Hemophilia is a hereditary disease characterized by increased bleeding.
In hemophilia, the internal mechanism of blood coagulation is impaired as a result of deficiency of VIII, IX, and XI coagulation factors. Hemophilia A is isolated (coagulation factor VIII deficiency), hemophilia B (coagulation factor IX deficiency), transmitted by the recessive sex-linked inheritance type (men are ill, women are carriers of the hemophilia gene) and hemophilia C (coagulation factor XI failure) - A rare disease with an autosomal dominant mode of inheritance (men and women are ill). The severity of the disease depends on the degree of deficiency of the corresponding coagulation factor.
It is clinically characterized by bleeding that occurs in early childhood (after umbilical cord ligation, teething). There are heavy bleeding after any, even minor injuries and surgical interventions; recurring acute hemorrhages in the abdominal organs, as well as in the joints of the limbs, mainly knee and ankle, which result in contractures, muscle atrophy , impaired function of the limbs. Subcutaneous, intramuscular, subperiosteal hematomas are observed, the appearance of blood in the urine (hematuria) is possible. Massive hematomas can cause compression of blood vessels and nerves and lead to the development of paralysis, ischemia and organ necrosis. Possible long-term bleeding from the mucous membranes of the nose, gums, less gastrointestinal tract.
Hemophilia should be suspected in males with increased bleeding that occurred in early childhood. The diagnosis is established on the basis of laboratory research methods (determination of blood coagulation time, the content of the corresponding blood coagulation factor, etc.).
During the period of bleeding, hemotherapy is prescribed, which must be differentiated: for hemophilia A, cryoprecipitate (purified concentrate of coagulation factor VIII), fresh or antihemophilic plasma transfusions are used; in hemophilia B and C, plasma, native plasma concentrate and PPSB preparation (complex II, VII, IX and X blood clotting factors) are used. If necessary, surgical interventions in patients with hemophilia are carried out in specialized medical institutions after filling the content of the missing coagulation factor. In the acute period of hemarthrosis, general hemotherapy is performed, against the background of which, according to indications, joint puncture and removal of spilled blood can be performed: The following shows physiotherapy treatment (mud therapy and water procedures, physiotherapy exercises).
Prevention of bleeding is reduced to the prevention of injuries, the inadmissibility of surgical interventions without prior preparation. In severe cases with hemophilia A, cryoprecipitate is prescribed prophylactically outside periods of exacerbations.