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多囊肾是一种先天性肾脏发育反常,常合并有其它器官畸形。婴儿型为常染色体隐性遗传,成人型为常染色体显性遗传。尸检发病率为1:222～1:1019,并不罕见,而且,仅次于肾炎、肾盂肾炎及动脉硬化肾而为慢性肾功能衰竭常见原因之一。但国内报告尚少,1952年至1980年仅共报告36例。本文报告我院1972～1981年住院的先天性多囊肾18例,并结合文献进行讨论。
Polycystic kidney disease is a congenital kidney abnormalities, often associated with other organ deformities. Infant type is autosomal recessive, adult type is autosomal dominant. Autopsy incidence of 1: 222-1: 1019, is not uncommon, and, second only to nephritis, pyelonephritis and atherosclerotic kidney and chronic renal failure is one of the common causes. However, there are still few domestic reports, with only 36 cases reported from 1952 to 1980. This article reports 18 cases of congenital polycystic kidney disease hospitalized in our hospital from 1972 to 1981, combined with the literature to discuss.