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Chronic pain in hypermobility syndrome and Ehlers-Danlos syndrome (hypermobility type)

Generalized joint hypermobility (GJH) is highly prevalent among patients diagnosed with chronic pain. When GJH is accompanied by pain in ≥4 joints over a period ≥3 months in the absence of other conditions that cause chronic pain, the hypermobility syndrome (HMS) may be diagnosed. In addition, GJH is also a clinical sign that is frequently present in hereditary diseases of the connective tissue, such as the Marfan syndrome, osteogenesis imperfecta, and the Ehlers-Danlos syndrome. However, within the Ehlers-Danlos spectrum, a similar subcategory of patients having similar clinical features as HMS but lacking a specific genetic profile was identified: Ehlers-Danlos syndrome hypermobility type (EDS-HT). Researchers and clinicians have struggled for decades with the highly diverse clinical presentation within the HMS and EDS-HT phenotypes (Challenge 1) and the lack of understanding of the pathological mechanisms that underlie the development of pain and its persistence (Challenge 2). In addition, within the HMS/EDS-HT phenotype, there is a high prevalence of psychosocial factors, which again presents a difficult issue that needs to be addressed (Challenge 3). Despite recent scientific advances, many obstacles for clinical care and research still remain. To gain further insight into the phenotype of HMS/EDS-HT and its mechanisms, clearer descriptions of these populations should be made available. Future research and clinical care should revise and create consensus on the diagnostic criteria for HMS/EDS-HT (Solution 1), account for clinical heterogeneity by the classification of subtypes within the HMS/EDS-HT spectrum (Solution 2), and create a clinical core set (Solution 3).

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Wheelchair Shuttle Test for Assessing Aerobic Fitness in Youth With Spina Bifida: Validity and Reliability

Background Testing aerobic fitness in youth is important because of expected relationships with health. Objective The purpose of the study was to estimate the validity and reliability of the Shuttle Ride Test in youth who have spina bifida and use a wheelchair for mobility and sport. Design Ths study is a validity and reliability study. Methods The Shuttle Ride Test, Graded Wheelchair Propulsion Test, and skill-related fitness tests were administered to 33 participants for the validity study (age = 14.5 ± 3.1 y) and to 28 participants for the reliability study (age = 14.7 ± 3.3 y). Results No significant differences were found between the Graded Wheelchair Propulsion Test and the Shuttle Ride Test for most cardiorespiratory responses. Correlations between the Graded Wheelchair Propulsion Test and the Shuttle Ride Test were moderate to high (r = .55–.97). The variance in peak oxygen uptake (VO2peak) could be predicted for 77% of the participants by height, number of shuttles completed, and weight, with large prediction intervals. High correlations were found between number of shuttles completed and skill-related fitness tests (CI = .73 to −.92). Intraclass correlation coefficients were high (.77–.98), with a smallest detectable change of 1.5 for number of shuttles completed and with coefficients of variation of 6.2% and 6.4% for absolute VO2peak and relative VO2peak, respectively. Conclusions When measuring VO2peak directly by using a mobile gas analysis system, the Shuttle Ride Test is highly valid for testing VO2peak in youth who have spina bifida and use a wheelchair for mobility and sport. The outcome measure of number of shuttles represents aerobic fitness and is also highly correlated with both anaerobic performance and agility. It is not possible to predict VO2peak accurately by using the number of shuttles completed. Moreover, the Shuttle Ride Test is highly reliable in youth with spina bifida, with a good smallest detectable change for the number of shuttles completed.

Exercise training in pediatric patients with end-stage renal disease

The objective of this study was to determine the feasibility and efficacy of an exercise training program to improve exercise capacity and fatigue level in pediatric patients with end-stage renal disease (ESRD). Twenty children on dialysis intended to perform a 12-week graded community-based exercise program. Exercise capacity and fatigue level were studied; muscle force and health-related quality of life were secondary outcomes. All outcomes were measured at baseline (T = 0) and after intervention (T = 1). Fourteen of the 20 patients (70%) either did not start the program or did not complete the program. Of these patients, seven did not complete or even start the exercise program because of a combination of lack of time and motivational problems. Six patients were not able to continue the program or were unable to do the follow-up measurements because of medical problems. Exercise capacity and muscle strength was higher after the exercise program in the children who completed the training. In conclusion, exercise training is difficult to perform in children with ESRD and is not always feasible in real-life situations for many children with ESRD.

