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[选择性免疫球蛋白A缺乏症的重要性]

[Importance of selective immunoglobulin A deficiency].

作者信息

Rojas-Torres D S, Bastidas-Yaguana D K, Sierra-Santos L, Aguilar-Shea A L

机构信息

Medicina de Familia y Comunitaria, Centro de Salud Canillejas, Madrid, España.

Medicina de Familia y Comunitaria, Centro de Salud Canillejas, Madrid, España.

出版信息

Semergen. 2014 Apr;40(3):e65-8. doi: 10.1016/j.semerg.2013.01.013. Epub 2013 Dec 19.

DOI:10.1016/j.semerg.2013.01.013
PMID:24360868
Abstract

Selective IgA deficiency is the most common primary immunodeficiency. Little is known about the pathogenetic mechanisms leading to this disease. It is estimated that the incidence of this disease in Spain is 1:163. We report 3 cases diagnosed in Primary Care. Most people with selective IgA deficiency are usually asymptomatic and are diagnosed by chance, but patients with recurrent respiratory infections; gastrointestinal, allergic and autoimmune diseases can be associated with this disease. Although there is no treatment at present, its importance lies in the association with different diseases, such as coeliac disease or idiopathic thrombocytopenic purpura. Another important feature is the risk of anaphylaxis after transfusion, and the possible progression to Common Variable Immunodeficiency. It is important to know the vaccines that we can use due to the risk of disease.

摘要

选择性IgA缺乏症是最常见的原发性免疫缺陷病。导致这种疾病的发病机制鲜为人知。据估计,该病在西班牙的发病率为1:163。我们报告了3例在基层医疗中诊断出的病例。大多数选择性IgA缺乏症患者通常无症状,是偶然被诊断出来的,但反复出现呼吸道感染、胃肠道疾病、过敏和自身免疫性疾病的患者可能与这种疾病有关。虽然目前尚无治疗方法,但其重要性在于与不同疾病的关联,如乳糜泻或特发性血小板减少性紫癜。另一个重要特征是输血后发生过敏反应的风险,以及可能进展为常见变异型免疫缺陷病。由于存在患病风险,了解我们可以使用的疫苗很重要。

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