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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.

DOI:10.1002/path.1711350305
PMID:7320813
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。

相似文献

1
The glomerular basal lamina in hereditary nephritis.遗传性肾炎中的肾小球基底膜。
J Pathol. 1981 Nov;135(3):199-209. doi: 10.1002/path.1711350305.
2
The thin glomerular basement membrane in children with haematuria.血尿患儿的薄肾小球基底膜
J Pathol. 1984 Apr;142(4):253-7. doi: 10.1002/path.1711420403.
3
Hereditary nephritis in children with and without characteristic glomerular basement membrane alterations.
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Hereditary nephritis mimicking immune complex-mediated glomerulonephritis.酷似免疫复合物介导性肾小球肾炎的遗传性肾炎。
Hum Pathol. 2006 May;37(5):547-54. doi: 10.1016/j.humpath.2005.12.017.
5
[Clinicopathological study on progressive hereditary nephritis: observations of ultrastructural lesions in the glomerular basement membrane].
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Early pathologic features of hereditary nephritis: a clinicopathologic correlation.遗传性肾炎的早期病理特征:临床病理相关性
Pathol Res Pract. 1980;168(1-3):146-62. doi: 10.1016/S0344-0338(80)80214-7.
7
Glomerular basement membrane changes in hereditary glomerular diseases.遗传性肾小球疾病中的肾小球基底膜变化
Ren Physiol. 1980;3(1-6):405-13. doi: 10.1159/000172790.
8
[Hereditary nephropathies].[遗传性肾病]
Pediatrie. 1990;45(1):5-11.
9
Glomerular basement membrane attenuation in familial nephritis and "benign" hematuria.家族性肾炎和“良性”血尿中的肾小球基底膜变薄
J Pediatr. 1982 Sep;101(3):358-65. doi: 10.1016/s0022-3476(82)80058-9.
10
Ultrastructural lesions of the glomerular basement membrane in hereditary chronic nephritis.遗传性慢性肾炎肾小球基底膜的超微结构病变
Adv Nephrol Necker Hosp. 1974;3:133-52.

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2
The Alport nephropathy: clinicopathological correlations.奥尔波特肾病:临床病理相关性
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Glomerular basement membrane thickness in primary diffuse IgA nephropathy: ultrastructural morphometric analysis.原发性弥漫性IgA肾病的肾小球基底膜厚度:超微结构形态计量分析
Int Urol Nephrol. 1998;30(4):513-9. doi: 10.1007/BF02550234.
4
Glomerular basement membrane abnormalities in infants with heavy proteinuria.
Pediatr Nephrol. 1993 Aug;7(4):401-3. doi: 10.1007/BF00857552.
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Permselectivity in thin membrane nephropathy.薄基底膜肾病中的分子屏障功能
J Clin Invest. 1994 May;93(5):1881-4. doi: 10.1172/JCI117178.
6
Ocular manifestations of Alport's syndrome: a hereditary disorder of basement membranes?阿尔波特综合征的眼部表现:一种基底膜的遗传性疾病?
Br J Ophthalmol. 1983 Aug;67(8):493-503. doi: 10.1136/bjo.67.8.493.
7
Alport's syndrome: specificity and pathogenesis of glomerular basement membrane alterations.
Pediatr Nephrol. 1987 Jul;1(3):422-7. doi: 10.1007/BF00849248.
8
Glomerular basement membrane thickness in children. A morphometric study.儿童肾小球基底膜厚度。一项形态计量学研究。
Pediatr Nephrol. 1988 Apr;2(2):190-5. doi: 10.1007/BF00862588.
9
Human tissue distribution of novel basement membrane collagen.新型基底膜胶原蛋白的人体组织分布
Am J Pathol. 1989 Apr;134(4):813-25.
10
The investigation of haematuria.血尿的调查
Arch Dis Child. 1989 Jan;64(1):159-65. doi: 10.1136/adc.64.1.159.