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第二例人类C3b抑制剂(KAF)缺乏症。

A second case of human C3b inhibitor (KAF) deficiency.

作者信息

Thompson R A, Lachmann P J

出版信息

Clin Exp Immunol. 1977 Jan;27(1):23-9.

PMID:849647
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC1540911/
Abstract

The second case of C3b inhibitor deficiency is described in an 11-year-old girl who presented with recurrent attacks of meningitis, in between which she was well. Her serum showed all of the complement component changes noted in the first described case, although showing only a relatively slight defect in its ability to opsonize bacteria for phagocytosis and killing by polymorphonuclear leucocytes. This correlated with the patient's freedom from other infections.

摘要

第二例C3b抑制剂缺乏症发生在一名11岁女孩身上,她反复出现脑膜炎发作,发作间歇期情况良好。她的血清显示出首例所述病例中所有补体成分的变化,尽管在调理细菌以便被多形核白细胞吞噬和杀灭方面仅表现出相对轻微的缺陷。这与该患者未发生其他感染的情况相符。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c94e/1540911/d74bdd288f28/clinexpimmunol00239-0035-b.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c94e/1540911/1a6ef5656f14/clinexpimmunol00239-0034-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c94e/1540911/85e13d94e353/clinexpimmunol00239-0034-b.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c94e/1540911/02222bc7f120/clinexpimmunol00239-0035-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c94e/1540911/d74bdd288f28/clinexpimmunol00239-0035-b.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c94e/1540911/1a6ef5656f14/clinexpimmunol00239-0034-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c94e/1540911/85e13d94e353/clinexpimmunol00239-0034-b.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c94e/1540911/02222bc7f120/clinexpimmunol00239-0035-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c94e/1540911/d74bdd288f28/clinexpimmunol00239-0035-b.jpg

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1
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2
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Front Immunol. 2019 Jun 7;10:1150. doi: 10.3389/fimmu.2019.01150. eCollection 2019.
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本文引用的文献

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A BIOCHEMICAL ABNORMALITY IN HEREDIATRY ANGIONEUROTIC EDEMA: ABSENCE OF SERUM INHIBITOR OF C' 1-ESTERASE.遗传性血管神经性水肿中的一种生化异常:血清C1酯酶抑制剂缺失
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In vitro bactericidal capacity of human polymorphonuclear leukocytes: diminished activity in chronic granulomatous disease of childhood.人多形核白细胞的体外杀菌能力:儿童慢性肉芽肿病中活性降低。
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Genetic aspects of the complement system.补体系统的遗传学方面。
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The molecular basis of hereditary complement factor I deficiency.遗传性补体因子I缺乏症的分子基础。
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Recurrent bacterial meningitis in patients with genetic defects of terminal complement components.终末补体成分基因缺陷患者的复发性细菌性脑膜炎
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Properdin deficiency in a family with fulminant meningococcal infections.一个患有暴发性脑膜炎球菌感染的家庭中的备解素缺乏症。
Clin Exp Immunol. 1982 Nov;50(2):291-7.
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C3b inactivator deficiency with immune complex manifestations.伴有免疫复合物表现的C3b灭活因子缺乏症。
Clin Exp Immunol. 1982 Jan;47(1):197-205.
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Complement activation in chronic liver disease.慢性肝病中的补体激活
Clin Exp Immunol. 1982 Mar;47(3):548-54.
9
Recurrent meningococcal infections associated with a functional deficiency of the C8 component of human complement.复发性脑膜炎球菌感染与人类补体C8成分的功能缺陷相关。
Clin Exp Immunol. 1980 Jan;39(1):53-9.
10
Functional deficiency of complement factor D in a monozygous twin.单卵双胞胎中补体因子D的功能缺陷
Clin Exp Immunol. 1984 Dec;58(3):724-30.
Adv Immunol. 1971;14:251-90. doi: 10.1016/s0065-2776(08)60286-2.
4
Studies on eosinophil leucocyte migration. II. Factors specifically chemotactic for eosinophils and neutrophils generated from guinea-pig serum by antigen-antibody complexes.嗜酸性白细胞迁移的研究。II. 抗原抗体复合物从豚鼠血清中产生的对嗜酸性粒细胞和中性粒细胞具有特异性趋化作用的因子。
Clin Exp Immunol. 1970 Nov;7(5):723-37.
5
Hereditary deficiency of the second component of complement (C2) in man: correlation of C2 haemolytic activity with immunochemical measurements of C2 protein.人类补体第二成分(C2)的遗传性缺陷:C2溶血活性与C2蛋白免疫化学测量的相关性。
Immunology. 1970 Jun;18(6):943-54.
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Increased susceptibility to infection in a patient with type II essential hypercatabolism of C3.患有C3 II型原发性高分解代谢的患者对感染的易感性增加。
N Engl J Med. 1973 Mar 22;288(12):601-6. doi: 10.1056/NEJM197303222881204.
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The alternate pathway of complement activation. The role of C3 and its inactivator (KAF).补体激活的替代途径。C3及其灭活剂(KAF)的作用。
Immunology. 1973 Feb;24(2):259-75.
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[Acute lupus erythematosus with total absence of the C4 fraction of complement].[伴有补体C4成分完全缺失的急性红斑狼疮]
Nouv Presse Med. 1974 Apr 6;3(14):881-2.
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Inactivator of the third component of complement as an inhibitor in the properdin pathway.补体第三成分灭活剂作为备解素途径中的一种抑制剂。
Proc Natl Acad Sci U S A. 1972 Oct;69(10):2910-3. doi: 10.1073/pnas.69.10.2910.
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Escherichia coli antibody: a screening test for immunodeficiency.大肠杆菌抗体:一种免疫缺陷筛查试验。
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