Sickle cell disease (SCD) is the most frequent life-threatening genetic hemoglobinopathy in the world and occurs due to the synthesis of abnormal hemoglobin S (HbS). hemoglobin S-containing red blood cells (RBC) are fragile, leading to hemolysis and anemia, and adhere to the endothelium, leading to hemorheological and hemodynamical disturbances. In its deoxygenated form, HbS may polymerize, leading to sickling of red blood cells and potentially to vasoocclusive crises. Recent findings observed that SCD patients demonstrate significant skeletal muscle remodeling and display reduced muscle functional capacities, contributing to exercise intolerance and poor quality of life. Although acute high-intensity exercise is not recommended for SCD patients because it may increase the risk of sickling, regular moderate-intensity physical activity could have beneficial effects on skeletal muscle and more generally on the well-being of SCD patients. This article reviews the literature regarding the impact of the disease on muscular tissue characteristics and function, as well as the corresponding implications for SCD patients' quality of life.
Lactate exchange and removal abilities in sickle cell patients and in untrained and trained healthy humans
Use of limb movement sensors as indicators of the level of everyday physical activity in chronic congestive heart failure
Cardio-pulmonary responses and gas exchange during exercise in adults with homozygous sickle-cell disease (sickle-cell anaemia)
The influence of wind resistance in running and walking and the mechanical efficiency of work against horizontal or vertical forces
Endothelial dysfunction in patients with sickle cell disease is related to selective impairment of shear stress-mediated vasodilation.
Physical fitness indices and anthropometrics profiles in schoolchildren with sickle cell trait/disease
Hemodynamic and functional assessment of patients with sickle cell disease and pulmonary hypertension
Cardiopulmonary stress testing in children with sickle cell disease who are on long-term erythrocytapheresis
Haemoglobin oxygen saturation is a determinant of cerebral artery blood flow velocity in children with sickle cell anaemia
Pain-related behaviors and neurochemical alterations in mice expressing sickle hemoglobin: modulation by cannabinoids
Red blood cell deformability and aggregation, cell adhesion molecules, oxidative stress and nitric oxide markers after a short term, submaximal, exercise in sickle cell trait carriers
Skeletal muscle structural and energetic characteristics in subjects with sickle cell trait, alpha-thalassemia, or dual hemoglobinopathy
Mild haemorheological changes induced by a moderate endurance exercise in patients with sickle cell anaemia
Delayed beneficial effect of acute exercise on red blood cell aggregate strength in patients with sickle cell anemia
Effects of regular physical activity on skeletal muscle structural, energetic, and microvascular properties in carriers of sickle cell trait
Antisickling fetal hemoglobin reduces hypoxia-inducible factor-1α expression in normoxic sickle mice: microvascular implications
Normal muscle oxygen consumption and fatigability in sickle cell patients despite reduced microvascular oxygenation and hemorheological abnormalities
Respiratory muscle force and lung volume changes in a population of children with sickle cell disease
Fatigue in adolescents and young adults with sickle cell disease: biological and behavioral correlates and health-related quality of life
Acute moderate exercise does not further alter the autonomic nervous system activity in patients with sickle cell anemia
Moderate endurance exercise in patients with sickle cell anaemia: effects on oxidative stress and endothelial activation
Evidence for a profound remodeling of skeletal muscle and its microvasculature in sickle cell anemia
Pilot randomized controlled trial to evaluate the effect of aquatic and land physical therapy on musculoskeletal dysfunction of sickle cell disease patients
Reduced fitness and abnormal cardiopulmonary responses to maximal exercise testing in children and young adults with sickle cell anemia
Physical activity level is not a determinant of autonomic nervous system activity and clinical severity in children/adolescents with sickle cell anemia: A pilot study
Changes in structural and metabolic muscle characteristics following exercise-based interventions in patients with COPD: a systematic review
Score Predicting Acute Chest Syndrome During Vaso-occlusive Crises in Adult Sickle-cell Disease Patients
Moderate and intense muscular exercises induce marked intramyocellular metabolic acidosis in sickle cell disease mice
Cerebral and muscle microvascular oxygenation in children with sickle cell disease: Influence of hematology, hemorheology and vasomotion
Exacerbated in vivo metabolic changes suggestive of a spontaneous muscular vaso-occlusive crisis in exercising muscle of a sickle cell mouse
Comparative NMR and NIRS analysis of oxygen-dependent metabolism in exercising finger flexor muscles
Endurance training reduces exercise-induced acidosis and improves muscle function in a mouse model of sickle cell disease
Beneficial effects of endurance exercise training on skeletal muscle microvasculature in sickle cell disease patients.
Muscle structural, energetic and functional benefits of endurance exercise training in sickle cell disease
Effect of acute exercise on RBC deformability and RBC nitric oxide synthase signalling pathway in young sickle cell anaemia patients
Blood Rheology: Key Parameters, Impact on Blood Flow, Role in Sickle Cell Disease and Effects of Exercise
In vivo muscle function and energetics in women with sickle cell anemia or trait: a 31P-magnetic resonance spectroscopy study.
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