Molecular Pathology of Rare Bleeding Disorders (RBDs) in India: A Systematic Review

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Molecular Pathology of Rare Bleeding Disorders (RBDs) in India: A Systematic Review

BACKGROUND Though rare in occurrence, patients with rare bleeding disorders (RBDs) are highly heterogeneous and may manifest with severe bleeding diathesis. Due to the high rate of consanguinity in many caste groups, these autosomal recessive bleeding disorders which are of rare occurrence in populations across the world, may not be as rare in India. OBJECTIVES To comprehensively analyze the ...

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rare bleeding disorders: a narrative review of epidemiology, molecular and clinical presentations, diagnosis and treatment

rare bleeding disorders (rbds) are a heterogeneous group of disorders including different types of coagulation factor deficiencies. the disorders are inherited in an autosomal recessive manner with different frequencies varying from 1:500000 to 1:2000000. patients affected with rbds are presented with a wide spectrum of clinical manifestations ranging from mild to life threatening bleeding diat...

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Rare bleeding disorders: a narrative review of epidemiology, molecular and clinical presentations, diagnosis and treatment

Majid Naderi Shadi Tabibian Maryam Sadat Hosseini Shaban Alizadeh Soudabeh Hosseini Hossein Karami Hassan Mahmoodi Nesheli Akbar Dorgalaleh Departement Of Pediatrics Hematology & Oncology, Ali Ebn-e Abitaleb Hospital Research Center For Children And Adolescents Health [RCCAH], Zahedan University of Medical Sciences, Zahedan, IR Iran Department of Hematology, Allied Medical School, Tehran Univer...

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Rare bleeding disorders.

During the haemostatic response, the formation of a primary platelet plug limits bleeding and provides a surface for clotting factors to assemble and become activated. The initial platelet plug is stabilized by fibrin monomers, covalently cross-linked by FXIII, forming a platelets-fibrin thrombus. Defects in platelets as well as inherited deficiencies of coagulation factors including fibrinogen...

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ژورنال

عنوان ژورنال: PLoS ONE

سال: 2014

ISSN: 1932-6203

DOI: 10.1371/journal.pone.0108683