bluuebunny/crossref_metadata_2025_split huggingface dataset:
Neurobehavioral Profile and Brain Imaging Study of the 22q13.3 Deletion Syndrome in Childhood
Authors: Philippe Anne , Boddaert Nathalie , Vaivre-Douret Laurence , Robel Laurence , Danon-Boileau Laurent , Malan Valérie , de Blois Marie-Christine , Heron Delphine , Colleaux Laurence , Golse Bernard , Zilbovicius Monica , Munnich Arnold 
Published: July 2008

OBJECTIVE. The 22q13.3 deletion syndrome (Online Mendelian Inheritance in Man No. 606232) is a neurodevelopmental disorder that includes hypotonia, severely impaired development of speech and language, autistic-like behavior, and minor dysmorphic features. Although the number of reported cases is increasing, the 22q13.3 deletion remains underdiagnosed because of failure in recognizing the clinical phenotype and detecting the 22qter deletion by routine chromosome analyses. Our goal is to contribute to the description of the neurobehavioral phenotype and brain abnormalities of this microdeletional syndrome.
METHODS. We assessed neuromotor, sensory, language, communication, and social development and performed cerebral MRI and study of regional cerebral blood flow measured by positron emission tomography in 8 children carrying the 22q13.3 deletion.
RESULTS. Despite variability in expression and severity, the children shared a common developmental profile characterized by hypotonia, sleep disorders, and poor response to their environment in early infancy; expressive language deficit contrasting with emergence of social reciprocity from ages ∼3 to 5 years; sensory processing dysfunction; and neuromotor disorders. Brain MRI findings were normal or showed a thin or morphologically atypical corpus callosum. Positron emission tomography study detected a localized dysfunction of the left temporal polar lobe and amygdala hypoperfusion.
CONCLUSIONS. The developmental course of the 22q13.3 deletion syndrome belongs to pervasive developmental disorders but is distinct from autism. An improved description of the natural history of this syndrome should help in recognizing this largely underdiagnosed condition.
A Prospective, Cross-sectional Survey Study of the Natural History of Niemann-Pick Disease Type B
Authors: McGovern Margaret M. , Wasserstein Melissa P. , Giugliani Roberto , Bembi Bruno , Vanier Marie T. , Mengel Eugen , Brodie Scott E. , Mendelson David , Skloot Gwen , Desnick Robert J. , Kuriyama Noriko , Cox Gerald F. 
Published: July 2008

OBJECTIVE. The objective of this study was to characterize the clinical features of patients with Niemann-Pick disease type B and to identify efficacy end points for future clinical trials of enzyme-replacement therapy.
METHODS. Fifty-nine patients who had Niemann-Pick disease type B, were at least 6 years of age, and manifested at least 2 disease symptoms participated in this multicenter, multinational, cross-sectional survey study. Medical histories; physical examinations; assessments of cardiorespiratory function, clinical laboratory data, and liver and spleen volumes; radiographic evaluation of the lungs and bone age; and quality-of-life assessments were obtained during a 2- to 3-day period.
RESULTS. Fifty-three percent of the patients were male, 92% were white, and the median age was 17.6 years. The R608del mutation accounted for 25% of all disease alleles. Most patients initially presented with splenomegaly (78%) or hepatomegaly (73%). Frequent symptoms included bleeding (49%), pulmonary infections and shortness of breath (42% each), and joint/limb pain (39%). Growth was markedly delayed during adolescence. Patients commonly had low levels of platelets and high-density lipoprotein, elevated levels of low-density lipoprotein, very-low-density lipoprotein, triglycerides, leukocyte sphingomyelin, and serum chitotriosidase, and abnormal liver function test results. Nearly all patients had documented splenomegaly and hepatomegaly and interstitial lung disease. Patients commonly showed restrictive lung disease physiology with impaired pulmonary gas exchange and decreased maximal exercise tolerance. Quality of life was only mildly decreased by standardized questionnaires. The degree of splenomegaly correlated with most aspects of disease, including hepatomegaly, growth, lipid profile, hematologic parameters, and pulmonary function.
CONCLUSIONS. This study documents the multisystem involvement and clinical variability of Niemann-Pick B disease. Several efficacy end points were identified for future clinical treatment studies. Because of its correlation with disease severity, spleen volume may be a useful surrogate end point in treatment trials, whereas biomarkers such as chitotriosidase also may play a role in monitoring patient treatment responses.
Maternal Smoking During Pregnancy, Other Prenatal and Perinatal Factors, and the Risk of Legg-Calvé-Perthes Disease
Authors: Bahmanyar Shahram , Montgomery Scott M. , Weiss Rüdiger J. , Ekbom Anders 
Published: July 2008

