Epsilon-amino-caproic acid in the treatment of Osler's hereditary angioneurotic edema.
This clinical entity described for the first time by Osler in 1888 presented a great therapeutic problem during many decades because of its severity. Landerman and later on Donaldson and Evans established the pathogenic mechanisms of this disease finding a deficiency in the inhibitor of the first activated component of complement, an alpha 2 aminoglycoprotein, to be the mechanism responsible of the same. More concretely, alterations in the plasmin, kinin and kallikrein systems are those that will lead to a change in vascular permeability with resultant tissue alterations. Four cases of hereditary angioneurotic edema are studied in female patients aged between 15 and 50 years and with family history consistent with angioneurotic familiar edema in which there were six cases of death due to edema of the glottis. Once the diagnosis had been made the patients were subjected to treatment with EACA at doses of 2.5 gm every 6 hours. The determinations of complement were similar in the four cases, with marked decreases in C4 and C1 inhibitor with a decrease in total complement in three cases. Regarding secondary effects, vomiting was found only in one cases, which as the dose was reduced did not necessitate termination of treatment. In summary, considering the results obtained in the cases above, we believe that due to its good tolerance and moderate cost, epsilon-amino-caproic acid at the abovementioned dosage is an excellent pharmacological agent in the treatment of Osler's hereditary angioneurotic edema.
D000293:Adolescent; D000614:Aminocaproates / Q000627:therapeutic use*; D015119:Aminocaproic Acid / Q000494:pharmacology / Q000627:therapeutic use*; D000799:Angioedema / Q000188:drug therapy* / Q000235:genetics / Q000276:immunology; D003174:Complement C1 Inactivator Proteins / Q000172:deficiency; D003181:Complement C4 / Q000172:deficiency; D003171:Complement Pathway, Classical / Q000187:drug effects; D005260:Female; D006801:Humans; D007136:Immunoglobulins / Q000276:immunology; D008875:Middle Aged
D000614:Aminocaproates; D003174:Complement C1 Inactivator Proteins; D003181:Complement C4; D007136:Immunoglobulins; D015119:Aminocaproic Acid