吞噬细胞数量及功能缺陷  

Congenital defects of phagocyte number or function

作　　者：贺建新[1] He Jianxin(National Children＇s Medical Center, Department of Respiratory, Beijing Children＇s Hospital, Capital Medical University, National Clinical Research Center for Respiratory Diseases, Beijing 100045, Chin)

机构地区：[1]国家儿童医学中心,首都医科大学附属北京儿童医院呼吸科,国家呼吸系统疾病临床医学研究中心,100045

出　　处：《中华实用儿科临床杂志》2018年第4期291-297,共7页Chinese Journal of Applied Clinical Pediatrics

摘　　要：吞噬细胞数量及功能缺陷属于最新原发性免疫缺陷病分类中的第5大类。最严重的吞噬细胞数量缺陷疾病为严重先天性中性粒细胞减少。吞噬细胞功能包括移动、趋化、黏附、吞噬和杀菌等功能。临床最常见的吞噬细胞功能缺陷包括黏附分子缺陷和慢性肉芽肿病。现分别阐述严重先天性中性粒细胞减少、白细胞黏附分子缺陷和慢性肉芽肿病3类疾病的发病机制、分子特征、临床特点、实验室发现、鉴别诊断、治疗及预后等。Congenital defects of phagocyte number, function or both was categorized to the fifth classification from the international union of immunological societies expert committee for primary immunodeficiency 2015.Severe congenital neutropenia was the most fatal phagocyte number defect.Phagocyte functions included motility, chemotaxis, adhesion, phagocytosis and killing.Leukocyte adhesion deficiency and chronic granulomatous disease were the most common diseases.This article will describe pathogenesis, molecular, clinical, laboratory features and treatment and prognosis, to supporting clues for paediatrician′s clinical operations.

关 键 词：严重先天性中性粒细胞减少 白细胞黏附分子缺陷 慢性肉芽肿病 

分 类 号：R725.9[医药卫生—儿科]