| Literature DB >> 10588838 |
P Labrune1, M Fabre, P Trioche, B Estournet-Mathiaud, M C Grangeponte, C Rambaud, C Maurage, O Bernard.
Abstract
Three children with Jeune syndrome (asphyxiating thoracic dystrophy) had clinical and laboratory evidence of liver disease. In two patients the disease evolved to biliary cirrhosis, whereas in the third it was recognized when extensive fibrosis was developing. In the three patients, treatment with ursodeoxycholic acid appeared to control the progression of the hepatic dysfunction. Copyright 1999 Wiley-Liss, Inc.Entities:
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Year: 1999 PMID： 10588838 DOI： 10.1002/(sici)1096-8628(19991203)87:4<324::aid-ajmg8>3.0.co;2-u
Source DB: PubMed Journal: Am J Med Genet ISSN： 0148-7299