Study of the Function and Muscle Metabolism in Drepanocytose Affected Patient During a Moderate Intensity Exercise

Public Assistance-Hospitals of Marseille (AP-HM)

Status: Unknown

Conditions: Drepanocytose

Treatments: Other: Spectroscopy magnetic resonance of phosphorus 31

Study type: Interventional

Funder types: Other

Identifiers

NCT02874170

2014-39

2014-A01486-41 (Other Identifier)

Details and patient eligibility

About

Drepanocytose disease is the first genetic disease in the world. It results in the synthesis of an abnormal hemoglobin (HbS), which in its deoxygenated form, polymerizes and leads to structural changes of red blood cells (RBC) which then take the shape of a sickle, become more fragile, more rigid and less deformable. The fragility of GR sickle causes their mass destruction, leading to chronic anemia (i.e. low levels of GR in the blood) associated with low tissue oxygenation. More rigid and less deformable, sickle GR tend to hang in the microvessels, leading to vaso-occlusive crises (CVO) particularly painful, can cause the failure of certain organs (spleen, kidneys, brain, lung, heart, liver , bone ...) and to life-threatening patients. Preliminary studies conducted on patients with drepanocytose disease (HbSS) have demonstrated changes of muscle tissue indicating a possible failure in the supply and use of oxygen. To date, the translation of this metabolic remodeling in the muscle work is not known.

This project's main objective is to evaluate muscle function in drepanocytose attempted patients. We hypothesized that muscle remodeling associated with sickle cell disease have a functional impact on strength and muscle metabolism. The main objective is to characterize the maximal voluntary plantar flexor muscles. The criteria for these targets will be based on the comparison between healthy subjects, carriers of the sickle cell trait (HbAS) and sickle cell patients (HbSS) oxygen saturation.

The strictly non-invasive approach proposed in this project will study the functional parameters of the muscle of sickle cell disease and the possible link with the clinical manifestations of the disease, including vaso-occlusive crisis, in which tissue oxygenation and pH plays a major role.

Enrollment: 45 estimated patients

Sex: All

Ages: 18+ years old

Volunteers: Accepts Healthy Volunteers

Inclusion criteria

• Patient affected by sickel cells and healthy volonteers presenting no contre indication to spectrometry exam.

Exclusion criteria

• Patient affected by hemoglobinopathies other than drepanocytose

Trial design

Primary purpose: Basic Science

Allocation: Non-Randomized

Interventional model: Parallel Assignment

Masking: None (Open label)

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45 participants in 2 patient groups

drepanocytose affected patient

Experimental group

Treatment:

Other: Spectroscopy magnetic resonance of phosphorus 31

Healthy volunteers

Other group

Treatment:

Other: Spectroscopy magnetic resonance of phosphorus 31