Hemophilia is a family of hereditary hemorrhagic diathesis, manifested by recurrent, hard to stop the bleeding. The disease is exclusively for men; hemophilia is inherited from practically healthy women of the family, one of whose members suffered from hemophilia.
Slow blood clotting depends on shortage of blood plasma these patients antihemophilic globulin (factor VIII blood clotting)participating in education thromboplastin. The disease develops in childhood prolonged bleeding due to injury or operational intervention (for example, removal of a tooth). Even the bite of a mosquito can cause a large haematoma. Hemorrhage can be in the skin, muscles, joints (hemarthrosis). There are bleeding from the nose, at least from the gastrointestinal tract. The characteristic wave-like in the course of the disease (the periods of exacerbation and remission). The main sign of hemophilia - prolonged blood clotting (see). Instead of the normal 6-10 minutes on Bazarno it reaches 30 minutes, sometimes a few hours. Other signs of bleeding missing. The differential diagnosis should exclude pseudohemophilia is a rare disease that occurs more often in women, in which the clotting of blood is normal, but the bleeding time (see) lengthened.
Treatment. In the period of exacerbation strict bed rest, massive (500-1000 ml) re (8-12 hours) transfusion swiezegowno blood (daily storage) or plasma in 250 to 500 ml under the supervision of a physician. Recently used antihemophilic globulin or antihemophilic plasma. To stop external bleeding - local tampon with thrombin.
Prevention. Hemophilia patients should avoid injuries and to be under medical supervision.