Fibrin Stabilizing Factor Deficiency

Deficiency of fibrin-stabilizing factor (factor XIII) is a rare hereditary disease of the hemostatic system, characterized by increased bleeding due to disruption of the process of fibrin stabilization.

Factor XIII is responsible for the formation of cross-links between fibrin molecules, which leads to the formation of a strong fibrin clot. With factor XIII deficiency, the fibrin clot remains loose and unstable, which leads to increased blood loss even with minor vascular damage.

Clinically, factor XIII deficiency is manifested by hemorrhagic diathesis - increased bleeding of the skin and mucous membranes, nasal, gingival, gastrointestinal, and uterine bleeding. Subcutaneous hematomas and hemorrhages into muscles and internal organs are characteristic. The disease can manifest immediately after birth or in childhood. Diagnosis is based on determining the activity of factor XIII in plasma.

Treatment consists of lifelong replacement therapy with factor XIII concentrates to prevent bleeding. The prognosis with adequate therapy is favorable. Thus, factor XIII deficiency is a severe hereditary disease of the hemostatic system, requiring lifelong replacement therapy to prevent life-threatening bleeding.

Fibrin-stabilizing factor deficiency or Glanzmann's thrombasthenia

Fibrinogen deficiency is a hereditary disease of the blood coagulation system, accompanied by a tendency to form blood clots, poor blood supply to tissues and wounds. They have the ability to decompose quickly. As a result, a substrate is created for the growth of microorganisms. With inflammation or infection of the wound, there is a possibility of carbuncle formation, followed by damage to other tissues and internal organs.

The disease can be acquired or hereditary. Hypofibrinogenemia develops as a result of a deficiency of fibrinogenic proteins in the prenatal period or during life. The compensatory mechanism is to reduce the formation of coagulation factors (prothrombin, prothrombinase), fibrin, and proteoglycans. If a patient is diagnosed with a deficiency of three or more factors, then there is a risk of developing disseminated intravascular coagulation syndrome.

**Formation of the disease** If there is a violation