OBJECTIVE. The causes of Legg-Calvé-Perthes disease are largely unknown, but this pediatric disease seems to result from interruption of the blood supply to the proximal femur and is considered a vascular disease. Because maternal smoking during pregnancy influences fetal development and is associated with cardiovascular diseases in offspring, we hypothesized that this exposure is a risk for Legg-Calvé-Perthes disease and also investigated other markers of impaired fetal development and early-life exposures.
MATERIALS AND METHODS. The Swedish Inpatient Register identified 852 individuals with a diagnosis of Legg-Calvé-Perthes disease from 1983 to 2005, individually matched by year of birth, age, sex, and region of residence with 4432 randomly selected control subjects. Linkage with the Swedish Medical Birth Register provided information on prenatal factors, including maternal smoking. Conditional logistic regression examined associations of maternal smoking during pregnancy and the other measures with the risk of Legg-Calvé-Perthes disease in offspring, adjusted for socioeconomic index and other potential confounding factors.
RESULTS. Maternal smoking during pregnancy was associated with an increased Legg-Calvé-Perthes disease risk, and heavy smoking was associated with a risk increase of almost 100%. Very low birth weight and cesarean section were independently associated with ∼240% and 36% increases in the risk of Legg-Calvé-Perthes disease, respectively.
CONCLUSION. Maternal smoking during pregnancy and other factors indicated by impaired fetal development may be associated with an increased risk of Legg-Calvé-Perthes disease.
Impacts of climate change and establishing a vegetation cover on water erosion of contaminated spoils for two contrasting United Kingdom regional climates: A case study approach
Authors: De Munck Cécile S , Hutchings Tony R , Moffat Andy J 
Published: July 2008

Abstract
This study examines how pollutant linkage of contaminants will be influenced by predicted changes in precipitation and subsequent rainfall erosion of soils and spoils in the United Kingdom during the 21st century. Two contrasting regional climates were used in conjunction with 2 extreme emissions scenarios (low and high greenhouse gas emissions) to run the Revised Universal Soil Loss Equation 2 (RUSLE2) model for a case study that represents a high risk of pollutant linkage through water erosion. Results for the 2 scenarios and the 2 regions showed a significant and gradual increase in erosion rates with time as a consequence of climate change, by up to 32% for the southwest and 6.6% for the southeast regions by the 2080s. Revegetation of the site showed a dramatic reduction in predicted future amounts of sediment production and subsequent contaminant movement, well below existing levels. Limitations and future improvements of the methodology are discussed.
A Web-Based Integrated Project-Management System Supporting Teamworking and Decision Making on Field-Development Projects
Authors: Piantanida M. , Rossi P. , Gaudioso G. 
Published: July 2008

