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The Bernard-Soulier Syndrome is a rare inherited bleeding disorder. It is one of the giant platelet syndromes that is caused by abnormal platelets and subsequent abnormal clotting. The platelet abnormality is described as the condition in which the platelets lack the ability to stick adequately to injured blood-vessel walls. This syndrome is transmitted in an autosomal recessive pattern. Signs of the syndrome are initially noticed during childhood. Symptoms vary from person to person. The following are some of them: a) Easy bruising b) Nosebleeds c) Bleeding from gums d) Heavy or prolonged menstrual bleeding or bleeding after childbirth e) Abnormal bleeding after surgery
HOW IS BERNARD-SOULIER SYNDROME DIAGNOSED?
The diagnosis requires analysis of medical history and certain laboratory tests that should be performed by haemophilia specialist.
HOW IS BERNARD-SOULIER SYNDROME TREATED?
People affected with this syndrome require treatment during surgical procedures. The other methods of treatment are: • Antifibrinolytic drugs • Recombinant • Desmopressin • Fibrin sealants • Hormonal contraceptives • Iron replacement • Platelet transfusions
DID YOU KNOW?
For patients with moderate to severe symptoms, some restriction of activity may be necessary