Clinical and immunological features of specific antibody deficiency in a pediatric hospital in Colombia

Lina M Castaño-Jaramillo, Alejandra Munevar, Andrea Carolina Marín, Milena Villamil, Sonia Restrepo, Natalia Vélez
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Abstract

Introduction. Specific antibody deficiency is an innate error of humoral immunity characterized by normal levels of immunoglobulin isotypes, recurrent infections, and a reduced reaction to polysaccharide antigens in vaccines. Objective. To describe the clinical and immunological characteristics of patients with specific antibody deficiency attending a pediatric hospital in Bogotá between May 2021 and September 2023. Materials and methods. We reviewed the medical records of 16 patients with specific antibody deficiency. Results. The median age at diagnosis was six and a half years. Nine were male, and 7 had a history of prematurity. Eleven patients had adequate nutritional status, and 7 had standard height. The most frequent recurrent infection was pneumonia, affecting 12 patients; more than half of them experienced some associated complications. The most common phenotype was moderate, and 15 of the individuals received immunoglobulin as definitive treatment. Conclusion. Specific antibody deficiency is a frequently underdiagnosed functional alteration of the immune system. It should be suspected in patients experiencing recurrent otitis media and pneumonia or in cases complicated by septic shock, pleural effusion, or necrotizing pneumonia.

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哥伦比亚儿科医院特异性抗体缺乏症的临床和免疫学特征
介绍。特异性抗体缺乏是一种体液免疫的先天错误,其特征是免疫球蛋白同型水平正常、反复感染和对疫苗中多糖抗原的反应降低。描述2021年5月至2023年9月期间在波哥大儿科医院就诊的特异性抗体缺乏症患者的临床和免疫学特征。材料和方法。我们回顾了16例特异性抗体缺乏症患者的病历。诊断时的中位年龄是6岁半。其中9例为男性,7例有早产史。11例营养状况良好,7例身高达标。最常见的复发感染是肺炎,12例;其中一半以上的人经历了一些相关的并发症。最常见的表型为中度,其中15例接受免疫球蛋白作为最终治疗。特异性抗体缺乏症是一种经常被误诊的免疫系统功能改变。反复出现中耳炎和肺炎的患者,或合并感染性休克、胸腔积液或坏死性肺炎的患者,应予以怀疑。
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