常染色体隐性遗传性多囊肾病:临床、病理与影像学表现

常染色体隐性多囊肾病是一种以肾集合管和肝内胆管扩张、畸形及肝脏和肾脏纤维化为特点的遗传性疾病。高血压、肾功能不全及门静脉高压是常见的临床症状。影像学表现为肾脏增大但保持肾的外形，超声显示肾实质呈弥漫性回声，静脉肾盂造影及增强CT显示肾脏呈纹状改变。肝脏影像学表现可正常或肝内胆管扩张，门静脉高压常表现脾脏增大和门静脉曲张.

Autosomal recessive polycystic kienay disease:clinical,pathological and radiological correlations

Autsosomal recessive polycysitc kidney disease is a heritable disorder characterized by nonobstrutive renal collecting duct ectasia,hepatic biliary duct rctasia and malformation,and fibrosis of both liver and kidneys.Hypertension and renal insufficiency and portal hypertension are common clinical manifestations.Imaging typically shows large but reniform kidneys,diffusely increased renal parenchymal echogenicity at ultrasonography,and striated nephrogram at CT and intravenoud pyelography.The liver may appear normal or may show intrahepatic biliary dilatation.Splenomegaly and varices are usually evident for portal hypertension.