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Background: Over the last decades the number of childhood cancer survivors has increased. However, a significant number of childhood cancer survivors suffer from side effects of treatment. This results in a lower quality of life, reduced physical activity (PA), decreased muscle strength and cardiorespiratory fitness compared to healthy peers. To improve PA and to be able to set up and evaluate training programmes, it is necessary to study the feasibility of accelerometery and current objectively measured PA level of children with childhood cancer. Methods: The PA level of twenty-five children undergoing cancer treatment was measured using accelerometery in a cross-sectional study design. Differences in PA levels were analysed by age, cancer type, sex, home or hospital setting and week or weekend days. Results: All participants reported the Actical was well-accepted and the burden and risks associated with participation were negligible.The median of minutes was 1325 min/24hrs of Sedentary Behaviour (SB), 111 min/24hrs of light PA and 4-min/24hrs of moderate-to-vigorous PA (MVPA). In a hospital setting significantly lower levels of PA were achieved, in comparison to a home setting. Conclusion: Accelerometery is feasible to objectively measure PA levels throughout the treatment, both in hospital or home settings. The level of PA per day of all participants was mostly SB, some light PA and hardly any MVPA. It is strongly recommended to determine benefits and limitations of PA in children during cancer treatment and to determine differences in perceptions of capability of PA of these children. With the growing population of childhood cancer survivors, substantiated PA guidelines and advices should be developed. PA for these children should be encouraged and stimulated to minimize the side effects during and after treatment. Keywords: Paediatric, Childhood cancer, Oncology, Physical activity and accelerometer

Background Although fatigue is a prevalent distressing symptom in children and adolescents with Pediatric Rheumatic Conditions (PRCs), intervention studies designed for reducing fatigue in PRCs are limited. Aim To systematically review evidence regarding the efficacy of interventions intended to reduce fatigue in patients with PRCs. Methods Comprehensive electronic searches were performed in PubMed/ MEDLINE, Embase, Web of Science and Cinahl. The risk of bias was assessed using the ‘Revised Cochrane risk-of-bias tool for randomized trials’ and ‘Quality Assessment Tool for Before-After Studies With No Control Group’ for respectively studies with and without a control group. Results Ten out of 418 studies were included with a total of 240 participants (age range 5–23 years). Interventions included land-based and aquatic-based exercise therapy, prednisolone, vitamin-D and creatine supplementation, psychological therapy and a transition program into an adult rheumatology program. Fatigue was assessed with self-reported questionnaires in all included studies. Land-based exercise therapy was effective in one pre-post intervention study, whereas not effective in two randomized controlled trials. Aquatic-based exercise therapy was found more effective than land-based exercise therapy. Two placebo-controlled studies showed a significant positive effect in reducing subjective fatigue with prednisolone and vitamin-D. Creatine was not found effective. Cognitive therapy was effective in one pre-post intervention study, while one RCT did not show an effect in reducing fatigue. A transition program based on health education showed a small reducing effect, however, it was not clear if this was a significant effect. Six studies showed a high risk of bias, three studies a moderate risk, and one study had a low risk of bias. Conclusions Insufficient evidence is provided to substantiate the efficacy of current interventions to reduce fatigue in PRCs. The low number of studies, non-comparable interventions, risk of bias, and inconclusive outcomes of the included studies denote future research should focus on intervention studies aimed at the treatment of fatigue in children and adolescents with PRCs. Identification of possible underlying biological and psychosocial mechanisms as possible treatment targets to reduce complaints of fatigue in children and adolescents with PRCs is warranted.

Introduction: Monitoring clinical outcome in persons with haemophilia (PWH) is essential in order to provide optimal treatment for individual patients and compare effectiveness of treatment strategies. Experience with measurement of activities and participation in haemophilia is limited and consensus on preferred tools is lacking. Aim: The aim of this study was to give a comprehensive overview of the measurement properties of a selection of commonly used tools developed to assess activities and participation in PWH. Methods: Electronic databases were searched for articles that reported on reliability, validity or responsiveness of predetermined measurement tools (5 self-reported and 4 performance based measurement tools). Methodological quality of the studies was assessed according to the COSMIN checklist. Best evidence synthesis was used to summarize evidence on the measurement properties. Results: The search resulted in 3453 unique hits. Forty-two articles were included. The self-reported Haemophilia Acitivity List (HAL), Pediatric HAL (PedHAL) and the performance based Functional Independence Score in Haemophilia (FISH) were studied most extensively. Methodological quality of the studies was limited. Measurement error, cross-cultural validity and responsiveness have been insufficiently evaluated. Conclusion: Albeit based on limited evidence, the measurement properties of the PedHAL, HAL and FISH are currently considered most satisfactory. Further research needs to focus on measurement error, responsiveness, interpretability and cross-cultural validity of the self-reported tools and validity of performance based tools which are able to assess limitations in sports and leisure activities.

Thrombotic microangiopathy (TMA) is a pattern of endothelial damage that can be found in association with diverse clinical conditions such as malignant hypertension. Although the pathophysiological mechanisms differ, accumulating evidence links complement dysregulation to various TMA syndromes and in particular the atypical hemolytic uremic syndrome. Here, we evaluated the role of complement in nine consecutive patients with biopsy-proven renal TMA attributed to severe hypertension. Profound hematologic symptoms of TMA were uncommon. In six Out of nine patients, we found mutations C3 in three, CFI in one, CD46 in one, and/or CFH in two patients either with or without the risk CFH-H3 haplotype in four patients. Elevated levels of the soluble C5b-9 and renal deposits of C3c and C5b-9 along the vasculature and/or glomerular capillary wall, confirmed complement activation in vivo. In contrast to patients without genetic defects, patients with complement defects invariably progressed to end-stage renal disease, and disease recurrence after kidney transplantation seems common. Thus, a subset of patients with hypertension-associated TMA falls within the spectrum of complement-mediated TMA, the prognosis of which is poor. Hence, testing for genetic complement abnormalities is warranted in patients with severe hypertension and TMA on renal biopsy to adopt suitable treatment options and prophylactic measures.

