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INTRODUCTION: Whether cardiac impairment can be fully discarded in McArdle disease-the paradigm of 'exercise intolerance', caused by inherited deficiency of the skeletal muscle-specific glycogen phosphorylase isoform ('myophosphorylase')-remains to be determined. METHODS: Eight patients with McArdle disease and seven age/sex-matched controls performed a 15-minute moderate, constant-load cycle-ergometer exercise bout followed by a maximal ramp test. Electrocardiographic and two-dimensional transthoracic (for cardiac dimension's assessment) and speckle tracking [for left-ventricle global longitudinal (GLS) assessments] echocardiographic evaluations were performed at baseline. Electrocardiographic and GLS assessments were also performed during constant-load exercise and immediately upon maximal exertion. Four human heart biopsies were obtained in individuals without McArdle disease, and in-depth histological/molecular analyses were performed in McArdle and wild-type mouse hearts. RESULTS: Exercise intolerance was confirmed in patients ('second wind' during constant-load exercise, -55% peak power output vs controls). As opposed to controls, patients showed a decrease in GLS during constant-load exercise, especially upon second wind occurrence, but with no other between-group difference in cardiac structure/function. Human cardiac biopsies showed that all three glycogen phosphorylase-myophosphorylase, but also liver and especially brain-isoforms are expressed in the normal adult heart, thereby theoretically compensating for eventual myophosphorylase deficiency. No overall histological (including glycogen depots), cytoskeleton, metabolic or mitochondrial (morphology/network/distribution) differences were found between McArdle and wild-type mouse hearts, except for lower levels of pyruvate kinase M2 and translocase of outer membrane 20 kDa subunit in the former. CONCLUSIONS: This study provides preliminary evidence that cardiac structure and function seem to be preserved in patients with McArdle disease. However, the role for an impaired cardiac contractility associated with the second wind phenomenon should be further explored.
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BACKGROUND & AIMS: Ketone supplementation is gaining popularity. Yet, its effects on exercise performance when muscle glycogen cannot be used remain to be determined. McArdle disease can provide insight into this question, as these patients are unable to obtain energy from muscle glycogen, presenting a severely impaired physical capacity. We therefore aimed to assess the effects of acute ketone supplementation in the absence of muscle glycogen utilization (McArdle disease). METHODS: In a randomized cross-over design, patients with an inherited block in muscle glycogen breakdown (i.e., McArdle disease, n = 8) and healthy controls (n = 7) underwent a submaximal (constant-load) test that was followed by a maximal ramp test, after the ingestion of a placebo or an exogenous ketone ester supplement (30 g of D-beta hydroxybutyrate/D 1,3 butanediol monoester). Patients were also assessed after carbohydrate (75 g) ingestion, which is currently considered best clinical practice in McArdle disease. RESULTS: Ketone supplementation induced ketosis in all participants (blood [ketones] = 3.7 ± 0.9 mM) and modified some gas-exchange responses (notably increasing respiratory exchange ratio, especially in patients). Patients showed an impaired exercise capacity (-65 % peak power output (PPO) compared to controls, p < 0.001) and ketone supplementation resulted in a further impairment (-11.6 % vs. placebo, p = 0.001), with no effects in controls (p = 0.268). In patients, carbohydrate supplementation resulted in a higher PPO compared to ketones (+21.5 %, p = 0.001) and a similar response was observed vs. placebo (+12.6 %, p = 0.057). CONCLUSIONS: In individuals who cannot utilize muscle glycogen but have a preserved ability to oxidize blood-borne glucose and fat (McArdle disease), acute ketone supplementation impairs exercise capacity, whereas carbohydrate ingestion exerts the opposite, beneficial effect.
