Dosanjh Amrita
Department of Pediatrics, Rady Childrens Hospital, San Diego, CA, USA.
J Asthma Allergy. 2017 Feb 28;10:31-33. doi: 10.2147/JAA.S129160. eCollection 2017.
Patients with autosomal-dominant (AD) hyper immunoglobulin E (IgE) syndrome (HIES) or Job syndrome develop frequent dermatologic and pulmonary infections. As patients have an extreme elevation of IgE levels, this database analysis study sought to study the association between AD HIES, Job syndrome, and anaphylaxis. HIES is a heterogeneous group of immune disorders characterized by extremely elevated levels of serum IgE. Although the molecular defects and clinical phenotypes found in association with Job syndrome are well characterized, the association with severe allergic reactions and anaphylaxis is a subject of ongoing investigation.
常染色体显性(AD)高免疫球蛋白E(IgE)综合征(HIES)或乔布综合征患者经常发生皮肤和肺部感染。由于患者的IgE水平极度升高,这项数据库分析研究旨在探讨AD HIES、乔布综合征与过敏反应之间的关联。HIES是一组异质性免疫疾病,其特征是血清IgE水平极度升高。尽管与乔布综合征相关的分子缺陷和临床表型已得到充分描述,但与严重过敏反应和过敏的关联仍在研究中。