病理学与病理生理学 泌尿系统疾病 多囊肾双语介绍.doc

多囊肾 该患有DPKD疾病患者的肾脏重量是3公斤。这种疾病为常染色体显性遗传性疾病，因此在家庭中的发病率为50％。这种囊肿在出生时通常不存在，但是随着时间慢慢地生长，因此肾衰竭通常发生在青少年或是成年以后。 病理变化 肉眼形态，常染色体显性遗传多囊肾病人的肾切除外面观及切面观：病变肾脏明显增大，肾内遍布大小不等的囊腔，使肾脏表面呈现球状的突起，疾病后期，大部分肾实质消失，只能在显微镜下看到。 组织学，这是多囊肾在显微镜下显示有一个特征性的大囊肿将立方形的上皮细胞压平并且侵入肾实质内，使肾实质纤维化，带蓝色的软骨岛，很少看到肾小球。 Dominant polycystic kidney disease This kidney in a patient with DPKD weighed 3 kilograms! This disease is inherited with an autosomal dominant pattern, so the recurrence risk in the family is 50%. The cysts are not usually present at birth, but develop slowly over time, so the onset of renal failure occurs in middle age to later adult life. Morphology Grossly, external and cut surface of a nephrectomy specimen from a patient with autosomal dominant polycystic kidney disease：The kidneys are markedly enlarged and have a bosselated outer cortical surface which is produced by multiple cysts of varying sizes . When the disease is severe, normal renal parenchyma is apparent only microscopically. Histopathology, the microscopic appearance of multicystic dysplastic kidney (cystic renal dysplasia, or Potters type II) is characterized by large cysts lined by flattened cuboidal epithelium and an intervening parenchyma that is fibrotic with islands of bluish cartilage and rare glomeruli.