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Enfermedad del Almacenamiento de Glucógeno Tipo V , Glucógeno , Humanos , Glucemia , Suplementos Dietéticos , Cetonas , Músculos , Estudios CruzadosRESUMEN
McArdle disease is an autosomal recessive inherited disease caused by pathogenic variants in the PYGM gene, resulting in virtual absence of the myophosphorylase enzyme in skeletal muscle. Patients experience physical activity intolerance, muscle pain, and muscle fatigue. This study aimed to investigate other fatigue domains with the Multidimensional Fatigue Inventory (MFI-20) along with an investigation of potential contributing factors, including relevant disease and lifestyle-related factors. We conducted a survey in an international cohort of patients with McArdle disease. The survey included questions on demographics and McArdle disease-related symptoms, and the questionnaires: MFI-20, Insomnia Severity Index (ISI), and International Physical Activity Questionnaire Short-Form (IPAQ-SF). One hundred seventy-four responses were included in the data analyses. We found relatively high fatigue scores in all five domains (general fatigue (12.9 ± 2.2), mental fatigue (10.1 ± 4.1), physical fatigue (13.7 ± 4.1), reduced activity (12.1 ± 4.1), and reduced motivation (10.4 ± 3.4)). Fatigue associated with McArdle symptom severity (p < 0.005), lower levels of physical activity (assessed by IPAQ-SF) (p < 0.05), and poor sleep (assessed by ISI) (p < 0.05). These findings call for clinical focus and future research into fatigue, sleep and mental health in patients with McArdle disease.
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Glucógeno Fosforilasa de Forma Muscular , Enfermedad del Almacenamiento de Glucógeno Tipo V , Humanos , Enfermedad del Almacenamiento de Glucógeno Tipo V/complicaciones , Enfermedad del Almacenamiento de Glucógeno Tipo V/genética , Enfermedad del Almacenamiento de Glucógeno Tipo V/diagnóstico , Glucógeno Fosforilasa de Forma Muscular/genética , Músculo Esquelético/patología , Encuestas y Cuestionarios , InternetRESUMEN
BACKGROUND: This study aimed to determine the effect of different carbohydrate (CHO) doses on exercise capacity in patients with McArdle disease-the paradigm of "exercise intolerance", characterized by complete muscle glycogen unavailability-and to determine whether higher exogenous glucose levels affect metabolic responses at the McArdle muscle cell (in vitro) level. METHODS: Patients with McArdle disease (nâ¯=â¯8) and healthy controls (nâ¯=â¯9) underwent a 12-min submaximal cycling constant-load bout followed by a maximal ramp test 15 min after ingesting a non-caloric placebo. In a randomized, double-blinded, cross-over design, patients repeated the tests after consuming either 75 g or 150 g of CHO (glucose:fructose = 2:1). Cardiorespiratory, biochemical, perceptual, and electromyographic (EMG) variables were assessed. Additionally, glucose uptake and lactate appearance were studied in vitro in wild-type and McArdle mouse myotubes cultured with increasing glucose concentrations (0.35, 1.00, 4.50, and 10.00 g/L). RESULTS: Compared with controls, patients showed the "classical" second-wind phenomenon (after prior disproportionate tachycardia, myalgia, and excess electromyographic activity during submaximal exercise, all p < 0.05) and an impaired endurance exercise capacity (-51% ventilatory threshold and -55% peak power output, both p < 0.001). Regardless of the CHO dose (p < 0.05 for both doses compared with the placebo), CHO intake increased blood glucose and lactate levels, decreased fat oxidation rates, and attenuated the second wind in the patients. However, only the higher dose increased ventilatory threshold (+27%, pâ¯=â¯0.010) and peak power output (+18%, pâ¯=â¯0.007). In vitro analyses revealed no differences in lactate levels across glucose concentrations in wild-type myotubes, whereas a dose-response effect was observed in McArdle myotubes. CONCLUSION: CHO intake exerts beneficial effects on exercise capacity in McArdle disease, a condition associated with total muscle glycogen unavailability. Some of these benefits were dose dependent.
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BACKGROUND: Melatonin is a hormone that has shown anti-inflammatory actions, reduced oxidative stress, and has effects on physical performance, so the aim of this study was to review the effects of melatonin supplementation on the performance of professional soccer players. METHODS: Critical and systematic review. Data were obtained by performing searches in the following bibliographic databases: Web of Science, MEDLINE (via PubMed), Embase, Cochrane Library, and Scopus. The terms used were "Soccer Athlete", "Melatonin", and "Soccer Performance", using "Humans" as a filter. The search update was in May 2023. RESULTS: Having applied the inclusion and exclusion criteria, eight articles were selected out of 59 retrieved references. The dose of melatonin administered in the studies ranged between 5 and 8 mg. The outcomes showed a decrease in oxidative stress, muscle damage, and inflammatory markers in the melatonin-treated group. CONCLUSIONS: Exogenously administered melatonin seems to attenuate some of the effects derived from physical exercise, such as oxidative stress, inflammation, and muscle damage, in professional football players, and since it has no potential adverse effects, it could be interesting to apply it in this population. However, the direct effects of melatonin supplementation on physical performance have not been demonstrated, so more research is needed on the intervention period and effective dose and with larger participant populations.
