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Impact of a submaximal mono-articular exercise on the skeletal muscle function of patients with sickle cell disease

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Abstract

Purpose

Sickle cell disease (SCD) patients exhibit a limited exercise tolerance commonly attributed to anaemia, as well as hemorheological and cardio-respiratory abnormalities, but the functional status of skeletal muscle at exercise is unknown. Moreover, the effect of SCD genotype on exercise tolerance and skeletal muscle function has been poorly investigated. The aim of this study was to investigate skeletal muscle function and fatigue during a submaximal exercise in SCD patients.

Methods

Nineteen healthy individuals (AA), 28 patients with sickle cell anaemia (SS) and 18 with sickle cell-haemoglobin C disease (SC) performed repeated knee extensions exercise (FAT). Maximal isometric torque (Tmax) was measured before and after the FAT to quantify muscle fatigability. Electromyographic activity and oxygenation by near-infrared spectroscopy of the Vastus Lateralis were recorded.

Results

FAT caused a reduction in Tmax in SS (− 17.0 ± 12.1%, p < 0.001) and SC (− 21.5 ± 14.5%, p < 0.05) but not in AA (+ 0.58 ± 29.9%). Root-mean-squared value of EMG signal (RMS) decreased only in SS after FAT, while the median power frequency (MPF) was unchanged in all groups. Oxygenation kinetics were determined in SS and AA and were not different.

Conclusion

These results show skeletal muscle dysfunction during exercise in SCD patients, and suggest different fatigue aetiology between SS and SC. The changes in EMG signal and oxygenation kinetics during exercise suggest that the greater skeletal muscle fatigue occurring in SCD patients would be rather due to intramuscular alterations modifications than decreased tissue oxygenation. Moreover, SS patients exhibit greater muscle fatigability than SC.

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Abbreviations

6-MWT:

6-Min walk test

AA:

Healthy subjects

ACS:

Acute chest syndrome

BP:

Blood pressure

EMG:

Electromyography

FAT:

Fatigability test

GPAQ:

Global Physical Activity Questionnaire

HbC:

Haemoglobin C

HbS:

Haemoglobin S

HR:

Heart rate

iMVC:

Isometric maximal voluntary contraction

MPF:

Median power frequency

NIRS:

Near-infrared spectroscopy

RBC:

Red blood cell

RMS:

Root Mean Square

RMSmax:

Maximal root mean square value

RPE:

Perceived exertion

SC:

Sickle cell-haemoglobin C disease

SCD:

Sickle cell disease

SpO2 :

Transcutaneous oxygen saturation

SS:

Sickle cell anaemia

Tmax:

Maximal isometric torque

TSI:

Tissue saturation index

VL:

Vastus Lateralis

VOC:

Vaso-occlusive crisis

References

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Acknowledgements

We thank Johan Cassirame, from Mtraining, for technical support on near-infrared spectroscopy.

Author information

Authors and Affiliations

Authors

Contributions

Study design: PC, GC, and CH. Acquisition of data: EG and SM. Analysis and interpretation of data: EG, PC, CF, and CH. Drafting of the manuscript: EG, PC, CF, and CH. Critical revision of the manuscript: AGV, SP, CR, CB, PJ, YB, AH, and GC.

Corresponding author

Correspondence to Christophe Hautier.

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Conflict of interests

The authors have no competing interests, including specific financial interests, relationships, and/or affiliations relevant to the subject matter or materials, included in this manuscript.

Additional information

Communicated by Nicolas Place.

Publisher's Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Philippe Connes and Alexandra Gauthier-Vasserot have equivalent position.

Camille Faes—deceased.

Giovanna Cannas and Christophe Hautier are co-last authors.

Supplementary Information

Below is the link to the electronic supplementary material.

421_2021_4716_MOESM1_ESM.tif

Supplementary file 1: Figure S1A: RMS/RMSmax within each bout of the FAT for each group. Data are presented by Mean ± SD. *: p < 0.05 (TIF 180 KB)

421_2021_4716_MOESM2_ESM.tif

Supplementary file 2: Figure S1B: MPF within each bout of the FAT for each group. Data are presented by Mean ± SD. *: p < 0.05 (TIF 140 KB)

421_2021_4716_MOESM3_ESM.tif

Supplementary file 3: Figure S1C: Typical trace for TSI obtained during a bout of FAT (A). ΔTSI (B) corresponds to the difference between baseline and mean TSI during the plateau phase. Time of deoxygenation (C) is the time between the beginning of the bout and of the plateau phase (TIF 88 KB)

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Gouraud, E., Connes, P., Gauthier-Vasserot, A. et al. Impact of a submaximal mono-articular exercise on the skeletal muscle function of patients with sickle cell disease. Eur J Appl Physiol 121, 2459–2470 (2021). https://doi.org/10.1007/s00421-021-04716-2

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  • DOI: https://doi.org/10.1007/s00421-021-04716-2

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