1、常染色體顯性遺傳多囊腎病囊腫去頂減壓術(shù)價值的探討,王滕滕 王新,Contents,多囊腎,多囊腎是是指雙側(cè)腎臟發(fā)生多個囊腫且進行性長大而導致腎臟結(jié)構(gòu)和功能損害的一種單基因遺傳性疾病。 按遺傳方式分為兩型： 常染色體顯性遺傳型（成人型） 常染色體隱性遺傳型（嬰兒型）,常染色體顯性遺傳多囊腎（ADPKD）,治療,流行病學,病理改變,臨床表現(xiàn),診斷,ADPKD,流行病學,該病發(fā)病率為1/5001/1000,是人類最常見的遺傳性腎病,也是人類最常見的威脅生命的單基因疾?。晃覈壳凹s有150300萬患者。平均發(fā)病年齡為40歲，到60 歲時, 50% 患者進入終末期腎衰。該病已成為僅次于高血壓、糖尿病、腎

2、小球腎炎引起人類終末期腎病的第四大疾病。疾病。,病理改變（一）,腎臟切面肉眼可見皮質(zhì)和髓質(zhì)滿布大小不等的囊腫，如一簇葡萄。囊呈球形、圓柱形或梭形，直徑0.1至數(shù)厘米。,病理改變（二）,囊壁為上皮細胞常有限局性增生，甚至形成息肉。囊內(nèi)充滿液體，外觀清亮至血性不等。即使是晚期病例，囊與囊之間仍可以見到少量正常腎組織。,病理改變（三）,鏡下觀察：囊腫來源于腎小管某一節(jié)段、或集合管、或腎小囊，并與它們相通。大的囊腫通常有繼發(fā)性閉鎖。囊壁上皮細胞的基底膜增厚，致密度下降。,常染色體顯性遺傳多囊腎,ThemeGallery is a Design Digital Content & Contents ma

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