Introduction The implementation of early long-term, regular clotting factor concentrate (CFC) replacement therapy (‘prophylaxis’) has made it possible to offer boys with haemophilia a near normal life. Many different regimens have reported favourable results, but the optimum treatment regimens have not been established and the cost of prophylaxis is very high. Both for optimizing treatment and reimbursement issues, there is a need to provide objective evidence of both short- and long-term results and benefits of prophylactic regimens. Aims This report presents a critical review of outcome measures for use in the assessment of musculoskeletal health in persons with haemophilia according to the International Classification of Functioning, Disability and Health (ICF). This framework considers structural and functional changes, activities and participation in a context of both personal and environmental factors. Methods Results were generated by a combination of a critical review of available literature plus expert opinion derived from a two day consensus conference between 48 health care experts from different disciplines involved in haemophilia assessment and care. Outcome tools used in haemophilia were reviewed for reliability and validity in different patient groups and for resources required. Results and conclusion Recommendations for choice of outcome tools were made according to the ICF domains, economic setting, and reason for use (clinical or research). The next step will be to identify a ‘core’ set of outcome measures for use in clinical care or studies evaluating treatment.

Summary Children with haemophilia often experience limitations in activities of daily life. Recently the Paediatric Haemophilia Activities List (PedHAL) has been developed and tested in Dutch children with intensive replacement therapy. The psychometric properties of the PedHAL in children not receiving intensive replacement therapy are not known. The objective was to gain further insight into the psychometric properties of the PedHAL and to study the functional health status of Romanian children and adolescents with haemophilia. Children attending to the rehabilitation centre of Buzias in Romania were sampled consecutively. Construct validity of the PedHAL was evaluated by concurrent testing with objective and subjective measures of physical function and functional ability. Reproducibility was tested by a 3-day test–retest by intraclass correlation coefficient (ICC) and limits of agreement (LOA). Responsiveness to rehabilitation was assessed by Haemophilia Joint Health Score (HJHS) and PedHAL. Twenty-nine children with severe (n = 25) or moderate (n = 4) haemophilia participated. Mean age was 13.2 years (SD 4.0). Median score of the PedHAL was 83.5 (IQR 47.9–90.5). The PedHAL correlated moderately with HJHS (rho = −0.59), Functional Independence Score in& Haemophilia (rho = 0.65) and Child Health Questionnaire-physical function (rho = 0.40) and not with Child Health Questionnaire-mental health, Child Health Questionnaire-behaviour and 6MWT. Test-retest reliability was good (ICC = 0.95). LOA was 17.4 points for the sum score. HJHS scores improved slightly after rehabilitation, whereas PedHAL scores did not change. In general, construct validity and test–retest reliability were good, test–retest agreement showed some variability. Therefore, currently the PedHAL may be more appropriate for research purposes than for individual patient monitoring in clinical practice.

The reaction of bromine or iodine with cyanine dyes results in the formation of an equilibrium mixture of the dye and the halogen used, their 1: 1 addition product, the mesohalogen derivative of the dye, the 1: 1 addition product of the latter and the halogen used, the protonated dye and its dimeric or polymeric forms. The equilibrium constants of the different reactions are presented. The original dyes are easily regenerated from the halogenated products if other halogen acceptors are present in the system. The processes described can be of importance in the chromatic sensitization of photographic materials.

Increased work of breathing is considered to be a limiting factor in patients with cystic fibrosis (CF) performing aerobic exercise. We hypothesized that adolescents with CF and with static hyperinflation are more prone to a ventilatorily limited exercise capacity than non-static hyperinflated adolescents with CF. Exercise data of 119 adolescents with CF [range 12-18 years], stratified for static hyperinflation, defined as ratio of residual volume to total lung capacity (RV/TLC) 30%, were obtained during a progressive bicycle ergometer test with gas analysis and analyzed for ventilatory limitation. Static hyperinflation showed a significant, though weak association (Φ 0.38; P 0.001) with a ventilatorily limited exercise capacity (breathing reserve index at maximal effort0.70; FEV(1) 80% predicted and reduced exercise capacity, defined as VO(2peak) 85% predicted). Analysis of association for increasing degrees of hyperinflation showed an increase to Φ 0.49 (P 0.001) for RV/TLC 50%. In adolescents with static hyperinflation, peak work rate (W(peak) ; 3.1 ± 0.7 W/kg (75.1 ± 17.3% of predicted), peak oxygen uptake (VO(2peak) /kg (ml/min/kg); 39.2 ± 9.2 ml/min/kg (91.0 ± 20.3% of predicted), peak heart rate (HR(peak) ; 176 ± 19 beats/min) were significantly (P 0.05) decreased when compared with non-static hyperinflated adolescents (W(peak) 3.5 ± 0.5 W/kg (81.4 ± 10.0% of predicted)); VO(2peak) /kg (ml/min/kg); 43.1 ± 7.5 ml/min/kg (98.0 ± 15.1% of predicted); and HR(peak) 185 ± 14 beats/min). Additionally, no difference was found in the degree of association of FEV(1) (%) and RV/TLC (%) with VO(2peak) /kg(pred) and W(peak) /kg(Pred) , but we found the RV/TLC (%) to be a slightly stronger predictor of VO(2peak) /kg(pred) and W(peak) /kg(Pred) than FEV(1) (%). These results indicate that the presence of static hyperinflation in adolescents with CF by itself does not strongly influence ventilatory constraints during exercise and that static hyperinflation is only a slightly stronger predictor of W(peak) /kg(Pred) and VO(2peak) /kg(Pred) than airflow obstruction (FEV(1) (%)).

