Premium
Biliary atresia and survival into adulthood without transplantation: a collaborative multicentre clinic review
Author(s) -
Kumagi Teru,
Drenth Joost P. H.,
Guttman Orlee,
Ng Vicky,
Lilly Les,
Therapondos George,
Hiasa Yoichi,
Michitaka Kojiro,
Onji Morikazu,
Watanabe Yuji,
Sen Sambit,
Griffiths William,
Roberts Eve,
Heathcote Jenny,
Hirschfield Gideon M.
Publication year - 2012
Publication title -
liver international
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.873
H-Index - 110
eISSN - 1478-3231
pISSN - 1478-3223
DOI - 10.1111/j.1478-3231.2011.02668.x
Subject(s) - medicine , biliary atresia , liver transplantation , portal hypertension , gastroenterology , jaundice , transplantation , primary sclerosing cholangitis , liver disease , bilirubin , atresia , surgery , disease , cirrhosis
Background Biliary atresia is a progressive biliary injury which occurs only in infants. Aims 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.