Treatment of hereditary angioedema

Springer Science and Business Media LLC - Tập 56 - Trang 819-823 - 1978
Bianca Marasini1, M. Cicardi1, G. C. Martignoni1, A. Agostoni1
13° Cattedra di Patologia Speciale Medica dell'Università, Milano, Italia

Tóm tắt

The purpose of this study was to report the results of different treatments in 20 patients with hereditary angioedema. Effectiveness of tranexamic acid in preventing swellings was evaluated in 15 patients: in all but 3 subjects tranexamic acid was effective without serious side effects. 15 severe attacks of edema were managed with intravenous infusions of either kallikrein inhibitor (8 cases) or concentrate of C 1 esterase inhibitor (7 cases). In only 1 case was the kallikrein inhibitor unsuccessful. C 1 esterase inhibitor concentrate proved highly effective in the treatment of acute attacks (the result was lacking in one patient because of too low dosage of the drug). No side effects were observed with both treatments, but improvement was more rapidly achieved with infusion of C 1 esterase inhibitor. The serum levels of C 4 and C 1 esterase inhibitor and the activity of C 1 esterase inhibitor before and after long-term prophylaxis and acute attacks treatment were investigated.

Tài liệu tham khảo

Agostoni, A., Marasini, B., Cicardi, M., Martignoni, G.C., Brenna, O.: Studio mediante dosaggio enzimatico e immunochimico dell'inibitore della C 1 esterasi in 59 pazienti affeti da angioedema ereditario. Bollettino Istituto Sieroterapico Milanese,56, 22 (1977) Agostoni, A., Marasini, B., Martignoni, G.C., Cicardi, M.: Hereditary angioneurotic oedema. Klin. Wschr.53, 679 (1975) Agostoni, A., Martignoni, G.C.: Hereditary angioneurotic oedema. Lancetii, 325 (1973) Brackertz, D., Kueppers, F.: Hereditary angioneurotic oedema. Lancetii, 680 (1973) Davies, D., Howell, D.A.: Tranexamic acid and arterial thrombosis. Lanceti, 49 (1977) Donaldson, V.H., Evans, R.R.: A biochemical abnormality in hereditary angioneurotic oedema: absence of serum inhibitor of C 1 esterase. Amer. J. Med.35, 37 (1963) Donaldson, V.H., Merler, E., Rosen, F.S., Kretschmer, K.W., Lepow, I.H.: A polypeptide kinin in hereditary angioneurotic oedema plasma: role of complement in its formation. J. Lab. Clin. Med.76, 986 (1970) Fine, S.R., Fogarty, M., Linz, A.: Fresh frozen plasma for prophylaxis in hereditary angioedema. J. All. Clin. Immunol.57, 624 (1976) Frank, M.M., Gelfand, J.A., Atkinson, J.P.: Hereditary angioedema: the clinical syndrome and its management. Ann. Int. Med.84, 580 (1976) Frank, M.M., Sergent, J.S., Kane, M.A., Alling, D.W.: Epsilon aminocaproic acid therapy of hereditary angioedema. New Engl. J. Med.286, 808 (1972) Gelfand, J.A., Sherins, R.J., Alling, D.W., Frank, M.M.: Treatment of hereditary angioedema with Danazol. Reversal of clinical and biochemical abnormalities. New Engl. J. Med.295, 1444 (1976) Jaffe, C.J., Atkinson, J.P., Gelfand, J.A.: Hereditary angioedema. The use of fresh frozen plasma for prophylaxis in patients undergoing oral surgery. J. All. Clin. Immunol.55, 386 (1975) Johns, M.E., Vanselow, N.A., Boles, R., Arbor, A.: Hereditary angioneurotic oedema, treatment with epsilon aminocaproic acid during surgery. Arch. Otolaryng.99, 388 (1974) Juhlin, L., Michaelson, G.: Use of a kallikrein inhibitor in the treatment of urticaria and hereditary angioneurotic edema. Acta Derm.-Venereol.49, 37 (1969) Klemperer, M.R., Rosen, F.S., Donaldson, V.H.: A polypeptide derived from the second component of human complement (C 2) which increases vascular permeability. J. Clin. Invest.48, 44 (abs.) (1969) Lachmann, P.J., Hobart, M.J., Aston, W.P.: Complement technology. In: “Handbook of experimental immunology” D.M. Weir ed., Oxford: Blackwell 1973, pp. 1–17 Landermann, N.S.: Hereditary angioneurotic edema. Case report and review of the literature. J. All.33, 316 (1962) Lundh, B., Laurell, A.B., Wetterquist, H., White, T., Granerus, G.: A case of hereditary angioneurotic oedema successfully treated with epsilon aminocaproic acid. Studies on C 1 esterase inhibitor, C 1 activation, plasminogen level and histamine metabolism. Clin. Exp. Immunol.3, 733 (1968) Pence, R.L., Evans, R., Guernsey, L.H., Gerhard, R.C.: Prophylactic use of epsilon aminocaproic acid for oral surgery in a patient with hereditary angioneurotic edema. J. All. Clin. Immunol.53, 298 (1974) Pickering, R.J., Kelly, J.R., Good, R.A., Gevurz, H.: Replacement therapy in hereditary angioedema. Successful treatment of two patients with fresh frozen plasma. Lanceti, 326 (1969) Proud, G., Chamberlain, J.: Anaphylactic reaction to aprotinin. Lancetii, 48 (1976) Rosen, F.S., Austen, K.F.: Androgen therapy in hereditary angioneurotic edema. New Engl. J. Med.295, 1476 (1976) Rosen, F.S., Charache, P., Pensky, J., Donaldson, V.H.: Hereditary angioneurotic edema: two genetic variants. Science148, 957 (1965) Ross, W.F., Logue, G.L., Silbermann, H.R.: The effect of synthetic androgens on clinical course and C 1 esterase inhibitor (C 1-INH) levels in hereditary angioneurotic edema (HANE). Clin. Res.24, 482 (abs.). (1976) Sjolm, I., Wiman, B., Wallen, P.: Studies on conformational changes of plasminogen induced during activation to plasmin and by 6-aminoexanoic acid. Eur. J. Biochem.39, 471 (1973) Soter, N.A., Austen, K.F., Gigli, I.: Inhibition by epsilon aminocaproic acid of the activation of the first component of the complement system. J. Immunol.114, 928 (1975) Violand, B.N., Sodetz, J.M., Castellino, F.J.: The effect of aminocaproic acid on the gross conformation of plasminogen and plasmin. Arch. Biochem. Biophys.170, 300 (1975) Vogelaar, E.F., Brummelhuis, H.G.J., Krunen, H.W.: Contribution to the optimal use of human blood. Vox Sang.26, 118 (1974) Zachariae, H., Laurberg, G., Hjortshoj, A.: Tranexamic acid (Cyklokapron) in hereditary angioneurotic edema. Ugeskrift for Laege137, 1106 (1975)