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Melatonina , Fútbol , Humanos , Fútbol/fisiología , Melatonina/farmacología , Estrés Oxidativo , Suplementos DietéticosRESUMEN
BACKGROUND: The European registry for individuals with GSD5 and other muscle glycogenosis (EUROMAC) was launched to register rare muscle glycogenosis in Europe, to facilitate recruitment for research trials and to learn about the phenotypes and disseminate knowledge about the diseases. A network of twenty collaborating partners from eight European countries and the US contributed data on rare muscle glycogenosis in the EUROMAC registry. METHODS: Following the initial report on demographics, neuromuscular features and comorbidity (2020), we here present the data on social participation, previous and current treatments (medication, supplements, diet and rehabilitation) and limitations. Furthermore, the following questionnaires were used: Fatigue severity scale (FSS), WHO Disability Assessment Scale (DAS 2.0), health related quality of life (SF36) and International Physical Activity Questionnaire (IPAQ). RESULTS: Of 282 participants with confirmed diagnoses of muscle glycogenosis, 269 had GSD5. Of them 196 (73%) completed all questionnaires; for the others, the data were incomplete. The majority, 180 (67%) were currently working. Previous medical treatments included pain medication (23%) and rehabilitation treatment (60%). The carbohydrate-rich diet was reported to be beneficial for 68%, the low sucrose diet for 76% and the ketogenic diet for 88%. Almost all participants (93%) reported difficulties climbing stairs. The median FSS score was 5.22, indicating severe fatigue. The data from the WHODAS and IPAQ was not of sufficient quality to be interpreted. CONCLUSIONS: The EUROMAC registry have provided insight into the functional and social status of participants with GSD5: most participants are socially active despite limitations in physical and daily life activities. Regular physical activity and different dietary approaches may alleviate fatigue and pain.
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Enfermedad del Almacenamiento de Glucógeno Tipo V , Enfermedad del Almacenamiento de Glucógeno , Humanos , Calidad de Vida , Participación Social , Estado Funcional , FatigaRESUMEN
Over the years, soccer has become more physically demanding; the number and frequency of high-intensity actions have increased, and these activities are decisive in determining the match outcome. Importantly, the reductionist approach commonly used to analyze high-intensity actions does not contemplate a more contextualized perspective on soccer performance. Traditionally, most investigations have only provided quantitative data regarding sprints (i. e. time, distances, frequency) without examining "how" (e. g. type of trajectory or starting position) and "why" (e. g. tactical role) soccer players sprint. In fact, other high-intensity actions, apart from running, are not even mentioned (i. e. curve sprints, change of direction, and specific-jump tasks). This has led to the use of tests and interventions that do not accurately reflect real game actions. Given the true technical-tactical-physical demands of each playing position, this narrative review collected a wide-spectrum of current soccer-related articles and provided a discussion regarding high-intensity actions, with a positional-based approach. In this narrative review, practitioners are encouraged to contemplate and consider the different elements that characterize high-intensity actions in soccer, in order to assess and train soccer players under a more sport-specific and integrative perspective.
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Rendimiento Atlético , Carrera , Fútbol , HumanosRESUMEN
The low-carbohydrate ketogenic diet (LCKD) has attracted increased attention in recent years as a potential treatment option for individuals with McArdle disease (glycogen storage disease type V), and despite the absence of strong scientific evidence of the LCKD's benefits, increased numbers of individuals with McArdle disease have tried a LCKD. The objective of this study was to collect patient-reported experiences with a LCKD. We aimed to estimate the immediate prevalence of individuals that had tried a LCKD in an international McArdle disease cohort, and we aimed to report on the patient-reported experiences with the diet, both positive and negative. A total of 183 responses were collected from individuals with McArdle disease from 18 countries. We found that one-third of the cohort had tried a LCKD, and almost 90% experienced some degree of positive effect, with the most prominent effects on McArdle disease-related core symptoms (e.g., activity intolerance, muscle pain, and muscle fatigue). Adverse effects were rare and generally rated as mild to moderate. These patient-reported findings underline the need for randomized clinical trials to decisively determine if a LCKD is a suitable nutritional strategy for patients with McArdle disease. The results from this study can prompt and contribute to the design of such a clinical trial.
