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Angioedema glossary

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ACE inhibitor

ACE (Angiotensin Converting Enzyme) inhibitors are commonly prescribed to patients with hypertension (high blood pressure) as well as those with cardiac insufficiency, following a heart attack or with renal insufficiency. About 0.1-0.6% of patients treated with ACE inhibitors develop angioedema. ACE inhibitors, whose generic or chemical names end in -pril, include: benazepril, captopril, cilazapril, enalapril, fosinopril, lisinopril, moexipril, perindopril, quinapril, ramipril, spirapril, trandolapril.

Acute abdomen

This is a situation characterised by the sudden manifestation of severe abdominal pain in patients with a possibly life-threatening disorder within the abdomen. Many patients require emergency surgery.

Adrenalin

A so-called “stress hormone”. In Medicine, adrenalin is used as an emergency medication in patients with severe allergic reactions, as well as for some other indications. Among its physiological actions, adrenalin causes constriction (narrowing) of the blood vessels and also reduces the permeability of the vascular wall.

Allergy

“Intolerance reactions” on the part of the body’s immune system, representing an exaggerated defence response against certain, usually harmless substances (allergens) encountered in the environment.

Angioedema

Swelling of the deeper skin layers and/or the mucous membranes caused by outflow of fluid from the blood vessels into these tissues.

Antihistamine

Drugs that counteract the effects of the natural mediator substance, histamine.

Ascites

Pathological accumulation of free fluid within the abdominal cavity.

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Aspirin

Aspirin is the brand name of the common analgesic (pain reliever), acetylsalicylic acid (ASA). This medication acts to relieve pain, reduce inflammation and lower fever. It can be purchased without a doctor’s prescription and is commonly used for headaches and other aches and pains.

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AT-1 Blocker

Angiotensin receptor (AT-1) blockers are medications frequently used to treat patients with hypertension (high blood pressure) as well as in cases with cardiac insufficiency, following heart attacks and in patients with renal insufficiency. Use of AT-1 blockers has been associated in individual cases with swelling attacks, though these are much less frequently reported than in patients taking ACE inhibitors. Medications belonging to the AT-1 blocker family, all ending in -sartan, include candesartan, losartan and valsartan.

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Autosomal dominant

Traits that are inherited independent of gender and that suppress other traits. Children of an affected parent have a 50% chance of inheriting a trait subject to autosomal dominant inheritance.

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Bradykinin

A peptide, actions of which are similar to those of histamine. Bradykinin binds to receptors in the cells of the blood vessels and, among other things, increases the permeability of the vascular wall. Bradykinin is formed through activation of the  kallikrein-kinin system.

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Bradykinin B2 receptors

Specific receptors located in the wall of the blood vessel. They bind bradykinin, which sets certain processes in motion, resulting in increased permeability of the vascular wall and increase passage of fluid into the surrounding tissues.

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Bradykinin receptor antagonist

A synthetically manufactured peptide (chemical name: icatibant), whose chemical structure resembles that of bradykinin and which is able to displace it from its B2 receptor. Icatibant is used in the treatment of swelling attacks in patients with hereditary angioedema (HAE) caused by a C1-INH deficiency.

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C1-esterase inhibitor

C1-esterase inhibitor (C1-INH) is an important protein that regulates a number of metabolic processes in the body. Persons with hereditary angioedema (HAE) either produce too little C1-INH or their C1-INH is defective in function. Among other functions, C1-INH acts to prevent the excessive production of bradykinin in association with inflammation, coagulation reactions and other processes in the body. Bradykinin increases the permeability of the blood vessel wall. If the synthesis of bradykinin is not kept within its proper limits by C1-INH, angioedema attacks may occur.

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C1-INH

See C1-esterase inhibitor.

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C1-INH concentrate

C1-esterase inhibitor derived from blood plasma and concentrated for use in the treatment of swelling attacks in patients with hereditary angioedema (HAE).

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C1q

One factor of the complement system. Determination of C1q levels can help to differentiate between hereditary angioedema (HAE) (concentration of C1q is normal) and acquired angioedema (AAE) (concentration of C1q is reduced).

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C4

One factor of the complement system. It is an important laboratory parameter for differentiating between types of hereditary angioedema (HAE) (concentration of C4 is reduced in types I and II, but normal in the rare type III HAE). 

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Chromosome

Structures located in the cell nucleus that consist of a chain of DNA and carry the organism’s genetic information.

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Complement factors

Any of several protein molecules comprising the complement system.

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Complement system

A specific reaction chain in the human body comprising more than 30 proteins, some dissolved in the blood, others bound to the surface of the cells. The complement system is part of the body’s immune system and is responsible for, among other duties, the destruction or dissolution of foreign cells, bacteria or viruses that have penetrated the body’s physical defences and for inflammatory reactions.

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Computed tomography (CT)

A radiological method that by visualization of the body in the form of slices permits examination of internal organs and pathological changes in these organs. The examination requires application of x-ray radiation to the part of the body to be examined.

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Coniotomy

Emergency surgical procedure for creating an artificial opening in the airway at the level of the larynx. Coniotomy consists of surgically dividing the cricothyroid membrane between the thyroid cartilage and cricoids cartilage of the larynx.

