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The purpose of this study was to investigate the influence of body function, activities and pain on the level of activity in adults with Kashin Beck Disease (KBD). Seventy-five KBD patients with a mean age of 54.8 years (SD 11.3) participated. Anthropometrics, range of joint motion (ROM) and muscle strength were measured as well as the time-up-and-go test and functional tests for the lower and upper extremities. Activity was assessed with the participation scale and the WHO DAS II. In the shoulder, elbow, hip and knee joints, a severe decrease in ROM and bilateral pain was noted. A decrease in muscle strength was observed in almost all muscles. The timed-up-and-go test scores decreased. No or mild restriction in activity was found in 35%, and 33% experienced a moderate restriction whereas 32% had severe to extreme restriction. Activities in the lower extremities were mildly to moderately correlated to ROM and muscle strength, whereas in the upper extremities activities were correlated to range of joint motion. Activity was significantly associated with ROM after correction for muscle strength, gender and age. Participation was borderline significantly associated with ROM after correction for muscle strength, gender, age and the activity time-up-and-go. In KBD adults, a severe decrease in activity is primarily caused by decrease in ROM. These findings have strong influence on rehabilitation and surgical intervention. [ABSTRACT FROM AUTHOR]

OBJECTIVE: To evaluate the physical function and fitness in survivors of childhood leukaemia 5-6 years after cessation of chemotherapy. MATERIALS AND METHODS: Thirteen children (six boys and seven girls; mean age 15.5 years) who were treated for leukaemia were studied 5-6 years after cessation of therapy. Physical function and fitness were determined by anthropometry, motor performance, muscle strength, anaerobic and aerobic exercise capacity. RESULTS: On motor performance, seven of the 13 patients showed significant problems in the hand-eye co-ordination domain. Muscle strength only showed a significantly lower value in the mean strength of the knee extensors. The aerobic and the anaerobic capacity were both significantly reduced compared to reference values. CONCLUSION: Even 5-6 years after cessation of childhood leukaemia treatment, there are still clear late effects on motor performance and physical fitness. Chemotherapy-induced neuropathy and muscle atrophies are probably the prominent cause for these reduced test results. Physical training might be indicated for patients surviving leukaemia to improve fitness levels and muscle strength. [ABSTRACT FROM AUTHOR]

Mark C Scheper,1,2 Janneke E de Vries,1–3 Jeanine Verbunt,3,4 Raoul HH Engelbert1,2 1School of Physiotherapy, Amsterdam University of Applied Sciences, Amsterdam, 2Department of Rehabilitation, Academic Medical Center, University of Amsterdam, Amsterdam, 3Department of Rehabilitation Medicine, CAPHRI School for Public Health and Primary Care, Maastricht University, Maastricht; 4Adelante, Center of expertise in Rehabilitation and Audiology, Hoensbroek, the Netherlands Abstract: 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). Keywords: chronic musculoskeletal pain, generalized joint hypermobility, hypermobility syndrome, Ehlers–Danlos hypermobility type

Purpose: Physical interventions during subacute rehabilitation have potential to improve functional recovery. This study explored the perspectives of children and adolescents with acquired brain injury (ABI) and their parents with respect to physical rehabilitation during the subacute phase., Methods: Thirteen children and adolescents with ABI and their parents were included and interviewed using semi-structured interviews. Interview transcripts were analysed using inductive thematic analysis approach., Results: Six themes were identified: 1) beliefs of physical rehabilitation, 2) content of physical rehabilitation, 3) tailored care, 4) impact of context, 5) communication and 6) transition. The importance of intensive physical practice was widely supported. The positive can-do mentality was emphasised to create an atmosphere of hope, meaning that every effort would be made to achieve maximum recovery. Intensive involvement of parents is considered essential during subacute rehabilitation including an open and mutual dialogue about the focus of rehabilitation, therapy goals and future participation in their own environment., Conclusions: Our findings highlight the need for an intensive rehabilitation approach, tailored to the individual's needs. The perspectives of children and adolescents and their parents in our study contribute to a better understanding of factors that are important for optimal recovery through physical rehabilitation during the subacute phase.

Objective: To develop consensus among experts to guide physical rehabilitation in children and adolescents with acquired brain injury during the subacute phase., Design: International Delphi study., Methods: A 3-round online Delphi study was conducted with 11 international experts in rehabilitation for children and adolescents with acquired brain injury. The first round consisted of open-ended questions; the second and third round consisted of ranking 139 statements on a 5-point Likert scale., Results: The panel reached consensus on 116/139 statements. Consensus was reached on the importance of age, pre-injury developmental stage and the clinical presentation of the child when determining content and focus of physical rehabilitation. In addition, consensus was reached on the importance of participation-focused interventions, and involvement of family members in goal-setting and therapeutic activities. Although dosage was deemed very important, no consensus was reached for determination of dose-response variables to suit and influence the child's needs., Conclusion: This study provides a framework for clinicians to design physical rehabilitation interventions in children with acquired brain injury in the sub-acute recovery phase. The promotion of physical activity in meaningful contexts and involvement of family members are considered as important components to optimize recovery.

Objective: 'Better By Moving' is a multifaceted intervention developed and implemented in collaboration with patients and healthcare professionals to improve physical activity in hospitalized adults. This study aimed to understand if, how and why 'Better By Moving' resulted in higher levels of physical activity by evaluating both outcomes and implementation process., Design: Mixed-methods study informed by the Medical Research Council guidance., Setting: Tertiary hospital., Participants: Adult patients admitted to surgery, haematology, infectious diseases and cardiology wards, and healthcare professionals., Measures: Physical activity was evaluated before and after implementation using the Physical Activity Monitor AM400 on one random day during hospital stay between 8 am and 8 pm. Furthermore, the time spent lying on bed, length of stay and discharge destination was investigated. The implementation process was evaluated using an audit trail, surveys and interviews., Results: There was no significant difference observed in physical activity (median [IQR] 23 [12-51] vs 27 [17-55] minutes, P  = 0.107) and secondary outcomes before-after implementation. The intervention components' reach was moderate and adoption was low among patients and healthcare professionals. Patients indicated they perceived more encouragement from the environment and performed exercises more frequently, and healthcare professionals signalled increased awareness and confidence among colleagues. Support (priority, resources and involvement) was perceived a key contextual factor influencing the implementation and outcomes., Conclusion: Although implementing 'Better By Moving' did not result in significant improvements in outcomes at our centre, the process evaluation yielded important insights that may improve the effectiveness of implementing multifaceted interventions aiming to improve physical activity during hospital stay.

Background: Chronic musculoskeletal pain (CMP), Generalized Joint Hypermobility (GJH) and pain-related fear have influence on physical functioning in adolescents., Aim: to evaluate differences in physical functioning between adolescents with CMP, GJH or the combination of both, and in addition evaluate the potential contribution of pain-related fear., Design: The design of this study was observational and cross-sectional., Setting: The adolescents with CMP were recruited by a physician in rehabilitation medicine and measured in the university outpatient rehabilitation clinic (Adelante/Maastricht University Medical Center+, the Netherlands). The adolescents without CMP were recruited in the Southern area of the Netherlands and measured in the university outpatient rehabilitation clinic (Adelante/Maastricht University Medical Center+, the Netherlands)., Population: Four subgroups of adolescents were included; 21 adolescents with CMP without GJH, 9 adolescents with CMP and GJH, 51 adolescents without CMP without GJH, and 11 adolescents without CMP with GJH., Methods: Outcome measures were muscle strength and endurance, motor performance, physical activity level, and pain-related fear. Hierarchical regression analyses were used to study differences in physical functioning and the contribution of pain-related fear in adolescents with/without CMP as well as with/without GJH., Results: Adolescents with CMP had decreased muscle strength (P=0.01), endurance (P=0.02), and lower motor performance (P<0.01) compared to adolescents without CMP. Higher levels of pain-related fear were related to decreased muscle strength (P=0.01), endurance (P<0.01), and motor performance (P<0.01). No differences in physical functioning and pain-related fear between hypermobile and non-hypermobile adolescents with CMP were found., Conclusions: Adolescents with CMP had decreased muscle strength and motor performance associated with increased levels of pain-related fear compared to adolescents without CMP. The association of being hypermobile with physical functioning is not more pronounced in adolescents with CMP., Clinical Rehabilitation Impact: No differences were found in physical functioning and pain-related fear between hypermobile adolescents with CMP compared to non-hypermobile adolescents with CMP. Future rehabilitation treatment in hypermobile adolescents with CMP should also focus on psychological components, such as pain-related fear.