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Dieta Cetogénica , Enfermedad del Almacenamiento de Glucógeno Tipo V , Humanos , Enfermedad del Almacenamiento de Glucógeno Tipo V/tratamiento farmacológico , Dieta Cetogénica/métodos , Dieta Baja en Carbohidratos/métodos , Cuerpos Cetónicos , Medición de Resultados Informados por el Paciente , CarbohidratosRESUMEN
PURPOSE: To assess durability in professional cyclists, as well as potential associated indicators. METHODS: Twelve male professional cyclists participated in the study (age: 26 [5] y, VO2max: 83.0 [3.6] mL·kg-1·min-1). They performed a 20-minute time trial (TT) on 2 different sessions separated by a 48-hour period: (1) with no previous fatigue (TTFresh) and (2) immediately after a long submaximal ride (approximately 4 h, 40 kJ/kg) (TTFatigue). We then assessed the decay (in percentage) in mean power output (PO) from TTFresh to TTFatigue and its association with different laboratory-based endurance indicators (ventilatory threshold, peak PO, and VO2max) determined through a previous maximal incremental cycling test, as well as with training loads during the 4 weeks preceding the TTs. RESULTS: While no differences were noted in the average heart rate (177 [7] vs 176 [6] beats·min-1, P = .118), there was a significant decay in PO between TTFresh and TTFatigue (386 [29] W vs 375 [28] W [-2.9%], respectively; P = .007), albeit with signs of interindividual variability (range = -8.5% to 1.1%; coefficient of variation = 105%). No significant associations were found between the PO decay and any of the analyzed indicators (all P > .05). CONCLUSIONS: Performance is significantly impaired after a certain amount of work completed (approximately 40 kJ·kg-1) in professional cyclists, and the magnitude of this impairment seems to be not related to "traditional" laboratory-based endurance indicators or to markers of training load. These findings might support the need for specifically assessing durability in cyclists and confirming potential determinants of this parameter.
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Consumo de Oxígeno , Resistencia Física , Humanos , Masculino , Adulto , Ciclismo , FatigaRESUMEN
Carbohydrate availability affects fat metabolism during exercise; however, the effects of complete muscle glycogen unavailability on maximal fat oxidation (MFO) rate remain unknown. Our purpose was to examine the MFO rate in patients with McArdle disease, comprising an inherited condition caused by complete blockade of muscle glycogen metabolism, compared to healthy controls. Nine patients (three women, aged 36 ± 12 years) and 12 healthy controls (four women, aged 40 ± 13 years) were studied. Several molecular markers of lipid transport/metabolism were also determined in skeletal muscle (gastrocnemius) and white adipose tissue of McArdle (Pygm p.50R*/p.50R*) and wild-type male mice. Peak oxygen uptake ( V Ì O 2 peak ${\dot V_{{{\rm{O}}_{\rm{2}}}{\rm{peak}}}}$ ), MFO rate, the exercise intensity eliciting MFO rate (FATmax) and the MFO rate-associated workload were determined by indirect calorimetry during an incremental cycle-ergometer test. Despite having a much lower V Ì O 2 peak ${\dot V_{{{\rm{O}}_{\rm{2}}}{\rm{peak}}}}$ (24.7 ± 4 vs. 42.5 ± 11.4 mL kg-1 min-1 , respectively; P < 0.0001), patients showed considerably higher values for the MFO rate (0.53 ± 0.12 vs. 0.33 ± 0.10 g min-1 , P = 0.001), and for the FATmax (94.4 ± 7.2 vs. 41.3 ± 9.1 % of V Ì O 2 peak ${\dot V_{{{\rm{O}}_{\rm{2}}}{\rm{peak}}}}$ , P < 0.0001) and MFO rate-associated workload (1.33 ± 0.35 vs. 0.81 ± 0.54 W kg-1 , P = 0.020) than controls. No between-group differences were found overall in molecular markers of lipid transport/metabolism in mice. In summary, patients with McArdle disease show an exceptionally high MFO rate, which they attained at near-maximal exercise capacity. Pending more mechanistic explanations, these findings support the influence of glycogen availability on MFO rate and suggest that these patients develop a unique fat oxidation capacity, possibly as an adaptation to compensate for the inherited blockade in glycogen metabolism, and point to MFO rate as a potential limiting factor of exercise tolerance in this disease. KEY POINTS: Physically active McArdle patients show an exceptional fat oxidation capacity. Maximal fat oxidation rate occurs near-maximal exercise capacity in these patients. McArdle patients' exercise tolerance might rely on maximal fat oxidation rate capacity. Hyperpnoea might cloud substrate oxidation measurements in some patients. An animal model revealed overall no higher molecular markers of lipid transport/metabolism.
