Afibrinogenaemia

Afibrinogenemia is a rare hereditary disease characterized by the absence or sharp decrease in plasma fibrinogen, a protein responsible for blood clotting.

With afibrinogenemia, the level of fibrinogen in plasma is less than 0.2 g/l (with the norm being 1.5-4 g/l). This leads to disruption of the last stage of blood coagulation and the formation of an unstable fibrin clot.

The main symptoms of afibrinogenemia: increased bleeding (frequent and heavy bleeding), spontaneous hemorrhages in muscles, joints and internal organs. Heavy bleeding is typical during injuries, operations, and childbirth.

Diagnosis is based on a coagulological study (determining the level of fibrinogen).

Treatment consists of lifelong replacement therapy with fibrinogen or cryoprecipitate. For bleeding, transfusions of fresh frozen plasma are administered.

Afibrinogenemia is inherited, as a rule, in an autosomal recessive manner. The prognosis with timely diagnosis and adequate therapy is generally favorable.

Afibrinogenemia

Afibrinognaemia is a term that describes low levels of a plasma protein called fibrinogen. Fibrinogen is a protein that is responsible for blood clotting and participation in the formation of blood clots. It is necessary for the formation of red blood cells - platelets - and helps regulate blood pressure. Low levels of fibrinogen can cause serious problems such as bleeding and the risk of developing diseases such as heart attack or stroke.

**Causes and symptoms of afibrinogenomia**

The main causes of anifibrinogenia. The disease occurs as a congenital type of blood clotting disorder, caused by disturbances in the synthesis of fibrinogen protein and changes in its structure. Hereditary causes include: * genetic defects in the synthesis of fibrin-binding peptide, coagulogram AII, DAI, PAI-2, SF, * genetic disorder in the synthesis of biologically active glycosaminoglycans, such as glycosaminoglycan and hyaluronidase types 1, 2, * diseases of a genetic nature, for example, syndrome Ehlers-Danlos asulphahydral type, von Willebrand disease (Von Willebrand disease (Von Willebrand disease, type 3), * rare genetic syndromes and pathological variants. These include Carlom's syndrome, Munt - chondrocystic type I type, congenital hypercholesterolemia with atopic dermatitis, phocochromosomal disease with severe joint dysplasia and structural features of the skin of the face, dewlap, external auditory canal and pterygium. Congenital bleeding disorders can be caused by deficiencies of thrombin, fibrin peroxidase, factors II, V, VII, IX and X, as well as inhibitors of these blood factors. Other forms of afibrinogenemia occur in patients with hematological, oncological diseases and after organ and bone marrow transplantation. Among the hereditary or acquired causes of afibrinohemia (hereditary multifactorial origin). Hereditary thrombin deficiency in Debre-de Tollis syndrome, familial hypercoagulation of blood plasma thromboplastin due to a defect in factor V of the coagulation system. Disorders of the blood coagulation system occur during transfusion of platelet mass, megakaryocytes when using aggressive methods of stimulating platelet formation, rare use with insufficient synthesis of fibrinopolymer, changes in the properties of adhesion, aggregation, anomalies of the hemostatic system in other conditions.

Afibrinogenemia or afibrinogenemia is an abnormality of hemostasis in which there are no blood clots or a fibrin basis for hemocoagulation. The disease develops in various diseases with disruption of the hemostasis system. If abnormalities in blood clotting are not noticed in time and the condition is not treated, this can lead to serious complications - heart attack, stroke. When treating after diagnosis, various drugs and methods are used. Typically, women with the first stage are prescribed massage, exercise therapy, hirudotherapy, and cold therapy. The later the diagnosis is made, the more difficult it is to get rid of the problem.

Afibrinohemia cause

Atony is a disorder manifested by a delay in the formation of a coagulation plug. Hormonal imbalances, liver dysfunction, shock, and neurological damage lead to the development of the disease. The lack of a protective inner lining of blood vessels leads to their increased vulnerability and decreased tone. Red blood cells burst, causing internal hemorrhages. The situation is temporarily corrected by increased blood pressure; in advanced cases, multiple organ failure and infections develop. The disorder can also occur due to a primary defect in the composition of blood plasma - hypo-, afibrinogenic coagulopathy. With this pathology, coagulation factors and fibrin are produced.