Alagille Syndrome
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Surgical biliary diversion in Alagille syndrome raises concerns

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Surgical biliary diversion (SBD) in children with Alagille syndrome (ALGS) appears to be associated with an increased risk of liver transplantation (LT) or death, according to a presentation at The Liver Meeting 2023.

The study, conducted across 26 centers, aimed to evaluate whether SBD alters the natural history of liver disease in patients with ALGS.

The results, based on data from 1673 patients with ALGS, found that 3.7% (n = 62) underwent SBD, with a median age of 2.5 years. The majority opted for a partial external biliary diversion (54.8%), followed by a partial internal biliary diversion (19.4%) and ileal exclusion (12.9%). All SBD patients reported pruritus at the time of the procedure, and 51.4% had xanthomas.

However, the study found that patients with ALGS-SBD faced a 2.5-fold greater risk of liver transplantation (LT) or death compared to those without SBD. There were no significant improvements in total bilirubin (TB), alanine transaminase (ALT), GGT, or cholesterol after SBD. Notably, post-SBD TB levels below 4.0 mg/dL were associated with longer native liver survival (NLS). Despite limited serum bile acids (SBA) data, a subset of patients with a substantial decrease in post-operative SBA levels showed higher rates of NLS.

Vandriel SM, et al. Surgical biliary diversion is associated with an increased risk of liver transplantation or death in Alagille syndrome. Presented at: The Liver Meeting 2023.

Rare Disease 360® is the Official Media Partner and Official Publication of The Alagille Syndrome Alliance (Alagille.org).