hemophilia b…

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x-linked recessive genetic defects, how boys, x-linked recessive genetic defects, how girls, x-linked recessive genetic defects, blood cells, blood clots, christmas disease, factor ix hemophilia, hemophilia b, hereditary blood coagulation disorder, a deficiency of a blood plasma protein, factor ix, clotting property of blood, hemophilia, hereditary bleeding disorder of specific blood clotting factors, several types of hemophilia, hemophilia a, b, hemophilia a is 7 times more common than hemophilia b, hemophilia b, result of a deficiency of clotting factor ix, disorder, an inherited x-linked recessive trait, the defective gene located on the x chromosome, disorder occurs primarily in males, females carry two copies, the x chromosome, factor ix gene on one chromosome is defective, compensate, males, carry only one x chromosome, factor ix gene on that chromosome is defective, have the disease, females, one defective factor ix gene, carriers of this trait, fifty percent, the male offspring of female carriers, have the disease, 50% of female offspring, carriers, all female children of a male hemophiliac, carriers, the trait, severity of symptoms, severe forms, become apparent early on, bleeding, hallmark, though not always, occurs, an infant is circumcised, additional bleeding manifestations make appearance, the infant becomes mobile, mild cases, go unnoticed, later in life, occur in response to surgery, trauma, internal bleeding, occur anywhere, bleeding into joints is common, risk factors, a family history of bleeding, male, hemophilia b occurs in, 1 out of 32,000 men, nosebleeds, nosebleed, bruising, spontaneous bleeding, bleeding into joints, associated pain, swelling, gastrointestinal tract, urinary tract hemorrhage, blood, in the urine, stool, prolonged bleeding from cuts, tooth extraction, surgery, excessive bleeding following circumcision, coagulation studies involving many tests, performed person tested, first one, in the family to have a bleeding disorder, once the defect has been identified, family members, need less testing to diagnose the disorder, ptt is prolonged, prothrombin time is normal, bleeding time is normal, fibrinogen level is normal, serum factor ix is reduced, standard treatment is infusion of factor ix concentrates to replace the defective clotting factor, amount infused depends upon the severity of bleeding, site, the bleeding, the patient, hepatitis b vaccine, individuals, hemophilia b, at increased risk of developing hepatitis, due to exposure to blood products, prevent a bleeding crisis, hemophilia, families, taught to administer factor ix concentrates at home, first signs of bleeding, severe forms, need regular prophylactic infusions, depending on the severity, factor ix concentrate given, prior to dental extractions, surgery, prevent bleeding, hemophilia support group, outcome, hemophilia, able to lead relatively normal lives, a small percentage of people develop inhibitors of factor ix, die from loss of blood, chronic joint deformities, recurrent bleeding, joint, managed, orthopedic specialist, recurrent transfusions, expose the individual to hiv, hepatitis, prior to 1985, blood screening procedures were improved, detecting the hiv virus, intracerebral hemorrhage, deep intracerebral hemorrhage, lobar intracerebral hemorrhage, thrombosis, occur following use of factor ix concentrate, symptoms of a bleeding disorder develop, screening, a family member has been diagnosed, hemophilia b, have hemophilia b, plan to have children, genetic counseling advised, female carriers, identified by testing.