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Purpose: To investigate associations between maternal and paternal sport participation, and children's leisure-time physical activity, and to explore differences by child gender. Method: The sample comprised 737 year five students (mean age: 11.0 ± 0.6 years, 52% male) recruited through the Fit for Pisa Project which was conducted in 2008 at 6 secondary schools in Goettingen, Germany. Maternal and paternal sport participation were assessed through child reports of mothers' and fathers' weekly participation in sport. Children's leisure-time physical activity was measured as minutes/week that children engaged in organized and nonorganized sport. Multiple linear regression was used to assess associations between maternal and paternal sport participation, and children's leisure-time physical activity. Results: Both maternal and paternal sport participation were positively associated with children's leisure-time physical activity (maternal: b = 34.20, p < .001; paternal: b = 25.32, p < .05). When stratifying analyses by child gender, maternal sport participation remained significantly associated with leisure-time physical activity in girls (b = 60.64, p < .001). In contrast, paternal sport participation remained significantly associated with leisure-time physical activity in boys (b = 43.88, p < .01). Conclusion: Both maternal and paternal modeling positively influence children's leisure-time physical activity. [ABSTRACT FROM AUTHOR]

Background: Pediatric lifestyle interventions have positive short-term effects on obese patients. Studies on long-term effects are still scarce in Europe. We investigated long-term weight patterns and sociodemographic predictors of a weight change in a large Central European (Germany, Austria and Switzerland) overweight pediatric cohort. Methods: The APV (Adiposity Patients Verlaufsbeobachtung) database was retrospectively analyzed; 157 specialized childhood obesity centers contributed standardized data of 29,181 patients [body mass index (BMI) ≥90th percentile; 5-25 years old] presenting between 2000 and 2012. BMI standard deviation scores (BMI-SDS) were analyzed in a 2-year follow-up and grouped according to BMI-SDS changes. Multiple logistic regression analyses were conducted to assess associations between sociodemographic factors and weight patterns. Results: 2-year follow-up data were available in 3,135 patients (54.6% female). Five distinct weight trajectories 'rapid weight loss' (n = 735, 23.4%), 'delayed success' (n = 697, 22.2%), 'cycling weight' (n = 43, 1.4%), 'initial weight loss' and 'weight rebound' (n = 383, 12.2%) and 'no weight loss throughout' (n = 1,277, 40.7%) best characterized long-term BMI-SDS changes. Younger and male patients were more likely to reduce weight and maintain weight loss. Conclusions: Our results suggest that an intervention before the onset of puberty seems promising for long-term weight maintenance in overweight children. Thus, new concepts are needed to improve long-term treatment success in patients with lower success rates. © 2014 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

Background and Objectives: GM2 gangliosidoses, a group of autosomal-recessive neurodegenerative lysosomal storage disorders, result from β-hexosaminidase (HEX) deficiency with GM2 ganglioside as its main substrate. Historically, GM2 gangliosidoses have been classified into infantile, juvenile, and late-onset forms. With disease-modifying treatment trials now on the horizon, a more fine-grained understanding of the disease course is needed., Methods: We aimed to map and stratify the clinical course of GM2 gangliosidoses in a multicenter cohort of pediatric and adult patients. Patients were stratified according to age at onset and age at diagnosis. The 2 resulting GM2 disease clusters were characterized in-depth for respective disease features (detailed standardized clinical, laboratory, and MRI assessments) and disease evolution., Results: In 21 patients with GM2 gangliosidosis (17 Tay-Sachs, 2 GM2 activator deficiency, 2 Sandhoff disease), 2 disease clusters were discriminated: an early-onset and early diagnosis cluster (type I; n = 8, including activator deficiency and Sandhoff disease) and a cluster with very variable onset and long interval until diagnosis (type II; n = 13 patients). In type I, rapid onset of developmental stagnation and regression, spasticity, and seizures dominated the clinical picture. Cherry red spot, startle reactions, and elevated AST were only seen in this cluster. In type II, problems with balance or gait, muscle weakness, dysarthria, and psychiatric symptoms were specific and frequent symptoms. Ocular signs were common, including supranuclear vertical gaze palsy in 30%. MRI involvement of basal ganglia and peritrigonal hyperintensity was seen only in type I, whereas predominant infratentorial atrophy (or normal MRI) was characteristic in type II. These types were, at least in part, associated with certain genetic variants., Discussion: Age at onset alone seems not sufficient to adequately predict different disease courses in GM2 gangliosidosis, as required for upcoming trial planning. We propose an alternative classification based on age at disease onset and dynamics, predicted by clinical features and biomarkers, into type I-an early-onset, rapid progression cluster-and type II-a variable onset, slow progression cluster. Specific diagnostic workup, including GM2 gangliosidosis, should be performed in patients with combined ataxia plus lower motor neuron weakness to identify type II patients.