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Inherited defects in the complement system.
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.
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  • Leonardi L; Department of Maternal Infantile and Urological Sciences, Sapienza University of Rome, Rome, Italy.
  • La Torre F; Department of Pediatrics, Giovanni XXIII Pediatric Hospital, University of Bari, Bari, Italy.
  • Soresina A; Unit of Pediatric Immunology, Pediatrics Clinic, University of Brescia, ASST Spedali Civili Brescia, Brescia, Italy.
  • Federici S; Division of Rheumatology, IRCCS, Ospedale Pediatrico Bambino Gesù, Rome, Italy.
  • Cancrini C; Department of Systems Medicine, University of Rome Tor Vergata, Rome, Italy.
  • Castagnoli R; Academic Department of Pediatrics, Immune, and Infectious Diseases Division, Research Unit of Primary Immunodeficiencies, Bambino Gesù, Children's Hospital, IRCCS, Rome, Italy.
  • Cinicola BL; Department of Pediatrics, Pediatric Clinic, Fondazione IRCCS Policlinico San Matteo, University of Pavia, Pavia, Italy.
  • Corrente S; Department of Maternal Infantile and Urological Sciences, Sapienza University of Rome, Rome, Italy.
  • Giardino G; Department of Experimental Medicine, Sapienza University of Rome, Rome, Italy.
  • Lougaris V; Division of Pediatrics, S. Camillo-Forlanini Hospital, Rome, Italy.
  • Volpi S; Pediatric Section, Department of Translational Medical Sciences, Federico II University, Naples, Italy.
  • Marseglia GL; Department of Clinical and Experimental Sciences, Pediatrics Clinic and Institute for Molecular Medicine A. Nocivelli, University of Brescia and ASST-Spedali Civili di Brescia, Brescia, Italy.
  • Cardinale F; Center for Autoinflammatory Diseases and Immunodeficiency, IRCCS Istituto Giannina Gaslini, Università degli Studi di Genova, Genoa, Italy.
Pediatr Allergy Immunol ; 33 Suppl 27: 73-76, 2022 01.
Article en En | MEDLINE | ID: mdl-35080299
ABSTRACT
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.
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Proteínas del Sistema Complemento Límite: Humans Idioma: En Revista: Pediatr Allergy Immunol Asunto de la revista: ALERGIA E IMUNOLOGIA / PEDIATRIA Año: 2022 Tipo del documento: Article País de afiliación: Italia
Texto completo
Añadir a Mi BVS
Imprimir
XML
PubMed Links
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Proteínas del Sistema Complemento Límite: Humans Idioma: En Revista: Pediatr Allergy Immunol Asunto de la revista: ALERGIA E IMUNOLOGIA / PEDIATRIA Año: 2022 Tipo del documento: Article País de afiliación: Italia