Paul J M Helders

Physical activity and health related physical fitness in children with juvenile idiopathic arthritis

Objective: To obtain insight into the interaction between daily physical activity and components of health related physical fitness in children with juvenile idiopathic arthritis. Methods: Forty five patients (10 male/35 female; mean (SD) age 8.9 (2.2) years) participated in the study. Body mass, height, skinfold thickness, number of swollen joints, and joint range of motion were determined. The maximal oxygen consumption (Vo2peak) was assessed during a graded maximal bicycle exercise test. Daily physical activity levels were measured with a Caltrac activity monitor and a parental physical activity rating (PAL) on a five point Likert scale. Results: Partial correlation coefficients (to control for age) between physical activity and indices of health related physical fitness showed significant relationships between Caltrac motion counts and absolute Vo2peak (r=0.31) and relative Vo2peak (r=0.34), but not with the indices of body composition. There was also a significant correlation between PAL and relative Vo2peak (r=0.33). Conclusions: Physical activity was significantly related to cardiorespiratory fitness but not to body composition in children with juvenile idiopathic arthritis. A longitudinal follow up should show whether an active lifestyle protects for loss of aerobic fitness in this patient group.

Osteogenesis imperfecta in childhood: treatment strategies.

Osteogenesis imperfecta (OI) is a skeletal disorder of remarkable clinical variability characterized by bone fragility, osteopenia, variable degrees of short stature, and progressive skeletal deformities. Additional clinical manifestations such as blue sclerae, dentinogenesis imperfecta, joint laxity, and maturity onset deafness are described in the literature. OI occurs in about 1 in 20,000 births and is caused by quantitative and qualitative defects in the synthesis of collagen I. Depending on the severity of the disease, a large impact on motor development, range of joint motion, muscle strength, and functional ability may occur. Treatment strategies should primarily focus on the improvement of functional ability and the adoption of compensatory strategies, rather than merely improving range of joint motion and muscle strength. Surgical treatment of the extremities may be indicated to stabilize the long bones to optimize functional ability and walking capacity. Surgical treatment of the spine may be indicated in patients with progressive spinal deformity and in those with symptomatic basilar impression.

Health‐related Quality of Life and Self‐perceived Competence of Children Assessed before and up to Two Years after Epilepsy Surgery

Summary:u2002 Purpose: To measure outcome of epilepsy surgery in terms of health‐related quality of life (HrQoL) and self‐perceived competence of children and adolescents.

Relation between physical fitness and gross motor capacity in children and adolescents with cerebral palsy

Aimu2002 To examine the relation between physical fitness and gross motor capacity in children with cerebral palsy (CP) who were classified in Gross Motor Function Classification System levels I or II.

Osteogenesis Imperfecta in Childhood: Impairment and Disability A Follow-Up Study

OBJECTIVESnTo evaluate differences over time (mean follow-up, 14 months) on impairment parameters (range of joint motion and muscle strength), functional limitation parameters (functional ability), and disability parameters (caregiver assistance in achieving functional skills) in osteogenesis imperfecta (OI), related to the different types of the disease.nnnDESIGNnA prospective, descriptive study.nnnMATERIALS AND METHODSnFifty-four children with OI and their parents participated at the start of the study. At the end, 44 children participated in the assessment of functional skills and 42 of them participated in clinical assessment (OI type I, n = 19; OI type III, n = 13; OI type IV, n = 10). Range of joint motion was measured by means of goniometry. Generalized hypermobility was scored according to Bulbena. Manual muscle strength was scored by means of the MRC grading system. The level of ambulation was scored according to Bleck, and functional skills and caregiver assistance were scored with the Pediatric Evaluation of Disability Inventory.nnnRESULTSnThe different types of OI have impact on impairment, functional limitation, and disability. Almost all impairment parameters did not change significantly over time, whereas some disability parameters seemed to improve significantly.nnnCONCLUSIONSnImpairment parameters in OI are presumably not always preconditions for functional limitation and disability. A 1-year follow-up revealed no significant changes in impairment parameters, whereas some disability parameters improved. Treatment strategies in OI should, therefore, focus primarily on improving functional ability, with respect to the natural course of the disease, and not only on impairment parameters.

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

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

No deterioration in epilepsy and motor function in children with medically intractable epilepsy ineligible for surgery.

The aim of this study was to examine whether severity of epilepsy, motor functioning, and epilepsy‐related restrictions change in children with medically intractable epilepsy who are ineligible for epilepsy surgery. The study was a prospective, longitudinal, 2‐year follow‐up of 28 children (14 females, 14 males). Their median age was 6 years 1 month (range 7mo–15y 4mo). Seizure types were: complex partial seizures (n=16), secondary generalized seizures (n=7), simple partial seizures (n=2), and mixed seizures (n=3). Severity of seizures, motor impairments, motor development, activities of daily life, and epilepsy‐related restrictions were rated at baseline and 6, 12, and 24 months thereafter. Seizure severity did not change significantly, nor did muscle strength, range of motion, or muscle tone. Motor retardation was ubiquitous but did not increase in 20 children without spasticity. Motor function of eight children with spasticity improved (Gross Motor Function Measure: baseline 70.5 [SD 35.5]; 24 months later 81.6 [SD 29.6], p < 0.05) but remained below reference values in four children. In the entire group, functional skills increased and caregiver assistance lessened. Restrictions did not change significantly. We conclude that during a 2‐year follow‐up period, in children with medically intractable epilepsy who do not have surgical intervention, seizure severity does not deteriorate, motor impairments do not increase, motor development does not deflect negatively, and activities of daily living and restrictions do not worsen.

Destructive synovitis in contralateral perthes; disease: A report of 2 cases

2 boys had unilateral Perthes disease at the age of 5 years. After 6 and 7 years, respectively, both patients developed contralateral femoral head necrosis with rapid destruction leading to ankylosis of the hip. Histology of the synovium showed nonspecific synovitis. Both patients fulfilled criteria for oligo-articular juvenile chronic arthritis (JCA). The association with Perthes disease suggests a common etiology.

Motor Performance after Bottom-shuffling in Early Childhood

Summary Objective A group of school-age children who were formerly idiopathic bottom-shufflers were investigated to look at their motor skills and the possible influence of physiotherapy in early infancy on later motor performance. Methods In a cross-sectional study, 20 subjects aged between four and eight years and who had been idiopathic bottom shufflers, were tested with the Movement Assessment Battery for Children (M-ABC). Results No statistically significant differences were found in the scores of children who shuffled in their first two years of life compared to the reference group of the M-ABC. In addition, no statistically significant differences were found between children who received physiotherapy and those who did not. Conclusion Children who shuffled in sitting position in early infancy showed normal motor performance in childhood, whether they received physiotherapy or not.

456 Change in Cerebral Palsy Incidence and Severity Among Children Born Preterm in 1990-2005: A Hospital-Based Cohort Study

456 Change in Cerebral Palsy Incidence and Severity Among Children Born Preterm in 1990-2005: A Hospital-Based Cohort Study