Petersen Claus
Department of Pediatric Surgery, Hannover Medical School, Hannover, Germany.
Semin Pediatr Surg. 2012 Aug;21(3):185-91. doi: 10.1053/j.sempedsurg.2012.05.002.
Biliary atresia (BA) is a progressive fibrosing process of the neonatal biliary tree and liver, of unknown origin, and an as-yet unexplained pathologic mechanism. The crucial point is to elucidate the origin of this rare disease to change palliative surgery to etiology-related procedures. Patient-based research can only begin at the time of the Kasai procedure and does not allow retracing of the pathology back to its origin. Basic research has focused on similar diseases in the veterinary literature and started to simulate BA in animal models. Unfortunately, even after 50 years of research, no knowledge has been gained from such models, which has led to a single clinical application. This article reviews BA in the context of the animal models available and discusses whether future studies are promising or futile.
胆道闭锁（BA）是一种新生儿胆管树和肝脏的进行性纤维化过程，病因不明，病理机制也尚未得到解释。关键在于阐明这种罕见疾病的起源，以便将姑息性手术转变为病因相关的手术。基于患者的研究只能在进行葛西手术时开始，无法追溯病理过程至其起源。基础研究聚焦于兽医文献中的类似疾病，并开始在动物模型中模拟BA。不幸的是，即使经过50年的研究，此类模型仍未带来任何有价值的知识，也未促成任何临床应用。本文结合现有的动物模型对BA进行综述，并探讨未来的研究是否有望取得成果或徒劳无功。
