of liver disease, In two patients the diseas e evolved to biliary cirrhosis, whereas in the third it was recognized when extensive fibrosis was developing. In the three patients, treatment with u rsodeoxycholic acid appeared to control the progression of the hepatic dysf unction, Am. (C) 1999 Wiley-Liss, Inc.