title: 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.
abstract: 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 μmol/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.
authors.affiliation: Department of Pediatric Gastroenterology, Hepatology and Nutrition, Astrid Lindgren Children's Hospital, CLINTEC, Karolinska Institute, Karolinska University Hospital Huddinge, Stockholm, Sweden.
authors.forename: S
authors.initials: S
authors.lastname: Malenicka
authors.affiliation: Division of Transplantation Surgery, CLINTEC, Karolinska Institute, Karolinska University Hospital Huddinge, Stockholm, Sweden.
authors.forename: B-G
authors.initials: BG
authors.lastname: Ericzon
authors.affiliation: Department of Pediatric and Adolescent Medicine, Rigshospitalet, Copenhagen, Denmark.
authors.forename: M H
authors.initials: MH
authors.lastname: Jørgensen
authors.affiliation: Department of Transplantation Surgery, Helsinki University Central Hospital, Helsinki, Finland.
authors.forename: H
authors.initials: H
authors.lastname: Isoniemi
authors.affiliation: Department of Gastroenterology, Oslo University Hospital, Rikshospitalet, Oslo, Norway.
authors.forename: T H
authors.initials: TH
authors.lastname: Karlsen
authors.affiliation: Department of Pediatric Gastroenterology, Hepatology and Nutrition, The Queen Silvia Children's Hospital, Gothenburg, Sweden.
authors.forename: M
authors.initials: M
authors.lastname: Krantz
authors.affiliation: Medical Faculty, Rigshospitalet, Copenhagen, Denmark.
authors.forename: V
authors.initials: V
authors.lastname: Naeser
authors.affiliation: Department of Transplantation Surgery, Sahlgrenska University Hospital, Gothenburg, Sweden.
authors.forename: M
authors.initials: M
authors.lastname: Olausson
authors.affiliation: Department of Transplantation Surgery, Rigshospitalet, Copenhagen, Denmark.
authors.forename: A
authors.initials: A
authors.lastname: Rasmussen
authors.affiliation: Department of Pediatric Nephrology, Helsinki University Central Hospital, Helsinki, Finland.
authors.forename: K
authors.initials: K
authors.lastname: Rönnholm
authors.affiliation: Department of Pediatric Gastroenterology, Oslo University Hospital, Rikshospitalet, Oslo, Norway.
authors.forename: T
authors.initials: T
authors.lastname: Sanengen
authors.affiliation: Department of Transplantation Surgery, Uppsala University Hospital, Uppsala, Sweden.
authors.forename: T
authors.initials: T
authors.lastname: Scholz
authors.affiliation: Department of Pediatric Gastroenterology, Hepatology and Nutrition, Astrid Lindgren Children's Hospital, CLINTEC, Karolinska Institute, Karolinska University Hospital Huddinge, Stockholm, Sweden.
authors.forename: B
authors.initials: B
authors.lastname: Fischler
authors.affiliation: Department of Pediatric Gastroenterology, Hepatology and Nutrition, Astrid Lindgren Children's Hospital, CLINTEC, Karolinska Institute, Karolinska University Hospital Huddinge, Stockholm, Sweden.
authors.forename: A
authors.initials: A
authors.lastname: Nemeth
mesh_terms: D000293:Adolescent; D001656:Biliary Atresia / Q000401:mortality* / Q000601:surgery*; D015992:Body Mass Index; D002648:Child; D002675:Child, Preschool; D005260:Female; D006085:Graft Survival; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D057194:Intention to Treat Analysis; D019934:International Normalized Ratio; D017093:Liver Failure / Q000401:mortality* / Q000601:surgery*; D016031:Liver Transplantation; D008297:Male; D015999:Multivariate Analysis; D009752:Nutritional Status; D011379:Prognosis; D011446:Prospective Studies; D012042:Registries; D012307:Risk Factors; D012537:Scandinavian and Nordic Countries; D061665:Time-to-Treatment; D016896:Treatment Outcome
keywords: PELD score; biliary atresia; chronic liver disease; intention-to-treat survival; pediatric liver transplantation