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一种伴有哮喘、湿疹和复发性皮肤感染的中性粒细胞趋化性家族性缺陷。

A familial defect of neutrophil chemotaxis with asthma, eczema, and recurrent skin infections.

作者信息

Jacobs J C, Norman M E

出版信息

Pediatr Res. 1977 Jun;11(6):732-6. doi: 10.1203/00006450-197706000-00007.

Abstract

A defect in chemotaxis of peripheral blood polymorphonuclear leukocytes (PMN's) was demonstrated in both parents and three of four children in a single family afflicted with varying degrees of respiratory allergy, unusual onset of severe eczema in the first month of life, and recurrent bacterial skin infections. Of great interest was the identification of HLA-B12 at the B locus in all affected members but not in the unaffected child. The two children known since infancy to be most severely affected with eczema and recurrent infections are HLA identical and homozygous for HLA-B12. The child without eczema and infections had an intermediate cellular chemotactic defect most apparent on kinetic studies.

摘要

在一个患有不同程度呼吸道过敏、出生后第一个月出现严重湿疹且反复发生细菌性皮肤感染的家庭中,父母及四个孩子中的三个外周血多形核白细胞(PMN)的趋化性存在缺陷。有趣的是,在所有患病成员的B位点均鉴定出HLA - B12,而未患病的孩子则没有。自婴儿期起就患有最严重湿疹和反复感染的两个孩子HLA相同且为HLA - B12纯合子。没有湿疹和感染的孩子存在中度细胞趋化性缺陷,在动力学研究中最为明显。

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