Summary. Worldwide, children with haemophilia suffer from limitations in performing activities of daily living. To measure such limitations in adults a disease-specific instrument, the Haemophilia Activities List (HAL), was created in 2004. The aim of this study was to adapt the HAL for children with haemophilia and to assess its psychometric properties. The structure and the main content were derived from the HAL. Additionally, items of the Childhood Health Assessment Questionnaire and the Activity Scale for Kids were considered for inclusion. This version was evaluated by health professionals (n = 6), patients (n = 4), and parents (n = 3). A pilot test in a sample of 32 Dutch children was performed to assess score distribution, construct validity (Spearman’s rho) and reproducibility. Administration of the pedhal was feasible for children from the age of 4 years onwards. The pedhal scores of the Dutch children were in the high end of the scale, reflecting a good functional status. Most subscales showed moderate associations with the joint examination (rho = 0.42–0.63) and moderate-to-good associations with the physical function subscale of the CHQ-50 (rho = 0.48–0.74). No significant associations were found for the pedhal and the subscales mental health and behaviour, except for the subscales leisure and sport and mental health (rho = 0.47). Test–retest agreement was good. The pedhal is a promising tool, but further testing in populations with a higher level of disability is warranted to study the full range of its psychometric properties.

Children with juvenile idiopathic arthritis (JIA) often have significant physical impairment. A minority is unresponsive to combinations of medications, and a possible treatment of resistant JIA is intense immunosuppression followed by autologous hematopoietic SCT (ASCT). Children resistant to conventional therapy have a poor prognosis with regard to long-term outcome of joint function, exercise tolerance and quality of life. It has previously been shown that ASCT can induce long-term remissions in such children. The long-term effects of this treatment are still largely unknown. This retrospective study investigates the exercise tolerance and functional ability in children with JIA who have undergone ASCT compared to healthy subjects. Ten children with JIA who received ASCT between 1997 and 2003 participated in this study. Patients were tested during their regular clinical follow-up. Exercise tolerance was determined using a maximal exercise test. Functional ability was measured using the Childhood Health Assessment Questionnaire and joint status. The study group showed significantly reduced exercise tolerance compared to healthy subjects. Functional ability and joint status were also decreased in patients after ASCT. Children with JIA postASCT have impaired exercise tolerance even 9 years postASCT.

Objectives The 6-minute walk test (6MWT) is a frequently used indicator of functional exercise capacity. The goals of this study were to compare the 6-minute walk performance of three paediatric patient groups with that of healthy peers, to assess differences between published reference values and to investigate which anthropometric characteristics best predict 6-minute walk performance. Methods 47 children with haemophilia (mean (SD) age 12.5 (2.9) years), 44 with juvenile idiopathic arthritis (JIA) (mean age 9.3 (2.2) years) and 22 with spina bifida (SB) (mean age 10.3 (3.1) years) were included. Subjects performed a 6MWT, and the distance walked (6MWD) was compared with published reference values. Results The haemophilia, JIA and SB patients achieved 90%–92%, 72%–75% and 60%–62% of predicted walking distances, respectively. There were significant associations between 6MWD and age, height and weight in the haemophilia group and 6MWD and height in the JIA group. None of the anthropometric variables was significantly related to 6MWD in the SB group. All anthropometric variables were strongly correlated with walking distance–body weight product (6Mwork) in all groups. Height explained 24% (haemophilia) and 11% (JIA) of the variance in 6MWD and 84% (haemophilia), 78% (JIA) and 73% (SB) of the variance in 6Mwork. Conclusions Walking distances of children with haemophilia, JIA and SB are significantly reduced compared with healthy references. Walking distance–body weight product seems to be a better outcome measure of the 6MWT compared with distance walked alone. Height is the best predictor of 6MWD and 6Mwork.

Introduction: Haemophilia Joint Health Score (HJHS) is the most sensitive validated score for physical examination of joint health in haemophilia. HJHS performed at regular intervals can be used for clinical monitoring as well as for comparative outcomes research. Aim: To determine whether routinely collected HJHS could be used to compare outcome of three different prophylactic regimens in children with severe haemophilia A (primary) and which parameters caused variability in HJHS (secondary). Methods: International retrospective observational multi-centre study comparing routine HJHS in 127 children with severe haemophilia A born from 1995 to 2009, from London, Stockholm and Utrecht centres. Patient and treatment data were collected from the European Paediatric Network for Haemophilia Management registry and patient files. The independent effects of regimens, physiotherapists, age and inhibitor status on HJHS were explored, using multivariable regression analysis. Results: Prophylaxis varied across participating centres, with differences in initial frequency of infusions (1× per week vs. 3× per week), age at reaching infusions ≥3× per week, and dose kg-1 week-1 at HJHS assessment. Evaluation at median age of 11 years showed an illogical association of HJHS with treatment regimen: the least intensive regimen had the lowest HJHS. The HJHS increased with age and history of inhibitor, as expected (internal validity). But the comparison of prophylactic regimens was obscured by systematic differences in assessment between physiotherapists, both within and between centres. Conclusion: Inter-physiotherapist discrepancies in routine HJHS hamper comparison of scores between treatment regimens. For multi-centre research, additional inter-observer standardization for HJHS scoring is needed.

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.

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.

Objective: To evaluate the safety and efficacy of autologous stem cell transplantation (ASCT) for refractory juvenile idiopathic arthritis (JIA). Design: Retrospective analysis of follow up data on 34 children with JIA who were treated with ASCT in nine different European transplant centres. Rheumatological evaluation employed a modified set of core criteria. Immunological reconstitution and infectious complications were monitored at three month intervals after transplantation. Results: Clinical follow up ranged from 12 to 60 months. Eighteen of the 34 patients (53%) with a follow up of 12 to 60 months achieved complete drug-free remission. Seven of these patients had previously failed treatment with anti-TNF. Six of the 34 patients (18%) showed a partial response (ranging from 30% to 70% improvement) and seven (21%) were resistant to ASCT. Infectious complications were common. There were three cases of transplant related mortality (9%) and two of disease related mortality (6%). Conclusions: ASCT in severely ill patients with JIA induces a drug-free remission of the disease and a profound increase in general wellbeing in a substantial proportion of patients, but the procedure carries a significant mortality risk. The following adjustments are proposed for future protocols: (1) elimination of total body irradiation from the conditioning regimen; (2) prophylactic administration of antiviral drugs and intravenous immunoglobulins until there is a normal CD4+ T cell count.

