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<p>The Japanese Biliary Atresia Registry, reported by Okubo et al., provides a comprehensive long-term perspective on biliary atresia (BA) across 3951 patients (1989–2023). With jaundice clearance rates exceeding 60% and 10-year native liver survival of 50.5%, outcomes in Japan remain consistently superior to those reported in Western cohorts. These disparities are likely multifactorial. BA represents a heterogeneous phenotype shaped by genetic, immunological, environmental, and developmental factors that may differ across populations. The lower prevalence of biliary atresia splenic malformation syndrome in Japan (2.2% vs. 8–15%) may partly contribute but does not fully explain the gap. Differences in management strategies, including earlier surgery, greater use of revision Kasai portoenterostomy, and adjuvant therapies, may also play a key role.</p>

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International disparities in biliary atresia outcomes

  • 期刊论文

  • 发表日期：

    2026年4月21日

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  • Rani Kassir,
  • Grégoire Schneider,
  • Rémi Dubois,
  • Guillaume Rossignol

摘要

The Japanese Biliary Atresia Registry, reported by Okubo et al., provides a comprehensive long-term perspective on biliary atresia (BA) across 3951 patients (1989–2023). With jaundice clearance rates exceeding 60% and 10-year native liver survival of 50.5%, outcomes in Japan remain consistently superior to those reported in Western cohorts. These disparities are likely multifactorial. BA represents a heterogeneous phenotype shaped by genetic, immunological, environmental, and developmental factors that may differ across populations. The lower prevalence of biliary atresia splenic malformation syndrome in Japan (2.2% vs. 8–15%) may partly contribute but does not fully explain the gap. Differences in management strategies, including earlier surgery, greater use of revision Kasai portoenterostomy, and adjuvant therapies, may also play a key role.