Course of Life Into Adulthood of Patients With Biliary Atresia: The Achievement of Developmental Milestones in a Nationwide Cohort

Potgieser, Adriaan R E; Vries, Willemien de; Sze, Yuk K.; Sieders, Egbert; Verkade, Henkjan J.; Porte, Robert J.; Hoekstra-Weebers, Josette E. H. M.; Hulscher, Jan B F

doi:10.1016/j.jadohealth.2011.10.007

Cited by 7 publications

(2 citation statements)

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“…Adult listing criteria might not be relevant to childhood liver diseases. Data on course of life in young adults transplanted in childhood show delay in reaching developmental milestones, but less risky behavior with regards to substance abuse and gambling compared to the general population[109]. Nonadherence to treatment is a major concern in the post-transplant setting and the long-term graft andpatient survival in patients transplanted between the ages of 12-17 years is poorer compared to the younger population.…”

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confidence: 99%

Biliary atresia and other cholestatic childhood diseases: Advances and future challenges

Verkade

Bezerra

Davenport

et al. 2016

Journal of Hepatology

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141

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Biliary Atresia and other cholestatic childhood diseases are rare conditions affecting the function and/or anatomy along the canalicular-bile duct continuum, characterised by onset of persistent cholestatic jaundice during the neonatal period. Biliary atresia (BA) is the most common among these, but still has an incidence of only 1 in 10-19,000 in Europe and North America. Other diseases such as the genetic conditions, Alagille syndrome (ALGS) and Progressive Familial Intrahepatic Cholestasis (PFIC), are less common. Choledochal malformations are amenable to surgical correction and require a high index of suspicion. The low incidence of such diseases hinder patient-based studies that include large cohorts, while the limited numbers of animal models of disease that recapitulate the spectrum of disease phenotypes hinders both basic research and the development of new treatments. Despite their individual rarity, collectively BA and other cholestatic childhood diseases are the commonest indications for liver transplantation during childhood. Here, we review the recent advances in basic research and clinical progress in these diseases, as well as the research needs. For the various diseases, we formulate current key questions and controversies and identify top priorities to guide future research.

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confidence: 99%

Biliary atresia and other cholestatic childhood diseases: Advances and future challenges

Verkade

Bezerra

Davenport

et al. 2016

Journal of Hepatology

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141

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“…Detailed demographic information was described previously. 24 The study was performed according to the guidelines of the Medical Ethical Committee of the University Medical Center Groningen. A letter explaining the goal of the study, the questionnaires, an informed consent form, and a prepaid return envelope were sent by post to all eligible patients.…”

Section: Patients and Proceduresmentioning

confidence: 99%

Overall Quality of Life in Adult Biliary Atresia Survivors with or without Liver Transplantation: Results from a National Cohort

et al. 2015

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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.

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Biliary Atresia in Transition Hepatology

Jain,

Alexander,

Burford

2022

In Clinical Practice

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Course of Life Into Adulthood of Patients With Biliary Atresia: The Achievement of Developmental Milestones in a Nationwide Cohort

Cited by 7 publications

References 9 publications

Biliary atresia and other cholestatic childhood diseases: Advances and future challenges

Biliary atresia and other cholestatic childhood diseases: Advances and future challenges

Overall Quality of Life in Adult Biliary Atresia Survivors with or without Liver Transplantation: Results from a National Cohort

Biliary Atresia in Transition Hepatology

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