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Hand <strong>book</strong> <strong>for</strong> <strong>dental</strong> <strong>surgeons</strong> on <strong>dental</strong> management of persons with hemophilia and other haemostatic disorders<br />

Introduction<br />

HEMOPHILIA<br />

Hemophilia is a hereditary disorder of coagulation results in deficiency of factor VIII (Hemophilia A) or<br />

factor IX (haemophilia B). The disease is almost exclusively see in males while females are asymptomatic<br />

carries. Rarely it can affect females. It can also be caused by mutation. It is characterized by spontaneous or<br />

trauma related bleeding typically into the large joints or muscles. Untreated or inadequately treated episodes<br />

lead to joint, muscles or nerve damage. Life threatening haemorrhages can result spontaneously or from trauma<br />

to the head or internal organs.<br />

Without proper care, hemophilia can lead to serious damage to limb and joint function within the first<br />

one to two decades of life. This is due to impaired joint mobility, contractures, muscle atrophy and chronic pan.<br />

Crippling joint de<strong>for</strong>mities can be effectively prevented by adequate care of each episode of bleeding, including<br />

replacement of the appropriate coagulation factor lacked by the patient and every deliberated and persistent<br />

physical therapy. Certain serious complications can further complicate the management of hemophilia.<br />

Between 10-20% of people with hemophilia A and 23% of those with hemophilia B develop inhibitors against<br />

the deficient factor. Such patients do not respond to usual factor replacement therapy. Patients exposed to<br />

factor replacement with products that have not been virus inactivate are prone to acquiring transfusion<br />

transmitted viral infections such as human immuno-deficiency virus (HIV), hepatitis B and C viruses. The care<br />

of people with hemophilia there<strong>for</strong>e often requires a multidisciplinary team to address different aspects of the<br />

patientís problems. This is the concept of comprehensive care that is recommended by the World Health<br />

Organization (WHO) and the World Federation of Hemophilia (WFH).<br />

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Hand <strong>book</strong> <strong>for</strong> <strong>dental</strong> <strong>surgeons</strong> on <strong>dental</strong> management of persons with hemophilia and other haemostatic disorders

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