CD154 deficiency and related syndromes

被引：5

作者：

Bonilla, FA

Geha, RS

机构：

[1] Childrens Hosp, Boston, MA 02115 USA

[2] Harvard Univ, Sch Med, Boston, MA USA

来源：

IMMUNOLOGY AND ALLERGY CLINICS OF NORTH AMERICA | 2001年 / 21卷 / 01期

关键词：

D O I：

10.1016/S0889-8561(05)70193-4

中图分类号：

R392 [医学免疫学];

学科分类号：

100102 ;

摘要：

The X-linked hyper-IgM syndrome results from mutations in the CD154 gene. The contact of CD154 with its ligand, CD40, underlies a wide variety of immunoregulatory cellular dialogues. This article provides an overview of the physiology of CD40-154 interactions and presents a detailed description of the clinical and immunologic features of more than 100 reported cases of XHIM. Aspects of the genetics, diagnosis, and treatment of XHIM and the clinical and immunologic features of the autosomal recessive forms of hyper-IgM syndrome in comparison with XHIM are also discussed.

引用

页码：65 / +

页数：26

共 155 条

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