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家族性中性粒细胞趋化性缺陷、复发性细菌感染、黏膜皮肤念珠菌病和高免疫球蛋白E血症。

Familial neutrophil chemotaxis defect, recurrent bacterial infections, mucocutaneous candidiasis, and hyperimmunoglobulinemia E.

作者信息

Van Scoy R E, Hill H R, Ritts R E, Quie P G

出版信息

Ann Intern Med. 1975 Jun;82(6):766-71. doi: 10.7326/0003-4819-82-6-766.

Abstract

A 20-year old women and her infant daughter had recurrent bacterial infections and chronic mucocutaneous candidiasis and were found to have extreme hyperimmunoglobulinemia E, defective neutrophil chemotaxis, and diminished lymphocyte responses to Candida antigen. Studies of members of the mother's family showed mild increases of IgE and mildly depressed chemotactic activity of neutrophils in a brother, the father, and the paternal grandfather. The recurrent bacterial infections in these two patients can be explained by the defective neutrophil chemotaxis. It is not known whether the mucocutaneous candidiasis is related to the neutrophil chemotaxis with the lymphocyte defect being secondary to the Candida infection or, alternatively, the Candida infection being secondary to the lymphocyte defect. Furthermore, the family data suggest a familial pattern of hyperimmunoglobulinemia E and defective neutrophil motility.

摘要

一名20岁女性及其婴儿女儿反复发生细菌感染和慢性黏膜皮肤念珠菌病,发现她们有极度高免疫球蛋白E血症、中性粒细胞趋化性缺陷以及淋巴细胞对念珠菌抗原的反应减弱。对母亲家族成员的研究显示,其兄弟、父亲和祖父的免疫球蛋白E轻度升高,中性粒细胞趋化活性轻度降低。这两名患者反复发生的细菌感染可通过中性粒细胞趋化性缺陷来解释。目前尚不清楚黏膜皮肤念珠菌病是否与中性粒细胞趋化性有关,淋巴细胞缺陷是念珠菌感染的继发表现,还是相反,念珠菌感染是淋巴细胞缺陷的继发表现。此外,家族数据提示高免疫球蛋白E血症和中性粒细胞运动性缺陷存在家族模式。

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