Background: Idiopathic toe walking (ITW) is an exclusionary diagnosis given to healthy children who persist in walking on their toes after they should typically have achieved a heel-toe gait. The literature discusses conservative and surgical interventions using a variety of treatment modalities. Young children and children without a limitation in ankle dorsiflexion (the upwards movement of the foot towards the shin of the leg) are commonly treated with conservative interventions. Older children who continue toe walking and present with limitations in ankle dorsiflexion are sometimes treated with surgical procedures. This systematic review is needed to evaluate the evidence for any intervention for the treatment of ITW. The conclusions of this review may support decision making by clinicians caring for children with ITW. It may also assist families when deciding on treatment options for their children with ITW. Many of the treatments employed have financial implications for parents or healthcare services. This review also aims to highlight any deficits in the current research base., Objectives: To assess the effects of conservative and surgical interventions in children with ITW, specifically effects on gait normalisation, ankle range of motion, pain, frequency of recurrence, and any adverse effects., Search Methods: On 29 April 2019, we searched the Cochrane Neuromuscular Specialised Register, CENTRAL, MEDLINE, Embase, CINAHL Plus, and PEDro. We searched the following registers of clinical trials for ongoing and recently completed trials: the World Health Organization (WHO) International Clinical Trials Registry Platform (ICTRP, apps.who.int/trialsearch), and ClinicalTrials.gov (clinicaltrials.gov). We searched conference proceedings and other grey literature in the BIOSIS databases and System for Information on Grey Literature in Europe (OpenGrey, opengrey.eu). We searched guidelines via the Turning Research Into Practice database (TRIP, tripdatabase.com) and National Guideline Clearinghouse (guideline.gov). We did not apply language restrictions., Selection Criteria: We considered randomised or quasi-randomised trials for inclusion in the review if they involved participants diagnosed with ITW gait in the absence of a medical condition known to cause toe walking, or associated with toe walking. As there is no universally accepted age group for ITW, this review includes ITW at any age, who have been toe walking for more than six months, who can or cannot walk with a heel-toe gait, and who may or may not have limited dorsiflexion of the ankle joint., Data Collection and Analysis: We used standard Cochrane methodological procedures. The primary outcome was improvement in toe walking (defined as greater than 50% of time spent heel-toe walking). Secondary outcomes were active and passive range of motion of the ankle joint, pain, recurrence of ITW after treatment, and adverse events. We assessed the certainty of the evidence using the GRADE framework., Main Results: Four studies, comprising 104 participants, met the inclusion criteria. One study did not report data within the appropriate follow-up timeframe and data from two studies were insufficient for analysis. The single study from which we extracted data had 47 participants and was a randomised, controlled, parallel-group trial conducted in Sweden. It tested the hypothesis that combined treatment with serial casting and botulinum toxin type A (BTX) was more effective than serial casting alone in reducing ITW gait.This study found that more participants treated with BTX improved (defined as toe walking less than 50% of the time, as reported by parents) (risk ratio (RR) 1.21, 95% confidence interval (CI) 0.57 to 2.55; 1 trial, 46 participants; very low-certainty evidence). However, there was little or no difference between groups in passive ankle joint dorsiflexion range of movement on the right with the knee extended (mean difference (MD) -1.48º, 95% CI -4.13 to 1.16; 1 trial, 47 participants), on the right with the knee flexed (MD -0.04º, 95% CI -1.80 to 1.73; 1 trial, 46 participants), on the left with the knee flexed (MD 1.07, 95% CI -1.22 to 3.37), or on the left with the knee extended (MD 0.05, 95% CI -0.91 to 1.91). Nor was there a clear difference between the groups in recurrence of toe-walking gait (assessed via severity of toe walking (graded 1 (mild), 2 (moderate), or 3 (severe)) on gait analysis, analysed as continuous data: MD 0.34 points, 95% CI -0.09 to 0.78; 46 participants). In principle, MDs greater than zero (i.e.) positive values) would favour BTX and casting and negative values would favour casting alone. We have not reported effects as better or worse because all results were from evidence of very low certainty. We downgraded the certainty of evidence because of study limitations (outcome assessment was not blinded) and imprecision. Outcomes of pain and active range of motion were not reported in the included study.In terms of adverse events, calf pain was reported twice in the casting-only group and three times in the BTX group. There were three minor skin problems in each group and one reported case of pain directly after BTX injection. The report did not state if calf pain and skin irritation were from the same or different participants. The study authors reported that adverse events did not alter treatment adherence., Authors' Conclusions: The certainty of evidence from one study, which compared serial casting with serial casting with BTX for ITW in children, was too low for conclusions to be drawn. A further three studies reported outcomes relating to BTX, footwear, exercises, and different types of orthoses as interventions, however the outcome data were too limited to assess their effects.

Objective: To evaluate the effectiveness of a specialized physical therapy (SPT) program on disability in cervical dystonia (CD) compared to regular physical therapy (RPT)., Design: A single-blinded randomized controlled trial., Setting: This study was performed by a physical therapist in a primary health care setting. Measurements were performed at baseline, 6 and 12 months in the botulinum toxin (BoNT) outpatient clinic of the neurology department., Participants: Patients with primary CD and stable on BoNT treatment for 1 year (N=96)., Main Outcome Measures: The primary outcome was disability assessed with the Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS). Secondary outcomes were pain, anxiety, depression, quality of life (QOL), and health related costs over 12 months., Results: A total of 72 participants (30 men, 42 women) finished the study: 40 received SPT, 32 RPT. No significant between group differences were found after 12 months of treatment (P=.326). Over these 12 months both groups improved significantly (P<.001) on the TWSTRS disability scale compared to baseline (SPT 1.7 points, RPT 1.0 points). Short Form 36 (SF-36) General Health Perceptions (P=.046) and self-perceived improvement (P=.007) showed significantly larger improvements after 12 months in favor of SPT. Total health related costs after 12 months were $1373±556 for SPT compared to $1614±917 for RPT., Conclusion: SPT revealed no significant differences compared to RPT after 12 months of treatment on the TWSTRS disability scale. Both groups showed similar improvements compared to baseline. Positive results in the SPT group were higher patient perceived effects and general health perception. Treatment costs were lower in the SPT group. With lower costs and similar effects, the SPT program seems to be the preferred program to treat CD., (Copyright © 2019 American Congress of Rehabilitation Medicine. Published by Elsevier Inc. All rights reserved.)

Background: For older adults, physical activity is vital for maintaining their health and ability to live independently. Home-based programs can help them achieve the recommended exercise frequency. An application for a tablet computer was developed to support older adults in following a personal training program. It featured goal setting, tailoring, progress tracking, and remote feedback., Objective: In line with the Medical Research Council Framework, which prescribes thorough testing before evaluating the efficacy with a randomized controlled trial, the aim of this study was to assess the usability of a tablet-based app that was designed to support older adults in doing exercises at home., Methods: A total of 15 older adults, age ranging from 69 to 99 years old, participated in a usability study that utilized a mixed-methods approach. In a laboratory setting, novice users were asked to complete a series of tasks while verbalizing their ongoing thoughts. The tasks ranged from looking up information about exercises and executing them to tailoring a weekly exercise schedule. Performance errors and time-on-task were calculated as proxies of effective and efficient usage. Overall satisfaction was assessed with a posttest interview. All responses were analyzed independently by 2 researchers., Results: The participants spent 13-85 seconds time-on-task. Moreover, 79% (11/14)-100% (14/14) participants completed the basic tasks with either no help or after having received 1 hint. For expert tasks, they needed a few more hints. During the posttest interview, the participants made 3 times more positive remarks about the app than negative remarks., Conclusions: The app that was developed to support older adults in doing exercises at home is usable by the target audience. First-time users were able to perform basic tasks in an effective and efficient manner. In general, they were satisfied with the app. Tasks that were associated with behavior execution and evaluation were performed with ease. Complex tasks such as tailoring a personal training schedule needed more effort. Learning effects, usefulness, and long-term satisfaction will be investigated through longitudinal follow-up studies., (©Sumit Mehra, Bart Visser, Nazli Cila, Jantine van den Helder, Raoul HH Engelbert, Peter JM Weijs, Ben JA Kröse. Originally published in JMIR Human Factors (http://humanfactors.jmir.org), 01.02.2019.)

