Thrombotic disease due to protein C deficiency
Protein C is a protein in plasma that enters into the cascade of biochemical events leading to the formation of a clot. Deficiency of protein C results in thrombotic (clotting) disease and excess platelets with recurrent thrombophlebitis (inflammation of the vein that occurs when a clot forms). The clot can break loose and travel through the blood stream (thromboembolism) to the lungs causing a pulmonary embolism, brain causing a stroke (cerebrovascular accident), heart causing an early heart attack, skin causing what in the newborn is called neonatal purpura fulminans, the adrenal gland causing hemorrhage with abdominal pain, abnormally low blood pressure (hypotension), and salt loss. Protein C deficiency is due to possession of one gene (heterozygosity) in chromosome band 2q13-14. The possession of two such genes (homozygosity) is usually lethal.
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A substance made by platelets that causes blood clotting and constriction of blood vessels. It also encourages platelet aggregation. There are two thromboxanes. Thromboxane A2 (TXA2) is active but is very unstable and has a half-life of only 30 seconds before it undergoes hydrolysis to form thromboxane B2 (TXB2) which is inactive. The thromboxanes are […]
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The active type of thromboxane. Abbreviated TXA2.
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The inactive product of thromboxane. Abbreviated TXB2
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