bluuebunny/crossref_metadata_2025_split huggingface dataset:
The Birth Prevalence of PKU in Populations of European, South Asian and Sub‐Saharan African Ancestry Living in South East England
Authors: Hardelid P. , Cortina‐Borja M. , Munro A. , Jones H. , Cleary M. , Champion M. P. , Foo Y. , Scriver C. R. , Dezateux C. 
Published: August 2007

SummaryPhenylketonuria (PKU) is an autosomal recessive inborn error of metabolism (OMIM 261600). Treatment with a low‐phenylalanine diet following early ascertainment by newborn screening prevents impaired cognitive development, the major disease phenotype in PKU. The overall birth prevalence of PKU in European, Chinese and Korean populations is ∼1/10,000. Since the human PAH locus contains PKU‐causing alleles and polymorphic core haplotypes that describe and corroborate an out‐of‐Africa range expansion in modern human populations, it is of interest to know the prevalence of PKU in different ethnic groups with diverse geographical origin. We estimated PKU prevalence in South East England, where a sizeable proportion of the population are of Sub‐Saharan African or South Asian ancestry. Over the period 1994 to 2004 167 children were diagnosed with PKU. Using birth registration and census data to derive denominators, PKU birth prevalence per 10,000 live births (95% Bayesian credible intervals) was estimated to be 1.14 (0.96–1.33) among white, 0.11 (0.02–0.37) among black, and 0.29 (0.10–0.63) among Asian ethnic groups. This suggests that PKU is up to an order of magnitude less prevalent in populations with Sub‐Saharan African and South Asian ancestry that have migrated to the UK.
A Novel Locus for Ectodermal Dysplasia of Hairs, Nails and Teeth Type Maps to Chromosome 18q22.1–22.3
Authors: Tariq M. , Chishti M. S. , Ali G. , Ahmad W. 
Published: August 2007

SummaryEctodermal dysplasias (EDs) are developmental disorders affecting tissues of ectodermal origin including hair, nails, teeth and sweat glands. Ectodermal dysplasia of hair, nails and teeth is a rare type of congenital disorder characterized by sparse and thin hair, dystrophic finger‐and toenails and missing and abnormal teeth. In an effort to understand the molecular basis of this form of ED a family of Pakistani origin with an autosomal recessive pattern of inheritance was ascertained from a remote region in Pakistan. The clinical features of the affected individuals included thin and fine hair on the scalp, dystrophic and flat nails, absent or sparse eyebrows and eyelashes, missing and abnormal teeth, and thin body hair. A human genome scan carried out using microsatellite markers mapped the disease locus in this family to chromosome 18q22.1–18q22.3. A maximum two‐point LOD score of 2.73 (θ= 0.0) was obtained at marker D18S541. Multipoint linkage analysis resulted in a maximum LOD score of 3.42 obtained with several markers, including D18S1125, ATA82B02, D18S848, D18S488, D18S1091, and D18S485, which supported the linkage. The linkage interval is flanked by markers D18S857 and D18S815, which corresponds to a region of 17.32 cM according to Rutgers combined linkage and physical map (build 36). This region covers 8.63 Mb according to the sequence‐based physical map. Three candidate genes, CDH7, CDH19 and ZNF407, from the linkage interval were sequenced and found to be negative for functional sequence variants. This study is the first step towards the identification of a gene involved in hair, nails and teeth type ED.
Electroencephalographic Characterization of Spike‐Wave Discharges in Cortex and Thalamus in WAG/Rij Rats
Authors: Sitnikova Evgenia , Van Luijtelaar Gilles 
Published: August 2007

