Analysis of Body Fluid Distribution, Phase Angle and Its Association With Maximal Oxygen Consumption in Facioscapulohumeral Dystrophy: An Observational Study.

Background And Aims: Body composition parameters associated with aerobic fitness, mirrored by maximal oxygen consumption (V̇Omax), have recently gained interest as indicators of physical efficiency in facioscapulohumeral dystrophy (FSHD). Bioimpedance analysis (BIA) allows a noninvasive and repeatable estimate of body composition but is based on the use of predictive equations which, if used in cohorts with different characteristics from those for which the equation was originally formulated, could give biased results. Instead, the phase angle (PhA), a BIA raw bioelectrical parameter reflecting body fluids distribution, could provide reliable data for such analysis.

Cross-cultural translation, validation, and responsiveness of the Pittsburgh Fatigability Scale - Italian version (PFS-I) in a mixed-sample of older adults.

Introduction: Older adults experience fatigue which impacts health-related quality of life. The Pittsburgh Fatigability Scale (PFS) was specifically designed to assess perceived physical and mental fatigability in older adults. The aim of this study was to translate the PFS into Italian (PFS-I) and to investigate its psychometric properties.

The first case of a competitive basketball player affected by carnitine palmitoyl transferase II deficiency presenting an undescribed compound heterozygous genetic mutation.

Purpose: The risk of exercise-induced rhabdomyolysis, followed by abrupt creatine kinase (CK) augmentation, associated with carnitine palmitoyl transferase II (CPTII) deficiency causes patients to abstain from physical training. However, the exercise adjustment to the disease-induced metabolic impairment, accompanied by a tailored nutritional and supplementation strategy, could make sporting activity feasible, even at a competitive level. Here, we report the case of an 18-year-old male basketball player at a competitive level diagnosed for CPTII deficiency after a rhabdomyolytic event.

Magnetic Sensor Array for Electric Arc Reconstruction in Circuit Breakers.

Noninvasive imaging of circuit breakers under short-circuit testing is addressed by recording the magnetic field produced over an array of external sensors and by solving an inverse problem to identify the causing current distribution. The temporal and spatial resolution of the sensing chain are studied and implemented in a physical set-up. A wire model is adopted to describe electrical current distribution.

Lower-Limb Exoskeletons for Gait Training in Parkinson's Disease: The State of the Art and Future Perspectives.

Gait dysfunction (GD) is a common impairment of Parkinson's disease (PD), which negatively impacts patients' quality of life. Among the most recent rehabilitation technologies, a lower-limb powered exoskeleton (LLEXO) arises as a useful instrument for gait training in several neurological conditions, including PD. However, some questions relating to methods of use, achievable results, and usefulness compared to traditional rehabilitation methodologies still require clear answers.

Maximal Oxygen Consumption Is Negatively Associated with Fat Mass in Facioscapulohumeral Dystrophy.

Facioscapulohumeral dystrophy (FSHD) leads to progressive changes in body composition such as loss of muscle mass and increase in adiposity. In healthy subjects, anthropometric parameters are associated with the maximum volume of oxygen consumed per minute (VOmax), which is a health and function indicator in several populations of subjects, both healthy and pathological. Since VOmax can be difficult to test in patients with FSHD due to exercise intolerance, the identification of associated anthropometric parameters could provide new easily obtainable elements for the patients' clinical stratification.

Bioimpedance analysis of fat free mass and its subcomponents and relative associations with maximal oxygen consumption in facioscapulohumeral dystrophy.

Purpose: Fat free mass (FFM) is considered the metabolically active component of human body and is positively associated with maximal oxygen uptake ( ). However, FFM is composed of metabolically active and inactive subcomponents whose proportion can vary depending on body composition and clinical condition, possibly affecting such association. Although it is known that in facioscapulohumeral dystrophy (FSHD) peculiar changes in body composition occur, it is unclear whether there are alterations in FFM composition and, if so, whether such alterations affect the association towards compared to healthy subjects (HS).

Case report: A creatine kinase-borg scale values-based approach to tailor physical training in a central core myopathy patient.

Background: Patients with central core myopathy (CCM) can be at risk of exercise-induced rhabdomyolysis and myalgia. Despite its possible positive effects, physical training has been long avoided in these patients as no population-specific exercise adaption strategies have been developed. Here we present the case of a 17-year-old male CCM patient who underwent a 3-month training program tailored to a preliminary test aimed at assessing his physical exertion tolerance measured via changes in serum creatine kinase (CK).

Effects of essential amino acid (EAA) and glutamine supplementation on skeletal muscle wasting in acute, subacute, and postacute conditions.

Under optimal physiological conditions, muscle mass maintenance is ensured by dietary protein, which balances the amino acid loss during the post-absorption period and preserves the body's protein homeostasis. Conversely, in critical clinical conditions (acute, subacute or postacute), particularly those related to hypomobility or immobility, combined with malnutrition, and local/systemic inflammation, the loss of muscle mass and strength can be quantitatively significant. A decline of more than 1% in muscle mass and of more than 3% in muscle strength has been registered in subjects with aged 20-37 yr after just five days of bed rest, similarly to those observed during one year of age-related decline in individuals over the age of 50.

