Definition

Blood clotting factors are twelve, all very important. When one of the twelve factors not adequately perform their work, others can not work together to form a clot and inhibit bleeding. It is true that hemophilia is a disorder of the clotting of blood, hereditary, by which their training takes longer to generate than normal and people who suffer lack the ability to stop bleeding. The peculiar name of “Hemophilia” means “Love of blood.” The romantic memory of the history of Russian monarchs Alexandra and Czar Nicholas II and the condition of his son, Alexis, contributed further to define hemophilia as a disease of kings ” but it is known that one inevitably affects of 10 thousand men , without distinction of race or social strata. It is passed from father to son, so it is a sex-linked inherited abnormality. There are several types of hemophilia, but the most common and best known include: Hemophilia A – factor VIII deficiency. Hemophilia B – factor IX deficiency. A hemophiliac who suffer from hemophilia A or B does not bleed faster than others, but they bleed longer than usual because the process of coagulation is not normal.

Causes

Hemophilia types A and B is an inherited disease that is transmitted by a gene located on chromosome X. Females have two X chromosomes, while males have one X chromosome and one Y chromosome A female carrier of hemophilia has the hemophilia gene on one of her X chromosomes, and there is a 50 percent chance that she may pass the defective gene to their male offspring. Men with hemophilia do not pass the gene to their sons. Generally, women are carriers of hemophilia A but do not suffer. Hemophilia B, also transmitted by sex-linked inheritance is less common than A. In approximately one third of hemophilia cases, there is no family history of the disease. These cases are due to a new or spontaneous development of the defective gene in women.

Symptoms

The most common symptoms of hemophilia is excessive, uncontrollable bleeding because of the missing clotting factor or is at low levels in the blood. The severity of symptoms depends on the degree of deficiency of clotting factor. Hemorrhages are characteristic of this disease and may occur without apparent cause. The most common symptoms of hemophilia are: Bruises can occur from blows little overwhelming, which can generate a large hematoma (a collection of blood under the skin, causing swelling). Spontaneous bleeding the tendency to bleed from the nose, mouth and gums with minor trauma or by regular brushing teeth, is often a sign of hemophilia. Bleeding into a joint Hemarthrosis (bleeding into a joint) can cause pain, immobility, and eventually deformity if not treated the right way. This is the most common complications due to hemophilia bleeding. These joint bleeds can lead to chronic arthritis pain, deformity and crippling with repeated occurrences. Bleeding into the muscles Bleeding into the muscles can cause swelling, pain and redness. Swelling from excessive blood in these areas can cause increased pressure on tissues and nerves in the area, causing damage and / or permanent deformation. Bleeding from injury or cerebral hemorrhage the injury or spontaneous bleeding in the brain is the most common cause of death in children with hemophilia and the most serious bleeding complication. Other sources of bleeding Blood in the urine or stool may also be a symptom of hemophilia. The wounds, dental extractions and surgeries cause prolonged bleeding.

Diagnosis

In addition to a complete medical history and physical examination is necessary to conduct a series of laboratory tests to evaluate the clotting process, including the clotting time, and also DNA testing. Also it determined in plasma levels of Factor VIII and IX to evaluate the severity of the deficiency.

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