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Acute-on-chronic liver failure in children with biliary atresia awaiting liver transplantation
Rashmi D'Souza1, Tassos Grammatikopoulos1, Akhilesh Pradhan2
1Paediatric Liver, Gastroenterology and Nutrition Centre, King's College Hospital, London, UK.
Insights
Acute-on-chronic liver failure (ACLF) in children with biliary atresia (BA) significantly increases mortality while awaiting liver transplantation (LT). Early identification and prioritization for LT are crucial for improved outcomes in these pediatric patients.
Area of Science:
- Pediatric Gastroenterology and Hepatology
- Transplantation Immunology
- Critical Care Medicine
Background:
- Biliary atresia (BA) is the leading indication for pediatric liver transplantation (LT) worldwide.
- Acute-on-chronic liver failure (ACLF) is a severe complication of cirrhosis with high mortality, but its occurrence in pediatric BA is understudied.
- Understanding ACLF in pediatric BA is critical for improving transplant outcomes.
Purpose of the Study:
- To evaluate the incidence and outcomes of ACLF in children with BA awaiting deceased donor LT.
- To identify predictors of ACLF development in this population.
- To assess the impact of ACLF on mortality and morbidity.
Main Methods:
- Subanalysis of a prospective cohort study of pediatric BA patients (0-18 years) undergoing portoenterostomy and listed for LT.
- Follow-up for development of ACLF, mortality, and complications.
- Analysis of biochemical parameters and clinical factors predictive of ACLF.
Main Results:
- Twenty of 99 children developed ACLF, associated with significantly higher mortality (20% vs. 4%) while awaiting LT.
- Elevated bilirubin levels 3 months post-portoenterostomy predicted ACLF development.
- Sepsis and gastrointestinal bleeding were common precipitants; ACLF patients required intensive care and experienced more complications.
Conclusions:
- ACLF in pediatric BA patients awaiting LT is linked to increased mortality and morbidity.
- Stratification for earlier wait-listing and LT prioritization is warranted.
- Further research into ACLF management in pediatric BA is essential.
Objectives:
Acute-on-chronic liver failure (ACLF) is an acute decompensation of cirrhosis complicated by other organ failure and is associated with increased mortality and morbidity. ACLF has not been studied in children with biliary atresia (BA), which is the commonest indication for pediatric liver transplantation (LT) worldwide. This study aims to evaluate ACLF and outcomes in children with BA while awaiting deceased donor LT.
Methods:
This was a subanalysis of the dataset from a prospective cohort study of patients aged 0-18 years who underwent portoenterostomy for BA and were listed for LT at King's College Hospital, London, between 1999 and 2003. Outcomes included the development of ACLF, mortality, and complications.
Results:
Ninety-nine (41 male) children were included, and follow-up was 10 [6.0-15.0] years. A total of 20/99 children developed ACLF. ACLF was associated with increased mortality while awaiting LT (20% vs 4%; P = 0.03). There were no associations between biochemical parameters at listing and death. Increased bilirubin levels 3 months post-portoenterostomy was predictive of development of ACLF (AUROC = 0.72, P < 0.01). Age at LT and time on the waiting list in the ACLF subgroup were both lower compared to the non-ACLF group (P > 0.05). Sepsis and gastrointestinal bleeding were the commonest precipitants of ACLF. Complications included ascites, hepatic encephalopathy, and hepatorenal syndrome; the ACLF subgroup required multisystem support and longer intensive care unit stay.
Conclusions:
ACLF in children with BA awaiting deceased donor LT carries increased mortality and morbidity. This warrants stratification of patients for earlier wait-listing and prioritization for LT.
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