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伴有IgA系膜沉积且与系统性疾病无关的肾小球肾炎。

Glomerulonephritis with mesangial deposits of IgA unassociated with systemic disease.

作者信息

Katz A, Underdown B J, Minta J O, Lepow I H

出版信息

Can Med Assoc J. 1976 Feb 7;114(3):209-15.

Abstract

Typical features of IgA-associated nephritis were found in renal biopsies from 16 of 355 consecutive patients. Generalized segmental mesangial proliferation was noted in biopsies from most patients, and dense deposits were detected by electron microscopy in mesangial regions of approximately 50% of biopsies. Immunofluorescent studies showed IgA to be the predominant immunoglobulin in glomueruli; IgG was present in less than 50% of biopsies and IgM in only 12%. The serum IgA value was significantly increased (P les than 0.001) in 50% of patients and the mean IgA/IgG ratio was significantly increase (P less than 0.001) for the patient group as a whole, which suggests a selective increase in IgA. Mesangial deposits of C3 were present in 15 of 16 biopsies and properdin was noted in all biopsies tested; C4 was not demonstrated in any biopsy. This suggests activation of the alternative complement pathway. The results of this study support the concept that IgA-associated nephritis is a unique condition that in some patients gives rise to idiopathic recurrent hematuria. Although the prognosis is good in the majority of patients, the renal disease may progress.

摘要

在355例连续患者中,16例患者的肾活检发现了IgA相关性肾炎的典型特征。大多数患者的活检显示弥漫性节段性系膜增生,约50%的活检标本经电子显微镜检查在系膜区检测到致密沉积物。免疫荧光研究显示IgA是肾小球中的主要免疫球蛋白;不到50%的活检标本中有IgG,只有12%的活检标本中有IgM。50%的患者血清IgA值显著升高(P小于0.001),整个患者组的平均IgA/IgG比值显著升高(P小于0.001),这表明IgA选择性升高。16例活检标本中有15例存在C3系膜沉积物,所有检测的活检标本中均发现备解素;所有活检标本中均未检测到C4。这表明替代补体途径被激活。本研究结果支持这样一种观点,即IgA相关性肾炎是一种独特的疾病,在某些患者中会导致特发性复发性血尿。虽然大多数患者的预后良好,但肾脏疾病可能会进展。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4807/1956928/2e21bd715eac/canmedaj01551-0037-a.jpg

引用本文的文献

1
Partial H (beta 1H) deficiency and glomerulonephritis in two families.
J Clin Immunol. 1982 Apr;2(2):110-7. doi: 10.1007/BF00916894.
2
IGA mesangial glomerulonephritis; significance and pathogenesis of segmental-focal glomerular lesions.
Virchows Arch A Pathol Anat Histopathol. 1983;402(1):11-24. doi: 10.1007/BF00695045.
5
Do genetic factors play a role in Berger's disease?
Pediatr Nephrol. 1987 Jul;1(3):447-54. doi: 10.1007/BF00849253.

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