Fryebarnett1445

Early identification and treatment of spinal muscular atrophy (SMA) are crucial but difficult. In this study, we aimed to assess the significance of compound motor action potential (CMAP) amplitude in patients identified through a newborn screening program.

We initiated a large-scale population newborn screening program for SMA in Taiwan in 2014. Patients had access to treatment through clinical trials or expanded use programs. Symptomatic patients were evaluated regularly, including CMAP exams.

Among 364,000 screened newborns, 21 were diagnosed with SMA. The incidence of SMA was around 1 in 17,000 live births, and 70% developed SMA type 1. All infants with two SMN2 copies became symptomatic before the age of 1 month. CMAP amplitudes of 12 newborns were available, including 6 who were subsequently treated with nusinersen. We found that a rapid decrease of CMAP amplitude was an early predictor of symptom onset. Selleck Rosuvastatin Pretreatment CMAP and rapid increment of post-treatment CMAP could predict better treatment outcomes.

This study prospectively demonstrated the incidence of SMA and its types. Our results imply the importance of pretreatment CMAP amplitude and rapid reversal of post-treatment CMAP amplitude with regard to disease presentation and also treatment outcomes.

This study prospectively demonstrated the incidence of SMA and its types. Our results imply the importance of pretreatment CMAP amplitude and rapid reversal of post-treatment CMAP amplitude with regard to disease presentation and also treatment outcomes.An amendment to this paper has been published and can be accessed via a link at the top of the paper.An amendment to this paper has been published and can be accessed via a link at the top of the paper.

We hypothesised that maternal diet-induced-obesity has adverse consequences for offspring energy expenditure and susceptibility to obesity in adulthood, and that the prebiotic polydextrose (PDX) would prevent the consequences of programming by maternal obesity.

Female mice were fed a control (Con) or obesogenic diet (Ob) for 6 weeks prior to mating and throughout pregnancy and lactation. Half the obese dams were supplemented with 5% PDX (ObPDX) in drinking water throughout pregnancy and lactation. Offspring were weaned onto standard chow. At 3 and 6 months, offspring energy intake (EI) and energy expenditure (EE by indirect calorimetry) were measured, and a glucose-tolerance test performed. Offspring of control (OffCon), obese (OffOb) and PDX supplemented (OffObP) dams were subsequently challenged for 3 weeks with Ob, and energy balanced reassessed. Potential modifiers of offspring energy balance including gut microbiota and biomarkers of mitochondrial activity were also evaluated.

Six-month-old male Ofpring energy balance and propensity for obesity, which is ameliorated by maternal PDX treatment with associated changes in gut microbiota composition and skeletal muscle mitochondrial function.

Maternal obesity adversely influences adult offspring energy balance and propensity for obesity, which is ameliorated by maternal PDX treatment with associated changes in gut microbiota composition and skeletal muscle mitochondrial function.Accurately quantifying dietary intake is essential to understanding the effect of diet on health and evaluating the efficacy of dietary interventions. Self-report methods (e.g., food records) are frequently utilized despite evident inaccuracy of these methods at assessing energy and nutrient intake. Methods that assess food intake via images of foods have overcome many of the limitations of traditional self-report. In cafeteria settings, digital photography has proven to be unobtrusive and accurate and is the method of choice for assessing food provision, plate waste, and food intake. In free-living conditions, image capture of food selection and plate waste via the user's smartphone, is promising and can produce accurate energy intake estimates, though accuracy is not guaranteed. These methods foster (near) real-time transfer of data and eliminate the need for portion size estimation by the user since the food images are analyzed by trained raters. A limitation that remains, similar to self-report methods where participants must truthfully record all consumed foods, is intentional and/or unintentional underreporting of foods due to social desirability or forgetfulness. Methods that rely on passive image capture via wearable cameras are promising and aim to reduce user burden; however, only pilot data with limited validity are currently available and these methods remain obtrusive and cumbersome. To reduce analysis-related staff burden and to allow real-time feedback to the user, recent approaches have aimed to automate the analysis of food images. The technology to support automatic food recognition and portion size estimation is, however, still in its infancy and fully automated food intake assessment with acceptable precision not yet a reality. This review further evaluates the benefits and challenges of current image-assisted methods of food intake assessment and concludes that less burdensome methods are less accurate and that no current method is adequate in all settings.

Large socioeconomic, gender, and ethnic inequalities exist in terms of childhood obesity worldwide. Children from low socioeconomic status families are more likely to have overweight/obesity and related cardiometabolic problems and future cancer risk. A wider concept are social vulnerabilities defined as social or economic characteristics or experiences negatively affecting children through behavioral, biological factors, or mental health. Social vulnerabilities include also therefore low subjective perceptions of social position.

This study aims to identify social vulnerabilities and to summarize their impact as obesity development risk factor. Preventive programs implemented targeting these vulnerable groups and their effectiveness are also discussed.

Literature review based on the experience of the authors social vulnerabilities identified as risk factors for childhood obesity were children whose parents lack of a social network, low support from formal and informal sources, parental unemployment, belonging to a minority group or having migrant background, adverse childhood experiences including household dysfunction, violence and childhood maltreatment and other traumatic experiences, gender inequalities and being part of nontraditional families.