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Diseases and Conditions › Pediatric Hematology › Hemophilia

Hemophilia
Dr Ira Shah
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Hemophilia - Introduction
What is hemophilia?
Hemophilia is an inherited disorder in which there is inability to form an effective clot and hence causes prolonged bleeding in a patient. It is seen exclusively in males. A person with hemophilia does not bleed faster, only longer.

How common is hemophilia?
Hemophilia affects approximately 1 in 5,000 live male births.

How does Hemophilia occur?
A. The normal clotting system consists of
Vascular Spasm
Platelet plug formation
Coagulation: Fibrin production
The normal coagulation of blood depends on the following clotting factors:
Factor I - Fibrinogen
Factor II - Prothrombin
Factor III - Tissue thromboplastin
Factor IV - Calcium ions
Factor - V - Labile Factor
Factor - VII - Stable Factor
Factor VIII - Antihemophilic Factor
Factor IX - Christmas Factor
Factor X - Stuart-Prower Factor
Factor XI - Plasma Thromboplastin Antecedent (PTA)
Factor XII - Hageman Factor
Factor XIII - Fibrin stabilizing factor
Coagulation cascade consists of two pathways - the intrinsic and extrinsic pathway. It is the deficiency of factor VIII or IX in the intrinsic pathway that causes hemophilia.
Hemophilia Hemophilia 12/20/2000
Clinical Presentation Of Hemophilia >>
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