Background: Physical activity can prevent or delay age-related impairments and prolong the ability of older adults to live independently. Community-based programs typically offer classes where older adults can exercise only once a week under the guidance of an instructor. The health benefits of such programs vary. Exercise frequency and the duration of the program play a key role in realizing effectiveness. An auxiliary home-based exercise program can provide older adults the opportunity to exercise more regularly over a prolonged period of time in the convenience of their own homes. Furthermore, mobile electronic devices can be used to motivate and remotely guide older adults to exercise in a safe manner. Such a blended intervention, where technology is combined with personal guidance, needs to incorporate behavior change principles to ensure effectiveness., Objective: The aim of this study was to identify theory-based components of a blended intervention that supports older adults to exercise at home., Methods: The Medical Research Council framework was used to develop the blended intervention. Insights from focus group, expert panels, and literature were combined into leading design considerations., Results: A client-server system had been developed that combined a tablet app with a database in the cloud and a Web-based dashboard that can be used by a personal coach to remotely monitor and guide older adults. The app contains several components that facilitate behavior change-an interactive module for goal setting, the ability to draw up a personal training schedule from a library containing over 50 exercise videos, progress monitoring, and possibilities to receive remote feedback and guidance of a personal coach., Conclusions: An evidence-based blended intervention was designed to promote physical activity among older adults. The underlying design choices were underpinned by behavior change techniques that are rooted in self-regulation. Key components of the tablet-supported intervention were a tailored program that accommodates individual needs, demonstrations of functional exercises, monitoring, and remote feedback. The blended approach combines the convenience of a home-based exercise program for older adults with the strengths of mobile health and personal guidance., (©Sumit Mehra, Bart Visser, Tessa Dadema, Jantine van den Helder, Raoul HH Engelbert, Peter JM Weijs, Ben JA Kröse. Originally published in JMIR Research Protocols (http://www.researchprotocols.org), 02.05.2018.)

Objective: Lowered pressure-pain thresholds have been demonstrated in adults with Ehlers-Danlos syndrome hypermobility type (EDS-HT), but whether these findings are also present in children is unclear. Therefore, the objectives of the study were to determine whether generalized hyperalgesia is present in children with hypermobility syndrome (HMS)/EDS-HT, explore potential differences in pressure-pain thresholds between children and adults with HMS/EDS-HT, and determine the discriminative value of generalized hyperalgesia., Methods: Patients were classified in 1 of 3 groups: HMS/EDS-HT, hypermobile (Beighton score ≥4 of 9), and healthy controls. Descriptive data of age, sex, body mass index, Beighton score, skin laxity, and medication usage were collected. Generalized hyperalgesia was quantified by the average pressure-pain thresholds collected from 12 locations. Confounders collected were pain locations/intensity, fatigue, and psychological distress. Comparisons between children with HMS/EDS-HT and normative values, between children and adults with HMS/EDS-HT, and corrected confounders were analyzed with multivariate analysis of covariance. The discriminative value of generalized hyperalgesia employed to differentiate between HMS/EDS-HT, hypermobility, and controls was quantified with logistic regression., Results: Significantly lower pressure-pain thresholds were found in children with HMS/EDS-HT compared to normative values (range -22.0% to -59.0%; P ≤ 0.05). When applying a threshold of 30.8 N/cm 2 for males and 29.0 N/cm 2 for females, the presence of generalized hyperalgesia discriminated between individuals with HMS/EDS-HT, hypermobility, and healthy controls (odds ratio 6.0)., Conclusion: Children and adults with HMS/EDS-HT are characterized by hypermobility, chronic pain, and generalized hyperalgesia. The presence of generalized hyperalgesia may indicate involvement of the central nervous system in the development of chronic pain., (© 2016, American College of Rheumatology.)

The purpose was to perform a systematic review of clinical assessment methods for classifying Generalized Joint Hypermobility (GJH), evaluate their clinimetric properties, and perform the best evidence synthesis of these methods. Four test assessment methods (Beighton Score [BS], Carter and Wilkinson, Hospital del Mar, Rotes-Querol) and two questionnaire assessment methods (Five-part questionnaire [5PQ], Beighton Score-self reported [BS-self]) were identified on children or adults. Using the Consensus-based Standards for selection of health Measurement Instrument (COSMIN) checklist for evaluating the methodological quality of the identified studies, all included studies were rated "fair" or "poor." Most studies were using BS, and for BS the reliability most of the studies showed limited positive to conflicting evidence, with some shortcomings on studies for the validity. The three other test assessment methods lack satisfactory information on both reliability and validity. For the questionnaire assessment methods, 5PQ was the most frequently used, and reliability showed conflicting evidence, while the validity had limited positive to conflicting evidence compared with test assessment methods. For BS-self, the validity showed unknown evidence compared with test assessment methods. In conclusion, following recommended uniformity of testing procedures, the recommendation for clinical use in adults is BS with cut-point of 5 of 9 including historical information, while in children it is BS with cut-point of at least 6 of 9. However, more studies are needed to conclude on the validity properties of these assessment methods, and before evidence-based recommendations can be made for clinical use on the "best" assessment method for classifying GJH. © 2017 Wiley Periodicals, Inc., (© 2017 Wiley Periodicals, Inc.)

New insights into the phenotype of Joint Hypermobility Syndrome (JHS) and Ehlers-Danlos Syndrome-hypermobile type (hEDS) have raised many issues in relation to classification, diagnosis, assessment, and treatment. Within the multidisciplinary team, physical therapy plays a central role in management of individuals with hypermobility related disorders. However, many physical therapists are not familiar with the diagnostic criteria, prevalence, common clinical presentation, and management. This guideline aims to provide practitioners with the state of the art regarding the assessment and management of children, adolescents, and adults with JHS/hEDS. Due to the complexity of the symptoms in the profile of JHS/hEDS, the International Classification of Functioning, Disability and Health (ICF) is adopted as a central framework whereby the umbrella term of disability is used to encompass functions, activities and participation, as well as environmental and personal factors. The current evidence-based literature regarding the management of JHS/hEDS is limited in size and quality and there is insufficient research exploring the clinical outcomes of a number of interventions. Multicenter randomized controlled trials are warranted to assess the clinical and cost-effectiveness of interventions for children and adults. Until further multicenter trials are conducted, clinical decision-making should be based on theoretical and the current limited research evidence. For all individuals diagnosed with JHS/hEDS, international consensus and combined efforts to identify risk profiles would create a better understanding of the pathological mechanisms and the potential for optimizing health care for affected individuals. © 2017 Wiley Periodicals, Inc., (© 2017 Wiley Periodicals, Inc.)

