Long-term benefits of physical activity in adult patients with late onset Pompe disease: a retrospective cohort study with 10 years of follow-up.

Background: In 2011 a 12 weeks personalized exercise training program in 23 mildly affected adult late onset Pompe patients (age 19.6-70.5 years) improved endurance, muscle strength and function.

Leg-focused high-weight resistance training improves ventricular stroke volume, exercise capacity and strength in young patients with a Fontan circulation.

Aims: Effective therapy to improve exercise capacity in Fontan patients is lacking. Leg-focused high-weight resistance training might augment the peripheral muscle pump and thereby improve exercise capacity.

Methods And Results: This randomized semi-cross-over controlled trial investigated the effects of a 12-week leg-focused high-weight resistance training plus high-protein diet, on (sub)maximal exercise capacity, cardiac function (assessed with cardiovascular magnetic resonance), muscle strength, and quality of life in paediatric Fontan patients.

Body composition in patients with Fontan physiology: a systematic review.

Fontan circulation is a highly abnormal circulatory state that may affect various organ systems. The effect on body composition is an important factor to assess the condition of the patient. This systematic review assesses body composition and possibly related adverse outcomes in patients with a Fontan circulation, to provide an overview of current insights.

Stress imaging in patients with a Fontan circulation: A systematic review.

Introduction: The aims of this study were to provide an overview of the cardiac stress response in Fontan patients and of the use, safety and clinical value of stress imaging in Fontan patients.

Methods: Studies evaluating cardiac function using stress imaging in Fontan patients published up until 12 December 2021 were included in this review.

Results: From 1603 potential studies, 32 studies met the inclusion criteria.

Sugar-responsive inhibition of Myc-dependent ribosome biogenesis by Clockwork orange.

The ability to feed on a sugar-containing diet depends on a gene regulatory network controlled by the intracellular sugar sensor Mondo/ChREBP-Mlx, which remains insufficiently characterized. Here, we present a genome-wide temporal clustering of sugar-responsive gene expression in Drosophila larvae. We identify gene expression programs responding to sugar feeding, including downregulation of ribosome biogenesis genes, known targets of Myc.

A Pre-registered sticky mittens study: active training does not increase reaching and grasping in a swedish context.

Several studies have previously investigated the effects of sticky mittens training on reaching and grasping development. However, recent critique casted doubts on the robustness of the motor effect of this training. The current study presents a pre-registered report that aimed to generalize these effects to Swedish infants.

Standardizing submaximal exercise intensities for use of supine push-pull exercise during cardiovascular magnetic resonance.

Background: Cardiovascular magnetic resonance (CMR) imaging during supine exercise at (sub)maximal oxygen consumption (VO ) offers unique diagnostic insights. However, maximal VO is not achievable in the supine position and standardizing submaximal exercise intensities remains challenging. Using heart rate or workload could be a viable option to translate VO -based submaximal exercise intensities.

Study Protocol of the Exercise Study: Unraveling Limitations for Physical Activity in Children With Chronic Diseases in Order to Target Them With Tailored Interventions-A Randomized Cross Over Trial.

Physical activity is associated with many physiological and psychological health benefits across the lifespan. Children with a chronic disease often have lower levels of daily physical activity, and a decreased exercise capacity compared to healthy peers. In order to learn more about limitations for physical activity, we investigate children with four different chronic diseases: children with a Fontan circulation, children with Broncho Pulmonary Dysplasia (BPD), Pompe disease and inflammatory bowel disease (IBD).

Translation and transcultural validation of the Dutch hospital for special surgery paediatric functional activity brief scale (HSS Pedi-FABS).

Background: There is a need for a validated simple Dutch paediatric activity scale. The purpose was to translate and transculturally validate the Dutch Hospital for Special Surgery Paediatric Functional Activity Brief Scale (HSS Pedi-FABS) questionnaire in healthy children and adolescents.

Methods: The original HSS Pedi-FABS was translated forward and backward and was transculturally adapted after performing a pilot study among children and professionals.

The sticky mittens paradigm: A critical appraisal of current results and explanations.

Almost two decades ago, the sticky mittens paradigm was demonstrated as a way to train reaching and grasping behaviors in pre-reaching infants, and consequently improve visual attentional abilities. In that first study, Needham and colleagues fitted 3-month-old infants with Velcro loop-covered mittens and allowed them to interact with Velcro hook-covered toys over the course of 2 weeks. In this review, we scrutinize the 17 studies that have followed those first sticky mittens results in regards to the motor, social perception, and visual attentional domains.