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Enfermedad del Almacenamiento de Glucógeno Tipo V , Masculino , Femenino , Animales , Ratones , Enfermedad del Almacenamiento de Glucógeno Tipo V/metabolismo , Glucógeno/metabolismo , Oxidación-Reducción , Músculo Esquelético/fisiología , Prueba de Esfuerzo , Lípidos , Consumo de Oxígeno/fisiología , Tejido Adiposo/metabolismoRESUMEN
The purpose of this study was to analyze the influence of tactical behavior on physical performance of the three best Spanish soccer teams. Team 1 employed a 1-4-4-2 formation with compact defence and direct attacks, Team 2 employed an indirect style of play with a 1-4-3-3 formation and Team 3 used a 1-4-3-3 formation with elaborate attacks and strong counter-attacks. 816 individual situations of 54 professional soccer players categorized as fullbacks (FB), central defenders (CD), central midfielders (MF), wide midfielders (WM), and strikers (S) were included in the analysis. Their performance was examined with a player-tracking system. The analyzed variables included total distance covered, distance covered above 14, 21 and 24 km·h-1, as well as distance covered in possession of the ball and without possession of the ball. Team 2 covered a substantially lower distance >14 km·h-1 than Team 1 and substantially lower distances >14, >21, and >25km·h-1 than Team 3. No differences in running activity were found between Teams 1 and 3. However, there were substantial differences between the specific positions of the three teams. CD in Team 1 covered the lowest distance by a substantial margin in almost all variables analysed, whereas MF travelled substantially greater distances than did other positions. WM in Team 1 covered the greatest distance >18 and >21km·h- 1, while S in Team 2 had the lowest distance covered in almost all the variables, and FB in Team 3 showed the lowest total distance covered and distance covered >14km·h-1. WM in Team 2 had the greatest distance covered in possession, while S in Team 3 had the greatest distance covered out of possession. The results indicate that different team formations and associated tactical demands have a significant influence on running performance.
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The purpose of this study was to compare the external load in competitive (official) and non-competitive matches (friendly, training and modified-sided games) in professional soccer players. Time-motion data for 10 elite male soccer players (age = 20.1 ± 2.1 years; body height = 178.8 ± 5.9; body mass = 71.4 ± 7.3; % body fat = 11.0 ± 1.1 and VO2max = 55.96 ± 3.3) from a professional Spanish first division team were recorded during official (n = 12), friendly (n = 7) and training (n = 6) matches and a 5 vs. 5 + goalkeepers modified-sided game (n = 3). GPS devices were used to monitor players' external loads: total distance covered, distance covered at different speeds (<13.9 km·h-1, >14, >18, >21 and >25 km·h-1), peak speed (km·h-1), and the number of accelerations and decelerations (1.5-2.5 m·s-2, 2.5-4 m·s-2 and 4-8 m·s-2). One-way analysis of variance of the magnitude-based inference was used to determine differences between matches. Data indicated that official matches scored statistically higher peak speeds (ES = 1.40-2.20). In modified-sided games more total distance was covered at <13.9 km·h-1 and >14 km·h-1 than in regular matches (ES = 0.72-2.21), but lower distances were covered at >21 km·h-1 and >25 km·h-1 than in official and friendly matches (ES = 0.51-2.53) and at >25 km·h-1 than in training matches (ES = 0.92). Likewise, the modified-side games showed a greater number of accelerations and decelerations than other types of matches (ES = 1.46-2.51). This work shows that friendly and training matches, in conjunction with modified-side games, are suitable tools to prepare soccer players for official matches.
