301 research outputs found

    Can women have children and a career? IV evidence from IVF treatments

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    This paper introduces a new IV strategy based on IVF (in vitro fertilization) induced fertility variation among childless women to estimate the causal effect of having children on their career. For this purpose, we use administrative data on IVF treated women in Denmark. Because observed chances of IVF success do not depend on labor market histories, IVF treatment success provides a plausible instrument for childbearing. Our IV estimates indicate that fertility effects on earnings are: (i) negative, large, and long-lasting; (ii) driven by fertility effects on hourly earnings and not so much on labor supply; and (iii) much stronger at the extensive margin than at the intensive margin

    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

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    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

    Prenatal diagnosis for haemophilia: A nationwide survey among female carriers in the Netherlands

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    Carriers of haemophilia face difficult choices regarding prenatal diagnosis, but little is known about the determinants that influence their decisions. The aim of this study was to assess the incidence of prenatal diagnosis and potential determinants affecting the choice for prenatal diagnosis. A nationwide survey was performed among all women who underwent carriership testing for haemophilia in the Netherlands between 1992 and 2004. Prenatal diagnosis was assessed i

    Classic infantile Pompe patients approaching adulthood: A cohort study on consequences for the brain

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    Aim: To examine the long-term consequences of glycogen storage in the central nervous system (CNS) for classic infantile Pompe disease using enzyme replacement therapy. Method: Using neuropsychological tests and brain magnetic resonance imaging (MRI), we prospectively assessed a cohort of 11 classic infantile Pompe patients aged up to 17 years. Results: From approximately age 2 years onwards, brain MRI showed involvement of the periventricular white matter and centrum semiovale. After 8 years of age, additional white-matter abnormalities occurred in the corpus callosum, internal and external capsule, and subcortical areas. From 11 years of age, white-matter abnormalities were also found in the brainstem. Although there seemed to be a characteristic pattern of involvement over time, there were considerable variations between patients, reflected by variations in neuropsychological development. Cognitive development ranged from stable and normal to declines that lead to intellectual disabilities. Interpretation: As treatment enables patients with classic infantile Pompe disease to reach adulthood, white-matter abnormalities are becoming increasingly evident, affecting the neuropsychological development. Therefore, we advise follow-up programs are expanded to capture CNS involvement in larger, international patient cohorts, to incorporate our findings in the counselling of parents before the start of treatment, and to include the brain as an additional target in the development of next-generation therapeutic strategies for classic infantile Pompe disease. What this paper adds: In our long-term survivors treated intravenously with enzyme replacement therapy, we found slowly progressive symmetric white-matter abnormalities. Cognitive development varied from stable and normal to declines towards intellectual disabilities

    Assessing the societal benefits of river restoration using the ecosystem services approach

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    This paper is a contribution from the EU seventh framework funded research project REFORM (Grant Agreement 282656).The success of river restoration was estimated using the ecosystem services approach. In eight pairs of restored–unrestored reaches and floodplains across Europe, we quantified provisioning (agricultural products, wood, reed for thatching, infiltrated drinking water), regulating (flooding and drainage, nutrient retention, carbon sequestration) and cultural (recreational hunting and fishing, kayaking, biodiversity conservation, appreciation of scenic landscapes) services for separate habitats within each reach, and summed these to annual economic value normalized per reach area. We used locally available data and literature, did surveys among inhabitants and visitors, and used a range of economic methods (market value, shadow price, replacement cost, avoided damage, willingness-to-pay survey, choice experiment) to provide final monetary service estimates. Total ecosystem service value was significantly increased in the restored reaches (difference 1400 ± 600 € ha−1 year−1; 2500 − 1100, p = 0.03, paired t test). Removal of one extreme case did not affect this outcome. We analysed the relation between services delivered and with floodplain and catchment characteristics after reducing these 23 variables to four principal components explaining 80% of the variance. Cultural and regulating services correlated positively with human population density, cattle density and agricultural N surplus in the catchment, but not with the fraction of arable land or forest, floodplain slope, mean river discharge or GDP. Our interpretation is that landscape appreciation and flood risk alleviation are a function of human population density, but not wealth, in areas where dairy farming is the prime form of agriculture.PostprintPeer reviewe

    Prediction of pH Change in Processed Acidified Turnips

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    The acetic acid uptake by turnips was studied during an acidification process in containers. The process was successfully described by a Fickian diffusion, using a correlation for the buffer effect. Diffusion coefficients (0.629 to 3.99 × 10-9 m2/sec) and partition coefficients (0.8 to 1.1) were obtained by optimization of the fit between experimental and theoretical values, using the simplex method. The partition coefficient did not show an evident dependence on temperature, while diffusivity followed an Arrhenius type behavior. The relationship between acid concentration and pH was described using a cubic model with parameters independent of temperature. Results showed that the combination of these models describing the acid diffusion into the food and the buffering effects of the food allowed accurate prediction of pH evolution in the acidification process
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