RARE-Bestpractices: a platform for sharing best practices for the management of rare diseases

From 7th European Conference on Rare Diseases and Orphan Products (ECRD 2014).Rare diseases; clinical practice guidelines; recommendations. RARE-Bestpractices (http://www.rarebestpractices.eu) is a 4-year project (2013-2016) funded by the EC FP7. The project aims at improving clinical management of patients with rare diseases (RD) and at narrowing the existing gap in quality of healthcare among countries. Methods: RARE-Bestpractices (http://www.rarebestpractices.eu) involves 9 EU countries, including 15 partners from academic institutions, governmental bodies, patient organizations and networks, which will exploit the added value of integrating different contributions and viewpoints. The platform is developed involving both experts in RD research as well as experts in clinical practice guidelines (CPG) and systematic reviews. Results: Project expected outputs include: 1) identification of challenges to be considered in deriving high quality standards for CPG on RD; 2) transparent procedures and criteria for the evaluation of CPG and their collection in a publicly searchable database; 3) identification of notation criteria to improve user understandability and implementation of CPG; 4) production of mechanisms to assess RD clinical research needs; 5) development of training activities targeted to key stakeholders to disseminate process and tools for developing and evaluating CPG; 6) the publication of a new scientific journal (http://rarejournal.org). Discussion: RARE-Bestpractices addresses the demands from both patients and health care providers for updated and high quality CPG on RD. The project will meet the requirements laid down by to the Directive 2011/24/EU, which endorses EU MS to develop European Reference Networks (ERNs) for RD; in fact, one main criterion for ERNs should be the competence to produce CPG and actively disseminate them among Centers of Expertise.N

Validation of Methods for Determination of Metabolic Rate in the Edholm Scale and ISO 8996

The aim of this study was to validate the Edholm scale (Edholm, 1966) and the ISO 8996 standard (International Organization for Standardization [ISO], 1990) by comparing the metabolic rates estimated for both methods with the actual measured metabolic rate (MMeas) in 6 manual material handling tasks simulated under laboratory conditions. The metabolic rate was calculated from oxygen consumption V˙ O2 (19 participants) according to Standard No. ISO 8996 (ISO, 1990). Additionally, the participants estimated perceived exertion using the Borg scale. The metabolic rates derived from the Edholm scale (MEdh) overestimated 5 of 6 activities by 34–50% (< =.05). The metabolic rates derived from ISO 8996 (MISO) overestimated all activities by 7–38% (< =.05)

Self-Rated Physical Loads of Work Tasks Among Firefighters

Objectives. The present study sought to identify firefighters’ rated physical demands for the most frequently occurring work tasks and to determine if the ratings differed between full-time and part-time firefighters to help create a basis for the development of physical employment tests for firefighters. Methods. An extensive questionnaire was completed by 125 and 68 firefighters in 2000 and 2010, respectively. The data were analysed with the Mann–Whitney U test and binominal test and ranked on the basis of the responses in each category. Results. Significant differences were seen between the full- and part-time firefighters. The work tasks rated as the most physically strenuous in terms of aerobic fitness, muscle strength, work posture and body control by most respondents were smoke diving upstairs (carrying a hose), victim rescue in different ways, carrying a stretcher over terrain and pulling a hose. Conclusions. Physically strenuous work tasks should be included in the end-point performance variables used to select physical performance tests for firefighters. The part-time firefighters with no experience in several of the work tasks suggests that work-related exercises are important if both groups of firefighters are expected to do similar work

Occupational exposure to cold thermal environments: a field study in Portugal

Abstract The present work is essentially dedicated to the study of cold thermal environments. The analysis includes 32 industrial units from 6 activity sectors and the measurements were carried out in 101 workplaces. Different environmental conditions were identified and a clear relationship with the different types of workplaces was established. The work environments were thus allocated to three typical exposure categories corresponding to freezing and refrigerating cold stores and free-running or controlled air temperature manufacturing workplaces. In order to characterize the level of cold exposure, the method proposed by ISO/TR 11079, Technical Report, 1st edn, International Organization for Standardization, Geneva (1993) was adopted. The results for each activity sector demonstrate that a significant percentage of workers are repeatedly exposed to extreme conditions with insufficient clothing insulation. A value between 20 and 40% corresponds to the most critical situation, where the selected clothing ensemble does not provide adequate insulation (I clr < IREQ min). The ideal scenario, represented by I clr values between IREQ min and IREQ neutral, shows the lowest percentages with an overall result of only 10%. When all the sectors are considered together, from a total of 3,667 workers, about one-third (1,151) are exposed to the cold. Among the workplaces under analysis, 14 are characterized by a continuous exposure greater than the DLE neutral. Those who work under such conditions, on average, have a time shift 60 min longer than the calculated DLE value

Field tests for evaluating the aerobic work capacity of firefighters

Working as a firefighter is physically strenuous, and a high level of physical fitness increases a firefighter's ability to cope with the physical stress of their profession. Direct measurements of aerobic capacity, however, are often complicated, time consuming, and expensive. The first aim of the present study was to evaluate the correlations between direct (laboratory) and indirect (field) aerobic capacity tests with common and physically demanding firefighting tasks. The second aim was to give recommendations as to which field tests may be the most useful for evaluating firefighters' aerobic work capacity. A total of 38 subjects (26 men and 12 women) were included. Two aerobic capacity tests, six field tests, and seven firefighting tasks were performed. Lactate threshold and onset of blood lactate accumulation were found to be correlated to the performance of one work task (rs = -0.65 and -0.63, p&lt;0.01, respectively). Absolute (mL·min(-1)) and relative (mL·kg(-1)·min(-1)) maximal aerobic capacity was correlated to all but one of the work tasks (rs = -0.79 to 0.55 and -0.74 to 0.47, p&lt;0.01, respectively). Aerobic capacity is important for firefighters' work performance, and we have concluded that the time to row 500 m, the time to run 3000 m relative to body weight (s·kg(-1)), and the percent of maximal heart rate achieved during treadmill walking are the most valid field tests for evaluating a firefighter's aerobic work capacity