DefinitionThis section has been translated automatically.
Acquired angioedema at the base of an (acquired)C1-esterase inhibitor deficiency. Typical are decreased values forC1-esterase inhibitor, CH50, CC1q, CC1 and CC2; no heredity.
ManifestationThis section has been translated automatically.
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Clinical featuresThis section has been translated automatically.
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Treatment of angioedema at the base of a C1-esterase inhibitor deficiency
Acute therapy/prophylaxis |
Therapy measures |
Acute therapy |
Analgesics for pain. |
AddC1-INH(500-1000 units in 10 ml physiological NaCl solution), slowly i.v., the injection can be repeated depending on the clinical picture. | |
If available, 500-2000 ml fresh plasma or "fresh frozen plasma". | |
If indicated, intubation, tracheotomy or criotomy. | |
Caution: Adrenalin, glucocorticoids and antihistamines are ineffective! | |
Preoperative short-term prophylaxis |
Danazol about 600 mg/day, 1-10 days before the procedure. |
Alternatively: EACA approx. 6 g/day, 2-3 days before the procedure. | |
Alternatively:C1-INH approx. 500-1000 units i.v., shortly before the procedure. | |
Long term prophylaxis |
Danazol at the beginning 600 mg/day, later reduction attempt to 200 mg/day or tranexamic acid 2-3 g/day (children: 1.5 g/day). |
Alternatively: EACA 6 g/day (children: 2 g/day). | |
Alternatively:C1-INH 500 units i.v., every 4-5 days. |
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Stanozolol;Disclaimer
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