Summary
Following the re-engineering of the Eni Development Management System (DMS), which occurred in early 2004, Eni developed an information technology (IT) integrated Project Management System (PMS) to support field-development projects.
The DMS promotes a stage-and-gate approach to the development of a field, starting from assessment of the value of the field, identification of the viable development scenarios, definition of the selected development strategy, and execution of the project (including the construction of the facilities and the handover to operations). At the end of each stage, a predefined set of data and documents are prepared by the project team to be submitted to a decision gate to support an informed decision by Eni's executives and authorize the start of the next phase.
The IT system was developed with a Web Portal technology and it assists in the following:
The multidisciplinary team involved in the project (geologists, reservoir and drilling engineers, facility and construction specialists, and HSE consultants) on a daily basis. In fact, it provides web-based tools to share and verify project documents; develop and control the schedule baseline of the project; automatically track cost expenditures against budget values; document and review risks and lessons learned; and manage milestones, issues, and project changes. The executives involved in decision making. In fact, it provides a high-level dashboard with an integrated visualization of the last updated schedule and cost figures for the project, as well as direct access to the main project documents relevant for the decision gate of the current project phase.
The system integrates the document-management capabilities of Lotus Domino, extends the planning and control functions of MS Project, automatically imports the budget/actual expenditures from the SAP R3 Enterprise Resource Planning system, and authorizes and profiles users according to their role. It has been used to support 116 project phases across 82 projects.
Rheology of Gas-to-Liquid Products, Alaska North Slope (ANS) Crude Oil, and Their Blends for Transportation Through the Trans-Alaska Pipeline System (TAPS)
Authors: Inamdar A. Abhijeet , Chukwu Godwin A. , Khataniar Santanu , Patil Shirish L. , Dandekar Abhijit Y. 
Published: July 2008

Summary
It is projected that by the year 2015, Alaska North Slope (ANS) crude-oil production will decline to such a level (200,000 to 400,000 B/D) that there will be a critical need for pumping additional liquids through the pipeline to maintain economic operation of the Trans-Alaska Pipeline System (TAPS). The estimated proven and recoverable gas reserves of 38 trillion cubic feet (Tcf) on the ANS can be converted to a high-premium liquid product using gas-to-liquids (GTL) technology. The GTL product can be transported from the ANS to the southern port of Valdez, Alaska through the TAPS.
One of the proposed modes of transportation of the GTL products from ANS to market is by commingling it with crude oil as a single phase and pumping the mixture through the TAPS. This mode of transportation changes the properties of the GTL as well as the crude oil. The focus of this work, therefore, was to determine the physical and chemical properties of GTL and its blends with ANS crude oil, analyze the energy requirements for flowing the mixture through TAPS, and determine the optimum blend ratio to maximize the transportation economics. Four blends of GTL/crude-oil mixture in the ratios of 1:1, 1:2, 1:3, and 1:4 were prepared for their rheological evaluation and pressure drops at different temperature conditions.
The results of the analysis show that flow behavior of the GTL and GTL blends are temperature sensitive. Viscosity and density of the blends were found to decrease with increasing amounts of GTL and increasing temperature. The optimum GTL/crude-oil blend ratio of 1:2.5 (28%:72%) was determined to take full advantage of the economics of commingled flow of GTL/crude-oil mixture through the TAPS.
The Cost of Carbon Capture and Storage in the Perth Region
Authors: Allinson W. G. , Dunsmore R. E. , Neal P. R. , Ho M. T. 
Published: July 2008

Summary
This paper describes and reports the results of analyses of the costs of carbon dioxide (CO2) capture and storage in southwest Western Australia. We analyze the costs of capturing approximately 22 million tonnes per year of CO2 and injecting it into one of three storage sites: the Gage sandstone offshore Perth, the Dongara depleted gas field, and the Neocomian subcrop offshore Geraldton.
The central estimates obtained are that the real cost to capture, transport, and inject CO2 for a period of 25 years is between 59 and 63 Australian dollars (AUD) per tonne of CO2 avoided (in 2005 terms), depending on the location of the injection site.
The sensitivity of these cost estimates to changes in key assumptions is also analyzed. These sensitivity analyses show that the costs of carbon capture and storage (CCS) can vary significantly with different assumptions for key variables.
Integrated Flow-Assurance Modeling of the BP Angola Block 18 Western Area Development
Authors: Watson M. J. , Hawkes N. J. , Pickering P. F. , Elliott J. , Studd L. W. 
Published: July 2008

