Primary immunodeficiency
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Overview
Primary immunodeficiencies are disorders in which part of the body's immune system is missing or does not function properly. To be considered a primary immunodeficiency, the cause of the immune deficiency must not be secondary in nature (i.e., caused by other disease, drug treatment, or environmental exposure to toxins, e.g.)
Examples
- X-linked agammaglobulinemia (Bruton type agammaglobulinemia)
- Common variable immunodeficiency (also called CVID or Hypogammaglobulinemia)
- Selective immunoglobulin A deficiency
- Severe combined immunodeficiency (SCID)
- IgG Sub-class deficiency
- Chronic granulomatous disease
- Wiskott-Aldrich syndrome
- hyper-IgM syndromes
- DiGeorge syndrome
- Ataxia telangiectasia
- Complement Deficiencies
- Myeloperoxidase deficiency
- Leukocyte adhesion deficiency
- Chédiak-Higashi syndrome
- Vici syndrome
Incidence
} It is believed that around one in every 1200 Americans has a Primary Immunodeficiency disorder. Conditions - there are over 130 recognised primary immunodeficiencies - common variable immunodeficiency, IgA deficiency, XLA, XLP all being worthy of mention in addition to those listed.
Patient support - there are a growing number of national patient organisations around the world to support those who are diagnosed or where one of the conditions are recorded in a family. Access via www.ipopi.org or e-mail info@ipopi.org [or] The Immune Deficiency Foundation [or] Jeffrey Modell Foundation (Links found under "External Links Section")
Treatment
Primary immunodeficiency is generally treated by immunotherapy either through intravenous infusions of immunoglobulin (IVIG) in an effort to induce passive immunity (Immunotherapy is not indicated for those who are IgA deficient). A new subcutaneous treatment (SC-Ig) was recently approved by the FDA, which is especially recommended in cases of severe adverse effects to the IV-Ig treatment or with individuals with poor venous access. Persons with immunodeficiencies are very vulnerable to opportunistic infections, and the use of antimicrobial agents, such as antibiotics and antifungals, is often required. One of the future prospects in the treatment primary immunodeficiency is gene therapy. However, this form of treatment is in its infancy, and the feasibility and long-term complications of gene therapy are not known.
Along with physical treatment, there are hotlines and patient groups; this enables support for family members as well as the actual patient. One can find a diversity of supports by clicking the link to the 'Immune Deficiency Foundation' or 'Jeffrey Modell Foundation under Wikipedia's section External Links on this page.
External links
Immune Deficiency Foundation [3]
Primary Immunodeficiency Resource Center Presented by the Jeffrey Modell Foundation Jeffrey Modell Foundation
International Patient Organisation for Primary Immunodeficiencies (IPOPI) [4]
Population Prevalence of Diagnosed Primary Immunodeficiency Diseases in the United States [5]
Immune system / Immunology | |
|---|---|
| Systems | Adaptive immune system vs. Innate immune system • Humoral immune system vs. Cellular immune system • Complement system (Anaphylatoxins) • Intrinsic immune system |
| Antibodies and antigens | Antibody (Monoclonal antibodies, Polyclonal antibodies, Autoantibody) • Allotype • Isotype • Idiotype • Antigen (Superantigen) |
| Immune cells | White blood cells (T cell, B cell, NK cell, Mast cell, Basophil, Eosinophil) • Phagocyte (Neutrophil, Macrophage, Dendritic cell) • Antigen-presenting cell • Reticuloendothelial system |
| Immunity vs. tolerance | Immunity • Autoimmunity • Allergy • Tolerance (Central) • Immunodeficiency |
| Immunogenetics | Somatic hypermutation • V(D)J recombination • Immunoglobulin class switching • MHC / HLA |
| Substances | Cytokines • Opsonin • Cytolysin |
| Other | Inflammation • Epitope (Hapten) • Cross-reactivity |
WikiDoc Research Resources for Primary immunodeficiency (Click show to right to view) | |
|---|---|
