Fatigue and associated factors in 172 patients with McArdle disease: An international web-based survey.

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.

Patient-Reported Experiences with a Low-Carbohydrate Ketogenic Diet: An International Survey in Patients with McArdle Disease.

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.

BNIP3 Is Involved in Muscle Fiber Atrophy in Late-Onset Pompe Disease Patients.

Late-onset Pompe disease (LOPD) is a rare genetic disorder produced by mutations in the GAA gene and is characterized by progressive muscle weakness. LOPD muscle biopsies show accumulation of glycogen along with the autophagic vacuoles associated with atrophic muscle fibers. The expression of molecules related to muscle fiber atrophy in muscle biopsies of LOPD patients was studied using immunofluorescence and real-time PCR.

Influenza A-Associated In-Hospital Mortality in Very Older People: Does Inflammation Also Play a Role?

Background: The aim of the study was to analyze the clinical manifestations and outcome of the oldest old (people aged ≥85 years) who were admitted to the hospital with a confirmed influenza A virus infection in comparison with younger patients and to assess the role of inflammation in the outcome of influenza infection in this population.

Methods: This is an observational prospective study including all adult patients with influenza A virus infection hospitalized in a tertiary teaching hospital in Madrid, in 2 consecutive influenza seasons (2016-17 and 2017-18).

Results: Five hundred nine hospitalized patients with influenza A infection were included, of whom 117 (23%) were older than 85 years (median age: 89.

Academic performance and psychosocial functioning in European schoolchildren: The role of cardiorespiratory fitness and weight status.

Background: Whether a high cardiorespiratory fitness (CRF) can attenuate the potential detrimental effect of childhood obesity on academic performance and particularly psychosocial functioning remains unclear.

Objectives: To analyse the academic performance/psychosocial functioning of schoolchildren attending to their CRF and weight status.

Methods: 470 schoolchildren (46% girls, 7 ± 0 years) from 20 schools in five European countries were categorized as (i) having overweight/obesity (n = 113) or normal weight (n = 357) attending to body mass index and (ii) 'fit' (n = 282) or 'unfit' (n = 188) based on 20-meter shuttle-run performance (CRF ≥ or <42 ml·kg ·min [boys] and 35 ml·kg ·min [girls], respectively).

The Postnatal Leptin Surge Supports Immune Cell Function in Rats.

Background: Leptin plays an important role in the regulation of the immune response. There is a physiological surge of leptin in rodents during the neonatal period, which has mainly been studied in the context of brain development. However, little is known about the effects of this neonatal leptin surge on immunity.

Physical Fitness and Health-related Quality of Life in Patients with Colorectal Cancer.

Colorectal cancer is now a frequently treatable illness for most and a chronic disease for many. The number of people living with a diagnosis of colorectal cancer is thus expected to rise. Yet even after successful treatment, colorectal cancer survivors, mostly the elderly, frequently experience health problems and impaired health-related quality of life.

Heart Rate Variability and Exceptional Longevity.

Centenarians are the paradigm of human extreme longevity and healthy aging, because they have postponed, if not avoided, mayor age-related diseases. The purpose of this study was to investigate potential differences in resting heart rate variability (HRV) between young adults, octogenarians, and centenarians and assess whether HRV variables are predictors of all-cause mortality in centenarians. To this end, three groups of participants: young adults ( = 20; 20.

Obesity can offset the cardiometabolic benefits of gestational exercise.

Pregnancy exercise can prevent excessive gestational weight gain (EGWG), gestational diabetes mellitus (GDM) and hypertension (GH), but inter-individual variability has not been explored. We aimed to analyze the prevalence--and potential sociodemographic and medical predictors of--non-responsiveness to gestational exercise, and the association of non-responsiveness with adverse pregnancy outcomes. Among 688 women who completed a supervised light-to-moderate intensity exercise program (three ~1-h sessions/week including aerobic, resistance, and pelvic floor muscle training) until near-term, those who showed EGWG, GDM or GH were considered 'non-responders'.

The supplementations with 2-hydroxyoleic acid and n-3 polyunsaturated fatty acids revert oxidative stress in various organs of diet-induced obese mice.

Obesity and its related diseases have been associated with oxidative stress. Thus, the search for nutritional strategies to ameliorate oxidative stress in obese individuals seems important. We hypothesized that the supplementation with monounsaturated (2-hydroxyoleic acid (2-OHOA)) and with combined n-3 polyunsaturated (eicosapentaenoic acid (EPA) and docosahexaenoic acid (DHA)) fatty acids would ameliorate oxidative stress in different organs, including brain, liver, lungs, and kidneys of adult diet-induced obese (DIO) mice.