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Corticosteroid

Any of a group of steroid hormones used as medications that can be used to suppress inflammatory reactions in allergies and in other conditions. A natural corticosteroid is the hormone, cortisone, which is produced by the adrenal cortex.

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Cortisone

A natural corticosteroid produced by the adrenal cortex. This substance is also frequently used as a medication to suppress inflammatory reactions in allergies and in other conditions.

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Dysphagia

Disorder of swallowing that may be a possible symptom of a swelling attack affecting the laryngeal area.

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Endoscopy

An examination of body cavities or of hollow organs performed by introducing a special optical device (“endoscope”).

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Erythema marginatum

Inflammatory annular (circular) reddening of the skin caused by increased perfusion. It may precede some angioedema attacks (prodromal symptom).

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Gene

A section of the genetic material inside the cell nucleus that encodes for the production of a certain protein that performs a specific function in the body. Genes are located on the chromosomes.

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HAE

See hereditary angioedema (HAE).

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Hereditary

Inherited. Used in reference to traits or diseases passed on from parents to children as part of their genetic material.

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Hereditary angioedema

Hereditary angioedema (HAE) is a disorder caused by a change (mutation) in the genetic information of the affected individual. The mutation affects a gene located on chromosome 11, which is responsible for the synthesis of the C1-esterase inhibitor (C1-INH). C1-INH is an important protein that regulates a variety of metabolic processes in the body. Persons with Hereditary angioedema (HAE) produce either too little C1-INH or a form of C1-INH that does not function correctly. Among other functions, C1-INH acts to prevent the excessive production of bradykinin in association with inflammation, coagulation reactions and other processes in the body. Bradykinin increases the permeability of the blood vessel wall. If the synthesis of bradykinin is not kept within its proper limits by C1-INH, angioedema attacks may occur.

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Histamine

A protein released in the body during allergic or allergy-like reactions. Among its other effects, histamine acts to temporarily increase the permeability of the blood vessel wall, allowing fluid to leave the blood vessel and enter the surrounding tissue. The result is swelling. Allergy-related angioedema is therefore described as histamine-mediated angioedema.

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Icatibant

A bradykinin receptor antagonist, icatibant is a synthetically manufactured protein whose chemical structure resembles that of bradykinin and acts to displace bradykinin from its receptor. Icatibant is used in the treatment of swelling attacks in patients with hereditary angioedema with C1-INH deficiency.

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Idiopathic

A disorder or change, whose cause is not known, and which occurs independently of other diseases.

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Intubation

Insertion of a tube through the mouth or nose to secure the airway and breathing.

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Kallikrein-kinin system

Also known as the kinin system. It consists of different proteins in the blood, which affect or control a variety of processes and reactions in the body, including inflammation, coagulation, blood pressure control and pain. One result of the activation of the kallikrein-kinin system is the production of bradykinin. Among its many functions, bradykinin causes dilatation of the blood vessels and increases the permeability of the vascular wall.

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Kinin system

Please see kallikrein-kinin system, above.

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Laryngoscopy

Examination of the larynx using an optical instrument (laryngoscope).

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Larynx

The larynx is a structure in the respiratory tract that is located at the point of transition from the throat to the trachea (the tube leading to the lungs).

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Magnetic resonance imaging (MRI)

A diagnostic imaging method that provides cross-sectional images (“slices”) of the body, permitting evaluation of the internal organs and any pathological changes. The method uses a magnetic field to obtain data that are processed by a computer to yield cross-sectional images.

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Mutation

Any change in the normal make-up of an individual’s genetic material.

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New mutation

A new or de novo mutation represents a change in the genetic material that has occurred spontaneously in an individual and has not been inherited from that individual’s parents.

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Opiates

Any of a group of pharmaceutical substances derived from opium. Certain opiates, such as morphine, are used to control severe pain.

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Oestrogens

Any of a group of steroid hormones that predominate in women and are related to the functioning of their reproductive system.

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Pathophysiology

The branch of medical science that studies and describes the abnormalities in the life functions of an organism associated with disease.

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Prodromes

Precursor symptoms. Among the recognised precursors of a swelling attack is erythema marginatum.

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Stridor

Whistling breathing sound. It is a possible sign for a swelling attack involving the larynx or adjacent tissue. This pathological breathing sound is caused by narrowing or partial obstruction of the airways.

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Symptoms

Physical signs or characteristics that accompany a disease or disorder.

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Tracheotomie

Surgical procedure to create an artificial opening in the trachea, the tube leading to the lungs.

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Urticaria

Urticaria is one of the most common disorders of the skin. It is also commonly known as hives. About one in four persons experiences urticaria at some point in their lives. Symptoms of urticaria include the sudden appearance of pruritic (itching) hives on the skin. For more information, please go to www.urtikaria.net)

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Vasoactive

The property of a natural or artificial substance at affects the blood vessel wall or the degree of dilatation or constriction of the blood vessel. Certain vasoactive substances, such as histamine or bradykinin, temporarily increase the permeability of the vascular wall, permitting increased amounts of fluid to escape into surrounding tissues, thus promoting swelling of the skin or mucous membrane.

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