Functional limitations in Romanian children with haemophilia: further testing of psychometric properties of the Paediatric Haemophilia Activities List.

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.

Determinants of participation in patients with severe haemophilia.

The multifactorial nature of disability makes it difficult to point out a specific cause for limitations in participation. The conceptual framework of the WHO-ICF (International Classification of Function, Disability and Health) was used to study the determinants participation in patients with severe haemophilia. Outcome was assessed in a single-centre cohort of 124 patients with severe haemophilia. Joint mobility and muscle strength of the elbows, knees and ankles, in combination with recent X-ray findings (N = 39 only) and the MPQ-DLV pain questionnaire were used to assess Body Functions and Structures. Four performance-based functional tasks and the HAL questionnaire were used to assess Activities. The IPA questionnaire was used to assess Participation. Stepwise and hierarchical regression analysis adjusted for age and psychological health (Dutch-AIMS 2) was used to associate the various domains of the ICF. Irrespective of age, joint mobility was an important factor in explaining self-reported and performance-based activities. Muscle strength had no significant association with participation. Self-reported activities showed a stronger association with participation than performance-based activities. Adjusted for age and psychological health, joint mobility and pain explained none of the variation in participation. Self-reported activities, however, significantly contributed in explaining participation (25%), whereas performance-based activities (3%) did not. This study adds to the knowledge of determinants of participation in haemophilia. As the currently used instruments on joint status and activities only partially explain differences in participation, this aspect of clinical outcome should be included to fully assess outcome in haemophilia.

The effectiveness of graded activity in patients with non-specific low-back pain: a systematic review.

BACKGROUND: Non-specific low-back pain (LBP) is considered a major health and economic problem in Western society. Nowadays a common used intervention on non-specific LBP is graded activity (GA). Graded Activity developed by Lindström et al., consisted of four parts: (i) measurements of functional capacity; (ii) a work-place visit; (iii) back school education and (iv) an individual, sub-maximal, gradually increased exercise program with an operant-conditioning behavioural approach as described by Fordyce et al. OBJECTIVE: To evaluate the effectiveness of GA in adults with non-specific LBP on pain, disabilities and return to work. DATA SOURCES: An extensive literature search of PubMed, Embase, CINAHL and The Cochrane Library was conducted in July 2011. REVIEW METHODS: Randomized controlled trials (RCTs) evaluating the effect of GA in patients with non-specific LBP were eligible. Methodological quality of the studies was assessed according to the PEDro scale. A best-evidence synthesis was conducted according to van Peppen et al. to interpret the outcomes of the included studies. RESULTS: Ten articles were included in this systematic review; these articles described five RCTs (680 patients). The best-evidence synthesis revealed that there was no or insufficient evidence for a positive effect of GA on pain, disabilities and return to work in patients with non-specific LBP. CONCLUSION: Currently there is no or insufficient evidence that GA results in better outcomes of patients with non-specific LBP.

Habitual physical activity in Dutch children and adolescents with haemophilia.

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.

Symptomatic asymmetry in very young infants: a Delphi study on the development of a screening instrument.

The objective of this study was to develop a screening instrument for pediatric physiotherapists to distinguish a symptomatic asymmetry in the clinical evaluation of young infants (age <6 months) with an asymmetric head posture. We chose two consensus methods, a two-round Delphi design and an expert meeting using nominal group technique, for reaching agreement about classification of diagnoses and clinical diagnostic criteria (CDC). Seventeen diagnoses with an expression of asymmetry with 69 matching CDC were assessed. In two Delphi rounds, six medical specialists and seven pediatric physiotherapists were polled anonymously on the classification, completeness, and relevance of the diagnoses and the CDC. Panel consistency in round 2, expressed as Cronbach's-α, was 0.89. In round 3, a face-to-face meeting with eight therapists, the previously selected diagnoses and CDC were prioritised, reduced to 10 diagnoses and 21 CDC, and completed with eight hard clinical signs (red flags). Finally, a differential diagnostic screening instrument, containing a classification scheme, the CDC for differential diagnostics, and a list of "red flags" was established on the basis of literature search and expert consensus. Cross-validity and reliability of the instrument will be investigated in future research.

Is static hyperinflation a limiting factor during exercise in adolescents with cystic fibrosis?

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 effort >0.70; 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) (%)).

High-intensity interval training in an adolescent with cystic fibrosis: a physiological perspective.

Nutritional, musculoskeletal, and/or ventilatory status can lead to a decreased exercise capacity in children with cystic fibrosis (CF). Exercise training is already part of the usual care; however, the "optimal" intensity and volume of exercise training to improve exercise capacity is still unknown. Six weeks of high-intensity interval training (HIT) for a patient with CF with a ventilatory limitation was evaluated by a cardiopulmonary exercise test (CPET). Peak oxygen uptake and peak workload increased 19% and 16%, respectively, and there was a rise in peak ventilation from 50 L/min to 75 L/min, with an increase in both breathing depth and respiratory rate. A relative short period of HIT resulted in a significant increase in exercise capacity. In patients with CF, HIT might be an effective and efficient training regimen, especially in CF patients with a ventilatory limitation. Further research is necessary to investigate whether HIT is a better alternative than traditional aerobic training programs especially in ventilatory limited patients with CF.

