Hemophilia 2
Hemophilia 2
In the human body, each cell contains 23 pairs of chromosomes, one of each pair inherited through the egg from the mother and the other from the sperm of the father. Of these chromosomes, those that determine sex are X and Y. Females have XX and males have XY. In addition to information concerning sex, the X chromosomes carry determinants for a number of other features of the body, including the levels of factor Vlll and lX. If the genetic information determining factors Vlll and lX is defective, hemophilia results. As a result, the protein factors needed for normal blood clotting are effected.
In males, the single X chromosome can not compensate for the lack, and will show the defect. In females however, only one of the two chromosomes will be abnormal. In very rare cases however, she will have inherited two defective X chromosomes and herself will be a hemophiliac. If she is not affected and the gene is recessive, she is now classified as a carrier. In some cases, there is no previous record of hemophilia in the family. Male children who would be born with hemophilia would be the result of the mutation of the gene.
There are two types of hemophilia: Hemophilia A and Hemophilia B. Hemophilia A, also known as "Classical Hemophilia" is caused by a factor Vlll deficiency. This is the most common form of hemophilia. The second type of hemophilia, is Hemophilia B, and is sometimes referred to as the "Christmas Disease." Hemophilia B is caused by a factor lX deficiency. Both types of hemophilia are characterized by prolonged episodes of bleeding, especially into joints, muscles, internal organs, or even brain hemorrhaging.
Hemophiliacs do not bleed harder of faster than a normal, unaffected person. Instead, they suffer from prolonged bleeding. The reason a hemophiliac will suffer from prolonged bleeding, is the inability of the blood to form clots. This is the result of the missing/ reduced clotting factors. When a normal person sustains a cut or wound, the platelets in their blood break apart and quickly form fibrin, which forms the clot. In hemophiliacs however, either the clotting factor Vlll or lX is reduced, thus making the hemophiliac unable to form an effective clot. A few of the warning signs of internal bleeding in hemophiliacs are: headaches, sleepiness, nausea, vomiting, unequal pupil size, slurred speech, and disorientation and confusion.
Hemophilia is classified as severe, moderate, or mild, which indicates the expected frequency of bleeding. The normal clotting factor levels are on average 40% to 200%. If a hemophiliac has less than 2% clotting factors, then they are said to have severe hemophilia. If a person has severe hemophilia, factor Vlll of lX, depending on the type of hemophilia the hemophiliac suffers from, they will need the clotting factor Vlll or lX replaced several times a...
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