Summary
Purpose: The waveform of spontaneous spike‐wave discharges (SWD) in the electroencephalogram (EEG) was delineated in the WAG/Rij rat model of absence epilepsy according to the definitions of clinical electroencephalography. We defined four elements in SWD based on the schema of Weir (1965): Spike 1 and 2, Positive Transient (PT), and Wave. The EEG patterns of generalized type I and local type II SWD in cortical and thalamic areas were analyzed.
Methods: EEGs were recorded in freely moving rats epidurally from different cortical regions and with deep electrodes from the specific and reticular thalamic nuclei. Grand average SWD waveforms were computed to assess spatiotemporal patterns of seizures.
Results: SWD I in the frontal cortex comprised of a large Spike 2 + Wave, and in the thalamus PT + Wave. Small transient spikes were associated with SWD I in the anterior‐middle part of the cortex. SWD II were found in the occipital cortex as a sequence of (occasional) Spike 1 + PT + Wave.
Conclusions: The EEG structure of SWD in WAG/Rij rats was comparable with that of epileptic patients, suggesting face validity of the WAG/Rij model. Fast transients spikes are an integrative part of SWD I. Time‐amplitude linkage between cortical and thalamic counterparts of SWD I suggests a complex spatiotemporal organization of SWD I. The thalamus sustained SWD I, but not SWD II.
Thermodynamic Characterization of the Eutectic Phase Mixture NaNbO3/Na3NbO4. II: Solid‐State Electrochemical Investigation
Authors: Näfe Helfried , Amin Ruhul , Aldinger Fritz 
Published: August 2007

The difference in the standard Gibbs‐free energies of NaNbO3 and Na3NbO4 has been determined by means of two electrochemical approaches based on potentiometric solid oxide electrolyte galvanic cells. The results are consistent with each other and prove the phase equilibrium between the niobates to be univariant. Quantitatively, the data can be represented by (525°–700°C)
image

The results are in between a wide range spanned by estimated literature data and are close to the findings of a previous manometric study.
Superior nuclear receptor selectivity and therapeutic index of methylprednisolone aceponate versus mometasone furoate
Authors: Mirshahpanah Parham , Döcke Wolf‐Dietrich , Merbold Udo , Asadullah Khusru , Röse Lars , Schäcke Heike , Zollner Thomas M. 
Published: August 2007

Abstract: Although introduced more than 50 years ago, topical glucocorticoids are still the first line therapy for many inflammatory skin disorders such as atopic eczema, contact dermatitis and many others. Recently, significant improvements have been made to optimize the ratio of desired to unwanted effects. While with early compounds such as triamcinolone, topical side effects such as skin atrophy and telangiectasias can be observed rather frequently, newer drugs such as methylprednisolone aceponate or mometasone furoate have a significantly improved therapeutic index. The present study compared these two modern topical glucocorticoids, which possess the highest therapeutic index currently found, in terms of nuclear receptor selectivity in vitro and induction of the most important local side effects (skin atrophy and telangiectasias) in a relevant rodent model in vivo. We demonstrate that methylprednisolone aceponate displays higher specificity in nuclear receptor binding compared with mometasone furoate. Methylprednisolone aceponate was also markedly superior in terms of minimizing induction of skin atrophy or telangiectasias when compared with mometasone furoate. Based on these observations, methylprednisolone aceponate is expected to have a greater therapeutic index as compared with mometasone furoate, at least in the test systems used here. The degree to which this observation may translate into a clinical setting requires confirmation.
Adoption of Food Safety and Quality Controls: Do Firm Characteristics Matter? Evidence from the Canadian Food Processing Sector
Authors: Herath Deepananda , Hassan Zuhair , Henson Spencer 
Published: August 2007

This study explores the association between the adoption of food safety and quality assurance practices in the Canadian food processing sector and firm characteristics. A conceptual model is developed recognizing that the relative importance of a firm's incentives to adopt enhanced food safety and quality assurance practices is influenced by the firm's characteristics and activities. Binomial logit models are estimated to explore the association between adoption of various combinations of food safety and quality assurance practices including hazard analysis and critical control point (HACCP). The findings show that the adoption of food safety and quality practices varies widely between individual firms according to, among others, firm size, country of ownership and control, level of innovativeness, level of export orientation, forms of food safety inspection, and the subsector in which the firm operates. Incentives of being able to access foreign markets play an important role in influencing HACCP adoption. La présente étude analyse le lien entre l'adoption de pratiques visant la salubrité et l'assurance de la qualité des aliments au sein de l'industrie canadienne de la transformation et les caractéristiques des entreprises. Nous avons élaboré un modèle conceptuel reconnaissant que l'importance relative des incitatifs qui motivent une entreprise à adopter des pratiques améliorées en matière de salubrité et d'assurance de la qualité des aliments est influencée par les caractéristiques et les activités de l'entreprise. Nous avons estimé des modèles logit binomiaux pour examiner le lien entre l'adoption de diverses combinaisons de pratiques visant la salubrité et l'assurance de la qualité des aliments, y compris les systèmes HACCP (analyse des risques et maîtrise des points critiques). Les résultats ont montré que l'adoption de ces pratiques variait considérablement d'une entreprise à l'autre en fonction, entre autres, de la taille de l'entreprise, du pays de propriété et de contrôle, du degré d'innovation, du degré de vocation exportatrice, des programmes d'inspection de la salubrité des aliments et du sous‐secteur dans lequel une entreprise évolue. La perspective de pénétrer les marchés étrangers joue un rôle important dans l'adoption de systèmes HACCP.
Vertical Economies and the Structure of U.S. Hog Farms
Authors: Azzam Azzeddine , Skinner Cari S. 
Published: August 2007

