Hemophilia
(Internal Disease - Circulatory)
Hemophilia - a form of hemorrhagic diathesis, in which the tendency to bleeding due to an impaired blood coagulation due to deficiency of plasmic thromboplastin generation.
Etiology and pathogenesis. Hemophilia occurs in the form of congenital, rarely sporadic forms. Hemophiliacs suffer from male carriers of the disease are women.
A-form of Hemophilia associated with the lack anti-hemophilic globulin and occurs in 80-90% of patients with hemophilia.
B-form of Hemophilia is caused by lack of the plasmic thromboplastin component, occurs in 10-15% of patients with hemophilia.
C-form of Hemophilia characterized by lack of plasmic thromboplastin precursor, occurs in approximately 5% of patients with hemophilia.
D-form of Dhemophilia is the lack of Hageman’s factor.
Symptoms and flow. The main importance of hemorrhage in the form of large bruises on the soft tissue and is especially characteristic in the joints with the development of the strain them. Also observed bleeding from the mucous membranes of the mouth, nose, gastrointestinal tract, kidney, arising even for minor injuries, cuts, bruises. In most patients the disease manifests itself at an early age and goes heavily in childhood and adolescence. After 20 years of the disease is becoming calmer.
Recognition is based on laboratory studies, history of disease and typical hemarthrosis symptoms. The sharp slowdown in the blood clotting time, reduced consumption of prothrombin and modified thromboplastin test gives an opportunity to identify violations in the first phase of blood coagulation. Detection of haemophilia by direct determination of the deficit factor or by correlation of the ingredients of donated blood, or vice versa, mixing of donor blood and patient blood. Normalization or change the previously obtained parameters of blood coagulation provides an indication of violation in a link thrombocyte.
Treatment is symptomatic. The most effective are the repeated transfusion of fresh, no more than the daily prescription of blood or plasma by 400-500 ml of ink-jet method. In B-form hemophilia you can transfuse blood and plasma for longer periods of storage, because the plasma thromboplastin component in contrast anti-hemophilic globulin is stable in storage. In order to improve hemostasis prescribe corticosteroids (prednisolone 25-30 mg / day for 2-4 weeks). Anti-hemophilic globulin, isolated in pure form, is the most effective treatment for hemophilia.
By bleeding surface (mucosa of the nose, mouth) should have put hemostatic cotton, sponge, gauze cloth and thrombin or plasma.
When hemarthrosis immobilized limb, apply cold (ice) and a pressure bandage, and further local radiotherapy.
Various surgical interventions in hemophiliacs, including tooth extraction should be carried out in institutions with the use of various preventive measures in the preoperative period, at the time and after the operation. Most of these activities is the use of various hemostatic means - blood transfusion and plasma.
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