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ACE Inhibitor
Class of medicines used to treat high blood pressure and other diseases.
Activating factor
A functioning plasma protein that works in the coagulation pathway to help form a clot.
Anabolic steroid
Also referred to as attenuated androgen, a class of steroid hormones used to prevent HAE attacks. Although their mechanism of action in HAE patients is unknown, these agents are believed to partially reverse the biochemical defect by increasing levels of C1-esterase inhibitor (C1-INH), helping to prevent angioedema attacks in patients with inherited C1-INH deficiency.
Antifibrolytic agent
Medicines used to prevent HAE attacks. These agents inhibit the activation of plasmin, a chemical in the blood that can make the C1-INH less effective.
Attenuated androgen
Also referred to as anabolic steroid, a class of steroid hormones used to prevent HAE attacks. Although their mechanism of action in HAE patients is unknown, these agents are believed to partially reverse the biochemical defect by increasing levels of C1-esterase inhibitor (C1-INH), helping to prevent angioedema attacks in patients with inherited C1-INH deficiency.
Bradykinin
A protein, which when released, causes blood vessels to expand (dilate) and leak fluid, such as serum, into surrounding tissue.
C1 inhibitor
Also referred to as C1-INH, a protein found in the fluid part of your blood that controls C1, a component of your body's complement system. Abnormal swelling can result when C1-INH is insufficient or defective.
C1-INH
Also referred to as C1 inhibitor, a protein found in the fluid part of your blood that controls C1, a component of your body's complement system. Abnormal swelling can result when C1-INH is insufficient or defective.
Cleave
To cut; to split
Coagulation pathway
A process in which blood cells work together to form a clot.
Complement system
The complement system is made up of proteins like C1 that float in your bloodstream, which work with the immune system to fight organisms like bacteria, viruses and parasites.
Drug formulary
A list of prescription medications selected for coverage under a health insurance plan.
Edema
Swelling caused by an excessive amount of fluid in the body tissues.
Endothelial cells
A thin layer of cells that line the surface of blood vessels.
Enzyme
A protein that changes the rate of chemical reactions in the body.
HAE
HAE, or Hereditary Angioedema, is a rare, hereditary disease of the immune system that affects an estimated 1 in 10,000 to 1 in 50,000 adults and children globally. HAE causes attacks of spontaneous swelling that are often painful and severe, and can be life-threatening.
Hereditary Angioedema
A rare, hereditary disease of the immune system that affects an estimated 1 in 10,000 to 1 in 50,000 adults and children globally. Hereditary Angioedema (HAE) causes attacks of spontaneous swelling that are often painful and severe, and can be life-threatening.
High-molecular-weight kininogen
HMWK, a protein that acts on the inflammation and coagulation processes; inactive until it is cleaved by kallikrein and in turn, releases bradykinin.
Hypotension
Low blood pressure.
Inflammatory pathway
A process in which the body responds to an injury either inside or outside of the body.
Inhibitor
That which reduces, prevents or stops; i.e. a substance that stops enzyme activity.
Kallikrein–kinin system
A system of proteins in the blood that has a role in the inflammatory pathway; through this system, bradykinin is released.
Kinin
A protein that is a component of the kallikrein-kinin system.
Laryngeal attack
An HAE attack that affects the larynx (throat); this can block or close the airway, leading to difficulty breathing and, potentially, death.
Liberate
To release
Plasma kallikrein
An enzyme present in blood plasma, urine and tissue; responsible for cleaving high-molecular-weight kininogen (HMWK), which then releases bradykinin.
Prekallikrein
Precursor to kallikrein; inactive until it cleaved by activated factor (specifically, activated factor XII) to generate active kallikrein.
Preventive therapy
In HAE, medicine taken to prevent attacks.
Prodrome
One or more physical warnings of an HAE attack; can appear as a rash or a feeling of skin tightness.
Protease
An enzyme that breaks proteins down into a simpler form.
Proteolytic cascade
A step-by-step process in which proteins are broken down into simpler forms.
Receptor
A component in a cell that can combine with a drug or a chemical in the body (i.e. hormone) to change the function of the cell.
Serum
The clear, watery, fluid-component of blood that resembles fluid in a blister.
Subcutaneous
Beneath the skin.
Substrate
A molecule or substance acted upon by an enzyme.
Trigger
In HAE, something that causes an attack. Stress and trauma are two common HAE attack triggers.
Vascular permeability
The capacity of blood vessels to allow fluids or molecules to pass in and out of the vessel.
Vasodilator
A substance that opens, or dilates, blood vessels; e.g. bradykinin and nitric oxide.
Header: Living with HAE
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Resources

As HAE becomes more widely understood, the resources available to patients will continue to increase. Here are some sites to visit (Note: by clicking any of the links below, you will leave HAE Hope):

HAEA.org: This nonprofit organization offers information about hereditary angioedema and forums where you can connect with other patients.

National Organization for Rare Diseases (NORD): This organization focuses on the issues patients face when they're living with a rare disease, including hereditary angioedema.

