原发性胆汁性肝硬化（ＰＢＣ）是一种缓慢进展的肝脏疾患，可引起胆道发炎、胆汁酸瘀积、肝硬化并最终导至肝功能衰竭。胆汁酸的分泌减少引起维生素Ａ、Ｄ、Ｅ、Ｋ的吸收障碍，从而造成维生素缺乏、高胆固醇血症、高脂血症、脂肪泻和骨质疏松症。 ＰＢＣ的病因不明，但可能与自 Primary biliary cirrhosis (PBC) is a slow-progressing liver disorder that causes biliary inflammation, bile acid stasis, cirrhosis and eventually liver failure. Reduced secretion of bile acids causes malabsorption of vitamins A, D, E, K, resulting in vitamin deficiency, hypercholesterolemia, hyperlipidemia, steatorrhea and osteoporosis. The cause of PBC is unknown, but may be related to self