Churg J, Strauss L, Sherman R L
Birth Defects Orig Artic Ser. 1974;10(4):89-92.
A characteristic electron microscopic lesion-longitudinal splitting of the glomerular basement membranes with accumulation of dark particles-was found in some cases of hereditary nephritis, especially in Alport syndrome. A somewhat similar but less specific alteration was present in the tubular basement membranes and in the Bowman capsule. The lesions tended to exhibit a familial segregation. The possible pathogenesis of the lesions and their relation to physiologic abnormalities are briefly discussed.
在一些遗传性肾炎病例中,尤其是在阿尔波特综合征中,发现了一种特征性的电子显微镜下病变——肾小球基底膜纵向分裂并伴有深色颗粒积聚。肾小管基底膜和鲍曼囊中也存在某种程度上相似但特异性较低的改变。这些病变往往呈现家族性分离现象。本文简要讨论了这些病变可能的发病机制及其与生理异常的关系。