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What is the central question of this study? Do intrinsic abnormalities in oxygenation and/or muscle oxidative metabolism contribute to exercise intolerance in adolescents with mild cystic fibrosis? What is the main finding and its importance? This study found no evidence that in adolescents with mild cystic fibrosis in a stable clinical state intrinsic abnormalities in skeletal muscle oxidative metabolism seem to play a clinical significant role. Based on these results, we concluded that there is no metabolic constraint to benefit from exercise training. Patients with cystic fibrosis (CF) are reported to have limited exercise capacity. There is no consensus about a possible abnormality in skeletal muscle oxidative metabolism in CF. Our aim was to test the hypothesis that abnormalities in oxygenation and/or muscle oxidative metabolism contribute to exercise intolerance in adolescents with mild CF. Ten adolescents with CF (12-18 years of age; forced expiratory volume in 1 s >80% of predicted; and resting oxygen saturation >94%) and 10 healthy age-matched control (HC) subjects were tested with supine cycle ergometry using near-infrared spectroscopy and (31)P magnetic resonance spectroscopy to study skeletal muscle oxygenation and oxidative metabolism during rest, exercise and recovery. No statistically significant (P > 0.1) differences in peak workload and peak oxygen uptake per kilogram lean body mass were found between CF and HC subjects. No differences were found between CF and HC subjects in bulk changes of quadriceps phosphocreatine (P = 0.550) and inorganic phosphate (P = 0.896) content and pH (P = 0.512) during symptom-limited exercise. Furthermore, we found statistically identical kinetics for phosphocreatine resynthesis during recovery for CF and HC subjects (P = 0.53). No statistically significant difference in peak exercise arbitrary units for total haemoglobin content was found between CF and HC subjects (P = 0.66). The results of this study provide evidence that in patients with mild CF and a stable clinical status (without signs of systemic inflammation and/or chronic Pseudomonas aeruginosa colonization), no intrinsic metabolic constraints and/or abnormalities in oxygenation and/or muscle oxidative metabolism contribute to exercise intolerance.

An idiopathic asymmetry in posture of the head is recognized as a risk factor to develop a deformational plagiocephaly (DP). In our neonatal follow-up clinic, an IA is often observed in infants born preterm at term-equivalent age (TEA).To explore (1) the prevalence of an idiopathic asymmetry in 192 infants (gestational age ≤32.0 weeks) at TEA and 6 months corrected age (CA), (2) whether demographical, perinatal, and medical factors were predictors of the asymmetry, and (3) differences in motor maturation between infants with and without asymmetry.In a retrospective study, frequencies of idiopathic asymmetry and DP, putative predictors, and Alberta Infant Motor Scale scores at 6 months CA were abstracted and analyzed with Chi(2), Mann-Whitney, logistic regression and T-test.The prevalence rate of a positional preference of the head at TEA was 44.8% (n=86), 10.4% (20/192) had a DP at TEA and 13% (25/192) at 6 months CA. Positional preference, multiple birth and male gender predicted the presence of DP (p.05, odds ratio 3.0, 3.2, and 3.1 respectively). Gross motor maturity at 6 months CA was less developed in infants with a positional preference at TEA compared to preterm norms (p=0.01).The high prevalence of a positional preference in infants born preterm at term equivalent age requires extra alertness to prevent the development of a deformational plagiocephaly, especially in boys and twins. Although, considering the lower prevalence of plagiocephaly at 6 months CA, therapists should be aware of over treating these infants.

Aim To explore whether active head lifting from supine (AHLS) in early infancy is associated with cognitive outcome in the second year of life. Method The presence of AHLS was always recorded in the notes of infants admitted to our tertiary neonatal intensive care unit. Random sampling was used to pair infants with AHLS with two comparison infants without AHLS whose sex, gestational age, birth year (1993–2009), time of assessment, and developmental test (Griffiths Mental Development Scales, Mental Scale of the Bayley Scales of Infant Development-II, or cognitive subtest of the Bayley Scales of Infant and Toddler Development-III) were comparable. Brain injury identified from neonatal cranial ultrasound scans was classified as no – mild or moderate – severe. Z-scores of cognitive test outcomes were calculated for multivariable analysis. Results Eighty-seven preterm (34 males, 53 females) and 40 term (17 males, 23 females) infants with AHLS were identified. AHLS was documented at a mean (corrected) age of 7.0 (SD 1.7) and 8.1 (SD 2.2) months respectively. The cognitive assessments were performed at a mean corrected age of 15.7 (SD 1.7) and 23.9 (SD 1.6) months in preterm infants, and 19.1 (SD 2.3) months in term infants. The mean cognitive outcome of preterm and term infants with AHLS was lower than that of infants without AHLS (p=0.002 and p=0.004 respectively). This remained after excluding infants with cerebral palsy with matching comparison infants (p=0.001 in preterm and p=0.001 in term infants). The mean difference was highest (1.35SD) between term male infants and comparison infants (p=0.001). Interpretation AHLS is associated with a less favourable cognitive outcome in the second year of life in preterm as well as in term-born infants than in comparison infants.

To summarize and discuss current evidence and understanding of clinical pediatric exercise physiology focusing on the work the research group at Utrecht and others have performed in the last decade in a variety of chronic childhood conditions as a continuation of the legacy of Dr Bar-Or.The report discusses current research findings on the cardiopulmonary exercise performance of children (and adolescents) with juvenile idiopathic arthritis, osteogenesis imperfecta, achondroplasia, hemophilia, cerebral palsy, spina bifida, cystic fibrosis, and childhood cancer. Exercise recommendations and contraindications are provided for each condition. Implications for clinical practice and future research in this area are discussed for each of the chronic conditions presented.The authors provide a basic framework for developing an individual and/or disease-specific training program, introduce the physical activity pyramid, and recommend a core set of clinical measures to be used in clinical research.

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 (age6 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.

The objective of this study was to investigate the characteristics of the submaximal Oxygen Uptake Efficiency Slope (OUES) in a healthy pediatric population. Bicycle ergometry exercise tests with gas-analyses were performed in 46 healthy children aged 7–17 years. Maximal OUES, submaximal OUES, V̇O2peak, VEpeak, and ventilatory threshold (VT) were determined. The submaximal OUES correlated highly with V̇O2peak, VEpeak, and VT. Strong correlations were found with basic anthropometric variables. The submaximal OUES could provide an objective, independent measure of cardiorespiratory function in children, reflecting efficiency of ventilation. We recommend expressing OUES values relative to Body Surface Area (BSA) or Fat Free Mass (FFM).