Aerobic capacity and muscle strength in juvenile-onset mixed connective tissue disease (MCTD).

OBJECTIVES: To study the aerobic capacity and muscle strength in children and adolescents with mixed connective tissue disease (MCTD). Frequently reported clinical symptoms include joint swelling, muscle weakness, fatigue, decreased stamina/exercise tolerance, and shortness of breath. The exercise capacity of patients with MCTD has not been studied systematically in this detail before. METHODS: Eleven children and adolescents diagnosed with MCTD (mean age 15.7 years, range 11.3­19.9 years) were studied. Maximal exercise testing on a cycle ergometer was used to determine the peak oxygen uptake (VO2peak) and a hand-held dynamometer was used to measure muscle strength. Cardiac and pulmonary function tests (ultrasonography, electrocardiography, spirometry) were used to measure cardiac function and obstructive or restrictive respiratory impairment. Complementary data (e.g. disease duration and concurrent symptoms) were collected from a medical chart review. RESULTS: VO2peak was significantly lower in patients with MCTD compared to the VO2peak of healthy subjects (Z-score ­1.9, p = 0.008). The strength of the proximal muscles (hip flexors, shoulder abductors, knee extensors) of the patients was significantly lower than in the controls, whereas the strength of the distal muscles (dorsal flexors of the foot and handgrip strength) showed no differences. In eight children, arthritis was observed. No clinically relevant impairment in cardiac or pulmonary function was observed. CONCLUSIONS: Aerobic capacity and also proximal muscle strength were significantly impaired in our sample of children and adolescents with MCTD. Because respiratory problems were non-dominant in our patient group, the decreased aerobic capacity and muscle strength were probably caused by musculoskeletal impairments. Further studies in larger multicentre samples are warranted to confirm our findings.

The Dutch translation of the revised Childhood Health Assessment Questionnaire: a preliminary study of score distribution.

BACKGROUND: 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. OBJECTIVES: To examine the score distribution of the revised CHAQ in a population of children with JIA. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.

Oxygen uptake to work rate slope in children with a heart, lung or muscle disease.

The purposes of this study were all to determine if DeltaVO2/DeltaWR is dependent on age, body mass, height and fitness and if DeltaVO2/DeltaWR could discriminate between healthy children and children with a chronic disease that limits O2 delivery or utilization. Four groups were included: muscle disease (Juvenile Dermatomyositis; JDM; n=12), lung disease (Cystic Fibrosis; CF; n=13), Congenital Heart Disease (CHD; (n=13), and healthy children (n=44). All children performed a cardiopulmonary exercise test on a cycle ergometer with respiratory gas analysis. The DeltaVO2/DeltaWR was determined by linear regression using data from unloaded cycling to peak exercise. No associations were found between the DeltaVO2/DeltaWR and age, body mass and height in healthy children. DeltaVO2/DeltaWR was significantly correlated with VO2peak/kg (r=0.44; p<0.01). Children with JDM had lower DeltaVO2/DeltaWR values than healthy children (p=0.02), and DeltaVO2/DeltaWR tended to be lower in CHD and higher in CF (p=0.09 and p=0.08, respectively). DeltaVO2/DeltaWR may be more sensitive for conditions that are characterized by local hypo perfusion (as in JDM), than for conditions that are characterized by impaired oxygen delivery (i. e. CF or CHD).

Development and preliminary testing of a Paediatric Version of the Haemophilia Activities List (pedhal).

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.

Reproducibility of energy cost of locomotion in ambulatory children with spina bifida.

OBJECTIVES: Many ambulatory children with Spina Bifida (SB) experience functional decline in ambulation despite stable or even improving motor exams. Improving or maintaining low energy cost of locomotion during childhood and throughout the teenage years, could be an important goal for children and adolescents with SB. Purpose of this study was to determine reproducibility of energy expenditure measures during gait in ambulatory children with SB. DESIGN: Reproducibility study. SETTING: Child Development and Exercise Center of the University Children's Hospital in Utrecht, the Netherlands. PARTICIPANTS: Fourteen ambulatory children (6 boys/8 girls) with SB. Mean age was 10.8 years (+ or - 3.4). INTERVENTIONS: Net and gross energy expenditure measures during locomotion were determined during a six-minute walking test. These measures consisted of energy consumption (ECS), expressed in J/kg/min, and energy cost (EC), expressed in J/kg/m. For reliability, the intra-class coefficient (ICC) was determined. For agreement, the smallest detectable difference (SDD) was calculated. RESULTS: ICCs vary from 0.86 to 0.96 for both EC and ECS. The SDD ranges from 18-24% for gross measures, up to over 30% for net values. CONCLUSION: Reproducibility of energy expenditure during ambulation in children with SB should be considered carefully when using these measures in the evaluation of gait. High reliability of energy expenditure measurements makes these measurements appropriate to use as discriminative tools in children with SB, while agreement of only gross EC seems acceptable to use as a evaluative tool in children with SB. Overall, measures of reliability and agreement seem higher in young children when compared to adolescents. Further research is recommended to determine clinically relevant changes in energy expenditure in children with SB.

