重症联合免疫缺陷——分类、微生物学关联及治疗
Severe Combined Immunodeficiency-Classification, Microbiology Association and Treatment.
作者信息
Justiz-Vaillant Angel A, Gopaul Darren, Akpaka Patrick Eberechi, Soodeen Sachin, Arozarena Fundora Rodolfo
机构信息
Department of Paraclinical Sciences, Faculty of Medical Sciences, The University of the West Indies, St. Augustine, Trinidad and Tobago.
Department of Internal Medicine, Port of Spain General Hospital, The University of the West Indies, St. Augustine, Trinidad and Tobago.
出版信息
Microorganisms. 2023 Jun 15;11(6):1589. doi: 10.3390/microorganisms11061589.
Abstract
Severe combined immunodeficiency (SCID) is a primary inherited immunodeficiency disease that presents before the age of three months and can be fatal. It is usually due to opportunistic infections caused by bacteria, viruses, fungi, and protozoa resulting in a decrease in number and impairment in the function of T and B cells. Autosomal, X-linked, and sporadic forms exist. Evidence of recurrent opportunistic infections and lymphopenia very early in life should prompt immunological investigation and suspicion of this rare disorder. Adequate stem cell transplantation is the treatment of choice. This review aimed to provide a comprehensive approach to the microorganisms associated with severe combined immunodeficiency (SCID) and its management. We describe SCID as a syndrome and summarize the different microorganisms that affect children and how they can be investigated and treated.
摘要
重症联合免疫缺陷病（SCID）是一种原发性遗传性免疫缺陷病，在三个月龄前发病，可能致命。它通常是由细菌、病毒、真菌和原生动物引起的机会性感染所致，导致T细胞和B细胞数量减少及功能受损。存在常染色体、X连锁和散发型。生命早期出现反复机会性感染和淋巴细胞减少的证据应促使进行免疫学检查并怀疑患有这种罕见疾病。充分的干细胞移植是首选治疗方法。本综述旨在提供一种全面的方法来处理与重症联合免疫缺陷病（SCID）相关的微生物及其管理。我们将SCID描述为一种综合征，并总结影响儿童的不同微生物以及如何对其进行检查和治疗。
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