Pilot study of a virtual weight management program for Duchenne muscular dystrophy.

INTRODUCTION/AIMS: Young people with Duchenne muscular dystrophy (DMD) are at increased risk of obesity. Weight management is important to families; however, several barriers exist. This pilot study aimed to investigate the feasibility and acceptability of a co-designed weight management program for DMD. METHODS: The Supporting Nutrition and Optimizing Wellbeing Program (SNOW-P) was a single-arm diet and behavior weight management intervention delivered via weekly telehealth/phone visits over 6 weeks to young people with DMD and obesity (body mass index (BMI) ≥95th percentile) and their caregivers. Using an online survey, caregivers of boys with DMD were consulted on the structure and topics delivered in SNOW-P. Primary outcomes were feasibility and acceptability; secondary outcomes were weight, physical function, and quality of life at 6- and 12-weeks follow-up. RESULTS: Of nineteen eligible participants, eight were enrolled (median age 11.4 years, range 4.9-15.8), and seven completed the program. Visit attendance was high (88%-100%); most participants reported high satisfaction and that participation was easy. Suggested changes included online and visual DMD-specific resources. At 6-weeks, median change in weight z-scores was -0.01 (IQR: -0.23, 0.17) indicating that on average, weight gain tracked as expected for age. Waist circumference measured by caregivers lacked accuracy and the completion rate of caregiver-reported secondary outcome measures (e.g., food diaries) was low. DISCUSSION: A co-designed, telehealth/phone weight management program appeared to be feasible and acceptable in a small group of boys with DMD. An adapted, hybrid telehealth and face-to-face program is recommended for efficacy testing.

Exploring caregivers' attitudes and beliefs about nutrition and weight management for young people with Duchenne muscular dystrophy.

INTRODUCTION/AIMS: Obesity disproportionately affects children and adolescents with Duchenne muscular dystrophy (DMD) and with adverse consequences for disease progression. This study aims to: explore barriers, enablers, attitudes, and beliefs about nutrition and weight management; and to obtain caregiver preferences for the design of a weight management program for DMD. METHODS: We surveyed caregivers of young people with DMD from four Australian pediatric neuromuscular clinics. Survey questions were informed by the Theoretical Domains Framework and purposefully designed to explore barriers and enablers to food and weight management. Caregivers were asked to identify their preferred features in a weight management program for families living with DMD. RESULTS: Fifty-three caregivers completed the survey. Almost half (48%) perceived their son as above healthy weight. Consequences for those children were perceived to be self-consciousness (71%), a negative impact on self-esteem (64%) and movement (57%). Preventing weight gain was a common reason for providing healthy food and healthy eating was a high priority for families. Barriers to that intention included: time constraints, selective food preferences, and insufficient nutrition information. Caregivers preferred an intensive six-week weight management program addressing appetite management and screen time. DISCUSSION: Managing weight is an important issue for caregivers of sons with DMD; yet several barriers exist. Individualized 6 week programs are preferred by caregivers to improve weight management for DMD.

A scoping review of nutrition issues and management strategies in individuals with skeletal dysplasia.

PURPOSE: Skeletal dysplasia are heterogeneous conditions affecting the skeleton. Common nutrition issues include feeding difficulties, obesity, and metabolic complications. This systematic scoping review aimed to identify key nutrition issues, management strategies, and gaps in knowledge regarding nutrition in skeletal dysplasia. METHODS: The databases Ovid MEDLINE, Ovid EMBASE, Ebsco CINAHL, Scopus, and Cochrane Central Register of Controlled Trials and Database of Systematic Reviews were searched. Reference lists and citing literature for included studies were searched. Eligible studies included participants with skeletal dysplasia and described: anthropometry, body composition, nutrition-related biochemistry, clinical issues, dietary intake, measured energy or nutrition requirements, or nutrition interventions. RESULTS: The literature search identified 8509 references from which 138 studies were included (130 observational, 3 intervention, 2 systematic reviews, and 3 clinical guidelines). Across 17 diagnoses identified, most studies described osteogenesis imperfecta (n = 50) and achondroplasia or hypochondroplasia (n = 47). Nutrition-related clinical issues, biochemistry, obesity, and metabolic complications were most commonly reported, and few studies measured energy requirements (n = 5). CONCLUSION: Nutrition-related comorbidities are documented in skeletal dysplasia; yet, evidence to guide management is scarce. Evidence describing nutrition in rarer skeletal dysplasia conditions is lacking. Advances in skeletal dysplasia nutrition knowledge is needed to optimize broader health outcomes.

