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亨诺-许兰综合征:肾活检的临床与形态学研究

Henoch-Schoenlein syndrome: a clinical and morphological study of renal biopsies.

作者信息

Sinniah R, Feng P H, Chen B T

出版信息

Clin Nephrol. 1978 Jun;9(6):219-28.

PMID:668222
Abstract

Twenty-four patients, 12 children and 12 adults, with the Henoch-Schoenlein syndrome had their renal biopsy specimens studied by light and electron microscopic and immunofluorescent antibody techniques. The principal glomerular lesion was a focal and segmental proliferative glomerulonephritis in 15, a diffuse proliferative glomerulonephritis in 6 and a animal or minor change lesion with mesangial hypertrophy in 3 cases. The proliferation of the cells was mainly mesangial. Renal biopsies taken earlier in the course of the disease showed a greater number with a focal lesion. Electron dense deposits with cellular proliferation and increased matrix were seen in the mesangium. Less frequent subendothelial and occasional subepithelial deposits were found. Capillary loop changes were seen more frequently in the later stages of the disease. Heavy deposits of IgA were found in the mesangium in all cases, and less intense deposits of IgG in 60%. beta 1 C globulin and fibrinogen were found in 80% and IgD and IgM less frequently. Complement activation was via the alternate pathway as early complement components C1q and C4 were absent. Overt allergies, streptococal infections and the HBsAg could not explain the pathogenesis of the disease. Henoch-Schoenlein syndrome is a chronic disease of the mesangium; only 5 patients showed complete recovery, 15 had persistent microscopic hematuria and 3 died or developed renal insufficiency within 8 years. The prognosis was worst with diffuse proliferative glomerulonephritis, widespread focal glomerulonephritis or epithelial cresents formation.

摘要

对24例患有过敏性紫癜的患者(12例儿童和12例成人)的肾活检标本进行了光镜、电镜及免疫荧光抗体技术研究。主要的肾小球病变为:局灶节段性增生性肾小球肾炎15例,弥漫性增生性肾小球肾炎6例,系膜增生伴轻微病变或轻微改变病变3例。细胞增生主要为系膜细胞增生。病程早期进行的肾活检显示局灶性病变的病例较多。在系膜区可见电子致密沉积物伴细胞增生和基质增加。较少见内皮下沉积物,偶见上皮下沉积物。毛细血管袢改变在疾病后期更为常见。所有病例系膜区均发现大量IgA沉积,60%的病例有较弱的IgG沉积。80%的病例发现β1C球蛋白和纤维蛋白原,IgD和IgM较少见。由于早期补体成分C1q和C4缺失,补体激活通过替代途径。明显的过敏反应、链球菌感染及HBsAg均不能解释该疾病的发病机制。过敏性紫癜是一种系膜的慢性疾病;仅5例患者完全恢复,15例有持续性镜下血尿,3例在8年内死亡或发展为肾功能不全。弥漫性增生性肾小球肾炎、广泛的局灶性肾小球肾炎或上皮新月体形成的预后最差。

相似文献

1
Henoch-Schoenlein syndrome: a clinical and morphological study of renal biopsies.亨诺-许兰综合征:肾活检的临床与形态学研究
Clin Nephrol. 1978 Jun;9(6):219-28.
2
Lupus nephritis: correlation between light, electron microscopic and immunofluorescent findings and renal function.狼疮性肾炎:光镜、电镜及免疫荧光检查结果与肾功能的相关性
Clin Nephrol. 1976 Aug;6(2):340-51.
3
Electron-dense subepithelial glomerular deposits in Henoch-Schönlein purpura syndrome.过敏性紫癜综合征中的电子致密性肾小球上皮下沉积物。
Arch Pathol Lab Med. 1979 Oct;103(11):595-8.
4
Hepatitis-B-associated glomerulonephritis: pathology, pathogenesis, and clinical course.乙型肝炎相关性肾小球肾炎:病理、发病机制及临床病程
Medicine (Baltimore). 1990 Jul;69(4):200-16.
5
Henoch-Schoenlein nephritis in adults: a clinical and morphological study.成人过敏性紫癜性肾炎:一项临床与形态学研究。
Clin Nephrol. 1986 Sep;26(3):125-30.
6
[IgA nephropathy and Henoch-Schönlein purpura nephritis: clinicopathological, histopathological immunohistological, and electronmicroscopical characteristics].[IgA肾病与过敏性紫癜性肾炎:临床病理、组织病理免疫组化及电镜特征]
Hokkaido Igaku Zasshi. 1984 Jul;59(4):456-70.
7
Glomerular lesions in asymptomatic microscopic hematuria discovered on routine medical examination.在常规体检中发现的无症状镜下血尿中的肾小球病变。
Clin Nephrol. 1976 May;5(5):216-28.
8
Primary IgA glomerulonephritis and Schönlein-Henoch purpura nephritis: Clinicopathological and immunohistological characteristics.原发性IgA肾小球肾炎和过敏性紫癜性肾炎:临床病理及免疫组织学特征
Q J Med. 1978 Oct;47(188):495-516.
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Glomerular lesions in patients with asymptomatic persistent andorthostatic proteinuria discovered on routine medical examination.在常规体检中发现的无症状持续性直立性蛋白尿患者的肾小球病变。
Clin Nephrol. 1977 Jan;7(1):1-14.
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[Demonstration of mesangial IgA deposits in kidney biopsies of pediatric patients: comparison with the clinical picture].[小儿患者肾活检中系膜IgA沉积的显示:与临床表现的比较]
Praxis (Bern 1994). 1995 Oct 10;84(41):1158-64.

引用本文的文献

1
Antineutrophil cytoplasm antibodies (ANCA) of IgA isotype in adult Henoch-Schönlein purpura.成人过敏性紫癜中IgA同种型抗中性粒细胞胞浆抗体(ANCA)
Clin Exp Immunol. 1994 Jan;95(1):49-55. doi: 10.1111/j.1365-2249.1994.tb06013.x.
2
Schönlein-Henoch glomerulonephritis. Characteristic ultrastructural changes in the glomerular basement membrane and localisation of osmiophilic deposits.过敏性紫癜性肾小球肾炎。肾小球基底膜的特征性超微结构改变及嗜锇性沉积物的定位。
Virchows Arch A Pathol Anat Histol. 1980;388(2):155-65. doi: 10.1007/BF00430684.