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一名患有C4缺乏症患者的系统性红斑狼疮

Systemic lupus erythematosus in a patient with C4 deficiency.

作者信息

Urowitz M B, Gladman D D, Minta J O

出版信息

J Rheumatol. 1981 Sep-Oct;8(5):741-6.

PMID:6975824
Abstract

A 40-year-old female with systemic lupus erythematosus was found to be totally deficient in the 4th component of complement and to be lacking in Chido and Rodgers blood group substances on red cells and in plasma. Family studies were suggestive of a linkage of the C4 deficiency with an HLA haplotype. C2 in the patient's serum was 50% of normal but other complement components and alternative pathway functions were normal. Chemotactic and opsonic activities in the patient's serum were diminished.

摘要

一名40岁的系统性红斑狼疮女性患者被发现完全缺乏补体第四成分,其红细胞和血浆中缺乏奇多(Chido)和罗杰斯(Rodgers)血型物质。家族研究提示C4缺乏与一种HLA单倍型存在连锁关系。该患者血清中的C2为正常水平的50%,但其他补体成分和替代途径功能正常。患者血清中的趋化活性和调理活性降低。

相似文献

1
Systemic lupus erythematosus in a patient with C4 deficiency.一名患有C4缺乏症患者的系统性红斑狼疮
J Rheumatol. 1981 Sep-Oct;8(5):741-6.
2
Selective deficiency of the fourth component of complement in a patient with systemic lupus erythematosus (SLE): immunochemical and biological studies.系统性红斑狼疮(SLE)患者补体第四成分的选择性缺乏:免疫化学和生物学研究
Clin Exp Immunol. 1981 Jul;45(1):72-80.
3
[Hereditary deficiency of the 4th component of complement (C4) associated with a lupic syndrome].与狼疮综合征相关的遗传性补体第四成分(C4)缺乏症
Arch Fr Pediatr. 1986 Apr;43(4):267-9.
4
[Systemic lupus erythematosus in hereditary complement 4 deficiency].
Hautarzt. 1984 Jan;35(1):27-32.
5
C3 metabolism in a patient with deficiency of the second component of complement (C2) and discoid lupus erythematosus.一名补体第二成分(C2)缺乏且患有盘状红斑狼疮患者的C3代谢
Clin Exp Immunol. 1976 May;24(2):238-48.
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Which complement assays and typings are necessary for the diagnosis of complement deficiency in patients with lupus erythematosus? A study of 25 patients.诊断红斑狼疮患者补体缺乏需要哪些补体检测和分型?对25例患者的研究。
Clin Immunol. 2006 Nov;121(2):198-202. doi: 10.1016/j.clim.2006.08.007. Epub 2006 Sep 20.
7
[Familial lupus erythematosus with partial C4 deficiency].
Wien Klin Wochenschr. 1993;105(11):323-6.
8
Deficiency of C2, the second complement component, in the family of a patient with SLE-like syndrome: the first case of hereditary C2 deficiency in Czechoslovakia.系统性红斑狼疮样综合征患者家族中第二补体成分C2缺乏:捷克斯洛伐克首例遗传性C2缺乏症。
Haematologia (Budap). 1987;20(4):215-20.
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Immune response of a patient with deficiency of the fourth component of complement and systemic lupus erythematosus.一名补体第四成分缺乏症患者与系统性红斑狼疮的免疫反应。
N Engl J Med. 1979 May 17;300(20):1124-9. doi: 10.1056/NEJM197905173002002.
10
Anti-C4 in the serum of a transfused C4-deficient patient with systemic lupus erythematosus.一名患有系统性红斑狼疮的C4缺陷输血患者血清中的抗C4
Vox Sang. 1984;46(5):291-9. doi: 10.1111/j.1423-0410.1984.tb00089.x.

引用本文的文献

1
Association of Sjögren's syndrome with C4 deficiency, defective reticuloendothelial function and circulating immune complexes.干燥综合征与C4缺乏、网状内皮系统功能缺陷及循环免疫复合物的关联。
Clin Exp Immunol. 1983 Jun;52(3):551-60.
2
Inherited incomplete deficiency of the fourth component of complement (C4) determined by a gene not linked to human histocompatibility leukocyte antigens.由一个与人类组织相容性白细胞抗原不连锁的基因所决定的遗传性补体第四成分(C4)不完全缺乏。
J Clin Invest. 1984 Oct;74(4):1509-14. doi: 10.1172/JCI111564.
3
Selective C4 deficiency, systemic lupus erythematosus, and Whipple's disease.
选择性C4缺乏、系统性红斑狼疮和惠普尔病。
Ann Rheum Dis. 1984 Feb;43(1):91-4. doi: 10.1136/ard.43.1.91.
4
Genetic deficiency of C4, C2 or C1q and lupus syndromes. Association with anti-Ro (SS-A) antibodies.C4、C2或C1q基因缺陷与狼疮综合征。与抗Ro(SS-A)抗体的关联。
Clin Exp Immunol. 1985 Dec;62(3):678-84.
5
Lupus diseases associated with hereditary and acquired deficiencies of complement.与遗传性和获得性补体缺陷相关的狼疮疾病。
Springer Semin Immunopathol. 1986;9(2-3):161-78. doi: 10.1007/BF02099020.