Six-minute walk test in children with chronic conditions.

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.

Cardiopulmonary exercise testing in congenital heart disease: equipment and test protocols.

Cardiopulmonary exercise testing (CPET) in paediatric cardiology differs in many aspects from the tests as performed in adult cardiology. Children's cardiovascular responses during exercise testing present different characteristics, particularly oxygen uptake, heart rate and blood pressure response, which are essential in interpreting haemodynamic data. Diseases that are associated with myocardial ischaemia are very rare in children. The main indications for CPET in children are evaluation of exercise capacity and the identification of exercise-induced arrhythmias. In this article we will review exercise equipment and test protocols for CPET in children with congenital heart disease. (Neth Heart J 2009;17:339-44.).

Cardiopulmonary exercise testing in congenital heart disease: (contra)indications and interpretation.

Cardiopulmonary exercise testing (CPET) in paediatric cardiology differs in many aspects from the tests performed in adult cardiology. Children's cardiovascular responses during exercise testing present different characteristics, particularly oxygen uptake, heart rate and blood pressure response, which are essential in interpreting haemodynamic data. Diseases that are associated with myocardial ischaemia are rare in children. The main indications for CPET in children are evaluation of exercise capacity and the identification of exercise-induced arrhythmias. In this article we will review the main indications for CPET in children with congenital heart disease, the contraindications for exercise testing and the indications for terminating an exercise test. Moreover, we will address the interpretation of gas exchange data from CPET in children with congenital heart disease. (Neth Heart J 2009;17:385-92.).

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.

Gross motor functional abilities in preterm-born children with cerebral palsy due to periventricular leukomalacia.

To describe the impact of periventricular leukomalacia (PVL) on gross motor function, data on 59 children (37 males, 22 females) with a gestational age (GA) of 34 weeks or less with cerebral palsy (CP) due to PVL grade I (n=20), II (n=13), III (n=25), and IV (n=1) were studied; (mean GA 29 wk 4d [SD 4 wk 6d]; mean birthweight 1318 g [SD 342]). Two independent raters used the Gross Motor Function Classification System (GMFCS) at four time points: T1, mean corrected age (CA) 9 months 15 days (SD 2 mo 6d); T2, mean CA 16 months (SD 1 mo 27 d); T3, mean CA 24 months 27 days (SD 2 mo 3d); and T4, median age 7 years 6 months (range 2 y 2 mo-16 y 8 mo). Interrater reliability and stability across time with respect to the total cohort were kappa>or=0.86 and rho>or=0.74 respectively. The association between PVL and gross motor outcome at T4 was strong (positive and negative predictive values 0.92 and 0.85 respectively). The proportion of children who remained in the same GMFCS level increased from 27% (T1-T4) to 53% (T2-T4) and 72% (T3-T4). PVL grade I to II, as diagnosed in the neonatal period, has a better functional mobility prognosis than PVL grade III-IV. These findings have implications for habilitation counselling and intervention strategies.

Limiting factors in peak oxygen uptake and the relationship with functional ambulation in ambulating children with spina bifida.

The objective of this study is to interpret the outcomes of peak oxygen uptake (VO(2peak)) in children with SB and explore the relationship between VO(2peak) and functional ambulation using retrospective cross-sectional study. Twenty-three ambulating children with SB participated at Wilhelmina's Children's Hospital Utrecht, the Netherlands. VO(2peak) was measured during a graded treadmill-test. Eschenbacher's and Maninna's algorithm was used to determine limiting factors in reaching low VO(2peak) values. Energy expenditure during locomotion (both O(2) rate and O(2) cost) and percentage of VO(2peak) and HR(peak) were determined during a 6-min walking test (6MWT). Differences between community and normal ambulators were analyzed. VO(2peak), VO(2peak)/kg, HR(peak), RER(peak) and VE (peak) were significantly lower compared to reference values, with significant differences between normal and community ambulators. Limiting factors according to the algorithm were mostly "muscular and/or deconditioning" (47%) and ventilatory "gasexchange" (35%). Distance walked during 6MWT was 48.5% of predicted distance. Both O(2) rate and O(2) cost were high with significant differences between normal and community ambulators [17.6 vs. 21.9 ml/(kg min) and 0.27 vs 0.43 ml/(kg m)]. Also %HR(peak) and %VO(2peak) were significantly higher in community ambulators when compared to normal ambulators (resp. 97.6 vs. 75% and 90.2 vs. 55.9%). VO(2peak) seems to be mostly limited by deconditioning and/or muscular components and possible ventilatory factors. For both peak values and functional ambulation, community ambulators were significantly more impaired than normal ambulators. High energy expenditure, %VO(2peak) and %HR(peak) reflect high level of strain during ambulation in the community ambulators. Future exercise testing in children with SB should include assessment of ventilatory reserve. Exercise training in ambulatory children should focus on increasing both VO(2peak) and muscular endurance, as well as decreasing energy cost of locomotion.

Exercise tolerance in children with juvenile idiopathic arthritis after autologous SCT.

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.

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.