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.

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.

Joint health and functional ability in children with haemophilia who receive intensive replacement therapy.

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.

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.

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.

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.

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).

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.

Juvenile chronic arthritis: T cell reactivity to human HSP60 in patients with a favorable course of arthritis.

Synovial fluid and peripheral blood mononuclear cell proliferative responses to the 60-kD human heat shock protein (HSP60) were studied in 23 patients with juvenile chronic arthritis (JCA) and 7 non-JCA control patients. All patients showed active arthritis at the time of study. The patients were divided into two groups according to the presence (group A) or absence (group B) of T lymphocyte reactivity to human HSP60. We show that reactivity to human HSP60 is primarily, though not exclusively, occurring in patients with a remitting course of disease, i.e., the subgroup of HLA-B27 negative JCA patients with an oligoarticular onset. Immunohistochemical analysis of HSP expression in synovial membranes showed a significantly higher intensity of staining in JCA patients than in non-JCA controls. The results suggest that, in accordance with the earlier observation made in experimental models, T lymphocyte reactivity to human HSP60 in this subgroup of JCA patients may be part of T cell regulatory mechanisms that control the development of arthritis.

The development of a clinical practice stroke guideline for physiotherapists in The Netherlands: a systematic review of available evidence.

PURPOSE: To develop a clinical practice guideline for the physiotherapy management of patients with stroke as support for the clinical decision-making process, especially with respect to the selection of appropriate interventions, prognostic factors and outcome measures. INTRODUCTION: Physiotherapists have a high caseload of patients with stroke, so there is a need to identify effective evidence-based physiotherapy procedures. The availability of a guideline that includes information about prognostic factors, interventions, and outcome measures would facilitate clinical decision-making. METHOD: A systematic computerized literature search was performed to identify evidence concerning the use of: (i) prognostic factors related to functional recovery; (ii) physiotherapy interventions in patients with stroke; and (iii) outcome measures to assess patients' progress in functional health. Experts, physiotherapists working in the field of stroke rehabilitation, and a multidisciplinary group of health professionals reviewed the clinical applicability and feasibility of the recommendations for clinical practice and their comments were used to compose the definitive guideline. RESULTS: Of 9482 relevant articles, 322 were selected. These were screened for methodological quality. Seventy-two recommendations for clinical practice were retrieved from these articles and included in the guideline: Six recommendations concerned the prediction of functional recovery of activities of daily living (ADL), including walking ability and hand/arm use; 65 recommendations concerned the choice of physiotherapy interventions; and 1 recommendation concerned the choice of outcome instrument to use. A core set of seven reliable, responsive, and valid outcome measures was established, to determine impairments and activity limitations in patients with stroke. CONCLUSIONS: The guideline provides physiotherapists with an evidence-based instrument to assist them in their clinical decision making regarding patients with stroke. As most of the recommendations included in the guideline came from studies of patients in the post acute and chronic phase of stroke, and in general involved patients with less severe and uncomplicated stroke, more needs to be learned about the more complex cases.

[Fewer lung complications following inspiratory muscle training in patients undergoing coronary bypass surgery: a randomized trial]. / Minder longcomplicaties door ademspiertraining bij patiënten die een coronaire bypassoperatie moeten ondergaan: een gerandomiseerde trial.

OBJECTIVE: The aim of this study was to evaluate the prophylactic efficacy of preoperative physiotherapy, including inspiratory muscle training (IMT), on the incidence of postoperative pulmonary complications (PPCs) in high-risk patients scheduled for elective coronary artery bypass grafting (CABG). DESIGN: Randomized controlled clinical trial (www.controlled-trials. com/isrctn17691887). METHOD: Of 655 patients referred to a university medical centre in The Netherlands for elective CABG, 299 met the criteria for being at high risk of developing PPCs. A total of 279 were enrolled and monitored up to discharge from hospital. Patients were randomly assigned to receive either preoperative IMT (n=140) or usual care (n=139). Both groups received the same postoperative treatment. RESULTS: Both groups were comparable at baseline. Before CABG, 2 control group patients and 1 IMT group patient died. After CABG surgery, PPCs were present in 25 (18%) of 139 patients in the IMT group and 48 (35%) of 137 patients in the control group (OR: 0.52; 95% CI: 0.30-0.92). Pneumonia occurred in 9 (6.5%) of 139 patients in the IMT group and in 22 (16.1%) of 137 patients in the usual care group (OR: 0.40; 95% CI: 0.19-0.84). Mean duration of postoperative hospitalization was 7 (range 5-41) days in the IMT group versus 8 (range 6-70) days in the usual care group (Mann-Whitney test; Z: -2.42; p = 0.015). CONCLUSION: Preoperative physiotherapy, including IMT, statistically significantly reduced the incidence ofPPCs and the duration ofpostoperative hospitalization in patients at high risk of developing a pulmonary complication on undergoing CABG.

