Hemophilia in children - congenital blood pathology

Hemophilia in children - hereditary hemorrhagic diathesis. The development of hemophilia is caused by congenital insufficiency in the blood of factors taking part in the formation of plasma thromboplastin, a substance necessary for the rapid folding of blood in trauma.

The disease occurs mainly in men. It is passed on to the grandchildren of the patient from clinically healthy daughters. There are three varieties of this pathology.

1. Hemophilia A is the most common form of the disease. It is caused by insufficiency in the blood of a patient with antihemophilic globulin.

2. Hemophilia B is caused by a deficiency in the blood of the plasma component of thromboplastin.

3. Hemophilia C is caused by the absence in the blood of the plasma precursor thromboplastin.

As a result of insufficiency in the blood of one of the named factors, its coagulability is violated. Such blood collected in a test tube does not turn off for a long time, and the uniform elements gradually settle to the bottom.

This disease is characterized by a hematomic type of bleeding. The main complaint of patients - the tendency to bleeding, which manifests itself from the first years of life. In anamnesis, hemophilia in children is manifested by umbilical cord bleeding, hematoma at birth, prolonged bleeding with teeth eruption, and skin wounds. Later, swelling and soreness of the joints occur with minor injuries. Adults often develop painful common hemorrhages in the subcutaneous basis, muscle hematomas. Less bleeding wounds with crushed edges. This is due to the fact that the deficiency of plasma thromboplastin is partially compensated by a significant secretion of the tissue.

There are bleeding from the mucous membranes of the mouth, nose, digestive tract, kidneys, bronchi. Possible hemorrhages in the brain or meninges. Threatening the life of the patient retroperitoneal hematomas, hemorrhages in the root of the tongue, the neck area, pharynx. Severe bleeding can lead to any medical manipulation, for example, tooth extraction, various injections, and even more surgical interventions. A feature of hemorrhagic syndrome with damage to small vessels is the later appearance of bleeding. It can develop a few hours after the injury.

Typical symptoms that hemophilia manifests in children are hemorrhages in the joints, called hemarthrosis. Most often hemarthrosis of the knee and elbow joints develop. They are very painful, accompanied by an increase in temperature. Repeated hemarthroses lead to the development of contractures.

Hemophilia is a serious blood disease. The most severe hemophilia in children and adolescents. In the past, most patients died from bleeding or hemorrhage to internal organs before the age of twenty. Complications of the disease include severe anemia and joint damage - hemarthrosis and contractures that develop against a background of hemarthrosis.

If hemophilia is diagnosed, treatment should be performed from the first months of the patient's life. The main method of treatment of bleeding is urgent replacement therapy. To treat hemophilia, blood products are used, in which factors of blood clotting are saved longer than in fresh blood. It is an antihemophilic plasma, cryoprecipitate or concentrate of the blood clotting factor. In severe cases, direct blood transfusion from a donor is sometimes used. In hemophilia B and C, enter native and dry plasma, as well as a complex preparation containing several factors of blood clotting. Of the other haemostatic drugs, aminocaproic acid and fibrinogen are used. Treatment of hemophilia prevents the development of complications of diseases, significantly improves the quality and life expectancy of the patient.