Sickle-cell disease overview: Difference between revisions
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==Overview== | ==Overview== | ||
'''Sickle-cell disease''' is a group of [[genetic disorders]] caused by | '''Sickle-cell disease''' is a group of [[genetic disorders]] caused by mutation in the β-globin chain gene of [[hemoglobin]] at the 6th position replacing [[glutamic acid]] to [[valine]]. HbS polymerizes reversibly when deoxygenated to form a network of fibrous [[hemoglobin]] polymers that stiffens the RBC [[Cell membrane|membrane]], giving it a sickle shape. These sickled cells loose the pliability to cross thin capillaries and possess a sticky membrane, giving it a property to adhere to the [[endothelium]] of blood vessels, thereby causing vaso-occlusion. It causes significant morbidity and mortality, particularly in people in the Mediterranean and African region. | ||
==References== | ==References== | ||
Revision as of 20:16, 20 August 2012
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Aarti Narayan, M.B.B.S [2]
Overview
Sickle-cell disease is a group of genetic disorders caused by mutation in the β-globin chain gene of hemoglobin at the 6th position replacing glutamic acid to valine. HbS polymerizes reversibly when deoxygenated to form a network of fibrous hemoglobin polymers that stiffens the RBC membrane, giving it a sickle shape. These sickled cells loose the pliability to cross thin capillaries and possess a sticky membrane, giving it a property to adhere to the endothelium of blood vessels, thereby causing vaso-occlusion. It causes significant morbidity and mortality, particularly in people in the Mediterranean and African region.