A New Parameter Set for the Relativistic Mean Field Theory

Subtracting the Strutinsky shell corrections from the selfconsistent energies obtained within the Relativistic Mean Field Theory (RMFT) we have got estimates for the macroscopic part of the binding energies of 142 spherical even-even nuclei. By minimizing their root mean square deviations from the values obtained with the Lublin-Srasbourg Drop (LSD) model with respect to the nine RMFT parameters we have found the optimal set (NL4). The new parameters reproduce also the radii of these nuclei with an accuracy comparable with that obtained with the NL1 and NL3 sets.Comment: Semiar given at the 10th Nuclear Physics Workshop in Kazimierz, Poland, Sep. 24-28, 200

Variability in feeding habitats of red deer sensu lato in Eurasia in the Late Pleistocene and Holocene

Red deer (Cervus elaphus) is one of the species that is rather wide spread and survived across Europe over the Holocene. The analyses of carbon and nitrogen stable isotopes in bone collagen of ungulate remains have been applied in paleoecological studies as environmental and dietary indicators. In this study we present the carbon and nitrogen stable isotope compositions of previously radiocarbon-dated red deer bone samples (N = 68) found in Central, Southern and Eastern Europe and Asia and aligned to one of two species: European red deer (Cervus elaphus) and wapiti (Cervus canadensis). We showed that the values of carbon and nitrogen stable isotope ratios of European red deer and wapiti overlapped. Among all analysed independent factors (determined for the locality and time period relevant for each of the analysed samples), the variability of δ13C values in European red deer dated to the Holocene is best explained by forest cover and mean July temperature, and variability of δ15N values by the mean July temperature, annual precipitation and altitude. Additionally, combining the results of the present study with isotopic data on European red deer collected from published sources, we revealed that the values of δ13C and of δ15N in C. elaphus bones changed according to environmental oscillations that took place in Europe over the last 50 000 years. We concluded that red deer shifted their feeding habitats in relation to changing environmental conditions, for example, forest expansion during the climate warming, and in the mid to later Holocene in response to deforestation caused by human activity and the spread of agriculture. We also found out that red deer reacted in varied ways to changing local conditions in different regions of Europe. Modern individuals of C. elaphus had the lowest δ13C values among all analysed specimens, so they probably inhabited the most densely forested areas in comparison to other European red deer populations during the last 50 000 years

Nijmegen paediatric CDG rating scale: a novel tool to assess disease progression

Congenital disorders of glycosylation (CDG) are a group of clinically heterogeneous inborn errors of metabolism. At present, treatment is available for only one CDG, but potential treatments for the other CDG are on the horizon. It will be vitally important in clinical trials of such agents to have a clear understanding of both the natural history of CDG and the corresponding burden of disability suffered by patients. To date, no multicentre studies have attempted to document the natural history of CDG. This is in part due to the lack of a reliable assessment tool to score CDG’s diverse clinical spectrum. Based on our earlier experience evaluating disease progression in disorders of oxidative phosphorylation, we developed a practical and semi-quantitative rating scale for children with CDG. The Nijmegen Paediatric CDG Rating Scale (NPCRS) has been validated in 12 children, offering a tool to objectively monitor disease progression. We undertook a successful trial of the NPCRS with a collaboration of nine experienced physicians, using video records of physical and neurological examination of patients. The use of NPCRS can facilitate both longitudinal and natural history studies that will be essential for future interventions

Winter Temperature and Forest Cover Have Shaped Red Deer Distribution in Europe and the Ural Mountains Since the Late Pleistocene

Aim: The Expansion-Contraction model has been used to explain the responses of species to climatic changes. During periods of unfavourable climatic conditions, species retreat to refugia from where they may later expand. This paper focuses on the palaeoecology of red deer over the past 54 ka across Europe and the Urals, to reveal patterns of change in their range and explore the role of environmental conditions in determining their distribution. Location: Europe and western Asia to 63°E. Taxon: Red deer (Cervus elaphus). Methods: We collected 984 records of radiocarbon-dated red deer subfossils from the Late Pleistocene and the Holocene, including 93 original dates. For each deer sample we compiled climatic and biome type data for the corresponding time intervals. Results: During the last 54 ka changes in red deer range in Europe and the Urals were asynchronous and differed between western and eastern Europe and western Asia due to different environmental conditions in those regions. The range of suitable areas for deer during the Last Glacial Maximum (LGM) was larger than previously thought and covered vast regions not only in southern but also in western and eastern Europe. Throughout the period investigated the majority of specimens inhabited forests in the temperate climatic zone. The contribution of forests in deer localities significantly decreased during the last 4 ka, due to deforestation of Europe caused by humans. Mean January temperature was the main limiting factor for species distribution. Over 90% of the samples were found in areas where mean January temperature was above −10°C. Main conclusions: Red deer response to climatic oscillations are in agreement with the Expansion-Contraction model but in contradiction to the statement of only the southernmost LGM refugia of the species. During the last 54 ka red deer occurred mostly in forests of the temperate climatic zone. © 2020 John Wiley & Sons Ltd.European Social Fund, Grant/Award Number: UDA-POKL.04.01.01-00-072/09-00; University of Wroclaw, Grant/Award Number: 0410/2990/18; Institute of Environmental Biology, University of Wrocław, Grant/Award Number: 0410/2990/18; Mammal Research Institute Polish Academy of Sciences; Narodowe Centrum Nauki , Grant/Award Number: DEC-2013/11/B/NZ8/00888 and UMO-2016/23/B/HS3/00387; Romanian National Authority for Scientific Research, UEFISCDI, Grant/Award Number: PN-IIIP4-ID-PCE-2016-0676; National Centre for Atmospheric Science and the Centre for Environmental Data Analysis, UK; Faunal Database of the Stage Three Project; Leverhulme Trust, Grant/Award Number: F00568W

