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Primary Immunodeficiency Diseases
1976Rare individuals, experiments of nature who suffer the often devastating consequences of a congenital defect within the immune system, have served to teach us much of what we know today about the complex immune response of man (36,39,54). From a careful study of them and their diseases, together with related studies in the laboratory, we have learned ...
Robert A. Good, M. A. Hansen
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Malakoplakia and Primary Immunodeficiency
The Journal of Pediatrics, 2014Malakoplakia, a rare granulomatous disease caused by impaired macrophage response, has been reported only rarely in children. We report 3 unique cases, with lesions occurring in unusual locations in children with primary immune deficiencies.
Sydney R. Archer +8 more
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2013
In general, immunodeficiencies are divided into those of the specific immune system (e.g. T cells or B cells or combined) or those of the innate or non-specific immune system (e.g. complement and neutrophils). Immunodeficiencies may also be divided into primary (usually genetic) and secondary, where the immune defect is caused by some other non ...
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In general, immunodeficiencies are divided into those of the specific immune system (e.g. T cells or B cells or combined) or those of the innate or non-specific immune system (e.g. complement and neutrophils). Immunodeficiencies may also be divided into primary (usually genetic) and secondary, where the immune defect is caused by some other non ...
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Progress in primary immunodeficiency
Immunology Today, 1992Clinically, primary immunodeficiencies can be grouped into several well-defined syndromes. This consistent clinical picture, however, belies the enormous complexity of lymphocyte maturation and activation, and it has long been suspected that numerous distinct underlying defects give rise to primary immunodeficiencies.
Richard B. Gallagher +4 more
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Primary T-cell immunodeficiencies
Current Opinion in Immunology, 1993The phenotypes of many primary T-cell immunodeficiencies have been described, including diseases associated with defective T-cell differentiation and/or activation. Recently, genotypes have been defined for some of them, such as X-linked severe combined immunodeficiency and CD3 deficiencies (or hyper IgM syndrome).
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PRIMARY IMMUNODEFICIENCY DISORDERS [PDF]
Primary Care: Clinics in Office Practice, 1998 The 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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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 Slatter, Andrew R. Gennery
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Primary Immunodeficiencies of Horses
Veterinary Clinics of North America: Equine Practice, 2000Primary immunodeficiency disorders are genetically determined failures of immune defense that increase susceptibility to infectious agents. This article reviews the salient features of equine primary immunodeficiency disorders, summarizes the molecular mechanisms of each disorder, and updates information that facilitates diagnosis and management of ...
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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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Immunodeficiency and Multiple Primary Malignancies [PDF]
, 2009 The 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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