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Biliary atresia and survival into adulthood without transplantation: a collaborative multicentre clinic review.
MedLine Citation:
PMID:  22098694     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
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.
Authors:
Teru Kumagi; Joost P H Drenth; Orlee Guttman; Vicky Ng; Les Lilly; George Therapondos; Yoichi Hiasa; Kojiro Michitaka; Morikazu Onji; Yuji Watanabe; Sambit Sen; William Griffiths; Eve Roberts; Jenny Heathcote; Gideon M Hirschfield
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2011-11-9
Journal Detail:
Title:  Liver international : official journal of the International Association for the Study of the Liver     Volume:  -     ISSN:  1478-3231     ISO Abbreviation:  -     Publication Date:  2011 Nov 
Date Detail:
Created Date:  2011-11-21     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  101160857     Medline TA:  Liver Int     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Copyright Information:
© 2011 John Wiley & Sons A/S.
Affiliation:
Liver Centre, Toronto Western Hospital, Toronto, Canada; Ehime University Graduate School of Medicine, Japan.
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