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Approach to primary immunodeficiency
Allergy and Asthma Proceedings, 2019Primary immunodeficiency diseases are inherited defects of the innate or adaptive arms of the immune system that lead to an increase in the incidence, frequency, or severity of infections and/or immune dysregulation. There may be defects in the adaptive arm of the immune system, including combined immunodeficiencies and antibody deficiency syndromes ...
Ashley L Devonshire, Melanie M. Makhija
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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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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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Gene Therapy for Primary Immunodeficiencies [PDF]
Hematology/Oncology Clinics of North America, 2010 Gene 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 ...
Alain Fischer +3 more
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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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Primary immunodeficiency registries
Current Opinion in Allergy & Clinical Immunology, 2007Research in the field of rare diseases such as primary immunodeficiencies can be significantly improved with sufficient patient numbers. Patient registries can help provide the basis for this by collecting data over a longer period of time and by connecting centres nationally or even internationally. The present article reviews recent publications both
Knerr, Viviane, Grimbacher, Bodo
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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 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 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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