The association between muscle strength and activity limitations in patients with the hypermobility type of Ehlers–Danlos syndrome : the impact of proprioception

Purpose: The patients diagnosed with Ehlers-Danlos Syndrome Hypermobility Type (EDS-HT) are characterized by pain, proprioceptive inacuity, muscle weakness, potentially leading to activity limitations. In EDS-HT, a direct relationship between muscle strength, proprioception and activity limitations has never been studied. The objective of the study was to establish the association between muscle strength and activity limitations and the impact of proprioception on this association in EDS-HT patients. Methods: Twenty-four EDS-HT patients were compared with 24 controls. Activity limitations were quantified by Health Assessment Questionnaire (HAQ), Six-Minute Walk test (6MWT) and 30-s chair-rise test (30CRT). Muscle strength was quantified by handheld dynamometry. Proprioception was quantified by movement detection paradigm. In analyses, the association between muscle strength and activity limitations was controlled for proprioception and confounders. Results: Muscle strength was associated with 30CRT (r = 0.67, p = <0.001), 6MWT (r = 0.58, p = <0.001) and HAQ (r = 0.63, p = <0.001). Proprioception was associated with 30CRT (r = 0.55, p <0.001), 6MWT (r = 0.40, p = <0.05) and HAQ (r = 0.46, p < 0.05). Muscle strength was found to be associated with activity limitations, however, proprioceptive inacuity confounded this association. Conclusions: Muscle strength is associated with activity limitations in EDS-HT patients. Joint proprioception is of influence on this association and should be considered in the development of new treatment strategies for patients with EDS-HT. Implications for rehabilitation : Reducing activity limitations by enhancing muscle strength is frequently applied in the treatment of EDS-HT patients. Although evidence regarding treatment efficacy is scarce, the current paper confirms the rationality that muscle strength is an important factor in the occurrence of activity limitations in EDS-HT patients. Although muscle strength is the most dominant factor that is associated with activity limitations, this association is confounded by proprioception. In contrast to common belief proprioception was not directly associated with activity limitations but confounded this association. Controlling muscle strength on the bases of proprioceptive input may be more important for reducing activity limitations than just enhancing sheer muscle strength

Kashin Beck Disease: more than just osteoarthrosis

The purpose of this study was to investigate the influence of body function, activities and pain on the level of activity in adults with Kashin Beck Disease (KBD). Seventy-five KBD patients with a mean age of 54.8 years (SD 11.3) participated. Anthropometrics, range of joint motion (ROM) and muscle strength were measured as well as the time-up-and-go test and functional tests for the lower and upper extremities. Activity was assessed with the participation scale and the WHO DAS II. In the shoulder, elbow, hip and knee joints, a severe decrease in ROM and bilateral pain was noted. A decrease in muscle strength was observed in almost all muscles. The timed-up-and-go test scores decreased. No or mild restriction in activity was found in 35%, and 33% experienced a moderate restriction whereas 32% had severe to extreme restriction. Activities in the lower extremities were mildly to moderately correlated to ROM and muscle strength, whereas in the upper extremities activities were correlated to range of joint motion. Activity was significantly associated with ROM after correction for muscle strength, gender and age. Participation was borderline significantly associated with ROM after correction for muscle strength, gender, age and the activity time-up-and-go. In KBD adults, a severe decrease in activity is primarily caused by decrease in ROM. These findings have strong influence on rehabilitation and surgical interventio

Motor performance and functional ability in preschool- and early school-aged children with Juvenile Idiopathic Arthritis: a cross-sectional study

