HEMOPHILIA

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Dr Ira Shah
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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.



Contributor Information and Disclosures Dr Ira Shah
Consultant Pediatrician, B.J.Wadia Hospital for Children, Mumbai, India


First Created : 12/20/2000
References
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