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Haemophilia:
Haemophilia
(also spelled as hemophilia in North America, from the Greek haima αἷμα
"blood" and philia friend") is a group of hereditary genetic disorders
that impair the body's ability to control blood clotting or coagulation,
which is used to stop bleeding when a blood vessel is broken. In its
most common form, Haemophilia A, clotting factor VIII is absent. In
Haemophilia B, factor IX is deficient. Haemophilia A occurs in about 1
in 5,000–10,000 male births, while Haemophilia B occurs at about 1 in
about 20,000–34,000.The effects of this sex-linked, X chromosome
disorder are manifested almost entirely in males, although the gene for
the disorder is inherited from the mother. Females have two X
chromosomes while
males
have only one, lacking a 'back up' copy for the defective gene. Females
are therefore almost exclusively carriers of the disorder, and may have
inherited it from either their mother or father. These genetic
deficiencies may lower blood plasma clotting factor levels of
coagulation factors needed for a normal clotting process. When a blood
vessel is injured, a temporary scab does form, but the missing
coagulation factors prevent fibrin formation which is necessary to
maintain the blood clot. Thus a hemophiliac does not bleed more
intensely than a normal person, but for a much longer amount of time. In
severe hemophiliacs even a minor injury could result in blood loss
lasting days, weeks, or not ever healing completely. The critical risk
here is with normally small injuries which, due to missing factor VIII,
take long times to heal. In areas such as the brain or inside joints
this can be fatal or permanently debilitating. The bleeding with
external injury is normal, but incidence of late re-bleeding and
internal bleeding is increased, especially into muscles, joints, or
bleeding into closed spaces.
Hemo RA-62 is the most effective medicine
for the patients having haemophilia. |