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Purpose: To compare endurance, strength and body composition indicators between cyclists of three different competition age categories. Methods: Fifty-one male road cyclists classified as either junior (n = 13, age 16.4 ± 0.5 years), under-23 [(U23), n = 24, 19.2 ± 1.3 years] or professional (n = 14, 26.1 ± 4.8 years) were studied. Endurance (assessed through a maximal incremental test and an 8-minute time-trial), strength/power (assessed through incremental loading tests for the squat, lunge and hip thrust exercises) and body composition (assessed through dual energy X-ray absorptiometry) were determined on three different testing sessions. Results: U23 and, particularly professional, cyclists attained significantly (p < 0.05) higher values than juniors for most of the analyzed endurance indicators [time-trial performance, maximum oxygen uptake (VO2max), peak power output (PPO), respiratory compensation point (RCP), and ventilatory threshold (VT)]. Significant differences (p < 0.05) between U23 and professionals were also found for time-trial performance, PPO and VT, but not for other markers such as VO2max or RCP. Professional cyclists also showed significantly (p < 0.05) lower relative fat mass and higher muscle mass levels than U23 and, particularly, juniors. No consistent differences between age categories were found for muscle strength/power indicators. Conclusion: Endurance (particularly time-trial performance, PPO and VT) and body composition (fat and muscle mass) appear as factors that best differentiate between cyclists of different age categories, whereas no consistent differences are found for muscle strength/power. These findings might help in performance prediction and/or talent identification and may aid in guiding coaches in the design of training programs focused on improving those variables that appear more determinant.
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Glycogen storage disease type V (GSDV, McArdle disease) is a rare genetic myopathy caused by deficiency of the muscle isoform of glycogen phosphorylase (PYGM). This results in a block in the use of muscle glycogen as an energetic substrate, with subsequent exercise intolerance. The pathobiology of GSDV is still not fully understood, especially with regard to some features such as persistent muscle damage (i.e., even without prior exercise). We aimed at identifying potential muscle protein biomarkers of GSDV by analyzing the muscle proteome and the molecular networks associated with muscle dysfunction in these patients. Muscle biopsies from eight patients and eight healthy controls showing none of the features of McArdle disease, such as frequent contractures and persistent muscle damage, were studied by quantitative protein expression using isobaric tags for relative and absolute quantitation (iTRAQ) followed by artificial neuronal networks (ANNs) and topology analysis. Protein candidate validation was performed by Western blot. Several proteins predominantly involved in the process of muscle contraction and/or calcium homeostasis, such as myosin, sarcoplasmic/endoplasmic reticulum calcium ATPase 1, tropomyosin alpha-1 chain, troponin isoforms, and alpha-actinin-3, showed significantly lower expression levels in the muscle of GSDV patients. These proteins could be potential biomarkers of the persistent muscle damage in the absence of prior exertion reported in GSDV patients. Further studies are needed to elucidate the molecular mechanisms by which PYGM controls the expression of these proteins.
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Enfermedad del Almacenamiento de Glucógeno Tipo V , Proteoma , Biomarcadores/metabolismo , Glucógeno/metabolismo , Enfermedad del Almacenamiento de Glucógeno Tipo V/genética , Humanos , Músculo Esquelético/metabolismo , Isoformas de Proteínas/metabolismo , Proteoma/metabolismoRESUMEN
OBJECTIVE: In soccer, vertical jump means jumping toward a ball. Since no vertical jump test includes the ball as a reference element, the effect that the ball would have in a vertical jump test is unknown. The aim of this study was to examine the biomechanical differences between run-up vertical jump measurements without (Run-up Vertical Jump) and with ball inclusion (Heading Test). METHODS: Twelve semi- and professional soccer players were recruited. Athletes performed both jump tests in a biomechanical laboratory, where kinetic and spatiotemporal variables were collected and compared using a Student's dependent t-test for paired samples. RESULTS: Overall, players performed a different jumping strategy during the heading test compared to the run-up vertical jump, exhibiting: 1) higher horizontal velocity during initial contact (+45.3%, P ≤ .001), 2) shorter contact time, greater rate of force development, and total impulse during push-off (+27.5%, +53%, and +10.6%, respectively, P ≤ .008), 3) higher CoM horizontal and resultant velocity during take-off (+76.1% and 20.5%, respectively, P ≤ .001), 4) better vertical jump performance (+4.3%, P ≤ .0001), and 5) larger body angle rotation during landing (+63.3%, P = .006), compared to run-up vertical jump (effect size: 0.78 to 3.7). CONCLUSION: In general, soccer players display greater vertical jump heights in heading test, which highlights the importance of including an overhead ball during soccer-specific jump tests. Coaches and practitioners are encouraged to assess, and perhaps develop, the jumping ability of soccer players using a suspended ball as a specific target.
