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Background and Aim: Factors promoting family adaptation to child's disability are poorly studied together. The aim of the study was to describe the family adaptation to disability and to identify determinants associated with using a global theoretical model.Materials and Methods: 286 families of teenagers [13-17 years] with cerebral palsy (CP) from 4 European disability registers were included and visited at home. Face to face interviews were performed in order to measure parental distress, perceived impact in various dimensions of family life, family resources and stressors. Relationships were modelled with structural equations.Results: 31.8% of parents living with an adolescent with CP showed clinically significant high stress requiring professional assistance. The main stressors were the level of motor impairment and behavioural disorders in adolescent. A good family functioning was the best protective factor. Respite in care and a parents' positive attitude were significantly related to less parental distress. Material support, socioeconomical level, marital status or parental qualifications did not appear to be significant protector factors.Conclusions: Particular attention must be paid not only on physical condition but also on adolescent psychological problems to improve family adaptation. Families at risk of experiencing severe distress should be targeted early and proactive caregiver interventions on the whole family should be performed.What This Paper Adds: Family is a dynamic system: facing disability, it tries to recover its balance with available resources and its perception of the situation. Literature highlights potential stressors and protecting factors that could affect the disabled child's family adaptation but few papers study a global model including most of these factors. This study validated a global theoretical model of family adaptation to disability at adolescence. It identified behaviour disorders and motor impairment level as main stressors, family functioning as the largest protecting factors, and equipment and financial support as non significant protective factors. [ABSTRACT FROM AUTHOR]

Aim: To test the hypothesis that higher seizure burden in Dravet syndrome is associated with increased comorbidities and lower quality of life (QoL) in a large cohort of patients with Dravet syndrome and their caregivers in Europe.Method: An extensive survey of caregivers of patients with Dravet syndrome on experiences of diagnosis, seizure burden, management, social and financial impact, and health services use was administered online in 10 languages.Results: The survey received 584 unique responses from caregivers of paediatric (83%) and adult (17%) patients with Dravet syndrome (aged <1-48y). Despite broadly following current treatment guidance, less than 10% of patients were seizure free in the previous 3 months. Nearly all (99.6%) patients aged 5 years or older experienced at least one or more motor, speech, learning, or behavioural impairment. High seizure frequency was related to more reports of emergency treatment, comorbidities, and a lower QoL (as measured by the standardized instrument EQ-5D-5L). If not diagnosed at the first instance, the majority (83%) of adults, but less than 20% of 6- to 11-year-olds were diagnosed after 4 or more years.Interpretation: Patients with Dravet syndrome with the highest current seizure frequency suffer from more comorbidities and have a lower QoL. Therefore, more effective antiepileptic treatments are needed.What This Paper Adds: The survey captured about 15% of all patients with Dravet syndrome in Europe. Less than 10% of patients had current seizure freedom. Patients with a high current seizure burden have more comorbidities and lower quality of life. [ABSTRACT FROM AUTHOR]

Background and Aims: Although much has been written about the conventional cardiovascular risk factor correlates of the extent of coronary artery calcification (CAC), few studies have been carried out on symptomatic patients. This paper assesses the potential ability of risk factors to associate with an increasing CAC score.Methods: From the European Calcific Coronary Artery Disease (Euro-CCAD) cohort, we retrospectively investigated 6309 symptomatic patients, 62% male, from Denmark, France, Germany, Italy, Spain and the USA. All had conventional cardiovascular risk factor assessment and CT scanning for CAC scoring.Results: Among all patients, male sex (OR = 4.85, p<0.001) and diabetes (OR = 2.36, p<0.001) were the most important risk factors of CAC extent, with age, hypertension, dyslipidemia and smoking also showing a relationship. Among patients with CAC, age, diabetes, hypertension and dyslipidemia were associated with an increasing CAC score in males and females, with diabetes being the strongest dichotomous risk factor (p<0.001 for both). These results were echoed in quantile regression, where diabetes was consistently the most important correlate with CAC extent in every quantile in both males and females. To a lesser extent, hypertension and dyslipidemia were also associated in the high CAC quantiles and the low CAC quantiles respectively.Conclusion: In addition to age and male sex in the total population, diabetes is the most important correlate of CAC extent in both sexes. [ABSTRACT FROM AUTHOR]

Purpose: Different health state utility (HSU) instruments produce different utilities for the same individuals, thereby compromising the intended comparability of economic evaluations of health care interventions. When developing crosswalks, previous studies have indicated nonlinear relationships. This paper inquires into the degree of nonlinearity across the four most widely used HSU-instruments and proposes exchange rates that differ depending on the severity levels of the health state utility scale.Methods: Overall, 7933 respondents from six countries, 1760 in a non-diagnosed healthy group and 6173 in seven disease groups, reported their health states using four different instruments: EQ-5D-5L, SF-6D, HUI-3 and 15D. Quantile regressions investigate the degree of nonlinear relationships between these instruments. To compare the instruments across different disease severities, we split the health state utility scale into utility intervals with 0.2 successive decrements in utility starting from perfect health at 1.00. Exchange rates (ERs) are calculated as the mean utility difference between two utility intervals on one HSU-instrument divided by the difference in mean utility on another HSU-instrument.Results: Quantile regressions reveal significant nonlinear relationships across all four HSU-instruments. The degrees of nonlinearities differ, with a maximum degree of difference in the coefficients along the health state utility scale of 3.34 when SF-6D is regressed on EQ-5D. At the lower end of the health state utility scale, the exchange rate from SF-6D to EQ-5D is 2.11, whilst at the upper end it is 0.38.Conclusion: Comparisons at different utility levels illustrate the fallacy of using linear functions as crosswalks between HSU-instruments. The existence of nonlinear relationships between different HSU-instruments suggests that level-specific exchange rates should be used when converting a change in utility on the instrument used, onto a corresponding utility change had another instrument been used. Accounting for nonlinearities will increase the validity of the comparison for decision makers when faced with a choice between interventions whose calculations of QALY gains have been based on different HSU-instruments. [ABSTRACT FROM AUTHOR]