DISEASES OF THE BLOOD SYSTEM : HAEMOPHILIA (HAEMOPHILIA) - etiology, pathogenesis, symptoms and course of, recognition, prediction and prevention
Etiology. rare family disease. Ill almost exclusively men; women, remaining for the most part healthy, transmit the disease to their offspring through the male line.
The pathogenesis. The main znachenie has a slow time of blood coagulation associated with the lack of production by the body of the patient with hemophilia enzyme thrombokinase. A role currently give insufficiency globulin fraction of serum proteins of the patient.
Symptoms. The disease begins in early childhood. A minor injury, a bruise, a cut, to cause sometimes uncontrollable profuse and sometimes fatal bleeding. On the skin due to the injury often have bruising, less frequent muscle hematoma. Bleeding from mucous membranes, most often from the mouth (gums), nasal. Tooth extraction often leads to persistent bleeding. There are fewer intestinal bleeding, bleeding from the kidneys and bladder. Especially characteristic hemarthrosis haemorrhage in the cavity of the various joints, often recurrent (cause persistent arthropathy). Haemorrhagic syndrome: abrupt deceleration of the clotting time of blood. Bleeding time is normal retraction blood clot is deviation from the norm, the number plates are the norm. Symptom Konchalovsky negative. Posthemorrhagic anemia type; depending on the volume of blood loss is a certain degree of gravity.
For chronic periodically, under the influence of trauma, bleeding coming from a variety of organs and tissues of the patient.
Recognition is placed after a careful study of history.
The prediction is doubtful, as even minor trauma leads to fatal blood loss. With age, the bleeding abated.
With the preventive purpose should encourage the patient to avoid injury, to prevent any surgery, even minor ones.
A particularly favorable effect is observed under the influence of repeated transfusions of citrate blood (citrate, because in vitro it is a stabilizer, a in vivo - coagulator) at least 400 - 600 ml of blood Transfusion should be prescribed and with the preventive purpose to warn may arise bleeding, once or twice a year at a dose of 300 - 500 ml is also Useful infusion serum and plasma in an amount of from 50 to 300 ml Should be poured or the same name with a group of recipient plasma and serum, or group AB. Sometimes been reported effect from the use of inogroupna blood - dose 5 - 10 ml re 1 every 5 days, the course is 4 to 5 intravenous infusion.