Absence of calf muscle metabolism alterations in active cystic fibrosis adults with mild to moderate lung disease.
Specific alterations in skeletal muscle related to genetic defects may be present in adults with cystic fibrosis (CF). Limb muscle dysfunction may contribute to physical impairment in CF.



We hypothesized that adults with CF would have altered calf muscle metabolism during exercise.



Fifteen adults with CF and fifteen healthy controls matched for age, gender and physical activity performed a maximal cycling test and an evaluation of calf muscle energetics by31P magnetic resonance spectroscopy before, during and after plantar flexions to exhaustion.



Maximal cycling test revealed lower exercise capacities in CF (VO2peak2.44±0.11 vs. 3.44±0.23L·Min-1, P=0.03). At rest, calf muscle phosphorus metabolites and pHi were similar in CF and controls (P>0.05). Maximal power output during plantar flexions was significantly lower in CF compared to controls (7.8±1.2 vs. 6.6±2.4W; P=0.013). At exhaustion, PCr concentration was similarly reduced in both groups (CF -33±7%, controls -34±6%, P=0.44), while PCr degradation at identical absolute workload was greater in CF patients (P=0.04). These differences disappeared when power output was normalized for differences in calf size (maximal power output: 0.10±0.02 vs. 0.10±0.03W/cm2; P=0.87). Pi/PCr ratio and pHi during exercise as well as PCr recovery after exercise were similar between groups.



Similar metabolic calf muscle responses during exercise and recovery were found in CF adults and controls. Overall, muscle anabolism rather than specific metabolic dysfunction may be critical regarding muscle function in CF.
HP2 Laboratory, Grenoble-Alpes University, 38000 Grenoble, France; INSERM, U1042, 38000, Grenoble, France. Electronic address: ndecorte@chu-grenoble.fr.
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Decorte
HP2 Laboratory, Grenoble-Alpes University, 38000 Grenoble, France; INSERM, U1042, 38000, Grenoble, France; LAMHESS EA 6312, Universities of Toulon and Nice Sophia-Antipolis, France.
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Gruet
Centre de ressources et de compétences de la mucoviscidose (CRCM), Clinique Universitaire de Pneumologie, Pôle Thorax et Vaisseaux, CHU Grenoble, France.
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Camara
Centre de ressources et de compétences de la mucoviscidose (CRCM), Clinique Universitaire de Pneumologie, Pôle Thorax et Vaisseaux, CHU Grenoble, France.
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Quetant
LAMHESS EA 6312, Universities of Toulon and Nice Sophia-Antipolis, France; Centre de ressources et de compétences de la mucoviscidose (CRCM), Hôpital Renée Sabran, Giens, France.
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Mely
LAMHESS EA 6312, Universities of Toulon and Nice Sophia-Antipolis, France.
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Vallier
HP2 Laboratory, Grenoble-Alpes University, 38000 Grenoble, France; INSERM, U1042, 38000, Grenoble, France.
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Verges
HP2 Laboratory, Grenoble-Alpes University, 38000 Grenoble, France; INSERM, U1042, 38000, Grenoble, France.
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Wuyam
D000328:Adult; D003550:Cystic Fibrosis / Q000150:complications / Q000175:diagnosis / Q000503:physiopathology; D004734:Energy Metabolism; D005080:Exercise Test / Q000379:methods; D017079:Exercise Tolerance; D005260:Female; D006801:Humans; D035002:Lower Extremity / Q000503:physiopathology*; D008171:Lung Diseases / Q000209:etiology / Q000503:physiopathology; D009682:Magnetic Resonance Spectroscopy / Q000379:methods; D008297:Male; D018482:Muscle, Skeletal / Q000378:metabolism / Q000503:physiopathology; D010101:Oxygen Consumption; D062072:Patient Acuity; D013223:Statistics as Topic
(31)P MR spectroscopy; Cystic fibrosis; Exercise tolerance; Muscle metabolism