Juveniele idiopathische artritis (jia) is een systemische auto-immuunziekte die vooral de gewrichten treft. De gevolgen van de ziekte zijn veelzijdig en liggen op het gebied van de groei en psychosociale en motorische ontwikkeling. In dit reviewartikel worden de nieuwe ontwikkelingen op het gebied van classificatie, etiologie, pathofysiologie, prognose en behandeling van jia besproken.

Objective. To study the relationships of muscle strength and maximal oxygen consumption (VO2peak) with Childhood Health Assessment Questionnaire (CHAQ) score, Childhood Myositis Assessment Score (CMAS) and Child Health Questionnaire wphysical summary (CHQ-PhS) and psychosocial summary (CHQ-PsS)x scores in juvenile dermatomyositis. Method. Fifteen patients (age 5–14 yr) participated. CMAS, CHAQ, CHQ, muscle strength and VO2peak were measured. Results. Correlations revealed significant relationships between CHAQ and (i) muscle strength (r=20.72) and (ii) absolute VO2peak (r=20.68); between CMAS and relative VO2peak (r=0.73); and between CHQ-PhS and (i) muscle strength (r=0.57) and (ii) relative VO2peak (r=0.58). Backward regression analysis showed that muscle strength was the best indicator of variation in CHAQ. Age and relative VO2peak were the best indicators for CMAS. Body mass and age were the best indicators for CHQ-PsS. Body mass and muscle strength were the best indicators for CHQ-PhS. Conclusion. CMAS, CHAQ and CHQ correlate with muscle strength and VO2peak. CMAS, CHAQ and CHQ depend on different physical and physiological variables.

To adapt the American PEDI into a Dutch version, and to establish the content validity of this pediatric functional status instrument.The adaptation process was based on current scientific guidelines in the field of cross-cultural research. Thirty-one allied health professionals completed a validity questionnaire for the content validity study.The topic 'bicycling' was added to the questionnaire, and adaptations of the text were made without losing the content of the original PEDI. At least 81% of the respondents rated that the most important facets in examining functional status in childhood were represented in the Dutch PEDI. Eighty-seven per cent and 71% of the respondents rated that the PEDI is feasible for discriminative purposes and evaluative purposes, respectively.The expert panel confirmed the functional content and feasibility of the Dutch PEDI for pediatric rehabilitation outcome measurement.

The development of malalignment of the wrist is understood to be a combination of pathological changes induced by the disease process, compressive forces on the carpus exerted by the forearm muscle tendons crossing the wrist and the geometry of the articular surfaces. Understanding how changes in compressive forces affect malalignment is important, in view of improving physiotherapeutic treatment of the wrist in children with juvenile chronic arthritis (JCA). Quantitative analysis, taking into account the various factors involved, has recently been made possible by the development of interactive graphics based musculoskeletal models. Therefore, individual models of wrists from one healthy as well as three JCA children, with different expressions and stages of malalignment, were made. Joint moments were calculated and shear forces were estimated by applying rigid body spring modeling (RBSM) to the modified wrist models. In the radial direction, the joint moment for all JCA subjects was lower than found in ...

The impact of systemic onset JCA on functional outcome was studied in a multidimensional construct. Twenty-one patients were subjected to auxologic evaluation, a laboratory check, pulmonary and cardiac function tests, radiographic evaluation, joint count on tenderness, swelling and function, ADL, health assessment (CHAQ), and psychosocial evaluation. Six of 21 patients had active systemic disease. Restrictive pulmonary function was found in 8/17 patients, 1/21 had pericarditis. Joint impairment was moderate. Functional limitations were mild. Self-esteem was positive. Parental report on functional limitation correlated significantly with joint impairment. Performance of daily activities correlated strongly with perceived competence. Active inflammatory disease did not correlate with joint impairment and functional limitation. Patients with systemic onset JCA develop mild functional limitations, which partially correlate with the more serious impairments. Pulmonary function disorders are a common impairment. Active inflammatory disease might influence functional outcome, but there is no evidence that it influences joint impairment outcome.

Increase of factor VIII activity (FVIII) after physical exercise has been reported in healthy subjects and small-scale studies in patients with coagulopathies. The aim was to study whether moderate and mild haemophilia A patients are able to increase their endogenous FVIII activity levels by physical activity. We studied changes in FVIII activity levels after high-intensity exercise in 15 haemophilia A patients, 20-39 years, eight with moderate, seven with mild haemophilia. Patients cycled until volitional exhaustion, blood samples were drawn before and 10 min after the exercise test. FVIII activity increased 2.5 times (range 1.8-7.0 times), for both severities. Absolute increases were markedly different: median 7 IU dL(-1) (range 3-9 IU dL(-1) ) in patients with moderate, compared to 15 IU dL(-1) (range 6-62 IU dL(-1) ) in mild haemophilia patients. VWF and VWFpp increased independently of severity; median 50% (range 8-123%) and median 165% (range 48-350%), respectively, reflecting acute release of VWF. These observations may be used to promote high-intensity activities before participating in sports for moderate and mild haemophilia A patients, to reduce bleeding risk. Further studies are warranted to fully appreciate the clinical significance of exercise on different levels of intensity in patients with mild and moderate haemophilia A.

