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Advances in the Care of Primary Immunodeficiencies (PIDs): from Birth to Adulthood

Lookup NU author(s): Professor Andrew Cant

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Abstract

© 2017 The Author(s) 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.

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Author(s): Mahlaoui N, Warnatz K, Jones A, Workman S, Cant A

Publication type: Article

Publication status: Published

Journal: Journal of Clinical Immunology

Year: 2017

Volume: 37

Issue: 5

Pages: 452-460

Print publication date: 01/07/2017

Online publication date: 18/05/2017

Acceptance date: 08/05/2017

Date deposited: 19/06/2017

ISSN (print): 0271-9142

ISSN (electronic): 1573-2592

Publisher: Springer New York LLC

URL: https://doi.org/10.1007/s10875-017-0401-y

DOI: 10.1007/s10875-017-0401-y

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