Peña-Pérez Carlos Alberto, Díaz-Ponce-Medrano Juan Alberto
Adult Intensive Therapy Unit. Hospital General Naval de Alta Especialidad, Secretaría de Marina/Armada de México, Mexico City. Mexico.
Medical Management Office. Hospital General Naval de Alta Especialidad, Secretaría de Marina/Armada de México, Mexico City. Mexico.
Cir Cir. 2019;86(1):49-55. doi: 10.24875/CIRUE.M18000008.
Primary sclerosing cholangitis (PSC) is a rare idiopathic condition with immunopathogenic mechanisms where there is chronic progressive destruction of the biliary tree. Secondary sclerosing cholangitis (SSC) is clinically comparable to PSC, but is caused by specific processes which directly damage the biliary tree; examples include recurrent pancreatitis, bile duct malignancy, congenital bile duct abnormalities. A new cause of SSC has been described during or following significant critical illness associated with severe respiratory insufficiency, vasopressor requirement, shock and sepsis. This condition rapidly progresses to cirrhosis, frequently requiring liver transplantation for definitive management.
原发性硬化性胆管炎（PSC）是一种罕见的特发性疾病，具有免疫致病机制，可导致胆管树的慢性进行性破坏。继发性硬化性胆管炎（SSC）在临床上与PSC相似，但由直接损害胆管树的特定过程引起；例如复发性胰腺炎、胆管恶性肿瘤、先天性胆管异常。在与严重呼吸功能不全、血管升压药使用、休克和脓毒症相关的重大危重病期间或之后，已发现一种新的SSC病因。这种情况会迅速发展为肝硬化，通常需要进行肝移植以进行最终治疗。
