Hemophilia in( a disease of kristmasa)
Hemophilia B is a hereditary hemorrhagic diathesis, characterized by a deficiency of the activity of factor IX of blood coagulation( plasma component of thromboplastin).Hemophilia B was first diagnosed as an independent disease in 1952. Like haemophilia A, the disease is transmitted through a recessive type of inheritance linked to a female X chromosome. The share of Christmass disease accounts for 8-15% of all cases of hemophilia.
By its manifestations, severity and complications of hemophilia B is identical to hemophilia A. These diseases can only be distinguished by laboratory data. This differentiation is important for proper substitution treatment with blood products.
Inhibitory forms of hemophilia B are less common than hemophilia A.
Treatment. Before the creation of highly active concentrates of factor IX blood clotting, hemophilia B was treated mainly with frozen or dry donor plasma. This method is widely used at the present time. With hemophilia B direct blood transfusions from the donor are not carried out. Intravenous injection of plasma is advisable, since the factor IX of blood coagulation is stable, well preserved in it and can be introduced into the body in a sufficiently large amount.
Factor IX is well preserved in frozen plasma( 90% activity is detected after 6 weeks and later) and other blood products. It lasts longer than factor VIII of blood coagulation, circulates in the blood after its intravenous administration. In this regard, the transfusion of plasma in hemophilia B can be done once a day.
Transfusion of plasma is carried out only intravenously struyno. A dose of 15-20 ml per 1 kg of body weight makes it possible to increase the level of factor IX of blood clotting by 10-15%, which is enough to stop acute hemorrhages in the joints and small spontaneous post-traumatic and postoperative bleeding, in particular after tooth extraction. Additional plasma injection at a dose of 10-15 ml / kg 12 hours after the first transfusion gives an increase in the level of this factor of coagulation by another 3-4%.Subsequent daily intravenous infusions of plasma maintain a concentration of factor IX in the blood plasma at a level of 7-14%.This is not enough to stop and prevent large bleeding, as well as for performing various cavitary, orthopedic or ENT operations.
Stop and prevent large bleeding with the help of concentrates of factor IX blood clotting, allowing to maintain a sufficient level of it in the plasma. Calculation of the dose of the drug is carried out individually.
Hepatitis virus infection of the liver, which can happen accidentally in the blood serum if it is not properly tested and is not fully tested before use, is an obstacle to the continuation of intravenous infusions. Impossible such infusions and with severe reactions to intravenous administration of blood products( some of them can be suppressed by the simultaneous administration of hormonal drugs).
Small bleeding( for example, with the removal of teeth) is often successfully prevented and stopped with e-aminocaproic acid.