C1-esterase inhibitor (activity)

C1-esterase inhibitor (activity)

Clinical significance

C1-esterase inhibitor (C1INH) is a plasma glycoprotein, produced mainlyóin the liver. It belongs to the plasma serine protease inhibitorów system. Under physiological conditions, it blocks the classical pathway of complement system activation by inactivating the C1s and C1r components. It also inhibits the activity of factor XIIa and kallikrein. C1 inhibitor prevents excessive activation of the complement system, as well as the blood coagulation and fibrinolysis system. Deficiencyór of this factor leads to excessive activation of the aforementioned systemsów and the occurrence of pathological changes in the body. Determination of C1 esterase inhibitor is used to detect congenital or acquired deficiency of this inhibitor. A rare condition caused by C1 esterase inhibitor deficiency is acquired angioedema, manifested by recurrent angioedema. The edema is most often localized to the skin or mucous membranes of the górne dróg respiratory and gastrointestinal tracts. Laryngeal localization of edema is an absolute life-threatening situation. C1 esterase inhibitor deficiencies can be congenital (caused by genetic mutations) or acquired (observed in proliferative, autoimmune and connective tissue diseases).

Patient preparation

Material: citrated plasma