Summary
As oilfield developments become more challenging and economically challenging, flow assurance has become crucial to the feasibility of projects. Consequently, flow-assurance issues such as hydrates or wax deposition must now be considered early in concept selection. Modern numerical methods, coupled with the latest software engineering techniques, now allow the rigorous calculation of multiphase thermal hydraulic behavior in an integrated asset model (IAM) on time scales acceptable for concept selection.
This paper describes the application of a new IAM tool to analyze options for the development of fields in the western part of BP's Angolan deepwater Block 18. Novel aspects include the embedding of field scheduling rules such that the drilling schedules were predicted automatically from the model. In addition, different field architectures were considered including tubing and pipeline sizes, looping of pipelines and subsea multiphase boosting, and the impact on production rates and drilling schedules was quantified. Furthermore, the option to tie back to the planned Greater Plutonio floating production storage and offloading (FPSO) vessel was also modeled with the forecast ullage profile being imposed on production from the new fields. All calculations were performed using rigorous multiphase thermal-hydraulic models allowing flow-assurance constraints to be analyzed simultaneously.
Treatment of Seawater With Cartridge Filtration–A Field Trial
Authors: Lee Joseph M. , Frankiewicz Ted , Walsh John 
Published: July 2008

Summary
In offshore operations, seawater is often injected to maintain reservoir pressure for enhanced oil recovery. A sulfate-reducing membrane (SRM) is typically used to reduce the sulfate content in the seawater from 2900 mg/L to less than 50 mg/L to prevent souring of the reservoir by sulfate reducing bacteria and formation of barium and strontium sulfate scale when seawater is mixed with formation water. A two-cartridge in-series filtration pilot unit installed on a deepwater platform in the Gulf of Mexico (GOM) has demonstrated that cartridge filtration can be used upstream of the SRM to pretreat seawater to meet the SRM feed seawater specification of a silt density index (SDI) &lt;5 and a nephelometric turbility unit (NTU) &lt; 1. Scanning electron microscopy/energy dispersive spectroscopy (SEM/EDS) was used to determine the composition of the material deposited on the surface of a used cartridge filter. From the SEM/EDS results, it was concluded that the filters were blocked predominantly by coccoliths (calcium compounds), diatoms (silicon compounds), iron oxide corrosion products, and broken, crushed marine debris, that formed a fine-grained membrane cake on the filters. It was found that a two-filter in-series configuration is a better seawater filtration system than using a single filter if seawater with an SDI &lt;3.5 is required. With the installation of a 50-micron nominal filter upstream of a 10-micron absolute filter, the service life of a 10-micron filter was increased by 20 times.
Effects of High Oil Viscosity on Oil/Gas Flow Behavior in Horizontal Pipes
Authors: Gokcal B. , Wang Q. , Zhang H. -Q. , Sarica C. 
Published: July 2008

Summary
Oil/gas pipe flows are expected to exhibit significantly different behavior at high oil viscosities. Effects of high-viscosity oil on flow pattern, pressure gradient, and liquid holdup are experimentally observed, and differences in flow behavior of high- and low-viscosity oils are identified. The experiments are performed on a flow loop with a test section of 50.8-mm ID and 18.9-m-long horizontal pipe. Superficial liquid and gas velocities vary from 0.01 to 1.75 m/s and from 0.1 to 20 m/s, respectively. Oil viscosities from 0.181 to 0.587 Pa·s are investigated. The experimental results are used to evaluate the performances of existing models for flow pattern and hydrodynamics predictions. Comparisons of the data with the existing models show significant discrepancies at high oil viscosities. Possible reasons for these discrepancies are carefully examined. Some modifications are identified and implemented to the closure relationships employed in the Zhang et al. (2003) model. After these modifications, the model predictions provide better agreement with experimental results for flow pattern transition, pressure gradient, and liquid holdup.
An Investigative Study of Potential Emulsion Problems Before Field Development
Authors: Kokal Sunil , Al-Ghamdi Abdullah , Meeranpillai N. S. 
Published: July 2008

