Hereditary Angioedema

Hereditary Angioedema is a genetic medical condition, which is associated with a deficiency of blood protein, called C1 inhibitor. C1 inhibitor is  Hereditary Angioedemathe complement system’s part, a protein group involved in some allergic and immune reactions. C1 inhibitor’s abnormal activity or deficiency results in swelling in skin’s local area and the tissues beneath it, or in the mucous membrane that is the lining body opening including gastrointestinal tract, throat, and the mouth.

Viral infections or injury frequently precipitates the attack, that may be caused by emotional distress. Attacks usually produce swelling areas, that are achy rather than itchy and are not accompanied by hives. Many individuals with Hereditary Angioedema have cramps, vomiting and nausea. The most severe complications include the upper airways swelling, which may affect breathing. Blood tests that measure activity or levels of C1 inhibitor, confirm diagnosis.

The treatment consists of medication called Aminocaprotic acid, which sometimes ends hereditary angioedema attacks. Corticosteroids, antihistamines, and epinephrine are frequently prescribed; although there is no proof that such medications are effective. Breathing may abruptly become obstructed, and a breathing tube can be required to be inserted in the individuals windpipe at the time of Hereditary Angioedema’s acute attack.

Specific treatment can help prevent the attacks. For instance, before undergoing dental procedure or minor surgery, an individual, who has Hereditary Angioedema can be given a fresh plasma transfusion to increase C1 inhibitor’s level in the person’s blood. Administration of purified C1 inhibitor, may prevent Hereditary Angioedema attack, but it is not yet avaliable for use. Androgens-oral anabolic steroid, including Danazol or Stanozolol may stimulate the person’s body to produce more C1 inhibitor,-for long term prevention. Because such medications may have masculinizing complications, the doses are monitored and carefully evaluated when given to females.

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