Fracture risk and impact in boys with Duchenne muscular dystrophy: A retrospective cohort study.

INTRODUCTION/AIMS: Boys with Duchenne muscular dystrophy (DMD) are at increased risk of fracture. This study investigated the incidence of fractures, factors contributing to risk of first fracture with emphasis on body mass index (BMI), and the impact of fractures on functional capacity in an Australian cohort of boys with DMD. METHODS: A retrospective cohort study included boys with DMD who attended a pediatric neuromuscular clinic from 2011 to 2018. Information regarding fractures, anthropometry measurements, body composition and functional assessment was collected. Factors associated with first fracture risk were analyzed with Cox-proportional hazards. Longitudinal analysis of function post-fracture was also conducted. RESULTS: This study included 155 boys with DMD. At least one fracture occurred in 71 (45%) boys; overall incidence of fractures was 399-per-10,000 persons-years. The first fracture was vertebral in 55%; 41% had non-vertebral fractures and 4% had both. Vertebral fractures occurred in significantly older (12.28 vs 9.28 y) boys with longer exposure to glucocorticoids (5.45 vs 2.50 y) compared to non-vertebral fractures. Boys with a history of fracture(s) had a steeper rate of functional decline (measured by Northstar Ambulatory Assessment score) than those with no recorded fractures. DISCUSSION: A high fracture burden was observed in a large Australian cohort of boys with DMD. Further investigation is required to understand preventative strategies and modifiable risk factors to reduce the incidence of fractures in DMD. The impact on fractures on ambulatory capacity should be closely monitored.

The association between dietary factors and body weight and composition in boys with Duchenne muscular dystrophy.

BACKGROUND: Duchenne muscular dystrophy (DMD) is a X-linked neuromuscular disorder. Boys with DMD have high rates of obesity, although little is known about dietary factors that may contribute to weight gain in this population. The present study aimed to explore the relationship between dietary factors, body mass index (BMI) z-score, body composition and motor function and to describe dietary intake in boys with DMD. METHODS: A cross-sectional analysis of 3-day food diaries from ambulant and steroid treated boys with DMD aged 5-13 years was conducted. Correlation analysis explored the relationship between dietary factors, BMI z-score, fat mass percentage (FM%) and lean mass percentage (LM%). RESULTS: The median age was 8.5 years (interquartile range [IQR] = 7.2-10.5 years). Median energy kg-1  day-1 in those within a healthy weight range (n = 11) was 316 kJ kg-1  day-1 (IQR = 276-355 kJ kg-1  day-1 ) and greater than estimated requirements and, for those above a healthy weight (n = 26), energy intake was 185 kJ kg-1  day-1 (IQR = 143-214 kJ kg-1  day-1 ) and lower than estimated requirements. Energy kg-1  day-1 was negatively associated with BMI z-score (r = -0. 650) and FM% (r = -0.817) but positively associated with LM% (r = 0.805; all analyses p = <0.01). Younger age was associated (r = -0.609 p = <0.01) with a higher energy kg-1  day-1 . For all participants, vegetable, grains, meat/alternatives and dairy intakes were sub-optimal. CONCLUSIONS: Younger boys with DMD within a healthy weight range are overconsuming energy dense nutrient poor foods. A focus on improving diet quality during early childhood may prove to be a useful strategy for reducing excess weight gain and supporting healthier eating habits in this vulnerable clinical population.

The relative price of healthy and less healthy foods available in Australian school canteens.