Objective To examine whether peak work rate (Wpeak) can predict peak oxygen uptake (VO2peak) in children with juvenile idiopathic arthritis (JIA). Methods Ninety-one patients with JIA with a mean ± SD age of 11.4 ± 2.9 years underwent a cardiopulmonary exercise test in which VO2peak and Wpeak were determined. A multivariate regression model was used to formulate a regression equation to predict VO2peak using Wpeak and anthropometric and demographic details. This regression equation was subsequently cross-validated using an unrelated data set from children with JIA (n = 17). Results The following linear regression equation to predict VO2peak was established: VO2peak (liters/minute) = 0.308 + 0.146 × sex (0 = female, 1 = male) + 0.005 × weight (kg) + 0.008 × Wpeak (W) (R2 = 0.91, standard error of the estimate = 0.18 liter/minute). Using this equation, the predicted VO2peak was strongly related to the measured VO2peak (r = 0.96, P < 0.0001). Bland and Altman analysis revealed a mean difference of 0.01 liter/minute and limits of agreement between −0.35 and 0.35 liter/minute. Conclusion This study suggests that Wpeak is a strong predictor of VO2peak in children with JIA and may be used as a surrogate measure of VO2peak in situations where it is not possible to formally assess VO2peak.

Study Design. Cross-cultural adaptation of an outcome questionnaire. Objective. The aim of the study was to cross-culturally adapt the Neck Bournemouth Questionnaire (NBQ) for the Dutch language in a population of people with subacute and chronic whiplash associated disorders (WAD), and to assess its psychometric qualities. SUMMARY OF BACKGROUND DATA.: The NBQ covers the salient dimensions of the biopsychosocial model of pain, and has been shown to be reliable, valid, and responsive to clinically significant change in patients with non specific neck pain. However, no Dutch validated version was available for patients with WAD at the time our study was initiated. Methods. The English version of the NBQ was translated into Dutch (NBQ-NL) and back-translated according to established guidelines. The internal consistency (with help of Cronbach's α), construct validity, and convergent validity were estimated in a different group of 92 patients. Agreement and correlation between the NBQ-NL scores and counterpart questionnaires (SF-36, Neck Disability Index, Hospital Anxiety and Depression Scale, the General Perceived Self-Efficacy and a numerical rating scale for perceived pain) were investigated, using Bland and Altman method and Spearman rank correlation coefficient. Subsequently, 34 patients with subacute WAD completed the questionnaire twice over 1 to 3 weeks, to assess its test-retest reliability. Results. Ninety-two patients, with subacute and chronic WAD, completed the study. Their mean age was 41 years (SD = 11). There were relative high mean scores on the Neck Disability Index (25.5; SD = 8.2), and the NBQ (43.9; SD = 14.8). The NBQ-NL was granted face-validity. Spearman rank correlation coefficient was 0.51 to 0.82 (except for General Perceived Self-Efficacy (0.21). The limits of agreement of normalized scores were relative variable, from small to wide. Cronbach's α (internal consistency) for the NBQ-NL whole scale was 0.87. The Intraclass correlation coefficient for the test-retest reliability was excellent (0.92) and the SEM was relatively low (3.7). Conclusion. Overall, the rank correlation level in general was good, whereas the agreement between questionnaires outcome was variable, most of which seems to be attributable to absolute scale differences. The NBQ-NL is a useable patient-orientated tool for assessing disability in clinical studies and clinical diagnosis in Dutch speaking patients with WAD. © 2009, Lippincott Williams & Wilkins.

BackgroundEarlier studies have demonstrated low peak oxygen uptake (V̇o2peak) in children with spina bifida. Low peak heart rate and low peak respiratory exchange ratio in these studies raised questions regarding the true maximal character of V̇o2peak values obtained with treadmill testing.ObjectiveThe aim of this study was to determine whether the V̇o2peak measured during an incremental treadmill test is a true reflection of the maximum oxygen uptake (V̇o2max) in children who have spina bifida and are ambulatory.DesignA cross-sectional design was used for this study.MethodsTwenty children who had spina bifida and were ambulatory participated. The V̇o2peak was measured during a graded treadmill exercise test. The validity of V̇o2peak measurements was evaluated by use of previously described guidelines for maximum exercise testing in children who are healthy, as well as differences between V̇o2peak and V̇o2 during a supramaximal protocol (V̇o2supramaximal).ResultsThe average values for V̇o2peak and normalized V̇o2peak were, respectively, 1.23 L/min (SD=0.6) and 34.1 mL/kg/min (SD=8.3). Fifteen children met at least 2 of the 3 previously described criteria; one child failed to meet any criteria. Although there were no significant differences between V̇o2peak and V̇o2supramaximal, 5 children did show improvement during supramaximal testing.LimitationsThese results apply to children who have spina bifida and are at least community ambulatory.ConclusionsThe V̇o2peak measured during an incremental treadmill test seems to reflect the true V̇o2max in children who have spina bifida and are ambulatory, validating the use of a treadmill test for these children. When confirmation of maximal effort is needed, the addition of supramaximal testing of children with disability is an easy and well-tolerated method.

Objective To examine the anaerobic-to-aerobic power ratio in children with juvenile idiopathic arthritis (JIA) compared with healthy peers. Methods Sixty-two patients with JIA (mean ± SD age 11.9 ± 2.1 years, range 7.2–15.9 years) with varying severity of disease and 50 healthy children (mean ± SD age 12.1 ± 2.1, range 8.4–15.9 years) participated in this study. Anaerobic power was measured using the Wingate Anaerobic Exercise Test. Aerobic power was measured using a cardiopulmonary exercise test. The power ratio was calculated as the ratio between the anaerobic mechanical power and aerobic mechanical power in watts. Results Mean ± SD anaerobic-to-aerobic power ratio and peak anaerobic-to-aerobic ratios in children with JIA were 1.98 ± 0.51 and 3.28 ± 1.15, respectively. Compared with healthy children these differences were not statistically significant (P = 0.52 and P = 0.99, respectively). The differences in these ratios were not statistically significant when corrected for age, height, and body mass. Statistical analyses showed no significant difference between disease-onset types of JIA for mean anaerobic-to-aerobic power ratio and peak anaerobic-to-aerobic ratio, respectively. Furthermore, there was no significant difference in the development of the power ratios between children with JIA and healthy controls, or between girls and boys. Conclusion This cross-sectional study suggests that the development of the anaerobic-to-aerobic power ratio is not statistically different in children with JIA compared with healthy peers. Exercise training of the anaerobic capacity through interval training, along with aerobic exercise training, seems warranted in the exercise therapy programs of children with JIA.

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

Objective: The aim of this study was to develop a 12-week exercise training program (comprising aerobic and strength exercises), and to study the feasibility and efficacy of this exercise program in children who survived acute lymphoblastic leukemia. Sample and methods: A 12-week exercise program was developed and tested for feasibility in nine children who survived cancer. Results: From the 16 eligible children for the intervention, 9 participated, while 4 were able to complete the entire program. Feasibility of the program was scored by five children, two of them reported the program as being too demanding. The participating physiotherapists were satisfied with training methodology and training progress. The efficacy of the program on muscle strength, exercise capacity, functional mobility and fatigue showed no significant differences between pre and post training. Conclusion: In designing a community-based exercise training program, not only the stage of the disease needs to be considered, but more so the age of the children, the variety of exercises, the location of implementation and even more importantly the views and motivation of the parents concerning the execution of an exercise training program. A careful balance between these parameters could lead to a greater adherence and by that, to a better outcome of these programs. Copyright © 2009 John Wiley & Sons, Ltd.