Preoperative functional status is a risk factor for developing postoperative complications (POC) in major abdominal and thoracic surgery, but this has hardly been evaluated in esophageal cancer patients undergoing esophagectomy. The aim of this prospective cohort study was to determine if preoperative functional status in esophageal cancer patients is associated with POC. From March 2012 to October 2014, esophageal cancer patients scheduled for esophagectomy at the outpatient clinic of a large tertiary referral center were eligible for the study. We measured inspiratory muscle strength, hand grip strength, physical activities, and health related quality of life as indicators of functional status one day before surgery. POC were scored according to the Clavien-Dindo Classification. We used univariate and multivariate backward regression analysis to determine the association between functional status and POC. We included 94 patients in the study and esophagectomy was performed in 90 patients from which 55 developed POC (61.1%). After multivariate analysis, none of the indicators of preoperative functional status were independently associated with POC (inspiratory muscle strength [OR 1.00; P = 0.779], hand grip strength [OR 0.99; P = 0.250], physical activities [OR 1.00; P = 0.174], and health related quality of life [OR 1.02; P = 0.222]). We concluded that preoperative functional status in our study cohort is not associated with POC after esophagectomy., (© 2016 International Society for Diseases of the Esophagus.)

In a continuation of a prospective longitudinal cohort study in a healthy population on the course of skull shape from birth to 24 months, at 5 years of age, 248 children participated in a follow-up assessment using plagiocephalometry (ODDI-oblique diameter difference index, CPI-cranio proportional index). Data from the original study sampled at birth, 7 weeks, 6, 12, and 24 months were used in two linear mixed models., Main Findings: (1) if deformational plagiocephaly (ODDI <104%) and/or positional preference at 7 weeks of age are absent, normal skull shape can be predicted at 5 years of age; (2) if positional preference occurs, ODDI is the highest at 7 weeks and decreases to a stable lowest value at 2 and 5 years of age; and (3) regarding brachycephaly, all children showed the highest CPI at 6 months of age with a gradual decrease over time., Conclusion: The course of skull deformation is favourable in most of the children in The Netherlands; at 5 years of age, brachycephaly is within the normal range for all children, whereas the severity of plagiocephaly is within the normal range in 80%, within the mild range in 19%, and within the moderate/severe range in 1%. Medical consumption may be reduced by providing early tailored counselling. What is Known: • Skull deformation prevalence increased after recommendations against Sudden Infant Death Syndrome, little is known about the longitudinal course. • Paediatric physical therapy intervention between 2 and 6 months of age reduces deformational plagiocephaly at 6 and 12 months of age. What is New: • The course of skull deformation is favourable in most of the children in The Netherlands; at 5 years of age, deformational brachycephaly is within the normal range for all children, whereas the severity of deformational plagiocephaly is within the normal range in 80%, within the mild range in 19%, and within the moderate to severe range in only 1%. • Paediatric physical therapy intervention does not influence the long-term outcome; it only influences the earlier decrease of the severity of deformational plagiocephaly., Competing Interests: Compliance with ethical standards All procedures performed in this study were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards. The Medical Ethics Committees of the University Medical Center Utrecht, The Netherlands (initial part of the study 0–24 months), of the Radboud University Medical Center Nijmegen, The Netherlands (5-year assessments) and of the Bernhoven Hospital Veghel (all assessments), The Netherlands, gave ethical approval. Written informed consent was obtained from all parents of the children in the cohort. Funding This study was made possible by a grant BU002/10 from the Scientific Committee of The Royal Dutch Association for Physiotherapy, Amersfoort, The Netherlands. There was independence of researchers from funders. Conflict of interest The authors declare that they have no conflicts of interest. The authors have indicated they have no financial relationships relevant to this article to disclose.

Objective: To (1) establish the association of the most common reported symptoms on disability; and (2) study the effectiveness of treatment on disability in patients with Ehlers-Danlos syndrome-hypermobility type (EDS-HT)/hypermobility syndrome (HMS)., Data Sources: An electronic search (Medical Subject Headings and free-text terms) was conducted in bibliographic databases CENTRAL/MEDLINE., Study Selection: Comparative, cross-sectional, longitudinal cohort studies and (randomized) controlled trials including patients with HMS/EDS-HT aged ≥17 years were considered for inclusion. A class of symptoms was included when 5 publications were available. In regards to treatment (physical, cognitive interventions), only (randomized) controlled trials were considered. Surgical and medicinal interventions were excluded., Data Extraction: Bias was assessed according to the methodological scoring tools of the Cochrane collaboration. Z-score transformations were applied to classify the extent of disability in comparison with healthy controls and to ensure comparability between studies., Data Synthesis: Initially, the electronic search yielded 714 publications, and 21 articles remained for analysis after selection. The following symptoms were included for meta-analysis: pain (n=12), fatigue (n=6), and psychological distress (n=7). Pain (r=.64, P=.021), fatigue (r=.91, P=.011), and psychological distress (r=.86, P=.018) had a significant impact on disability. Regarding treatment, a significant pain reduction was achieved by a variety of physical and cognitive approaches. Treatment effectiveness on disability was not established., Conclusions: Disability can affect patients with HMS/EDS-HT significantly and is highly correlated with both physical and psychological factors. Although evidence is available that physical and psychological treatment modalities can induce significant pain reduction, the evidence regarding disability reduction is lacking., (Copyright © 2016 American Congress of Rehabilitation Medicine. Published by Elsevier Inc. All rights reserved.)

Ageing is associated with a decline in daily functioning and mobility. A physically active life and physical exercise can minimize the decline of daily functioning and improve the physical-, psychological- and social functioning of older adults. Despite several advantages of group-based exercise programs, older adults participating in such interventions often do not meet the frequency, intensity or duration of exercises needed to gain health benefits. An exercise program that combines the advantages of group-based exercises led by an instructor with tailored home-based exercises can increase the effectiveness. Technology can assist in delivering a personalized program. The aim of the study was to determine the susceptibility of older adults currently participating in a nationwide group-based exercise program to such a blended exercise program. Eight focus-groups were held with adults of 55 years of age or older. Two researchers coded independently the remarks of the 30 participants that were included in the analysis according to the three key concepts of the Self Determination Theory: autonomy, competence, and relatedness. The results show that maintaining self-reliance and keeping in touch with others were the main motives to participate in the weekly group-based exercises. Participants recognized benefits of doing additional home-based exercises, but had concerns regarding guidance, safety, and motivation. Furthermore, some participants strongly rejected the idea to use technology to support them in doing exercises at home, but the majority was open to it. Insights are discussed how these findings can help design novel interventions that can increase the wellbeing of older adults and preserve an independent living.

Background: Cervical dystonia (CD) is characterized by involuntary muscle contractions causing abnormal postures and/or twisting movements of the head and neck. These motor symptoms can have a major impact on disability. Treatment with botulinum toxin injections aims to reduce motor symptoms, and therefore disability. Despite motor improvements, many patients still experience difficulties with performing daily life activities. To optimize treatment, other factors that determine disability should be identified., Objective: To explore and identify clinical characteristics that relate to disability in CD., Methods: Data on disability, severity of dystonia, anxiety, depression, pain and quality of life of 96 CD patients was analyzed with a principal component analysis (PCA). Multiple regression analysis was performed to determine which components derived from the PCA explain most of the variance in disability., Results: PCA revealed five components (disability, psychiatric features, pain, physical function and severity of dystonia), explaining 74.4% of the variance in disability. Multivariate association between disability and the other components was statistically significant (R 2 change 0.433, F change (4-86) = 22.39, p = .000). Psychiatric features had the largest contribution to disability (standardized beta = 0.555, p = 0.000) followed by pain (standardized beta = 0.232 p = 0.004). Physical functioning (standardized beta = 0.059 p = 0.507) and severity of dystonia (standardized beta = -0.001 p = 0.991) had no significant contribution., Conclusions: In CD patients, psychiatric features and pain are important determinants of disability. Interventions to reduce psychiatric problems and pain should have a more prominent role in the treatment of CD patients in order to improve disability levels., (Copyright © 2016 Elsevier Ltd. All rights reserved.)