School canteens have an important role in modelling a healthy food environment. Price is a strong predictor of food and beverage choice. This study compared the relative price of healthy and less healthy lunch and snack items sold within Australian school canteens. A convenience sample of online canteen menus from five Australian states were selected (100 primary and 100 secondary schools). State-specific canteen guidelines were used to classify menu items into 'green' (eat most), 'amber' (select carefully) and 'red' (not recommended in schools). The price of the cheapest 'healthy' lunch (vegetable-based 'green') and snack ('green' fruit) item was compared to the cheapest 'less healthy' ('amber/red') lunch and snack item, respectively, using an un-paired t-test. The relative price of the 'healthy' items and the 'less healthy' items was calculated to determine the proportion of schools that sold the 'less healthy' item cheaper. The mean cost of the 'healthy' lunch items was greater than the 'less healthy' lunch items for both primary (AUD $0.70 greater) and secondary schools ($0.50 greater; p < 0.01). For 75% of primary and 57% of secondary schools, the selected 'less healthy' lunch item was cheaper than the 'healthy' lunch item. For 41% of primary and 48% of secondary schools, the selected 'less healthy' snack was cheaper than the 'healthy' snack. These proportions were greatest for primary schools located in more, compared to less, disadvantaged areas. The relative price of foods sold within Australian school canteens appears to favour less healthy foods. School canteen healthy food policies should consider the price of foods sold.

The effect of sugar-sweetened beverage front-of-pack labels on drink selection, health knowledge and awareness: An online randomised controlled trial.

PURPOSE AND AIM: Sugar-sweetened beverages (SSBs) provide little nutritional value and are associated with an increased risk of diet-related diseases. Despite this, SSB consumption is high globally. One emerging strategy aimed at reducing SSB consumption involves the use of front-of-pack (FOP) labels that clearly identify the risks associated with SSB consumption. The aim of this research study was to determine whether FOP labels with a graphic warning, text warning, sugar information (with the number of teaspoons of added sugar) or Health Star Rating (HSR) reduces intended choice of a SSB in an online choice experiment with young Australian adults. RESULTS: 994 participants were recruited and completed the online choice experiment. Compared to the control group who were not exposed to a label, the graphic warning, text warning, sugar information and HSR labels all significantly reduced selection of a SSB in the choice scenario. The magnitude of effect was greatest for the graphic warning label (OR 0.22 95% CI 0.14-0.35). Compared to the control group, only the HSR label significantly increased selections of the high HSR drinks (OR 2.18 95% CI 1.20-3.97). CONCLUSIONS: FOP labels, particularly those with graphic warnings, have the potential to reduce intended SSB purchases. Labels that also identify healthier alternatives may influence consumers to substitute SSBs with healthier drinks.

A scoping review of Australian nutrition resources for feeding children under 5 years of age.

INTRODUCTION: This review identified and appraised Australian open-access online resources relating to feeding and nutrition during the first 5 years of life. METHODS: Eligible resources were identified by hand searching plus a targeted search of pertinent source websites (government, hospitals and health services, peak bodies, and nutrition organisations) published in English with/without translations to other languages between 2012 and 2022. Search terms relating to the population (children) AND topic (nutrition OR feeding) were entered into Google and Bing. Critical appraisal of each resource was conducted using a modified version of the Centres for Disease Control and Prevention Clear Communication Index. RESULTS: The search identified 1327 nutrition resources, of which 1067 were appraised. Forty-five percent were paper-based resources, 47% were online content only, and 8% audio-visual resources. Almost half of the resources (45%) broadly addressed the 0-5 years age range, and 24% provided information on breast or formula feeding. Limited resources were found specifically addressing growth and development (5%) and introducing solids (8%). Only 10% of resources were culturally tailored and only 3% were translated into a language other than English. Appraisal showed the majority included visual cues and appropriate language, but less included images to support the main message of the resource. DISCUSSION: Although there were a large volume of evidence-based nutrition resources available in Australia for feeding children under 5 years, they were highly dispersed and of varying quality. Collating and presenting these resources in an open-access resource hub would empower families and education and health professionals in the nutritional care of young children.

Describing Energy Expenditure in Children with a Chronic Disease: A Systematic Review.

