Klinische Wochenschrift

, Volume 51, Issue 12, pp 620–622 | Cite as

Possible therapy in hereditary angioneurotic edema (HAE)

  • D. Brackertz
  • F. Kueppers
Originalien

Summary

The symptomatic therapy of hereditary angioneurotic edema with tranexamic acid and suramin in two patients is reported. The first patient responded well to tranexamic acid (Cyclocapron®) but not to suramin. In the second patient, attacks of edema could apparently be better prevented with suramin.

Key words

Hereditary angioneurotic edema tranexamic acid suramin C\(\bar 1\)-inhibitor 

Mögliche neue Therapie beim hereditären angioneurotischen Ödem

Zusammenfassung

Es wird über die symptomatische Therapie des hereditären angioneurotischen Ödems mit Tranexamsäure und Suramin bei zwei Patienten berichtet. Während der erste Patient gut auf eine Therapie mit Tranexamsäure (Cyclocapron®), nicht jedoch auf Suramin ansprach, ließen sich beim zweiten Patienten die Ödemanfälle mit Suramin unterbinden.

Schlüsselwörter

Hereditäres angioneurotisches Ödem Tranexamsäure Suramin C\(\bar 1\)-Inhibitor 

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  1. 1.
    Ablondi, F.B., Hagan, J.J., Philips, M., De Renzo, E.C.: Inhibition of plasmin, trypsin, and the streptokinase-activated fibrinolytic system by ε-aminocaproic acid. Arch. Biochem. Biophys.82, 153 (1959).PubMedGoogle Scholar
  2. 2.
    Alkaersig, N., Fletcher, A.P., Sherry, S.: ε-aminocaproic acid: An inhibitor of plasminogen activation. J. biol. Chem.234, 832 (1959).PubMedGoogle Scholar
  3. 3.
    Austen, K.F.: Inborn and acquired abnormalities of the complement system of man. Hopkins med. J.128, 75 (1971).Google Scholar
  4. 4.
    Bruhn, H.D., Mueller, L., Duckert, F.: Quantitative determination of plasminogen. A caseinolytic method. Thrombos. Diathes. haemorrh. (Stuttg.)23, 191 (1970).Google Scholar
  5. 5.
    Champion, R.H., Lachmann, P.J.: Hereditary angio-oedema treated with ε-aminocaproic acid. Brit. J. Derm.81, 763 (1969).PubMedGoogle Scholar
  6. 6.
    Donaldson, V.H., Evans, R.R.: Biochemical abnormality in hereditary angioneurotic edema: Absence of serum inhibitor of C′1 esterase. Amer. J. Med.35, 37 (1963).PubMedGoogle Scholar
  7. 7.
    Fong, J.S.C., Good, R.A.: Suramin—a potent reversible and competitive inhibitor of complement systems. Clin. exp. Immunol.10, 127 (1972).PubMedGoogle Scholar
  8. 8.
    Frank, M.M., Sergent, J.S., Kane, M.A., Alling, D.W.: Epsilon aminocaproic acid therapy of hereditary angioneurotic edema. New Engl. J. Med.286, 808 (1972).PubMedGoogle Scholar
  9. 9.
    Juhlin, L., Michaelsson, G.: Vascular reactions in hereditary angioneurotic edema. Acta derm.-venereol (Stockh.)49, 20 (1969).PubMedGoogle Scholar
  10. 10.
    Kueppers, F., Berendes, U., Schöpf, E.: Inherited high serum levels of a functionally deficient complement esterase inhibitor associated with hereditary angioedema. Hum. Genet.15, 119 (1972).Google Scholar
  11. 11.
    Laurell, A.B., Martensson, U.: C1 inactivator protein complexed with albumin in plasma from a patient with angioneurotic edema. Europ. J. Immunol.1, 146 (1971).Google Scholar
  12. 12.
    Lundh, B., Laurell, A.B., Wetterquist, H., White, T.: A case of hereditary angioneurotic oedema successfully treated with ε-aminocaproic acid. Studies on C′1 esterase inhibitor, C′1 activation, plasminogen level and histamine metabolism. Clin. exp. Immunol.3, 733 (1968).PubMedGoogle Scholar
  13. 13.
    Mancini, G., Carbonara, O.A., Heremans, J.F.: Immunochemical quantitation of antigen by single radial immunodiffusion. Immunochemistry2, 235 (1965).PubMedGoogle Scholar
  14. 14.
    Nilsson, J.M., Andersson, L., Bjorkman, S.E.: Epsilon-aminocaproic acid (E-ACA) as a therapeutic agent based on 5 years' clinical experience. Acta med. scand., Suppl.448, 1 (1966).Google Scholar
  15. 15.
    Nyman, D.: The preparation of an artificial reagent for the onestage factor VIII assay. Thrombos. Diathes. haemorrh. (Stuttg.)23, 306 (1970).Google Scholar
  16. 16.
    Ratnoff, O.D., Pensky, J., Ogston, D.: Inhibition of plasmin, plasma kallikrein, plasma permeability factor, and the C′1r subcomponent of the first component of complement by serum C′1 esterase inhibitor. J. exp. Med.129, 315 (1969).PubMedGoogle Scholar
  17. 17.
    Rosen, F.S., Charche, P., Pensky, J., Donaldson, V.H.: Hereditary angioneurotic edema: two genetic variants. Science148, 952 (1965).Google Scholar
  18. 18.
    Rosen, F.S., Alper, C.A., Pensky, J., Klemperer, M.R., Donaldson, V.H.: Genetically determined heterogeneity of the C1 esterase inhibitor in patients with hereditary angioneurotic edema. J. clin. Invest.50, 2143 (1971).PubMedGoogle Scholar
  19. 19.
    Schreiber, A.D., Kaplan, A.P., Austen, K.F.: The common activation and regulation of the coagulation, fibrinolytic, and kinin-generating pathways in human plasma. J. clin. Invest.51, 87a (1972).Google Scholar
  20. 20.
    Schumann, G., Mueller, H.-L.: Eine einfache Methode zur serienmäßigen Bestimmung der Komplement-Aktivität im Serum. Z. Immun.-Forsch.141, 201 (1971).Google Scholar
  21. 21.
    Sheffer, A.L., Austen, K.F., Rosen, F.S.: Treatment of hereditary angioedema with trans-AMCHA. J. Allergy49, 133 (1972).Google Scholar

Copyright information

© Springer-Verlag 1973

Authors and Affiliations

  • D. Brackertz
    • 1
    • 2
  • F. Kueppers
    • 1
    • 2
  1. 1.Rheumatologische Universitätsklinik BaselSwitzerland
  2. 2.Universitäts-Hautklinik HamburgGermany

Personalised recommendations