Mukasa C, Nakamura K, Chijiiwa Y, Sakai H, Nawata H
The Third Department of Internal Medicine, Faculty of Medicine, Kyushu University, Fukuoka, Japan.
Am J Gastroenterol. 1998 Mar;93(3):471-3. doi: 10.1111/j.1572-0241.1998.468_3.x.
Hereditary hemorrhagic telangiectasia is a systemic vascular disease with autosomal dominant inheritance that results in telangiectasia, arteriovenous malformations, and hemangiomas. The liver is one of the organs commonly affected in hereditary hemorrhagic telangiectasia, and hepatic lesions consist of angiodysplasia and fibrosis. A patient with hereditary hemorrhagic telangiectasia and significant impairment of synthetic liver function is reported. Dynamic computed tomography revealed marked enlargement of the common hepatic and intrahepatic arteries, heterogeneous parenchymography, and early opacification of the hepatic veins consistent with telangiectasias and arteriovenous shunting. Overall, the liver was predominantly occupied by vascular structures and scarce residual hepatic parenchyma. Other causes of liver dysfunction, such as viral hepatitis and alcohol abuse, were excluded. In general, hepatic fibrovascular dysplasia seen in hereditary hemorrhagic telangiectasia usually results in only mild liver dysfunction; however, this case shows that hepatic involvement may rarely result in hepatic failure.
遗传性出血性毛细血管扩张症是一种常染色体显性遗传的全身性血管疾病，可导致毛细血管扩张、动静脉畸形和血管瘤。肝脏是遗传性出血性毛细血管扩张症常见受累器官之一，肝脏病变包括血管发育异常和纤维化。本文报道了1例患有遗传性出血性毛细血管扩张症且肝功能合成功能严重受损的患者。动态计算机断层扫描显示肝总动脉和肝内动脉明显增粗，实质造影不均匀，肝静脉早期显影，符合毛细血管扩张和动静脉分流表现。总体而言，肝脏主要被血管结构占据，残余肝实质稀少。排除了其他导致肝功能障碍的原因，如病毒性肝炎和酒精滥用。一般来说，遗传性出血性毛细血管扩张症中所见的肝纤维血管发育异常通常仅导致轻度肝功能障碍；然而，该病例表明肝脏受累很少会导致肝衰竭。