Background: The study objective was to obtain consensus on physical therapy (PT) in the rehabilitation of critical illness survivors after hospital discharge. Research questions were: what are PT goals, what are recommended measurement tools, and what constitutes an optimal PT intervention for survivors of critical illness?, Methods: A Delphi consensus study was conducted. Panelists were included based on relevant fields of expertise, years of clinical experience, and publication record. A literature review determined five themes, forming the basis for Delphi round one, which was aimed at generating ideas. Statements were drafted and ranked on a 5-point Likert scale in two additional rounds with the objective to reach consensus. Results were expressed as median and semi-interquartile range, with the consensus threshold set at ≤0.5., Results: Ten internationally established researchers and clinicians participated in this Delphi panel, with a response rate of 80 %, 100 %, and 100 % across three rounds. Consensus was reached on 88.5 % of the statements, resulting in a framework for PT after hospital discharge. Essential handover information should include information on 15 parameters. A core set of outcomes should test exercise capacity, skeletal muscle strength, function in activities of daily living, mobility, quality of life, and pain. PT interventions should include functional exercises, circuit and endurance training, strengthening exercises for limb and respiratory muscles, education on recovery, and a nutritional component. Screening tools to identify impairments in other health domains and referral to specialists are proposed., Conclusions: A consensus-based framework for optimal PT after hospital discharge is proposed. Future research should focus on feasibility testing of this framework, developing risk stratification tools and validating core outcome measures for ICU survivors.

Background: Intensive care unit (ICU) stays often lead to reduced physical functioning. Change in physical functioning in patients in the ICU is inadequately assessed through available instruments. The de Morton Mobility Index (DEMMI), developed to assess mobility in elderly hospitalized patients, is promising for use in patients who are critically ill., Objective: The aim of this study was to evaluate the clinimetric properties of the DEMMI for patients in the ICU., Design: A prospective, observational reliability and validity study was conducted., Methods: To evaluate interrater and intrarater reliability (intraclass correlation coefficients), patients admitted to the ICU were assessed with the DEMMI during and after ICU stay. Validity was evaluated by correlating the DEMMI with the Barthel Index (BI), the Katz Index of Independence in Activities of Daily Living (Katz ADL), and manual muscle testing (MMT). Feasibility was evaluated based on the percentage of participants in which the DEMMI could be assessed, the floor and ceiling effects, and the number of adverse events., Results: One hundred fifteen participants were included (Acute Physiology and Chronic Health Evaluation II [APACHE II] mean score=15.2 and Sepsis-related Organ Failure Assessment [SOFA] mean score=7). Interrater reliability was .93 in the ICU and .97 on the wards, whereas intrarater reliability during the ICU stay was .68. Validity (Spearman rho coefficient) during the ICU stay was .56, -.45, and .57 for the BI, Katz ADL, and MMT, respectively. The DEMMI showed low floor and ceiling effects (2.6%) during and after ICU discharge. There were no major adverse events., Limitations: Rapid changes in participants' health status may have led to underestimation of intrarater reliability., Conclusion: The DEMMI was found to be clinically feasible, reliable, and valid for measuring mobility in an ICU population. Therefore, the DEMMI should be considered a preferred instrument for measuring mobility in patients during and after their ICU stay., (© 2016 American Physical Therapy Association.)

Purpose: To investigate the reliability and validity of the SQUASH physical activity (PA) questionnaire in a multi-ethnic population living in the Netherlands., Methods: We included participants from the HELIUS study, a population-based cohort study. In this study we included Dutch (n = 114), Turkish (n = 88), Moroccan (n = 74), South-Asian Surinamese (n = 98) and African Surinamese (n = 91) adults, aged 18-70 years. The SQUASH was self-administered twice to assess test-re-test reliability (mean interval 6-7 weeks) and participants wore an accelerometer and heart rate monitor (Actiheart) to enable assessment of construct validity., Results: We observed low test-re-test reliability; Intra class correlation coefficients ranged from low (0.05 for moderate/high intensity PA in African Surinamese women) to acceptable (0.78 for light intensity PA in Moroccan women). The discrepancy between self-reported and measured PA differed on the basis of the intensity of activity: self-reported light intensity PA was lower than measured but self-reported moderate/high intensity PA was higher than measured, with wide limits of agreement. The discrepancy between questionnaire and Actiheart measures of moderate intensity PA did not differ between ethnic minority and Dutch participants with correction for relevant confounders. Additionally, the SQUASH overestimated the number of participants meeting the Dutch PA norm; Cohen's kappas for the agreement were poor, the highest being 0.30 in Dutch women., Conclusion: We found considerable variation in the test-re-test reliability and validity of self-reported PA with no consistency based on ethnic origin. Our findings imply that the SQUASH does not provide a valid basis for comparison of PA between ethnic groups., Competing Interests: The authors have declared that no competing interests exist.

Background: Over 30 % of older patients experience hospitalization-associated disability (HAD) (i.e., loss of independence in Activities of Daily Living (ADLs)) after an acute hospitalization. Despite its high prevalence, the mechanisms that underlie HAD remain elusive. This paper describes the protocol for the Hospital-Associated Disability and impact on daily Life (Hospital-ADL) study, which aims to unravel the potential mechanisms behind HAD from admission to three months post-discharge., Methods/design: The Hospital-ADL study is a multicenter, observational, prospective cohort study aiming to recruit 400 patients aged ≥70 years that are acutely hospitalized at departments of Internal Medicine, Cardiology or Geriatrics, involving six hospitals in the Netherlands. Eligible are patients hospitalized for at least 48 h, without major cognitive impairment (Mini Mental State Examination score ≥15), who have a life expectancy of more than three months, and without disablement in all six ADLs. The study will assess possible cognitive, behavioral, psychosocial, physical, and biological factors of HAD. Data will be collected through: 1] medical and demographical data; 2] personal interviews, which includes assessment of cognitive impairment, behavioral and psychosocial functioning, physical functioning, and health care utilization; 3] physical performance tests, which includes gait speed, hand grip strength, balance, bioelectrical impedance analysis (BIA), and an activity tracker (Fitbit Flex), and; 4] analyses of blood samples to assess inflammatory and metabolic markers. The primary endpoint is additional disabilities in ADLs three months post-hospital discharge compared to ADL function two weeks prior to hospital admission. Secondary outcomes are health care utilization, health-related quality of life (HRQoL), physical performance tests, and mortality. There will be at least five data collection points; within 48 h after admission (H1), at discharge (H3), and at one (P1; home visit), two (P2; by telephone) and three months (P3; home visit) post-discharge. If the patient is admitted for more than five days, additional measurements will be planned during hospitalization on Monday, Wednesday, and Friday (H2)., Discussion: The Hospital-ADL study will provide information on cognitive, behavioral, psychosocial, physical, and biological factors associated with HAD and will be collected during and following hospitalization. These data may inform new interventions to prevent or restore hospitalization-associated disability.

Objective: To develop evidence-based recommendations for effective and safe diagnostic assessment and intervention strategies for the physiotherapy treatment of patients in intensive care units., Methods: We used the EBRO method, as recommended by the 'Dutch Evidence Based Guideline Development Platform' to develop an 'evidence statement for physiotherapy in the intensive care unit'. This method consists of the identification of clinically relevant questions, followed by a systematic literature search, and summary of the evidence with final recommendations being moderated by feedback from experts., Results: Three relevant clinical domains were identified by experts: criteria to initiate treatment; measures to assess patients; evidence for effectiveness of treatments. In a systematic literature search, 129 relevant studies were identified and assessed for methodological quality and classified according to the level of evidence. The final evidence statement consisted of recommendations on eight absolute and four relative contra-indications to mobilization; a core set of nine specific instruments to assess impairments and activity restrictions; and six passive and four active effective interventions, with advice on (a) physiological measures to observe during treatment (with stopping criteria) and (b) what to record after the treatment., Conclusions: These recommendations form a protocol for treating people in an intensive care unit, based on best available evidence in mid-2014., (© The Author(s) 2015.)