Elevation of serum ferritin levels for predicting a poor outcome in hospitalized patients with influenza infection.

Objectives: There is increasing evidence that ferritin is a key marker of macrophage activation, but its potential role in influenza infection remains unexplored. Our aim was to assess whether hyperferritinaemia (ferritin ≥500 ng/mL) could be a marker of poor prognosis in hospitalized patients with confirmed influenza A infection.

Methods: We prospectively recruited all hospitalized adult patients who tested positive for the influenza A rRT-PCR assay performed on respiratory samples in two consecutive influenza periods (2016-17 and 2017-18).

Physical activity and prenatal depression: going beyond statistical significance by assessing the impact of reliable and clinical significant change.

Background: Previous literature supports exercise as a preventative agent for prenatal depression; however, treatment effects for women at risk for prenatal depression remain unexplored. The purpose of the study was to examine whether exercise can lower depressive symptoms among women who began pregnancy at risk for depression using both a statistical significance and reliable and clinically significant change criteria.

Methods: This study is a secondary analysis of two randomized controlled trials that followed the same exercise protocol.

Reliable change indices for the 37-item version of the MMSE in Spanish older adults.

Objective: The aim of this study was to establish reliable change and regression-based change score norms on the 37-item version of MMSE in older Spanish adults at the three-year follow-up.

Method: All subjects of this research were selected from the Neurological Disorders in Central Spain (NEDICES), a prospective population-based cohort study of older adults (65 years and over). Of the 4208 individuals free from neurological disorders in this cohort, 2073 participants completed the MMSE-37 at baseline and at the three-year follow-up.

Blood-based systems biology biomarkers for next-generation clinical trials in Alzheimer's disease .

Alzheimer's disease (AD)-a complex disease showing multiple pathomechanistic alterations-is triggered by nonlinear dynamic interactions of genetic/epigenetic and environmental risk factors, which, ultimately, converge into a biologically heterogeneous disease. To tackle the burden of AD during early preclinical stages, accessible blood-based biomarkers are currently being developed. Specifically, next-generation clinical trials are expected to integrate positive and negative predictive blood-based biomarkers into study designs to evaluate, at the individual level, target druggability and potential drug resistance mechanisms.

Improvement of Redox State and Functions of Immune Cells as Well as of Behavioral Response in Aged Mice After Two-Week Supplementation of Fermented Milk with Probiotics.

The homeostatic systems, such as the nervous and immune systems, show deterioration in aging as a consequence of the age-related oxidative-inflammatory stress establishment. The supplementation with fermented milk containing probiotic bacteria could be a good nutritional strategy to improve homeostatic system functions in aged individuals through the modulation of their redox state. The aim of the present study was to evaluate the effect of 2-week supplementation with a commercial fermented milk containing yogurt species (Lactobacillus delbrueckii subsp.

Physical Exercise and Mitochondrial Disease: Insights From a Mouse Model.

Mitochondrial diseases (MD) are among the most prevalent neuromuscular disorders. Unfortunately, no curative treatment is yet available. This study analyzed the effects of exercise training in an animal model of respiratory chain complex I deficiency, the Harlequin () mouse, which replicates the clinical features of this condition.

Late-onset thymidine kinase 2 deficiency: a review of 18 cases.

Background: TK2 gene encodes for mitochondrial thymidine kinase, which phosphorylates the pyrimidine nucleosides thymidine and deoxycytidine. Recessive mutations in the TK2 gene are responsible for the 'myopathic form' of the mitochondrial depletion/multiple deletions syndrome, with a wide spectrum of severity.

Methods: We describe 18 patients with mitochondrial myopathy due to mutations in the TK2 gene with absence of clinical symptoms until the age of 12.

Impacts of the late adulthood diet-induced obesity onset on behavior, immune function, redox state and life span of male and female mice.

The aim of the present study was to investigate whether the late onset of diet-induced obesity (DIO) in middle-aged mice affected behavioral, immunological and oxidative stress parameters as well as life span of male and female mice. Also, it was analyzed whether the late DIO onset aggravated immunosenescence in old female mice. Late-adult male and female ICR/CD1 mice (28 weeks old) were fed either a high-fat diet or a standard diet during 14 weeks.