This cross-sectional study investigates deficits and associations in muscle strength, 6-minute walking distance (6MWD), aerobic capacity (VO(2peak)), and physical activity (PA) in independent ambulatory children with lumbosacral spina bifida.Twenty-tree children participated (13 boys, 10 girls). Mean age (SD): 10.4 (+/-3.1) years. Muscle strength (manual muscle testing and hand-held dynamometry), 6MWD, VO(2peak) (maximal exercise test on a treadmill), and PA (quantity and energy expenditure [EE]), were measured and compared with aged-matched reference values.Strength of upper and lower extremity muscles, and VO(2peak) were significantly lower compared to reference values. Mean Z-scores ranged from -1.2 to -2.9 for muscle strength, and from -1.7 to -4.1 for VO2peak. EE ranged from 73 - 84% of predicted EE. 6MWD was significantly associated with muscle strength of hip abductors and foot dorsal flexors. VO(2peak) was significantly associated with strength of hip flexors, hip abductors, knee extensors, foot dorsal flexors, and calf muscles.These children have significantly reduced muscle strength, 6MWD, VO(2peak) and lower levels of PA, compared to reference values. VO(2peak) and 6MWD were significantly associated with muscle strength, especially with hip abductor and ankle muscles. Therefore, even in independent ambulating children training on endurance and muscle strength seems indicated.

Schmitt MA, van Meeteren NL, de Wijer A, van Genderen FR, van der Graaf Y, Helders PJ: Patients with chronic whiplash-associated disorders: Relationship between clinical and psychological factors and functional health status. Am J Phys Med Rehabil 2009;88:231-238. Objectives: To examine the relative contribution of cervical impairments and psychosocial factors to perceived disability among people with chronic whiplash-associated disorders. Design: A total of 86 patients with chronic whiplash-associated disorders participated in this observational, cross-sectional study. All patients were presented to outpatient physical therapy clinics. All patients completed the neck disability index. Depression, anxiety, and catastrophizing were measured with the Hospital Anxiety and Depression scale and the pain coping and cognition list, respectively. Cervical function was assessed by measuring the active range of motion. Stepwise and hierarchical regression analysis was used to estimate the contribution of cervical impairment and psychosocial functions to the variance in neck disability. Results: Depressive symptomatology and catastrophizing explained 61% of the variance in neck disability index scores. Catastrophizing explained 57% of the variance in neck disability index scores and 15% of the variance in the sum scores of active cervical rotations. Conclusions: Catastrophizing explained the variance in both perceived neck disability and, to a lesser extent, active range of cervical motion, which suggests that pain-related catastrophizing plays an important role in the physical complaints of patients with chronic whiplash-associated disorders when referred to a physical therapist. Copyright © 2009 by Lippincott Williams & Wilkins.

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.

To evaluate the effects of an 8-month training program with standardized exercises on aerobic and anaerobic capacity in children and adolescents with cerebral palsy.Pragmatic randomized controlled clinical trial with blinded outcome evaluation between July 2005 and October 2006.Participants were recruited from 4 schools for special education in the Netherlands.A total of 86 children with cerebral palsy (aged 7-18 years) classified at Gross Motor Function Classification System level I or II.Participants were randomly assigned to either the training group (n = 32) or the control group (n = 33). The training group met twice per week for 45 minutes to circuit train in a group format that focused on aerobic and anaerobic exercises.Aerobic capacity was assessed by the 10-m shuttle run test, and anaerobic capacity was assessed by the Muscle Power Sprint Test. Secondary outcome measures included agility, muscle strength, self-competence, gross motor function, participation level, and health-related quality of life.A significant training effect was found for aerobic (P.001) and anaerobic capacity (P = .004). A significant effect was also found for agility (P.001), muscle strength (P.001), and athletic competence (P = .005). The intensity of participation showed a similar effect for formal (P.001), overall (P = .002), physical (P = .005), and skilled-based activities (P.001). On the health-related quality of life measure, a significant improvement was found for the motor (P = .001), autonomy (P = .02), and cognition (P = .04) domains.An exercise training program improves physical fitness, participation level, and quality of life in children with cerebral palsy when added to standard care.isrctn.org Identifier: ISRCTN77274716.

The aim of the current literature study was to perform a literature review of the factors contributing to exercise limitation and physiological response to exercise in patients with Fontan circulation. In patients with Fontan circulation, peak oxygen uptake ranged from about 14.4 to 32.3 ml/min/kg, and showed a slowed acceleration in the kinetics of oxygen uptake at the onset of exercise. Peak heart rate during exercise was decreased to an average of 153 +/- 10 bpm and arterial oxygen saturation was also decreased at peak exercise, with an average of 89.5 +/- 1.94%. Cardiac output was subnormal, owing to reduced stroke volume, heart rate response and affected pulmonary venous return. Ventilatory anaerobic threshold was below normal values. Moreover, the ventilatory equivalent for carbon dioxide was found to be higher. Patients with Fontan circulation possess a unique physiological response to exercise. Although there is a wide range in exercise capacity among patients, all patients have reduced tolerance to exercise. Cardiac, pulmonary, and muscular factors might play a role in reduced exercise capacity and this distinct response to exercise.

Objective To study in children with achondroplasia the response to exercise and muscle strength compared with healthy peers and to describe the relation between exercise capacity, anthropometric factors, and physical activity. Study design Patients (7 boys and 10 girls; mean age, 11.8 ± 3.3 years) with achondroplasia performed a maximal treadmill exercise test. Anthropometric variables and muscle strength were measured and compared with the general population. The level of everyday physical activity was measured by using a diary. Functional ability was measured by using the Activity Scale for Kids. Results The exercise capacity of the children with achondroplasia was significantly reduced compared with reference values. All anthropometrical measurements differed significantly from reference values. There was a decrease in muscle strength in almost all muscle groups. We found a reduced physical activity level and impairments in functional ability. Conclusions Cardiopulmonary exercise capacity and muscle strength in children with achondroplasia was reduced compared with reference values. Children with achondroplasia have a unique response to exercise. Clinicians should take these characteristic differences into account when the exercise capacity of subjects with achondroplasia is being tested.

We investigated reliability, construct validity, and feasibility of two sprint tests for children with cerebral palsy (CP).Twenty-six children with CP participated (7-18 years of age; Gross Motor Function Classification System [GMFCS] level I or II). On different occasions, the 10 x 5-Meter Sprint Test and the Muscle Power Sprint Test were scored by different assessors.Excellent interobserver reliability (intraclass correlation [ICC] = 1.0 and ICCor= 0.97) and test-retest reliability (ICC = 0.97 and ICCor= 0.97) were obtained. Scores differed significantly on both sprint tests for children classified at GMFCS level I and level II. Mean scores for feasibility ranged from 8.8 to 9.2 on a 10-cm visual analog scale (10 = the best).Both exercise tests are reliable and have good feasibility for children and adolescents with CP (GMFCS level I or II). Construct validity is supported for both sprint tests in children classified at GMFCS level I and level II.

