Patient survival and graft survival rates following liver transplantation are similar between patients with Alagille syndrome (ALGS) and those with biliary atresia, according to a new study published in the Journal of Pediatric Gastroenterology and Nutrition.

This is the case despite higher congenital heart defect and cardiac intervention rates, creatinine levels, and model for end-stage liver disease (MELD) and pediatric end-stage liver disease (PELD) scores at the time of the transplantation in patients with ALGS.

Around one-third of patients with ALGS end up needing a liver transplant, which can be associated with significant morbidity. Whether a patient is eligible for transplantation is partly based on the evaluation of heart and kidney involvement.


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To clarify liver transplant outcomes and heart disease manifestations in children with ALGS, a team of researchers led by Lynette A. Gillis, MD, from the Division of Pediatric Gastroenterology, Hepatology, and Nutrition at Vanderbilt University Medical Center in Nashville, Tennessee, conducted a database analysis using a novel linkage between the Scientific Registry of Transplant Recipients and Pediatric Health Information System databases.

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The researchers identified 156 patients with ALGS under the age of 21 years and 312 age-matched patients with biliary atresia who underwent liver transplantation between 2002 and 2018.

The team found that the majority (80.7%) of patients with ALGS also had congenital heart disease. In contrast, only 16.4% of patients with biliary atresia had associated congenital heart disease. 

A little more than a quarter of patients with ALGS (25.6%) required cardiac interventions such as catheterization or surgery, either before or after liver transplantation. Finally, patients with ALGS had higher creatinine levels and laboratory MELD and PELD scores before the transplant. 

Despite these differences, the researchers reported no difference in terms of patient or graft survival between patients with ALGS and those with biliary atresia.

Reference

Black K, Ziogas IA, Thurm C, et al. Pediatric liver transplant survival in Alagille syndrome is comparable to biliary atresia- a linked database analysis. J Pediatr Gastroenterol Nutr. Published online June 13, 2022. doi:10.1097/MPG.0000000000003522