Treatment of hereditary angioedema

Therapie des hereditären angioneurotischen Ödems

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Zusammenfassung

In der vorliegenden Studie wird über die Ergebnisse verschiedener Behandlungsverfahren bei 20 Patienten mit hereditärem angioneurotischem Ödem berichtet. Die Wirksamkeit des Esterase-Inhibitors Trancxam-Säure zur Verhütung des Ödems wurde bei 15 Patienten erprobt. Bei 12 der 15 Patienten war Trancxam-Säure wirksam und ohne wesentliche Nebenwirkungen. 15 schwere Anfälle von angioneurotischem Ödem wurden behandelt mit intravenösen Infusionen entweder von Kallikrein-Inhibitor (8 Fälle) oder einem Konzentrat eines C1-Esterase-Inhibitors (7 Fälle). Nach dem Kallikrein-Inhibitor blieb nur in 1 Fall ein Erfolg aus. Das Konzentrat von C 1-Esterase-Inhibitor zeigte sich äußerst wirksam in der Behandlung akuter Anfälle (1 Mißerfolg infolge zu geringer Dosierung). Nebenwirkungen wurden bei beiden Behandlungsverfahren nicht beobachtet, jedoch konnte die Besserung mit Hilfe des C 1-Esterase-Inhibitors schneller erreicht werden. Die Serumspiegel von C 4- und C 1-Esterase-Inhibitor und die Aktivität von C 1-Esterase-Inhibitor vor und nach Langzeitprophylaxe und nach Therapie akuter Anfälle wurden untersucht.

Summary

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.

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Correspondence to Bianca Marasini M.D..

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Marasini, B., Cicardi, M., Martignoni, G.C. et al. Treatment of hereditary angioedema. Klin Wochenschr 56, 819–823 (1978). https://doi.org/10.1007/BF01489716

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Schlüsselwörter

  • Hereditäres angioneurotisches Ödem
  • Trancxam-Säure
  • C 1-Esterase-Inhibitor
  • Kallikrein-Inhibitor

Key words

  • Hereditary angioedema
  • Tranexamic acid
  • C 1 esterase inhibitor
  • Kallikrein inhibitor