Department of Hepatobiliary Surgery and Organ Transplantation Center, The First Affiliated Hospital of of USTC, Division of Life Science and Medicine, University of Science and Technology of China, Hefei, Anhui, China.
Department of Pathology, the First Affiliated Hospital of University of Science and Technology of China, He Fei, Anhui, China.
Transplant Proc. 2024 Jun;56(5):1161-1164. doi: 10.1016/j.transproceed.2024.05.012. Epub 2024 May 29.
Caroli disease (CD) is a congenital disease of the intrahepatic biliary system, which manifests as cystic dilatation of the intrahepatic bile ducts. The disease has a low incidence and atypical clinical manifestations; therefore, it can be easily misdiagnosed. Hepatitis B infection is a viral infection that affects liver cells, leading to degeneration, necrosis, and regeneration of the cells and formation of false lobules, and ultimately nodular cirrhosis, which can lead to liver dysfunction and liver failure. Herein, we report a case of decompensated liver cirrhosis because of a diffuse form of CD, which was misdiagnosed because of long-term hepatitis B virus (HBV) infection. Finally, orthotopic liver transplantation (OLT) was performed, and the patient was cured. We believe that this congenital factor combined with HBV infection accelerated cirrhosis progression in this patient. This transplant was carried out in accordance with the Helsinki Congress and the Declaration of Istanbul.
先天性肝内胆管囊状扩张症（Caroli 病，CD）是一种先天性肝内胆管疾病，表现为肝内胆管囊性扩张。该病发病率低，临床表现不典型，易误诊。乙型肝炎病毒（hepatitis B virus，HBV）感染是一种影响肝细胞的病毒感染，导致肝细胞变性、坏死和再生，形成假小叶，最终导致结节性肝硬化，导致肝功能衰竭。本研究报告了一例因弥漫型 CD 导致肝功能失代偿的病例，因长期 HBV 感染而误诊。最终，患者接受了原位肝移植（orthotopic liver transplantation，OLT），治愈。我们认为，这种先天性因素与 HBV 感染共同加速了患者的肝硬化进展。该移植符合赫尔辛基大会和伊斯坦布尔宣言的规定。