Objective: To determine the prevalence of generalized joint hypermobility (GJH) in Dutch children aged 5.5 years, and to examine the association between GJH and motor performance and development over time., Study Design: A prospective cohort of 249 children was recruited. GJH was assessed with the Beighton test at age 5.5 years. Motor performance was evaluated at age 2.0 years using the Bayley Scales of Infant Development, Second Edition and at age 5.5 years using the Movement Assessment Battery for Children-Second Edition (subscore categories: manual dexterity, aiming and catching, and static and dynamic balance)., Results: In 249 children, the prevalence of GJH, defined by the Beighton test score, was 34.1% for a score ≥ 4, 22.5% for a score ≥ 5, and 16.5% for a score ≥ 6. No significant association was found between GJH and total motor performance. Manual dexterity in girls (Beighton score ≥ 4) was positively associated with higher level of motor performance (β [SE] = 0.38 [0.17]; P = .028), ranging from +0.04 SD to +0.72 SD, even after correction for covariates. A significant interaction between GJH and body mass index (BMI) growth was found, indicating that the effect of GJH on the rate of development of motor performance declines with increasing BMI growth (β = 0.05 [0.02]; P = .031)., Conclusion: In this healthy pediatric cohort, GJH was present in one-third of the sample, and no significant association was found between GJH and total motor performance. The effect of GJH on the rate of development of motor performance appears to decline with increasing BMI growth. Longitudinal prospective studies are recommended to detect influences of GJH on motor performance over time, as well as the influence of body composition and Beighton cutoff points., (Copyright © 2015 Elsevier Inc. All rights reserved.)

Background: Generalized Joint Hypermobility (GJH) has been found to be associated with musculoskeletal complaints and disability. For others GJH is seen as a prerequisite in order to excel in certain sports like dance. However, it remains unclear what the role is of GJH in human performance. Therefore, the purpose of the study was to establish the association between GJH and functional status and to explore the contribution of physical fitness and musculoskeletal complaints to this association., Methods: A total of 72 female participants (mean age (SD; range): 19.6 (2.2; 17-24)) were recruited among students from the Amsterdam School of Health Professions (ASHP) (n = 36) and the Amsterdam School of Arts (ASA), Academy for dance and theater (n = 36) in Amsterdam, The Netherlands. From each participant the following data was collected: Functional status performance (self-reported Physical activity level) and capacity (walking distance and jumping capacity: side hop (SH) and square hop (SQH)), presence of GJH (Beighton score ≥4), muscle strength, musculoskeletal complaints (pain and fatigue) and demographic characteristics (age and BMI)., Results: GJH was negatively associated with all capacity measures of functional status. Subjects with GJH had a reduced walking distance (B(SE):-75.5(10.5), p = <.0001) and jumping capacity (SH: B(SE):-10.10(5.0), p = .048, and SQH: B(SE):-11.2(5.1), p = .024) in comparison to subjects without GJH, when controlling for confounding: age, BMI and musculoskeletal complaints. In participants with GJH, functional status was not associated with performance measures., Conclusion: GJH was independently associated with lower walking and jumping capacity, potentially due to the compromised structural integrity of connective tissue. However, pain, fatigue and muscle strength were also important contributors to functional status.

Objectives: In health evaluations, physical activity (PA) and cardiorespiratory fitness (maximal oxygen uptake [VO2max]) are important variables. It is not always possible to assess both of them. If the association between self-reported PA and VO2max was strong, it would be possible to use the information on PA to make assumptions about VO2max and vice versa. However, little is known about this relation, in particular among women at high risk for cardiovascular disease. Our aim was to study the association between self-reported PA (Short QUestionnaire to ASses Health enhancing PA) and fitness (determined using the Siconolfi step test) among sedentary women in a multiethnic population., Study Design and Setting: Participants were sampled from an exercise program for sedentary women (The Netherlands, 2008-09). Linear regression was performed with VO2max (dependent variable) and self-reported PA (independent variable); covariates were age and body mass index., Results: One hundred ninety-seven women from different ethnic backgrounds were included. No significant association was found between VO2max and PA (R(2) = 0.60)., Conclusion: A poor association was found between self-reported PA and estimated VO2max. Hence, PA and VO2max represent two different aspects of health in sedentary women and cannot be used interchangeably. This should be taken into account when evaluating health promotion interventions or when making health risks statements in sedentary women in a multiethnic population., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Background: Cervical dystonia is characterized by involuntary muscle contractions of the neck and abnormal head positions that affect daily life activities and social life of patients. Patients are usually treated with botulinum toxin injections into affected neck muscles to relief pain and improve control of head postures. In addition, many patients are referred for physical therapy to improve their ability to perform activities of daily living. A recent review on allied health interventions in cervical dystonia showed a lack of randomized controlled intervention studies regarding the effectiveness of physical therapy interventions., Methods/design: The (cost-) effectiveness of a standardized physical therapy program compared to regular physical therapy, both as add-on treatment to botulinum toxin injections will be determined in a multi-centre, single blinded randomized controlled trial with 100 cervical dystonia patients. Primary outcomes are disability in daily functioning assessed with the disability subscale of the Toronto Western Spasmodic Torticollis Rating Scale. Secondary outcomes are pain, severity of dystonia, active range of motion of the head, quality of life, anxiety and depression. Data will be collected at baseline, after six months and one year by an independent blind assessor just prior to botulinum toxin injections. For the cost effectiveness, an additional economic evaluation will be performed with the costs per quality adjusted life-year as primary outcome parameter., Discussion: Our study will provide new evidence regarding the (cost-) effectiveness of a standardized, tailored physical therapy program for patients with cervical dystonia. It is widely felt that allied health interventions, including physical therapy, may offer a valuable supplement to the current therapeutic options. A positive outcome will lead to a greater use of the standardized physical therapy program. For the Dutch situation a positive outcome implies that the standardized physical therapy program forms the basis for a national treatment guideline for cervical dystonia., Trial Registration: Number Dutch Trial registration (Nederlands Trial Register): NTR3437.

Objective: To study the impact of generalized joint hypermobility (GJH) in professional dancers on physical fitness, musculoskeletal complaints and psychological distress., Methods: Thirty-six professional dancers were recruited and compared with control subjects (mean age 20.1, range 17-27). Height, weight, Beighton score, physical fitness (walking distance, muscle strength, estimated VO2max), musculoskeletal complaints (pain, fatigue) and psychological distress (anxiety, depression) were measured., Results: Univariate analysis revealed, in between-group analysis, that dancers (with and without GJH) had higher physical fitness [the six-minute walk test (6MWT): ΔD = +8.4%, P = 0.001; VO2max: ΔD = +12.8%, P = 0.01], fatigue (checklist individual strength: ΔD = +80.3%, P < 0.0001) and greater psychological distress (Hospital Anxiety and Depression Scale: ΔD = +115.0%, P < 0.0001). When comparing dancers and control subjects with GJH to those without GJH, lower levels of physical fitness (muscle strength: ΔD = -11.3%, P < 0.0001; 6MWT: ΔD = -9.9%, P < 0.0001), more fatigue (checklist individual strength: ΔD = +84.4%, P < 0.0001) and greater psychological distress (Hospital Anxiety and Depression Scale: ΔD = +79.6%, P < 0.0001) were observed in subjects with GJH. Multivariate analysis showed that dancers have higher levels of physical fitness (6MWT, P = 0.001; VO2max, P = 0.020); however, when taking GJH into account, this advantage disappeared, indicating lower levels of physical fitness in comparison with control subjects (6MWT, P = 0.001; muscle strength, P < 0.0001; VO2max, P = 0.040). Dancers experienced more fatigue (P = 0.001) and psychological distress (P < 0.0001). This was associated with even more fatigue (P = 0.010) and psychological distress (P = 0.040) when GJH was present., Conclusion: Dancers with GJH seem more vulnerable to musculoskeletal and psychological complaints. In addition, GJH was also associated with lower physical fitness, despite training. Caregivers for professional dancers should monitor closely the physical capabilities and the amount of psychological strain.

