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Severe Combined Immunodeficiency

National Organization for Rare Disorders, Inc.

Important

It is possible that the main title of the report Severe Combined Immunodeficiency is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.

Synonyms

  • SCID
  • Bubble Boy Syndrome

Disorder Subdivisions

  • Autosomal Recessive Severe Combined Immunodeficiency
  • ADA Deficiency
  • X-Linked Recessive Severe Combined Immunodeficiency with Leukopenia
  • Bare Lymphocyte Syndrome
  • Reticular Dysgenesis
  • Adenosine Deaminase Deficiency

General Discussion

Severe Combined Immunodeficiency (SCID) is a group of rare congenital syndromes characterized by little if any immune responses. This results in frequent recurring infections. Cellular immune responses involve specialized white blood cells known as T lymphocytes or "killer cells". These cells assist other white blood cells (B lymphocytes) to respond to infectious, foreign agents that invade the body (i.e., bacteria or viruses). The B lymphocytes maintain immunity by enabling the body to produce and preserve circulating antibodies.



People with Severe Combined Immunodeficiency are unusually susceptible to recurrent infections with bacteria, viruses, fungi, and other infectious agents that can be life-threatening.



There are several types of Severe Combined Immunodeficiencies. These include: Autosomal Recessive Severe Combined Immunodeficiency, X-Linked Recessive Severe Combined Immunodeficiency, Adenosine Deaminase Deficiency (ADA), Bare Lymphocyte Syndrome, Severe Combined Immunodeficiency with Leukopenia (Reticular Dysgenesis), and Swiss-type Agammaglobulinemia. Each type of Severe Combined Immune Deficiency is caused by a different genetic defect, but the primary symptom is reduced or absent immune functions, and all types are hereditary.

Resources

March of Dimes Birth Defects Foundation

1275 Mamaroneck Avenue

White Plains, NY 10605

Tel: (914)997-4488

Fax: (914)997-4763

Tel: (888)663-4637

Email: Askus@marchofdimes.com

Internet: http://www.marchofdimes.com



Immune Deficiency Foundation

40 W. Chesapeake Avenue

Suite 308

Towson, MD 21204

Tel: (410)321-6647

Fax: (410)321-9165

Tel: (800)296-4433

Email: idf@primaryimmune.org

Internet: http://www.primaryimmune.org



NIH/National Institute of Allergy and Infectious Diseases

Office of Communications and Government Relations

6610 Rockledge Drive, MSC 6612

Bethesda, MD 20892-6612

Tel: (301)496-5717

Fax: (301)402-3573

Tel: (866)284-4107

TDD: (800)877-8339

Email: ocpostoffice@niaid.nih.gov

Internet: http://www.niaid.nih.gov/



American Academy of Allergy, Asthma and Immunology

611 East Wells Street

Milwaukee, WI 53202

Tel: (414)272-6071

Fax: (414)276-3349

Tel: (800)822-2762

Email: info@aaaai.org

Internet: http://www.aaaai.org



International Patient Organization for Primary Immunodeficiencies

Firside Main Road

Downderry

Cornwall, PL11 3LE

United Kingdom

Tel: 441503250668

Fax: 441503250961

Email: info@ipopi.org

Internet: http://www.ipopi.org/



Genetic and Rare Diseases (GARD) Information Center

PO Box 8126

Gaithersburg, MD 20898-8126

Tel: (301)251-4925

Fax: (301)251-4911

Tel: (888)205-2311

TDD: (888)205-3223

Internet: http://rarediseases.info.nih.gov/GARD/



Madisons Foundation

PO Box 241956

Los Angeles, CA 90024

Tel: (310)264-0826

Fax: (310)264-4766

Email: getinfo@madisonsfoundation.org

Internet: http://www.madisonsfoundation.org



European Society for Immunodeficiencies

1-3 rue de Chantepoulet

Geneva, CH 1211

Switzerland

Tel: 410229080484

Fax: 41229069140

Email: esid@kenes.com

Internet: http://www.esid.org



For a Complete Report

This is an abstract of a report from the National Organization for Rare Disorders (NORD). A copy of the complete report can be downloaded free from the NORD website for registered users. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational therapies (if available), and references from medical literature. For a full-text version of this topic, go to MyD-H, the Dartmouth-Hitchcock patient portal. You must be a registered MyD-H user for the Lebanon, Manchester, or Nashua locations to access this site.

The information provided in this report is not intended for diagnostic purposes. It is provided for informational purposes only. NORD recommends that affected individuals seek the advice or counsel of their own personal physicians.

It is possible that the title of this topic is not the name you selected. Please check the Synonyms listing to find the alternate name(s) and Disorder Subdivision(s) covered by this report

This disease entry is based upon medical information available through the date at the end of the topic. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder.

For additional information and assistance about rare disorders, please contact the National Organization for Rare Disorders at P.O. Box 1968, Danbury, CT 06813-1968; phone (203) 744-0100; web site www.rarediseases.org or email orphan@rarediseases.org

Last Updated:  7/19/2010

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