肝导管减少症是一种病理诊断,其特征是由于各种潜在病因导致肝内胆管数量减少。一些病因,比如原发性硬化性胆管炎,原发性胆汁性胆管炎,缺血性胆管炎,通常有独特的影像学发现。相比之下,其他原因,如肝移植后的慢性排斥反应,药物性胆道损伤,感染,恶性肿瘤如淋巴瘤,移植物抗宿主病可能仅有辅助或非特异性影像学发现。因此,在具有非特异性影像学表现的情况下诊断导管减少症需要多维方法,包括临床评估,血清学检测,成像,和肝脏组织学来确定根本原因。这些病因导致胆汁流动受损,导致胆汁淤积,肝功能障碍,and,最终,肝硬化和肝功能衰竭,如果根本原因仍未治疗或未被发现。在大多数情况下,诊断为导管减少症的个体对解决病因或停止病原体的治疗表现出积极的反应。本文重点介绍了导管减少的获得性原因,其临床表现,组织病理学,影像诊断,和管理。
Hepatic
ductopenia is a pathologic diagnosis characterized by a decrease in the number of intrahepatic bile ducts as a consequence of various underlying etiologies. Some etiologies, such as primary sclerosing cholangitis, primary biliary cholangitis, and ischemic cholangitis, often have distinctive imaging findings. In contrast, other causes such as chronic rejection following liver transplantation, drug-induced biliary injury, infection, malignancy such as lymphoma, and graft-versus-host disease may only have ancillary or non-specific imaging findings. Thus, diagnosing
ductopenia in conditions with nonspecific imaging findings requires a multidimensional approach, including clinical evaluation, serological testing, imaging, and liver histology to identify the underlying cause. These etiologies lead to impaired bile flow, resulting in cholestasis, liver dysfunction, and, ultimately, cirrhosis and liver failure if the underlying cause remains untreated or undetected. In the majority of instances, individuals diagnosed with
ductopenia exhibit a positive response to treatment addressing the root cause or cessation of the causative agent. This article focuses on acquired causes of
ductopenia, its clinical manifestation, histopathology, imaging diagnosis, and management.