Improvement in Atopic Dermatitis and Recurrent Infection With Dupilumab in Children With Distinct Genetic Types of Hyper-IgE Syndrome: A Case Series and Literature Review.

IF 16.4 1区化学 Q1 CHEMISTRY, MULTIDISCIPLINARY

Accounts of Chemical Research Pub Date : 2024-10-18 DOI:10.1111/pde.15780

Jenna K Dick, Christina Boull, Tamara C Pozos, Sheilagh M Maguiness

引用次数: 0

Abstract

Hyperimmunoglobulin E syndrome (HIES) is a group of rare genetic disorders characterized by severe atopic dermatitis and recurrent skin and pulmonary infections. The efficacy of dupilumab in pediatric patients with HIES-associated severe atopic dermatitis is relatively understudied. Here, we present a series of three children with HIES, two with AD-HIES caused by STAT3 mutations, and one with AR-HIES caused by biallelic mutations in ZNF341. In all cases, dupilumab treatment led to sustained clearance of severe atopic dermatitis over multiple years, as well as improvements in systemic symptoms of HIES.

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使用杜匹单抗可改善不同基因型高 IgE 综合征患儿的特应性皮炎和复发性感染：病例系列和文献综述

高免疫球蛋白 E 综合征（HIES）是一组罕见的遗传性疾病，以严重特应性皮炎和反复皮肤及肺部感染为特征。杜比鲁单抗对 HIES 相关重度特应性皮炎儿科患者的疗效研究相对较少。在此，我们介绍了三例 HIES 患儿，其中两例是由 STAT3 突变引起的 AD-HIES 患儿，另一例是由 ZNF341 双重突变引起的 AR-HIES 患儿。在所有病例中，dupilumab 治疗均能使严重特应性皮炎在数年内持续消失，并改善 HIES 的全身症状。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Accounts of Chemical Research 化学-化学综合

CiteScore

31.40

自引率

1.10%

发文量

312

审稿时长

2 months

期刊介绍： Accounts of Chemical Research presents short, concise and critical articles offering easy-to-read overviews of basic research and applications in all areas of chemistry and biochemistry. These short reviews focus on research from the author’s own laboratory and are designed to teach the reader about a research project. In addition, Accounts of Chemical Research publishes commentaries that give an informed opinion on a current research problem. Special Issues online are devoted to a single topic of unusual activity and significance. Accounts of Chemical Research replaces the traditional article abstract with an article "Conspectus." These entries synopsize the research affording the reader a closer look at the content and significance of an article. Through this provision of a more detailed description of the article contents, the Conspectus enhances the article's discoverability by search engines and the exposure for the research.