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Inherited defects in the complement system
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 differ...
| Autores principales: | , , , , , , , , , , , , |
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| Formato: | Online Artículo Texto |
| Lenguaje: | English |
| Publicado: |
John Wiley and Sons Inc.
2022
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| Materias: | |
| Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9544610/ https://www.ncbi.nlm.nih.gov/pubmed/35080299 http://dx.doi.org/10.1111/pai.13635 |
| _version_ | 1784804634787840000 |
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| author | 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 |
| author_facet | 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 |
| author_sort | Leonardi, Lucia |
| collection | PubMed |
| description | 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. |
| format | Online Article Text |
| id | pubmed-9544610 |
| institution | National Center for Biotechnology Information |
| language | English |
| publishDate | 2022 |
| publisher | John Wiley and Sons Inc. |
| record_format | MEDLINE/PubMed |
| spelling | pubmed-95446102022-10-14 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 Pediatr Allergy Immunol Special Issue: 2021 Update From The Italian Society Of Pediatric Allergy And Immunology 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. John Wiley and Sons Inc. 2022-01-25 2022-01 /pmc/articles/PMC9544610/ /pubmed/35080299 http://dx.doi.org/10.1111/pai.13635 Text en © 2022 The Authors. Pediatric Allergy and Immunology published by European Academy of Allergy and Clinical Immunology and John Wiley & Sons Ltd. https://creativecommons.org/licenses/by-nc/4.0/This is an open access article under the terms of the http://creativecommons.org/licenses/by-nc/4.0/ (https://creativecommons.org/licenses/by-nc/4.0/) License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited and is not used for commercial purposes. |
| spellingShingle | Special Issue: 2021 Update From The Italian Society Of Pediatric Allergy And Immunology 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 Inherited defects in the complement system |
| title | Inherited defects in the complement system |
| title_full | Inherited defects in the complement system |
| title_fullStr | Inherited defects in the complement system |
| title_full_unstemmed | Inherited defects in the complement system |
| title_short | Inherited defects in the complement system |
| title_sort | inherited defects in the complement system |
| topic | Special Issue: 2021 Update From The Italian Society Of Pediatric Allergy And Immunology |
| url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9544610/ https://www.ncbi.nlm.nih.gov/pubmed/35080299 http://dx.doi.org/10.1111/pai.13635 |
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