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 Printable version |
Vascular aspects of sickle cell disease |
Sang Thrombose Vaisseaux. Volume 20, Number 4, 191-6, Avril 2008, Mini-revue
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 Résumé
Article gratuit
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Author(s) : Daniel Lemogoum, Luc Van Bortel, Philippe Van de Borne |
Summary : Sickle cell disease is a common genetic disease in sub-saharian Africa. This is caused by the persistence of foetal haemoglobin at adult age. Acute episodes of haemolysis are caused by environmental stress (hypoxia, cold etc), and lead to acute and chronic cardiovascular disease. The pathogenesis involves chronic endothelial dysfunction by excessive oxidative stress and decreased bioavailability of NO. Small arteries are prone to spasm and occlusion, whereas we describe a paradoxical decrease in arterial stiffness and reduced wave reflection. Heart failure and pulmonary hypertension are frequent. Stroke is devastating, especially in younger patients and may be predicted by paradoxical increase in cerebral blood flow. Renal involvement (essentially glomerular) is frequent and severe, and leads to end stage renal disease at an early age. Prevention of target organ damage requires early and vigorous treatment of hemolytic crise with a planned transfusion policy. |
Keywords : sickle cell disease/physiopathology, artery, arterial pressure, haemoglobin |
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