ArticleJGH open : an open access journal of gastroenterology and hepatology2022
Different clinical and genetic features of Alagille patients with progressive disease
Article in JGH open : an open access journal of gastroenterology and hepatology, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 4 papers.
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4 citing papers in PubMed, 5 citations in OpenAlex.
- Alagille syndrome case series: five new variants and two large deletions.European journal of pediatrics · 2026Article
- Native liver survival and genetic associations in Korean patients with Alagille syndrome.European journal of pediatrics · 2026Article
- Clinical and genetic characteristics of patients with Alagille syndrome in China: identification of six novelTranslational pediatrics · 2024Article
- Clinical Characterization of Alagille Syndrome in Patients with Cholestatic Liver Disease.International journal of molecular sciences · 2023Article
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Authors and funding
14 authors at 5 institutions in 1 country.
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No grant is acknowledged in the PubMed record.
Abstract
Background and Aim: Alagille syndrome (ALGS) is a multisystem disorder with variable clinical courses. This study investigated the clinical and genetic features of ALGS patients with different outcomes and analyzed the liver pathology at liver transplantation (LT) compared with that in biliary atresia (BA). Methods: We report the clinical characteristics, outcomes, and genetic mutations of 25 children with ALGS followed for a median of 7.3 years. Patients were classified into (i) jaundice-free (JF) group (resolving jaundice after 2 years of age); (ii) progressive disease (PD) group (persistent jaundice or progressive cholestasis). In addition, we analyzed the explant liver in 10 ALGS patients compared with 20 age-matched BA patients at the time of LT. Results: Nine patients (36%) in the JF group had a favorable outcome, with longer native liver survival than patients with PD ( Conclusion: One-third of ALGS patients had JF and a favorable course. Children with ALGS presenting with persistent jaundice beyond 2 years of age should be cautioned for poor prognosis. ALGS patients tend to have a lesser extent of cirrhosis, and more growth problems than BA patients at the time of LT.
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