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12. Primary immunodeficiency diseases
Journal of Allergy and Clinical Immunology, 2003Although primary immunodeficiency disorders are relatively rare, intensive investigation of these disorders has yielded a great wealth of understanding of basic immunologic mechanisms in host defense, inflammation, and autoimmunity. These advances have led to important developments for the treatment not only of the primary immunodeficiencies but also ...
Francisco A, Bonilla, Raif S, Geha
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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 ...
R A, Good, M A, Hansen
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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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Neutropenia and Primary Immunodeficiency Diseases
International Reviews of Immunology, 2009Primary immunodeficiency diseases (PID) are a heterogeneous group of congenital disorders of the immune system leading to recurrent infections, autoimmunity, malignancies, and hematological disorders. This review focuses specifically on inherited disorders associated with neutropenia, which may occur in isolation or as a feature of more complex immune ...
Nima, Rezaei +3 more
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Primary immunodeficiency diseases: An update
Journal of Allergy and Clinical Immunology, 2004Although relatively rare, primary immune deficiency diseases (PIDs) provide an excellent window into the functioning of the immune system. In the late 1960s, observations on these diseases, with their associated infections and genetics, bisected the immune system into humoral immunity and cell-mediated immunity.
Luigi, Notarangelo +6 more
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Primary immunodeficiency diseases in adulthood
Internal Medicine Journal, 2004Abstract Occasionally, patients present with clinical episodes of infectious disease that seem to fall beyond the scope of normal immunocompetence. Examples include infections that are unusually persistent, recurrent or resistant to treatment, or those involving unexpected dissemination of disease or atypical pathogens.
D S, Riminton, S, Limaye
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Mycobacterial diseases in primary immunodeficiencies
Current Opinion in Allergy and Clinical Immunology, 2001Primary immunodeficiency diseases comprise over 100 conditions, each associated with a variety of viral, bacterial, fungal and protozoan infections. M. tuberculosis and less virulent mycobacteria, such as bacille Calmette-Guérin vaccines and environmental non-tuberculous mycobacteria, may cause severe disease in patients with primary immunodeficiency ...
J, Reichenbach +5 more
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Primary Immunodeficiency Diseases
2019Primary immunodeficiency diseases (PIDs) are a heterogeneous group of monogenic disorders in which part of the immune system is deficient or does not function properly. Patients with PID commonly present with recurrent infections, which explains the importance of the immune system in protection from organisms.
Fayhan Alroqi +2 more
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Primary Immunodeficiency Diseases
2006Abstract The second edition of Primary Immunodeficiency Diseases presents discussions of gene identification, mutation detection, and clinical and research applications for over 100 genetic immune disorders - disorders featuring an increased susceptibility to infections and, in, certain conditions, an increased rate of malignancies and ...
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