title: Health status and quality of life in adult biliary atresia patients surviving with their native livers.
abstract: BACKGROUND
  Gaining an insight into the quality of life (QOL) in long-term biliary atresia (BA) survivors is becoming more important. Identifying patients with limitations might make tailor made interventions possible. This is the first study investigating the health status (HS) and QOL in adults surviving BA with their native livers, and comparing them with healthy peers.
  
  
  METHODS
  BA patients surviving with their native liver were identified in the Netherlands Study Group on Biliary Atresia Registry database. The RAND-36 and the World Health Organization Quality of Life assessment instrument-100 (WHOQOL-100) were used to measure HS and QOL, respectively. Correlation between the RAND-36 and WHOQOL-100 was also assessed. Hospital Anxiety and Depression Scale (HADS) and the Impact Event Scale (IES) were also completed.
  
  
  RESULTS
  In total, 25 (83%) of the 30 eligible patients after Kasai portoenterostomy completed the questionnaires (median age 23.2 years). A lower perceived level of general health in HS was found as compared with the reference group and a higher score on the social domain was reported in QOL. Correlations between HS and QOL questionnaires were moderate to good. For the group, overall HADS and IES scores were good, though individual patients did score above the cutoff of both the questionnaires.
  
  
  CONCLUSIONS
  Adult BA patients surviving with their native liver have similar HS and QOL as compared with their healthy peers. RAND-36 and WHOQOL-100 questionnaires are not interchangeable, but complementary to assess the patients' outcomes. The overall scores did not point to increased levels of anxiety and depression. However, on an individual level, three and six patients had a score above the cutoff of the HADS subscales demonstrating clinically relevant levels of anxiety and depression, respectively.
authors.affiliation: Department of Pediatric Surgery, University Medical Center Groningen, Groningen, The Netherlands.
authors.forename: Robert C
authors.initials: RC
authors.lastname: Lind
authors.affiliation: Division of Pediatrics, Section of Gastroenterology/Hepatology, University Medical Center Groningen, Groningen, The Netherlands.
authors.forename: Willemien
authors.initials: W
authors.lastname: de Vries
authors.forename: Claudia M G
authors.initials: CM
authors.lastname: Keyzer-Dekker
authors.affiliation: Department of Pediatric Surgery, University Medical Center Groningen, Groningen, The Netherlands.
authors.forename: Claudia M G
authors.initials: CM
authors.lastname: Keyzer
authors.affiliation: Department of Hepatobiliary Surgery and Liver Transplantation, University Medical Center Groningen, Groningen, The Netherlands.
authors.forename: Paul M J G
authors.initials: PM
authors.lastname: Peeters
authors.affiliation: Division of Pediatrics, Section of Gastroenterology/Hepatology, University Medical Center Groningen, Groningen, The Netherlands.
authors.forename: Henkjan J
authors.initials: HJ
authors.lastname: Verkade
authors.affiliation: Wenckebach Institute Groningen, University Medical Center Groningen, Groningen, The Netherlands.
authors.forename: Josette E H M
authors.initials: JE
authors.lastname: Hoekstra-Weebers
authors.affiliation: Department of Pediatric Surgery, Pediatric Surgical Center of Amsterdam, Emma Children's Hospital AMC and VU University Medical Center, Amsterdam, The Netherlands.
authors.forename: Alida F W
authors.initials: AF
authors.lastname: van der Steeg
authors.affiliation: Department of Pediatric Surgery, University Medical Center Groningen, Groningen, The Netherlands.
authors.forename: Jan B F
authors.initials: JB
authors.lastname: Hulscher
mesh_terms: D000293:Adolescent; D000328:Adult; D001007:Anxiety / Q000175:diagnosis / Q000209:etiology; D001656:Biliary Atresia / Q000523:psychology / Q000601:surgery*; D016022:Case-Control Studies; D003863:Depression / Q000175:diagnosis / Q000209:etiology; D005260:Female; D006304:Health Status; D006305:Health Status Indicators; D006801:Humans; D008099:Liver; D008297:Male; D011171:Portoenterostomy, Hepatic / Q000523:psychology*; D011788:Quality of Life; D012042:Registries; D011795:Surveys and Questionnaires; D017741:Survivors / Q000523:psychology*; D055815:Young Adult