Summary
Before the development of a field, it is worthwhile to determine the characteristics of crude-oil emulsions. This may be even more important if the wells are to be equipped with electricalsubmersible pumps (ESPs) which can create a shearing environment resulting in tight emulsions. One such study was conducted to determine the characteristics of emulsions from a cluster of oil fields planned for development. The proposed development is to produce from three fields and several different reservoirs. The central processing facility (CPF) will process ≈500,000 B/D oil and 50,000 B/D of water. Previous studies determine the impact of ESP on emulsions and on the ESP design (horsepower) and the possibility of demulsifier injection downhole. The emulsion properties are also necessary for the design of central wet crude handling facilities. A series of emulsion tests was conducted with crudes from different reservoirs at bottomhole and surface temperatures, range of water cuts, and shearing conditions. The results are provided in terms of "relative" tightness of emulsions from different reservoirs. The viscosities of various emulsions were also determined. The results indicate that demulsifier injection facilities will be needed at the wet crude handling facilities and possibly in wells from one reservoir that has a high asphaltenic and viscous crude. This paper provides a framework of laboratory emulsions studies that can provide valuable information for the design of CPFs before they are built. This paper also discusses some practical aspects of demulsifier treatment programs, including demulsifier selection and optimization and methods to prevent emulsion problems.
Advances in Deepwater-Pipeline Route Selection—A Gulf of Mexico Case Study
Authors: Tootill N. P. , Vandenbossche M. P. , Morrison M. L. 
Published: July 2008

Summary
The selection of pipeline routes and the location of the associated tie-in structures require detailed knowledge of the seabed conditions and shallow geology. The areas along the routes and the areas regionally around the routes must be accurately surveyed. This is especially important for the complex geology and seabed topography found in the deepwater Gulf of Mexico.
The Mardi Gras project used an autonomous underwater vehicle (AUV) to provide high-quality survey data. These data allowed the project to make decisions with a high degree of confidence regarding pipeline routing, ultimately leading to successful installation of the Mardi Gras pipelines.
Harmonics in Offshore Electrical Power Systems
Authors: Kumar Balesh , Sethi V. K. , Bhattacharjee Saibal 
Published: July 2008

Summary
Recently, electrical submersible pumps (ESPs) have become widely used in depleting oil fields with low pressure (dead crude) and in deep wells with marginal pressure to achieve oil production. These types of ESPs are variable-speed drives (VSDs) and operated at variable voltages and frequencies to optimize production. The introduction of a large amount of ESPs driven by VSDs into an electrical power system of an offshore oil field that is electrically weak leads to distortion of voltage and current profile beyond acceptable limits, thereby producing harmonics. The more distortion there is in the waveforms, the higher the harmonics.
The concept of harmonics, along with their impact and mitigation measures to be exercised in an offshore power system, is discussed in this paper. Furthermore, a method statement is provided for sizing the active harmonic filter (AHF). An attempt is made to arrive at an empirical relationship for an AHF rating in kVAR for offshore electrical power systems, but it is not yet well documented. A case study of harmonic analysis for an integrated offshore field in the South China Sea has been discussed.
The information contained in this paper is useful for design engineers as well as operators handling electrical power systems for offshore oil fields with significant amounts of nonlinear loads or VSD loads. This enables them to predict the requirement of harmonic treatment in offshore electrical power systems. It also helps them to take early action at the planning/design stage to avoid probable damage to equipment or loss of power resulting from harmonics and to conduct detailed harmonic analysis.
Leak Detection for Gas and Liquid Pipelines by Online Modeling
Authors: Wang Shouxi , Carroll John J. 
Published: July 2008

Summary
The leakage of hydrocarbon products from a pipeline not only represents the loss of natural resources, but it also is a serious and dangerous environmental pollution and potential fire disaster. Quick awareness and accurate location of the leak event are important to reduce losses and avoid disaster.
A leak-detection method using transient modeling is introduced in this paper. This method is suitable for both gas and liquid pipelines, with comprehensive consideration of the transient-flow features of compressible flows and stochastic processing and noise filtering of the meter readings. The correlations for diagnosing the leak location and amount are derived on the basis of the online real-time observation and the readings of pressure, temperature, and flow rate at both ends of the pipeline. As an online real-time system, great attention has been paid to the stochastic processing and noise filtering of the meter readings and the models to reduce the impact of signal noise. It is also essential for the robust realtime pipeline observer to have the self-study and adjustment abilities needed to respond to the large varieties of pipeline configuration, pipeline operation conditions, and fluid properties.
Real application cases are presented here to demonstrate this leak-detection method. For example, in the leak detection of a crude-oil pipeline of 34.5 km long and 219 mm (nominal diameter), this method located the leak at 16.6 km from the pipe-line upstream end, which is only 0.6 km away from the actual leak location.