Nephrology Center and Okinaka Memorial Institute for Medical Research, Toranomon Hospital Kajigaya, Japan.
Department of Pathology, Toranomon Hospital Kajigaya, Japan.
Intern Med. 2024 Nov 1;63(21):2965-2970. doi: 10.2169/internalmedicine.2485-23. Epub 2024 Feb 1.
A 37-year-old man with autosomal dominant polycystic kidney disease (ADPKD) was admitted to our hospital with a liver volume of 8,000 cm. Hepatic arterial embolization was performed using a microcoil but was ineffective. Eight years later, the hepatomegaly progressed to liver failure and death. At autopsy, the liver weighed 21.5 kg, and the entire liver had been replaced by cysts; in the few remaining areas of liver parenchyma, microscopic, small cysts of various sizes and fibrosis were evident, with only a few normal hepatocytes observed. Hepatic arterial branches developed; however, the portal vein could not be observed.
一名 37 岁男性，患有常染色体显性多囊肾病（ADPKD），因肝脏体积为 8000cm³而入院。使用微线圈进行了肝动脉栓塞，但无效。八年后，肝肿大进展为肝衰竭并死亡。尸检时，肝脏重达 21.5 公斤，整个肝脏已被囊肿取代；在仅剩的少数肝实质区域中，显微镜下可见各种大小的小囊肿和纤维化，仅观察到少数正常肝细胞。肝动脉分支发育；然而，门静脉无法观察到。
