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四名患有中性粒细胞减少、嗜酸性粒细胞增多、免疫球蛋白A升高和中性粒细胞趋化性缺陷的兄弟姐妹反复发生细菌感染。

Recurrent bacterial infections in four siblings with neutropenia, eosinophilia, hyperimmunoglobulinemia A, and defective neutrophil chemotaxis.

作者信息

Björkstén B, Lundmark K M

出版信息

J Infect Dis. 1976 Jan;133(1):63-71. doi: 10.1093/infdis/133.1.63.

Abstract

Four siblings with recurrent bacterial infections, neutrophil chemotactic defect, neutropenia, and eosinophilia were studied. During periods of infection the peripheral neutrophil count increased to normal, while the eosinophilia disappeared. In addition, these children had high levels of serum IgA and poor antibody responses to tetanus and polio vaccinations. A defect in cell-mediated immunity was demonstrated by an absent or weak reactivity to various skin test antigens and by abnormal lymph node histology. Thus these siblings had an unusual combination of defective inflammatory response and immunologic abnormalities.

摘要

对四名患有复发性细菌感染、中性粒细胞趋化缺陷、中性粒细胞减少和嗜酸性粒细胞增多的兄弟姐妹进行了研究。在感染期间,外周血中性粒细胞计数恢复正常,而嗜酸性粒细胞增多消失。此外,这些儿童血清IgA水平较高,对破伤风和脊髓灰质炎疫苗的抗体反应较差。对各种皮肤试验抗原缺乏或反应较弱以及淋巴结组织学异常证明了细胞介导免疫存在缺陷。因此,这些兄弟姐妹存在炎症反应缺陷和免疫异常的不寻常组合。

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