Cancer Centre, Faculty of Health Sciences, University of Macau, Macau SAR, China.
Division of Paediatric Surgery, Department of Surgery, School of Clinical Medicine, Li Ka Shing Faculty of Medicine, The University of Hong Kong, Hong Kong SAR, China.
Int J Mol Sci. 2022 Apr 27;23(9):4841. doi: 10.3390/ijms23094841.
Biliary atresia is a severe obliterative cholangiopathy in early infancy that is by far the most common cause of surgical jaundice and the most common indicator for liver transplantation in children. With the advanced knowledge gained from different clinical trials and the development of research models, a more precise clinical classification of BA (i.e., isolated BA (IBA), cystic BA (CBA), syndromic BA (SBA), and cytomegalovirus-associated BA (CMVBA)) is proposed. Different BA subtypes have similar yet distinguishable clinical manifestations. The clinical and etiological heterogeneity leads to dramatically different prognoses; hence, treatment needs to be specific. In this study, we reviewed the clinical characteristics of different BA subtypes and revealed the molecular mechanisms of their developmental contributors. We aimed to highlight the differences among these various subtypes of BA which ultimately contribute to the development of a specific management protocol for each subtype.
先天性胆道闭锁是一种婴儿期严重的阻塞性胆管病，是迄今为止导致手术性黄疸的最常见原因，也是儿童肝移植最常见的指征。随着不同临床试验和研究模型的发展，人们对 BA 的更精确的临床分类（即孤立性 BA（IBA）、囊性 BA（CBA）、综合征性 BA（SBA）和巨细胞病毒相关性 BA（CMVBA））有了更深入的了解。不同的 BA 亚型具有相似但可区分的临床表现。临床和病因学的异质性导致预后明显不同，因此治疗需要具有针对性。在本研究中，我们回顾了不同 BA 亚型的临床特征，并揭示了其发育相关因素的分子机制。我们旨在强调这些不同 BA 亚型之间的差异，最终为每个亚型制定特定的管理方案。
