C1-esterase inhibitor (quantitative)

C1-esterase inhibitor (quantitative)

Clinical significance

C1-esterase inhibitor (C1INH) is a plasma glycoprotein, produced mainlyóin the liver. It belongs to the human plasma serine protease inhibitorów system. In the plasma of a healthy person it is present in a constant concentration, while in inflammation its concentration can increase. 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 (involved in blood clotting). Determination of C1 esterase inhibitor levels 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. Deficiencies of C1 esterase inhibitor can be congenital ( caused by genetic mutations) or acquired (observed in proliferative, autoimmune and connective tissue diseases).

Patient preparation

Material: Serum