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{{SI}}
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{{Primary immunodeficiency}}
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{{SK}} PID, congenital immunodeficiency syndromes, primary immunodeficiency syndromes, hereditary immunodeficiency syndromes, congenital immunodeficiency disorders, primary immunodeficiency disorders, hereditary immunodeficiency disorders, congenital immunodeficiency diseases, primary immunodeficiency diseases, ereditary immunodeficiency diseases


==Overview==
==[[Primary immunodeficiency overview|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.)''' 
==[[Primary immunodeficiency historical perspective|Historical Perspective]]==


== Examples ==
==[[Primary immunodeficiency classification|Classification]]==
*[[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 syndrome|hyper-IgM syndromes]]
*[[DiGeorge syndrome]]
*[[Ataxia telangiectasia]]
*Complement Deficiencies
*[[Myeloperoxidase deficiency]]
*[[Leukocyte adhesion deficiency]]
*[[Chédiak-Higashi syndrome]]
*[[Vici syndrome]]


==Incidence==
==[[Primary immunodeficiency pathophysiology|Pathophysiology]]==


It is believed that around one in every 1200 Americans has a Primary Immunodeficiency disorder.
==[[Primary immunodeficiency causes|Causes]]==
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")
==[[Primary immunodeficiency differential diagnosis|Differentiating Primary immunodeficiency from other Diseases]]==


==Treatment==
==[[Primary immunodeficiency epidemiology and demographics|Epidemiology and Demographics]]==
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 infection]]s, and the use of [[antimicrobial]] agents, such as [[antibiotic]]s and [[antifungal]]s, 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.
 
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==[[Primary immunodeficiency risk factors|Risk Factors]]==
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==[[Primary immunodeficiency screening|Screening]]==
 
==[[Primary immunodeficiency natural history, complications and prognosis|Natural History, Complications and Prognosis]]==
 
==Diagnosis==


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.
[[Primary immunodeficiency history and symptoms|History and Symptoms]] | [[Primary immunodeficiency physical examination|Physical Examination]] | [[Primary immunodeficiency laboratory findings|Laboratory Findings]] | [[Primary immunodeficiency chest x ray|Chest X ray]] | [[Primary immunodeficiency other imaging findings|Other Imaging Findings]] | [[Primary immunodeficiency other diagnostic studies|Other Diagnostic Studies]]


==External links==
==Treatment==
Immune Deficiency Foundation [http://www.primaryimmune.org]


Primary Immunodeficiency Resource Center Presented by the Jeffrey Modell Foundation [http://www.info4pi.org/index.cfm?CFID=11982666&CFTOKEN=43062451.com Jeffrey Modell Foundation]
[[Primary immunodeficiency medical therapy|Medical Therapy]] | [[Primary immunodeficiency primary prevention|Primary Prevention]] | [[Primary immunodeficiency secondary prevention|Secondary Prevention]] | [[Primary immunodeficiency cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Primary immunodeficiency future or investigational therapies|Future or Investigational Therapies]]


[[Category:Immune system disorders]]
==Case Studies==
[[Primary immunodeficiency case study one|Case #1]]


International Patient Organisation for Primary Immunodeficiencies (IPOPI) [http://www.ipopi.org]
==Related Chpaters==
*[[Immunodeficiency]]


Population Prevalence of Diagnosed Primary Immunodeficiency Diseases in the United States [http://www.springerlink.com/content/b5r702t2820mk6l8/fulltext.pdf]


{{Immune_system}}
{{Immune_system}}

Latest revision as of 23:17, 15 February 2015

Primary immunodeficiency Microchapters

Home

Patient Information

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Primary immunodeficiency from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

History and Symptoms

Physical Examination

Laboratory Findings

Chest X Ray

Other Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Primary Prevention

Secondary Prevention

Cost-Effectiveness of Therapy

Future or Investigational Therapies

Case Studies

Case #1

Primary immunodeficiency On the Web

Most recent articles

Most cited articles

Review articles

CME Programs

Powerpoint slides

Images

American Roentgen Ray Society Images of Primary immunodeficiency

All Images
X-rays
Echo & Ultrasound
CT Images
MRI

Ongoing Trials at Clinical Trials.gov

US National Guidelines Clearinghouse

NICE Guidance

FDA on Primary immunodeficiency

CDC on Primary immunodeficiency

Primary immunodeficiency in the news

Blogs on Primary immunodeficiency

Directions to Hospitals Treating Primary immunodeficiency

Risk calculators and risk factors for Primary immunodeficiency

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Synonyms and keywords: PID, congenital immunodeficiency syndromes, primary immunodeficiency syndromes, hereditary immunodeficiency syndromes, congenital immunodeficiency disorders, primary immunodeficiency disorders, hereditary immunodeficiency disorders, congenital immunodeficiency diseases, primary immunodeficiency diseases, ereditary immunodeficiency diseases

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Primary immunodeficiency from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

History and Symptoms | Physical Examination | Laboratory Findings | Chest X ray | Other Imaging Findings | Other Diagnostic Studies

Treatment

Medical Therapy | Primary Prevention | Secondary Prevention | Cost-Effectiveness of Therapy | Future or Investigational Therapies

Case Studies

Case #1

Related Chpaters


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