Objective: To determine in a pilot study the feasibility and effects of preoperative inspiratory muscle training in patients at high risk of postoperative pulmonary complications who were scheduled for coronary artery bypass graft surgery. Design: Single-blind, randomized controlled pilot study. Setting: University Medical Centre Utrecht, the Netherlands. Subjects: Twenty-six patients at high risk of postoperative pulmonary complications were selected. Intervention: The intervention group ( N = 14) received 2-4 weeks of preoperative inspiratory muscle training on top of the usual care received by the patients in the control group. Main measures: Primary outcome variables of feasibility were the occurrence of adverse events, and patient satisfaction and motivation. Secondary outcome variables were postoperative pulmonary complications and length of hospital stay. Results: The feasibility of inspiratory muscle training was good and no adverse events were observed. Treatment satisfaction and motivation, scored on 10-point scales, were 7.9 (± 0.7) and 8.2 (± 1.0), respectively. Postoperative atelectasis occurred in significantly fewer patients in the intervention group than in the control group (ϰ2DF1 = 3.85; P = 0.05): Length of hospital stay was 7.93 (± 1.94) days in the intervention group and 9.92 (± 5.78) days in the control group ( P = 0.24). Conclusion: Inspiratory muscle training for 2-4 weeks before coronary artery bypass graft surgery was well tolerated by patients at risk of postoperative pulmonary complications and prevented the occurrence of atelectasis in these patients. A larger randomized clinical trial is warranted.

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.

Background and Purpose. The purpose of this study was to examine the reliability and validity of data obtained with 2 newly developed shuttle run tests (SRT-I and SRT-II) to measure aerobic power in children with cerebral palsy (CP) who were classified at level I or II on the Gross Motor Function Classification System (GMFCS). The SRT-I was developed for children at GMFCS level I, and the SRT-II was developed for children at GMFCS level II. Subjects. Twenty-five children and adolescents with CP (10 female, 15 male; mean age=11.9 years, SD=2.9), classified at GMFCS level I (n=14) or level II (n=11), participated in the study. Methods. To assess test-retest reliability of data for the 10-m shuttle run tests, the subjects performed the same test within 2 weeks. To examine validity, the shuttle run tests were compared with a GMFCS level–based treadmill test designed to measure peak oxygen uptake. Results. Statistical analyses revealed test-retest reliability for exercise time (number of levels completed) (intraclass correlation coefficients of .97 for the SRT-I and .99 for the SRT-II) and reliability for peak heart rate attained during the final level (intraclass correlation coefficients of .87 for the SRT-I and .94 for the SRT-II). High correlations were found for the relationship between data for both shuttle run tests and data for the treadmill test (r=.96 for both). Discussion and Conclusion. The results suggest that both 10-m shuttle run tests yield reliable and valid data. Moreover, the shuttle run tests have advantages over a treadmill test for children with CP who are able to walk and run (GMFCS level I or II). [Verschuren O, Takken T, Ketelaar M, et al. Reliability and validity of data for 2 newly developed shuttle run tests in children with cerebral palsy. Phys Ther. 2006;86:1107–1117.]

Deformational plagiocephaly (DP) in newborns and very young children is a common problem in daily practice. The intrarater and interrater reliability of plagio- cephalometry (PCM), a new, non-invasive, inexpensive instrument to assess and quantify the asymmetry of the skull, is evaluated at the outpatient Department of Phys- ical Therapy of the Bernhoven Hospital at Veghel, The Netherlands. Using a thermoplastic material to mould the outline of the infant's skull, a reproduction of the skull shape is performed on paper, allowing for accurate ceph- alometric measurements. Fifty children (aged 0-24 months), with or without positional preference of the head, and with or without DP, were measured three times by two separate, experienced pediatric physical therapists. Intraclass corre- lation coefficients (ICC) regarding the measurements of the drawn lines were all above 0.92 (intrarater reliability) and 0.90 (interrater reliability). The ICCs of the plagio- cephaly indicators ear deviation (ED), antero-sinistra� antero-dextra (ASAD), postero-dextrapostero-sinistra (PDPS) and oblique diameter difference (ODD) were 0.88, 0.57, 0.92 and 0.96, respectively, for the intrarater reliability and 0.90, 0.65, 0.94 and 0.96, respectively, for the interrater reliability. The ICCs of the two indices oblique diameter difference index (ODDI) and cranial proportional index (CPI) were 0.97 and 0.96, respectively, for the intrarater reliability and 0.95 and 0.92, respec- tively, for the interrater reliability. The limits of agreement according to Bland Altman, comprising 95% of the dif- ferences between two measurements (2 sd), were 4.3 mm (ED), 5.9 mm (ASAD), 3.0 mm (PDPS), 3.4 mm (ODD), 2.7% (ODDI) and 4.5% (CPI) for the intrarater reliability, and 3.7 mm (ED), 5.2 mm (ASAD), 2.4 mm (PDPS), 3.3 mm (ODD), 2.9% (ODDI) and 5.8% (CPI) for the interrater reliability. Conclusion: We conclude that PCM is an easy-to-apply, non-invasive and reliable measurement instrument to assess skull asymmetry with good clinical accuracy and low application costs. PCM might serve as an instrument to be used in all levels of care for children with DP, and might provide information concerning the natural course of DP, as well as the assessment of the effects of conservative treatment strategies on DP.

The goal of this systematic literature review was to establish the effects of manual therapy, chiropractic, or osteopathic treatment of the kinetic imbalance due to suboccipital strain (KISS) syndrome in infants with positional preference, plagiocephaly, and colic. We searched PubMed, Embase, and the Cochrane Library for articles on the effects of manual therapy, chiropractic, and osteopathy for the KISS syndrome. In addition, experts in the field of manual medicine and osteopathy were asked to provide relevant articles, and the bibliography in a textbook of manual therapy for children was hand-searched for additional references to the syndrome. This search strategy located no clinical trials that evaluated the effects of manual therapy or osteopathy on either the KISS syndrome or its symptoms. However, pooled analysis of two randomized clinical trials on the effects of chiropractic in infantile colic showed no statistically significant difference between active and control treatments. In addition...

