Food choice and diet of the bearded vulture Gypaetus barbatus in southern Africa

When offered a selection of food items, bearded vultures Gypaetus barbatus in southern Africa chose bones in preference to meat or to feeding from a fleshed carcass. Once a carcass had been stripped of soft tissue by Gyps vultures, bearded vultures disarticulated sections or individual bones (depending on the size of the dead animal) in the order: limbs, ribs, vertebrae, skull. Their overall diet was estimated as 70% bone with marrow, 25% meat and 5% skin. This diet is about 15% higher in energy than an equivalent mass of meat. Of 683 identified prey items from five sources of data, over 80% consisted of domestic livestock; about 60% of this was sheep and goats. Even birds nesting within conservation areas derived more than half of their food from domestic stock which they found by foraging over adjacent commercial and subsistence farming areas. Bearded vultures obtain all their food by scavenging, and reports of attacks on live animals and even humans are rejected

Hemophilia on the treshold of the 21st century

LUMCKlinische epidemiologi

Breaking continuity?: Site formation and temporal depth at Çatalhöyük and Tell Sabi Abyad

Archaeology of the Near Eas

How Do Spouses Share their Full Income in Russia?: Identification of the Sharing Rule Using Self-reported Income

Accepté dans une version révisée par la Review of Income and WealthInternational audienceThe paper applies the collective model to the analysis of intra-household inequality using self-reported income scales and provides a test for its assumptions. We assume a correspondence between the income level that household members report and their true income sharing. Using Russian data, we first show that this assumption is supported by the data, and then use couples who report the same level of income to identify the full sharing rule for the whole sample. From simulations for an average couple living in the Urals, we find that a full income share of 45% is allocated to the wife

Cost-effectiveness of enzyme replacement therapy with alglucosidase alfa in classic-infantile patients with Pompe disease

Background: Infantile Pompe disease is a rare metabolic disease. Patients generally do not survive the first year of life. Enzyme replacement therapy (ERT) has proven to have substantial effects on survival in infantile Pompe disease. However, the costs of therapy are very high. In this paper, we assess the cost-effectiveness of enzyme replacement therapy in infantile Pompe disease. Methods. A patient simulation model was used to compare costs and effects of ERT with costs of effects of supportive therapy (ST). The model was filled with data on survival, quality of life and costs. For both arms of the model, data on survival w

Impact of enzyme replacement therapy on survival in adults with Pompe disease: Results from a prospective international observational study

Background: Pompe disease is a rare metabolic myopathy for which disease-specific enzyme replacement therapy (ERT) has been available since 2006. ERT has shown efficacy concerning muscle strength and pulmonary function in adult patients. However, no data on the effect of ERT on the survival of adult patients are currently available. The aim of this study was to assess the effect of ERT on survival in adult patients with Pompe disease. Methods. Data were collected as part of an international observational study conducted between 2002 and 2011, in which patients were followed on an annual basis. Time-dependent Cox's proportional hazards models were used for univariable and multivariable analyses. Results: Overall, 283 adult patients with a median age of 48 years (range, 19 to 81 years) were included in the study. Seventy-two percent of patients started ERT at some time during follow-up, and 28% never received ERT. During follow-up (median, 6 years; range, 0.04 to 9 years), 46 patients died, 28 (61%) of whom had never received ERT. After adjustment for age, sex, country of residence, and disease severity (based on wheelchair and ventilator use), ERT was positively associated with survival (hazard ratio, 0.41; 95% CI, 0.19 to 0.87). Conclusion: This prospective study was the first to demonstrate the positive effect of ERT on survival in adults with Pompe disease. Given the relatively recent registration of ERT for Pompe disease, these findings further support its beneficial impact in adult patients

Effects of a higher dose of alglucosidase alfa on ventilator-free survival and motor outcome in classic infantile Pompe disease: an open-label single-center study

Background: Though enzyme-replacement therapy (ERT) with alglucosidase alfa has significantly improved the prospects for patients with classic infantile Pompe disease, some 50 % of treated infants do not survive ventilator-free beyond the age of 3 years. We investigated whether higher and more frequent dosing of alglucosidase alfa improves outcome. Methods: Eight cross-reactive immun