Hereditary angioedema: an appraisal of 104 cases.
One hundred and four patients affected by hereditary angioedema belonging to 31 families have been studied. Twenty-two percent had the variant form related to the deficiency of the functional activity of serum C1 esterase inhibitor. The remaining 78% of patients had the predominant form, characterized by low antigenic levels and low functional activity of serum C1 esterase inhibitor. Attacks of swelling affected the subcutaneous tissue in 86% of patients; the upper airways in 76% of patients, and the bowel mucose in 75% of patients. Before treatment was available the mortality rate was 56%. One or more attacks a month were present in 46% of cases. The infusion of C1 inhibitor concentrate promptly reversed 14 severe attacks without any side effect. Twenty-nine patients were given long term prophylactic treatment with androgen derivatives with full success. Tranexamic acid reduced the frequency of swelling of 70% of the patients.
D000293:Adolescent; D000328:Adult; D000368:Aged; D000799:Angioedema / Q000188:drug therapy / Q000235:genetics* / Q000517:prevention & control; D002648:Child; D002675:Child, Preschool; D003174:Complement C1 Inactivator Proteins / Q000097:blood / Q000627:therapeutic use; D003613:Danazol / Q000627:therapeutic use; D004487:Edema / Q000473:pathology; D005260:Female; D006801:Humans; D007819:Laryngeal Edema / Q000473:pathology; D008297:Male; D008875:Middle Aged; D011247:Pregnancy; D011654:Pulmonary Edema / Q000473:pathology; D014148:Tranexamic Acid / Q000627:therapeutic use
D003174:Complement C1 Inactivator Proteins; D014148:Tranexamic Acid; D003613:Danazol