Objective: To investigate determinants of functional independence and study which functional abilities were determinants for ‘health-related quality of life’ in children with myelomeningocele. Design: Cross-sectional study by means of clinical assessment, ‘disability’ measurement and questionnaires. Uniand multivariate logistic regression models were used to investigate factors that were determinants for these outcomes. Results were expressed as odds ratios (OR) and 95% confidence intervals (95% CI). Setting: Outpatient spina bifida clinic at a university hospital. Subjects: One hundred and twenty-two children with myelomeningocele. Mean age 7.9; range 1-18 years. Main measures: Functional independence as measured by the Pediatric Evaluation of Disability Inventory (PEDI), and quality of life as measured by the Spina Bifida Health Related Quality of Life Questionnaire. Results: Lesion level below L3 (OR 0.4, 95% CI 0.1-1.0), mental status of IQ] - 80 (OR 4.2, 95% CI 1.2-14.9), having no contractures in lower extremities (OR 3.4, 95% CI 1.3 -8.8), and having normal strength of knee extensor muscles (OR 4.1, 95% CI 1.4 -11.5) were most strongly associated with independence in self-care. Mental status (OR 16.1, 95% CI 2.8 -93.9), having no contractures in lower extremities (OR 1.5, 95% CI 1.4 -5.3), and normal strength in knee extensors (OR 11.0, 95% CI 1.3-97.0) were the most important determinants for independence in mobility. Concerning functional abilities, being independent with regard to mobility was the most important determinant for ‘health-related quality of life’ (OR 5.3, 95% CI 1.6-17.4). Conclusions: In children with myelomeningocele, good muscle strength, mental ability and being independent in mobility appeared to be much more important for daily life function and quality of life than other medical indicators of the disorder.

The functioning of 12 patients with chronic inflammatory demyelinating polyneuropathy (CIDP) and 18 patients with multifocal motor neuropathy (MMN) was evaluated to obtain health profiles and appropriate clinimetric instruments. Assessment was made in a cross-sectional study by means of a performance-based body function test (hand-held dynamometry), two performance-based activity tests (10-m walk test and Berg balance scale), a self-reported activity test (Canadian Occupational Performance Measure), and a self-reported functioning test (sickness impact profile 68). In both patient groups, CIDP and MMN, specific health profiles were manifest. A clear relationship between body function, activities, and functioning was not found. Therefore, to assess a patient with inflammatory neuropathy, it is recommended to assess body function as well as activities and functioning and to select appropriate clinimetric instruments specific for each type of neuropathy.

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

Summary: Purpose: To measure outcome of epilepsy surgery in terms of health-related quality of life (HrQoL) and self-perceived competence of children and adolescents. Methods: Prospective longitudinal follow-up study of 21 patients (aged 6.2 to 16.8 years). Frequency and severity of seizures and epilepsy-related restrictions, HrQoL, and self-perceived competence were rated before and 6, 12, and 24 months after epilepsy surgery. Data were analysed nonparametrically and using analysis of variance for repeated measures. Results: Group-wise, seizure parameters had almost normalized 6 months after surgery (p < 0.001) and remained so. Two years after surgery, 15 (72%) patients were free of seizures. At the first postsurgical assessment, parents and children evaluated the frequency of activities as improved and that of seizures as diminished (p < 0.05). Parents evaluated their children as having positive emotions more frequently (p < 0.05). Children started to feel better about seizure variables in the second year after surgery. Two years after surgery, children perceived themselves as being socially more competent and having greater self-worth (p = 0.05). In the adolescent group, several aspects of self-perceived competence improved shortly after surgery (p < 0.05), whereas 2 years after surgery, athletic competence and romance had improved (p < 0.05). Conclusions: In children and adolescents, epilepsy surgery sets the stage for improvement in HrQoL and in competence to participate in social and societal domains. Most improvement occurs in the first 6 months after surgery.

In contrast to the adult population, little is known regarding health-related quality of life and exercise tolerance in children with end-stage renal disease (ESRD) undergoing chronic intermittent hemodialysis. We designed a pilot study to investigate whether research into this area is indicated. The aim of this study was to describe the motor skills, exercise tolerance, and health-related quality of life in children with ESRD. The study population consisted of ten hemodialysis patients (aged 7-16 years). In eight children motor proficiency according to Bruininks-Oseretsky was determined. In all ten children a progressive exercise test on a treadmill was performed. The results were compared with an age-matched healthy reference group. Nine children filled in the TNO-AZL Child Quality of Life (TACQOL) scoring list. One child had a markedly reduced fine motor skills capacity; another five children scored less than or equal to-2 SD compared with healthy children in gross motor skills. Seven children showed a diminished VO(2)max (per kilogram body weight); six of these are physically inactive. Four of these seven children did not sustain the maximum workload. The self-assessed physical and mental health of children on dialysis seems comparable to the general population. We found no correlation between exercise performance or motor skills and hemoglobin levels, Kt/V, and time on dialysis. In conclusion, in this study most children had a reduced exercise tolerance and gross motor skills. There was no difference in fine motor skills. Pediatric dialysis patients report a good health-related quality of life.

Engelbert RH, Uiterwall CS, Gerver W-J, van der Net J-J, Pruijs HE, Helders PJ. Osteogenesis imperfecta in childhood: impairment and disability. A prospective study with 4-year follow-up. Arch Phys Med Rehabil 2004;85:772-8. Objectives To study (1) changes in anthropometrics, joint range of motion (ROM), muscle strength, functional ability, caregiver assistance, and level of ambulation in children with osteogenesis imperfecta (OI) and (2) the prediction of clinical characteristics at the level of ambulation at follow-up and the prediction of clinical characteristics on progression or regression at the level of ambulation over time. Design Prospective study with follow-up of 4 years. Setting A children's hospital that serves a nationwide center for treatment and research in children with OI in the Netherlands. Participants At follow-up, 49 children (24 boys, 25 girls; mean age ± standard deviation, 11.3±3.8y; range, 5.2–19.4y) participated. Interventions Not applicable. Main outcome measures Anthropometry, joint ROM, muscle strength, fracture frequency, intramedullary rodding, level of ambulation, functional ability, and caregiver assistance. Results In type I OI, total joint ROM decreased significantly over time, especially in the lower extremities, with a significant decrease in generalized joint hypermobility according to Bulbena (median start, 7.5; interquartile range [IQR], 4–9; median end, 6; IQR, 2–7; P P =.003) and social function ( P =.008) was measured; in type III, a significant increase in self-care ( P =.003), mobility ( P =.004), and social function ( P =.005) was measured, with a significant decrease in parental assistance in self-care ( P =.02) and mobility ( P =.005). In type IV, a significant increase was observed in the self-care ( P =.01) and social function domains ( P =.02). Type of OI (regression coefficient=−1.0; 95% confidence interval [CI], −1.64 to −0.47) and total muscle strength were the only significant predictors for level of ambulation (regression coefficient=.01; 95% CI, .17–.32). Body weight was significantly lower in the group that progressed in level of ambulation ( P =.03), whereas children with a decline in level of ambulation had significantly higher body weight ( P =.05). Conclusions Ours is the first study with a long-term follow-up that provides information concerning the natural course of developmental outcome parameters of OI in childhood. Joint ROM and muscle strength did not change significantly over time, possibly because of the biomechanical skeletal properties of the different OI types. Functional ability improved significantly over time, but, especially in types III and IV, did not reach normative values, possibly because of a plateau phase in functional ability. Knowledge of the natural course of the disease is essential to interpret the results from intervention studies.