Winter temperature and forest cover have shaped red deer distribution in Europe and the Ural Mountains since the Late Pleistocene

Aim: The Expansion-Contraction model has been used to explain the responses of species to climatic changes. During periods of unfavourable climatic conditions, species retreat to refugia from where they may later expand. This paper focuses on the palaeoecology of red deer over the past 54 ka across Europe and the Urals, to reveal patterns of change in their range and explore the role of environmental conditions in determining their distribution. Location: Europe and western Asia to 63°E. Taxon: Red deer (Cervus elaphus). Methods: We collected 984 records of radiocarbon-dated red deer subfossils from the Late Pleistocene and the Holocene, including 93 original dates. For each deer sample we compiled climatic and biome type data for the corresponding time intervals. Results: During the last 54 ka changes in red deer range in Europe and the Urals were asynchronous and differed between western and eastern Europe and western Asia due to different environmental conditions in those regions. The range of suitable areas for deer during the Last Glacial Maximum (LGM) was larger than previously thought and covered vast regions not only in southern but also in western and eastern Europe. Throughout the period investigated the majority of specimens inhabited forests in the temperate climatic zone. The contribution of forests in deer localities significantly decreased during the last 4 ka, due to deforestation of Europe caused by humans. Mean January temperature was the main limiting factor for species distribution. Over 90% of the samples were found in areas where mean January temperature was above −10°C. Main conclusions: Red deer response to climatic oscillations are in agreement with the Expansion-Contraction model but in contradiction to the statement of only the southernmost LGM refugia of the species. During the last 54 ka red deer occurred mostly in forests of the temperate climatic zone. © 2020 John Wiley & Sons Ltd.European Social Fund, Grant/Award Number: UDA-POKL.04.01.01-00-072/09-00; University of Wroclaw, Grant/Award Number: 0410/2990/18; Institute of Environmental Biology, University of Wrocław, Grant/Award Number: 0410/2990/18; Mammal Research Institute Polish Academy of Sciences; Narodowe Centrum Nauki , Grant/Award Number: DEC-2013/11/B/NZ8/00888 and UMO-2016/23/B/HS3/00387; Romanian National Authority for Scientific Research, UEFISCDI, Grant/Award Number: PN-IIIP4-ID-PCE-2016-0676; National Centre for Atmospheric Science and the Centre for Environmental Data Analysis, UK; Faunal Database of the Stage Three Project; Leverhulme Trust, Grant/Award Number: F00568W

Long-term effects of medical management on growth and weight in individuals with urea cycle disorders

Low protein diet and sodium or glycerol phenylbutyrate, two pillars of recommended long-term therapy of individuals with urea cycle disorders (UCDs), involve the risk of iatrogenic growth failure. Limited evidence-based studies hamper our knowledge on the long-term effects of the proposed medical management in individuals with UCDs. We studied the impact of medical management on growth and weight development in 307 individuals longitudinally followed by the Urea Cycle Disorders Consortium (UCDC) and the European registry and network for Intoxication type Metabolic Diseases (E-IMD). Intrauterine growth of all investigated UCDs and postnatal linear growth of asymptomatic individuals remained unaffected. Symptomatic individuals were at risk of progressive growth retardation independent from the underlying disease and the degree of natural protein restriction. Growth impairment was determined by disease severity and associated with reduced or borderline plasma branched-chain amino acid (BCAA) concentrations. Liver transplantation appeared to have a beneficial effect on growth. Weight development remained unaffected both in asymptomatic and symptomatic individuals. Progressive growth impairment depends on disease severity and plasma BCAA concentrations, but cannot be predicted by the amount of natural protein intake alone. Future clinical trials are necessary to evaluate whether supplementation with BCAAs might improve growth in UCDs