2. diagnosis and treatment of hemophilia
Hemophilia - a hereditary origin of a disease in which blood clotting occurs.The disease is characterized by slowing down the process of blood clotting and increase bleeding time.
Most disease hemophilia affects boys, but carriers of the disease are women.
blood coagulation, or hemostasis, - quite an important protective reaction of the human body, which is activated by bleeding or vascular damage.She provided special substances (clotting factors) and small blood cells (platelets).Only if there is a sufficient number of platelets and clotting factors, it is possible to stop the bleeding.With a lack of a blood clotting factor hemostasis process is impossible, and there is hemophilia.
Depending on the clotting factor that is missing in the blood, there are three forms of the disease:
carriers of hemophilia gene disease - women who usually does not exist signs of the disease.The sons of these women have a 50% risk of developing the disease.
hemophilia disease can occur at any age.But more often the first symptoms of the disease occur in children.Hemophilia in children can manifest itself in the first year of life while teething.Even a newborn baby can be bleeding from the umbilical cord bandaged, bleeding in the skin or subcutaneous tissue, cephalohematoma (bleeding under the periosteum of the skull bones).
But often the signs of hemophilia occur in children after the first year of life.At this age children are active, they begin to walk, which leads to increased risk of injury.Patients with hemophilia often have extensive hemorrhage, which can be spread over large areas.
Another symptom of hemophilia in children and adults are hematoma - a painful, deep intermuscular bleeding.They are resorbed slowly.The blood that has been poured, the liquid remains for a long time, which contributes to its light penetration in tissues and along the fascia (connective tissue membranes that cover organs, nerves, blood vessels, muscles).In some cases, the disease hemophilia bruises are very large.At the same time, they can squeeze the large arteries, or peripheral nerve trunks, causing gangrene and paralysis.These processes are accompanied by severe pain.
signs of the disease are quite prolonged bleeding from mucosal tissues of the mouth, gums, nose, sometimes the kidneys and gastrointestinal tract.Very dangerous is bleeding from the mucous membrane of the larynx.It contributes to the development of acute airway obstruction.
beginning of serious bleeding in patients with hemophilia may contribute to any medical procedure, particularly intramuscular injection.Tonsillectomy (complete removal of the tonsils) and tooth extraction can cause prolonged bleeding.In some cases, there is bleeding in the brain and meninges, leading to a serious threat to the health and life of the patient.
characteristic feature of hemophilia is hemarthrosis - bleeding into the joints.It is characterized by severe pain and a significant increase in body temperature.Usually affects the large joints - ankle, elbow, knee, hip.When repetitive hemorrhages in patients with hemophilia in the joint capsule and cartilage forming fibrous clots.Over time, they germinate connective tissue, joint cavity loses its function, and in patients with hemophilia develop ankylosis (complete joint immobility).
In this disease are often hemorrhage into the bone tissue, which promote bone decalcification, aseptic necrosis (necrosis) of tissue.
characteristic for hemophilia later delayed bleeding.It does not appear immediately after the injury, and 5-10 hours or more.Time of occurrence depends on the severity of disease and injury rate.
Diagnostics of hemophilia is based on family history, the establishment of clinical symptoms and laboratory findings.
Laboratory studies include holding thrombin and prothrombin test.These tests are conducted to determine the clotting time.
In order to establish the form of hemophilia patients undergoing corrective sample in autokoagulogramme.
If a pregnant woman or one of her family members suffer from the disease, carried out genetic diagnosis of the disease.Genetic Diagnosis consists of a direct genetic analysis, indirect gene analysis and prenatal diagnosis.
main treatment for hemophilia is replacement therapy.It consists in applying VIIІ and concentrates of coagulation factors IX.The required dose of the concentrate depends on the level of these factors in a patient with hemophilia and the type of bleeding.
the treatment of hemophilia A is usually used a concentrated preparation of antihemophilic globulin (cryoprecipitate).It is prepared from fresh frozen human blood plasma.
In the treatment of hemophilia B using a complex tool PPSB, which contains prothrombin factors proconvertin, plasma thromboplastin component, Stuart-Prauera.
antihemophilic drugs are administered immediately after their re-entry, intravenously.
In case of bleeding in the joints in the treatment of hemophilia is a must complete patient comfort.In this case the patient is carried out short-term immobilization of a limb in the desired position.Massive bleeding requires urgent joint puncture with aspiration (suction) of blood and administration of hydrocortisone into the joint cavity.
The period of rehabilitation treatment for hemophilia is carried out using physical therapy, therapeutic exercises and special massage.
hemophilia treatment in children and adults aimed at reducing symptoms and preventing complications.The cure of this disease in our time is not possible.
great importance is the prevention of hemophilia, which is to prevent bleeding.In hemophilia in children parents need to teach them the correct way of life in which you need to avoid physical stress and traumatic sports.Medicaments hemophiliacs administered orally or intravenously just as intramuscular injection can cause bleeding.Routine inspection by a dentist should be done every three months to avoid having to remove the tooth.
This article is available exclusively in the educational purposes and is not research material or professional medical advice.
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