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Primary Immunodeficiency Disorders
Immunology and Allergy Clinics of North America, 2015We are very pleased to be presenting this issue on Primary Immunodeficiency Disorders (PIDD). PIDD is an essential clinical topic for Allergy/Immunology practitioners as well as primary care providers. Given the delay in diagnosis of over ten years in most patients, it is incumbent on us to have a better understanding of these disorders and to get the ...
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Gene Therapy for Primary Immunodeficiencies
Hematology/Oncology Clinics of North America, 2010Gene therapy has effectively entered Medicine via the field of primary immunodeficiencies (PID). Because hematopoietic stem cells are accessible and because it was understood that genetic correction of lymphocyte progenitor cells carrying a genetic defect impairing differentiation, could result in the production of long‐lived T lymphocytes, it was ...
A, Fischer +3 more
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Primary Immunodeficiency Diseases
JAMA: The Journal of the American Medical Association, 1997Primary immunodeficiencies are rare, but important for 3 reasons. First, a high index of suspicion and prompt diagnosis can lead to lifesaving treatment or significant improvement in quality of life. Second, appreciation of the genetic nature of a host defense defect makes possible family counseling and carrier and prenatal diagnosis.
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Primary Immunodeficiency Syndromes
2010Several DNA repair pathways have evolved to recognise and repair DNA damaged by exogenous and endogenous agents, in order to maintain genomic integrity. Defects in these pathways can lead to replication errors, loss or rearrangement ofgenomic material, mutation or cancer and eventual death.
Mary A, Slatter, Andrew R, Gennery
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Primary Immunodeficiency in the NICU
NeoReviews, 2019Primary immunodeficiency disorders (PIDs) are genetic diseases that lead to increased susceptibility to infection. Hundreds of PIDs have now been described, but a select subset commonly presents in the neonatal period. Neonates, especially premature newborns, have relative immune immaturity that makes it challenging to differentiate PIDs from intrinsic
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Primary cellular immunodeficiencies
Journal of Allergy and Clinical Immunology, 2002Genetic defects in T-cell function lead to susceptibility to infections or to other clinical problems that are more grave than those seen in disorders resulting in antibody deficiency alone. Those affected usually present during infancy with either common or opportunistic infections and rarely survive beyond infancy or childhood. The spectrum of T-cell
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PRIMARY IMMUNODEFICIENCY DISORDERS
Primary Care: Clinics in Office Practice, 1998The primary immunodeficiency diseases are a relatively rare group of congenital disorders that are linked by the expression of an excessive number, duration, or severity of infections. The clinical features of most of the primary immunodeficiency diseases have been well described by astute physicians over several decades and have provided important ...
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Immunodeficiency and Multiple Primary Malignancies
2009The relationship between cancer onset and the immune response became a subject of great interest in the early 1900s, and led Ehrlich [1] to establish what came to be known as the theory of immunological surveillance. This theory is based on three principles: (1) cancer cells are antigenic, (2) these cells can be destroyed by the immune response of the ...
SANTANGELO, MICHELE +13 more
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Gene therapy for primary immunodeficiencies: up-to-date
Expert Opinion on Biological Therapy, 2021Claire Booth, Kritika Chetty
exaly