[Clinical features, pathogenesis, and therapy of hereditary angioneurotic edema (author's transl)].
The hereditary angioneurotic edema is probably a more frequent disease than presumed until now. It is inherited as an autosomal dominant trait, and is due to diminished or functionally deficient C1 esterase inhibitor. Family history is negative in cases of spontaneous mutations. Two thirds of the patients are already affected in infancy with peripheral edema, facial edema, abdominal symptoms and/or edema of the glottis occuring spontaneously or secondary to trauma. Recently the prognosis has become better due to early diagnosis and the possibility of substitution therapy with C1 esterase inhibitor. A long-term prophylaxis may be achieved with synthetic androgens.
D000293:Adolescent; D000328:Adult; D000728:Androgens / Q000627:therapeutic use; D000799:Angioedema / Q000175:diagnosis / Q000188:drug therapy / Q000235:genetics*; D003173:Complement C1s / Q000172:deficiency; D005260:Female; D006801:Humans; D008297:Male; D011247:Pregnancy; D011379:Prognosis
D000728:Androgens; D003173:Complement C1s
Klinik, Pathogenese und Therapie des hereditären angioneurotischen Odems.