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

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.