原发性免疫缺陷病：儿童和成人疾病——综述。
Primary Immunodeficiencies: Diseases of Children and Adults - A Review.
机构信息
Third Department and Clinic of Pediatrics, Immunology and Rheumatology of Developmental Age, Wroclaw Medical University, Wroclaw, Poland.
Department of Immunology and Pediatrics, The J. Gromkowski Provincial Hospital, Wroclaw, Poland.
出版信息
Adv Exp Med Biol. 2021;1289:37-54. doi: 10.1007/5584_2020_556.
Primary immunodeficiencies (PIDs) belong to a group of rare congenital diseases occurring all over the world that may be seen in both children and adults. In most cases, genetic predispositions are already known. As shown in this review, genetic abnormalities may be related to dysfunction of the immune system, which manifests itself as recurrent infections, increased risk of cancer, and autoimmune diseases. This article reviews the various forms of PIDs, including their characterization, management strategies, and complications. Novel aspects of the diagnostics and monitoring of PIDs are presented.
原发性免疫缺陷病（PIDs）属于一组在世界各地都可能发生的罕见先天性疾病，可发生于儿童及成人。在大多数情况下，其遗传易感性已被知晓。正如本综述中所显示的，遗传异常可能与免疫系统功能障碍有关，其表现为反复感染、癌症风险增加和自身免疫性疾病。本文综述了各种形式的 PID，包括其特征、管理策略和并发症。本文还介绍了 PID 诊断和监测的新方面。
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