|Anna Ciebiada, Marek L. Kowalski|
Angioedema and urticaria are among the most common diseases affecting approximately 15-20% of population. C1 inhibitor deficiency is rare as it is found in relatively small number among all angioedemas. C1 inhibitor is the only plasma protease inhibitor of activated C1 complement component and potent inhibitor of coagulation system and kininogenesis. Deficiency of the C1 inhibitor activity leads to nonphysiological activation of these systems. It may result in severe gastrointestinal symptoms and life - threatening laryngeal edema. Four forms of deficiency of the inhibitor with angioedema have been recognised. Two are hereditary (HAE) and two acquired. Most patients present I type of HAE with low plasma concentration of C1 inhibitor. Others (15%) have the II type with dysfunctional C1 inhibitor protein. In acquired form of disease first type is usually associated with lymphoproliferative diseases. In the second type, antibodies directed toward C1 inhibitor molecule have been described.
In this review the pathomechanisms of hereditary and acquired forms of C1 deficiency, the molecular genetics and the management of the disease are discussed.
Alergia Astma Immunol 2000; 5(1): 43-49
keywords: obrzęk naczynioruchowy, niedobór C1 inhibitora, dopełniacz, danazol, angioedema, C1 inhibitor deficiency, complement, danazol
pages: from 43 to 49
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