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Rendimiento Atlético , Fútbol , Atletas , Humanos , Cinética , RotaciónRESUMEN
INTRODUCTION: The long-term effects of exercise in patients with McArdle disease-the paradigm of "exercise intolerance"-are unknown. This is an important question because the severity of the disease frequently increases with time. PURPOSE: This study aimed to study the effects of a long-term exercise intervention on clinical and fitness-related outcomes in McArdle patients. METHODS: Seventeen patients (exercise group: n = 10, 6 male, 38 ± 18 yr; control: n = 7, 4 male, 38 ± 18 yr) participated in a 2-yr unsupervised intervention including moderate-intensity aerobic (cycle-ergometer exercise for 1 h) and resistance (high load-low repetition circuit) training on 5 and 2-3 d·wk -1 , respectively. Patients were assessed at baseline and postintervention. Besides safety, outcomes included clinical severity (e.g., exercise intolerance features) on a 0-3 scale (primary outcome), and aerobic fitness, gross muscle efficiency, and body composition (total/regional fat, muscle, and bone mass; secondary outcomes). RESULTS: The exercise program was safe and resulted in a reduction of 1 point (-1.0; 95% confidence interval, -1.6 to -0.5; P = 0.025) in clinical severity versus the control group, with 60% of participants in the exercise group becoming virtually asymptomatic and with no functional limitation in daily life activities. Compared with controls, the intervention induced significant and large benefits (all P < 0.05) in the workload eliciting the ventilatory threshold (both in absolute (watts, +37%) and relative units (watts per kilogram of total body mass or of lower-limb muscle mass, +44%)), peak oxygen uptake (in milliliters per kilogram per minute, +28%), and peak workload (in absolute (+27%) and relative units (+33%)). However, no significant changes were found for muscle efficiency or for any measure of body composition. CONCLUSIONS: A 2-yr unsupervised intervention including aerobic and resistance exercise is safe and induces major benefits in the clinical course and aerobic fitness of patients with McArdle disease.
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Enfermedad del Almacenamiento de Glucógeno Tipo V , Densidad Ósea , Ejercicio Físico , Terapia por Ejercicio/métodos , Enfermedad del Almacenamiento de Glucógeno Tipo V/terapia , Humanos , MasculinoRESUMEN
Research has shown that soccer players regularly execute curved sprints during matches. The purpose of this study was to determine the age-related effects on curve sprint (CS) performance to both sides, asymmetry, and association with linear sprint (LS). Eighty-four soccer players (aged 16.1 ± 1.6 categorized in U15, U17, and U20) were recruited, who performed CS and LS tests. One-way analysis of variance (ANOVA) and effect size (ES) were used to compare CS performance between age categories, and relationships between physical performance measures were calculated using Pearson's correlation coefficient. The main findings of this study were that: 1) there were significant differences in the "good" side CS among age groups (p < 0.001; ES from moderate to large), but not in the "weak" side CS, 2) curve asymmetry was significantly higher in U20 than U15 (p < 0.05; ES large) and U17 players (p < 0.05; ES moderate), and 3) relationships between CS and LS times decreased with age (from significant and very large [p < 0.001] to non-significant and smallmoderate [p > 0.05]). This study highlights the importance of assessing and training CS in different age categories, an action that becomes less correlated with LS as age increases, with the aim of mitigating the increase in asymmetries as a result of the specialization process, focusing interventions mainly on improving the CS "weak" side.
