BackgroundFibrodysplasia ossificans progressiva (FOP) is a rare, severely disabling, and life-shortening genetic disorder that causes the formation of heterotopic bone within soft connective tissue. Previous studies found that the FOP prevalence was about one in every two million lives. The aim of this study is to estimate the FOP prevalence in France by probabilistic record-linkage of 2 national databases: 1) the PMSI (Programme de médicalisation des systèmes d’information), an administrative database that records all hospitalization activities in France and 2) CEMARA, a registry database developed by the French Centres of Reference for Rare Diseases.ResultsUsing a capture-recapture methodology to adjust the crude number of patients identified in both data sources, 89 FOP patients were identified, which results in a prevalence of 1.36 per million inhabitants (CI95% = [1.10; 1.68]). FOP patients’ mean age was 25 years, only 14.9% were above 40 years, and 53% of them were males. The first symptoms – beside toe malformations- occurred after birth for 97.3% of them. Mean age at identified symptoms was 7 years and above 18 years for only 6.9% of patients. Mean age at diagnosis was 10 years, and above 18 years for 14.9% of the patients. FOP patients were distributed across France.ConclusionsDespite the challenge of ascertaining patients with rare diseases, we report a much higher prevalence of FOP in France than in previous studies elsewhere. We suggest that efforts to identify patients and confirm the diagnosis of FOP should be reinforced and extended at both national and European level.

Biomechanical and Psychosocial Work Exposures and Musculoskeletal Symptoms amongVineyard Workers: Christophe Bernard, et al. CCMSA, France-Objectives: This study explored the associations between biomechanical and psychosocial work factors and musculoskeletal symptoms in vineyard workers. Methods: This crosssectional study was based on a random sample of 2,824 male and 1,123 female vineyard workers in France. Data were collected using a self-administered questionnaire. Neck/shoulder, back and upper and lower extremity symptoms were evaluated using the Nordic questionnaire. Biomechanical exposures included 15 tasks related to vineyard activities. Psychosocial work factors included effort-reward imbalance and overcommitment, measured using the effort-reward imbalance model, and low job control and insufficient material means. Statistical analysis was performed using logistic regression analysis, and the results were adjusted for age, body mass index, educational level, work status and years in vineyard. Results: Pruning-related factors increased the risk of upper extremity pain for both genders, of back pain for men and of neck/shoulder and lower extremity pain for women. Driving increased the risk of neck/shoulder and back pain among men. Psychosocial work factors, which were insufficient material means, overcommitment (both genders), effort-reward imbalance (men) and low j o b c o n t r o l ( w o m e n ) , w e r e a s s o c i a t e d w i t h musculoskeletal symptoms, back and upper extremity pain for both genders and neck/shoulder and lower extremity pain for men. Conclusions: These results underlined that both biomechanical and psychosocial work factors may play a role in musculoskeletal pain among vineyard workers. Prevention policies focusing on both biomechanical and psychosocial work exposures may be useful to prevent musculoskeletal symptoms. (J Occup Health 2011; 53: 297-311) Key words: Biomechanical exposures, Musculoskeletal symptoms, Psychosocial work factors, Vineyard workers Musculoskeletal symptoms are considered to be a major occupational health issue especially among blue collar and service workers. The prevalence of these symptoms may be high, as the prevalence of back or neck/shoulder pain may be up to 40% 1) and often more in some sectors of work. These disorders lead to high human, social and economic costs (health care, disability, sickness absence, early retirement, etc.) [2][3][4] . In addition, musculoskeletal disorders are one of the leading occupational diseases in France and in many other industrialized countries. The etiology of these disorders is probably multifactorial and involves both biomechanical and psychosocial work exposures. However, whereas biomechanical factors are recognized as well-known risk factors, the etiological role of psychosocial work factors may be more controversial, as showed in literature reviews that provided little evidence for this role [5][6][7] . These reviews also underlined the lack of adjustment for biomechanical factors in the published s...

Background Certain telemedicine programmes for heart failure (HF) have been shown to reduce all-cause mortality and heart failure-related hospitalisations, but their cost-effectiveness remains controversial. The SCAD programme is a home-based interactive telemonitoring service for HF, which is one of the largest and longest-running telemonitoring programmes for HF in France. The objective of this cost-utility analysis was to evaluate the cost-effectiveness of the SCAD programme with respect to standard hospital-based care in patients with HF. Methods A Markov model simulating hospitalisations and mortality in patients with HF was constructed to estimate outcomes and costs. The model included six distinct health states (three ‘not hospitalised’ states, two ‘hospitalisation for heart failure’ states, both depending on the number of previous hospitalisations, and one death state). The model lifetime in the base case was 10 years. Model inputs were based on published literature. Outputs (costs and QALYs) were compared between SCAD participants and standard care. Deterministic and probabilistic sensitivity analyses were performed to assess uncertainty in the input parameters of the model. Results The number of quality-adjusted life years (QALYs) was 3.75 in the standard care setting and 4.41 in the SCAD setting. This corresponds to a gain in QALYs provided by the SCAD programme of 0.65 over the 10 years lifetime of the model. The estimated total cost was €30,932 in the standard care setting and €35,177 in the SCAD setting, with an incremental cost of €4245. The incremental cost-effectiveness ratio (ICER) for the SCAD programme over standard care was estimated at €4579/QALY. In the deterministic sensitivity analysis, the variables that had the most impact on the ICER were HF management costs. The likelihood of the SCAD programme being considered cost-effective was 90% at a willingness-to-pay threshold of €11,800. Conclusions Enrolment of patients into the SCAD programme is highly cost-effective. Extension of the programme to other hospitals and more patients would have a limited budget impact but provide important clinical benefits. This finding should also be taken into account in new public health policies aimed at encouraging a shift from inpatient to ambulatory care.

