Skeletal Muscle Metabolism in Cystic Fibrosis and Primary Ciliary Dyskinesia

Wells, Greg D.; Wilkes, Donna L.; Schneiderman, Jane E.; Rayner, Tammy; Elmi, Maryam; Selvadurai, Hiran; Dell, Sharon D.; Noseworthy, Michael D.; Ratjen, Félix; Tein, Ingrid; Coates, Allan L.

doi:10.1203/pdr.0b013e3181fff35f

Cited by 61 publications

(115 citation statements)

References 38 publications

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“…An a priori sample size of 16 participants per group was calculated for determination for half-time PCr recovery, with a minimum detectable difference of 9 and SD of 9; power was set at 0.8 and ␣ ϭ .05. For secondary outcomes, including changes in pH after exercise (resting pH Ϫ end-exercise pH) values from HC children collected with similar protocols and equipment, we calculated that 12 participants per group would be sufficient to detect an effect size difference of 0.2, with an SD of 0.15 in pH change, at a significance of ␣ ϭ .05 and power of 0.8 (26).…”

Section: Discussionmentioning

confidence: 99%

Skeletal Muscle Abnormalities in Girls and Adolescents With Turner Syndrome

Wells

O’Gorman

Rayner

et al. 2013

The Journal of Clinical Endocrinology &Amp; Metabolism

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Context:Turner syndrome (TS) is a chromosomal disorder occurring in approximately 1 in 2500 live births. Individuals with TS report lower levels of physical activity than healthy control (HC) subjects. Cardiorespiratory limitations may contribute to the observed reduction in physical activity.Objective: The objective of this study was to compare muscle metabolism of patients with TS vs HC subjects before and after exercise using exercise testing, magnetic resonance imaging, and magnetic resonance spectroscopy techniques. Design:We hypothesized that girls and adolescents with TS would have muscle metabolic abnormalities not present in the HC population. Setting:The research was conducted at the Hospital for Sick Children in Toronto, Ontario, Canada.Participants: Fifteen participants with TS were age-, activity-, and body mass index Z-scorematched with 16 HC subjects. Main Outcome Measures:31 P magnetic resonance spectroscopy was used to characterize muscle metabolism at rest and after 30 seconds of high-intensity exercise, 60 seconds of moderate-intensity exercise, and 5 minutes of low-intensity exercise.Results: While achieving the same workloads, participants with TS exhibited a greater difference between rest and end-exercise pH compared with HC subjects after 30 seconds (TS, 0.29 Ϯ 0.04; HC, 0.21 Ϯ 0.08; P ϭ .03) and 90 seconds (TS, 0.47 Ϯ 0.22; HC, 0.32 Ϯ 0.13; P ϭ .02) of exercise. During the 5-minute exercise test, similar workloads were achieved between groups; however, ATP production was greater in participants with TS vs the HC subjects via all 3 bioenergetic pathways (total ATP: TS, 0.90 Ϯ 0.34; HC, 0.60 Ϯ 0.25; P ϭ .01). Conclusions:The results of this study suggest that patients with TS exhibit greater anaerobic stress during exercise than HC subjects, which may lead to symptoms of increased muscle fatigue with short bursts of activity. Recovery metabolism after exercise appears to be similar between participants with TS and HC subjects, which is suggestive of normal mitochondrial metabolism and oxygen transport. (J Clin Endocrinol Metab 98:

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Section: Discussionmentioning

confidence: 99%

Skeletal Muscle Abnormalities in Girls and Adolescents With Turner Syndrome

Wells

O’Gorman

Rayner

et al. 2013

The Journal of Clinical Endocrinology &Amp; Metabolism

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“…However, muscle metabolism seems to be impaired, especially during high-intensity exercises [35]. Malnutrition and electrolyte disturbances [36], physical inactivity [13,25], systemic inflammation [37], oxidative stress [38], oral corticosteroid use [39] and CF-specific gene defects [35,40,41] are suggested to be involved in muscle dysfunction. Frequent exacerbations might further play a role in the long-term development of muscle dysfunction.…”

Section: Reasons For a Reduced Exercise Capacitymentioning

confidence: 99%

Rehabilitation in Patients with Chronic Respiratory Disease Other than Chronic Obstructive Pulmonary Disease: Exercise and Physical Activity Interventions in Cystic Fibrosis and Non-Cystic Fibrosis Bronchiectasis

Burtin

Hebestreit

2015

Respiration

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A relevant proportion of children and adults with cystic fibrosis (CF) have a marked decrease in exercise tolerance, which can be partly related to impaired muscle function and decreased physical activity levels in daily life, in addition to lung disease. Preliminary findings suggest that patients with non-CF bronchiectasis face the same problems. These patients might be excellent candidates for exercise and physical activity interventions. This review elaborates on the rationale for exercise training and activity behaviour changes and summarizes the existing evidence for these rehabilitation strategies in patients with bronchiectasis, both CF and non-CF bronchiectasis. Furthermore, practical considerations and safety aspects are discussed.

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“…Thus, the hypothesis of specific physiological impairments in the CF skeletal muscles can be raised. Wells et al [9] found abnormalities in muscle metabolism in CF children as compared with age, sex and habitual physical activity (PA)-matched healthy controls, suggesting intrinsic dysfunction of skeletal muscle function. Lamhonwah et al [10] recently demonstrated that CF transmembrane conductance regulator (CFTR) is expressed at the sarcoplasmic reticulum of human skeletal muscle.…”

Section: Introductionmentioning

confidence: 99%