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系膜纤维蛋白原沉积在儿童新月体性紫癜性肾炎发病机制中的作用

Role of mesangial fibrinogen deposition in the pathogenesis of crescentic Henoch-Schonlein nephritis in children.

作者信息

Shin J I, Park J M, Shin Y H, Lee J S, Jeong H J

机构信息

The Institute of Kidney Disease, Department of Pediatrics, Yonsei University College of Medicine, Seoul, Korea.

出版信息

J Clin Pathol. 2005 Nov;58(11):1147-51. doi: 10.1136/jcp.2005.027409.

Abstract

AIMS

To clarify the role of mesangial fibrinogen deposition in crescentic Henoch-Schönlein nephritis (HSN).

METHODS

A retrospective analysis of 21 children with HSN treated with immunosuppressants. Serial renal biopsies were performed before and after treatment. They were divided into two groups according to the immunofluorescent course of fibrinogen deposition: group I (n = 9), no or decreased deposition; group II (n = 12), persistent or increased deposition.

RESULTS

There were no differences between the two groups in renal manifestations or laboratory and histological findings at presentation. However, the activity index after immunosuppressive treatment was significantly decreased in group I (mean, 7.9 (SEM, 0.7) v 2.9 (0.4); p = 0.008) and unchanged in group II (mean, 6.8 (SEM, 0.3) v 6.0 (2.1)). The chronicity index was unchanged in group I, but increased in group II (mean, 0.8 (SEM, 0.3) v 1.8 (0.3); p = 0.02). Univariate analysis revealed that the only factor significantly related to persistent or increased fibrinogen deposition was age more than 9 years (p = 0.03). Furthermore, the intensity of fibrinogen deposition at the second biopsy correlated positively with the age at onset (R2= 0.306; p = 0.009) and changes in the percentage of crescents (post-treatment crescents (%) minus pretreatment crescents (%)) correlated positively with the intensity of fibrinogen deposition at the second biopsy (R2= 0.193; p = 0.046).

CONCLUSIONS

This study indicates that fibrinogen deposition has an important role to play in renal injury of crescentic HSN and reflects persistent severe histological activity.

摘要

目的

阐明系膜纤维蛋白原沉积在新月体性紫癜性肾炎(HSN)中的作用。

方法

对21例接受免疫抑制剂治疗的HSN患儿进行回顾性分析。在治疗前后进行系列肾活检。根据纤维蛋白原沉积的免疫荧光过程将其分为两组:I组(n = 9),无沉积或沉积减少;II组(n = 12),持续沉积或沉积增加。

结果

两组在就诊时的肾脏表现、实验室检查及组织学结果方面无差异。然而,免疫抑制治疗后的活动指数在I组显著降低(均值,7.9(标准误,0.7)对2.9(0.4);p = 0.008),而在II组无变化(均值,6.8(标准误,0.3)对6.0(2.1))。I组的慢性指数无变化,但II组增加(均值,0.8(标准误,0.3)对1.8(0.3);p = 0.02)。单因素分析显示,与纤维蛋白原持续沉积或沉积增加显著相关的唯一因素是年龄大于9岁(p = 0.03)。此外,第二次活检时纤维蛋白原沉积的强度与发病年龄呈正相关(R2 = 0.306;p = 0.009),新月体百分比的变化(治疗后新月体(%)减去治疗前新月体(%))与第二次活检时纤维蛋白原沉积的强度呈正相关(R2 = 0.193;p = 0.046)。

结论

本研究表明纤维蛋白原沉积在新月体性HSN的肾损伤中起重要作用,并反映持续的严重组织学活动。

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