• 文献检索
  • 文档翻译
  • 深度研究
  • 学术资讯
  • Suppr Zotero 插件Zotero 插件
  • 邀请有礼
  • 套餐&价格
  • 历史记录
应用&插件
Suppr Zotero 插件Zotero 插件浏览器插件Mac 客户端Windows 客户端微信小程序
定价
高级版会员购买积分包购买API积分包
服务
文献检索文档翻译深度研究API 文档MCP 服务
关于我们
关于 Suppr公司介绍联系我们用户协议隐私条款
关注我们

Suppr 超能文献

核心技术专利:CN118964589B侵权必究
粤ICP备2023148730 号-1Suppr @ 2026

文献检索

告别复杂PubMed语法,用中文像聊天一样搜索,搜遍4000万医学文献。AI智能推荐,让科研检索更轻松。

立即免费搜索

文件翻译

保留排版,准确专业,支持PDF/Word/PPT等文件格式,支持 12+语言互译。

免费翻译文档

深度研究

AI帮你快速写综述,25分钟生成高质量综述,智能提取关键信息,辅助科研写作。

立即免费体验

通过给患有与遗传性C3功能障碍相关的系统性红斑狼疮样综合征的患者输注血浆来重建补体替代途径。

Reconstitution of the alternative pathway of complement by plasma infusions given to a patient with an SLE-like syndrome associated with a hereditary C3 dysfunction.

作者信息

Nilsson B, Nilsson U R, Karlsson-Parra A, Sjölin-Forsberg G, Hällgren R

机构信息

Department of Clinical Immunology and Transfusion Medicine, University Hospital, Uppsala, Sweden.

出版信息

Ann Rheum Dis. 1994 Oct;53(10):691-4. doi: 10.1136/ard.53.10.691.

DOI:10.1136/ard.53.10.691
PMID:7979584
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC1005438/
Abstract

OBJECTIVE

To reconstitute a dysfunctional form of complement factor C3 in a patient with a systemic lupus erythematosus (SLE)-like syndrome.

METHODS

The propositus was treated with plasma infusions during five sessions over a period of eight months.

RESULTS

The alternative pathway was reconstituted to normal levels for approximately two to three days after each infusion. C3 fragments were incorporated into previously detected deposits of IgG and IgM at the dermal-epidermal junction and the immune complex levels gradually decreased during the whole treatment period.

CONCLUSION

The reconstitution appears to result in the solubilisation of tissue immune complexes and a subsequent transportation to the fixed macrophage system.

摘要

目的

在一名患有系统性红斑狼疮(SLE)样综合征的患者中重建功能失调的补体因子C3形式。

方法

先证者在八个月的时间里分五次接受了血浆输注治疗。

结果

每次输注后,替代途径在大约两到三天内恢复到正常水平。C3片段被整合到先前在真皮 - 表皮交界处检测到的IgG和IgM沉积物中,并且在整个治疗期间免疫复合物水平逐渐降低。

结论

这种重建似乎导致组织免疫复合物溶解,并随后转运至固定巨噬细胞系统。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9006/1005438/c6c3a4c04631/annrheumd00498-0073-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9006/1005438/c6c3a4c04631/annrheumd00498-0073-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9006/1005438/c6c3a4c04631/annrheumd00498-0073-a.jpg

