Thrombophilia is the tendency for abnormal blood clotting. In a person with thrombophilia, a blood clot could form inside an unbroken blood vessel. If left unchecked, it can lead to loss of blood supply to tissues - sometimes with fatal consequences.
Thrombophilia is treatable. Treatments can prevent recurrent thrombosis in the future. The Thrombophilia Program at Children’s includes:
- Diagnosis
- Prevention treatment of thromboembolic events
- Use of anticoagulant therapy in other conditions such as cardiac catheterization, cardiopulmonary bypass, dialysis, extra corporeal membrane oxygenation, stents and others
Children inherit thrombophilia from one parent or both parents. A genetic mutation can also cause it.
How do You Know if Your Child has Thrombophilia?
If he/she has a history of thrombosis, chances of having one or more forms of thrombophilia vary from less than one to 40 percent depending on:
- Age
- Sex
- Ethnic background
- Whether or not thrombosis was related to surgery, trauma, estrogen administration or prolonged bedrest
Reasons for increasing incidence and severity include:
- The capacity to cure most previously life-threatening diseases which then permits the development of new diseases such as thromboembolic events.
- The necessity for venous and arterial catheters to treat critically ill children.
- The availability of sensitive radiographic tools that can diagnose thromboembolic events in unusual locations which were previously undetected.
- The capacity to identify children with congenital prothrombotic disorders.
Developmental Hemostasis
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The coagulation system
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Coagulation proteins
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Coagulation inhibitors
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The fibrinolytic system
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The platelets and the vessel wall
Venous Thromboembolic Disease
- Incidence
- Congenital prothrombotic disorders
- Factor V Leiden
- Prothrombin mutant
- Protein C deficiency
- Protein S deficiency
- Antithrombin
- Dysfibrinogenemia
- Defects within the fibrinolytic system
- Platelet defect
- Central venous catheters
- Underlying medical conditions
- Clinical presentation
Arterial Thromboembolic Disease
- Incidence
- Congenital causes of arterial disease
- Familial homozygous hypercholesterolemia
- Hyperhomocystinuria
- Acquired disorders
- Takayasu's arteritis
- Occlusion of giant coronary aneurysm secondary to Kawasaki disease
- Occlusion of arteries in transplanted organs
- Acquired heart disease
- Paroxysmal nocturnal hemoglobinuria
- Central venous catheters
- Clinical Presentation
Antithrombotic Agents
- Heparin
- Low molecular weight heparin
- Coumadin
Antiplatelet Therapy
- Aspirin
- Dipyridamole
Thrombolytic Therapy
- Tissue plasminogen activator