Abstract
Combined immune deficiencies are a heterogeneous group of congenital disorders usually attributable to a single gene defect. The hallmark of these disorders is recurrent infections resulting from defects in both T- and B-cell function. This article reviews the basic components of the immune system as well as the clinical presentation, laboratory findings, and treatment for 3 distinct combined immune deficiencies.
MeSH terms
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Algorithms
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Anti-Bacterial Agents / therapeutic use
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Antibody Formation / immunology
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B-Lymphocytes / immunology
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Bone Marrow Transplantation
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Child
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Decision Trees
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DiGeorge Syndrome* / diagnosis
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DiGeorge Syndrome* / etiology
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DiGeorge Syndrome* / therapy
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Fluid Therapy
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Genetic Counseling
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Humans
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Immunoglobulin G / chemistry
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Immunoglobulin G / immunology
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Immunoglobulins, Intravenous / therapeutic use
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Infections / drug therapy
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Infections / etiology
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Inheritance Patterns / genetics
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Inheritance Patterns / immunology
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Prognosis
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Severe Combined Immunodeficiency* / diagnosis
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Severe Combined Immunodeficiency* / etiology
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Severe Combined Immunodeficiency* / therapy
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Stem Cell Transplantation
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T-Lymphocytes / immunology
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Wiskott-Aldrich Syndrome* / diagnosis
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Wiskott-Aldrich Syndrome* / etiology
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Wiskott-Aldrich Syndrome* / therapy
Substances
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Anti-Bacterial Agents
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Immunoglobulin G
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Immunoglobulins, Intravenous