Bernard–Soulier syndrome (BSS), also called hemorrhagiparous thrombocytic dystrophy, is a rare autosomal recessive coagulopathy (bleeding disorder) that causes a deficiency of glycoprotein Ib (GpIb), the receptor for von Willebrand factor, an important glycoprotein involved in hemostasis. The incidence of BSS is estimated to be less than 1 case ...
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http://en.wikipedia.org/wiki/Bernard–Soulier_syndrome
(from the article `blood disease`) ...thrombasthenia, an inherited disorder associated with a mild bleeding tendency, is due to a deficiency of the platelet glycoprotein IIb–IIIa, ...
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http://www.britannica.com/eb/a-z/b/53
<haematology, syndrome> A congenital disorder where the platelets lack receptors which allow them to adhere to the walls of blood vessels. The genetic deficiency in platelet membrane glycoprotein Ib allows platelets to aggregate normally (c.f. Glanzmann's thrombasthenia) but they do not stick to collagen of sub endothelial basement membrane. ...
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http://www.encyclo.co.uk/local/20973
(BSS) (bār-nahr´ sldbomacl-ya´) an autosomal recessive disorder characterized by giant platelets with membranes lacking glycoprotein Ib, the probable receptor for plasma von Willebrand factor; this keeps the platelets from binding the factor, which is necessary for their adhesion to the suben...
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http://www.encyclo.co.uk/local/21001
Genetic deficiency in platelet membrane glycoprotein Ib (CD42); platelets aggregate normally (cf Glanzmann's thrombasthenia) but do not stick to collagen of sub-endothelial basement membrane.
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http://www.encyclo.co.uk/visitor-contributions.php
Type: Term Pronunciation: bār-nahr′ sūl-yā′ Definitions: 1. a coagulation disorder characterized by thrombocytopenia, giant platelets, and a bleeding tendency. 2. an autosomal recessive disorder of absent or decreased platelet membrane glycoproteins Ib, IX, and V (the receptor for factor VIII R). This deficiency can lead ...
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http://www.medilexicon.com/medicaldictionary.php?t=87726
Bernard-Soulier syndrome (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 synd...
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http://www.medterms.com/script/main/art.asp?articlekey=7085
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