<p>Abstract</p> <p>Objective</p> <p>To describe the level of motor performance and functional skills in young children with JIA.</p> <p>Methods</p> <p>In a cross-sectional study in 56 preschool-aged (PSA) and early school- aged children (ESA) with JIA according to ILAR classification, motor performance was measured with the Bayley Scales of Infant Development II (BSID₂) and the Movement Assessment Battery for Children (M-ABC). Functional skills were measured with the Pediatric Evaluation of Disability Inventory (PEDI). Disease outcome was measured with a joint count on swelling/range of joint motion, functional ability and joint pain.</p> <p>Results</p> <p>Twenty two PSA children (mean age 2.1 years) with a mean Developmental Index of the BSID₂of 77.9 indicating a delayed motor performance; 45% of PSA children showed a severe delayed motor performance. Mean PEDI scores were normal, 38% of PSA scored below -2 SD in one or more domains of the PEDI. Thirty four ESA children (mean age 5.2 years) with a mean M-ABC 42.7, indicating a normal motor performance, 12% of ESA children had an abnormal score. Mean PEDI scores showed impaired mobility skills, 70% of ESA children scored below -2 SD in one or more domains of the PEDI. Disease outcome in both age groups demonstrated low to moderate scores. Significant correlations were found between age at disease onset, disease duration and BSID₂or M-ABC and between disease outcome and PEDI in both age cohorts.</p> <p>Conclusion</p> <p>More PSA children have more impaired motor performance than impaired functional skills, while ESA children have more impairment in functional skills. Disease onset and disease duration are correlated with motor performance in both groups. Impaired motor performance and delayed functional skills is primarily found in children with a polyarticular disease course. Clinical follow up and rehabilitation programs should also focus on motor performance and functional skills development in young children with JIA.</p

Effect of pediatric physical therapy on deformational plagiocephaly in children with positional preference: a randomized controlled trial

Objective To study the effect of pediatric physical therapy on positional preference and deformational plagiocephaly.\ud \ud Design Randomized controlled trial.\ud \ud Setting Bernhoven Hospital, Veghel, the Netherlands.\ud \ud Participants Of 380 infants referred to the examiners at age 7 weeks, 68 (17.9%) met criteria for positional preference, and 65 (17.1%) were enrolled and followed up at ages 6 and 12 months.\ud \ud Intervention Infants with positional preference were randomly assigned to receive either physical therapy (n = 33) or usual care (n = 32).\ud \ud Main Outcome Measures The primary outcome was severe deformational plagiocephaly assessed by plagiocephalometry. The secondary outcomes were positional preference, motor development, and cervical passive range of motion.\ud \ud Results Both groups were comparable at baseline. In the intervention group, the risk for severe deformational plagiocephaly was reduced by 46% at age 6 months (relative risk, 0.54; 95% confidence interval, 0.30-0.98) and 57% at age 12 months (0.43; 0.22-0.85). The numbers of infants with positional preference needed to treat were 3.85 and 3.13 at ages 6 and 12 months, respectively. No infant demonstrated positional preference at follow-up. Motor development was not significantly different between the intervention and usual care groups. Cervical passive range of motion was within the normal range at baseline and at follow-up. When infants were aged 6 months, parents in the intervention group demonstrated significantly more symmetry and less left orientation in nursing, positioning, and handling.\ud \ud Conclusion A 4-month standardized pediatric physical therapy program to treat positional preference significantly reduced the prevalence of severe deformational plagiocephaly compared with usual care

Idiopathic toe-walking in children, adolescents and young adults: a matter of local or generalised stiffness?

<p>Abstract</p> <p>Background</p> <p>Idiopathic Toe Walking (ITW) is present in children older than 3 years of age still walking on their toes without signs of neurological, orthopaedic or psychiatric diseases. ITW has been estimated to occur in 7% to 24% of the childhood population. To study associations between Idiopathic Toe Walking (ITW) and decrease in range of joint motion of the ankle joint. To study associations between ITW (with stiff ankles) and stiffness in other joints, muscle strength and bone density.</p> <p>Methods</p> <p>In a cross-sectional study, 362 healthy children, adolescents and young adults (mean age (sd): 14.2 (3.9) years) participated. Range of joint motion (ROM), muscle strength, anthropometrics sport activities and bone density were measured.</p> <p>Results</p> <p>A prevalence of 12% of ITW was found. Nine percent had ITW and severely restricted ROM of the ankle joint. Children with ITW had three times higher chance of severe ROM restriction of the ankle joint. Participants with ITW and stiff ankle joints had a decreased ROM in other joints, whereas bone density and muscle strength were comparable.</p> <p>Conclusion</p> <p>ITW and a decrease in ankle joint ROM might be due to local stiffness. Differential etiological diagnosis should be considered.</p