Scale economies are often touted as the factor behind the trend in the structure of the U.S. hog industry toward fewer and larger hog farms. However, since hog production is multistage and farms either integrate or separate the stages, the appropriate measures are multistage economies. In theory, a smaller and, presumably, high‐cost operation, by the standards of single stage/output scale economies, may still be cost‐competitive if it enjoys multistage economies — that is if vertical scope economies more than offset stage‐specific scale diseconomies. Whether that holds in practice remains heretofore unexplored in the agricultural economics literature. Using a unique data set on hog farms in the U.S. Midwest, we estimate a multistage cost function and provide the first‐ever estimates of multistage economies, stage‐specific economies, and vertical scope economies in hog production. Les économies d'échelle sont souvent soupçonnées d'être le facteur qui influence la tendance observée dans la structure de l'industrie porcine aux États‐Unis, soit des porcheries moins nombreuses mais de plus grande taille. Cependant, comme la production porcine est une production à plusieurs stades et que les producteurs peuvent choisir d'intégrer ces stades ou d'exploiter un seul stade, les mesures appropriées sont les économies d'échelle liées aux stades multiples. En théorie, une petite porcherie, qui a probablement des coûts élevés selon les critères des économies d'échelle liées à un seul stade, peut tout de même être concurrentielle quant aux coûts si elle profite des économies d'échelle liées aux stades multiples, c'est‐à‐dire si les économies de gamme verticales font plus que compenser les déséconomies d'échelle liées à un seul stade. La vérification de cette théorie dans la pratique demeure, jusqu'ici, inexploitée dans la littérature agroéconomique. En utilisant un ensemble de données sur des porcheries situées dans le Midwest américain, nous avons estimé une fonction de coût lié aux stades multiples et nous présentons les premières estimations d'économies d'échelle liées aux stades multiples, d'économies d'échelle liées à un seul stade et d'économies de gamme verticales dans la production porcine.
Accounting for Risk and Stability in Technology Adoption
Authors: Engler‐Palma Alejandra , Hoag Dana L. 
Published: August 2007

The reasons for sluggish adoption of long‐run agricultural systems are not well understood. Researchers have identified risk and uneven cash flows as two likely culprits, yet the literature has done little to investigate their impacts simultaneously. We explore the unique influence of risk, risk preference, stability, and stability preferences on the adoption of long‐run investments. We developed risk‐stability‐segregated expected utility (RSSEU) to disentangle risk from stability and then compute the impacts of risk, stability, and preferences over a range of values found in previous studies. Results clearly demonstrate that adoption decisions are influenced differentially by risk and stability. An unstable income can overwhelm the risk effect or visa versa, depending on a person's preferences for risk and stability. Disentangling risk and stability could be very important if economists are to understand how decisions are made. For example, we found that the impact of risk on individual behavior is very low when the expected income flow is unstable. In this case, policies that smooth expected income over time will be more effective than ones that reduce risk. In contrast, risky technologies with a stable income path are more appropriately addressed with instruments like insurance or facilitating futures markets. Les raisons de la lente adoption des systèmes agricoles à long terme ne sont pas bien comprises. Selon certains chercheurs, le risque et les flux de trésorerie irréguliers sont deux facteurs probables; toutefois peu de travaux se sont penchés sur leur impact simultané. Nous avons exploré l'influence du risque, des préférences pour le risque, de la stabilité et des préférences pour la stabilité sur l'adoption d'investissements à long terme. Nous avons élaboré une espérance d'utilité distincte risque‐stabilité(risk‐stability‐segregated expected utility) pour dégager le risque de la stabilité et pour calculer l'impact du risque, de la stabilité et des préférences à partir d'une série de valeurs tirées d’études antérieures. Les résultats ont clairement montré que les décisions d'adoption sont influencées différemment par le risque et la stabilité. Un revenu instable peut accabler l'effet du risque ou vice versa, selon les préférences d'une personne pour le risque et pour la stabilité. Si les économistes veulent comprendre de quelles façons les décisions sont prises, il serait important de dégager le risque de la stabilité. Par exemple, nous avons observé que l'impact du risque sur le comportement individuel est très faible lorsque les flux de revenus sont instables. Dans ce cas, les politiques qui adoucissent les revenus prévus au fil du temps seront plus efficaces que celles qui diminuent le risque. En revanche, des instruments tels que les assurances et les marchés à termes sont des moyens plus efficaces lorsque les technologies comportent des risques et que les revenus sont stables.
Clay Aquitards as Archives of Holocene Paleoclimate: δ18O and Thermal Profiling
Authors: Hendry M. Jim , Woodbury A.D. 
Published: August 2007

