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Issue title: Selected Proceedings of the 16th Conference of the European Society for Clinical Hemorheology and Microcirculation (ESCHM), 18–21 June, 2011, Munich, Germany
Article type: Research Article
Authors: Connes, Philippe; | Machado, Roberto | Hue, Olivier | Reid, Harvey
Affiliations: Laboratory ACTES, Department of Physiology, University of the French West Indies, Pointe a Pitre, Guadeloupe, French West Indies | Inserm, Pointe-à-Pitre, Guadeloupe, Université des Antilles et de la Guyane, Pointe-à-Pitre, France | Section of Pulmonary, Critical Care Medicine, Sleep and Allergy, University of Illinois Chicago, Chicago, USA | Department of Basic Medical Sciences (Physiology Section), Faculty of Medical Sciences, University of the West Indies, Mona Campus, Kingston, Jamaica, West Indies
Note: [] Corresponding author: Philippe Connes, Laboratory ACTES (EA 3596), Department of Physiology, University of the French West Indies, 97159 Pointe a Pitre, Guadeloupe, French West Indies. Tel.: 590 690 36 76 28; Fax: 590 590 83 05 13; E-mail: [email protected]
Abstract: Sickle cell anemia (SCA or SS homozygous sickle cell disease) is an inherited blood disorder caused by single nucleotide substitution in the β-globin gene that renders their hemoglobin (HbS) much less soluble than normal hemoglobin (HbA) when deoxygenated. The polymerization of HbS upon deoxygenation is the basic pathophysiologic event leading to RBC sickling, hemolysis, vasoocclusion and ultimately to chronic organ damage. The metabolic changes imposed by exercise may initiate sickling and vaso-occlusive episodes. Further, in patients with SCA, exercise limitation may be related to anemia or chronic complications such as pulmonary vascular disease, congestive heart failure and chronic parenchymal lung disease. Few studies have investigated the cardiorespiratory responses of patients with SCA during either symptom-limited maximal exercise test on cyclo-ergometer or during a six minute walk test. Therefore, patients are advised to start exercise slowly and progressively, to maintain adequate hydration during and after exercise, to avoid cold exposure or sudden change in temperature, and to avoid sports associated with mechanical trauma. There are, however, lack of evidence to allow practitioners to prescribe an exercise program for patients with SCA, and individuals are usually encouraged to exercise on a symptom-limited basis. Finally, this review will also highlight the basic principles that are often used for exercise practice and could be used for exercise prescription and rehabilitation in patients with sickle cell anemia.
Keywords: Sickle cell disease, exercise rehabilitation, exercise testing, clinical complications, physical fitness
DOI: 10.3233/CH-2011-1465
Journal: Clinical Hemorheology and Microcirculation, vol. 49, no. 1-4, pp. 151-163, 2011
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