Objective: Evaluate clinical outcome of early cyclic intravenous pamidronate treatment in children with moderate-to-severe osteogenesis imperfecta (OI), commenced before three years of age., Methods: A retrospective review of 17 patients with moderate-to-severe OI. Development, anthropometry, fracture history, bone mineral density (BMD) and biochemistry were collected at baseline, 12 and 24 months., Results: Four had OI type I, eleven had type III, one OI-FKBP10 type and one OI type V. Mean age at start of pamidronate was 14 ± 11 months. Pamidronate ranged from 6 to 12 mg/kg/year. No adverse reaction apart from fever and vomiting was noted. Long bone fracture decreased from a mean of 10.4/year to 1.2/year after 12 months and 1.4/year after 24 months (p = 0.02). Lumbar spine age- and height-matched BMD Z-scores increased (p < 0.005). Sixteen with vertebral compression fractures at baseline all showed improved vertebral shape (p < 0.001). Concavity index, likewise, improved (p < 0.005). Motor milestones compared to historical data show earlier attainment in rolling over, crawling, pulling to stand and walking independently but not sitting., Conclusion: Cyclic intravenous pamidronate, started under 3 years of age in children with moderate-to-severe OI, was well tolerated and associated with an increase in lumbar spine BMD, reduced fracture frequency, vertebral remodelling and attainment of motor milestones at an earlier age., (Copyright © 2013 S. Karger AG, Basel.)

Introduction: To provide a state of the art on diagnostics, clinical characteristics, and treatment of paediatric generalised joint hypermobility (GJH) and joint hypermobility syndrome (JHS)., Method: A narrative review was performed regarding diagnostics and clinical characteristics. Effectiveness of treatment was evaluated by systematic review. Searches of Medline and Central were performed and included nonsymptomatic and symptomatic forms of GJH (JHS, collagen diseases)., Results: In the last decade, scientific research has accumulated on all domains of the ICF. GJH/JHS can be considered as a clinical entity, which can have serious effects during all stages of life. However research regarding the pathological mechanism has resulted in new potential opportunities for treatment. When regarding the effectiveness of current treatments, the search identified 1318 studies, from which three were included (JHS: n = 2, Osteogenesis Imperfecta: n = 1). According to the best evidence synthesis, there was strong evidence that enhancing physical fitness is an effective treatment for children with JHS. However this was based on only two studies., Conclusion: Based on the sparsely available knowledge on intervention studies, future longitudinal studies should focus on the effect of physical activity, fitness, and joint stabilisation. In JHS and chronic pain, the effectiveness of a multidisciplinary approach should be investigated.

Objective: To assess whether there is an increased prevalence of joint hypermobility in patients with vesico-ureteric reflux (VUR)., Materials and Methods: We studied 50 patients with primary VUR and matched controls drawn from a reference population. Joint mobility was assessed using the Bulbena hypermobility score., Results: We identified significantly more patients with VUR with generalized joint hypermobility than controls (24% vs 6.7%, P= 0.007)., Conclusion: Our findings confirm our clinical observation of an increased rate of joint hypermobility in patients with VUR. We speculate that an altered composition of the connective tissue may contribute to the severity of the (pre-existing) VUR phenotype., (© 2011 THE AUTHORS. BJU INTERNATIONAL © 2011 BJU INTERNATIONAL.)

Background: Patients undergoing total knee arthroplasty (TKA) often experience strength deficits both pre- and post-operatively. As these deficits may have a direct impact on functional recovery, strength assessment should be performed in this patient population. For these assessments, reliable measurements should be used. This study aimed to determine the inter- and intrarater reliability of hand-held dynamometry (HHD) in measuring isometric knee strength in patients awaiting TKA., Methods: To determine interrater reliability, 32 patients (81.3% female) were assessed by two examiners. Patients were assessed consecutively by both examiners on the same individual test dates. To determine intrarater reliability, a subgroup (n = 13) was again assessed by the examiners within four weeks of the initial testing procedure. Maximal isometric knee flexor and extensor strength were tested using a modified Citec hand-held dynamometer. Both the affected and unaffected knee were tested. Reliability was assessed using the Intraclass Correlation Coefficient (ICC). In addition, the Standard Error of Measurement (SEM) and the Smallest Detectable Difference (SDD) were used to determine reliability., Results: In both the affected and unaffected knee, the inter- and intrarater reliability were good for knee flexors (ICC range 0.76-0.94) and excellent for knee extensors (ICC range 0.92-0.97). However, measurement error was high, displaying SDD ranges between 21.7% and 36.2% for interrater reliability and between 19.0% and 57.5% for intrarater reliability. Overall, measurement error was higher for the knee flexors than for the knee extensors., Conclusions: Modified HHD appears to be a reliable strength measure, producing good to excellent ICC values for both inter- and intrarater reliability in a group of TKA patients. High SEM and SDD values, however, indicate high measurement error for individual measures. This study demonstrates that a modified HHD is appropriate to evaluate knee strength changes in TKA patient groups. However, it also demonstrates that modified HHD is not suitable to measure individual strength changes. The use of modified HHD is, therefore, not advised for use in a clinical setting.

The primary purpose of this study was to examine whether grip strength is related to total muscle strength in children, adolescents, and young adults. The second purpose was to provide reference charts for grip strength, which could be used in the clinical and research setting. This cross-sectional study was performed at primary and secondary schools and the University of Applied Sciences. Three hundred and eighty-four healthy Dutch children, adolescents, and young adults at the age of 8 to 20 years participated. Isometric muscle strength was measured with a handheld dynamometer of four muscle groups (shoulder abductors, grip strength, hip flexors, and ankle dorsiflexors). Total muscle strength was a summing up of shoulder abductors, hip flexors, and ankle dorsiflexors. All physical therapists participated in a reliability study. The study was started when intratester and intertester reliability was high (Pearson correlation coefficient >0.8). Grip strength was strongly correlated with total muscle strength, with correlation coefficients between 0.736 and 0.890 (p < 0.01). However, the correlation was weaker when controlled for weight (0.485-0.564, p < 0.01). Grip strength is related to total muscle strength. This indicates, in the clinical setting, that grip strength can be used as a tool to have a rapid indication of someone's general muscle strength. The developed reference charts are suitable for evaluating muscle strength in children, adolescents, and young adults in clinical and research settings.

Although children with haemophilia are advised to participate in physical activities, their physical fitness has not been studied in a large group. In addition, children with haemophilia may be at increased risk for becoming overweight as a result of inactivity because of joint bleedings or because of overprotection. This study aimed to assess physical fitness (aerobic capacity), joint status, muscle strength, quality of life (QoL), self-reported motor competence and also prevalence of overweight and its association with physical parameters. Weight and height were measured. Skin folds were measured unilaterally at biceps, triceps, subscapular and supra-iliac sites. Aerobic capacity was determined on a cycle ergometer or with a 6-min walk test (6MWT). Muscle strength and active range of motion of elbows, knees and ankle joints were measured. Self-reported motor competence was measured with the 'Competentie BelevingsSchaal voor Kinderen'. Joint pain was scored on a Visual Analogue Scale. The Haemo-QoL Index was used to measure QoL. In 158 Dutch boys with haemophilia, with a mean age of 12.7 years (SD 2.9), normal aerobic capacity and muscle strength were found. Joint pain was reported by 16% of the participants. The prevalence of overweight (16%) was slightly increased when compared with healthy Dutch boys (13.5%). Being overweight had a negative association with the6MWT and QoL. Dutch children with haemophilia have normal aerobic exercise capacity and muscle strength. The majority also has normal joint mobility. Prevalence of overweight is slightly increased.

