Abstract
C1-inhibitor (C1-INH) is the only known inhibitor of the classical pathway of complement system and the most important inhibitor of factor XII-mediated contact activation. The genetic deficiency of C1-INH (hereditary angioedema) causes acute edema formation which is in most cases locally restricted to the upper extremities, neck, and face. Acute onset of an attack often results in life-threatening events such as laryngeal edema, which is resistant to treatment with corticosteroids.
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Eisele, B., Delvos, U. (1994). From Localized Angioedema to Generalized Capillary Leak Syndrome: Evidence for a Pivotal Role of C1-Inhibitor in Septic Shock-like Syndromes. In: Reinhart, K., Eyrich, K., Sprung, C. (eds) Sepsis. Update in Intensive Care and Emergency Medicine, vol 18. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-85036-3_39
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