An 8-month adapted motor activity program in a young CMT1A male patient.

Background: It is unclear whether prolonged periods of training can be well tolerated. In Charcot-Marie Tooth disease (CMT). We report the effects of an 8-month, adapted motor activity (AMA) program in a 16-years-old CMT1A male patient.

Fatigue in Spinal Muscular Atrophy: a fundamental open issue.

Hereditary proximal 5q Spinal Muscular Atrophy (SMA) is a severe neuromuscular disorder with onset mainly in infancy or childhood. The underlying pathogenic mechanism is the loss of alpha motor neurons in the anterior horns of spine, due to deficiency of the survival motor neuron (SMN) protein as a consequence of the deletion of the SMN1 gene. Clinically, SMA is characterized by progressive loss of muscle strength and motor function ranging from the extremely severe, the neonatal onset type 1, to the mild type 4 arising in the adult life.

Physical activity practiced at a young age is associated with a less severe subsequent clinical presentation in facioscapulohumeral muscular dystrophy.

Background: In facioscapulohumeral muscular dystrophy (FSHD), it is not known whether physical activity (PA) practiced at young age is associated with the clinical presentation of disease. To assess this issue, we performed a retrospective cohort study concerning the previous practice of sports and, among them, those with medium-high cardiovascular commitment in clinically categorized carriers of a D4Z4 reduced allele (DRA).

Methods: People aged between 18 and 60 were recruited as being DRA carriers.

Gut-muscle-brain axis: Molecular mechanisms in neurodegenerative disorders and potential therapeutic efficacy of probiotic supplementation coupled with exercise.

Increased longevity is often associated with age-related conditions. The most common neurodegenerative disorders in the older population are Alzheimer's disease (AD) and Parkinson's disease (PD), associated with progressive neuronal loss leading to functional and cognitive impairments. Although symptomatic treatments are available, there is currently no cure for these conditions.

Type 1 diabetes management in a competitive athlete: A five-year case report.

Type I diabetes has an incidence of 15 per 100,000 people. Though it is a metabolic disorder, it can be seen in top, even professional athletes. Physical activity is recommended to manage diabetes, but there is a lack of specific knowledge on diabetes management and exercise from dedicated medical staff.

A balanced formula of essential amino acids promotes brain mitochondrial biogenesis and protects neurons from ischemic insult.

Mitochondrial dysfunction plays a key role in the aging process, and aging is a strong risk factor for neurodegenerative diseases or brain injury characterized by impairment of mitochondrial function. Among these, ischemic stroke is one of the leading causes of death and permanent disability worldwide. Pharmacological approaches for its prevention and therapy are limited.

How much resistance exercise is beneficial for healthy aging and longevity?

• Resistance exercise (RE) is an important physical activity modality conferring major health benefits that are partially complementary to benefits from endurance exercise. • Recent meta-analyses reveal a U-shaped relationship between RE volumes and mortality, with optimal benefits achieved at about 60 min of RE per week. • The mechanisms for potential adverse effects at higher RE volumes are unknown and may be due to hitherto undefined confounders and/or specific individual vulnerabilities.

Strength training in elderly: An useful tool against sarcopenia.

The loss of muscle mass and strength in elderly population (especially after the age of 65-70) represents a public health problem. Due to the high prevalence of frailty in older adults, cardiovascular or low-intensity exercise is implemented as first choice option. Although beneficial these training schemes are not as effective as strength-based resistance training for increasing muscle strength and hypertrophy.

Effects of a Single Dose of a Creatine-Based Multi-Ingredient Pre-workout Supplement Compared to Creatine Alone on Performance Fatigability After Resistance Exercise: A Double-Blind Crossover Design Study.

Background: This study aims to investigate the acute effects of a single oral administration of a creatine-based multi-ingredient pre-workout supplement (MIPS) on performance fatigability and maximal force production after a resistance exercise protocol (REP).

Methods: Eighteen adult males (age: 23 ± 1 years; body mass: 76.4 ± 1.

Bioelectrical Impedance Vector Analysis Discriminates Aerobic Power in Futsal Players: The Role of Body Composition.

Aims: The present study aimed to assess the ability of bioelectrical impedance vector analysis (BIVA) in discriminating fitness levels in futsal players, exploring the association of body composition and bioelectrical parameters with aerobic power. Methods: Forty-eight professional futsal players (age 23.8 ± 5.

The Ketogenic Diet: Is It an Answer for Sarcopenic Obesity?

This review aims to define the effectiveness of the ketogenic diet (KD) for the management of sarcopenic obesity. As the combination of sarcopenia and obesity appears to have multiple negative metabolic effects, this narrative review discusses the effects of the ketogenic diet as a possible synergic intervention to decrease visceral adipose tissue (VAT) and fatty infiltration of the liver as well as modulate and improve the gut microbiota, inflammation and body composition. The results of this review support the evidence that the KD improves metabolic health and expands adipose tissue γδ T cells that are important for glycaemia control during obesity.