Pathophysiological insights in sickle cell disease.

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dc.contributor.authorOdièvre, Marie-Hélène
dc.contributor.authorVerger, Emmanuelle
dc.contributor.authorSilva-Pinto, Ana Cristina
dc.contributor.authorElion, Jacques
dc.date.accessioned2012-01-10T07:13:06Z
dc.date.available2012-01-10T07:13:06Z
dc.date.issued2011-10
dc.description.abstractThe first coherent pathophysiological scheme for sickle cell disease (SCD) emerged in the sixties-seventies based on an extremely detailed description of the molecular mechanism by which HbS in its deoxy-form polymerises and forms long fibres within the red blood cell that deform it and make it fragile. This scheme explains the haemolytic anaemia, and the mechanistic aspects of the vaso-occlusive crises (VOCs), but, even though it constitutes the basic mechanism of the disease, it does not account for the processes that actually trigger VOCs. This paper reviews recent data which imply: red blood cell dehydration, its abnormal adhesion properties to the endothelium, the participation of inflammatory phenomenon and of a global activation of all the cells present in the vessel, and finally, abnormalities of the vascular tone and of nitric oxide metabolism. These data altogether have shed a new light on the pathophysiology of the first molecular disease i.e. sickle cell disease.en_US
dc.identifier.citationOdièvre Marie-Hélène, Verger Emmanuelle, Silva-Pinto Ana Cristina, Elion Jacques. Pathophysiological insights in sickle cell disease. Indian Journal of Medical Research. 2011 Oct; 134(4): 532-537.en_US
dc.identifier.urihttps://imsear.searo.who.int/handle/123456789/136354
dc.language.isoenen_US
dc.source.urihttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC3237253/en_US
dc.subjectCell activationen_US
dc.subjectcell adhesionen_US
dc.subjecthaemoglobin Sen_US
dc.subjectinflammationen_US
dc.subjection channelsen_US
dc.subjectnitric oxideen_US
dc.subjectsickle cell diseaseen_US
dc.subject.meshAnemia, Sickle Cell --blood
dc.subject.meshAnemia, Sickle Cell --metabolism
dc.subject.meshCell Adhesion
dc.subject.meshEndothelium, Vascular --metabolism
dc.subject.meshErythrocytes --metabolism
dc.subject.meshErythrocytes --pathology
dc.subject.meshHemoglobin, Sickle --genetics
dc.subject.meshHemoglobin, Sickle --metabolism
dc.subject.meshHemolysis
dc.subject.meshHumans
dc.subject.meshIon Channels --metabolism
dc.subject.meshNitric Oxide --metabolism
dc.titlePathophysiological insights in sickle cell disease.en_US
dc.typeArticleen_US
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