期刊简介

               本杂志是由中华人民共和国卫生主管,同济医科大学附属协和医院主办,国内外公开发行的学术性期刊,1996年被评为全国优秀期刊。辟有专家论坛、临床研究、实验研究、技术与方法、研究报告、综述、进修苑、学术争鸣、经验介绍及病例报告等多个栏目。                

首页>临床耳鼻咽喉头颈外科杂志
  • 杂志名称:临床耳鼻咽喉头颈外科杂志
  • 主管单位:中华人民共和国教育部
  • 主办单位:华中科技大学同济医学院附属协和医院
  • 国际刊号:1001-1781
  • 国内刊号:42-1764/R
  • 出版周期:半月刊
期刊荣誉:第二届国家期刊奖提名奖期刊收录:万方收录(中), 维普收录(中), 国家图书馆馆藏, 知网收录(中), 上海图书馆馆藏, CA 化学文摘(美), 文摘与引文数据库, 北大核心期刊(中国人文社会科学核心期刊), 统计源核心期刊(中国科技论文核心期刊), JST 日本科学技术振兴机构数据库(日)
临床耳鼻咽喉头颈外科杂志2017年第11期

高IgE综合征

何颖瑶;刘斌

关键词:高IgE综合征, STAT3, DOCK8, TYK2
摘要:A hyper-IgE syndrome is a rare immunodeficiesncy disease,often accompanied by high serum IgE.It often characterized by facial features,repeated skin infections,eczema and pulmonary infection,including autosomal dominant HIES(AD-HIES) and autosomal recessive HIES(AR-HIES).AR-HIES is caused by mutations in STAT3,which is presented as connective tissue,bone,vascular disease,and high brain white matter signal.ADHIES is mainly caused by mutations in DOCK8 and TYK2,which is presented as severe viral infection and poor prognosis.The treatment for Hyper-IgE syndromes is mainly to control infection,skin care and other symptomatic treatment,if necessary,should be done as early as possible hematopoietic stem cell transplantation.This article reviews the clinical manifestations,pathogenesis and treatment of high IgE syndrome.