描述 ARPC1B 中的一种新型致病变体和两名婴儿的严重过敏症。

IF 1.2 4区 医学 Q4 ALLERGY Iranian journal of allergy, asthma, and immunology Pub Date : 2024-02-11 DOI:10.18502/ijaai.v23i1.14960
Oscar Zaveleta Martínez, Ana Eunice Fregoso-Zuñiga, Cielo Razo Requena, Sara Espinosa Padilla, Lzbeth Blancas Galicia
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引用次数: 0

摘要

肌动蛋白相关蛋白 2/3 复合物亚基 1B(ARPC1B)缺乏症是一种先天性免疫错误(IEI),其特征是免疫缺陷和免疫调节失调的结合,被归类为具有过敏表现的 IEI。在此,我们描述了两名患有 ARPC1B 基因致病变异的患者。第一例患者在六个月大时出现湿疹和支气管痉挛。第二名患者在五个月大时出现湿疹和牛奶蛋白过敏。c.899_944(p.Glu300Glyfs*7)致病变异是以前描述过的,而c.863del(p.Pro288Leufs*9)变异则是新发现的。ARPC1B缺乏症患者在幼年时就会出现严重的过敏表现,因此应考虑该病。
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Description of a Novel Pathogenic Variant in the ARPC1B and a Severe Allergy in Two Infants.

Actinrelated protein 2/3 complex subunit 1B (ARPC1B) deficiency is an inborn error of immunity (IEI) characterized by a combination of immunodeficiency and immune dysregulation and classified as an IEI with allergic manifestations. Here, we describe two patients with pathogenic variants in the ARPC1B gene. The first patient presented with eczema and bronchospasm at six months of age. The second patient presented with eczema and milk protein allergy at five months of age. The c.899_944 (p.Glu300Glyfs*7) pathogenic variant was previously described, whereas the c.863del (p.Pro288Leufs*9) variant was novel. ARPC1B deficiency should be considered because of the severe allergic manifestations at an early age.

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来源期刊
CiteScore
2.60
自引率
6.70%
发文量
64
审稿时长
>12 weeks
期刊介绍: The Iranian Journal of Allergy, Asthma and Immunology (IJAAI), an international peer-reviewed scientific and research journal, seeks to publish original papers, selected review articles, case-based reviews, and other articles of special interest related to the fields of asthma, allergy and immunology. The journal is an official publication of the Iranian Society of Asthma and Allergy (ISAA), which is supported by the Immunology, Asthma and Allergy Research Institute (IAARI) and published by Tehran University of Medical Sciences (TUMS). The journal seeks to provide its readers with the highest quality materials published through a process of careful peer reviews and editorial comments. All papers are published in English.
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