Exercise training in pediatric patients with end-stage renal disease

The objective of this study was to determine the feasibility and efficacy of an exercise training program to improve exercise capacity and fatigue level in pediatric patients with end-stage renal disease (ESRD). Twenty children on dialysis intended to perform a 12-week graded community-based exercise program. Exercise capacity and fatigue level were studied; muscle force and health-related quality of life were secondary outcomes. All outcomes were measured at baseline (T = 0) and after intervention (T = 1). Fourteen of the 20 patients (70%) either did not start the program or did not complete the program. Of these patients, seven did not complete or even start the exercise program because of a combination of lack of time and motivational problems. Six patients were not able to continue the program or were unable to do the follow-up measurements because of medical problems. Exercise capacity and muscle strength was higher after the exercise program in the children who completed the training. In conclusion, exercise training is difficult to perform in children with ESRD and is not always feasible in real-life situations for many children with ESRD

Six-minute walking test in children with ESRD: discrimination validity and construct validity

The six-minute walking test (6MWT) may be a practical test for the evaluation functional exercise capacity in children with end-stage renal disease (ESRD). The aim of this study was to investigate the 6MWT performance in children with ESRD compared to reference values obtained in healthy children and, secondly, to study the relationship between 6MWT performance with anthropometric variables, clinical parameters, aerobic capacity and muscle strength. Twenty patients (13 boys and seven girls; mean age 14.1 ± 3.4 years) on dialysis participated in this study. Anthropometrics were taken in a standardized manner. The 6MWT was performed in a 20-m-long track in a straight hallway. Aerobic fitness was measured using a cycle ergometer test to determine peak oxygen uptake \documentclass[12pt]{minimal} \usepackage{amsmath} \usepackage{wasysym} \usepackage{amsfonts} \usepackage{amssymb} \usepackage{amsbsy} \usepackage{mathrsfs} \usepackage{upgreek} \setlength{\oddsidemargin}{-69pt} \begin{document}$$\left( {\mathop {\text{V}}\limits^\cdot {{\text{O}}_{\text{2peak}}}} \right)$$\end{document}, peak rate (Wpeak) and ventilatory threshold (VT). Muscle strength was measured using hand-held myometry. Children with ESRD showed a reduced 6MWT performance (83% of predicted, p < 0.0001), irrespective of the reference values used. The strongest predictors of 6MWT performance were haematocrit and height. Regression models explained 59% (haematocrit and height) to 60% (haematocrit) of the variance in 6MWT performance. 6MWT performance was not associated with \documentclass[12pt]{minimal} \usepackage{amsmath} \usepackage{wasysym} \usepackage{amsfonts} \usepackage{amssymb} \usepackage{amsbsy} \usepackage{mathrsfs} \usepackage{upgreek} \setlength{\oddsidemargin}{-69pt} \begin{document}$${\mathop {\text{V}}\limits^\cdot {{\text{O}}_{\text{2peak}}}}$$\end{document}, strength, or other anthropometric variables, but it was significantly associated with haematocrit and height. Children with ESRD scored lower on the 6MWT than healthy children. Based on these results, the 6MWT may be a useful instrument for monitoring clinical status in children with ESRD, however it cannot substitute for other fitness tests, such as a progressive exercise test to measure \documentclass[12pt]{minimal} \usepackage{amsmath} \usepackage{wasysym} \usepackage{amsfonts} \usepackage{amssymb} \usepackage{amsbsy} \usepackage{mathrsfs} \usepackage{upgreek} \setlength{\oddsidemargin}{-69pt} \begin{document}$${\mathop {\text{V}}\limits^\cdot {{\text{O}}_{\text{2peak}}}}$$\end{document} or muscle strength tests