Geonzon-Gonzales Mariben R
Orthopedic Trauma Unit, Thomas Jefferson University Hospital, Philadelphia, Pennsylvania, USA.
Gastroenterol Nurs. 2007 Mar-Apr;30(2):102-5; quiz 105-7. doi: 10.1097/01.SGA.0000267929.62298.64.
Primary sclerosing cholangitis is a chronic cholestatic liver disease of unknown origin characterized by progressive inflammation, destruction, and fibrosis of the intrahepatic and extrahepatic bile ducts. The disease leads to obliteration of intrahepatic bile ducts and to biliary cirrhosis, end-stage liver disease, and portal hypertension. Primary sclerosing cholangitis commonly occurs in the presence of inflammatory bowel disease. Its exact etiology remains unknown. As a result, there is no existing effective medical management to delay or modify the progression of the disease. Ursodeoxycholic acid, the most well-studied drug for primary sclerosing cholangitis, has demonstrated promising results when used in combination with an immunosuppressant or antibiotic. To date, liver transplantation remains the only confirmed long-term treatment of primary sclerosing cholangitis, which now accounts for 6% of adult and 1% of pediatric liver transplantations in the United States. Primary sclerosing cholangitis represents an important liver disease with major morbidity and mortality.
原发性硬化性胆管炎是一种病因不明的慢性胆汁淤积性肝病，其特征为肝内和肝外胆管进行性炎症、破坏和纤维化。该疾病导致肝内胆管闭塞，并引发胆汁性肝硬化、终末期肝病和门静脉高压。原发性硬化性胆管炎通常在炎症性肠病存在的情况下发生。其确切病因仍不清楚。因此，目前尚无有效的药物治疗方法来延缓或改变疾病的进展。熊去氧胆酸是对原发性硬化性胆管炎研究最多的药物，与免疫抑制剂或抗生素联合使用时已显示出有前景的结果。迄今为止，肝移植仍然是原发性硬化性胆管炎唯一确定的长期治疗方法，在美国，原发性硬化性胆管炎导致的肝移植占成人肝移植的6%，儿童肝移植的1%。原发性硬化性胆管炎是一种具有重大发病率和死亡率的重要肝病。