Joint physical examination is an important outcome in haemophilia; however its relationship with functional ability is not well established in children with intensive replacement therapy. Boys aged 4-16 years were recruited from two European and three North American treatment centres. Joint physical structure and function was measured with the Haemophilia Joint Health Score (HJHS) while functional ability was measured with the revised Childhood Health Assessment Questionnaire (CHAQ₃₈. Two haemophilia-specific domains were created by selecting items of the CHAQ₃₈ that cover haemophilia-specific problems. Associations between CHAQ, HJHS, cumulative number of haemarthroses and age were assessed. A total of 226 subjects - mean 10.8 years old (SD 3.8) - participated; the majority (68%) had severe haemophilia. Most severe patients (91%) were on prophylactic treatment. Lifetime number of haemarthroses [median=5; interquartile range (IQR)=1-12] and total HJHS (median = 5; IQR=1-12) correlated strongly (ρ = 0.51). Total HJHS did not correlate with age and only weakly (ρ=-0.19) with functional ability scores (median=0; IQR=-0.06-0). Overall, haemarthroses were reported most frequently in the ankles. Detailed analysis of ankle joint health scores revealed moderate associations (ρ=0.3-0.5) of strength, gait and atrophy with lower extremity tasks (e.g. stair climbing). In this population, HJHS summating six joints did not perform as well as individual joint scores, however, certain elements of ankle impairment, specifically muscle strength, atrophy and gait associated significantly with functional loss in lower extremity activities. Mild abnormalities in ankle assessment by HJHS may lead to functional loss. Therefore, ankle joints may warrant special attention in the follow up of these children.

Joint physical examination is an important outcome in haemophilia; however its relationship with functional ability is not well established in children with intensive replacement therapy. Boys aged 4-16 years were recruited from two European and three North American treatment centres. Joint physical structure and function was measured with the Haemophilia Joint Health Score (HJHS) while functional ability was measured with the revised Childhood Health Assessment Questionnaire (CHAQ₃₈. Two haemophilia-specific domains were created by selecting items of the CHAQ₃₈ that cover haemophilia-specific problems. Associations between CHAQ, HJHS, cumulative number of haemarthroses and age were assessed. A total of 226 subjects - mean 10.8 years old (SD 3.8) - participated; the majority (68%) had severe haemophilia. Most severe patients (91%) were on prophylactic treatment. Lifetime number of haemarthroses [median=5; interquartile range (IQR)=1-12] and total HJHS (median = 5; IQR=1-12) correlated strongly (ρ = 0.51). Total HJHS did not correlate with age and only weakly (ρ=-0.19) with functional ability scores (median=0; IQR=-0.06-0). Overall, haemarthroses were reported most frequently in the ankles. Detailed analysis of ankle joint health scores revealed moderate associations (ρ=0.3-0.5) of strength, gait and atrophy with lower extremity tasks (e.g. stair climbing). In this population, HJHS summating six joints did not perform as well as individual joint scores, however, certain elements of ankle impairment, specifically muscle strength, atrophy and gait associated significantly with functional loss in lower extremity activities. Mild abnormalities in ankle assessment by HJHS may lead to functional loss. Therefore, ankle joints may warrant special attention in the follow up of these children.

For patients with haemophilia, a physically active lifestyle is important to maintain musculoskeletal health and to prevent chronic diseases, such as cardiovascular disease. Therefore, we studied physical activity levels, in Dutch children and adolescents with haemophilia as well as its association with aerobic fitness and joint health. Forty-seven boys with haemophilia (aged 8-18) participated. Physical activity was measured using the Modifiable Activity Questionnaire (MAQ) and was compared with the general population. Aerobic fitness was determined using peak oxygen uptake (VO(₂peak)). Joint health was measured using the Haemophilia Joint Health Score (HJHS). Associations between physical activity, joint health and aerobic fitness were evaluated by correlation analysis. Subjects were 12.5 (SD 2.9) years old, had a Body Mass Index (BMI) of 19.5 (SD 3.1; z-score 0.5) and a median HJHS score of 0 (range 0-6). Cycling, physical education and swimming were most frequently reported (86%, 69% and 50% respectively). Children with severe haemophilia participated significantly less in competitive soccer and more in swimming than children with non-severe haemophilia. Physical activity levels were similar across haemophilia severities and comparable to the general population. VO(₂peak) kg⁻¹ was slightly lower than healthy boys (42.9 ± 8.6 vs. 46.9 ± 1.9 mL kg⁻¹ min⁻¹; P = 0.03). Joint health, aerobic fitness and physical activity showed no correlation. Dutch children with haemophilia engaged in a wide range of activities of different intensities and showed comparable levels of physical activity to the general population. Aerobic fitness was well preserved and showed no associations with physical activity levels or joint health.

Summary. For patients with haemophilia, a physically active lifestyle is important to maintain musculoskeletal health and to prevent chronic diseases, such as cardiovascular disease. Therefore, we studied physical activity levels, in Dutch children and adolescents with haemophilia as well as its association with aerobic fitness and joint health. Forty-seven boys with haemophilia (aged 8–18) participated. Physical activity was measured using the Modifiable Activity Questionnaire (MAQ) and was compared with the general population. Aerobic fitness was determined using peak oxygen uptake (VO2peak). Joint health was measured using the Haemophilia Joint Health Score (HJHS). Associations between physical activity, joint health and aerobic fitness were evaluated by correlation analysis. Subjects were 12.5 (SD 2.9) years old, had a Body Mass Index (BMI) of 19.5 (SD 3.1; z-score 0.5) and a median HJHS score of 0 (range 0–6). Cycling, physical education and swimming were most frequently reported (86%, 69% and 50% respectively). Children with severe haemophilia participated significantly less in competitive soccer and more in swimming than children with non-severe haemophilia. Physical activity levels were similar across haemophilia severities and comparable to the general population. VO2peak kg−1 was slightly lower than healthy boys (42.9 ± 8.6 vs. 46.9 ± 1.9 mL kg−1 min−1; P = 0.03). Joint health, aerobic fitness and physical activity showed no correlation. Dutch children with haemophilia engaged in a wide range of activities of different intensities and showed comparable levels of physical activity to the general population. Aerobic fitness was well preserved and showed no associations with physical activity levels or joint health.