The comparison of the interpersonal action component of woman-centred care reported by healthy pregnant women in different sized practices in the Netherlands: A cross-sectional study.

Background: The number of interventions is lower, and the level of satisfaction is higher among women who receive midwife-led primary care from one or two midwives, compared to more midwives. This suggests that midwives in small-sized practices practice more women-centred. This has yet to be explored.

Development of a Biosecurity Checklist for Laboratory Assessment and Monitoring.

Introduction: Laboratory biosecurity is of continuously growing interest due to increasing concerns about deliberate misuse of biological materials and emerging biological risks. These risks continue to be magnified by globalization, the rapid pace of scientific development, and dual-use technologies. Worldwide laboratory capacities are expanding, which calls for concrete actions to improve laboratory biosafety and biosecurity practices to protect researchers and the community.

The Vulnerability Scan, a Web Tool to Increase Institutional Biosecurity Resilience.

The importance of vigilance within organizations working with high-risk biological material receives increasing attention. However, an in-depth and comprehensive tool, dedicated to increase awareness of potential risks and to assess an organization's current biosecurity vulnerabilities, has not been available yet. We developed the "Biosecurity Vulnerability Scan," a web tool that identifies biosecurity gaps in an organization based on eight biosecurity pillars of good practice.

Infants' center bias in free viewing of real-world scenes.

This study examines how salience and a center bias drive infants' first fixation while looking at complex scenes. Adults are known to have a strong center bias, their first point of gaze is nearly always in the center of the scene. The center bias is likely to be a strategic bias, as looking towards the center minimizes the distance to other parts of the scene and important objects are often located at the center.

Contribution of allelic imbalance to colorectal cancer.

Point mutations in cancer have been extensively studied but chromosomal gains and losses have been more challenging to interpret due to their unspecific nature. Here we examine high-resolution allelic imbalance (AI) landscape in 1699 colorectal cancers, 256 of which have been whole-genome sequenced (WGSed). The imbalances pinpoint 38 genes as plausible AI targets based on previous knowledge.

Bioinformatics in the Netherlands: the value of a nationwide community.

This review provides a historical overview of the inception and development of bioinformatics research in the Netherlands. Rooted in theoretical biology by foundational figures such as Paulien Hogeweg (at Utrecht University since the 1970s), the developments leading to organizational structures supporting a relatively large Dutch bioinformatics community will be reviewed. We will show that the most valuable resource that we have built over these years is the close-knit national expert community that is well engaged in basic and translational life science research programmes.

Genome-wide association study and meta-analysis in Northern European populations replicate multiple colorectal cancer risk loci.

Genome-wide association studies have been successful in elucidating the genetic basis of colorectal cancer (CRC), but there remains unexplained variability in genetic risk. To identify new risk variants and to confirm reported associations, we conducted a genome-wide association study in 1,701 CRC cases and 14,082 cancer-free controls from the Finnish population. A total of 9,068,015 genetic variants were imputed and tested, and 30 promising variants were studied in additional 11,647 cases and 12,356 controls of European ancestry.

Relationships Between Results of an Internal and External Match Load Determining Method in Male, Singles Badminton Players.

Abdullahi, Y, Coetzee, B, and van den Berg, L. Relationships between results of an internal and external match load determining method in male, singles badminton players. J Strength Cond Res 33(4): 1111-1118, 2019-The purpose of study was to determine relationships between results of an internal and external match load determining method.

The key role of extinction learning in anxiety disorders: behavioral strategies to enhance exposure-based treatments.

Purpose Of Review: Extinction learning is a major mechanism for fear reduction by means of exposure. Current research targets innovative strategies to enhance fear extinction and thereby optimize exposure-based treatments for anxiety disorders. This selective review updates novel behavioral strategies that may provide cutting-edge clinical implications.

Chemically Induced Oncogenesis in the Peripheral Nervous System Is Suppressed in Congenic BDIX.BDIV-Mss1 and -Mss7 Rats.

Human malignant peripheral nerve sheath tumors (MPNSTs) are highly aggressive soft-tissue sarcomas with a poor prognosis that arise either in the context of neurofibromatosis 1 or sporadically. Inbred BDIX and BDIV rat strains highly susceptible and resistant, respectively, to the development of ethylnitrosourea-induced MPNST enable us to identify, by using methods not applicable in humans, variant alleles involved in the pathways underlying individual MPNST risk. On the basis of a genome-wide association analysis using reciprocal intercrosses of BDIX and BDIV, BDIV alleles of two loci on chromosome 10, Mss1 and Mss7, were predicted to lower the risk of MPNST, the latter locus with a female bias.