| Articles on Primary immunodeficiency | Most recent articles on Primary immunodeficiency • Most cited articles on Primary immunodeficiency • Review articles on Primary immunodeficiency • Articles on Primary immunodeficiency in N Eng J Med, Lancet, BMJ |
| Media (Slides, Video, Images, MP3) on Primary immunodeficiency | Powerpoint slides on Primary immunodeficiency • Images of Primary immunodeficiency • Photos of Primary immunodeficiency • Podcasts & MP3s on Primary immunodeficiency • Videos on Primary immunodeficiency |
| Evidence Based Medicine Regarding Primary immunodeficiency | Cochrane Collaboration on Primary immunodeficiency • Bandolier on Primary immunodeficiency • TRIP on Primary immunodeficiency |
| Cost Effectiveness of Primary immunodeficiency | Cost Effectiveness of Primary immunodeficiency |
| Clinical Trials Involving Primary immunodeficiency | Ongoing Trials on Primary immunodeficiency at Clinical Trials.gov • Trial results on Primary immunodeficiency • Clinical Trials on Primary immunodeficiency at Google |
| Guidelines / Policies / Government Resources (FDA/CDC) Regarding Primary immunodeficiency | US National Guidelines Clearinghouse on Primary immunodeficiency • NICE Guidance on Primary immunodeficiency • NHS PRODIGY Guidance • FDA on Primary immunodeficiency • CDC on Primary immunodeficiency |
| Textbook Information on Primary immunodeficiency | Books and Textbook Information on Primary immunodeficiency |
| Pharmacology Resources on Primary immunodeficiency | Dosing of Primary immunodeficiency • Drug interactions with Primary immunodeficiency • Side effects of Primary immunodeficiency • Allergic reactions to Primary immunodeficiency • Overdose information on Primary immunodeficiency • Carcinogenicity information on Primary immunodeficiency • Primary immunodeficiency in pregnancy • Pharmacokinetics of Primary immunodeficiency • |
| Genetics, Pharmacogenomics, and Proteinomics of Primary immunodeficiency | Genetics of Primary immunodeficiency • Pharmacogenomics of Primary immunodeficiency • Proteomics of Primary immunodeficiency |
| Newstories on Primary immunodeficiency | Primary immunodeficiency in the news • Be alerted to news on Primary immunodeficiency • News trends on Primary immunodeficiency |
| Commentary on Primary immunodeficiency | Blogs on Primary immunodeficiency |
| Patient Resources on Primary immunodeficiency | Patient resources on Primary immunodeficiency • Discussion groups on Primary immunodeficiency • Patient Handouts on Primary immunodeficiency • Directions to Hospitals Treating Primary immunodeficiency • Risk calculators and risk factors for Primary immunodeficiency |
| Healthcare Provider Resources on Primary immunodeficiency | Symptoms of Primary immunodeficiency • Causes & Risk Factors for Primary immunodeficiency • Diagnostic studies for Primary immunodeficiency • Treatment of Primary immunodeficiency |
| Continuing Medical Education (CME) Programs on Primary immunodeficiency | CME Programs on Primary immunodeficiency |
| International Resources on Primary immunodeficiency | Primary immunodeficiency en Espanol • Primary immunodeficiency en Francais |
| Business Resources on Primary immunodeficiency | Primary immunodeficiency in the Marketplace • Patents on Primary immunodeficiency |
| Informatics Resources on Primary immunodeficiency | List of terms related to Primary immunodeficiency |
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Acknowledgement and Attribution Regarding Sources of Content
Some of the initial content on this page may be incorporated in part from copyleft sources in the public domain including wikis such as Wikipedia and AskDrWiki. Drug information for patients came from the The National Library of Medicine. Infectious disease information may have come from the Centers for Disease Control (CDC). Differential Diagnoses are drawn from clinicians as well as an amalgamation of 3 sources: 1.The Disease Database; 2. Kahan, Scott, Smith, Ellen G. In A Page: Signs and Symptoms. Malden, Massachusetts: Blackwell Publishing, 2004:3; 3. Sailer, Christian, Wasner, Susanne. Differential Diagnosis Pocket. Hermosa Beach, CA: Borm Bruckmeir Publishing LLC, 2002:7 .
