The process of blood coagulation is a vital
mechanism of defence against excessive loss of blood. There are nearly 13
factors involved in the mechanism of blood clotting. If any one or more of
these factors are not synthesised adequately and properly that results in
defect in blood clotting and thereby hemorrhage.
A number of inherited deficiency of the blood
clotting factors are found in human and are collectively called as hemophilias.
Hemophilia is an inherited disease, where
clotting occurs at an abnormally slow rate due to the absence of one or more of
the blood clotting factors. The sufferers are known as ‘ hemophiliacs ’ or ‘
bleeders’. It is peculiar that it affects only males. The most common
deficiency is that deficiency of the factor VIII, causes hemophilia A.
Deficiency of factor IX causes hemophilia B. Deficiency of factor XI causes
hemophilia C.
The characteristic findings of this disease are:
·
Bleeding
which does not stop. These individuals should be extremely careful not to
contract even minor injuries like trauma or extraction of tooth, since this may
result in severe hemorrhage ( blood loss ).
·
Marked
prolongation of the coagulation time of the blood, while prothrombin time being
normal.
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