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本文引用的文献

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Genetic analysis of C4 deficiency.C4缺乏症的基因分析。
J Clin Invest. 1981 Jan;67(1):260-3. doi: 10.1172/JCI110021.
2
The 1982 revised criteria for the classification of systemic lupus erythematosus.1982年系统性红斑狼疮分类的修订标准。
Arthritis Rheum. 1982 Nov;25(11):1271-7. doi: 10.1002/art.1780251101.
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Statement on the nomenclature of human C4 allotypes.关于人类C4同种异型命名的声明。
Immunobiology. 1983 Mar;164(2):184-91. doi: 10.1016/s0171-2985(83)80009-6.
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The lupus activity criteria count (LACC).狼疮活动标准计数(LACC)。
J Rheumatol. 1984 Dec;11(6):783-7.
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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.
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Family study of the major histocompatibility complex in patients with systemic lupus erythematosus: importance of null alleles of C4A and C4B in determining disease susceptibility.系统性红斑狼疮患者主要组织相容性复合体的家系研究:C4A和C4B无效等位基因在决定疾病易感性中的重要性。
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Genetics of human C4 polymorphism: detection and segregation of rare and duplicated haplotypes.人类C4多态性的遗传学:罕见和重复单倍型的检测与分离
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Serum C4 concentration in the monitoring of systemic lupus erythematosus: requirement for C4 allotyping.
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Reference ranges for serum C4 concentrations in subjects with and without C4 null alleles.具有和不具有C4无效等位基因的受试者血清C4浓度的参考范围。
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10
Component deficiencies. 2. The fourth component.
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系统性红斑狼疮中的C4浓度及C4缺陷等位基因

C4 concentrations and C4 deficiency alleles in systemic lupus erythematosus.

作者信息

Wilson W A, Armatis P E, Perez M C

机构信息

Department of Medicine, Louisiana State University Medical Center, New Orleans 70112.

出版信息

Ann Rheum Dis. 1989 Jul;48(7):600-4. doi: 10.1136/ard.48.7.600.

DOI:10.1136/ard.48.7.600
PMID:2774701
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC1003824/
Abstract

In a study of 66 patients with systemic lupus erythematosus (SLE) and 80 controls it was found that the presence of two deficiency (null) alleles of C4 had a significant effect on mean C4 concentrations in serum. In six controls who each had two C4 null alleles the mean C4 concentration in serum was 56% lower than in 43 controls without C4 null alleles; the nadir of the C4 concentration in four patients with SLE with two null alleles was also lower by a mean of 55% than in 32 patients who did not have null alleles. Reduced production of C4 allotypes in subjects with two null alleles may be an important determinant of total C4 concentration in patients with SLE. For optimal interpretation of C4 concentrations in SLE, C4 allotyping appears to be indicated, particularly to identify patients who have two null alleles of C4.

摘要

在一项针对66例系统性红斑狼疮(SLE)患者和80例对照者的研究中发现,C4的两个缺陷(无效)等位基因的存在对血清中C4的平均浓度有显著影响。在6名各自拥有两个C4无效等位基因的对照者中,血清中C4的平均浓度比43名没有C4无效等位基因的对照者低56%;4例拥有两个无效等位基因的SLE患者中C4浓度的最低点也比32例没有无效等位基因的患者平均低55%。拥有两个无效等位基因的受试者中C4同种异型的产生减少可能是SLE患者总C4浓度的一个重要决定因素。为了对SLE患者的C4浓度进行最佳解读,似乎有必要进行C4同种异型分型,特别是要识别出拥有两个C4无效等位基因的患者。