Understanding energy expenditure in children with chronic disease is critical due to the impact on energy homeostasis and growth. This systematic review aimed to describe available literature of resting (REE) and total energy expenditure (TEE) in children with chronic disease measured by gold-standard methods of indirect calorimetry (IC) and doubly labeled water (DLW), respectively. A literature search was conducted using OVID Medline, Embase, CINAHL Plus, Cochrane, and Scopus until July 2023. Studies were included if the mean age of the participants was ≤18 y, participants had a chronic disease, and measurement of REE or TEE was conducted using IC or DLW, respectively. Studies investigating energy expenditure in premature infants, patients with acute illness, and intensive care patients were excluded. The primary outcomes were the type of data (REE, TEE) obtained and REE/TEE stratified by disease group. In total, 271 studies across 24 chronic conditions were identified. Over 60% of retrieved studies were published >10 y ago and conducted on relatively small population sizes (n range = 1-398). Most studies obtained REE samples (82%) rather than that of TEE (8%), with very few exploring both samples (10%). There was variability in the difference in energy expenditure in children with chronic disease compared with that of healthy control group across and within disease groups. Eighteen predictive energy equations were generated across the included studies. Quality assessment of the studies identified poor reporting of energy expenditure protocols, which may limit the validity of results. Current literature on energy expenditure in children with chronic disease, although extensive, reveals key future research opportunities. International collaboration and robust measurement of energy expenditure should be conducted to generate meaningful predictive energy equations to provide updated evidence that is reflective of emerging disease-modifying therapies. This study was registered in PROSPERO as CRD42020204690.

Weight management interventions that include dietary components for young people with chronic health care needs: A systematic review.

AIMS: To identify and describe weight management interventions that include a dietary component for young people with chronic healthcare needs and overweight or obesity and their effect on body mass index (BMI) or weight. METHODS: Six databases were searched in 2017 and 2020 for experimental studies in English: Ovid MEDLINE, Ovid Embase, Ovid AMED, EBSCO CINAHL, Scopus and Cochrane Central Register of Controlled Trials and Database of Systematic Reviews. Two independent reviewers conducted data extraction and quality assessment using the Cochrane Risk of Bias tool. Eligible studies included young people with chronic healthcare needs ≤18 years with overweight or obesity with an intervention that included a dietary component. Eligible outcomes were BMI or weight. Data were synthesised narratively. RESULTS: The search identified 15 293 references, 12 studies were included (randomised controlled trials n = 5, before-after comparisons n = 7). Participant diagnoses were neurodevelopmental disabilities (n = 5) and mental illness (n = 1); survivors of cancers or tumours (n = 4); congenital heart disease (n = 1) and; migraine (n = 1). No studies addressed weight management in physical disabilities. Eight studies demonstrated a significant reduction in BMI or weight. Of these, most interventions used dietary counselling or an energy deficit, were family-focused, multicomponent and delivered by a multidisciplinary team including dietitians. A high risk of bias was detected across studies. CONCLUSIONS: There is limited high-quality evidence about effective dietary solutions for the management of overweight and obesity for young people with chronic healthcare needs. While more research is required, dietary management appears to be important to manage weight in these populations.

The Relationship between Obesity and Clinical Outcomes in Young People with Duchenne Muscular Dystrophy.

BACKGROUND: Duchenne muscular dystrophy (DMD) is a severe X-linked neuromuscular disorder. Young people with DMD have high rates of obesity. There is emerging evidence that a higher BMI may negatively affect clinical outcomes in DMD. This study aimed to explore the relationship between obesity and clinical outcomes in DMD. METHODS: This was a retrospective clinical audit of young people (two-21 years) with DMD. Height and weight were collected to calculate BMI z-scores to classify obesity, overweight and no overweight or obesity (reference category). Cox proportional hazards models determined the impact of obesity at five to nine years on clinical milestones including time to: loss of ambulation, timed function test cut-offs, obstructive sleep apnoea (OSA) diagnosis and first fracture. RESULTS: 158 young people with DMD were included; most (89.9%) were steroid-treated. Mean follow-up was 8.7 ± 4.7 years. Obesity prevalence increased from age five (16.7%) to 11 years (50.6%). Boys with obesity at nine years sustained a fracture earlier (hazard ratio, HR: 2.050; 95% CI: 1.038-4.046). Boys with obesity at six to nine years were diagnosed with OSA earlier (e.g., obesity nine years HR: 2.883; 95% CI: 1.481-5.612). Obesity at eight years was associated with a 10 m walk/run in 7-10 s occurring at an older age (HR: 0.428; 95% CI: 0.207-0.887), but did not impact other physical function milestones. CONCLUSIONS: Although 50% of boys with DMD developed early obesity, the impact of obesity on physical function remains unclear. Obesity puts boys with DMD at risk of OSA and fractures at a younger age. Early weight management interventions are therefore important.

