title: Jeune syndrome and liver disease: report of three cases treated with ursodeoxycholic acid.
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.
authors.affiliation: Service de Pédiatrie, Hôpital Antoine Béclère (AP-HP), Clamart, France. philippe.labrune@abc.ap-hop-paris.fr
authors.forename: P
authors.initials: P
authors.lastname: Labrune
authors.forename: M
authors.initials: M
authors.lastname: Fabre
authors.forename: P
authors.initials: P
authors.lastname: Trioche
authors.forename: B
authors.initials: B
authors.lastname: Estournet-Mathiaud
authors.forename: M C
authors.initials: MC
authors.lastname: Grangeponte
authors.forename: C
authors.initials: C
authors.lastname: Rambaud
authors.forename: C
authors.initials: C
authors.lastname: Maurage
authors.forename: O
authors.initials: O
authors.lastname: Bernard
mesh_terms: D000410:Alanine Transaminase / Q000097:blood / Q000187:drug effects; D001219:Aspartate Aminotransferases / Q000097:blood / Q000187:drug effects; D001238:Asphyxia Neonatorum / Q000150:complications* / Q000188:drug therapy / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D002756:Cholagogues and Choleretics / Q000627:therapeutic use; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008107:Liver Diseases / Q000150:complications* / Q000188:drug therapy / Q000473:pathology; D008297:Male; D010009:Osteochondrodysplasias / Q000150:complications* / Q000188:drug therapy / Q000473:pathology; D016896:Treatment Outcome; D014580:Ursodeoxycholic Acid / Q000627:therapeutic use; D005723:gamma-Glutamyltransferase / Q000097:blood / Q000187:drug effects
chemical_list: D002756:Cholagogues and Choleretics; D014580:Ursodeoxycholic Acid; D005723:gamma-Glutamyltransferase; D001219:Aspartate Aminotransferases; D000410:Alanine Transaminase