Hemophilia belongs to a family of inherited conditions permanent bleeding that prevents blood clotting properly. Patients with these disorders bleed for longer than normal, due to accident or spontaneously without any external cause.
The severity of bleeding depends on the amount of clotting factor that is missing or not working correctly, that in Hemophilia a and B-the most common types of hemophilia-is the clotting factors VIII and IX, respectively.
In addition to external bleeding patients commonly have internal bleeding around the joints and muscles, which can be extremely painful and cause of permanent disability. Haemorrhages in organs such as the brain is particularly difficult to manage, and can be fatal.
Here are 2 educational videos hemophilia from CDC: playing it safe with hemophilia: Hemophilia friends talk about playing sport grows and the importance of making smart decisions.
Start the conversation: Hemophilia. How to talk to your friends of hemophilia. A group of friends ask their friend Billy questions about his Hemophilia:
Treatment of hemophilia has undergone significant improvements over the past 40-50 years. First blood coagulation factor concentrates were not sufficiently refined to enable the self-treatment at home until 1970.
The long-term replacement therapy (prophylaxis) clotting factor missing is the recommended treatment in severe hemophilia. The major side effect of the treatment, the development of inhibitors for the concentrated infusion, is the main threat for the health of patients.
Mnemonic: differential diagnosis of bleeding disorders: F-CAP
Fibrinolysis-tPA
Coagulopathy-haemophilia, vWD
Angiopathy-conditions that affect the blood vessels, for example la sindrome di Rendu-Osler-Weber
Thrombocytopenia or platelet-thrombocytopathia
Initial diagnostic tests = 3 P:
Platelets
PT-INR
PTT
References:
http://www.cdc.gov/NCBDDD/video/Hemophilia_sports/index.html
http://www.cdc.gov/NCBDDD/video/Hemophilia_Friends/index.html
Making hemophilia a global priority -The Lancet, 2012.
Modern hemophilia care -The Lancet, 2012.
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