Skip to main content
OpenTrials
Completed

NCT Number: NCT03243812

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.

Completed

Looking for future studies?

Notify Me

Key information

Conditions

Sickle Cell Disease Anemia Anemia, Hemolytic Anemia, Hemolytic, Congenital Anemia, Sickle Cell Congenital, Hereditary, and Neonatal Diseases and Abnormalities Genetic Diseases, Inborn Hematologic Diseases Hemic and Lymphatic Diseases Hemoglobinopathies

Age range

15 year–60 year

Sex eligibility

All sexes

Study type

Interventional

Phase

Not applicable

Primary location

Hôpital Edouard Herriot

Lyon, 69003, France

Who can participate

Healthy volunteers accepted: Yes

Only the study team can determine whether someone qualifies for participation.

Inclusion criteria

For Sickle cell patients :

  • age ≥ 15 and < 60 years old,
  • SS homozygote or SC heterozygote
  • in clinical steady state (i.e. without vaso-occlusive crisis or recent blood transfusion)
  • identified by systematic neonatal screening programs,
  • registered in the French medical social security national program

For Healthy and non sickle cell subjects:

  • age ≥ 18 and < 60 years old
  • without cardiovascular/respiratory/muscle disease,
  • registered in the French medical social security national program.

Exclusion criteria

  • other hemoglobinopathies,
  • stroke or vasculopathy history,
  • presence of leg ulcers or osteonecrosis,
  • recent infectious episode (less than 1 month),
  • chronic transfusion therapy programs,
  • recent blood transfusion or phlebotomies (less than 3 months),
  • patients not at steady state,
  • pregnancy or breast feeding

Treatment and study plan

Blood sampling

Biological

Blood sampling will be performed to assess hematological and hemorheological parameters

Maximum Voluntary Contraction (MVC) test force

Other

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

Localized muscle endurance test

Other

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

Self-paced six-minute walk test

Other

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

Primary outcomes

  1. Maximum isometric muscular strength

    Time frame: Day 1

    Isometric muscular strength will be determined by Maximum Voluntary Contraction (MVC) test force on dominant leg.

    Muscular function will be evaluated using Maximum Voluntary Contraction (MVC) test force and the muscle endurance ability, which will be highlighted by the degree of decline of MVC after a short localized muscle effort using the formula: ((post MVC force - pre MVC force) / pre MVC force)x100.

    Muscle weakness will be determined by a loss of maximum isometric strength ≥ 20 % compared with control group.

Secondary outcomes

  1. Surface Electromyography (EMG) Activity

    Time frame: Day 1

    Surface EMG signals will be recorded by non-invasive electrodes on the dominant leg.

  2. Muscle oxygenation measurement

    Time frame: Day 1

    oxyhemoglobin (HbO2) and deoxyhemoglobin (HHb) levels will be measured using Near-Infrared spectroscopy on the dominant leg.

  3. Measurement of six-minute walk distance (6MWD)

    Time frame: Day 1

    In order to investigate the association between muscle endurance ability and physical fitness in sickle cell patients, patients will realize a six-minute walk test (6MWT).

    6MWD will be measured = the distance that a patient has walked on a flat, hard surface in a period of 6 minutes (6MWT).

  4. Complete Blood Count (CBC)

    Time frame: Day 1

    CBC will be performed in order to evaluate the role of hematological disorders in the muscle fatigue of sickle cell patients.

  5. Hematocrit

    Time frame: Day 1

    Hematocrit will be measured in order to evaluate the role of hematological disorders in the muscle fatigue of sickle cell patients.

  6. Blood viscosity

    Time frame: Day 1

    Blood viscosity will be measured by using viscosimetry, in order to evaluate the role of hemorheological disorders in the muscle fatigue of sickle cell patients.

  7. Red blood cell (RBC) deformability

    Time frame: Day 1

    RBC deformability will be assessed by using ektacytometry, in order to evaluate the role of hemorheological disorders in the muscle fatigue of sickle cell patients.

  8. Aggregation properties

    Time frame: Day 1

    Aggregation properties will be assessed by using syllectometry, in order to evaluate the role of hemorheological disorders in the muscle fatigue of sickle cell patients.

  9. Hemoglobin oxygenation level

    Time frame: Day 1

    Hemoglobin oxygenation level will be measured in order to evaluate the role of hemorheological disorders in the muscle fatigue of sickle cell patients.

  10. Number of vaso-occlusive crises and acute chest syndrome within a 5 years retrospective period.

    Time frame: Day 1

    Number of vaso-occlusive crises and acute chest syndrome reflects of clinical severity of the sickle cell disease.

    Clinical severity will be retrospectively (5 years) collected in clinical record of sickle cell patients.

    These clinical data will be used to study the relationships between the degree of muscle dysfunction and the degree of clinical severity in sickle cell patients.

Sponsors and collaborators

Lead sponsor

Hospices Civils de Lyon

Other

Registry information

Acronym: DREPAMUSCLE

Important dates

Study start
2017
Primary completion
2019
Study completion
2019
First posted
Aug 9, 2017
Registry last updated
Dec 19, 2025

OpenTrials presents study information sourced from ClinicalTrials.gov. The official registry record should be consulted for the latest information.

View the official ClinicalTrials.gov record (opens in a new tab)

This listing is for discovery and informational purposes only. It is not medical advice, does not guarantee that a study is recruiting, and does not determine eligibility. Contact the study team and a qualified healthcare professional when considering participation.

Related clinical trials

Published trials that share one or more normalized conditions with this study.