17 April – World Hemophilia Day

According to the World Health Organization, today there are more than 400 outside. person with hemophilia. Hemophilia is a rare inherited disease, characterized by a disorder of blood clotting processes. The disease has been known since ancient times, the first such cases were mentioned in the 2nd century BC. The term "hemophilia" was first proposed in 1828 year and comes from the Greek words “hemo” and “philia”, what does "tendency to bleed" mean?.

Hemophilia used to be called "liquid blood", "royal disease" or "Victorian disease". Queen Victoria of England is considered the first known carrier of this disease.. According to her family, this disease was transmitted to the reigning families of Germany, Spain and Russia. In the Russian Empire, the son of the last Tsar Nicholas II suffered from this disease..

The disease manifests itself in a decrease in blood clotting ability, this could make a serious bruise or cut dangerous. Hemorrhage may occur several hours after traumatic exposure.

The clinical picture of patients with hemophilia is characterized by an increased tendency to lose blood. Manifestations of the disease are bleeding of different locations: formation of large bruises or large hematomas after minor pressure or impact, prolonged bleeding after blood tests, more frequent nosebleeds 15 minutes; hemarthrosis (accumulation of blood in joints); intermuscular hematomas, spontaneous occurrence of hemorrhagic exanthems (rash), gastrointestinal bleeding, hematuria, hemorrhages in the brain or internal organs.

AT 70% cases of hemophilia are characterized by severe course, steadily progresses and leads to early disability.

Hemophilia varies depending on the type of clotting factor deficiency: Hemophilia A is characterized by the absence of coagulation factor VIII (antihemophilic globulin), for hemophilia B – factor IX deficiency (plasma component thromboplastin, of the Christmas factor), for hemophilia C – coagulation factor XI deficiency (thromboplastin precursor). Besides, According to the severity of the disease, mild, moderate and severe forms. With a mild degree, the activity of the concentration of coagulation factors in the blood reaches 5-30%, with average – 1-5%, in severe form less 1%.

Genes, causing the development of hemophilia, linked to the sex X chromosome and transmitted according to a recessive mode of inheritance. This pathology is diagnosed only in males.. Women act as carriers of the hemophilia gene and pass it on to their sons..

Today, hemophilia still occurs throughout the world.. It is impossible to recover from it, You can only improve the patient's health with drugs, increasing blood clotting. The main thing in the treatment of hemophilia is replacement therapy, which involves regular administration of deficient blood clotting factors.

In the study of hemophilia, modern science places much more emphasis on genetics. It is now possible to detect it even at the stage of intrauterine development with the help of special genetic examinations. For women and men, who are carriers of the defective hemophilia gene, It is recommended to undergo examination in the early stages of planning a child.

Preventive measures among people, patients with hemophilia, will be: protective mode to avoid various injuries, dispensary observation by medical specialists (pediatrician, hematologist, pediatric dentist, pediatric orthopedist, etc.), dispensary observation of a specialized institution, maintaining a healthy lifestyle, reduction of physical activity. Swimming will have a healing effect on the body., physical therapy and balanced nutrition.

The general purpose of the events held on this day is to, to attract public attention to the problems of hemophilia and do everything possible to improve the quality of medical care, who turns out to be sick with this incurable genetic disease.

17 April 2024 years from 14.00 to 15.00 hours in the State Institution "Republican Scientific and Practical Center for Radiation Medicine and Human Ecology" a direct line will work on the number 8(0232)38-99-35.

Tolkacheva Ekaterina Alexandrovna,

doctor for medical prevention of the state public health department institution "Gomel Regional Hygiene Center, Epidemiology and Public Health "