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Hemophilia: Bleeding
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Hemophilia types A and B are inherited diseases passed on to children from a gene located on the X chromosome. Females have two X chromosomes, while males have one X and one Y chromosome. A female carrier of hemophilia has the hemophilia gene on one of her X chromosomes. When a hemophilia carrier female is pregnant, there is a 50/50 chance that the hemophilia gene will be passed on. If the gene is passed on to a son, he will have the disease. If the gene is passed on to a daughter, she will be a carrier. If the father has hemophilia but the mother does not carry the hemophilia gene, then none of the sons will have hemophilia disease, but all of the daughters will be carriers.
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Hemophilia is a genetic disease. A defective gene located on the X chromosome causes hemophilia. Men who have the defective gene will have hemophilia since they possess only one X chromosome. Since females have two X chromosomes, they will develop hemophilia only if both chromosomes carry the defective gene. However, females are carriers of this trait and will pass on the defective chromosome to fifty percent of their offspring. Approximately one fifth of hemophilia B cases and one third of hemophila A cases occur from a spontaneous gene mutation with no family history of the disorder.
A young Queen Victoria passed hemophilia on to many of her descendants. Hemophilia is quite rare only about 1 in every 10,000 births (or 1 in 5,000 male births) for hemophila A and 1 in 50,000 births for hemophila B.[5] About 18,000 people in the United States have hemophilia. Each year in the US, about 400 babies are born with the disorder. Hemophilia usually occurs in males and less often in females.[6] It is estimated that about 2500 Canadians have hemophilia A and about 500 Canadians have hemophila B. [7]
Hemophilia A affects between one in 5,000 to one in 10,000 males in most populations. Hemophilia B occurs in one in 40,000 to 50,000. The prevalence of hemophilia is estimated to be 13.4 cases per 100,000 U.S. males (10.5 hemophilia A and 2.9 hemophilia B). By race/ethnicity, the prevalence is 13.2 cases in 100,000 among white males, 11.0 among African-American males, and 11.5 among Hispanic males. Hemophilia C occurs primarily among individuals of Jewish descent.
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Hemophilia A affects one in 10,000 males and can cause uncontrolled bleeding that can lead to disability or death. It is commonly been associated with British and Russian nobility but affects many others. Cures for hemophilia have ranged from faith healing by the Russian monk Rasputin in the early 20th century to clinical trials using gene therapy, but none of these approaches have amounted to a cure.
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Hemophilia occurs in one out of every 7,500 males. Of these, about 85 percent of cases are Factor VIII (Hemophilia A) and 14 percent are Factor IX (Hemophilia B). About one percent of hemophilia cases is due to deficiencies of Factor XI.
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