Emergency Therapy in Hereditary Angioedema due to C1-INH-Deficiency
Hereditary angioedema (HAE)
- Frequent symptoms of HAE due to (proven) C1-INH-deficiency are periodic skin swelling (oedema) in the face, arms, legs or genital region as well as swelling in the gastro-intestinal region that are accompanied by colic-like pain and sometimes circulatory disorders, diarrhoea and vomiting.
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Oedema in the upper respiratory tract (including swelling around the inner throat and larynx) is rare
(approximately 1% of all cases) but potentially life-threatening. - Facial swellings can develop into oedema of the upper respiratory tract (palate, throat, larynx), which may then become life-threatening.
Attention!
Oedema in the upper respiratory tract is a medical emergency!
Imprint
This information was compiled by:
Competency Centre for HAE in accordance with Section 116b, Department of Paediatrics and Internal Medicine, Dr W. Kreuz,
Dr E. Aygören-Pürsün, Dr I. Martinez-Saguer, Johann Wolfgang Goethe University Hospital, Frankfurt am Main
www.klinik.uni-frankfurt.de
HAE Centre Mainz, Department of Dermatology, Prof. Dr K. Bork, Dr P. Staubach-Renz, Johannes Gutenberg University Hospital, Mainz
www.hautklinik-mainz.de
Applicability of this emergency therapy recommendation: Federal Republic of Germany
Status: July 2011
More information on HAE:
www.schwellungen.de www.angioedema.de