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Failure of immunological competence: when to suspect?

OBJECTIVES: To draw physicians’ attention to the different warning signs of diseases of inborn errors of immunity. DATA SOURCES: A non-systematic review of the literature was carried out in the PubMed, LILACS, and SciELO databases, in addition to consultation of reference textbooks. SUMMARY OF THE F...

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Autor principal: Pinto-Mariz, Fernanda
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9432048/
https://www.ncbi.nlm.nih.gov/pubmed/33176165
http://dx.doi.org/10.1016/j.jped.2020.10.007
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author Pinto-Mariz, Fernanda
author_facet Pinto-Mariz, Fernanda
author_sort Pinto-Mariz, Fernanda
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description OBJECTIVES: To draw physicians’ attention to the different warning signs of diseases of inborn errors of immunity. DATA SOURCES: A non-systematic review of the literature was carried out in the PubMed, LILACS, and SciELO databases, in addition to consultation of reference textbooks. SUMMARY OF THE FINDINGS: It is known that the lack of immunological competence observed in patients with inborn errors of immunity diseases causes particularly serious and/or recurrent infections. However, manifestations related to autoimmunity, inflammation, allergies, and malignancy can also occur. Aiming at the early identification of these patients, a list of warning signs for inborn errors of immunity was created, in which the need for intravenous antibiotics or prolonged antibiotics use to control infection, failure to thrive, and positive family history for this group of diseases are considered the most sensitive. Regarding non-infectious manifestations, early onset, difficulty in controlling with the usual treatments, atypical presentations or association with other warning signs are noteworthy, and investigation for inborn errors of immunity in these situations is recommended. CONCLUSIONS: This article highlights the importance of considering this group of diseases even in the face of patients with non-infectious manifestations. Disclosure of inborn errors of immunity diseases, especially to non-specialists, is essential for early diagnosis and, consequently, for the reduction of these patients' morbidity and mortality.
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spelling pubmed-94320482022-09-08 Failure of immunological competence: when to suspect? Pinto-Mariz, Fernanda J Pediatr (Rio J) Review Article OBJECTIVES: To draw physicians’ attention to the different warning signs of diseases of inborn errors of immunity. DATA SOURCES: A non-systematic review of the literature was carried out in the PubMed, LILACS, and SciELO databases, in addition to consultation of reference textbooks. SUMMARY OF THE FINDINGS: It is known that the lack of immunological competence observed in patients with inborn errors of immunity diseases causes particularly serious and/or recurrent infections. However, manifestations related to autoimmunity, inflammation, allergies, and malignancy can also occur. Aiming at the early identification of these patients, a list of warning signs for inborn errors of immunity was created, in which the need for intravenous antibiotics or prolonged antibiotics use to control infection, failure to thrive, and positive family history for this group of diseases are considered the most sensitive. Regarding non-infectious manifestations, early onset, difficulty in controlling with the usual treatments, atypical presentations or association with other warning signs are noteworthy, and investigation for inborn errors of immunity in these situations is recommended. CONCLUSIONS: This article highlights the importance of considering this group of diseases even in the face of patients with non-infectious manifestations. Disclosure of inborn errors of immunity diseases, especially to non-specialists, is essential for early diagnosis and, consequently, for the reduction of these patients' morbidity and mortality. Elsevier 2020-11-08 /pmc/articles/PMC9432048/ /pubmed/33176165 http://dx.doi.org/10.1016/j.jped.2020.10.007 Text en © 2020 Published by Elsevier Editora Ltda. on behalf of Sociedade Brasileira de Pediatria. https://creativecommons.org/licenses/by-nc-nd/4.0/This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
spellingShingle Review Article
Pinto-Mariz, Fernanda
Failure of immunological competence: when to suspect?
title Failure of immunological competence: when to suspect?
title_full Failure of immunological competence: when to suspect?
title_fullStr Failure of immunological competence: when to suspect?
title_full_unstemmed Failure of immunological competence: when to suspect?
title_short Failure of immunological competence: when to suspect?
title_sort failure of immunological competence: when to suspect?
topic Review Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9432048/
https://www.ncbi.nlm.nih.gov/pubmed/33176165
http://dx.doi.org/10.1016/j.jped.2020.10.007
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