Ratcliffe P J, Reeders S, Theaker J M
Br Med J (Clin Res Ed). 1984 May 5;288(6427):1330-1. doi: 10.1136/bmj.288.6427.1330.
A patient with adult polycystic liver and kidney disease presented with haematemesis and melaena and was found to have raised serum creatinine, aspartate transaminase, and alkaline phosphatase values; hypoalbuminaemia; and a prolonged prothrombin ratio. She also had oesophageal varices. With haemodialysis her aspartate transaminase activity fell to normal but she remained hypoalbuminaemic with a prolonged prothrombin ratio. She died after three weeks. Although hepatic cysts do occur in adult polycystic kidney disease, they have been thought not to cause major liver disease. The hepatic cysts in this patient, however, did appear to be associated with portal hypertension and impaired hepatocellular function.
一名患有成人多囊肝和多囊肾病的患者出现呕血和黑便,检查发现其血清肌酐、天冬氨酸转氨酶和碱性磷酸酶值升高;存在低白蛋白血症;凝血酶原比值延长。她还患有食管静脉曲张。经过血液透析,她的天冬氨酸转氨酶活性降至正常,但仍存在低白蛋白血症,凝血酶原比值延长。三周后她去世。虽然肝囊肿确实会出现在成人多囊肾病中,但过去认为它们不会导致严重的肝脏疾病。然而,该患者的肝囊肿似乎与门静脉高压和肝细胞功能受损有关。