Hyper-immunoglobulin E syndrome
10.3760/cma.j.issn.2095-428X.2018.04.009
- VernacularTitle:高免疫球蛋白E综合征
- Author:
Yong YIN
1
;
Jing ZHANG
Author Information
1. 国家儿童医学中心/上海交通大学医学院附属上海儿童医学中心呼吸科
- Keywords:
Hyper-immunoglobulin syndrome;
STAT3 gene;
Mutation
- From:
Chinese Journal of Applied Clinical Pediatrics
2018;33(4):277-280
- CountryChina
- Language:Chinese
-
Abstract:
Hyper-immunoglobulin syndrome (HIES) is a multisystem disorder characterized by eczema,skin abscesses,recurrent staphylococcal infections of the skin and lungs,pneumatocele formation,candidiasis,cosinophilia,and elevated serum levels of IgE.The number of helper T lymphocyte 17 cells is reduced or absent,and dominant-negative heterozygous mutation in STAT3 gene are helpful for the diagnosis of the disease.The use of prophylactic antibiotics is a necessary measure to prevent suppurative pneumonia,pulmonary cystic cavity formation and bronchiectasis.