Exercise prescription for patients with a Fontan circulation: current evidence and future directions.

It is well documented that children with a Fontan circulation have a reduced exercise capacity. One of the modalities to improve exercise capacity might be exercise training. We performed a systematic literature review on the effects of exercise training in patients with a Fontan circulation. Six published studies were included that reported on the effects of exercise training in 40 patients. All studies had a small sample size and/or did not include a control group.Based on the six published studies we can conclude that children who have undergone a Fontan operation and who are in a stable haemodynamic condition can safely participate in an exercise training programme and that exercise training results in an improved exercise capacity. However, more research is needed to establish the optimal exercise mode, dose-response relation, and the effects of exercise training on cardiac function, peripheral muscle function, physical activity, and health-related quality of life. (Neth Heart J 2007;15:142-7.).

Epilepsy surgery does not harm motor performance of children and adolescents.

The impact of epilepsy surgery on motor performance, activities of daily life (ADL) and caregiver assistance was assessed in 37 children (age range 0.1-15.4 years) with pharmacologically untreatable epilepsy, 17 of whom were also diagnosed as having spasticity of cerebral origin. All patients underwent epilepsy surgery between 1996 and 2001 at the Wilhelmina University Children's Hospital and were assessed using a standard protocol at fixed intervals: before surgery and 6 months, 1 year and 2 years after surgery. The type of surgery was hemispherectomy (n = 14) and temporal (n = 14), frontal (n = 4), parietal (n = 2) and central (n = 2) resection. One child underwent callosotomy. Engel's classification was used to determine seizure outcome. Impairments were measured in terms of muscle strength, range of motion and muscle tone. Motor performance of infants and children without spasticity was measured using the Movement Assessment Battery for Children (M-ABC). The Gross Motor Function Measure (GMFM-88) was used in children with spasticity, the severity of motor disability in this group being determined by means of the Gross Motor Function Classification System (GMFCS). Daily activities and caregiver's assistance were measured in all children using the Pediatric Evaluation of Disability Inventory (PEDI). Twenty-four months after surgery 74% of the children could be classified as Engel class 1, indicating a significant seizure reduction. Impairments revealed some decrease in muscle strength and range of motion in the group with spasticity. Scores improved statistically significantly at group level on M-ABC and GMFM (P < 0.05). Improvement in activities of daily life and caregiver's assistance could not be measured in children without spasticity because of the ceiling effect of the PEDI, but children with spasticity improved significantly with respect to these parameters (PEDI) (P < 0.05). Hence, epilepsy surgery does not harm motor performance in children with or without spasticity.

Is physical fitness decreased in survivors of childhood leukemia? A systematic review.

The aim of this review is to determine whether physical fitness, assessed by peak oxygen uptake (VO(2peak)) 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 VO(2peak) (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 VO(2peak) 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.

Thoracic excursion measurement in children with cystic fibrosis.