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The aims of this study were (a) to assess intra-session reliability and usefulness of the soccer-specific maximum vertical jump (heading test, HT) and (b) to analyse the validity of the easy-to-use and cost-effective instrument (smartphone camera, MOB) compared with gold-standard instrument (3D motion capture system, MOCAP) to obtain the vertical jump performance during HT. Twelve semi-professional high-level and fifteen amateur soccer male players (23.9 ± 3.6 years) performed three HT attempts, and kinematic data were recorded with MOB and MOCAP. Intra-class correlation coefficient (ICC) and coefficient of variation (CV) were used as measures of intra-session reliability. T-test with Cohen's effect size (ES), Pearson's product moment and Bland-Altman analysis were used to obtain MOB validity. Regarding intra-session reliability, the CV was 1.13%, and ICC was 0.98, considered acceptable. Respecting validity criteria did not reveal significant differences (p < 0.05; effect size = 0.06, considered trivial), 'almost perfect' correlation (Pearson) (r = 0.98; p < 0.05), and strong agreement were obtained between MOB and MOCAP. This finding showed a test (HT) with a specific character, using cost-effective instrument and applicable to all soccer fields (adjusted to the standardised lines in the soccer field), all of them backed-up by reliability, usefulness and validity criteria.
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Background: The "second wind" (SW) phenomenon-commonly referring to both an initial period of marked intolerance to dynamic exercise (e.g., brisk walking) that is not followed by perceived improvement and disappearance of previous tachycardia (i.e., the actual "SW") until 6-10 min has elapsed-is an almost unique feature of McArdle disease that limits adherence to an active lifestyle. In this regard, an increase in the workload eliciting the SW could potentially translate into an improved patients' exercise tolerance in daily life. We aimed to determine whether aerobic fitness and physical activity (PA) levels are correlated with the minimum workload eliciting the SW in McArdle patients-as well as with the corresponding heart rate value. We also compared the SW variables and aerobic fitness indicators in inactive vs. active patients. Methods: Fifty-four McArdle patients (24 women, mean ± SD age 33 ± 12 years) performed 12-min constant-load and maximum ramp-like cycle-ergometer tests for SW detection and aerobic fitness [peak oxygen uptake (VO2 peak) and workload and ventilatory threshold] determination, respectively. They were categorized as physically active/inactive during the prior 6 months (active = reporting ≥150 min/week or ≥75 min/week in moderate or vigorous-intensity aerobic PA, respectively) and were also asked on their self-report of the SW. Results: Both peak and submaximal indicators of aerobic fitness obtained in the ramp tests were significantly correlated with the workload of the SW test, with a particularly strong correlation for the VO2 peak and peak workload attained by the patients (both Pearson's coefficients > 0.70). Twenty (seven women) and 24 patients (18 women) were categorized as physically active and inactive, respectively. Not only the aerobic fitness level [â¼18-19% higher values of VO2 peak (mlâ kg-1â min-1)] but also the workload of the SW tests was significantly higher in active than in inactive patients. All the inactive patients reported that they experienced the SW during walking/brisk walking in daily life, whereas active patients only reported experiencing this phenomenon during more strenuous activities (very brisk walking/jogging and bicycling). Conclusion: A higher aerobic fitness and an active lifestyle are associated with a higher workload eliciting the so-called SW phenomenon in patients with McArdle disease, which has a positive impact on their exercise tolerance during daily living.
RESUMEN
McArdle disease is an autosomal recessive disorder of muscle glycogen metabolism caused by pathogenic mutations in the PYGM gene, which encodes the skeletal muscle-specific isoform of glycogen phosphorylase. Clinical symptoms are mainly characterized by transient acute "crises" of early fatigue, myalgia and contractures, which can be accompanied by rhabdomyolysis. Owing to the difficulty of performing mechanistic studies in patients that often rely on invasive techniques, preclinical models have been used for decades, thereby contributing to gain insight into the pathophysiology and pathobiology of human diseases. In the present work, we describe the existing in vitro and in vivo preclinical models for McArdle disease and review the insights these models have provided. In addition, despite presenting some differences with the typical patient's phenotype, these models allow for a deep study of the different features of the disease while representing a necessary preclinical step to assess the efficacy and safety of possible treatments before they are tested in patients.