与牙周相关的白细胞黏附缺陷综合征-Ⅰ型研究进展  

Research progress of periodontally related leukocyte adhesion deficiency-Ⅰ

作　　者：李阳 李厚轩[1] 闫福华[1] LI Yang;LI Hou-xuan;YAN Fuhua(Department o f Periodontology, Nanjing Stomatological Hospital, Medical School o f Nanjing University, Nanjing 210008,Chirm)

机构地区：[1]南京大学医学院附属口腔医院,南京市口腔医院牙周病科,江苏南京210008

出　　处：《中国实用口腔科杂志》2019年第5期257-260,共4页Chinese Journal of Practical Stomatology

基　　金：江苏省科教强卫工程医学创新团队(CXTDB2017014);南京市卫生青年人才培养工程培养对象(QRX17025)

摘　　要：白细胞黏附缺陷综合征(leukocyte adhesion deficiency,LAD)是一种罕见的常染色体隐性遗传病。LAD被分为3种亚型:LAD-Ⅰ、LAD-Ⅱ和LAD-Ⅲ,其中LAD-Ⅰ最常见,但国内有关LAD-Ⅰ的研究报道不多,国外文献报道其发病率为1:1 000 000。LAD-Ⅰ的发病机制主要是由于CD18基因突变或缺陷导致白细胞黏附和迁移障碍,其口腔表现以快速进展的牙龈炎、牙周炎及复发性口腔溃疡为主,全身症状为局限于皮肤和黏膜表面的复发性感染、危及生命的细菌和真菌感染。文章就与牙周相关的LAD-Ⅰ研究进展做一介绍。Leukocyte adhesion deficiency(LAD)is a rare autosomal recessive disease. There are three types of LADs:LAD-Ⅰ,which is the most common type,LAD-Ⅱand LAD-Ⅲ. Domestic report on LAD-Ⅰ is scarce,prevalence of 1:1 000 000 people in foreign reports. LAD-Ⅰ is characterized by disorder of leukocyte adhesion and migration caused by mutations or deficiency in the gene encoding CD18.Systemic symptoms of LAD-Ⅰ are mainly recurrent,lifethreatening bacterial and fungal infections confined to skin and mucosal surfaces. Oral manifestations include rapid progressive gingivitis,periodontitis and recurrent oral ulcer. In this review,we try to highlight the recent updates on LAD-Ⅰrelated to periodontitis.

关 键 词：白细胞黏附缺陷综合征-Ⅰ型 牙周病 CD18 基因诊断 

分 类 号：R78[医药卫生—口腔医学]