Effectiveness of physiotherapy with telerehabilitation in surgical patients: a systematic review and meta-analysis.

BACKGROUND: Over the last few years, telerehabilitation services have developed rapidly, and patients value benefits such as reduced travelling barriers, flexible exercise hours, and the possibility to better integrate skills into daily life. However, the effects of physiotherapy with telerehabilitation on postoperative functional outcomes compared with usual care in surgical populations are still inconclusive. OBJECTIVES: To study the effectiveness of physiotherapy with telerehabilitation on postoperative functional outcomes and quality of life in surgical patients. DATA SOURCES: Relevant studies were obtained from MEDLINE, EMBASE, CINAHL, the Cochrane Library, PEDro, Google Scholar and the World Health Organization International Clinical Trials Registry Platform. STUDY SELECTION: Randomised controlled trials, controlled clinical trials, quasi-randomised studies and quasi-experimental studies with comparative controls were included with no restrictions in terms of language or date of publication. DATA EXTRACTION AND SYNTHESIS: Methodological quality was assessed using the Cochrane risk of bias tool. Twenty-three records were included for qualitative synthesis. Seven studies were eligible for quantitative synthesis on quality of life, and the overall pooled standardised mean difference was 1.01 (95% confidence interval 0.18 to 1.84), indicating an increase in favour of telerehabilitation in surgical patients. LIMITATIONS: The variety in contents of intervention and outcome measures restricted the performance of a meta-analysis on all clinical outcome measures. CONCLUSIONS: Physiotherapy with telerehabilitation has the potential to increase quality of life, is feasible, and is at least equally effective as usual care in surgical populations. This may be sufficient reason to choose physiotherapy with telerehabilitation for surgical populations, although the overall effectiveness on physical outcomes remains unclear. PROSPERO registration number: CRD42015017744.

The cardiac care bridge program: design of a randomized trial of nurse-coordinated transitional care in older hospitalized cardiac patients at high risk of readmission and mortality.

BACKGROUND: After hospitalization for cardiac disease, older patients are at high risk of readmission and death. Although geriatric conditions increase this risk, treatment of older cardiac patients is limited to the management of cardiac diseases. The aim of this study is to investigate if unplanned hospital readmission and mortality can be reduced by the Cardiac Care Bridge transitional care program (CCB program) that integrates case management, disease management and home-based cardiac rehabilitation. METHODS: In a randomized trial on patient level, 500 eligible patients ≥ 70 years and at high risk of readmission and mortality will be enrolled in six hospitals in the Netherlands. Included patients will receive a Comprehensive Geriatric Assessment (CGA) at admission. Randomization with stratified blocks will be used with pre-stratification by study site and cognitive status based on the Mini-Mental State Examination (15-23 vs ≥ 24). Patients enrolled in the intervention group will receive a CGA-based integrated care plan, a face-to-face handover with the community care registered nurse (CCRN) before discharge and four home visits post-discharge. The CCRNs collaborate with physical therapists, who will perform home-based cardiac rehabilitation and with a pharmacist who advices the CCRNs in medication management The control group will receive care as usual. The primary outcome is the incidence of first all-cause unplanned readmission or mortality within 6 months post-randomization. Secondary outcomes at three, six and 12 months after randomization are physical functioning, functional capacity, depression, anxiety, medication adherence, health-related quality of life, healthcare utilization and care giver burden. DISCUSSION: This study will provide new knowledge on the effectiveness of the integration of geriatric and cardiac care. TRIAL REGISTRATION: NTR6316 . Date of registration: April 6, 2017.

Hip Fractures in Older Patients: Trajectories of Disability after Surgery.

BACKGROUND: Hip fracture in older patients often lead to permanent disabilities and can result in mortality. OBJECTIVE: To identify distinct disability trajectories from admission to one-year post-discharge in acutely hospitalized older patients after hip fracture. DESIGN: Prospective cohort study, with assessments at admission, three-months and one-year post-discharge. SETTING AND PARTICIPANTS: Patients ≥ 65 years admitted to a 1024-bed tertiary teaching hospital in the Netherlands. METHODS: Disability was the primary outcome and measured with the modified Katz ADL-index score. A secondary outcome was mortality. Latent class growth analysis was performed to detect distinct disability trajectories from admission and Cox regression was used to analyze the effect of the deceased patients to one-year after discharge. RESULTS: The mean (SD) age of the 267 patients was 84.0 (6.9) years. We identified 3 disability trajectories based on the Katz ADL-index score from admission to one-year post-discharge: 'mild'- (n=54 (20.2%)), 'moderate'- (n=110 (41.2%)) and 'severe' disability (n=103 (38.6%)). Patients in all three trajectories showed an increase of disabilities at three months, in relation to baseline and 80% did not return to baseline one-year post-discharge. Seventy-three patients (27.3%) deceased within one-year post-discharge, particularly in the 'moderate'- (n=22 (8.2%)) and 'severe' disability trajectory (n=47 (17.6%)). CONCLUSIONS: Three disability trajectories were identified from hospital admission until one-year follow-up in acutely hospitalized older patients after hip fracture. Most patients had substantial functional decline and 27% of the patient's deceased one-year post-discharge, mainly patients in the 'moderate'- 'and severe' disability trajectories.