Food and Drug Administration (FDA): Find information on clinical trials and new drug approvals.

Development & Approval Process (Drugs): Learn about the drug approval process.

ClinicalTrials.gov: You’ll find details on which treatments are currently in clinical trials.

Sources used to write this site include:   

Agostoni A, Cicardi M. Hereditary and acquired C1-inhibitor deficiency: Biological and clinical characteristics in 235 patients. Medicine 1992; 71:206-15.

Bork K, Barnstedt S, Koch P, Traupe H. Hereditary angioedema with normal C1-inhibitor activity in women. Lancet 2000; 356:213-7.

Bork K, Fischer B, Dewald G. Recurrent Episodes of Skin Angioedema and Severe Attacks of Abdominal Pain Induced by Oral Contraceptives or Hormone Replacement Therapy. Am J Med 2003; 114:294-8.

Bork K, Hardt J, Schicketanz K-H, Ressel N. Clinical Studies of Sudden Upper Airway Obstruction in Patients With Hereditary Angioedema Due to C-1 Esterase Inhibitor Deficiency. Arch Intern Med/163. 2003; 163:1229-1235

Bork K, Meng G, Staubach P, Hardt J. Hereditary angioedema: New findings concerning symptoms, affected organs, and course. American Journal of Medicine 2006; 119:267-74.

Bork K, Siedlecki K, Bosch S, Schopf RE, Kreuz W. Asphyxiation by laryngeal edema in patients with hereditary angioedema. Mayo Clin Proc 2000; 75:349-54.

Bouillet L, Longhurst, H, Boccon-Gibod I, et al. Disease expression in women with hereditary angioedema. Am J Obstet Gynecol. 2008;199(Issue 5), 484.e1-484.e4

Bowen T, Cicardi M, Bork K, Zuraw B, Frank M, Ritchie B, et al. Hereditary angioedema: a current state-of-the-art review, VII: Canadian Hungarian 2007 International Consensus Algorithm for the Diagnosis, Therapy, and Management of Hereditary Angioedema. Ann Allergy Asthma Immunol 2008; 100:S30-40.

Frank MM. Urticaria and Angioedema. In: Goldman:Cecil Textbook of Medicine. 22 ed. Philadelphia: W.B Saunders Co.; 2004. p. 1611-4.

Frank MM, Gelfand JA, Atkinson JP. Hereditary angioedema: the clinical syndrome and its management. Annals of Internal Medicine 1976; 84:580-93.

Gompels MM, Lock RJ, Abinun M, Bethune CA, Davies G, Grattan C, et al. C1 inhibitor deficiency: consensus document. Clinical and Experimental Immunology 2005; 139:379-94.

Goring HD, Bork K, Spath PJ, Bauer R, Ziemer A, Hintner H, et al. [Hereditary angioedema in the German-speaking region]. Hautarzt. 1998 Feb;49(2):114-22.

HAE frequently asked questions. United States Hereditary Angioedema Association Web site. Available at: http://www.haea.org/. Accessed Jan 7, 2009.

Heymann W, Rossy K. Angioedema, Hereditary. Emedicine Web site. 2008. Available at: http://emedicine.medscape.com/article/1048994-overview. Accessed Jan 7, 2009.

Hereditary Angioedema. AARP website. Retrieved September 18, 2009 from: http://symptomchecker.aarp.org/adamcontent/hereditary-angioedema/2

Information for consumers: approved drugs. Food and Drug Administration Web site. Available at: http://www.accessdata.fda.gov/scripts/cder/drugsatfda/index.cfm. Accessed Jan 13, 2010.

Levy JH, O'Donnell PS. The therapeutic potential of a kallikrein inhibitor for treating hereditary angioedema. Expert Opin Investig Drugs 2006; 15:1077-90.

NORD policy position: insurance reimbursement for orphan drugs. National Organization for Rare Disorders (NORD) Web site. Available at: http://www.rarediseases.org/nord/news/policy/insur. Updated Oct 22, 2004. Accessed Jan 6, 2009.

Nzeako UC, Frigas E, Tremaine WJ. Hereditary angioedema, a broad review for clinicians. Archives of Internal Medicine 2001; 161:2417-29.

Pappalardo E, Cicardi M, Duponchel C, Carugati A, Choquet S, Agostoni A, et al. Frequent de novo mutations and exon deletions in the C1inhibitor gene of patients with angioedema. J Allergy Clin Immunol 2000; 106:1147-54.

Sullivan K, Winkelstein J. Genetically Determined Disorders of the Complement System. In: Scriver C, Beaudet A, Sly W, et al., eds. Metabolic and Molecular Bases of Inherited Disease. New York: McGraw Hill, 2001:4785-815.

Zuraw BL. Clinical practice. Hereditary angioedema. N Engl J Med 2008; 359:1027-36.

Zuraw BL. Hereditary angiodema: a current state-of-the-art review, IV: short- and long-term treatment of hereditary angioedema: out with the old and in with the new? Ann Allergy Asthma Immunol. 2008;100(Suppl 2):S13-S18

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