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

Journal of Clinical Immunology
Nizar MahlaouiAndrew Cant

Abstract

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.

References

Nov 1, 1993·The Journal of Adolescent Health : Official Publication of the Society for Adolescent Medicine·R W BlumG B Slap
Apr 20, 2002·Current Opinion in Infectious Diseases·Desa Lilic
Jun 26, 2002·Annals of the New York Academy of Sciences·Polly Matzinger
Feb 29, 2008·The New England Journal of Medicine·Anisur Rahman, David A Isenberg
Apr 19, 2008·Clinical Infectious Diseases : an Official Publication of the Infectious Diseases Society of America·Eric OksenhendlerUNKNOWN DEFI Study Group
Oct 8, 2009·Clinical Infectious Diseases : an Official Publication of the Infectious Diseases Society of America·Marion MalphettesUNKNOWN DEFI Study Group
Mar 25, 2010·Blood·Charlotte Cunningham-Rundles
May 4, 2010·Journal of Clinical Immunology·Gaël MouillotUNKNOWN DEFI Study Group
Aug 3, 2010·The Journal of Allergy and Clinical Immunology·Andrew R GenneryUNKNOWN European Society for Immunodeficiency
Aug 3, 2010·Clinical Immunology : the Official Journal of the Clinical Immunology Society·Jordan S OrangeMahlon M Wilkes
Jul 1, 2011·The New England Journal of Medicine·Frank L van de VeerdonkMihai G Netea
Jul 6, 2011·The Journal of Experimental Medicine·Luyan LiuJean-Laurent Casanova
Dec 20, 2011·Blood·Elena S ResnickCharlotte Cunningham-Rundles
May 23, 2012·American Journal of Human Genetics·Gabriela Lopez-HerreraBodo Grimbacher
Jul 24, 2012·The Journal of Allergy and Clinical Immunology·Helen ChapelEric Oksenhendler
Oct 13, 2012·Frontiers in Immunology·Thomas Pradeu, Edwin L Cooper
Feb 5, 2013·The Journal of Allergy and Clinical Immunology·Nima ParvanehMary Ellen Conley
Sep 27, 2013·PloS One·Joon ParkCharlotte Cunningham-Rundles
Oct 29, 2013·Lancet·Tayfun GüngörUNKNOWN Inborn Errors Working Party of the European Society for Blood and Marrow Transplantation
Mar 4, 2014·The Journal of Allergy and Clinical Immunology·Benjamin GathmannUNKNOWN European Society for Immunodeficiencies Registry Working Party
Apr 3, 2014·Journal of Clinical Immunology·H B GasparS Borte
Jul 21, 2014·Nature Genetics·Sarah E FlanaganAndrew T Hattersley
Aug 21, 2014·JAMA : the Journal of the American Medical Association·Antonia KwanVincent R Bonagura
Aug 27, 2014·European Journal of Immunology·Capucine Picard, Alain Fischer
Sep 17, 2014·The Journal of Experimental Medicine·Matthieu PerreauGiuseppe Pantaleo
Oct 14, 2014·Frontiers in Immunology·Rohan AmeratungaSee-Tarn Woon
Oct 21, 2014·Nature Medicine·Desirée SchubertBodo Grimbacher
Oct 24, 2014·Journal of Clinical Immunology·Sujal GhoshArndt Borkhardt
Jan 18, 2015·The Journal of Allergy and Clinical Immunology·Claudia WehrUNKNOWN Inborn Errors Working Party of the European Society for Blood and Marrow Transplantation and the European Society for Immuno
Feb 25, 2015·Clinical Genetics·A FischerM Cavazzana
Apr 11, 2015·Human Gene Therapy·Maria Pia Cicalese, Alessandro Aiuti
Oct 16, 2015·Nature·Luigi Naldini
Oct 21, 2015·Current Opinion in Allergy and Clinical Immunology·Annick A J M van de Ven, Klaus Warnatz
Nov 14, 2015·The Journal of Allergy and Clinical Immunology. in Practice·Francisco A BonillaKlaus Warnatz
Jan 16, 2016·The Journal of Allergy and Clinical Immunology·Laura Gámez-DíazBodo Grimbacher

❮ Previous
Next ❯

Citations

Jun 25, 2019·Clinical Genetics·Juan A BuerenGuillermo Guenechea
Nov 14, 2018·Orphanet Journal of Rare Diseases·Irina OdnoletkovaUNKNOWN Plasma Protein Therapeutics Association (PPTA) Taskforce
Aug 1, 2019·Genetics in Medicine : Official Journal of the American College of Medical Genetics·Henri MargotDavid Geneviève
Apr 28, 2021·Archives de pédiatrie : organe officiel de la Sociéte française de pédiatrie·C FlatrèsE Launay
May 11, 2021·Frontiers in Immunology·Isabella QuintiAntonio Condino-Neto
Dec 24, 2021·European Journal of Pediatrics·Aleksandra Szczawinska-PoplonykMonika Wiczuk-Wiczewska

❮ Previous
Next ❯

Related Concepts

Related Feeds

Autoimmune Diseases

Autoimmune diseases occur as a result of an attack by the immune system on the body’s own tissues resulting in damage and dysfunction. There are different types of autoimmune diseases, in which there is a complex and unknown interaction between genetics and the environment. Discover the latest research on autoimmune diseases here.

Allergy and Asthma

Allergy and asthma are inflammatory disorders that are triggered by the activation of an allergen-specific regulatory t cell. These t cells become activated when allergens are recognized by allergen-presenting cells. Here is the latest research on allergy and asthma.

Related Papers

Current Opinion in Allergy and Clinical Immunology
Marina CavazzanaAlain Fischer
Adolescent Medicine: State of the Art Reviews
Lisa J Kobrynski
The Journal of Allergy and Clinical Immunology
Isaac J NijmanMarielle E van Gijn
© 2022 Meta ULC. All rights reserved