Background Children's physical activity patterns are characterized by short-term anaerobic activities. Anaerobic exercise performance in children with cystic fibrosis (CF) has received little attention compared to aerobic performance. This study investigated the effects of anaerobic training in children with CF. Design and methods Twenty patients were randomly assigned to the training group (TG) [11 patients; mean (± SD) age, 13.6 ± 1.3 years; mean FEV 1 , 75.2 ± 20.7% predicted] or the control group (CG) [9 patients; mean age, 14.2 ± 2.1 years; FEV 1 , 82.1 ± 19.1% predicted]. The TG trained 2 days per week for 12 weeks, with each session lasting 30 to 45 min. The training program consisted of anaerobic activities lasting 20 to 30 s. The control subjects were asked not to change their normal daily activities. Body composition, pulmonary function, peripheral muscle force, habitual physical activity, aerobic and anaerobic exercise performance, and quality of life were reevaluated at the end of the training program, and again after a 12-week follow-up period. Results Patients in the TG significantly improved their anaerobic performance, aerobic performance, and quality of life. No significant changes were seen in other parameters, and no improvements were found in CG. After the follow-up period, only anaerobic performance and quality of life in TG were significantly higher compared to pretraining values. Conclusions Anaerobic training has measurable effects on aerobic performance (although not sustained), anaerobic performance, and health-related quality of life in children with CF. Therefore, anaerobic training could be an important component of therapeutic programs for CF patients.

Objective. To describe the clinical features, osseal characteristics, and collagen biochemistry in children who attended our clinic with predominantly generalized hypomobility of the joints, in combination with musculoskeletal complaints or abnormal walking, and no known syndrome or known rheumatic, neurologic, skeletal, metabolic, or connective tissue disorder was present. Methods. Nineteen children who attended the Children’s Hospital of the University Medical Center Utrecht for generalized hypomobility of the joints (mean age: 11.6; standard deviation: 2.7), in combination with musculoskeletal complaints or abnormal walking as primary complaints (symptomatic generalized hypomobility [SGH]), were compared with an age-matched reference group of 284 healthy children with normal mobility of the joints. Anthropometrics, range of joint motion, muscle strength, exercise tolerance, motor development, quantitative ultrasound measurements of bone, and degradation products of collagen in urine were studied. Collagen modifications were determined in skin biopsies of 3 children and in hypertrophic scar tissue of another child, all with SGH. Results. The range of joint motion was significantly decreased in almost all joints of all 19 children and after adjustment for age, gender, body weight, and height, significantly lower than that of the reference group (−108.3 degrees; 95% confidence interval [CI]: −136.9 to −79.8). Quantitative ultrasound measurements as well as urinary pyridinoline cross-link levels were, after adjustment for possible confounders, significantly lower in SGH children (broad-band ultrasound attenuation: −9.6 dB/MHz [95% CI: −17.4 to −1.9]; speed of sound: −25.0 m/s [95% CI: −39.7 to −10.3]; hydroxylysylpyridinoline: −50.1 μmol/mmol [95% CI: −87.6 to −12.6], lysylpyridinoline: −21.3 μmol/mmol [95% CI: −34.0 to −8.6]). An increased amount of pyridinoline cross-links per collagen molecule was observed in skin and hypertrophic scar tissue, in combination with increased amounts of collagen. Conclusion. SGH in children is considered a new clinical entity with specific clinical characteristics and might be related to an increased stiffness of connective tissue as a result of higher amounts of collagen with increased cross-linking.

To evaluate cardiopulmonary function, muscle strength, and cardiopulmonary fitness (VO 2 peak) in patients with osteogenesis imperfecta (OI). In 17 patients with OI type I (mean age 13.3 +/- 3.9 years) cardiopulmonary function was assessed at rest using spirometry, plethysmography, electrocardiography, and echocardiography. Exercise capacity was measured using a maximal exercise test on a bicycle ergometer and an expired gas analysis system. Muscle strength in shoulder abductors, hip flexors, ankle dorsal flexor, and grip strength were measured. All results were compared with reference values. Cardiopulmonary function at rest was within normal ranges, but when it was compared with normal height for age and sex, vital capacities were reduced. Mean absolute and relative VO 2 peak were respectively -1.17 (+/- 0.67) and -1.41 (+/- 1.52) standard deviations lower compared with reference values ( P

Several cross-sectional studies in patients with cystic fibrosis (CF) have shown that nutritional status and lung function are important determinants of peak aerobic capacity (peak oxygen uptake [O(2)peak]). In order to account for individual changes, the aim of this study was to determine the longitudinal relationship of changes in nutritional status, lung function, and O(2)peak in children with CF.Fat-free mass (FFM), lung function, and O(2)peak were assessed in 65 children with CF at baseline (mean +/- SD age, 10.5 +/- 2.9 years; mean FEV(1), 92.6 +/- 20.5%) and again 2 years later. FFM was calculated using skinfold thickness, and O(2)peak was measured using an incremental treadmill test for children12 years old or an incremental cycle ergometry test for childrenor =12 years old. Lung function was measured before the exercise test.Over the 2-year study period, an increase was found for absolute values of FFM (6.1 kg, p0.001), FEV(1) (229 mL, p0.001), and O(2)peak (240 mL, p0.001), while a decrease was found for predicted values of FEV(1) (- 8.9%, p0.001) and O(2)peak (- 4.4%, p0.05). DeltaO(2)peak over the 2-year period best correlated with DeltaFEV(1) (r = 0.619, p0.001) and to a lesser degree with DeltaFFM (r = 0.506, p0.001). Multiple regression analysis demonstrated that DeltaFEV(1) and DeltaFFM explained 47% of the variation of the DeltaO(2)peak over the 2-year period.Our results show that longitudinal changes in O(2)peak are associated with changes in lung function and to a lesser extent with changes in nutritional status in children with CF. Special consideration should be given to exercise training and nutritional intervention, which might improve long-term clinical outcome in children with CF.

In the present study, temperature increase patterns were determined by thermal imaging in a pig cadaver hind limb. The temperature increase patterns were measured with a 0.5-min interval during an insonation using a circular and stationary technique. The treatment time was 5 and 2.5 min, respectively. In all experiments, temperature peaks in the superficial tissue layers were obtained regardless of the technique used. It is concluded that temperature peaks on the inside and frontal of the bone are avoided when using a moving technique. Physical therapists should take into account that the moving technique decreased the overall temperature compared with the stationary technique.

