常染色体隐性遗传多囊性肾病(ARPKD)是一种遗传性囊性肾脏病。估计其发生率在1/10000至1/40000。发病率的差异与所研究的人群和方法不同有关。患者常在胎儿和婴儿期出现肾功能不全,但临床表现也可延迟发生。患者表现为肾脏囊肿和肝脏纤维化。在年青成人出现典型的ARPKD的临床表现,而肾脏表现在胎儿和婴儿也可发现。 Autosomal recessive polycystic kidney disease (ARPKD) is a hereditary cystic kidney disease. The estimated rate of occurrence is between 1/10000 and 1/40000. The differences in morbidity are related to different populations and methods studied. Patients often have renal insufficiency in the fetus and infancy, but clinical manifestations can also be delayed. Patients showed renal cysts and liver fibrosis. In young adults typical clinical manifestations of ARPKD appear, while kidney manifestations are also detectable in fetuses and infants.