Preoperative functional status is not associated with postoperative surgical complications in low risk patients undergoing esophagectomy.

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.

Generalized Hyperalgesia in Children and Adults Diagnosed With Hypermobility Syndrome and Ehlers-Danlos Syndrome Hypermobility Type: A Discriminative Analysis.

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/cm2 for males and 29.0 N/cm2 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.

Surviving critical illness: what is next? An expert consensus statement on physical rehabilitation after hospital discharge.

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.

Determinants of disability in cervical dystonia.

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 (R2 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.

Validation of the SQUASH Physical Activity Questionnaire in a Multi-Ethnic Population: The HELIUS Study.

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.

Children with generalised joint hypermobility and musculoskeletal complaints: state of the art on diagnostics, clinical characteristics, and treatment.

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.

Intravenous pamidronate treatment in children with moderate-to-severe osteogenesis imperfecta started under three years of age.

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.

Physical fitness in children with haemophilia and the effect of overweight.

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.

Exercise therapy in juvenile idiopathic arthritis: a Cochrane Review.

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.

Extremes in vitamin K status of bone are related to bone ultrasound properties in children with juvenile idiopathic arthritis.

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.

Exercise therapy in juvenile idiopathic arthritis.

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.

Aerobic and anaerobic exercise capacity in children with juvenile idiopathic arthritis.

OBJECTIVE: To compare the aerobic and anaerobic exercise capacity of children with juvenile idiopathic arthritis (JIA) with healthy controls, to determine if there were differences based on disease onset type, and to examine the relationship between aerobic and anaerobic exercise capacity in children with JIA. METHODS: Sixty-two patients with JIA (mean +/- SD age 11.9 +/- 2.2 years, range 6.7-15.9) participated in this study. Aerobic exercise capacity was measured using a cardiopulmonary exercise test. Anaerobic exercise capacity was measured using the Wingate Anaerobic Exercise Test (WAnT). RESULTS: All patients were able to perform the cardiopulmonary exercise test and WAnT without adverse events. On average, the maximal oxygen uptake (VO(2peak)) and VO(2peak/kg) were 69.8% and 74.8%, respectively, of that predicted compared with healthy controls. Mean +/- SD power was 66.7% +/- 37.2% of that predicted compared with healthy children. Mean +/- SD peak power was 65.5% +/- 43.1% of that predicted compared with healthy children. There were significant differences between subgroups of JIA; the oligoarticular-onset group values did not significantly differ from healthy control values; the polyarticular rheumatoid factor positive-onset subgroup had the greatest impairment in both aerobic and anaerobic exercise capacity. The correlations of mean power and peak power with VO(2peak) were r = 0.884 and r = 0.697, respectively (P < 0.05). CONCLUSION: This study demonstrates that both the aerobic and anaerobic exercise capacity in children with JIA are significantly decreased. The WAnT might be a valuable adjunct to other assessment tools in the followup of patients with JIA.

Physical fitness, functional ability and quality of life in children with severe haemophilia: a pilot study.

In the Netherlands comparable levels of sports-participation between persons with haemophilia and healthy controls have been reported. This raises the question if children with haemophilia under the currently available prophylaxis do reach comparable levels of physical fitness and health-related quality of life (HRQoL) as their healthy peers. The aim of this study was to investigate the level of physical fitness, functional ability and quality of life and to determine the feasibility to safely test the exercise capacity of boys with severe haemophilia A. Thirteen subjects participated in this study. Physical fitness was determined using the measurement of maximal oxygen uptake (VO2peak) attained during a graded maximal exercise test to volitional exhaustion. Joint health, physical activity levels and health-related quality of life (Haemo-Qol) were also measured. Mean VO2peak was 1.86+/-0.77 L min-1 (Z-score: -0.39+/-1.61) which was not significantly different from reference values. Relative VO2peak was 47.42+/-8.29 mL min-1 kg-1 (Z-score: -0.52+/-1.43), which did not differ significantly from reference values either. One boy suffered a joint bleeding one day after the test. Haemo-Qol scores in parents and children ranged from 3.2% to 36.7% (100% reflects poor outcome). Relationship between the child or parent reports of Haemo-QoL and both absolute and relative VO2peak ranged from R=0.00 and R=0.4. Exercise testing in children with severe haemophilia A was a safe procedure. Patients with severe haemophilia A with good joint health and no limitations of activities have comparable physical fitness and physical active lifestyle with healthy peers and good HRQoL.

