In spite of similar measured total activity, Fontan patients reported less time engaged in regular physical exercise than healthy controls and their quality of life was lower than the controls. We speculate that promoting structured regular physical exercise could improve the quality of life of Fontan patients.

Child Health Questionnaire-physical functioning was lowest in children who were dependent on orthoses (Ort-D) for walking. Both Ort-D and Ort-ND were similar satisfied with their orthoses. Clinical relevance This study contributes to knowledge about health-related quality of life in a group of ambulatory children with arthrogryposis multiplex congenita. For children using orthoses, it is relevant to capture their opinion about their orthoses but a questionnaire specifically for children should be developed.

Objective:To study physical activity and sleep in Fontan patients and healthy controls before and after an endurance training program, and after 1 year.Method:Fontan patients (n = 30) and healthy controls (n = 25) wore accelerometers for seven consecutive days and nights during a school week before and after a 12-week endurance training program and after 1 year.Results:Patients had similar sleep duration and sleep efficiency as healthy controls. Latency to sleep onset in minutes was longer for patients than controls (22.4 (4.3–55.3) minutes versus 14.8 (8.6–29.4) minutes, p < 0.01). More time in moderate-to-vigorous activity daytime was correlated with increased sleep time (p < 0.05; r2 = 0.20), improved sleep efficiency (p < 0.01; r2 = 0.24) and less time as wake after sleep onset (p < 0.05; r2 = 0.21) for patients but not controls. Sleep variables did not change after the exercise intervention for patients or controls. After 1 year, patients had decreased total sleep time, decreased sleep efficiency, increased accelerometer counts during sleep and more time as wake after sleep onset during sleep time, but not controls.Conclusions:Fontan patients have prolonged latency to sleep onset compared with controls. More time in physical activities was correlated with better sleep quality for the patients. Also, subjects with low sleep efficiency and long latency to sleep onset may benefit most from physical exercise. These patients should be encouraged to engage in individually designed physical exercise as this could improve sleep quality.

PurposeExcessive movements during walking have been observed by gait analysis in children with arthrogryposis (AMC) using orthoses compared to children using only shoes. The aim of this study was to evaluate energy expenditure and functional exercise capacity in children with AMC.MethodsTwenty-four children with AMC and 25 typically developing (TD) children underwent oxygen measurement and the 6-minute walk test (6MWT). Children were divided into AMC1 using knee–ankle–foot orthoses with locked knee joints (KAFO-LK); AMC2 KAFOs with open knee joints (KAFO-O) or ankle–foot orthoses (AFO); and AMC3 using shoes.ResultsThe net non-dimensional oxygen cost (NNcost) was lower in TD (0.308) than in AMC2 (0.455, n = 10) (p = 0.002). There were no differences in the net non-dimensional consumption (NNconsumption) or normalised walking velocity. The lowest NNconsumption (0.082), NNcost (0.385) and normalised walking velocity (0.214) were found in AMC1 (n = 3), but no statistical calculation was performed. In the 6MWT, both AMC2 (402.7, n = 11) and AMC3 (476.8, n = 10) walked shorter distances (m) than TD (565.1) (p < 0.001 and p = 0.043, respectively). AMC2 (0.435) had lower normalised walking velocity than TD (0.564) (p < 0.001).ConclusionsChildren with AMC using open KAFOs or AFOs (AMC2) had higher energy effort represented by significantly higher NNcost than TD, whereas AMC children requiring only shoes (AMC3) did not differ significantly from TD. To maintain the NNconsumption at an acceptable level, children using locked KAFOs (AMC1) slowed down their walking velocity. Compared to TD, the exercise capacity was lower in children with AMC using open KAFOs or AFOs and shoes, represented by lower walking velocity and shorter distance walked during the 6MWT.

Social aspects such as parental support for physical activity and refraining from tobacco were found to be important for healthy behaviours. Moreover, environmental factors such as socio-economic and geographical living area favourably influenced food choices and physical activity. Parental attitudes and economy are therefore important for physical activity, healthy food choices and refraining from tobacco in children. Consequently, future interventions need to address the psychological and environmental influences of the home environment through the active involvement of parents, even in school-based interventions.

More than 400 acquired and genetic diseases are labeled as arthrogryposis. Because of their rarity and complexity coordinated patient management is often lacking. Multidisciplinary clinics are the ideal setting to provide coordinated and comprehensive care to patients with special needs. Two similar experiences of multidisciplinary clinics for the care of patients with arthrogryposis were reported at the Symposium on Arthrogryposis held in Saint Petersburg in September 2014. These clinics are organized to bring together professionals from several disciplines, with the aim to provide patient-centered, comprehensive clinical care, and reduce the burden of multiple medical appointments for the families.

Time trends and age are both important determinants of health behaviour in pre-teen children. Even the small age increase from 11 to 13 years had an important negative influence. The results of the present study underline that the window of opportunities to promote a healthy lifestyle in children is narrow. It is evident that a successful health intervention programme must be initiated at an early age, continued and repeated over time, and structured to counteract trends in age as well as time.

No abstract