title: Biliary atresia: Clinical advances and perspectives.
abstract: Biliary atresia (BA) is a rare and severe inflammatory and obliterative cholangiopathy that affects both extra- and intrahepatic bile ducts. BA symptoms occur shortly after birth with jaundice, pale stools and dark urines. The prognosis of BA has dramatically changed in the last decades: before the Kasai operation most BA patients died, while nowadays with the sequential treatment with Kasai operation±liver transplantation BA patient survival is close to 90%. Early diagnosis is very important since the chances of success of the Kasai procedure decrease with time. The causes of BA remain actually unknown but several mechanisms including genetic and immune dysregulation may probably lead to the obliterative cholangiopathy. Current research focuses on the identification of blood or liver factors linked to the pathogenesis of BA that could become therapeutic targets and avoid the need for liver transplantation. No similar disease leading to total obstruction of the biliary tree exists in older children or adults. But understanding the physiopathology of BA may highlight the mechanisms of other destructive cholangiopathies, such as sclerosing cholangitis.
authors.affiliation: Pediatric Hepatology Unit, hôpital Necker-Enfants-Malades, 149, rue de Sèvres, 75015 Paris, France.
authors.forename: Laure
authors.initials: L
authors.lastname: Nizery
authors.affiliation: Pediatric Surgery Unit, hôpital Necker-Enfants-Malades, Paris, France.
authors.forename: Christophe
authors.initials: C
authors.lastname: Chardot
authors.affiliation: Pediatric Hepatology Unit, hôpital Necker-Enfants-Malades, 149, rue de Sèvres, 75015 Paris, France.
authors.forename: Samira
authors.initials: S
authors.lastname: Sissaoui
authors.affiliation: Pediatric Surgery Unit, hôpital Necker-Enfants-Malades, Paris, France.
authors.forename: Carmen
authors.initials: C
authors.lastname: Capito
authors.affiliation: UMR1163, Imagine Institute, hôpital Necker-Enfants-Malades, Paris, France.
authors.forename: Alexandra
authors.initials: A
authors.lastname: Henrion-Caude
authors.affiliation: Pediatric Hepatology Unit, hôpital Necker-Enfants-Malades, 149, rue de Sèvres, 75015 Paris, France.
authors.forename: Dominique
authors.initials: D
authors.lastname: Debray
authors.affiliation: Pediatric Hepatology Unit, hôpital Necker-Enfants-Malades, 149, rue de Sèvres, 75015 Paris, France; UMR1163, Imagine Institute, hôpital Necker-Enfants-Malades, Paris, France. Electronic address: muriel.girard@aphp.fr.
authors.forename: Muriel
authors.initials: M
authors.lastname: Girard
mesh_terms: D001656:Biliary Atresia / Q000175:diagnosis / Q000209:etiology / Q000628:therapy*; D002756:Cholagogues and Choleretics / Q000627:therapeutic use; D042241:Early Diagnosis; D006801:Humans; D016031:Liver Transplantation; D011171:Portoenterostomy, Hepatic; D011379:Prognosis; D014580:Ursodeoxycholic Acid / Q000627:therapeutic use
chemical_list: D002756:Cholagogues and Choleretics; D014580:Ursodeoxycholic Acid