Pediatric allergy and immunology : official publication of the European Society of Pediatric Allergy and Immunology · 2022
Inherited defects in the complement system.
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Leonardi Lucia, La Torre Francesco, Soresina Annarosa, Federici Silvia, Cancrini Caterina, Castagnoli Riccardo, Cinicola Bianca Laura, Corrente Stefania, Giardino Giuliana, Lougaris Vassilios, Volpi Stefano, Marseglia Gian Luigi, Cardinale Fabio, Immunology Task Force of the Italian Society of Pediatric Allergy and Immunology (SIAIP)
Аннотация
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.