The purpose of this study was to develop an activity energy expenditure (AEE) prediction equation for the Actiheart activity monitor for use in children with chronic disease.In total, 63 children, aged 8-18 years with different types of chronic disease (juvenile arthritis, hemophilia, dermatomyositis, neuromuscular disease, cystic fibrosis or congenital heart disease) participated in an activity testing session, which consisted of a resting protocol, working on the computer, sweeping, hallway walking, steps and treadmill walking at three different speeds. During all activities, actual AEE was measured with indirect calorimetry and the participants wore an Actiheart on the chest. Resting EE and resting heart rate were measured during the resting protocol and heart rate above sleep (HRaS) was calculated.Mixed linear modeling produced the following prediction equation: This equation results in a nonsignificant mean difference of 2.1 J/kg/min (limits of agreement: -144.2 to 148.4 J/kg/min) for the prediction of AEE from the Actiheart compared with actual AEE.The Actiheart is valid for the use of AEE determination when using the new prediction equation for groups of children with chronic disease. However, the prediction error limits the use of the equation in individual subjects.

Background The original version of the Childhood Health Assessment Questionnaire (CHAQ30orig) suffers from a ceiling effect and hence has reduced clinical validity. The purpose of this study was to evaluate the effect of adding eight more demanding items (CHAQ38) and a new categorical response option (CATII) on discriminant validity and score distribution in a European patient sample. Methods Eighty-nine children with Juvenile Idiopathic arthritis (JIA) and 22 healthy controls, aged 7-16 years, were recruited from eight centres across Europe. Eight new CHAQ items and scoring option were translated back and forth for the countries in which they were not already present. Demographic, clinical, and CHAQ data were collected on-site. Subsequently, five different scoring methods were applied, i.e. the original method (CHAQ30orig) and four alternatives. These alternatives consisted of the mean item scores for the 30 and 38-question versions with either the original (CATI), or the new categorical response option (CATII). The five versions were tested for their ability to distinguish between patients and controls. Furthermore score distributions were evaluated and visualized by box and whisker plots. Results Two CHAQ revisions with the new response option showed poor discriminative ability, whereas one revised version (CHAQ38CATI) had comparable discriminative ability comparable to the original CHAQ. A profound ceiling effect was observed in the original scoring method of the CHAQ (27%). The addition of eight more demanding items and application of a plain mean item score reduced this significantly to 14% (χ2 = 4.21; p < 0.05). Conclusions Revising the CHAQ by adding eight more demanding items and applying a plain mean item scoring (CHAQ38CATI) maintained discriminant ability and reduced the ceiling effect in a European patient sample. The new categorical response option (CATII) seemed promising, but was less able to distinguish children with JIA from healthy controls and had less favourable distribution characteristics. The CHAQ38CATI is advocated for future use in mildly affected JIA patients.

Transition of care for adolescents includes a transfer from paediatric to adult health care. This requires a transfer of specific measurements, which evaluate disease profiles such as functional ability. One of the most common measurements is the Health Assessment Questionnaire (HAQ).Results of the Childhood HAQ (CHAQ) and HAQ were compared among adolescents diagnosed with rheumatic diseases involving the musculoskeletal system. All adolescents had recently dealt with or would in the near future be dealing with transition.Overall results of both questionnaires were comparable; intra-class correlation for consistency (ICC) was 0.95 (95% confidence interval 0.93-0.97). For a smooth transfer from CHAQ to HAQ, both correlation and agreement are required. Agreement between both questionnaires was not found. Described by limits of agreement, results of HAQ can differ from CHAQ as much as 0.95.Despite strong correlations for consistency, lack of agreement was found in the results of CHAQ and HAQ. If correlation persists over time, this study suggests evaluating both the childhood and adult version of the HAQ during the transition period. When transfer into adulthood is completed, comparison to earlier tests at younger age is available and reliable.

Worldwide, children with haemophilia suffer from limitations in performing activities of daily living. To measure such limitations in adults a disease-specific instrument, the Haemophilia Activities List (HAL), was created in 2004. The aim of this study was to adapt the HAL for children with haemophilia and to assess its psychometric properties. The structure and the main content were derived from the HAL. Additionally, items of the Childhood Health Assessment Questionnaire and the Activity Scale for Kids were considered for inclusion. This version was evaluated by health professionals (n = 6), patients (n = 4), and parents (n = 3). A pilot test in a sample of 32 Dutch children was performed to assess score distribution, construct validity (Spearman's rho) and reproducibility. Administration of the pedhal was feasible for children from the age of 4 years onwards. The pedhal scores of the Dutch children were in the high end of the scale, reflecting a good functional status. Most subscales showed moderate associations with the joint examination (rho = 0.42-0.63) and moderate-to-good associations with the physical function subscale of the CHQ-50 (rho = 0.48-0.74). No significant associations were found for the pedhal and the subscales mental health and behaviour, except for the subscales leisure and sport and mental health (rho = 0.47). Test-retest agreement was good. The pedhal is a promising tool, but further testing in populations with a higher level of disability is warranted to study the full range of its psychometric properties.

The Childhood Health Assessment Questionnaire (CHAQ30) is the most commonly used physical functioning questionnaire for children with Juvenile Idiopathic Arthritis (JIA). By revising the CHAQ30 Lam et al. succeeded in decreasing the ceiling effect of this questionnaire in a North American population of children with diverse musculoskeletal diseases.To examine the score distribution of the revised CHAQ in a population of children with JIA.In this Dutch multicentre study 72 children with JIA participated (55 girls), with a mean age of 11.0 (+/- 3.1) and a mean disease duration of 4.6 year (+/- 3.7). The score distribution of the original CHAQ30 and four versions of the revised CHAQ was analysed with the median, range and interquartile range (IQR) and visualised with box-and-whisker plots. The normality of the score distribution was tested by the Kolmogorov-Smirnov one-sample test of normality.Although the addition of 8 more challenging items improved the spread of the scores of the revised CHAQ versions, the original CHAQ30 showed a better distribution of the scores.The revised CHAQ38 with the distribution characteristics, found in this study, might be especially relevant in interventions for patients with JIA at the mild end of the disability spectrum.

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).To assess the effects of exercise therapy on functional ability, quality of life and aerobic capacity in children with JIA.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.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.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.

