Muscle Function and Its Biological and Physiological Determinants in Sickle Cell Disease

Summary

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.

Conditions

• Sickle Cell Disease

Interventions

BIOLOGICAL

Blood sampling

Blood sampling will be performed to assess hematological and hemorheological parameters

OTHER

Maximum Voluntary Contraction (MVC) test force

Maximum Voluntary Contraction (MVC) test force will be performed before and after a localized muscle endurance test

OTHER

Localized muscle endurance test

Subject will perform 4 series of 20 submaximal dynamic contractions at 50% of the MVC interspaced with 1 min recovery.

OTHER

Self-paced six-minute walk test

Self-paced six-minute walk test will be conducted according to the guidelines of the American Thoracic Society

Sponsors & Collaborators

• Hospices Civils de Lyon

  lead OTHER

Principal Investigators

• Giovanna CANNAS, MD · Hospices Civils de Lyon

Study Design

Allocation

NON_RANDOMIZED

Purpose

BASIC_SCIENCE

Masking

NONE

Model

PARALLEL

Eligibility

Min Age

15 Years

Max Age

60 Years

Sex

ALL

Healthy Volunteers

Yes

Timeline & Regulatory

Start

2017-09-15

Primary Completion

2019-12-13

Completion

2019-12-13

Countries

• France

Study Locations