Hereditary angioedema is a rare but serious problem with the immune system. The problem is passed down through families. It causes swelling, particularly of the face and airways, and abdominal cramping.Hereditary angioedema (HAE) is caused by a low level or improper function of a protein called the C1 inhibitor. With HAE the blood vessels are affected. An HAE attack can result in rapid swelling of the hands, feet, limbs, face, intestinal tract, larynx (voicebox), or trachea (windpipe). Attacks of swelling can become more severe in late childhood and adolescence.Physical signs of HAE include overt, non inflammatory swelling of the skin and mucous membranes. Attenuated androgens are currently the initial mode of prophylactic treatment. Therapy should be minimized, balancing disease severity with minimizing adverse effects. The drug most commonly used is danazol, but all attenuated androgens are useful in treatment.
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Last date updated on October, 2020