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红斑狼疮女性中X连锁慢性肉芽肿病携带者状态的频率。

Frequency of the carrier state for X-linked chronic granulomatous disease among females with lupus erythematosus.

作者信息

Humbert J R, Fishman C B, Weston W L, DeArmey P A, Thoren C H

出版信息

Clin Genet. 1976 Jul;10(1):16-20. doi: 10.1111/j.1399-0004.1976.tb00003.x.

Abstract

Carriers for chronic granulomatous disease (CGD) and patients with lupus erythematosus (LE) share several characteristics: They are mostly females, they reduce nitroblue tetrazolium (NBT) poorly in their neutrophils, and, in some cases, they have similar skin lesions. We thus investigated 19 female LE patients for the presence of laboratory findings characteristic of the carrier state for CGD. None of these patients turned out to have the combined abnormality of neutrophil bactericidal activity and neutrophil NBT-reduction that is diagnostic of CGD carrier state in the X-linked form. An increased frequency of CGD carriers among female LE patients thus appear to be unlikely. Why some CGD carriers develop skin lesions typical of LE remains unexplained.

摘要

慢性肉芽肿病(CGD)携带者和红斑狼疮(LE)患者有几个共同特征:他们大多为女性,其嗜中性粒细胞对硝基蓝四氮唑(NBT)的还原能力较差,并且在某些情况下,他们有相似的皮肤病变。因此,我们对19名女性LE患者进行了调查,以寻找具有CGD携带者状态特征性实验室检查结果的情况。这些患者中没有一人出现嗜中性粒细胞杀菌活性和嗜中性粒细胞NBT还原能力的联合异常,而这种联合异常是X连锁型CGD携带者状态的诊断依据。因此,女性LE患者中CGD携带者的频率增加似乎不太可能。为何一些CGD携带者会出现典型的LE皮肤病变仍无法解释。

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