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遗传性肾炎中的肾小球基底膜。

The glomerular basal lamina in hereditary nephritis.

作者信息

Yoshikawa N, Cameron A H, White R H

出版信息

J Pathol. 1981 Nov;135(3):199-209. doi: 10.1002/path.1711350305.

Abstract

Characteristic ultrastructural alterations of the glomerular basal lamina have been reported in hereditary nephritis. The basal lamina is irregularly thickened and the lamina densa shows replication with a "basket weave" pattern, enclosing electron-lucent lacunae which frequently contain small dense particles. However there is controversy regarding the specificity of this lesion in hereditary nephritis. To determine the specificity, 366 renal biopsies from 310 children were studied retrospectively. Twenty-four out of 27 patients with hereditary nephritis showed the characteristic changes of the basal lamina and they were widespread in 17. Two patients with recurrent haematuria but no family history of deafness or haematuria showed similar extensive changes and are regarded as new mutant cases of hereditary nephritis. Similar changes were seen in 17 of the 281 patients with other conditions but were always localized to a few capillary loops. We conclude that a widespread "basket weave" pattern appears to be confined to hereditary nephritis and is seen in the great majority of such cases.

摘要

遗传性肾炎中已报道了肾小球基底膜特征性的超微结构改变。基底膜不规则增厚,致密层呈现“篮状编织”模式的复制,包绕着电子透亮的腔隙,这些腔隙常含有小的致密颗粒。然而,关于这种病变在遗传性肾炎中的特异性存在争议。为了确定其特异性,我们对310名儿童的366份肾活检标本进行了回顾性研究。27例遗传性肾炎患者中有24例表现出基底膜的特征性改变,其中17例改变广泛。2例复发性血尿但无耳聋或血尿家族史的患者也表现出类似的广泛改变,被视为遗传性肾炎的新突变病例。281例患有其他疾病的患者中有17例也出现了类似改变,但总是局限于少数毛细血管袢。我们得出结论,广泛的“篮状编织”模式似乎仅限于遗传性肾炎,并且在大多数此类病例中可见。

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