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Advances in the Care of Primary Immunodeficiencies (PIDs): from Birth to Adulthood
Primary immunodeficiencies (PIDs) are a widely heterogeneous group of inherited defects of the immune system consisting of many clinical phenotypes with at least 300 underlying genetic deficits currently known. Patients with PIDs can present with, or develop during the course of their life, a suscep...
Autores principales: | , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Springer US
2017
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5489581/ https://www.ncbi.nlm.nih.gov/pubmed/28523402 http://dx.doi.org/10.1007/s10875-017-0401-y |
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author | Mahlaoui, Nizar Warnatz, Klaus Jones, Alison Workman, Sarita Cant, Andrew |
author_facet | Mahlaoui, Nizar Warnatz, Klaus Jones, Alison Workman, Sarita Cant, Andrew |
author_sort | Mahlaoui, Nizar |
collection | PubMed |
description | Primary immunodeficiencies (PIDs) are a widely heterogeneous group of inherited defects of the immune system consisting of many clinical phenotypes with at least 300 underlying genetic deficits currently known. Patients with PIDs can present with, or develop during the course of their life, a susceptibility to recurrent and chronic infection along with autoimmune, allergic, inflammatory, and/or proliferative disorders, all potentially leading to end-organ damage. In recent years, a combination of basic and clinical research has greatly improved understanding of the underlying immunological and genetic defects in PIDs, leading to improved diagnosis, classification, and treatment approaches. In this review, we consider some of the key understandings that should direct diagnostic and treatment approaches in PID and offer insights into current and emerging management approaches and the lifelong care of patients from childhood through to adulthood. |
format | Online Article Text |
id | pubmed-5489581 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2017 |
publisher | Springer US |
record_format | MEDLINE/PubMed |
spelling | pubmed-54895812017-07-03 Advances in the Care of Primary Immunodeficiencies (PIDs): from Birth to Adulthood Mahlaoui, Nizar Warnatz, Klaus Jones, Alison Workman, Sarita Cant, Andrew J Clin Immunol Original Article Primary immunodeficiencies (PIDs) are a widely heterogeneous group of inherited defects of the immune system consisting of many clinical phenotypes with at least 300 underlying genetic deficits currently known. Patients with PIDs can present with, or develop during the course of their life, a susceptibility to recurrent and chronic infection along with autoimmune, allergic, inflammatory, and/or proliferative disorders, all potentially leading to end-organ damage. In recent years, a combination of basic and clinical research has greatly improved understanding of the underlying immunological and genetic defects in PIDs, leading to improved diagnosis, classification, and treatment approaches. In this review, we consider some of the key understandings that should direct diagnostic and treatment approaches in PID and offer insights into current and emerging management approaches and the lifelong care of patients from childhood through to adulthood. Springer US 2017-05-18 2017 /pmc/articles/PMC5489581/ /pubmed/28523402 http://dx.doi.org/10.1007/s10875-017-0401-y Text en © The Author(s) 2017 Open Access This article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. |
spellingShingle | Original Article Mahlaoui, Nizar Warnatz, Klaus Jones, Alison Workman, Sarita Cant, Andrew Advances in the Care of Primary Immunodeficiencies (PIDs): from Birth to Adulthood |
title | Advances in the Care of Primary Immunodeficiencies (PIDs): from Birth to Adulthood |
title_full | Advances in the Care of Primary Immunodeficiencies (PIDs): from Birth to Adulthood |
title_fullStr | Advances in the Care of Primary Immunodeficiencies (PIDs): from Birth to Adulthood |
title_full_unstemmed | Advances in the Care of Primary Immunodeficiencies (PIDs): from Birth to Adulthood |
title_short | Advances in the Care of Primary Immunodeficiencies (PIDs): from Birth to Adulthood |
title_sort | advances in the care of primary immunodeficiencies (pids): from birth to adulthood |
topic | Original Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5489581/ https://www.ncbi.nlm.nih.gov/pubmed/28523402 http://dx.doi.org/10.1007/s10875-017-0401-y |
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