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1856年Bristowe首先记述了并发多囊肾的成人多囊肝病(APLD),但直到最近,APLD和成人多囊肾病(APKD)才被认为是一种单基因显性遗传病。有关APKD的基因位于人第16对染色体,然而有些作者指出APLD可以单独发病,这样其基因传递的确切模式还有待于阐明。93%以上的APLD患者合并有APKD,但APKD仅34～78%伴发肝囊肿。本组患者肝内的囊肿大小不一,大者可含数升液体,肝脏增大有时可达盆腔。尽管肝内有大量囊肿形成,CT显示肝实质的体积仍可维持。在疾病早期,有时较难与单纯性肝囊肿(亦可多发)鉴别,因二者
In 1856 Bristowe first described adult polycystic liver disease (APLD) complicated with polycystic kidney disease, but until recently, APLD and adult polycystic kidney disease (APKD) were considered as a single dominant dominant disease. The APKD gene is located on the 16th chromosome of a human, however, some authors state that APLD can develop independently and that its exact pattern of gene delivery remains to be elucidated. More than 93% of APLD patients incorporate APKDs, but APKDs only 34-78% have hepatic cysts. The group of patients with varying sizes of intrahepatic cysts, the larger may contain a few liters of fluid, liver enlargement sometimes up to the pelvis. Despite massive cysts in the liver, CT showed that the volume of the liver parenchyma was maintained. Early in the disease, sometimes more difficult and simple hepatic cysts (also can be multiple) identification, because both