[Hereditary angioedema by defict of C1 esterase. Our experience in 8 cases].
Eight cases of hereditary angioedema, all of them with low values of C1-sterase inhibitor are analyzed. In 7 cases the C3 and C4 components of the complement were assessed; the results showed marked descent of C4. The 8 patients came from 4 different families; only 2 of them were males. Six patients presented digestive disorders, reporting colic pain, nausea and vomiting. In 1 of them the abdominal picture was the only evidence of the disease. In 5 patients the angioedema episodes occurred following traumatisms and in 3 because of emotional states. The duration of the attacks varied from several hours to six days. There was a familial history in all cases. Three of the patients had repeated episodes of pharyngolaryngeal angioedema, two of them requiring emergency tracheotomy because of suffocating crisis. Six patients were treated with Epsilon aminocaproic acid (16 to 20 gr daily) or with tranexamic acid (1 to 3 gr. daily). In 4 cases the results were excellent with either of these antifibrinolytic drugs. No side effects were observed in the tranexamic acid therapy whilst they were frequent in the treatment with Epsilon aminocaproic acid.
D000293:Adolescent; D000328:Adult; D000799:Angioedema / Q000235:genetics / Q000276:immunology*; D002648:Child; D002675:Child, Preschool; D003166:Complement Activating Enzymes / Q000172:deficiency*; D003174:Complement C1 Inactivator Proteins / Q000172:deficiency*; D003173:Complement C1s / Q000172:deficiency*; D003176:Complement C3 / Q000032:analysis; D003181:Complement C4 / Q000032:analysis; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged
D003174:Complement C1 Inactivator Proteins; D003176:Complement C3; D003181:Complement C4; D003166:Complement Activating Enzymes; D003173:Complement C1s
Angioedema hereditario por déficit del inhibidor de la C1 esterasa. Nuestra experiencia en ocho casos.