相似文献

1
Reconstitution of the alternative pathway of complement by plasma infusions given to a patient with an SLE-like syndrome associated with a hereditary C3 dysfunction.通过给患有与遗传性C3功能障碍相关的系统性红斑狼疮样综合征的患者输注血浆来重建补体替代途径。
Ann Rheum Dis. 1994 Oct;53(10):691-4. doi: 10.1136/ard.53.10.691.
2
Increased amounts of C4-containing immune complexes and inefficient activation of C3 and the terminal complement pathway in a patient with homozygous C2 deficiency and systemic lupus erythematosus.纯合子C2缺乏症和系统性红斑狼疮患者体内含C4免疫复合物数量增加,C3及补体终末途径激活效率低下。
Scand J Immunol. 1990 Jan;31(1):59-64. doi: 10.1111/j.1365-3083.1990.tb02743.x.
3
Demonstration of beta 1H globulin together with C3 in the dermal-epidermal junction of patients with systemic lupus erythematosus.系统性红斑狼疮患者真皮-表皮交界处β1H球蛋白与C3的显示。
Arthritis Rheum. 1979 Jan;22(1):13-8. doi: 10.1002/art.1780220103.
4
Hypercatabolism of C3 and C4 in active and inactive systemic lupus erythematosus.活动期和非活动期系统性红斑狼疮中C3和C4的高分解代谢
Ann Rheum Dis. 1989 Feb;48(2):153-9. doi: 10.1136/ard.48.2.153.
5
Factor B activation products in patients with systemic lupus erythematosus. A marker of severe disease activity.系统性红斑狼疮患者体内的B因子激活产物。一种严重疾病活动的标志物。
Arthritis Rheum. 1989 Nov;32(11):1406-13. doi: 10.1002/anr.1780321109.
6
A decrease in complement is associated with increased renal and hematologic activity in patients with systemic lupus erythematosus.系统性红斑狼疮患者体内补体水平降低与肾脏及血液学活性增加相关。
Arthritis Rheum. 2001 Oct;44(10):2350-7. doi: 10.1002/1529-0131(200110)44:10<2350::aid-art398>3.0.co;2-a.
7
Suppression of Fc Receptor and C4.C3 receptors of the granulocytes from patients with systemic lupus erythematosus.系统性红斑狼疮患者粒细胞的Fc受体和C4、C3受体的抑制作用
J Rheumatol. 1981 Nov-Dec;8(6):910-6.
8
Hereditary dysfunction of the third component of complement associated with a systemic lupus erythematosus-like syndrome and meningococcal meningitis.遗传性补体第三成分功能障碍伴系统性红斑狼疮样综合征和脑膜炎球菌性脑膜炎。
Arthritis Rheum. 1992 May;35(5):580-6. doi: 10.1002/art.1780350516.
9
Activation of the alternative complement pathway accompanies disease flares in systemic lupus erythematosus during pregnancy.在妊娠期间,系统性红斑狼疮患者疾病发作时会伴随替代补体途径的激活。
Arthritis Rheum. 1992 Jan;35(1):55-61. doi: 10.1002/art.1780350109.
10
Monoclonal antibodies against complement 3 neoantigens for detection of immune complexes and complement activation. Relationship between immune complex levels, state of C3, and numbers of receptors for C3b.用于检测免疫复合物和补体激活的抗补体3新抗原单克隆抗体。免疫复合物水平、C3状态与C3b受体数量之间的关系。
J Clin Invest. 1985 Oct;76(4):1418-26. doi: 10.1172/JCI112119.

引用本文的文献

1
Rare loss-of-function mutation in complement component C3 provides insight into molecular and pathophysiological determinants of complement activity.补体成分C3中罕见的功能丧失突变有助于深入了解补体活性的分子和病理生理决定因素。
J Immunol. 2015 Apr 1;194(7):3305-16. doi: 10.4049/jimmunol.1402781. Epub 2015 Feb 23.
2
Progress and Trends in Complement Therapeutics.补体疗法的进展与趋势
Adv Exp Med Biol. 2013;735:1-22. doi: 10.1007/978-1-4614-4118-2_1.
3
Assessing periarticular bone mineral density in patients with early psoriatic arthritis or rheumatoid arthritis.

本文引用的文献

1
Measurement of the complement C3 breakdown product C3d by rocket immunoelectrophoresis.通过火箭免疫电泳法检测补体C3裂解产物C3d
J Immunol Methods. 1982;48(1):97-108. doi: 10.1016/0022-1759(82)90214-9.
2
Simplified assays of hemolytic activity of the classical and alternative complement pathways.经典和替代补体途径溶血活性的简化检测方法。
J Immunol Methods. 1984 Aug 3;72(1):49-59. doi: 10.1016/0022-1759(84)90432-0.
3
Complement deficiency states and infection: epidemiology, pathogenesis and consequences of neisserial and other infections in an immune deficiency.
评估早期银屑病关节炎或类风湿关节炎患者的关节周围骨密度。
Ann Rheum Dis. 2002 Nov;61(11):1007-11. doi: 10.1136/ard.61.11.1007.
补体缺陷状态与感染:免疫缺陷状态下奈瑟菌及其他感染的流行病学、发病机制及后果
Medicine (Baltimore). 1984 Sep;63(5):243-73.
4
Successful plasma infusion treatment of a patient with C2 deficiency and systemic lupus erythematosus: clinical experience over forty-five months.C2缺乏症合并系统性红斑狼疮患者的成功血浆输注治疗:45个月的临床经验
Arthritis Rheum. 1989 Jul;32(7):906-13.
5
Defective Fc receptor-mediated clearance in patients with primary biliary cirrhosis.原发性胆汁性肝硬化患者中Fc受体介导的清除功能缺陷。
Gastroenterology. 1991 Oct;101(4):1076-82. doi: 10.1016/0016-5085(91)90736-5.
6
Complement deficiency and disease.补体缺陷与疾病
Immunol Today. 1991 Sep;12(9):301-6. doi: 10.1016/0167-5699(91)90003-C.
7
Generation of iC3 at the interface between blood and gas.在血液与气体的界面处生成iC3。
Scand J Immunol. 1992 Jan;35(1):85-91. doi: 10.1111/j.1365-3083.1992.tb02837.x.
8
Hereditary dysfunction of the third component of complement associated with a systemic lupus erythematosus-like syndrome and meningococcal meningitis.遗传性补体第三成分功能障碍伴系统性红斑狼疮样综合征和脑膜炎球菌性脑膜炎。
Arthritis Rheum. 1992 May;35(5):580-6. doi: 10.1002/art.1780350516.