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Four neonates with a positive phenylalanine screening test (Phe concentrations between 258 and 1250 μmol/L) were investigated further to differentiate between phenylalanine hydroxylase (PAH) deficiency and variant hyperphenylalaninaemia (HPA) forms. In patients 1 and 2 a tetrahydrobiopterin (BH 4) load caused a significant decrease of the plasma Phe levels. A combined phenylalanine/BH 4 loading te

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The Transfermium Working Group (TWG) was set up in 1986 under the joint auspices of the International Union of Pure and Applied Chemistry (IUPAC) and the International Union of Pure and Applied Physics (IUPAP). Its conclusions, duly endorsed by IUPAC and IUPAP, were published in the following three reports: 1. Criteria that must be satisfied for the discovery of a new chemical element to be recognThe Transfermium Working Group (TWG) was set up in 1986 under the joint auspices of the International Union of Pure and Applied Chemistry (IUPAC) and the International Union of Pure and Applied Physics (IUPAP). Its conclusions, duly endorsed by IUPAC and IUPAP, were published in the following three reports:1. Criteria that must be satisfied for the discovery of a new chemical element to be recogni

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The impact of torture on the distribution of psychiatric disorders among refugees is unknown.We surveyed a population-based sample of 418 tortured and 392 nontortured Bhutanese refugees living in camps in Nepal. Trained interviewers assessed International Classification of Diseases, 10th Revision (ICD-10) disorders through structured diagnostic psychiatric interviews.Except for male sex, history o

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Harvesting of wood for bioenergy purpose will probably increase in importance in the future, in order to replace fossil fuel. However, the environmental impact of increased harvesting might be considerable, e.g. on soil and water chemistry, biodiversity and long-term productivity, and in this study we investigate thresholds for sustainable harvesting volumes. The study is based on scientific revie

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This thesis employs a combination of quantitative and qualitative methods of linguistic analysis to investigate how business organizations use discourse as a strategic tool to build and repair stakeholders’ trust. In addition, the thesis makes a methodological contribution by developing a novel corpus annotation method that offers solutions to some of the most significant challenges involved in re

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N2O is currently the third most important anthropogenic greenhouse gas in terms of radiative forcing and its atmospheric mole fraction is rising steadily. To quantify the growth rate and its causes over the past decades, we performed a multi-site reconstruction of the atmospheric N2O mole fraction and isotopic composition using new and previously published firn air data collected from Greenland an

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Bacterial taxonomic community analyses using PCR-amplification of the 16S rRNA gene and high-throughput sequencing has become a cornerstone in microbiology research. To reliably detect the members, or operational taxonomic units (OTUs), that make up bacterial communities, taxonomic surveys rely on the use of the most informative PCR primers to amplify the broad range of phylotypes present in up-to

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The relationship between use of corticosteroids and fracture risk was estimated in a meta-analysis of data from seven cohort studies of similar to42,000 men and women. Current and past use of corticosteroids was an important predictor of fracture risk that was independent of prior fracture and BMD.

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Background: Calcipotriene and betamethasone dipropionate are topical treatments for psoriasis vulgaris. Their mode of action is different. Improved risk/benefit may result with concomitant use of the two compounds together. A new vehicle has been created with the objective of obtaining optimal stability of both calcipotriene and betamethasone dipropionate in the combination product. Objective. We

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Four whole- lake inorganic C-13 addition experiments were conducted in lakes of differing trophic status. Inorganic C-13 addition enriched algal carbon in C-13 and changed the delta C-13- DOC by + 1.5 parts per thousand to + 9.5 parts per thousand, depending on the specific lake. This change in delta C-13- DOC represented a significant input of algal DOC that was not completely consumed by bacteri

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Background: A medical treatment for urethral stricture (US) is not yet available. Objective: To evaluate if local injection of human adipose tissue-derived stem cells (hADSC) prevents urethral fibrosis in a rat model of US. Design, setting, and participants: Male rats were divided into three groups: sham, US, and hADSC (n = 12 each). Sham rats received a vehicle injection in the urethral wall. US

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São Francisco-kratonen i Sydamerika är en mycket gammal landmassa som bär spår av upprepade extensionella händelser då magmor från manteln transporterades uppåt och kristalliserade som diabas. Tidigare försök att åldersbestämma dessa gångbergarter är få och begränsade till radiometriska metoder som är känsliga för metamorfos och vittring. I denna studie har diabaser från Uauá-blocket och Curaçá-bäThe São Francisco Craton (SFC) is an Archaean craton that hosts a significant number of mafic intrusions. Previous attempts to date the igneous emplacement of these rocks are limited to a few dates of poor precision or comprise isotopic techniques known to be sensitive to alteration and low-grade metamorphism. Here, dykes from the Uauá Block and the Curaçá belt, in the northeastern portion of the

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The development of improved biomass pyrolysis models is vital for more accurate modelling and design of biomass conversion equipment. Such improved models must be based on reliable experimental data: biomass should be pyrolyzed at high heating rates and the reaction products should be measured using an on-line, non-intrusive method. Therefore, a heated grid reactor with heating rate of 300-600 K/s

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The role of inflammation in atherosclerotic disease is well established, but the role of autoantibodies against modified apolipoprotein (apo) B-100 remains unclear. The metabolic syndrome is associated with a proinflammatory state, a predominance of small dense low-density lipoprotein (LDL) particles, and an increased risk for atherosclerotic diseases. Previous studies have shown specific autoanti

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The Kruppel-like protein ZNF224 is a co-factor of the Wilms' tumor 1 protein, WT1. We have previously shown that ZNF224 exerts a specific proapoptotic role in chronic myelogenous leukemia (CML) K562 cells and contributes to cytosine arabinoside-induced apoptosis, by modulating WT1-dependent transcription of apoptotic genes. Here we demonstrate that ZNF224 gene expression is down-regulated both in

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Situated at the junction of Cognitive Semantics and Experimental Phenomenology, this study investigates how participants perceive the structure of 18 perceptual dimensions of opposites across the visual, auditory, tactile, gustatory and olfactory sensory modalities. The structures include three components: two poles (HIGH; LOW) and an intermediate (NEITHER HIGH NOR LOW). Participants were asked to

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Based on ethnographic observations, this article seeks to empirically illustrate how one of the main functions of the Smart City Expo in Barcelona in 2024 is to perform smartcity-as-ideology and its technological-solutionist stance that considerably narrows our understanding of urban problems and the solutions for it. The article introduces the Expo and describes our arrival and visit to the Expo.

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We report on the commissioning of an ultrafast timing diagnostic for measuring a time-offset signal between two different synchronized ultrashort light pulses. The method is based on sum-frequency generation in a nonlinear crystal. The setup is similar to an auto/cross-correlator setup. In this case, one of the beams is a relatively weak diagnostic beam of visible light from a bending magnet at Fe

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Movement disorders are common in inherited metabolic diseases (IMDs) and significantly impact quality of life. Unfortunately, they are often underrecognised by metabolic physicians. This study investigated whether a new screening tool improves recognition of movement disorders in IMD patients by non-neurologists. Internists and paediatricians specialized in IMDs assessed videos of 44 IMD patients

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A possible role for infections of the pregnant mother in the development of childhood acute leukemias and lymphomas has been suggested. However, no specific infectious agent has been identified. Offspring of 74,000 mothers who had serum samples taken during pregnancy and stored in a large-scale biobank were followed up to the age of 15 years (750,000 person years) through over-generation linkages