[Treatment of hereditary angioedema].
Hereditary angioedema is a rare familial disease caused by the defect of complement C1esterase inhibitor (C1-INH). It is characterized by recurrent acute edema of the extremities, the face, the respiratory tract and the gastrointestinal tract. Acute laryngeal edema usually produces laryngeal obstruction. Two cases have been treated since 1986, one of them had been admitted for forty-five times because of recurrent acute laryngeal edema. Investigations showed two families with a high incidence of this disease. Laboratory examination showed a remarkable decrease of C1-INH and C4. Tracheotomy is indicated in patients with laryngeal edema. Great success was achieved in two patients treated with danazol.
Qian
X
X
ENT Institute, Shanghai Medical University.
D000799:Angioedema / Q000188:drug therapy* / Q000235:genetics / Q000378:metabolism; D003181:Complement C4 / Q000378:metabolism; D003169:Complement Inactivator Proteins / Q000378:metabolism; D003613:Danazol / Q000008:administration & dosage / Q000627:therapeutic use*; D006801:Humans; D007819:Laryngeal Edema / Q000188:drug therapy* / Q000235:genetics / Q000378:metabolism; D008297:Male; D008875:Middle Aged
D003181:Complement C4; D003169:Complement Inactivator Proteins; D003613:Danazol