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

✍ Scribed by Labrune, Philippe; Fabre, Monique; Trioche, Pascale; Estournet-Mathiaud, Brigitte; Grangeponte, Marie-Claire; Rambaud, Caroline; Maurage, Chantal; Bernard, Olivier

Publisher: John Wiley and Sons
Year: 1999
Tongue: English
Weight: 33 KB
Volume: 87
Category: Article
ISSN: 0148-7299
DOI: 10.1002/(sici)1096-8628(19991203)87:4<324::aid-ajmg8>3.0.co;2-u

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✦ Synopsis

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. Am.

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