AbstractPaleoclimatic conditions in the Holocene were reconstructed from a detailed vertical profile of pore water δ18O and a series of downhole thermal profiles at a thick, hydrogeologically simple, aquitard research site in the Northern Great Plains of Saskatchewan. Reconstructions were obtained using the theory of one‐dimensional diffusive transport and an empirical Bayesian inversion technique. Inversion of the δ18O profile shows that input signal consists of a sudden increase of +6‰ (corresponding to a temperature increase of about 18°C) at about 12,000 years before present (BP), after which no measurable change in δ18O is apparent to present day. This research shows, at this location, that there is no evidence of large amplitude temperature shifts in the Holocene and supports the commonly used assumption in ground water studies of constant atmospheric δ18O values throughout the Holocene. Inversion of the temperature profiles suggests the ground surface temperature increased primarily in the last half of the 20th century, with a peak temperature (about 3°C) circa 1990. For both profiles, the ability to resolve historical variability decays rapidly with time. For the temperature profiles, the decay in resolution precluded the development of reliable estimates of climatic conditions prior to about 1950 and, in the case of the δ18O profile, it prevented the precise definition of climate changes (e.g., Hypsithermal and Little Ice Age) in the Holocene.
Modeling Transient Discharge into a Tunnel Drilled in a Heterogeneous Formation
Authors: Perrochet Pierre , Dematteis Antonio 
Published: August 2007

AbstractAn analytical model is developed to predict transient discharge flow into a tunnel drilled at various speeds through a heterogeneous formation. This model relies on simplifying assumptions commonly enforced in hydrogeologic engineering, and combines the convolution and superposition principles to account for composite sections with arbitrary parametric contrasts. An application to the data monitored during the exploratory drilling of an Alpine tunnel confirms the validity of the approach.
Three‐Dimensional Flow in the Storative Semiconfining Layers of a Leaky Aquifer
Authors: Sepúlveda Nicasio 
Published: August 2007

AbstractAn analytical solution for three‐dimensional (3D) flow in the storative semiconfining layers of a leaky aquifer fully penetrated by a production well is developed in this article to provide a method from which accurate hydraulic parameters in the semiconfining layers can be derived from aquifer test data. The analysis of synthetic aquifer test data with the 3D analytical solution in the semiconfining layers provided more accurate optimal hydraulic parameters than those derived using the available quasi‐two‐dimensional (2D) solution. Differences between the 3D and 2D flow solutions in the semiconfining layers become larger when a no flow boundary condition is imposed at either at the top of the upper semiconfining layer or at the bottom of the lower semiconfining layer or when the hydraulic conductivity ratio of the semiconfining layer to the aquifer is larger than 0.001. In addition, differences between the 3D and 2D flow solutions in the semiconfining layers are illustrated when the thickness ratio of the semiconfining layer to the aquifer is changed. Analysis of water level data from two hypothetical and one real aquifer test showed that the 3D solution in the semiconfining layers provides lower correlation coefficients among hydraulic parameters than the 2D solution.
A Simple Pore Water Hydrogen Diffusion Syringe Sampler
Authors: Vroblesky Don A. , Chapelle Francis H. , Bradley Paul M. 
Published: August 2007