The effect of front-of-package labels or point-of-sale signage on consumer knowledge, attitudes and behavior regarding sugar-sweetened beverages: a systematic review.

CONTEXT: Globally, the use of labels or signage targeting SSBs remains in its infancy and there is limited evidence available regarding its ability to decrease purchase and consumption of SSBs. OBJECTIVE: This systematic review aimed to synthesize the evidence on the effect of sugar- or health-related sugar-sweetened beverage (SSB) warning labels or signage on knowledge, attitudes, and beliefs, and SSB purchase and consumption. DATA SOURCES: Nine databases - Ovid Medline, Emerald Insight, Scopus, Informit, Business Source Complete, CINAHL, Global Health, PsychINFO, and SocIndex - were searched along with grey literature from inception to December 2019. The PRISMA guidelines were applied for reporting this systematic review. STUDY SELECTION: Studies examining the impact of front of pack (FOP) labels and/or point of sale (POS) signage highlighting added sugar content or its health risks were included. DATA EXTRACTION: Two authors independently extracted data on items, including study details, study design, population characteristics, intervention label details (type, duration, and settings), and outcomes measures. The Effective Public Health Practice Project tool was used to assess the study quality. DATA ANALYSIS: Findings were synthesized narratively. RESULTS: Twenty-one studies published between 1992 and 2019 were included. Of these, 16 studies examined the impact of FOP labels and 5 studies examined the impact of POS signage. Both FOP labels and POS signage were associated with improved health knowledge, attitudes, and beliefs regarding SSBs and reduced SSB purchases. Warning labels with diet-related health consequences were found to be particularly effective. Overall quality of studies was assessed as mixed. CONCLUSION: Health- or sugar-related FOP labels and POS signage for SSBs are promising public health measures and may improve consumers' health behaviors toward reduced SSB purchase and consumption.

Investigating business outcomes of healthy food retail strategies: A systematic scoping review.

Large changes to food retail settings are required to improve population diet. However, limited research has comprehensively considered the business implications of healthy food retail strategies for food retailers. We performed a systematic scoping review to identify types of business outcomes that have been reported in healthy food retail strategy evaluations. Peer-reviewed and grey literature were searched. We identified qualitative or quantitative real-world food or beverage retail strategies designed to improve the healthiness of the consumer nutrition environment (eg, changes to the "marketing mix" of product, price, promotion, and/or placement). Eligible studies reported store- or chain-level outcomes for measures of commercial viability, retailer perspectives, customer perspectives, and/or community outcomes. 11 682 titles and abstracts were screened with 107 studies included for review from 15 countries. Overall item sales, revenue, store patronage, and customer level of satisfaction with strategy were the most frequently examined outcomes. There was a large heterogeneity in outcome measures reported and in favourability for retailers of outcomes across studies. We recommend more consistent reporting of business outcomes and increased development and use of validated and reliable measurement tools. This may help generate more robust research evidence to aid retailers and policymakers to select feasible and sustainable healthy food retail strategies to benefit population health within and across countries.

Preschool and School Meal Policies: An Overview of What We Know about Regulation, Implementation, and Impact on Diet in the UK, Sweden, and Australia.

School meals make significant contributions to healthy dietary behaviour, at a time when eating habits and food preferences are being formed. We provide an overview of the approaches to the provision, regulation, and improvement of preschool and primary school meals in the UK, Sweden, and Australia, three countries which vary in their degree of centralisation and regulation of school meals. Sweden has a centralised approach; all children receive free meals, and a pedagogical approach to meals is encouraged. Legislation demands that meals are nutritious. The UK system is varied and decentralised. Meals in most primary schools are regulated by food-based standards, but preschool-specific meal standards only exist in Scotland. The UK uses food groups (starchy foods, fruit and vegetables, proteins and dairy) in a healthy plate approach. Australian States and Territories all employ guidelines for school canteen food, predominantly using a "traffic light" approach outlining recommended and discouraged foods; however, most children bring food from home and are not covered by this guidance. The preschool standards state that food provided should be nutritious. We find that action is often lacking in the preschool years, and suggest that consistent policies, strong incentives for compliance, systematic monitoring, and an acknowledgement of the broader school eating environment (including home provided food) would be beneficial.