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Factor XIII (fibrin stabilizing factor)
Last reviewed: 23.04.2024
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The reference value (norm) of activity of factor XIII in blood plasma is 100%.
Factor XIII (fibrin-stabilizing factor, fibrinase) refers to β 2 -glycoproteides. It is present in the vascular wall, platelets, erythrocytes, kidneys, lungs, muscles, placenta. In plasma it is in the form of a proenzyme, connected with fibrinogen.
Factor XIII under the influence of thrombin is converted to the active form of XIIIa, which during the formation of the fibrin clot ensures the formation of cross-linked forms of fibrin. Thrombi formed in the presence of fibrinase are very slowly lysed. With a decrease in the activity of factor XIII, the clots decay very quickly, even if the fibrinolytic activity of the blood is normal. If the wall of the blood vessel is damaged, factor XIII is involved in the process of aggregation and adhesion of blood platelets. It has been established that a decrease in fibrinase activity is accompanied by a decrease in adhesiveness and aggregation of platelets, and when the activity of fibrinase increases, these properties of platelets, on the contrary, increase.
Factor XIII characterizes the III phase of blood coagulation: a decrease or increase in fibrinase activity is considered as a factor of hemorrhagic or thrombotic risk.
Congenital deficiency of factor XIII
It is inherited by autosomal recessive type mainly by men. The first clinical sign of a deficiency of fibrinase in 80% of patients is a prolonged (during days, sometimes weeks) bleeding from the umbilical wound. The bleeding of the petechial type is characteristic. Possible hemorrhages in the brain. The slow healing of wounds is noted, post-operative hernia is often formed, fractures are poorly coalesced. All parameters in the coagulogram, in addition to reducing the concentration of factor XIII in the blood plasma, remain within normal limits.
In this disease, the coagulation of blood (according to the coagulogram) proceeds normally, but the fibrin clot is structurally unstable due to factor XIII deficiency. First of all draws attention to bleeding from the umbilical wound after the ablation or excision of the umbilical cord, and the bleeding may be moderate, but necessarily prolonged, within 2-5 weeks, so the deficiency of factor XIII is also called the "bleeding navel". In addition, long-term gastrointestinal and intracranial hemorrhages are possible.
The diagnosis is confirmed by determining the content of factor XIII (significant decrease). Other indicators of coagulogram, as well as the number of platelets are not changed.
[9], [10], [11], [12], [13], [14], [15], [16], [17], [18]
Treatment
Treatment is based on replacement therapy with preparations of the blood coagulation factor VIII: antihemophilic plasma from the calculation of 10-20 ml / kg intravenously drip or cryoprecipitate (1 dose contains 75 units of factor XIII), based on 0.3 doses / kg body weight of the child intravenously.
Acquired factor XIII deficiency
It is found in patients with avitaminosis C, radiation sickness, leukemia, cirrhosis, hepatitis, cancer with metastases to the liver, lymphoma, with DIC syndromes, in the transferred adrenalectomy, after taking anticoagulants of indirect action. Reduction of factor XIII in the blood for these diseases is due to a violation of its synthesis or expenditure in the process of DIC syndrome.
With long and poorly healing wounds and fractures, it is recommended to carry out a study of the activity of factor XIII, since in some cases such phenomena can be associated with its deficiency (factor XIII stimulates the development of fibroblasts).
The minimum haemostatic level of activity of factor XIII in the blood to stop bleeding is 1-2%, with a lower content of bleeding stopping without the introduction of a factor XIII patient is impossible.
In patients with thromboembolic complications, atherosclerosis, after surgery, childbirth, after administration of adrenaline, glucocorticosteroids, pituitrin, the activity of fibrinase is often increased.