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Alagille Syndrome

Pediatric liver transplant survival in ALGS comparable to biliary atresia

Posted on June 16, 2022

Children with Alagille Syndrome (ALGS) had comparable patient and graft survival to children with biliary atresia (BA) despite having increased rates of congenital heart defects/cardiac interventions, higher creatinine, and higher laboratory MELD/PELD scores at the time of liver transplant, according to a study.

In this linked database analysis, 156 liver transplant recipients with ALGS were identified and age-matched 1:2 to a control group of 312 liver transplant recipients with BA. All children were ≤21 years of age at the time of their first liver transplant.

Overall, 80.7% of children with ALGS had an associated diagnosis of congenital heart disease compared to just 16.4% of children with BA. Cardiac intervention of some type was needed before or after liver transplant in 25.6% (n = 40) of children with ALGS.

Prior to liver transplantation, patients with ALGS had higher creatinine and laboratory MELD and PELD scores.

There was no difference found regarding patient or graft survival between children with ALGS and children with BA (P = 0.08 and P = 0.27, respectively).

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. 2022;doi: 10.1097/MPG.0000000000003522. Epub ahead of print. PMID: 35696710.

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