This is just two with the definitions.
Epistaxis: Medical term for nosebleed. The nose is a part of the body that is very rich in blood vessels (vascular) and is situated in a vulnerable position on the face. As a result, any trauma to the face can cause bleeding which may be profuse. Nosebleeds can occur spontaneously when the nasal membranes dry out, crust, and crack, as is common in dry climates, or during the winter months when the air is dry and warm from household heaters. People are more susceptible if they are taking medications which prevent normal blood clotting (Coumadin, warfarin, aspirin, or any anti-inflammatory medication). Other predisposing factors include infection, trauma, allergic and non-allergic rhinitis, hypertension, alcohol abuse, and inherited bleeding problems. "Epistaxis" is a Greek word meaning "a dripping," especially of blood from the nose.
(Giant Platelet Syndrome)
The Bernard-Soulier syndrome is a primary problem of platelets in which the platelets lack the ability to stick adequately to injured blood vessel walls. This is a crucial aspect of the process of forming a blood clot, and as a result of this problem there is abnormal bleeding.
Bernard-Soulier syndrome usually presents in the newborn period, infancy, or early childhood with bruises, nose bleeds (epistaxis), and/or gum (gingival) bleeding. Later problems can occur with anything which can induce bleeding such as menstruation, trauma, surgery, or stomach ulcers.
Bernard-Soulier syndrome is an inherited disease and is transmitted in an autosomal recessive pattern. Both parents must carry a gene for the Bernard-Soulier syndrome and transmit that gene to the child for the child to have the disease. The molecular basis is known and is due to a deficiency in platelet glycoproteins Ib, V, and IX. The parents have a decrease in the glycoprotein but no impairment of platelet function and no abnormal bleeding. The Bernard-Soulier gene has been mapped to the short (p) arm of chromosome 17 (in the region 17pter-17p12).
There is no specific treatment for Bernard-Soulier syndrome. Bleeding episodes may require platelet transfusions. A coagulation drug known as DDAVP may also be of short-term benefit. These abnormal platelets are usually considerably larger than normal platelets when viewed on blood films or sized by automated instruments. However, this is not the only syndrome with large platelets. Specific platelet function tests as well as tests for the glycoproteins can confirm the diagnosis.
This disease was first recognized in 1948 by two French hematologists: Jean Bernard and Jean-Pierre Soulier. (Because the disease is named not for one man whose name was Bernard Soulier but for these two men, there should be a hyphen in Bernard-Soulier syndrome). Bernard (1907-) is a very distinguished medical scientist who for many years was professor of hematology and director of the Institute for Leukemia at the University of Paris. Soulier (1915-) was director of the National Blood Transfusion Service, professor of hematology at the University of Paris, and a pioneer in the treatment of hemophilia and other bleeding disorders.
2007-02-16 23:02:05
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answer #1
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answered by Ex Head 6
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