1、常染色体显性遗传多囊肾病囊肿去顶减压术价值的探讨,王滕滕 王新,Contents,多囊肾,多囊肾是是指双侧肾脏发生多个囊肿且进行性长大而导致肾脏结构和功能损害的一种单基因遗传性疾病。 按遗传方式分为两型： 常染色体显性遗传型（成人型） 常染色体隐性遗传型（婴儿型）,常染色体显性遗传多囊肾（ADPKD）,治疗,流行病学,病理改变,临床表现,诊断,ADPKD,流行病学,该病发病率为1/5001/1000,是人类最常见的遗传性肾病,也是人类最常见的威胁生命的单基因疾病；我国目前约有150300万患者。平均发病年龄为40岁，到60 岁时, 50% 患者进入终末期肾衰。该病已成为仅次于高血压、糖尿病、肾
2、小球肾炎引起人类终末期肾病的第四大疾病。疾病。,病理改变（一）,肾脏切面肉眼可见皮质和髓质满布大小不等的囊肿，如一簇葡萄。囊呈球形、圆柱形或梭形，直径0.1至数厘米。,病理改变（二）,囊壁为上皮细胞常有限局性增生，甚至形成息肉。囊内充满液体，外观清亮至血性不等。即使是晚期病例，囊与囊之间仍可以见到少量正常肾组织。,病理改变（三）,镜下观察：囊肿来源于肾小管某一节段、或集合管、或肾小囊，并与它们相通。大的囊肿通常有继发性闭锁。囊壁上皮细胞的基底膜增厚，致密度下降。,常染色体显性遗传多囊肾,ThemeGallery is a Design Digital Content & Contents ma
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