Sickle Cell Disease Clinical Trial
Official title:
Muscle Function and Its Biological and Physiological Determinants in Sickle Cell Disease
Background : Sickle cell patients have profound remodeling of their muscle microcirculation
networks with signs of amyotrophy. However, the consequences of these muscle alterations on
the functional status of muscles are unknown. In addition, whether the poor physical fitness
of sickle cell patients can be attributed, at least partly, to an hypothetical muscle
dysfunction has never been tested.
Purpose : this study will compare the muscle function of legs between sickle cell patients
(SS and SC genotypes) and healthy individuals (AA genotype) before, during and after a short
localized muscle endurance exercise.
Abstract : Very recently, a study reported large differences between the muscle
microcirculation networks of sickle cell patients compared to healthy individuals with
decreased capillary density and higher proportion of large capillaries in the former
population. In addition, the same study showed signs of amyotrophy in sickle cell patients.
However, the muscle function of sickle cell patients has not been investigated and one may
suggest that muscle dysfunction could participate in the decrease of physical fitness, in
association with the hematological and hemorheological disorders, already reported in this
population. The hypothesis is that muscle fatigue during a short localized muscle endurance
exercise should be higher in sickle cell patients compared to healthy individuals, due to a
greater recruitment of glycolytic fibers and a faster decrease of muscle oxygenation during
exercise.
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