Background: Exercise therapy is considered an important component of the treatment of arthritis. The efficacy of exercise therapy has been reviewed in adults with rheumatoid arthritis but not in children with juvenile idiopathic arthritis (JIA)., Objectives: To assess the effects of exercise therapy on functional ability, quality of life and aerobic capacity in children with JIA., Methods: Several electronic databases were searched up to October 2007 and references were tracked. The selection criteria were randomized controlled trials (RCTs) of exercise treatment in JIA. As for data collection and analysis, potentially relevant references were evaluated and all data were extracted by two review authors working independently., Results: Three out of 16 identified studies met the inclusion criteria, with a total of 212 participants. All the included studies fulfilled at least seven of 10 methodological criteria. The outcome data of the following measures were homogenous and were pooled in a meta-analysis: functional ability (N=198; weighted mean difference [WMD] -0.07, 95% CI -0.22 to 0.08), quality of life (CHQ-PhS: N=115; WMD -3.96, 95% CI -8.91 to 1.00) and aerobic capacity (N=124; WMD 0.04, 95% CI -0.11 to 0.19). The results suggest that the outcome measures all favoured the exercise therapy but none were statistically significant. None of the studies reported negative effects of the exercise therapy., Conclusions: Overall, based on ''silver-level'' evidence there was no clinically important or statistically significant evidence that exercise therapy can improve functional ability, quality of life, aerobic capacity or pain. The included and excluded studies were all consistent about the adverse effects of exercise therapy; no short-term detrimental effects of exercise therapy were found in any study. Both included and excluded studies showed that exercise does not exacerbate arthritis. Although the short-term effects look promising, the long-term effect of exercise therapy remains unclear.

Objective: To determine whether aerobic capacity is normal in boys with different types of hemophilia compared with healthy peers and whether the level of aerobic capacity correlates with the amount of physical activity, joint health status, muscle strength, and anthropometrics., Study Design: 47 patients (mean [SD] age, 12.9 [3.2] years; age range, 8.2-17.4 years) from the "Van Creveldkliniek" of the University Medical Center Utrecht, participated. Anthropometry, muscle strength, joint impairment, functional ability, and aerobic capacity were measured. The amount of energy expenditure during daily living was assessed., Results: All boys were able to perform at maximal or near-maximal level on exercise tests, and none of them reported bleeds or other adverse events. Relative peak oxygen, peak heart rate, and peak working capacity were significantly lower compared with healthy control subjects. 30% had Z-scores >2 for weight. Total muscle strength was normal, and almost no joint impairment and no decrease in functional ability were found., Conclusion: The aerobic capacity of children with hemophilia is still lower than the normal population, whereas their overall muscle strength is comparable with healthy peers. The functional ability does not differ from healthy peers, and joint health status showed very minor impairments. A substantial proportion of Dutch children with hemophilia was overweight, without showing a reduction in the amount of self-reported physical activities.

Objective: Osteopenia is a common complication of juvenile idiopathic arthritis (JIA). In adults, low bone density and increased fracture risk are associated with low vitamin K status of bone. The vitamin K-dependent protein osteocalcin plays an important role in bone metabolism. Its activity depends upon post-translational carboxylation in which vitamin K is an essential co-factor. Hence, vitamin K deficiency leads to under-carboxylated (i.e., inactive) osteocalcin (ucOC). Little is known about the vitamin K status and bone health in children with juvenile idiopathic arthritis (JIA). We studied the vitamin K status of bone and its association with bone mass properties in children with JIA compared to healthy children., Methods: We performed a cross sectional study in 55 children with JIA and 54 healthy controls between 6-18 years of age. Bone markers, ultrasound bone mass properties and vitamin K status of bone were determined., Results: Overall, no differences in vitamin K status of bone were found between the study groups. Among children with JIA, a high ratio of ucOC/cOC indicating low vitamin K status was associated with low bone ultrasound parameters, whereas children with a high vitamin K status had markedly higher bone properties. This association was independent of physical activity, age, gender and BMI., Conclusion: These results suggest that vitamin K may be one of multiple risk factors for low bone mass in children with JIA, in addition to other recognized determinants of bone mass. The question remains whether JIA patients would benefit from increased dietary vitamin K intake.

Background: Exercise therapy is considered an important component of the treatment of arthritis. The efficacy of exercise therapy has been reviewed in adults with rheumatoid arthritis but not in children with juvenile idiopathic arthritis (JIA)., Objectives: To assess the effects of exercise therapy on functional ability, quality of life and aerobic capacity in children with JIA., Search Strategy: The Cochrane Central Register of Controlled Trials (CENTRAL), Cochrane Database of Systematic Reviews (The Cochrane Library), MEDLINE (January 1966 to April 2007), CINAHL (January 1982 to April 2007), EMBASE (January 1966 to October 2007), PEDro (January 1966 to October 2007), SportDiscus (January 1966 to October 2007), Google Scholar (to October 2007), AMED (Allied and Alternative Medicine) (January 1985 to October 2007), Health Technologies Assessment database (January 1988 to October 2007), ISI Web Science Index to Scientific and Technical Proceedings (January 1966 to October 2007) and the Chartered Society of Physiotherapy website (http://www.cps.uk.org) were searched and references tracked., Selection Criteria: Randomised controlled trials (RCTs) of exercise treatment in JIA., Data Collection and Analysis: Potentially relevant references were evaluated and all data were extracted by two review authors working independently., Main Results: Three out of 16 identified studies met the inclusion criteria, with a total of 212 participants. All the included studies fulfilled at least seven of 10 methodological criteria. The outcome data of the following measures were homogenous and were pooled in a meta-analysis: functional ability (n = 198; WMD -0.07, 95% CI -0.22 to 0.08), quality of life (CHQ-PhS: n = 115; WMD -3.96, 95% CI -8.91 to 1.00) and aerobic capacity (n = 124; WMD 0.04, 95% CI -0.11 to 0.19). The results suggest that the outcome measures all favoured the exercise therapy but none were statistically significant. None of the studies reported negative effects of the exercise therapy., Authors' Conclusions: Overall, based on 'silver-level' evidence (www.cochranemsk.org) there was no clinically important or statistically significant evidence that exercise therapy can improve functional ability, quality of life, aerobic capacity or pain. The low number of available RCTs limits the generalisability. The included and excluded studies were all consistent about the adverse effects of exercise therapy; no short-term detrimental effects of exercise therapy were found in any study. Both included and excluded studies showed that exercise does not exacerbate arthritis. The large heterogeneity in outcome measures, as seen in this review, emphasises the need for a standardised assessment or a core set of functional and physical outcome measurements suited for health research to generate evidence about the possible benefits of exercise therapy for patients with JIA. Although the short-term effects look promising, the long-term effect of exercise therapy remains unclear.

Objective: To describe the level of motor performance and functional skills in young children with JIA., Methods: In a cross-sectional study in 56 preschool-aged (PSA) and early school- aged children (ESA) with JIA according to ILAR classification, motor performance was measured with the Bayley Scales of Infant Development II (BSID2) and the Movement Assessment Battery for Children (M-ABC). Functional skills were measured with the Pediatric Evaluation of Disability Inventory (PEDI). Disease outcome was measured with a joint count on swelling/range of joint motion, functional ability and joint pain., Results: Twenty two PSA children (mean age 2.1 years) with a mean Developmental Index of the BSID2 of 77.9 indicating a delayed motor performance; 45% of PSA children showed a severe delayed motor performance. Mean PEDI scores were normal, 38% of PSA scored below -2 SD in one or more domains of the PEDI. Thirty four ESA children (mean age 5.2 years) with a mean M-ABC 42.7, indicating a normal motor performance, 12% of ESA children had an abnormal score. Mean PEDI scores showed impaired mobility skills, 70% of ESA children scored below -2 SD in one or more domains of the PEDI. Disease outcome in both age groups demonstrated low to moderate scores. Significant correlations were found between age at disease onset, disease duration and BSID2 or M-ABC and between disease outcome and PEDI in both age cohorts., Conclusion: More PSA children have more impaired motor performance than impaired functional skills, while ESA children have more impairment in functional skills. Disease onset and disease duration are correlated with motor performance in both groups. Impaired motor performance and delayed functional skills is primarily found in children with a polyarticular disease course. Clinical follow up and rehabilitation programs should also focus on motor performance and functional skills development in young children with JIA.