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Impaired intention-to-treat survival after listing for liver transplantation in children with biliary atresia compared to other chronic liver diseases: 20 years' experience from the Nordic countries
Karolinska Inst, Karolinska Univ Hosp Huddinge, Astrid Lindgren Childrens Hosp, Dept Pediat Gastroenterol Hepatol & Nutr,CLINTEC, Stockholm, Sweden..
Rigshosp, Dept Pediat & Adolescent Med, Copenhagen, Denmark..
Univ Helsinki, Dept Transplantat Surg, Cent Hosp, Helsinki, Finland..
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2017 (English)In: Pediatric Transplantation, ISSN 1397-3142, E-ISSN 1399-3046, Vol. 21, no 2, e12851Article in journal (Refereed) Published
Abstract [en]

Biliary atresia (BA) is the most common indication for LT in children. We investigated whether this diagnosis per se, compared to other chronic liver diseases (OCLD), had an influence on patient survival. Data from 421 Scandinavian children, 194 with BA and 227 with OCLD, listed for LT between 1990 and 2010 were analyzed. The intention-to-treat survival and influencing risk factors were studied. Patients with BA had higher risk of death after listing than patients with OCLD. The youngest (< 1 year) and smallest (< 10 kg) children with the highest bilirubin (> 510 mu mol/L), highest INR (> 1.6), and highest PELD score (> 20) listed during 1990s had the worst outcome. Given the same PELD score, patients with BA had higher risk of death than patients with OCLD. For adolescents, low weight/BMI was the only prognostic marker. Impaired intention-to-treat survival in patients with BA was mainly explained by more advanced liver disease in younger ages and higher proportion of young children in the BA group rather than diagnosis per se. PELD score predicted death, but seemed to underestimate the severity of liver disease in patients with BA. Poor nutritional status and severe cholestasis had negative impact on survival, supporting the "sickest children first" allocation policy and correction of malnutrition before surgery.

Place, publisher, year, edition, pages
2017. Vol. 21, no 2, e12851
Keyword [en]
biliary atresia, chronic liver disease, intention-to-treat survival, pediatric liver transplantation, PELD score
National Category
Medical and Health Sciences
Identifiers
URN: urn:nbn:se:uu:diva-318942DOI: 10.1111/petr.12851ISI: 000393856700014OAI: oai:DiVA.org:uu-318942DiVA: diva2:1087109
Available from: 2017-04-05 Created: 2017-04-05 Last updated: 2017-04-05Bibliographically approved

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