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Title of Journal: J Thromb Thrombolysis

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Abbravation: Journal of Thrombosis and Thrombolysis

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Springer US

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DOI

10.1016/0304-3975(92)90016-9

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1573-742X

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Evaluation of von Willebrand factor and ADAMTS13

Authors: Anwar AlAwadhi Adekunle Adekile Rajaa Marouf
Publish Date: 2016/09/09
Volume: 43, Issue: 1, Pages: 117-123
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Abstract

Sickle cell disease SCD is a severe form of hemolytic anemia characterized by chronic hemolysis and is associated with increased thrombotic risk Elevated von Willebrand factor vWF levels in SCD have been attributed to increased secretion and impaired processing by its cleaving protease ADAMTS13 In this study we measured vWF and ADAMTS13 antigen and activity levels in our SCD patients Hematological and biochemical parameters for 59 SCD patients 20 children and 39 adults were analyzed and compared to 59 age and sexmatched controls Commercially available ELISA kits were used to measure vWF and ADAMTS13 antigen and activity levels in patients and controls Patients had significantly higher levels of vWF p  0006 and ADAMTS13 activity p  0006 compared to controls When patients were analyzed according to age and genotype adult patients 23 SS and 16 Sβ0thal maintained higher vWF antigen levels p  0001 but with reduced ADAMTS13 activity to vWFAg ratio p  0003 compared to controls Pediatric patients 8 SS and 12 Sβ0thal had comparable vWF antigen levels to controls p  005 but had higher levels of ADAMTS13 activity p  0011 and ADAMTS13 activity to vWFAg ratio p  0038 Age is an important factor to consider when vWF and ADAMTS13 proteins are analyzed among our patients Increased vWF in adult patients may be attributed to increased production and resistance of vWF to proteolysis rather than ADAMTS13 deficiency This outcome was not seen in pediatric patients as higher ADAMTS13 activity maintained vWF antigen at comparable levels to normal controlsThis work was supported and funded by Kuwait University Research Grant No NM02/13 Special thanks to Mrs Liena S Abdulaziz for performing ELISA work The authors also thank Rasha Abdullah Hadeel AlMuzaini and Nada Mustafa for their help in sample and data collection The technical assistance of Mohammad Ezzat Matra Salem and Mays Abdulhadi is appreciatedAll procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards

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