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两名患有系统性红斑狼疮样症状的姐妹存在遗传性补体第三成分缺陷。

Hereditary deficiency of the third component of complement in two sisters with systemic lupus erythematosus-like symptoms.

作者信息

Sano Y, Nishimukai H, Kitamura H, Nagaki K, Inai S, Hamasaki Y, Maruyama I, Igata A

出版信息

Arthritis Rheum. 1981 Oct;24(10):1255-60. doi: 10.1002/art.1780241005.

DOI:10.1002/art.1780241005
PMID:7306227
Abstract

We observed two sisters with lupus-like syndrome with homozygous C3 deficiencies. A 19-year-old woman and her 15-year-old sister developed malar rash, arthralgia, and photosensitivity, but antinuclear antibodies and LE cell preparations were negative. The older sister experienced recurrent bronchitis in her childhood, but the younger sister had no recurrent infections. Serum C3 was not detected immunochemically in either sister, and total complement activity and C3 hemolytic activity were extremely low.

摘要

我们观察了两名患有纯合子C3缺乏症的狼疮样综合征姐妹。一名19岁女性及其15岁妹妹出现了颧部皮疹、关节痛和光过敏,但抗核抗体和LE细胞检查均为阴性。姐姐童年时有反复支气管炎发作,但妹妹没有反复感染史。两名姐妹的血清C3均未通过免疫化学方法检测到,总补体活性和C3溶血活性极低。

相似文献

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Hereditary deficiency of the third component of complement in two sisters with systemic lupus erythematosus-like symptoms.两名患有系统性红斑狼疮样症状的姐妹存在遗传性补体第三成分缺陷。
Arthritis Rheum. 1981 Oct;24(10):1255-60. doi: 10.1002/art.1780241005.
2
Molecular analysis of hereditary deficiency of the third component of complement (C3) in two sisters.两姐妹遗传性补体第三成分(C3)缺乏症的分子分析
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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。
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Clin Exp Immunol. 1976 May;24(2):238-48.
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