Objectives. Children with generalized hypermobility of the joints and musculoskeletal complaints frequently visit pediatric clinics, but many show no currently known collagen or other possibly related diseases. Whether the symptoms are confined to the musculoskeletal system is unknown. We assessed whether such children have detectable differences in laxity of connective tissue present in organ systems other than joints. We also assessed whether children with generalized joint hypermobility and musculoskeletal complaints have more profound systemic changes in connective tissue of various organ systems as compared with children with generalized joint hypermobility without musculoskeletal complaints. Methods. Anthropometrics, range of joint motion, muscle strength, skin extensibility, blood pressure, quantitative ultrasound measurements of bone, and degradation products of collagen were studied in 15 prepubertal children with generalized joint hypermobility and musculoskeletal complaints and compared with a population-based reference group of 95 nonsymptomatic prepubertal children. Symptomatic hypermobile children were also compared with children of the population-based reference group who had asymptomatic hypermobility of the joints ( n = 16). Results. Children with symptomatic generalized joint hypermobility had significantly higher skin extensibility (5.6 mm/15 kPa, 95% confidence interval [CI]: 4.0–7.1), lower quantitative ultrasound measurements (speed of sound: −26.8 m/s; 95% CI: −41.1 to −12.6) in bone, and lower systolic and diastolic blood pressure (−8.0 mmHg, 95% CI: −13.3 to −2.8; and −6.0 mmHg, 95% CI: −10.0 to −2.2, respectively) as compared with the total reference group. Also, they had significantly lower excretion of urinary hydroxylysylpyridinoline cross-links (mean difference: −51.3 μmol/mmol; 95% CI: −92.2 to −10.4) as well as lysylpyridinoline cross-links (−18.7 μmol/mmol; 95% CI: −36.9 to −0.5). Age, gender, body weight, height, and particularly cross-links excretion did not explain group differences in clinical and bone characteristics. After adjustment for age, gender, body weight, and height, children with symptomatic generalized joint hypermobility ( n = 15) had significantly higher total range of joint motion (117.8 degrees; 95% CI: 77.7–158.0), skin extensibility (3.5 mm/15 kPa; 95% CI: 1.6–5.3), lower quantitative ultrasound measurements in bone (speed of sound: −27.9 m/s; 95% CI: −48.4 to −7.5), borderline lower diastolic blood pressure (−4.9 mmHg; 95% CI: −10.7–0.9), and significantly higher degradation products in urine (hydroxyproline/creatinine: 21.2 μmol/mmol; 95% CI: 2.3–40.1) as compared with asymptomatic hypermobile children of the total reference group ( n = 16). After adjustment for possible confounders, children with generalized joint hypermobility without musculoskeletal complaints had a significantly higher total range of joint motion and more profound skin extensibility, as compared with the reference group ( n = 79). Conclusions. Clinically manifested symptoms in otherwise healthy children with generalized joint hypermobility are accompanied by increases in the laxity of other body tissues. Thus, generalized joint hypermobility with musculoskeletal symptoms does not seem to be restricted to joint tissues. In symptomatic hypermobile children, a more systemic derangement was also present as compared with asymptomatic hypermobile children.

Paediatric rehabilitation as a discipline is rapidly changing, especially during the last decades. In the past, paediatric rehabilitation was characterized by merely adult intervention strategies in a miniaturized form, delivered by a merely adult patients-oriented profession. Theories on childhood development, however, changed, as did the focus of interventions: from impairments to function, from the child itself to family, community and peers. The call for outcome-oriented and evidence-based medicine lastly, changed paediatric rehabilitation into a mature paediatric profession with it's own scientific framework. This is reflected among other things in the increasing number of paediatric measures and instruments specifically geared to the paediatric rehabilitation profession, for example the Gross Motor Function Measure, Paediatric Evaluation of Disability Inventory and Movement ABC. More recently, paediatric exercise physiologists are pointing to the benefits of an active lifestyle and training for patients with chronic diseases and disabilities. Several studies have evaluated the effects of such training programmes and came up with positive results. It shows that paediatric rehabilitation continues to develop as a dynamic profession, having growth, childhood development and childhood activities as it's core business.

Custers JW, van der Net J, Hoijtink H, Wassenberg-Severijnen JE, Vermeer A, Helders PJ. Discriminative validity of the Dutch Pediatric Evaluation of Disability Inventory. Arch Phys Med Rehabil 2002;83:1437-41. Objective: To examine the discriminative validity of the Dutch Pediatric Evaluation of Disability Inventory (PEDI) to differentiate functional status between children with and without disabilities. Design: Cross-sectional study. Setting: A university children's hospital in the Netherlands. Participants: A clinical sample comprising 197 children with disabilities (infantile encephalopathy, n=40; juvenile idiopathic arthritis, n=20; neurometabolic conditions, n=36; neuromuscular disorders, n=9; skeletal disorders, n=28; spina bifida, n=41; traumatic injury, n=23), and 62 children without disabilities. Interventions: Not applicable. Main Outcome Measure: Functional status was measured by using a Dutch version of the PEDI. Results: Discriminant analysis established the sensitivity and specificity of the PEDI. Correct predictions of group membership (disabled vs nondisabled) were found in both children without disabilities (93.5% correctly predicted) and children with disabling conditions (91.6% correctly predicted). Conclusion: The discriminative validity of the Dutch PEDI between children with and without disabilities was excellent. © 2002 by the American Congress of Rehabilitation Medicine and the American Academy of Physical Medicine and Rehabilitation

Hand function in children is very important in the development of skills needed for daily childhood activities; therefore, preserving this function warrants special attention. One of the interventions aiming to preserve functionis splinting of the affected wrist joint. The rationale for the use of splinting is to balance rest and activity, therebypreserving wrist function. However, the evidence-based knowledge to support this rationale is scarce. To determine the validity of “balancing rest and activity of the affected wrists to preserve function,” we reviewed ourown data, data in the literature regarding rheumatic wrist problems in children, and data from a 15-year followup study of rheumatic wrist problems in an adult cohort of our adult rheumatology counterpart within the medicalcenter.

Motor learning is an important factor in the rehabilitation process. We suggest that measuring the motor learning capacity or "motor intelligence" of patients will help rehabilitation professionals to better adapt the intervention to this capacity, leading to a more effective intervention. We explored the use of the concept of motor learning in clinical practice and theory. It turns out that rehabilitation professionals hardly use the concept of motor learning in their interventions, although a lot of highly relevant literature is available on this subject. The development of cognitive intelligence and the intelligence quotient is analysed, because the framework of cognitive intelligence and inimportant tests could help us to operationalize motor intelligence, and, in the long run, to develop a motor intelligence test. With this paper, we want to start a discussion on the subject.

We investigated whether haemophilic children who are on prophylactic therapy differ from their healthy peers in terms of motor performance and disability. Thirty-nine children, aged 4-12 years, with moderate (eight) and severe (31) haemophilia were included. Patients with severe haemophilia received primary prophylactic therapy that was individually tailored. The number of target joints, amount of swelling, range of motion, muscular strength and pain were measured, as well as motor skills and disability. The scores were compared to the normal population. No patients had target joints. Normal range of motion in all joints was seen in 97% (38/39) of the patients. Strength of elbow, knee, and ankle muscles were within the normal ranges. Ninety-five percent (37/39) of the patients had normal motor performance. Although 90% of our patients (35/39) had no disabilities in activities of daily living (ADL), 79% (31/39) of them reported that the disease impacted on their lives. Seventy-two percent (28/39) of the patients had pain, and in 21% (6/28) of them this was mainly caused by injections. Restrictions in sports or gymnastics were seen in 56% (22/39) of the patients. Those who indicated that they experienced pain and those who indicated restrictions in sports had a higher chance of experiencing disease impact compared to those who did not have these limitations. There were no significant differences between patients with moderate and severe haemophilia. In general, Dutch children with moderate or severe haemophilia are comparable with their healthy peers with regard to motor performance and ADL. However, a majority of the patients perceive an impact of their disease associated with pain and restrictions in sports.

