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Bernard Soulier Syndrome: Presenting As High-grade Fever In A Young Male
ISSN: 2617 - 9482
Publisher: author
Bernard Soulier Syndrome: Presenting As High-grade Fever In A Young Male
Indexed in
Health Sciences
ARTICLE-FACTOR
1.3
Article Basics Score: 3
Article Transparency Score: 2
Article Operation Score: 3
Article Articles Score: 3
Article Accessibility Score: 2
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International Category Code (ICC):
ICC-0702
Publisher: Bahria University Medical And Dental College
International Journal Address (IAA):
IAA.ZONE/261798469482
eISSN
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2617 - 9482
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Abstract
Bernard-Soulier syndrome (BSS) is a rare inherited disorder of blood clotting (coagulation). They may be life-threateningand demand immediate attention. Congenital bleeding disorders especially pose a diagnostic challenge to the clinicianbecause of their rarity and the need to be differentiated from the other common causes of bleeding in children. We presenta case of young male presenting with generalized weakness, high grade fever with chills, dry cough and black tarry stools(2-3 stools/day). No response to steroids and further evaluation by platelet, ADP, collegen, epinephrine and defectiveresponse to ristocetin led to the correct diagnosis – Bernard soulier syndrome (BSS). However, it is imperative to havearrived at correct diagnosis in order to save unnecessary therapy and to take due precautions for prevention of bleeding.Key Words: Bernard Soulier Syndrome, Bleeding Disorder, Decrease expression of glycoprotein
