title: Overall Quality of Life in Adult Biliary Atresia Survivors with or without Liver Transplantation: Results from a National Cohort.
abstract: Background Biliary atresia (BA) is a rare cholestatic disease of infancy. Kasai portoenterostomy and liver transplantation (LT) are the two sequential treatment options. An increasing number of patients survive into adulthood. Little is known about their health-related quality of life (HRQOL). This study aims to compare HRQOL of transplanted and nontransplanted patients in a cohort of young adult BA survivors. Patients and Methods RAND-36 and Liver Disease Index Score (LDSI) questionnaires were sent to eligible adult patients with BA. Clinical characteristics were obtained from the NeSBAR (Netherlands Study group on Biliary Atresia Registry) and the national pediatric LT database. RAND-36 domain and summary scores were compared with those of an age-matched Dutch reference group. The correlations between several clinical variables and HRQOL were analyzed. Results Mean RAND-36 domain and summary scores of transplanted (n = 15) and nontransplanted (n = 25) patients with BA (response 74%) were similar to the reference scores, with the exception of a decreased general health perception in nontransplanted patients (63 ± 21 vs. 75 ± 17; [p < 0.001], particularly in females. RAND-36 domain and summary scores were not significantly correlated to age at LT, time since LT, serum bilirubin, aspartate amino transferase or albumin levels, but were moderately to strongly correlated to LDSI total scores (r values 0.35-0.77). Conclusions Overall, young adult patients with BA have a HRQOL similar to an age-matched reference group. However, general health perception of nontransplanted patients, particularly of females, was decreased. HRQOL is correlated to liver disease symptoms but not to liver biochemistry parameters. Nontransplanted females and patients suffering from liver disease-associated symptoms may be a target for tailored supportive interventions.
authors.affiliation: Section of Gastroenterology/Hepatology, Department of Paediatrics, University Medical Center Groningen, Groningen, The Netherlands.
authors.forename: Willemien
authors.initials: W
authors.lastname: de Vries
authors.affiliation: Section of Paediatric Surgery, Department of Surgery, University Medical Center Groningen, Groningen, The Netherlands.
authors.forename: Robert C
authors.initials: RC
authors.lastname: Lind
authors.affiliation: Section of Paediatric Surgery, Department of Surgery, University Medical Center Groningen, Groningen, The Netherlands.
authors.forename: Yuk-Kueng
authors.initials: YK
authors.lastname: Sze
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 Hepatobiliary Surgery and Liver Transplantation, University Medical Center Groningen, Groningen, The Netherlands.
authors.forename: Egbert
authors.initials: E
authors.lastname: Sieders
authors.affiliation: Department of Hepatobiliary Surgery and Liver Transplantation, University Medical Center Groningen, Groningen, The Netherlands.
authors.forename: Robert Jack
authors.initials: RJ
authors.lastname: Porte
authors.affiliation: Section of Gastroenterology/Hepatology, Department of Paediatrics, University Medical Center Groningen, Groningen, The Netherlands.
authors.forename: Henkjan J
authors.initials: HJ
authors.lastname: Verkade
authors.affiliation: Section of Paediatric Surgery, Department of Surgery, University Medical Center Groningen, Groningen, The Netherlands.
authors.forename: Jan B F
authors.initials: JB
authors.lastname: Hulscher
authors.affiliation: Wenckebach Institute, University Medical Center Groningen, Groningen, The Netherlands.
authors.forename: Josette E H M
authors.initials: JE
authors.lastname: Hoekstra-Weebers
mesh_terms: D000328:Adult; D001656:Biliary Atresia / Q000523:psychology* / Q000601:surgery; D015331:Cohort Studies; D003430:Cross-Sectional Studies; D005260:Female; D006304:Health Status; D006801:Humans; D016031:Liver Transplantation; D008297:Male; D009426:Netherlands; D011788:Quality of Life; D012042:Registries; D012720:Severity of Illness Index; D012737:Sex Factors; D018709:Statistics, Nonparametric; D011795:Surveys and Questionnaires; D017741:Survivors / Q000523:psychology* / Q000706:statistics & numerical data; D063189:Symptom Assessment; D055815:Young Adult