Address: 1Department of Pediatric Physical Therapy & Paediatric Exercise Physiology, Het Wilhelmina Kinderziekenhuis, University Children's Hospital, Utrecht, Utrecht, Netherlands, 2Department of Pediatric Physiotherapy, Sophia Children's Hospital, Erasmus University, Rotterdam, Zuid-Holland, Netherlands, 3Department of Physiotherapy (HO-Q), Leiden University Medical Centre, Leiden, Zuid-Holland, Netherlands, 4Department Physiotherapy, University Hospital Groningen, Groningen, Groningen, Netherlands and 5Department Paediatric Physiotherapy, University Medical Center Nijmegen St. Radboud, Nijmegen, Gelderland, Netherlands * Corresponding author

OBJECTIVE: To examine the aerobic and anaerobic exercise capacity in adolescents with juvenile idiopathic arthritis (JIA) compared with age- and sex-matched healthy individuals, and to assess associations between disease-related variables and aerobic and anaerobic exercise capacity.METHODS: Of 25 patients enrolled in a JIA transition outpatient clinic, 22 patients with JIA were included in this study (mean +/- SD age 17.1 +/- 0.7 years, range 16-18 years). Aerobic capacity was examined using a Symptom Limited Bicycle Ergometry test. Anaerobic capacity was assessed with the Wingate Anaerobic Test. Functional ability was assessed with the Childhood Health Assessment Questionnaire. Pain and overall well-being were measured using a visual analog scale. Disease duration and disease activity were also assessed.RESULTS: Absolute and relative maximal oxygen consumption in the JIA group were significantly impaired (85% and 83% for boys, respectively; 81% and 78% for girls, respectively) compared with healthy controls. Mean power was also significantly impaired (88% for boys and 74% for girls), whereas peak power was significantly impaired for girls and just failed significance for boys (67% for girls and 92% for boys). A post hoc analysis correcting for underweight and overweight demonstrated that body composition did not influence the results substantially.CONCLUSION: This study demonstrated that adolescents with JIA have an impaired aerobic and anaerobic exercise capacity compared with healthy age- and sex-matched peers. The likely cause for this significant impairment is multifactorial and needs to be revealed to improve treatment strategies.

The aim of this study was to examine the psychometric characteristics of the childhood health assessment questionnaire-disability index (CHAQ-DI). Seventy-six patients with juvenile idiopathic arthritis (JIA), age range 4.8-15.8 years, completed a CHAQ questionnaire one or more times. In total, 321 CHAQ questionnaires were available for analysis. Factor analysis and correlation were used to analyse the data. The analysis indicated that 12 items could be removed from the original 30 items of the CHAQ-DI. Also the addition of "aids and assistance" to the overall scoring method of the CHAQ-DI did not contribute to the overall measuring concept of the CHAQ-DI. The psychometric characteristics of the CHAQ-DI could be improved by removing 12 items from the original 30 items. Moreover, a simple scoring method, without the addition of aids and assistance to the total CHAQ-DI improves sensitivity to change of the CHAQ-DI.

Objective To determine the physiologic response of the 6-minute walk test (6-mwt) in children with juvenile idiopathic arthritis (JIA). Methods Eighteen children with JIA (age 7–17 years; 6 boys, 12 girls) performed a 6-mwt and a maximal exercise test. Results The physiologic response of the 6-mwt was on average between 80% and 85% of the peak values of heart rate and oxygen uptake (VO2peak) during the maximal exercise test, except for the minute ventilation, which had a mean percentage of 68.5%. Backward regression analysis showed that height and distance walked were the best predictors of VO2peak during cycling (R2 = 0.883, P < 0.001). During the 6-mwt, the difference between the first and second minute was significant in every variable, except for heart rate. The range of walking distance of children with JIA was comparable with that of healthy elderly people. Conclusion The physiologic response of the 6-mwt is at a submaximal, intense level of exercise. The course of the responses during the 6-mwt was normal. The 6-mwt can be regarded as a good test for measuring functional exercise capacity.

The aim of this review is to determine whether physical fitness, assessed by peak oxygen uptake (VO2peak) measurement, is reduced in survivors of acute lymphoblastic leukemia (ALL) compared to healthy children. A systematic literature search (up to June 2004) was performed using Medline, Sportdiscus, Cinahl, Embase, Cochrane and PEDro database and reference tracking. The VO2peak (ml kg-1 min-1) reached during a maximal exercise test until volitional exhaustion was used as the main outcome for this review. In all, 17 studies were identified in the literature. Data from three studies (102 ALL survivors, age ranging from 7 to 19 years) were pooled in a meta-analysis. Although there was a significant heterogeneity between the included studies (P=0.0006), the standardized mean difference (SMD) value of -0.61 (P=0.07) indicated that VO2peak tended to be reduced in survivors of childhood ALL compared to healthy control subjects, that is, decrease of -5.97 ml kg-1 min-1 (95% confidence interval (CI): (-12.35, 0.41); P=0.07) or -13% (95 % CI: (-27, 0.004)). Physical fitness tends to be reduced in survivors of ALL during childhood, which suggests the need for this population group to engage in regular physical activities with the purpose of increasing their functional capacity. Although more research is needed, this functional improvement might ameliorate the quality of life of ALL survivors as physical and outdoors activities are an essential part of daily routine during childhood. Sin financiación 6.612 JCR (2005) Q1, 13/123 Oncology, 5/60 Hematology UEM

To analyze the displacement response of juvenile arthritic wrists during grasp in order to diagnose early ligamental laxity and facilitate early splinting.X-rays of the wrists, made under standardized conditions, of 30 children with juvenile chronic arthritis (mean age 10.4 years, range 4.5-16.9) were analyzed after being digitalized. Osseous landmarks were identified, and coordinates were calculated from measured angles and lengths with an accuracy of 0.01'. Lunate and carpal-ulnar distance were obtained according to Youm, and ulnar variance according to Häfner.Overall, an increase in ulnar-lunate displacement and carpal narrowing and a decrease in ulnar variance were found. However, not all wrists responded to the same extent. Radial displacement of the lunate, though slight, was found in 2 wrists and the amount of ulnar displacement varied substantially (3.1% to 22.5%). The variance in amount of displacement could suggest that juvenile wrists do not respond to increased compressive forces to the same extent.The changes found are similar to those found in the healthy wrist. Furthermore, our findings suggest that the juvenile wrist acts in accordance with the generally accepted explanation for the development of malalignment in adult wrists. It seems that laxity of ligaments can be diagnosed early by the force grip maneuver during x-ray. It would have a significant impact on the moment of orthotic intervention as well as the design of the orthotic device. Further study along this line seems justified.

