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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., (Georg Thieme Verlag KG Stuttgart · New York.)

Introduction: 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., Conclusion: 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., (Georg Thieme Verlag KG Stuttgart · New York.)

Objective: This prospective follow-up study examines the psychometric properties of the World Health Organization Quality of Life assessment instrument (WHOQOL-100) for assessing quality of life in women suspected of having breast cancer and disease-free breast cancer survivors., Study Design and Setting: The WHOQOL-100 was tested at five points in time in women with a palpable lump in the breast or an abnormality on a screening mammography (N=356) and breast cancer survivors (N=140). Furthermore, all participants completed measures of anxiety (State Trait Anxiety Inventory) and depression (Center for Epidemiologic Studies Depression scale). Moreover, women who were diagnosed with breast cancer also completed the EORTC-QLQ-BR-23 at time points 2-5. Reliability (internal consistency; test-retest reliability) and construct validity were tested., Results: Confirmatory factor analyses on the WHOQOL-100 items showed a good fit with models reflecting six factors (physical health, psychological health, level of independence, social relationships, environment, spirituality/religion/personal beliefs) or four factors (physical health, psychological health, social relationships, environment). Internal consistency was adequate. Test-retest correlations were high. The WHOQOL-100 correlated highly with related constructs and showed low correlations with unrelated constructs., Conclusion: The WHOQOL-100 is a reliable and valid instrument for measuring QOL in women suspected of having breast cancer and disease-free breast cancer survivors.