Primary Care and Emergency Department Management of the Patient With Duchenne Muscular Dystrophy

Primary care providers (PCPs) are usually the first point of contact with the health care system for patients with Duchenne muscular dystrophy (DMD), and patients often present to emergency departments in which providers have little experience in dealing with this condition. With this article, we give primary care and emergency medicine providers a background in the common issues that affect people with DMD. By acquiring some specialized knowledge about the multisystem medical complications of DMD and by applying general principles of primary care, such as timely immunization, anticipatory safety counseling, behavioral screening, and routine nutritional and developmental assessments, the PCP can be a valued and effective medical provider to patients with DMD. The PCP can provide access to and effective coordination among the patient's specialty caregivers. Moreover, the PCP can become a trusted advisor to the patient and his family about important medical decisions, as well as issues in the psychosocial, behavioral, and educational domains. This article also contains a "pocket guide" used to assess and manage common urgent medical problems that cause patients with DMD to seek care in the emergency department. With the background information discussed in this article, both PCPs and emergency medicine physicians can skillfully care for patients with DMD in their respective settings, optimizing patient outcomes

Uniaxial compressive strengths of rocks drilled at Gale crater, Mars

Measuring the physical properties of geological materials is important for understanding geologic history. Yet there has never been an instrument with the purpose of measuring mechanical properties of rocks sent to another planet. The Mars Science Laboratory (MSL) rover employs the Powder Acquisition Drill System (PADS), which provides direct mechanical interaction with Martian outcrops. While the objective of the drill system is not to make scientific measurements, the drill's performance is directly influenced by the mechanical properties of the rocks it drills into. We have developed a methodology that uses the drill to indicate the uniaxial compressive strengths of rocks through comparison with performance of an identically assembled drill system in terrestrial samples of comparable sedimentary class. During this investigation, we utilize engineering data collected on Mars to calculate the percussive energy needed to maintain a prescribed rate of penetration and correlate that to rock strength

Effects of steroids and angiotensin converting enzyme inhibition on circumferential strain in boys with Duchenne muscular dystrophy: a cross-sectional and longitudinal study utilizing cardiovascular magnetic resonance

<p>Abstract</p> <p>Background</p> <p>Steroid use has prolonged ambulation in Duchenne muscular dystrophy (DMD) and combined with advances in respiratory care overall management has improved such that cardiac manifestations have become the major cause of death. Unfortunately, there is no consensus for DMD-associated cardiac disease management. Our purpose was to assess effects of steroid use alone or in combination with angiotensin converting enzyme inhibitors (ACEI) or angiotension receptor blocker (ARB) on cardiovascular magnetic resonance (CMR) derived circumferential strain (ε_cc).</p> <p>Methods</p> <p>We used CMR to assess effects of corticosteroids alone (Group A) or in combination with ACEI or ARB (Group B) on heart rate (HR), left ventricular ejection fraction (LVEF), mass (LVM), end diastolic volume (LVEDV) and circumferential strain (ε_cc) in a cohort of 171 DMD patients >5 years of age. Treatment decisions were made independently by physicians at both our institution and referral centers and not based on CMR results.</p> <p>Results</p> <p>Patients in Group A (114 studies) were younger than those in Group B (92 studies)(10 ± 2.4 vs. 12.4 ± 3.2 years, p < 0.0001), but HR, LVEF, LVEDV and LVM were not different. Although ε_ccmagnitude was lower in Group B than Group A (-13.8 ± 1.9 vs. -12.8 ± 2.0, p = 0.0004), age correction using covariance analysis eliminated this effect. In a subset of patients who underwent serial CMR exams with an inter-study time of ~15 months, ε_ccworsened regardless of treatment group.</p> <p>Conclusions</p> <p>These results support the need for prospective clinical trials to identify more effective treatment regimens for DMD associated cardiac disease.</p

Exploring adults’ experiences of sedentary behaviour and participation in nonworkplace interventions designed to reduce sedentary behaviour: a thematic synthesis of qualitative studies

Background: Sedentary behaviour is any waking behaviour characterised by an energy expenditure of ≤1.5 metabolic equivalent of task while in a sitting or reclining posture. Prolonged bouts of sedentary behaviour have been associated with negative health outcomes in all age groups. We examined qualitative research investigating perceptions and experiences of sedentary behaviour and of participation in non-workplace interventions designed to reduce sedentary behaviour in adult populations. Method: A systematic search of seven databases (MEDLINE, AMED, Cochrane, PsychINFO, SPORTDiscus, CINAHL and Web of Science) was conducted in September 2017. Studies were assessed for methodological quality and a thematic synthesis was conducted. Prospero database ID: CRD42017083436. Results: Thirty individual studies capturing the experiences of 918 individuals were included. Eleven studies examined experiences and/or perceptions of sedentary behaviour in older adults (typically ≥60 years); ten studies focused on sedentary behaviour in people experiencing a clinical condition, four explored influences on sedentary behaviour in adults living in socio-economically disadvantaged communities, two examined university students’ experiences of sedentary behaviour, two on those of working-age adults, and one focused on cultural influences on sedentary behaviour. Three analytical themes were identified: 1) the impact of different life stages on sedentary behaviour 2) lifestyle factors influencing sedentary behaviour and 3) barriers and facilitators to changing sedentary behaviour. Conclusions: Sedentary behaviour is multifaceted and influenced by a complex interaction between individual, environmental and socio-cultural factors. Micro and macro pressures are experienced at different life stages and in the context of illness; these shape individuals’ beliefs and behaviour related to sedentariness. Knowledge of sedentary behaviour and the associated health consequences appears limited in adult populations, therefore there is a need for provision of accessible information about ways in which sedentary behaviour reduction can be integrated in people’s daily lives. Interventions targeting a reduction in sedentary behaviour need to consider the multiple influences on sedentariness when designing and implementing interventions

Results of a feasibility randomised controlled study of the guidelines for exercise in multiple sclerosis project

There is increasing recognition that exercise is an efficacious strategy for managing many consequences of multiple sclerosis (MS), yet persons with MS are not engaging in sufficient exercise for accruing health benefits. Poor exercise uptake might be associated with the design of previous research. We conducted a randomised controlled trial (RCT) for examining the feasibility of a 4-month home-based, exercise-training program designed based on recent physical activity guidelines for MS and supplemented by behavioural strategies for compliance. Feasibility was assessed in the domains of process (e.g., recruitment), resource (e.g., monetary costs), management (e.g., personnel time requirements) and scientific outcomes (e.g., treatment effect). We recruited persons with mild-to-moderate MS who were randomised into an intervention or wait-list control condition. Intervention participants received a pedometer, elastic resistance bands, DVD, training manual, calendars, log-book, video coaching calls and newsletters. Participants in both conditions completed home-based assessments before and after the 4-month period. Ninety-nine persons with MS were assessed for eligibility, and 57 were randomised. Fifty-one persons completed the study (90%). Total costs of the study were US $5331.03. Personnel time to conduct the study totaled 263 h. Participants in the intervention group complied fully with 71% of all exercise sessions. There was a moderate increase in self-reported exercise behaviour of the intervention participants as measured by the Godin Leisure-Time Exercise Questionnaire (d ≥ 0.5). The results support the feasibility and acceptability of a home-based exercise intervention based on physical activity guidelines and supplemented with behavioural strategies for adults with mild-to-moderate MS