Since the publication of the Duchenne muscular dystrophy (DMD) care considerations in 2010, multidisciplinary care of this severe, progressive neuromuscular disease has evolved. In conjunction with improved patient survival, a shift to more anticipatory diagnostic and therapeutic strategies has occurred, with a renewed focus on patient quality of life. In 2014, a steering committee of experts from a wide range of disciplines was established to update the 2010 DMD care considerations, with the goal of improving patient care. The new care considerations aim to address the needs of patients with prolonged survival, to provide guidance on advances in assessments and interventions, and to consider the implications of emerging genetic and molecular therapies for DMD. The committee identified 11 topics to be included in the update, eight of which were addressed in the original care considerations. The three new topics are primary care and emergency management, endocrine management, and transitions of care across the lifespan. In part 1 of this three-part update, we present care considerations for diagnosis of DMD and neuromuscular, rehabilitation, endocrine (growth, puberty, and adrenal insufficiency), and gastrointestinal (including nutrition and dysphagia) management.

A coordinated, multidisciplinary approach to care is essential for optimum management of the primary manifestations and secondary complications of Duchenne muscular dystrophy (DMD). Contemporary care has been shaped by the availability of more sensitive diagnostic techniques and the earlier use of therapeutic interventions, which have the potential to improve patients’ duration and quality of life. In part 2 of this update of the DMD care considerations, we present the latest recommendations for respiratory, cardiac, bone health and osteoporosis, and orthopaedic and surgical management for boys and men with DMD. Additionally, we provide guidance on cardiac management for female carriers of a disease-causing mutation. The new care considerations acknowledge the effects of long-term glucocorticoid use on the natural history of DMD, and the need for care guidance across the lifespan as patients live longer. The management of DMD looks set to change substantially as new genetic and molecular therapies become available.

clinicaltrials.gov Identifier: NCT00195858.

OBJECTIVETo determine in Canadian children aged <18 years the 1) incidence of type 2 diabetes, medication-induced diabetes, and monogenic diabetes; 2) clinical features of type 2 diabetes; and 3) coexisting morbidity associated with type 2 diabetes at diagnosis.RESEARCH DESIGN AND METHODSThis Canadian prospective national surveillance study involved a network of pediatricians, pediatric endocrinologists, family physicians, and adult endocrinologists. Incidence rates were calculated using Canadian Census population data. Descriptive statistics were used to illustrate demographic and clinical features.RESULTSFrom a population of 7.3 million children, 345 cases of non–type 1 diabetes were reported. The observed minimum incidence rates of type 2, medication-induced, and monogenic diabetes were 1.54, 0.4, and 0.2 cases per 100,000 children aged <18 years per year, respectively. On average, children with type 2 diabetes were aged 13.7 years and 8% (19 of 227) presented before 10 years. Ethnic minorities were overrepresented, but 25% (57 of 227) of children with type 2 diabetes were Caucasian. Of children with type 2 diabetes, 95% (206 of 216) were obese and 37% (43 of 115) had at least one comorbidity at diagnosis.CONCLUSIONSThis is the first prospective national surveillance study in Canada to report the incidence of type 2 diabetes in children and also the first in the world to report the incidence of medication-induced and monogenic diabetes. Rates of type 2 diabetes were higher than expected with important regional variation. These results support recommendations that screening for comorbidity should occur at diagnosis of type 2 diabetes.

Background:In adults, anthropometric measures of central adiposity, such as waist-height ratio (WHtR) and waist circumference (WC), are more strongly associated with cardiometabolic risks than BMI. Methods: To provide similar quantitative tools for North American children, we created smoothed centile charts and LMS tables for WHtR and WC based on data from the US National Health and Nutrition Survey, cycle III (NHANES III, N = 11,930 aged 2-24 y 1988-1994. results: Applying these reference charts to subsequent NHANES survey cycles, 1999-2012) demonstrated a significant mean increase in both Z-scores of approximately 0.30 SD. In measuring the strength of the association between anthropometric measures and cardio-metabolic risk factors, a unit change in Z-scores for WHtR, WC, and BMI significantly increased the odds of an adverse outcome in all cases (1.18-2.03, P < 0.0001). Z-scores for both measures of central adiposity were significantly more strongly associated with cardio-metabolic comorbidities than BMI-Z. conclusion: Since Z-scores permit standardized comparisons across ages and genders, they are useful measures of central adiposity in both clinical or research settings. By providing LMS tables for children and adolescents based on North American reference data, we hope to provide quantitative tools for the study of obesity and its complications.W ith increasing prevalence of obesity at younger ages, anthropometric measures other than BMI have been proposed as predictors for the development of cardio-metabolic risks, such as diabetes and hyperlipidemia. In particular, a waist-height ratio (WHtR) > 0.5 has been shown to be a stronger predictor than BMI at all ages, presumably because the former is a better direct measure of central adiposity (1-3). Nevertheless, this "one-size-fits all" approach to WHtR ignores the natural variation of this measure with age and gender in children and adolescents.In younger subjects, waist circumference (WC) has also been shown to be at least as strong a predictor as BMI for metabolic complications (4,5). Although several groups have applied the LMS model to create smoothed centile charts for waist-circumference in North American children and adolescents (6-8), none published tables of LMS parameters needed to calculate Z-score or exact centiles in subsequent studies. In addition to dissimilarities between populations, there were also differences in measurement techniques: Although both Statistics Canada and NHANES recommend a validated protocol for measuring WC at the level of the iliac crest in the mid-axillary line (9), reference data from outside North America are often based on different choices for the physical landmarks used to measure WC (see Discussion for details).Our primary objective was to apply the LMS method to create gender-specific reference growth charts for both WHtR and WC in children aged 5-19 y of age in the North American NHANES III cohort (1988)(1989)(1990)(1991)(1992)(1993)(1994), a North American reference population that predates much of the recent...

Resistance training, alone or in combination with aerobic training, may provide psychological benefits in adolescents with overweight or obesity, and therefore could be an alternative to aerobic training for some individuals in the biological and psychological management of adolescent obesity.

ObjectiveTo examine the association between duration and type of screen time (TV, video games, computer time) and blood pressure (BP) and lipids in overweight and obese adolescents.DesignThis is a cross-sectional study of 282 overweight or obese adolescents aged 14–18 years (86 males, 196 females) assessed at baseline prior to beginning a lifestyle intervention study for weight control. Sedentary behaviours, defined as hours per day spent watching TV, playing video games, recreational computer use and total screen time were measured by self-report. We examined the associations between sedentary behaviours and BP and lipids using multiple linear regression.ResultsSeated video gaming was the only sedentary behaviour associated with elevated BP and lipids before and after adjustment for age, sex, pubertal stage, parental education, body mass index (BMI), caloric intake, percent intake in dietary fat, physical activity (PA) duration, and PA intensity. Specifically, video gaming remained positively associated with systolic BP (adjusted r = 0.13, β = 1.1, p<0.05) and total cholesterol/HDL ratio (adjusted r = 0.12, β = 0.14, p<0.05).ConclusionsPlaying video games was the only form of sedentary behaviour that was independently associated with increased BP and lipids. Our findings provide support for reducing time spent playing seated video games as a possible means to promote health and prevent the incidence of cardiovascular disease (CVD) risk factors in this high risk group of overweight and obese adolescents. Future research is needed to first replicate these findings and subsequently aim to elucidate the mechanisms linking seated video gaming and elevated BP and lipids in this high risk population.Trial RegistrationClinicaltrials.gov NCT00195858

clinicaltrials.gov Identifier: NCT01825798.