Systematic review: The quality of life of patients with biliary atresia

Abstract

Background: The quality of life of patients with Biliary Atresia (BA) have not been systematically examined. The goal of this meta-analysis is to determine patients' postoperative health-related Quality of life (HrQoL) with native or transplanted livers.

Methods: From 2000 to August 2021, a literature-based search for relevant cohorts was conducted using Pubmed/Medline, the Cochrane Library, and Embase. Original research on BA, Hepatoportoenterostomy (HPE), portoenterostomy, Kasai, Liver transplantation and HrQoL was included. Using RevMan, a forest plot analysis of HrQoL after surgical treatment after BA was calculated (version 5.4). Using MetaXL, a pooled prevalence for cholangitis, secondary liver transplantation, or related malformations was computed (version 5.3).

Results: Nine studies compared individuals with BA to an age-matched healthy control group. 4/9 (n = 352) of these studies found poorer scores for BA patients, while 5/9 (n = 81) found equivalent health status. Factors associated with HrQoL: older age at the time of the survey was linked to greater HrQoL; whereas females, higher total bilirubin and the amount of immunosuppressive medicines were associated with lower HrQoL in BA patients.

Conclusion: The current study emphasises the critical need to improve the many parameters influencing HrQoL in BA patients, as well as the methods utilized to assess those factors. This includes immunosuppression, withdrawal from polydrug regimes and recognizing the differences in disease burden between males and females.

Type of study: Systematic review.

Level of evidence: Level III.

Keywords: Biliary atresia; Health-related quality of life; Kasai hepatoportoenterostomy; Liver transplantation; Pediatrics.