遗传性补体系统缺陷。
Inherited defects in the complement system.
机构信息
Department of Maternal Infantile and Urological Sciences, Sapienza University of Rome, Rome, Italy.
Department of Pediatrics, Giovanni XXIII Pediatric Hospital, University of Bari, Bari, Italy.
出版信息
Pediatr Allergy Immunol. 2022 Jan;33 Suppl 27(Suppl 27):73-76. doi: 10.1111/pai.13635.
The complement system plays an essential role in both innate and adaptive immune responses. Any dysregulation in this system can disturb normal host defense and alter inflammatory response leading to both infections and autoimmune diseases. The complement system can be activated through three different pathways. Inherited complement deficiencies have been described for all complement components and their regulators. Despite being rare diseases, complement deficiencies are often severe, with a frequent onset during childhood. We provide an overview of clinical disorders related to these disorders and describe current diagnostic strategies required for their comprehensive characterization and management.
补体系统在先天和适应性免疫反应中都起着至关重要的作用。该系统的任何失调都可能扰乱正常的宿主防御并改变炎症反应，从而导致感染和自身免疫性疾病。补体系统可以通过三种不同的途径被激活。所有补体成分及其调节剂的遗传性补体缺陷都已被描述。尽管这些疾病较为罕见，但补体缺陷通常较为严重，且常在儿童时期发病。我们提供了与这些疾病相关的临床疾病概述，并描述了目前为全面描述和管理这些疾病所需的诊断策略。
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