The effects of wheelchair mobility skills and exercise training on physical activity, fitness, skills and confidence in youth using a manual wheelchair

Purpose: To evaluate the effects of a combination of wheelchair mobility skills (WMS) training and exercise training on physical activity (PA), WMS, confidence in wheelchair mobility, and physical fitness. Methods: Youth using a manual wheelchair (n = 60) participated in this practice-based intervention, with a waiting list period (16 weeks), exercise training (8 weeks), WMS training (8 weeks), and follow-up (16 weeks). Repeated measures included: PA (Activ8), WMS (Utrecht Pediatric Wheelchair Mobility Skills Test), confidence in wheelchair mobility (Wheelchair Mobility Confidence Scale), and physical fitness (cardiorespiratory fitness, (an)aerobic performance) and were analysed per outcome parameter using a multilevel model analyses. Differences between the waiting list and training period were determined with an unpaired sample t-test. Results: Multilevel model analysis showed significant positive effects for PA (p = 0.01), WMS (p < 0.001), confidence in wheelchair mobility (p < 0.001), aerobic (p < 0.001), and anaerobic performance (p < 0.001). Unpaired sample t-tests underscored these effects for PA (p < 0.01) and WMS (p < 0.001). There were no effects on cardiorespiratory fitness. The order of training (exercise before WMS) had a significant effect on confidence in wheelchair mobility. Conclusions: A combination of exercise and WMS training appears to have significant positive long-term effects on PA, WMS, confidence in wheelchair mobility, and (an)aerobic performance in youth using a manual wheelchair.Implications for rehabilitationExercise training and wheelchair mobility skills (WMS) training can lead to a sustained improvement in physical activity (PA) in youth using a manual wheelchair.These combined trainings can also lead to a sustained increase in WMS, confidence in wheelchair mobility, and (an)aerobic performance.More attention is needed in clinical practice and in research towards improving PA in youth using a manual wheelchair.

The association between muscle strength and activity limitations in patients with the hypermobility type of Ehlers-Danlos syndrome: the impact of proprioception

PURPOSE: The patients diagnosed with Ehlers-Danlos Syndrome Hypermobility Type (EDS-HT) are characterized by pain, proprioceptive inacuity, muscle weakness, potentially leading to activity limitations. In EDS-HT, a direct relationship between muscle strength, proprioception and activity limitations has never been studied. The objective of the study was to establish the association between muscle strength and activity limitations and the impact of proprioception on this association in EDS-HT patients.METHODS: Twenty-four EDS-HT patients were compared with 24 controls. Activity limitations were quantified by Health Assessment Questionnaire (HAQ), Six-Minute Walk test (6MWT) and 30-s chair-rise test (30CRT). Muscle strength was quantified by handheld dynamometry. Proprioception was quantified by movement detection paradigm. In analyses, the association between muscle strength and activity limitations was controlled for proprioception and confounders.RESULTS: Muscle strength was associated with 30CRT (r = 0.67, p = <0.001), 6MWT (r = 0.58, p = <0.001) and HAQ (r = 0.63, p= <0.001). Proprioception was associated with 30CRT (r = 0.55, p < 0.001), 6MWT (r = 0.40, p = <0.05) and HAQ (r = 0.46, p < 0.05). Muscle strength was found to be associated with activity limitations, however, proprioceptive inacuity confounded this association.CONCLUSIONS: Muscle strength is associated with activity limitations in EDS-HT patients. Joint proprioception is of influence on this association and should be considered in the development of new treatment strategies for patients with EDS-HT. Implications for rehabilitation Reducing activity limitations by enhancing muscle strength is frequently applied in the treatment of EDS-HT patients. Although evidence regarding treatment efficacy is scarce, the current paper confirms the rationality that muscle strength is an important factor in the occurrence of activity limitations in EDS-HT patients. Although muscle strength is the most dominant factor that is associated with activity limitations, this association is confounded by proprioception. In contrast to common belief proprioception was not directly associated with activity limitations but confounded this association. Controlling muscle strength on the bases of proprioceptive input may be more important for reducing activity limitations than just enhancing sheer muscle strength.