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Published on: February 10, 2015
Jeune syndrome and liver disease: report of three cases treated with ursodeoxycholic acid
P Labrune1, M Fabre, P Trioche
1Service de Pédiatrie, Hôpital Antoine Béclère (AP-HP), Clamart, France. philippe.labrune@abc.ap-hop-paris.fr
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.
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