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.