In 2002, all children in the Netherlands from four to 18 years of age, including the Juvenile Idiopathic Arthritis (JIA) population, were vaccinated with the group-C meningococci conjugate vaccine. The aim of this study is to determine whether the vaccine increases JIA disease activity by monitoring the core set criteria of JIA patients as well as investigates the efficacy of this vaccine in those receiving DMARDs. The core set criteria included Childhood Health Assessment Questionnaire (CHAQ) disability, CHAQ pain, CHAQ well being, number of inflamed joints, range of motion, ESR, CRP, and semi-quantitative physicians global assessment. These were monitored for six months prior to as well as after vaccination for any changes in 86 JIA patients. Blood samples were taken from patients to measure antibody production using ELISA as well as T cell proliferation through intracellular fluorescent dye CFSE. In summary, no significant differences (P>0.05) were found when comparing the core set criteria six months before as well as six months after vaccination. However, there were significant increases of CD4+ T cell proliferation both against the carrier protein of the vaccine (P=0.034) as well as the whole vaccine (P=0.035). Furthermore, anti-Men C IgG antibody production increased significantly (>18µg/ml) irrespective of DMARD therapy. In conclusion, our data suggests that the group-C meningococci conjugated vaccine does not increase JIA disease activity. Moreover, B as well as T lymphocyte activity against group-C meningococci are not significantly diminished in patients undergoing immune suppressive treatments.

Objective: To obtain insight into the interaction between daily physical activity and components of health related physical fitness in children with juvenile idiopathic arthritis. Methods: Forty five patients (10 male/35 female; mean (SD) age 8.9 (2.2) years) participated in the study. Body mass, height, skinfold thickness, number of swollen joints, and joint range of motion were determined. The maximal oxygen consumption (Vo 2peak ) was assessed during a graded maximal bicycle exercise test. Daily physical activity levels were measured with a Caltrac activity monitor and a parental physical activity rating (PAL) on a five point Likert scale. Results: Partial correlation coefficients (to control for age) between physical activity and indices of health related physical fitness showed significant relationships between Caltrac motion counts and absolute Vo 2peak ( r =0.31) and relative Vo 2peak ( r =0.34), but not with the indices of body composition. There was also a significant correlation between PAL and relative Vo 2peak ( r =0.33). Conclusions: Physical activity was significantly related to cardiorespiratory fitness but not to body composition in children with juvenile idiopathic arthritis. A longitudinal follow up should show whether an active lifestyle protects for loss of aerobic fitness in this patient group.

Objective: To determine the relationship between aerobic and anaerobic physical fitness and functional ability in children with juvenile idiopathic arthritis (JIA).Methods: Eighteen children with JIA (age 7 to 14 yr., 3 male/15 female) performed a maximal aerobic exercise test and a Wingate anaerobic exercise test. Functional ability was concurrently assessed using the Childhood Health Assessment Questionnaire (CHAQ).Results: A low relationship between aerobic fitness and functional ability was found (r=0.0 to 0.4, p>0.05, except for eating r=0.46, p

Objective To evaluate the effects of an aquatic training programme for JIA patients. Methods Fifty-four patients with JIA (age range 5 to 13 yr) participated in this study and were randomized into an experimental (n = 27) and a control (n = 27) group. The children in the experimental group received a training programme consisting of a 1 h per week supervised training programme in a local pool of approximately 20 sessions. Effects were analysed on the following domains: functional ability, health-related quality of life, joint status and physical fitness. Results Although all measures improved more in the experimental group than the control group, none of the differences was statistically significant. Conclusions The current research found no significant effect of an aquatic fitness training programme in children with JIA. Since there were no signs of worsening in health status, one can conclude that this was a safe exercise programme.

We report herein the results of the cross-cultural adaptation and validation into the Dutch language of the parent's version of two health related quality of life instruments. The Childhood Health Assessment Questionnaire (CHAQ) is a disease specific health instrument that measures functional ability in daily living activities in children with juvenile idiopathic arthritis (JIA). The Child Health Questionnaire (CHQ) is a generic health instrument designed to capture the physical and psychosocial well-being of children independently from the underlying disease. The Dutch CHAQ was fully validated with 3 forward and 3 backward translations while the CHQ was already published and therefore it was revalidated. A total of 180 subjects were enrolled: 100 patients with JIA (17% systemic onset, 31% polyarticular onset, 18% extended oligoarticular subtype, and 34% persistent oligoarticular subtype) and 80 healthy children. The CHAQ clinically discriminated between healthy subjects and JIA patients, with the systemic, polyarticular and extended oligoarticular subtypes having a higher degree of disability, pain, and a lower overall well-being when compared to their healthy peers. Also the CHQ clinically discriminated between healthy subjects and JIA patients, with the systemic onset, polyarticular onset and extended oligoarticular subtypes having a lower physical and psychosocial well-being when compared to their healthy peers. In conclusion the Dutch version of the CHAQ-CHQ is a reliable, and valid tool for the functional, physical and psychosocial assessment of children with JIA.

More and more researchers are questioning the theoretical and scientific foundations as well as the efficacy and effects of many physiotherapy interventions. The same applies for many of the neurophysiological based interventions that are being used in paediatric rehabilitation. Opinions and views regarding the development of motor behaviour of infants and children are significantly changing. Paediatric interventionists should consider bringing their interventions and focus of treatment into agreement with changed scientific knowledge. Moreover, for almost all other medical problems in childhood, paediatric rehabilitation has little to offer but mostly miniaturized forms of adult treatment. It not only means that we have to make a paradigm shift, but also are in the need of a broader view on paediatric rehabilitation as a specialized professional activity.