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Hemophilia: Severe Hemophilia
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Hemophilia is a lifelong bleeding disorder that prevents blood from clotting properly. People with hemophilia do not have enough clotting factor, a protein in blood that controls bleeding. Bleeding into joints and muscles can cause stiffness, pain, severe joint damage, disability, and sometimes death. People with more severe hemophilia require larger and more frequent doses of blood clotting factors. For more information about the WFH, hemophilia and other bleeding disorders go to http://www.wfh.org/.
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Hemophilia is a bleeding disorder that slows the blood clotting process. People with this condition often experience prolonged bleeding or oozing following an injury, surgery, or having a tooth pulled. In severe cases of hemophilia, heavy bleeding occurs after minor trauma or even in the absence of injury (spontaneous bleeding). Serious complications can result from bleeding into the joints, muscles, brain, or other internal organs. Milder forms of hemophilia do not involve spontaneous bleeding, and the condition may only become apparent when abnormal bleeding occurs following surgery or a serious injury.
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Severe Hemophilia: Patients with hemophilia have varying severities of clotting factor deficiency. If the person's deficiency clotting factor is less than one percent of the normal, the condition is generally termed "severe hemophilia." These patients frequently have spontaneous joint bleeds and are prone to develop joint inflammation and arthritis.
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Hemophilia A and hemophilia B are caused by an inherited defect in a pair of chromosomes. The defect affects how much clotting factor a person will produce and how the factor will function. Hemophilia is mild when the clotting factor functions are close to normal and the amount of clotting factor is almost normal. The less normal the function and amount of clotting factor, the more severe the hemophilia.
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Hemophilia A... known as factor VIII deficiency, is the cause of about 80% of cases. Hemophilia B, which makes up the majority of the remaining 20% of cases, is a deficiency of factor IX. Patients are classified as mild, moderate, or severe, based on the amount of factor present in the blood.
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Hemophilia is usually discovered when an injury initiates bleeding and the bleeding will not stop. In very young children, spontaneous musculoskeletal bleeding may occur around the time the child begins to walk; these episodes may be the first sign of hemophilia. In some children, a simple surgical procedure, such as a tooth extraction or injection, may present with uncontrolled bleeding. Any signs of deep bruises or the presence of prolonged bleeding after a bump or an injury that breaks the skin should be reported to a physician or emergency service immediately. Bleeding under the skin (hematoma), which looks like a severe bruise, should ... be reported and medical care sought immediately.
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Severe Hemophilia