Recent advances in the study of etiology of biliary atresia, focusing on the concept of developmental anomaly

DOI
  • Nakamura Kazuaki
    Department of Pharmacology, National Research Institute for Child Health and Development
  • Tanoue Akito
    Department of Pharmacology, National Research Institute for Child Health and Development

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Other Title
  • 胆道閉鎖症の病因に関する最近の知見
  • 特に発生異常説に関して

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Abstract

Biliary atresia (BA), the most common cause of pediatric liver transplantation, remains a challenge for clinicians and investigators. Patient survival and QOL have been greatly improved by liver transplantation compared to the time when hepatoportoenterostomy, or Kasai operation is the only treatment. However, organ transplantation has intrinsic demerits, such as donor shortage and chronic rejection. To develop new therapeutic options, the greater understanding of its pathogenesis is indispensable. The etiology of BA is still unclear, although there is some evidence for viral, toxic,and multiple genetic factors. The fact that the pathology of BA proceeds during a period of biliary growth and remodeling suggests an involvement of developmental anomalies. Recent studies indicate the association of the etiology of BA with some gene functions such as laterality genes,epigenetics regulation and/or miRNAs function. In this review, we present a perspective of advances in the understanding of the disease focusing on bile duct developmental anomaly.

Journal

  • Organ Biology

    Organ Biology 18 (3), 259-264, 2011

    The Japan Society for Organ Preservation and Biology

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