title: Biliary atresia and survival into adulthood without transplantation: a collaborative multicentre clinic review.
abstract: BACKGROUND
  Biliary atresia is a progressive biliary injury which occurs only in infants.
  
  
  OBJECTIVE
  To review the experience of patients surviving into adulthood without the need for liver transplantation in childhood.
  
  
  METHODS
  A multicentre review of patients with biliary atresia treated surgically who survived into adulthood without the need for transplantation.
  
  
  RESULTS
  Twenty-two patients were identified across four centres. Median age at the last follow-up was 25 years (range: 18-46), and 21 patients had clinical features of portal hypertension. At last follow-up values of liver enzymes varied from normal to 15 × the upper limit of normal (ULN) for ALT (median 2.11 × ULN) and 9 × the ULN for ALP (median 2.02 × ULN). Six patients had a serum bilirubin > 50 μmol/l. Pruritus and jaundice were noted in 8 of 20 patients (40%) and 11 of 22 patients (50%) respectively. Thirteen patients (59.1%) were shown to have imaging features of sclerosing cholangitis, with strictures of intrahepatic bile duct(s) (IHBD), dilatation of IHBD (n = 8), or stone(s) within the IHBD (n = 5). A history of presumed bacterial cholangitis was present in 11 patients (50%). Successful pregnancies were recorded in three of fourteen female patients. Four patients underwent transplant between the ages of 20-27 years. Twenty-one patients (95.5%) were alive, including 18 (81.8%) with their native liver at the time of last follow-up.
  
  
  CONCLUSIONS
  Some patients treated for biliary atresia will survive into adulthood with their native liver, but commonly with secondary biliary disease including cholangitis and portal hypertension.
authors.affiliation: Toronto Western Hospital, Toronto, Canada.
authors.forename: Teru
authors.initials: T
authors.lastname: Kumagi
authors.forename: Joost P H
authors.initials: JP
authors.lastname: Drenth
authors.forename: Orlee
authors.initials: O
authors.lastname: Guttman
authors.forename: Vicky
authors.initials: V
authors.lastname: Ng
authors.forename: Les
authors.initials: L
authors.lastname: Lilly
authors.forename: George
authors.initials: G
authors.lastname: Therapondos
authors.forename: Yoichi
authors.initials: Y
authors.lastname: Hiasa
authors.forename: Kojiro
authors.initials: K
authors.lastname: Michitaka
authors.forename: Morikazu
authors.initials: M
authors.lastname: Onji
authors.forename: Yuji
authors.initials: Y
authors.lastname: Watanabe
authors.forename: Sambit
authors.initials: S
authors.lastname: Sen
authors.forename: William
authors.initials: W
authors.lastname: Griffiths
authors.forename: Eve
authors.initials: E
authors.lastname: Roberts
authors.forename: Jenny
authors.initials: J
authors.lastname: Heathcote
authors.forename: Gideon M
authors.initials: GM
authors.lastname: Hirschfield
mesh_terms: D000293:Adolescent; D000328:Adult; D001653:Bile Ducts, Intrahepatic / Q000473:pathology; D001656:Biliary Atresia / Q000150:complications / Q000503:physiopathology* / Q000601:surgery; D049448:Cholangiopancreatography, Magnetic Resonance; D015209:Cholangitis, Sclerosing / Q000000981:diagnostic imaging* / Q000209:etiology; D002779:Cholestasis / Q000209:etiology / Q000473:pathology*; D005260:Female; D006801:Humans; D006975:Hypertension, Portal / Q000209:etiology / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D011171:Portoenterostomy, Hepatic; D017741:Survivors; D016896:Treatment Outcome; D014463:Ultrasonography