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Copyright 1986, 1987, 1989, 1991, 1992, 1993, 1996, 1997, 1999, 2002, 2005, 2007, 2008
Synonyms of Angioedema, Hereditary
- Angioneurotic Edema, Hereditary
- C1-INH
- C1NH
- Complement Component 1 Inhibitor Deficiciency
- Complement Component C1, Regulatory Component Deficiency
- Esterase Inhibitor Deficiency
- HAE
- HANE
Disorder Subdivisions
- C1 Esterase Inhibitor Deficiency, Type I, Angioedema
- C1 Esterase Inhibitor Dysfunction, Type II, Angioedema
General Discussion
Hereditary angioedema is a rare inherited disorder characterized by recurrent episodes of the accumulation of fluids outside of the blood vessels, blocking the normal flow of blood or lymphatic fluid and causing rapid swelling of tissues in the hands, feet, limbs, face, intestinal tract, or airway. Usually, this swelling is not accompanied by itching, as it might be with an allergic reaction. Swelling of the gastrointestinal tract leads to cramping. Swelling of the airway may lead to obstruction, a potentially very serious complication. These symptoms develop as the result of deficiency or improper functioning of certain proteins that help to maintain the normal flow of fluids through very small blood vessels (capillaries). In some cases, fluid may accumulate in other internal organs. The severity of the disease varies greatly among affected individuals.
The most common form of the disorder is hereditary angioedema type I, which is the result of abnormally low levels of certain complex proteins in the blood (C1 esterase inhibitors), known as complements. They help to regulate various body functions (e.g., flow of body fluids in and out of cells). Hereditary angioedema type II, a more uncommon form of the disorder, occurs as the result of the production of abnormal complement proteins.
Organizations related to Angioedema, Hereditary
- American Academy of Allergy, Asthma and Immunology
555 East Wells Street, Suite 1100
Milwaukee WI 53202-3823
Phone #: 414-272-6071
800 #: 800-822-2762
e-mail: info@aaaai.org
Home page: http://www.aaaai.org
- European Society for Immunodeficiencies (ESID)
c/o Dr. Bodo Grimbacher (ESID Board Member)
Division of Rheumatology and Clinical Immunology
Department of Medicine, University Hospital Freiburg
Hugstetter Strasse 55
Freiburg None D-79106
Phone #: +31- 73--6992965
800 #: N/A
e-mail: info@esid.org
Home page: http://www.esid.org
- Genetic and Rare Diseases (GARD) Information Center
PO Box 8126
Gaithersburg MD 20898-8126
Phone #: 301-251-4925
800 #: 888-205-2311
e-mail: ordr@od.nih.gov
Home page: http://rarediseases.info.nih.gov/Default.aspx
- Immune Deficiency Foundation
40 West Chesapeake Avenue
Suite 308
Towson MD 21204
Phone #: 410-321-6647
800 #: 800-296-4433
e-mail: idf@primaryimmune.org
Home page: http://www.primaryimmune.org
- Madisons Foundation
PO Box 241956
Los Angeles CA 90024
Phone #: 310-264-0826
800 #: N/A
e-mail: getinfo@madisonsfoundation.org
Home page: http://www.madisonsfoundation.org
- NIH/National Institute of Allergy and Infectious Diseases
6610 Rockledge Drive
MSC 6612
Bethesda MD 20892-6612
Phone #: 301-496-5717
800 #: --
e-mail: N/A
Home page: http://www.niaid.nih.gov/
- US Hereditary Angioedema Association, Inc.
Seven Waterfront Plaza
500 Ala Moana Blvd., Suite 400
Honolulu HI 96813
Phone #: 774-206-5555
800 #: 866-798-5598
e-mail: generalinfo@hereditaryangioedema.com
Home page: http://www.haea.org
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