J. van der Net

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

Aerobic and anaerobic exercise capacity in adolescents with juvenile idiopathic arthritis

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

Relationship between functional ability and physical fitness in juvenile idiopathic arthritis patients

Objective: To determine the relationship between aerobic and anaerobic physical fitness and functional ability in children with juvenile idiopathic arthritis (JIA). Methods: Eighteen children with JIA (age 7 to 14 yr., 3 male/15 female) performed a maximal aerobic exercise test and a Wingate anaerobic exercise test. Functional ability was concurrently assessed using the Childhood Health Assessment Questionnaire (CHAQ). Results: A low relationship between aerobic fitness and functional ability was found (r=0.0 to 0.4, p>0.05, except for eating r=0.46, p<0.05). The correlations between anaerobic physical fitness and functional ability in JIA patients were strong (r=0.5 to 0.75, p<0.05). This indicated a good relationship between anaerobic fitness and functional ability. Conclusion: The strong association between anaerobic physical fitness and functional ability showed the importance of anaerobic physical fitness for children with JIA.

Recent developments in clinimetric instruments

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

Validation of the Actiheart activity monitor for measurement of activity energy expenditure in children and adolescents with chronic disease

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

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

Summary.  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.

Is Static Hyperinflation a Limiting Factor During Exercise in Adolescents With Cystic Fibrosis

Increased work of breathing is considered to be a limiting factor in patients with cystic fibrosis (CF) performing aerobic exercise. We hypothesized that adolescents with CF and with static hyperinflation are more prone to a ventilatorily limited exercise capacity than non‐static hyperinflated adolescents with CF.

Using routine Haemophilia Joint Health Score for international comparisons of haemophilia outcome: standardization is needed

Haemophilia Joint Health Score (HJHS) is the most sensitive validated score for physical examination of joint health in haemophilia. HJHS performed at regular intervals can be used for clinical monitoring as well as for comparative outcomes research.

Protected by nature? Effects of strenuous physical exercise on FVIII activity in moderate and mild haemophilia A patients: a pilot study

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