What causes hereditary angioedema to occur?

Abnl or absent prote. Hae is the result of missing or a poorly functioning protein called c1 esterase inhibitor. When this protein is missing or abnormal then a patient may to prone to swelling problems which can be life threatening.

Related Questions

What cause hereditary angioedema?

HAE. Hereditary angioedema (hae) is caused by a an uncontrolled activation of a complex cascade of proteins called complement. Hae is a result of a deficiency or lack of functional c1 esterase inhibitor protein. Some patients are born with this problem while other may acquire a lack of c1 esterase inhibitor from an underlying disease such as cancer. There are new treatments available.

Whats hereditary angioedema?

Severe Swelling. Hereditary angioedema is rare but serious genetic immune system problem that is due to a deficiency of c1 esterase inhibitor. It is characterized by swelling of the face and airways, and my be life threatening.
Different types. There are several different types of hereditary angioedema. The most common forms are the result of a deficient protein, called c1 esterase inhibitor, or because this protein does not work correctly. Other forms of hae may be related to hormone problems.
Recurrent swelling. Hae is a blood disorder that leads to episodic attacks of recurrent swelling of the face, lips, tongue, throat, extremities, or other areas of the body. Swelling of the intestines can also lead to severe abdominal pain and vomiting. Triggers include trauma, surgery, dental work, menstruation, some medications, viral illness and stress. Diagnosis is made based on clinical history and via bloodwork.
Swelling hereditary. Hereditary angioedema is an inherited disorder of c1 inhibitor that is involved in inflammatory reactions. This deficiency lead to swelling of skin, larynx, and abdominal pain occurring recurrently. The deficiency runs in the family. The episodes are usually self limited.
Genetic disorder. HAE is genetic disorder resulting in unpredictable swelling of any part of the body. It is not caused by histamine, but a chemical caused bradykinin. When released in uncontrolled manner the part of the body where this occurs swells. Swelling occurs within 6-36 hours and if untreated resolves in 2-5 days. There is now a replacement enzyme for Type I and II. 4 on demand meds are FDA approved.

Can anyone have hereditary angioedema?

Yes. Hereditary angioedema types 1 and 2 have a very strong genetic component and typically runs in families. However, spontaneous mutations can occur and hae I and ii can occur on anyone.

Anyone here treat hereditary angioedema (hae)?

allergists can help. There been exciting and new pharmaceutical options developed for the treatment of acute attacks of hereditary angioedema. Typically board certified allergists are well-versed in the diagnosis and management of hereditary angioedema.
Allergist. HAE is a rare genetic disorder with a family hist 75% of the time. There have been 6000 pts in the USA diagnosed. 25% of pts have a new genetic mutation therefore no family history. Allergists almost always treat this disorder but only a small percentage are experts. Treatment should be in association with an expert. Contact the HAEA for an expert in your region.

At what age do attacks of hereditary angioedema start?

Most before age 15. About 40% of people with hereditary angioedema (hae) experience their first episode before age 5, and 75% present before age 15. There is one type of hae (type 3), found in the second decade of life or later, and rarely occurs before puberty.
Most before puberty. 40% of individuals with hereditary angioedema (hae) have attacks before age 5 and 75% have attacks before age 15. Swelling is not itchy and there are no hives in hae. Frequency of attacks increases after puberty. Diagnosis usually made in 2nd or 3rd decade of life by history with confirmatory blood tests. See your md for diagnosis and if indicated preventive treatment.
HAE presentation. One should keep in mind that the angioedema of hae is not associated with hives. Additionally, the swelling does not respond to antihistamines and corticosteroids.

Does anyone have experience with hereditary angioedema?

Hereditary angio ede. Hereditary angioedema (ha) is a disease that is typically noted to run in families. It is causes by low levels or poor function in a controller protein called c-1 esterase inhibitor. If swelling (angioedema) occurs with hives, then the angio edema is unlikely to be due to this rare problem. Typical angioedema develops quickly, often maximally within one hour. Ha may swell slowly over many hrs.

Is hereditary angioedema different from regular angioedema?

Inherited v acquired. Angioedema, a condition involving rapid swelling & accumulation of fluid in the skin & deeper tissues, is usually classified as hereditary (similar episodes run in the family) or acquired (due to medications or other allergic reaction). A third category sometimes used is idiopathic, meaning no obvious pathology or cause can be determined. Angioedema can involve the airways & cut off breathing.
Allergy or not. Angioedema such as swelling of the tongue or lips can occur as the result of an allergy to food or medication. It is usually associated with hives (urticaria). The presence of hives essentially rules out the allergic cause making hereditary or acquired c-1 inhibitor deficiency likely. However, the lack of hives does not rule out allergy. See an allergist for further evaluation either way!

Hereditary angioedema - can anybody tell me anything about?

Abnl or absent prote. Hae is the result of missing or a poorly functioning protein called c1 esterase inhibitor. When this protein is missing or abnormal then a patient may to prone to swelling problems which can be life threatening.

How long does a hereditary angioedema attack typically last?

Hereditary angioedem. Very difficult to say since hae patients can have many attacks, even oneon top of another. The "typical" time course for an angioedema attack is a 72-hour progression to fully swollen and then another 72-hours until resolution (with or without therapy, which will shorten time course).
36 to 48 hours. Typically a pt who has a hist of HAE attacks can "sense" hours or even a day or two before. The swelling is noted over a 6-24 hour period and typically peaks at 36-48 hours. The most important point here is to treat at the very first symptom with your on demand medication. It's important to recognize even the slightest sense of a laryngeal attack gets med ASAPand then to the ER to be watched.