Aims Management of patients with recently decompensated heart failure by hospital services is expensive, complicated to plan, and not always effective. Telemedicine programmes in heart failure may improve the quality of care, but their effectiveness is poorly documented in real‐world settings. The study aims to evaluate the impact of patient engagement in home‐based telemonitoring for heart failure (SCAD programme) on rehospitalization and mortality rates. Methods and results A retrospective observational study was performed in 659 SCAD participants. SCAD is a patient‐oriented service of home‐based interactive telemonitoring offered to heart failure patients during hospitalization who agree to participate in a therapeutic education programme. Patients were telemonitored for at least 3 months, and rehospitalization and mortality were documented at 12 months and 5 years. During the telemonitoring period, patients provided daily information on health and lifestyle through an internet‐based interface. Data were linked on a patient‐by‐patient basis between the SCAD database and the French national health insurance database (Système National des Données de Santé). Outcomes were compared as a function of use of the programme. Low, intermediate, and high users were classified by tercile of data return during telemonitoring. Patients were followed for a median of 32.9 months. Rehospitalization rates for cardiovascular disease decreased from 79.4% in the year preceding enrolment to 41.1% in the following year and from 52.8% to 18.8% for hospitalizations for heart failure. The 12 month mortality rate was 11.2%. Significant associations were observed between level of use of the SCAD programme and all‐cause rehospitalization (P = 0.0085), rehospitalization for cardiovascular disease (P = 0.0010), rehospitalization for heart failure (27.8% in low users, 12.9% in intermediate users, and 13.5% in high users; P < 0.0001), and mortality (26.8%, 15.2%, and 15.9% respectively; P = 0.0157) in the 12 months following enrolment. The mean number of days alive outside hospital were 279 ± 111 in low users, 312 ± 90 in intermediate users, and 304 ± 100 in high users (P = 0.0022). Conclusions Educational home telemonitoring of patients with heart failure following hospitalization provides long‐term clinical benefits in terms of rehospitalization and death in real‐world settings, according to the level of use of the programme by the patient. These benefits would be expected to have a major impact on the burden of this disease. Low engagement in telemonitoring could be used as a signal of poor prognosis and taken into account in the management strategy.

Background SCAD is a 3-month telemonitoring program for HF patients, associated with therapeutic education, proposed after an acute HF episode. SCAD is based on an interactive algorithm allowing to generate educative messages and alarms based on patients' responses registered on a digital tablet. It is funded by the French Health Insurance through a pilot program: ETAPES (470€/patient). Purpose To describe the profile of patients using SCAD & assess the medico-economic impact of the SCAD system. Methods Multicenter retrospective cohort study using SCAD data matched with French Health Insurance data. All patients telemonitored by SCAD in 7 centers have been included, since 01/01/2010 to 12/31/2016. Only direct costs were considered, estimated from a societal perspective limited to reimbursements. Analyses were performed to assess the difference in healthcare consumptions and costs between the year before and the year after inclusion in the SCAD program. Patients who died in the 12 months after SCAD initiation were excluded. Results 627 patients benefited from SCAD program between 01/01/2010 and 12/31/2016 and were retrieved in French Health Insurance data through probabilistic matching. Out of the 627 patients, 99 died in the 12 months after SCAD initiation. Analyses were performed on 528 patients.72.2% were male, mean age was 66.0 years old and mean BMI 28.2. HFrEF represented 51.9% of patients, HFmrEF 25.9% and HFpEF 22.2%. 58.0% were in NYHA class 2 at baseline, 29.2% in class 3, 8.5% in class 1 and 4.3% in class 4. Mean Charlson Comorbidity Index score was 2.6 at baseline. Patients reported their level of fatigue (10 representing significant fatigue) and morale (10 = good morale) at baseline: mean fatigue=4.0/10 & mean morale=7.4/10. Medico economic results are presented in table 1 and show an important & significant decrease of hospitalizations costs and some transfer of cost toward ambulatory care. Conclusion On the year following remote monitoring, total health expenditure has been reduced by 18% on average (mean=3 210€/patient) and 42% in median (5 500€/patient) vs 12 months before. Funding Acknowledgement Type of funding source: Private company. Main funding source(s): This analysis has been funded through an institutional grant from Amgen