Risk of Variceal Hemorrhage and Pretransplant Mortality in Children with Biliary Atresia

Authors

Lee M Bass
Wen Ye
Kieran Hawthorne
Daniel H Leung
Karen F Murray
Jean P Molleston
Rene Romero
Saul Karpen
Philip Rosenthal
Kathleen M Loomes
Kasper S Wang
Robert H Squires
Alexander Miethke
Vicky L Ng
Simon Horslen
M Kyle Jensen
Ronald J Sokol
John C Magee
Benjamin L Shneider
ChiLDReN

Language

English

Publication Date

9-1-2022

Journal

Hepatology

DOI

10.1002/hep.32451

PMID

35271743

PMCID

PMC9378352

PubMedCentral® Posted Date

4-18-2022

PubMedCentral® Full Text Version

Post-print

Abstract

BACKGROUND AND AIMS: The natural history of gastroesophageal variceal hemorrhage (VH) in biliary atresia (BA) is not well characterized. We analyzed risk factors, incidence, and outcomes of VH in a longitudinal multicenter study.

APPROACH AND RESULTS: Participants enrolled in either an incident (Prospective Database of Infants with Cholestasis [PROBE]) or prevalent (Biliary Atresia Study of Infants and Children [BASIC]) cohort of BA were included. Variceal hemorrhage (VH) was defined based on gastrointestinal bleeding in the presence of varices accompanied by endoscopic or nontransplant surgical intervention. Cumulative incidence of VH and transplant-free survival was compared based on features of portal hypertension (e.g., splenomegaly, thrombocytopenia) and clinical parameters at baseline in each cohort (PROBE: 1.5 to 4.5 months after hepatoportoenterostomy [HPE]; BASIC: at enrollment > 3 years of age). Analyses were conducted on 869 children with BA enrolled between June 2004 and December 2020 (521 in PROBE [262 (51%) with a functioning HPE] and 348 in BASIC). The overall incidence of first observed VH at 5 years was 9.4% (95% CI: 7.0-12.4) in PROBE and 8.0% (5.2-11.5) in BASIC. Features of portal hypertension, platelet count, total bilirubin, aspartate aminotransferase (AST), albumin, and AST-to-platelet ratio index at baseline were associated with an increased risk of subsequent VH in both cohorts. Transplant-free survival at 5 years was 45.1% (40.5-49.6) in PROBE and 79.2% (74.1-83.4) in BASIC. Two (2.5%) of 80 participants who had VH died, whereas 10 (12.5%) underwent transplant within 6 weeks of VH.

CONCLUSIONS: The low risk of VH and associated mortality in children with BA needs to be considered in decisions related to screening for varices and primary prophylaxis of VH.

Keywords

Biliary Atresia, Child, Esophageal and Gastric Varices, Gastrointestinal Hemorrhage, Humans, Hypertension, Portal, Infant, Varicose Veins

Published Open-Access

yes

Recommended Citation

Bass, Lee M; Ye, Wen; Hawthorne, Kieran; et al., "Risk of Variceal Hemorrhage and Pretransplant Mortality in Children with Biliary Atresia" (2022). Faculty and Staff Publications. 3108.
https://digitalcommons.library.tmc.edu/baylor_docs/3108