Hereditary Deficiency Of Blood Coagulation Factor Vii – Hypoproconvertinamia Republican Specialized Scientific And Practical Medical Center Of Hematology Of The Ministry Of Health Of The Republic Of Uzbekistan.

Abstract

Hypoproconvertinemia is a familial form of hemorrhagic diathesis caused by a deficiency of proconvertin (factor VII). Factor VII is a protein that migrates during electrophoresis between alpha and beta globulins. It is stable during storage, is not utilized in the process of blood coagulation, and therefore is found in both plasma and serum. Synthesized in the liver. The half-life in vivo is 4-6 hours. The disease is associated with a pathological gene located on the autosome and responsible for insufficient synthesis of proconvertin. Inheritance of proconvertin deficiency is recessive and not related to gender. Hemorrhagic diathesis caused by the presence of pathological factor VII, with structural changes in its molecule, is described. With hypoproconvertinemia of any etiology, the second phase of blood coagulation, the formation of thrombin, is disrupted, since factor VII takes part in the external mechanism of blood coagulation, contributing (together with tissue thromboplastin, factor V and calcium ions) to the activation of factor X, which converts prothrombin into thrombin.