Middlesworth W, Altman R P
Division of Pediatric Surgery, College of Physicians and Surgeons, Columbia University, Babies and Children's Hospital of New York, NY 10022, USA.
Curr Opin Pediatr. 1997 Jun;9(3):265-9. doi: 10.1097/00008480-199706000-00015.
Biliary atresia, a progressive obliterative process involving the bile ducts, has its onset in the newborn period. It is characterized by worsening cholestasis, hepatic fibrosis, and cirrhosis, which lead to portal hypertension and a decline in hepatic synthetic function. Untreated, the outcome is uniformly fatal. The two major milestones toward improved treatment of this disease have been the Kasai portoenterostomy and orthotopic liver transplantation. There has been discussion regarding transplantation as primary therapy, but portoenterostomy remains the standard of care as first-line intervention. Hepatic transplantation, done more frequently for biliary atresia than for any other cause of liver failure in the pediatric population, offers improved survival and quality of life to those for whom the Kasai operation fails. The etiology of biliary atresia remains poorly understood. Working toward a better understanding of this disease, recent investigations target more precise characterization of the hepatic pathology and seek to identify possible causative agents and predictors of favorable outcome. Recent advances in the understanding of biliary atresia published between December 1995 and November 1996 are the focus of this review.
胆道闭锁是一种累及胆管的进行性闭塞性病变，起病于新生儿期。其特征为胆汁淤积加重、肝纤维化及肝硬化，进而导致门静脉高压和肝脏合成功能下降。若不治疗，结局必然是死亡。改善该病治疗效果的两个主要里程碑是 Kasai 肝门空肠吻合术和原位肝移植。关于将移植作为主要治疗方法一直存在讨论，但肝门空肠吻合术仍是作为一线干预措施的标准治疗方法。在儿科人群中，因胆道闭锁进行肝移植的频率高于因任何其他肝衰竭病因进行肝移植的频率，对于 Kasai 手术失败的患者，肝移植可提高其生存率和生活质量。胆道闭锁的病因仍知之甚少。为更好地了解这种疾病，近期的研究旨在更精确地描述肝脏病理特征，并试图确定可能的致病因素和预后良好的预测指标。本文综述聚焦于 1995 年 12 月至 1996 年 11 月间发表的关于胆道闭锁认识的最新进展。
