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Hereditary Angioedema (HAE) SymptomsHereditary Angioedema (HAE) patients experience recurrent episodes of swelling in the hands, feet, face, gastrointestinal tract, genitals, and larynx (throat) that can last from two to five days. The frequency of and severity of attacks varies dramatically among patients, and even among those within the same family. A survey of HAE patients in Italy (over 500 identified and followed) revealed that slightly less than one half of untreated patients have more than one attack per month, and forty percent have 6 to 11 swellings per year.
HAE-related swelling is non-whealing and non-pruritic with ill-defined margins, and generally remains unrelieved by antihistamines and corticosteroids. About 25 percent of Hereditary Angioedema (HAE) patients experience a non-pruritic erythematous rash that often occurs during the attack prodrome.
Swelling involving the feet and hands is uncomfortable and can be extremely painful, and often prevents patients from being able to participate in normal daily routines.
Gastrointestinal attacks are characterized by excruciating abdominal pain, nausea, vomiting, and diarrhea caused by swelling in the intestinal wall. These symptoms are distinguishing features of Hereditary Angioedema (HAE) because abdominal pain is rarely seen in other types of angioedema. Hypovolemia (an abnormal decrease in blood volume) can occur from a combination of fluid loss, plasma extravasation, and vasodilation, and can progress into hypovolemic shock.
Approximately one third of patients with undiagnosed Hereditary Angioedema (HAE) undergo unnecessary surgery during abdominal attacks because the symptoms mimic a surgical emergency.
Laryngeal edema is the most significant feature of HAE, because laryngeal swelling can close the airway and cause death by asphyxiation. Studies of Hereditary Angioedema (HAE) families indicate that death from laryngeal edema can be as high as 30 percent of those affected by the disease. Surveys of Hereditary Angioedema (HAE) patients reveal that approximately 50 percent experienced at least one laryngeal episode in their lives. A study by German investigators that focused exclusively on airway attacks in 123 patients revealed that nearly 80% of the laryngeal edemas occurred between the ages of 11 and 45 years, and the mean interval between onset and maximum development of laryngeal edema was 8.3 hours. Nevertheless, laryngeal edema must be treated as a medical emergency, and patients should seek prompt treatment as soon as throat involvement is suspected.
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What causes Hereditary Angiodema (HAE) attacks?
Most attacks occur spontaneously, with no apparent reason; however anxiety, stress, minor trauma, surgery, and illnesses such as colds and flu have been cited as triggers. Trauma to the oral cavity caused by dental procedures make HAE patients particularly vulnerable to airway attacks. Patients have also reported swelling in extremities following typing, prolonged writing, pushing a lawn mower, hammering, shoveling, and other physical activities.
In women, menstruation and pregnancy seem to have a major effect on disease activity. Some women patients report a definite increase in the number of attacks during their menstrual periods. During pregnancy, some patients note an increase in the frequency of attacks, while others have reported a decrease. Use of oral contraceptives and hormone replacement therapy is associated with an increase in the frequency and severity of attacks.
ACE Inhibitors have been known to increase the frequency and intensity of HAE attacks. ACE Inhibitors are often prescribed to treat high blood pressure. Below is a list of some of the ACE Inhibitors licensed in the US:
* captopril (Capoten)
* benazepril (Lotensin)
* enalapril (Vasotec)
* lisinopril (Prinivil, Zestril)
* fosinopril (Monopril)
* ramipril (Altace)
* perindopril (Aceon)
* quinapril (Accupril)
* moexipril (Univasc)
* trandolapril (Mavik)
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