How fatigue is related to other somatic symptoms.

AIMS: To assess the relation between fatigue and somatic symptoms in healthy adolescents and adolescents with chronic fatigue syndrome/myalgic encephalopathy (CFS/ME). METHODS: Seventy two adolescents with CFS were compared within a cross-sectional study design with 167 healthy controls. Fatigue and somatic complaints were measured using self-report questionnaires, respectively the subscale subjective fatigue of the Checklist Individual Strength (CIS-20) and the Children's Somatization Inventory. RESULTS: Healthy adolescents reported the same somatic symptoms as adolescents with CFS/ME, but with a lower score of severity. The top 10 somatic complaints were the same: low energy, headache, heaviness in arms/legs, dizziness, sore muscles, hot/cold spells, weakness in body parts, pain in joints, nausea/upset stomach, back pain. There was a clear positive relation between log somatic symptoms and fatigue (linear regression coefficient: 0.041 points log somatic complaints per score point fatigue, 95% CI 0.033 to 0.049) which did not depend on disease status. CONCLUSIONS: Results suggest a continuum with a gradual transition from fatigue with associated symptoms in healthy adolescents to the symptom complex of CFS/ME.

Recent developments in clinimetric instruments.

Assessment of impairment and function is essential in order to monitor joint status and evaluate therapeutic interventions in patients with haemophilia. The improvements in the treatment of haemophilia have required the development of more sensitive tools to detect the more minor dysfunctions that may now be apparent. This paper outlines some of the recent developments in this field. The Haemophilia Joint Health Score (HJHS) provides a systematic and robust measure of joint impairment. The MRI Scoring System has been designed to provide a comprehensive scoring system combining both progressive and additive scales. The Functional Independence Score for Haemophilia (FISH) has been developed to assess performance of functional activities and can be used in conjunction with the Haemophilia Activities List (HAL) which provides a self report measure of function. It is recommended that both measures are evaluated as these tools measure different constructs. Further refinement and testing of the psychometric properties of all of these tools is in progress. More widespread use of these tools will enable the sharing of data across the world so promoting best practice and ultimately enhancing patient care.

Torticollis and plagiocephaly in infancy: therapeutic strategies.

BACKGROUND: Asymmetry in infancy is a diagnosis with a large spectrum of features, expressing an abnormal shape of parts of the body or unequal postures and movements, which might be structural and/or functional, with localized or generalized expression. PURPOSE: The purpose of the present study is to highlight different therapeutic aspects of the most occurring asymmetries in infancy: congenital muscular torticollis, positional torticollis and plagiocephaly, based on best evidence in current literature. RESULTS: A flow chart is presented showing different pathways in therapeutic strategies, such as physical therapy, orthotic devices (helmet treatment and Dynamic Orthotic Cranioplasty) and surgery. CONCLUSION: It is concluded that there are different views towards management on torticollis and plagiocephaly. A systematic therapeutic management to evaluate these asymmetries is indicated. The presented therapeutic flow chart might serve as a basis in order to achieve uniformity in therapeutic thinking and performance.

The relationship between generalized joint hypermobility and motor development.

PURPOSE: The aim of this study was to investigate the relationship between the presence and localization of joint hypermobility and delay in motor development. METHODS: A retrospective chart review was performed. All children younger than 2.5 years of age and between four and 12 years of age with generalized joint hypermobility were included. Generalized joint hypermobility was assessed using the criteria of Bulbena. In children between one and 2.5 years of age, motor development was measured using the Bayley Scales of Infant Development. In children four to 12 years of age, the Movement Assessment Battery for Children was used. RESULTS: Data from 72 children were available for analysis. In nine of 16 children younger than 2.5 years of age, a delay in motor development was found without a significant association between the delay in motor development (yes/no) and the Bulbena score (odds ratio: 0.4; 95% confidence interval: 0.07-2.1). In children between four and 12 years of age, a severe delay in motor development was present in 14 of 56 children (25%), while 12 of 56 children (21%) were at risk and 30 of 56 (54%) were age appropriate. No significant association between delay in motor development and the Bulbena score was found (odds ratio: 1.3; 95% confidence interval: 0.8-2.1). The age of independent walking was not significantly associated with the number of hypermobile joints (Bulbena score) (linear regression coefficient: 0.3; 95% confidence interval: -1.5 - 2.1). CONCLUSIONS: Although severe delays in motor development may be observed in approximately one third of children with generalized joint hypermobility, there is no association between the amount of generalized joint hypermobility and delay in motor development.