BACKGROUND: Measurement of thoracic excursion (TEM) during maximal ventilation is part of the physiotherapy assessment in children with cystic fibrosis (CF). OBJECTIVES: The purpose of our study was to examine the reliability of TEM and its relation with pulmonary function. METHODS: Thoracic excursions were measured using a measuring tape. Thirty children participated in an intra-observer and inter-observer reliability study. Reliability was determined by calculating the typical error in repeated measurement, limits of agreement and correlation coefficients. Cross-sectional data from the annual check-ups were used to measure the relation between TEM and pulmonary function. RESULTS: In the intra-observer reliability study the typical error was 0.31 cm, the limits of agreement were +/-0.86 cm. Pearson's r and ICC were 0.96 and 0.95, respectively. In the inter-observer reliability study these values were 0.56 cm, +/-1.55 cm, 0.85 and 0.85, respectively. TEM correlated significantly with height (0.31, P<0.001), FVC (0.44, P<0.001), FEV1 (0.41, P<0.001) and TLC (0.19, P<0.05), and there was a significant inverse correlation with RV%TLC (-0.45, P<0.001). CONCLUSIONS: TEM is a reliable assessment tool. Thoracic excursion is significantly, although moderately correlated with pulmonary function.

Functional consequences of hemispherectomy.

Using the International Classification of Functioning Disability and Health (ICF) (WHO, 2001), impairments, activities and social participation are reported in 12 children (mean age at surgery 5.9 years) who were investigated before and three times over a 2-year period after hemispherectomy. Impairments were assessed (i) in terms of seizure frequency (Engel classification) and seizure severity (HASS) and (ii) with respect to muscle strength (MRC), range of motion (JAM score) and muscle tone (modified Ashworth scale). Activities were assessed in terms of gross motor functioning (GMFM) and self-care, mobility and social function (PEDI). Participation was assessed in terms of epilepsy-related restrictions and quantified by means of the Hague Restrictions in Childhood Epilepsy Scale (HARCES). Nine out of 12 children could be classified as free of seizures (Engel class I), and in the remaining three seizure frequency was Engel class III. HASS scores showed maximum improvement in 10 out of 12 children and near-maximum improvement in the two remaining children. Muscle strength and muscle tone on the side of the body contralateral to the hemispherectomy, which were already decreased preoperatively, decreased even further in the first 6 months after surgery, but returned to the presurgical baseline thereafter, except for the distal part of the arm. Range of motion was abnormal prior to operation and remained so after operation. Mean GMFM increase was 20% after 2 years (95% confidence interval 10-33); all five dimensions improved statistically significantly (P < 0.05). Mean PEDI increase was more than 20 scale points (95% confidence interval 10-35); again, all domains improved significantly (P < 0.05). In nearly all children, HARCES scores had normalized 2 years after surgery. In conclusion, decrease of seizure frequency and severity widens the scope of motor and social functioning, which overrides the effects of remaining motor impairments.

[Systematic review of the effects of therapy in infants with the KISS-syndrome (kinetic imbalance due to suboccipital strain)]. / Systematisch literatuuronderzoek naar de effecten van behandeling bij zuigelingen met 'kopgewrichteninvloed bij storingen in de symmetrie' ('KISS-syndroom').

OBJECTIVE: To establish the effects of manual therapy, chiropractic, or osteopathic treatment of the KISS-syndrome (kinetic imbalance due to suboccipital strain) in infants with positional preference, plagiocephaly, and colic. DESIGN: Systematic review of the literature. METHOD: PubMed, Embase and the Cochrane Library were searched for articles on the effects of manual therapy, chiropractic and osteopathy on the KISS-syndrome. Experts in the field of manual medicine and osteopathy were asked to provide relevant articles. The bibliography in a textbook of manual therapy for children was hand-searched for additional references to the KISS-syndrome. RESULTS: No clinical trials were found that evaluated the effects of manual therapy or osteopathy on either the KISS-syndrome or its symptoms. Pooled analysis of two randomised clinical trials on the effects of chiropractic in infantile colic showed no statistically significant difference between active and control treatments. In addition, we found that 22% of infants showed short episodes of apnoea during manual therapy of the spine, and that one case has been described in which such apnoea resulted in death. CONCLUSION: Given the absence of evidence of beneficial effects of spinal manipulation in infants and in view of its potential risks, manual therapy, chiropractic and osteopathy should not be used in infants with the KISS-syndrome, except within the context of randomised double-blind controlled trials.