493 A systematic review of social outcomes in patients with biliary atresia

Abstract

<h3>Background</h3> Biliary atresia is an inflammatory obliterative cholangiopathy which presents in early infancy with obstructive jaundice. It is the most common indication for liver transplant in childhood. Following improved medical outcomes for children with biliary atresia, a new clinical and research focus is on promoting long-term well-being and quality of life. However, information on social outcomes, including markers of societal integration such as education, employment, and family outcomes, are inconsistently documented in existing studies, and have never previously been systematically reviewed. <h3>Objectives</h3> We aimed to systematically review the social outcomes of patients with biliary atresia (BA) including education, employment, family and social functioning outcomes. <h3>Methods</h3> A systematic review of four databases (Medline, Embase, Global Health, Maternity and Infant Care Database) was conducted in April 2020, supplemented by reference searching, with NHLBI scoring for quality appraisal. Each abstract and full text was independently reviewed by two researchers, and quality appraisal was independently conducted by two researchers. The protocol was registered on PROSPERO (CRD42020178846). <h3>Results</h3> 55 studies met inclusion criteria (39 cohort, 11 cross-sectional, five case-control), including 5,156 participants from 16 countries. BA post liver transplant (LT) (18 studies), native liver survival (NLS) (16 studies), mixed LT/NLS (16 studies) and 5 of other cohorts (e.g. listed for LT) were incorporated. Study outcomes included; education (n=39), employment (n=19), pregnancy (n=24), and social functioning (n=25). Patients with BA had lower school functioning scores than controls, with no difference when comparing those with and without LT. The need for additional educational support ranged between 2% and 48% of children. Adult patients with BA were employed at high rates (range 60–100%), with no difference between post-LT and NLS cohorts when compared in 1 study. Successful pregnancies were reported in 19 studies, with mostly small sample sizes. In 6/7 NLS studies mentioning pregnancy, complications included derangement in liver function and variceal bleeding secondary to portal hypertension. Social functioning scores did not significantly differ from healthy controls when compared in 7/9 studies. <h3>Conclusions</h3> Existing evidence on social outcomes for children and adolescents with BA is predominantly from non-controlled, single centre studies and based on parental surveys. The current findings suggest that school functioning is lower compared to peer groups and not influenced by having a liver transplant. Employment outcomes are encouraging and pregnancy considered an at-risk period. These findings provide justification for routine psychosocial assessment of these patients during follow up and larger multi-centre collaborations in order to develop the evidence base for future patients.