Objective To analyze the displacement response of juvenile arthritic wrists during grasp in order to diagnose early ligamental laxity and facilitate early splinting. Methods 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 Hafner. Results 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. Conclusion 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.

Purpose: The authors postulated that physiotherapy as an adjuvant to the surgical treatment of anterior chest wall deformities is only indicated if specific abnormalities can be found that could be corrected by physiotherapy. The purpose of this study is to investigate whether such abnormalities can be found and to evaluate their course during a postoperative period of 18 months. Methods: Twenty-one patients, 16 with pectus excavatum and 5 with pectus carinatum, were evaluated 6 weeks before and 6 weeks, 6 months, and 18 months after surgical correction. Postural impairments, spinal mobility and curvature, muscle strength, and muscle length were evaluated. Results: Preoperatively, poor posture was seen in 10 patients, nonstructural scoliosis in 11, and abdominal muscle weakness in 4 patients. None of the patients had restriction of spinal mobility or shortened pectoral muscles. Six weeks after surgery, poor posture was seen in 9, nonstructural scoliosis in 11, and abdominal muscle weakness in 10 patients. The authors found a higher percentage of recovery for abdominal muscle weakness than for poor posture (90% versus 33%, respectively). Conclusions: The authors found preoperative postural impairments in 52% of their patients, in patients with pectus carinatum as well as in patients with pectus excavatum. In patients without postural impairments, physiotherapy is not necessary, with the exception of postoperative pulmonary care.

This study was performed to achieve more detailed information regarding the age and sequence in the development of motor milestones in the different types of osteogenesis imperfecta (OI). The parents of 98 patients with a diagnosis of OI were sent a questionnaire regarding the age at which patients achieved motor milestones. All patients were attending the outpatient clinic for children with OI at the Wilhelmina Children's Hospital. The motor milestones were classified into static motor milestones and dynamic motor milestones and all data were checked with health care records. The age of development of motor milestones was compared to reference values of the healthy population. The severity of the disease was classified according to Sillence based on clinical, genetic and radiological data. The age of intramedullary rodding of the first nail in the lower and upper extremity and the localisation was noted. A total of 76 parents responded to the 98 questionnaires (78%). In OI type I, a delay exists in achieving motor milestones, comparable to the 95th percentile of the normal population. In type III, the development of all motor milestones was significantly delayed compared to types I and IV with a discrepancy between static and dynamic milestones. In OI type IV, a retardation in motor development developed after the milestone `sitting without support' was achieved. Motor development in types I and IV was not influenced by intramedullary rodding of the lower extremities, since rodding was rarely performed before the milestone `unsupported standing' was achieved. In type III, the influence of intramedullary rodding on the age of achieving motor milestones remains questionable. Conclusion The severity of osteogenesis imperfecta has a large influence on the age and sequence in the development of motor milestones. No influence of intramedullary rodding of the lower extremities on motor development was found in osteogenesis imperfecta types I and IV, whereas the influence in type III remains questionable.

In their pursuit of evidence-based practice, rehabilitation sciences, physical therapy included, seem to be reluctant to use a basic sciences approach. This is quite understandable, but at the same time illogical, as by excluding such an approach progress within the discipline might be hindered. Consequently, we here propose the judicious involvement of rehabilitation professionals in the field of basic sciences.

For many reasons it is preferable to use established health related outcome instruments. The validity of an instrument, however, can be affected when used in another culture or language other than what it was originally developed. In this paper, the outcome on functional status measurement using a preliminary version of the Dutch translated ‘Pediatric Evaluation of Disability Inventory’ (PEDI) was studied involving a sample of 20 non-disabled Dutch children and American peers, to see if a cross-cultural validation procedure is needed before using the instrument in the Netherlands. The Rasch model was used to analyse the Dutch data. Score profiles were not found to be compatible with the score profiles of American children. In particular, ten items were scored differently with strong indications that these were based on inter-cultural differences. Based on our study, it is argued that cross-cultural validation of the PEDI is necessary before using the instrument in the Netherlands.

Exercise training is currently advocated as part of the treatment of patients with cystic fibrosis (CF). However, data are few that document physiologic benefits or changes in patients' perceptions of long-term training programs in children with CF. The aim of this study was to investigate the effects and acceptability of a home cycling program in children with CF. Fourteen patients (9 boys, 5 girls) with CF, mean (SD) age 14.1 (2.0) years, with mild to moderate impairment of lung function (forced expiratory volume in 1 s, mean (SD) 58.3 (16.3)% of predicted) were studied for 1 year. The first half of the study year was used to obtain baseline values at 0 and 6 months. During the second half of the year, a cycle program was carried out 5 times a week, for 20 min each day at a level of work that resulted in a heart rate of 140-160 beats/min. Once a week the cycle program was supervised by a physiotherapist. Measurements were repeated at 12 months. Effects of the exercise program were measured in terms of lung function, nutritional status, growth, muscle strength, exercise performance, perceived competence, and attitude towards the training program. Differences between the changes during the 6-month training period as compared to the 6-month control period were analyzed by multivariate statistics and nonparametric tests. Statistically significant differences (P < 0.05) between the two periods were found with respect to muscle strength of knee extensors and ankle dorsiflexors, and with respect to maximal oxygen consumption per kg body weight as well as per kg fat free mass. All changes were positive. No adverse effects were found. Perceived competence showed significant positive changes in feelings about physical appearance, general self-worth, and Total Perceived Competence Score. Scores concerning perceived acceptability of the program were significantly lower at the end of the training period; however, patients reported that they did want to continue with other sorts of training. We conclude that an exercise training program in the home can produce beneficial effects on oxygen consumption, muscle force, and perceived competence in children with CF. However, acceptability of the program was low, suggesting that long-term adherence would be poor, and hence, other sorts of training need to be identified.

PTJ does not usually publish tributes, as there are so many physical therapists who have given so much to their profession and to rehabilitation science. We make an exception in this case because of the direct impact that Dr Beverly Bishop has had on PTJ 's Editorial Board over the years . Born in Corning, NY, Bishop earned a bachelor of science degree in mathematics from Syracuse University in 1944, a master of science degree in experimental psychology at the University of Rochester in 1946, and a PhD in physiology at the University of Buffalo in 1957. She was a State University of New York (SUNY) Distinguished Teaching Professor at the University of Buffalo and also received the Chancellor's Award for Excellence in Teaching. Her teaching career spanned more than 50 years, during which she taught neurophysiology to almost 40 cohorts of physical therapist students. She also authored more than 150 scholarly articles, many in PTJ 1–19; edited 4 books; and produced seminal monographs and book chapters in the area of neurophysiology. Her research interests included the identification and analysis of motor control in humans and animals. An honorary member of APTA, …