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DEFINITION
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