Hemophilia B

Hemophilia B – a disease of the Christmas family

**Hemophilia is a rare genetic disease** that affects the blood clotting process. The immune system begins to fight not only against infections and viruses, but also against healthy red blood cells. According to the World Health Organization (WHO), there are approximately **38,000

Hemophilia B

Hemophilia is a chronic disease characterized by a bleeding disorder due to factor VIII deficiency. It reduces the body's ability to clot blood during bleeding, which can lead to dangerous consequences. Hemophilia is also called Christ's disease, or winter's disease, as it occurs during the cold season. Most often it affects men. Based on the type of transmission, there are hereditary (autosomal recessive) and acquired types. Carriers can be people who do not suffer from hemorrhagic diathesis. Transfer of the pathological gene to them is possible transplacentally from the mother at birth. The female is sometimes the carrier. The disease can also manifest itself in offspring (in 60-80% of cases). A genetic defect encodes the production of a clotting factor - hemophilia B. Consequently, it is reduced and prevents the formation of a proper blood clot at the site of vessel damage. Factor VIII synthesis deficiency occurs for various reasons. The main ones are a mutation in the factor VIII gene and the effect of a negative external factor on the receptors of molecules in the inner layer of blood vessels. The second type of disease is idiopathic hemophilia. The basis of genetic damage is the development of mutations in individual cells of the circulatory system. Its carriers may suffer from another disease – scleroderma. Induced form - occurs as a result of exposure to the drug "Gravistat". It is characterized by increased blood viscosity. The production of coagulation factor is reduced under the influence of medications - for example, anticoagulants, diuretics, antipyretics, or as a result of chronic diseases affecting the liver (for example, glomerulonephritis, cirrhosis). Severe form (fulminant) - occurred in the patient. who was given 4 grams of calcium gluconate with a plasma infusion, which contains an insufficient amount of enzyme. The acute type (progressive) develops in the younger generation of people who are injected with the whole blood of their father or older brothers for emergency surgery or