Bach Nancy, Odin Joseph A
Division of Liver Diseases, Department of Medicine, Box 1123, Mount Sinai School of Medicine, One East 100th Street, New York, NY 10029, USA.
Mt Sinai J Med. 2003 Sep;70(4):242-50.
Individuals afflicted with primary biliary cirrhosis (PBC) first undergo chronic, nonsuppurative destruction of their intrahepatic bile ducts, eventually leading to cirrhosis. Over nearly 50 years, many faculty members at the Mount Sinai School of Medicine, including Dr. Hans Popper and Dr. Fenton Schaffner, have made important contributions to our understanding of the natural history and histopathologic evolution of PBC. And today, many patients with PBC continue to be cared for at Mount Sinai. In the absence of a cure for the disease, these patients continue to be enrolled in clinical trials and, when necessary, in the Mount Sinai liver transplant program. The establishment of the Center for the Study of Primary Biliary Cirrhosis at Mount Sinai, supported by the Artzt Family Foundation Trust, has enabled the faculty to expand both clinical and basic science initiatives related to primary biliary cirrhosis. Several of these new initiatives are described below and placed in the context of our current understanding of the immunopathogenesis of PBC.
原发性胆汁性肝硬化（PBC）患者首先经历肝内胆管的慢性非化脓性破坏，最终导致肝硬化。在近50年的时间里，西奈山医学院的许多教员，包括汉斯·波珀博士和芬顿·沙夫纳博士，为我们理解PBC的自然史和组织病理学演变做出了重要贡献。如今，许多PBC患者仍在西奈山接受治疗。由于该疾病无法治愈，这些患者继续参加临床试验，并在必要时加入西奈山肝移植项目。在阿茨特家族基金会信托基金的支持下，西奈山原发性胆汁性肝硬化研究中心的成立使教员们能够扩大与原发性胆汁性肝硬化相关的临床和基础科学研究项目。以下将介绍其中的一些新举措，并结合我们目前对PBC免疫发病机制的理解进行阐述。
