{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,13]],"date-time":"2026-10-13T17:27:55Z","timestamp":1791912475395,"version":"4.3.4"},"reference-count":10,"publisher":"Wiley","issue":"S27","license":[{"start":{"date-parts":[[2022,1,25]],"date-time":"2022-01-25T00:00:00Z","timestamp":1643068800000},"content-version":"vor","delay-in-days":24,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Allergy Immunology"],"published-print":{"date-parts":[[2022,1]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>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.<\/jats:p>","DOI":"10.1111\/pai.13635","type":"journal-article","created":{"date-parts":[[2022,1,26]],"date-time":"2022-01-26T06:29:44Z","timestamp":1643178584000},"page":"73-76","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":15,"title":["Inherited defects in the complement system"],"prefix":"10.1111","volume":"33","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-2554-1263","authenticated-orcid":false,"given":"Lucia","family":"Leonardi","sequence":"first","affiliation":[{"name":"Department of Maternal Infantile and Urological Sciences Sapienza University of Rome  Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5235-2900","authenticated-orcid":false,"given":"Francesco","family":"La Torre","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Giovanni XXIII Pediatric Hospital University of Bari  Bari Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Annarosa","family":"Soresina","sequence":"additional","affiliation":[{"name":"Unit of Pediatric Immunology Pediatrics Clinic University of Brescia ASST Spedali Civili Brescia  Brescia Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7780-6430","authenticated-orcid":false,"given":"Silvia","family":"Federici","sequence":"additional","affiliation":[{"name":"Division of Rheumatology IRCCS Ospedale Pediatrico Bambino Ges\u00f9  Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8410-9617","authenticated-orcid":false,"given":"Caterina","family":"Cancrini","sequence":"additional","affiliation":[{"name":"Department of Systems Medicine University of Rome Tor Vergata  Rome Italy"},{"name":"Academic Department of Pediatrics, Immune, and Infectious Diseases Division Research Unit of Primary Immunodeficiencies Bambino Ges\u00f9 Children\u2019s Hospital IRCCS  Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0029-9383","authenticated-orcid":false,"given":"Riccardo","family":"Castagnoli","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Pediatric Clinic Fondazione IRCCS Policlinico San Matteo University of Pavia  Pavia Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1268-5038","authenticated-orcid":false,"given":"Bianca Laura","family":"Cinicola","sequence":"additional","affiliation":[{"name":"Department of Maternal Infantile and Urological Sciences Sapienza University of Rome  Rome Italy"},{"name":"Department of Experimental Medicine Sapienza University of Rome  Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stefania","family":"Corrente","sequence":"additional","affiliation":[{"name":"Division of Pediatrics S. 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