AbstractMolecular hydrogen (H2) is an important intermediate product and electron donor in microbial metabolism. Concentrations of dissolved H2 are often diagnostic of the predominant terminal electron‐accepting processes in ground water systems or aquatic sediments. H2 concentrations are routinely measured in ground water monitoring wells but are rarely measured in saturated aquatic sediments due to a lack of simple and practical sampling methods. This report describes the design and development (including laboratory and field testing) of a simple, syringe‐based H2 sampler in (1) saturated, riparian sediments, (2) surface water bed sediments, and (3) packed intervals of a fractured bedrock borehole that are inaccessible by standard pumped methods.
Deep Air Sparging—15 to 46 m beneath the Water Table
Authors: Klinchuch Leslie A. , Goulding Nigel , James Steven R. , Gies Jerehmy J. 
Published: August 2007

AbstractAir sparging can be successfully applied to deep aquifers to remediate significant hydrocarbon mass in order to ultimately reach a point at which monitored natural attenuation can manage dissolved phase plumes. Air sparging is typically applied in shallow ground water conditions, less than 15 m beneath the water table (bwt). Deep air sparging, 15 to 46 m bwt, is more challenging but a viable alternative for treatment of residual petroleum hydrocarbons and dissolved phase constituents within a thick smear zone. An application to remediate an aquifer impacted by gasoline‐range petroleum hydrocarbons at depths greater than 76 m below ground surface (bgs) and up to 40 m thick (vertically) is reported here. Deep air sparging at 15, 31, and 46 m bwt was assessed using diagnostic tools involving flow rate, pressure, tracer gas, water table elevation, vapor concentration, and ground water chemistry measurements. The diagnostic tools identified preferential flow directions and a maximum depth of effective air injection. The full‐scale deep air sparging/soil vapor extraction (SVE) system removed more than 150,000 kg of hydrocarbon mass at the study site over a 3‐year period and ultimately greater than 100,000 kg/year with system expansion.
Comparison of Factorial and Scenario Analysis Methods for Assessing Uncertainty in the Design of Permeable Reactive Barriers
Authors: Painter Brett D.M. , Milke Mark W. 
Published: August 2007

AbstractThe method chosen to incorporate uncertainty into remediation design can have a significant effect on the feasibility and potential success of a remediation project. Three methods for analyzing uncertainty are explored to better allow their application to optimization of remediation designs: scenario analysis combined with one‐way sensitivity analysis, factorial analysis, and fractional factorial analysis. The three methods are analyzed using a deterministic optimization model for design of a permeable reactive barrier (PRB) to remediate nitrate contamination. Although the combined scenario/one‐way sensitivity approach is simpler than the factorial approaches, it has the comparative disadvantage of missing potentially important interactions between design inputs. Fractional factorial analysis is shown to have the added advantage of being useful in screening the importance of a number of model inputs without a large number of computer experiments. Hypothetical examples highlight the likely importance of uncertainty in hydraulic conductivity when choosing the optimal PRB design specification and the potential interaction of this input and the incoming pollutant concentration.
Combined Treatment of Perchlorate and RDX in Ground Water Using a Fluidized Bed Reactor
Authors: Fuller Mark E. , Hatzinger Paul B. , Condee Charles W. , Togna A. Paul 
Published: August 2007

AbstractA bench‐scale study was conducted to assess the effectiveness of biological treatment of comingled perchlorate and hexahydro‐1,3,5‐trinitro‐1,3,5‐triazine (RDX) in ground water using fluidized bed reactors (FBRs). In addition, the ability of FBRs to treat low concentrations of perchlorate was examined. Pilot‐scale FBRs were fed either acetic acid or molasses as an electron donor to facilitate simultaneous perchlorate and RDX reduction. The acetic acid‐fed FBR consistently removed perchlorate from an influent level of 100 μg/L to below 6 μg/L, and values below the method detection limit of 1 μg/L occurred approximately 70% of the time. RDX breakthrough from all FBRs was minimal due to sorption of the explosive on the granulated activated carbon media, but mass balance calculations indicated that more than 99.5% of the total RDX entering the acetic acid‐fed FBR was biologically degraded. Approximately 80% of the RDX fed to the control FBR (i.e., no substrate addition) was accounted for at the end of the study; the remainder was assumed to have been degraded. Molasses was much less effective than acetic acid as an electron donor for treatment of perchlorate and RDX. This work demonstrates that combined treatment of perchlorate and RDX is feasible using FBR technology, and that very low levels of perchlorate (less than 100 μg/L) can be effectively treated in an FBR.