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HEMOPHILIA

DEFINITION

It is a bleeding disorder characterized by


deficiency of factor 8, 9 and 11.

ETIOLOGY

Hereditary

CLASSIFICATON
Hemophilia A Classical hemophilia , occur
due to deficiency of plasma factor 8. found
in 80-85%.
Hemophilia B- Christmas disease, result
from deficiency of plasma factor 9. found in
15-20%.
Hemophilia C-result from deficiency of
factor 11,found in few cases

CLINICAL FEATURE
Bleeding from gum nose lip and joints.
GI bleeding(malena)
Intracranial haemorrhage
Hematuria

DIAGNOSIS
Blood test-CT, PTT is prolonged
X-ray of affected joint
Gene analysis for antenatal diagnosis

MANGEMENT
Replacement therapy
Fresh whole blood transfussion
Antifibrinolytics agent aminocaproic acid
and tranexamic acid
Supportive management
Synovectomy
Gene therapy
Genetic counselling

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