bluuebunny/crossref_metadata_2025_split huggingface dataset:
Alstrom syndrome (OMIM 203800): a case report and literature review
Authors: Joy Tisha , Cao Henian , Black Graeme , Malik Rayaz , Charlton-Menys Valentine , Hegele Robert A , Durrington Paul N 
Published: December 2007

Abstract

Background
Alstrom syndrome (AS) is a rare autosomal recessive disease characterized by multiorgan dysfunction. The key features are childhood obesity, blindness due to congenital retinal dystrophy, and sensorineural hearing loss. Associated endocrinologic features include hyperinsulinemia, early-onset type 2 diabetes, and hypertriglyceridemia. Thus, AS shares several features with the common metabolic syndrome, namely obesity, hyperinsulinemia, and hypertriglyceridemia. Mutations in the ALMS1 gene have been found to be causative for AS with a total of 79 disease-causing mutations having been described.

Case presentation
We describe the case of a 27-year old female from an English (Caucasian) kindred. She had been initially referred for hypertriglyceridemia, but demonstrated other features suggestive of AS, including blindness, obesity, type 2 diabetes, renal dysfunction, and hypertension. DNA analysis revealed that she is a compound heterozygote with two novel mutations in the ALMS1 gene – H3882Y and V424I. Examination of her family revealed that her phenotypically unaffected mother and younger sister also had heterozygous mutations in the ALMS1 gene. In addition to presenting these novel molecular findings for AS, we review the clinical and genetic features of AS in the context of our case.

Conclusion
Two novel mutations in the ALMS1 gene causative for AS have been reported here, thereby increasing the number of reported mutations to 81 and providing a wider basis for mutational screening among affected individuals.
Improving surface integrity and economics of grinding by optimum coolant application, with consideration of abrasive tool and process regime
Authors: Webster J A 
Published: December 2007

As compared to machining, the chip thickness in grinding is small and the specific grinding energy is generally much higher than the specific cutting energy in machining. With such small chip thickness values, a lot of heat is created by non-chip removal interactions, such as ploughing and rubbing. These interactions occur between the chip and grain, chip and bond, and the chip and the work. Identification and manipulation of these interactions can allow the engineer to minimize the thermal limitation, improve surface finish, increase wheel life, etc. This paper discusses the thermal energy partitioning that occurs with different modes of grinding, and guides the reader towards processes with a lower specific energy and more desirable energy partitioning with respect to the energy that enters the final workpiece surface. The high efficiency deep grinding regime, is compared to creep-feed and shallow-cut grinding. The paper also looks at the cooling effect of grinding fluids and how they can be applied more effectively to cool and lubricate the workpiece surface. Best practices will be presented, based on analytical models and field experience. Flowrate and pressure models are used to optimize the main fluid jet that is applied to the grinding process. The heat transfer characteristics of superabrasive wheels, as compared to conventional abrasives, will also be presented and discussed. Finally, a case study is presented, that clearly shows the economic and technological advantages of coherent-jet nozzles over a more conventional approach.
An investigation into the ball burnishing of aluminium alloy 6061-T6
Authors: El-Axir M H 
Published: December 2007

Burnishing has been widely used to produce excellent surface finish, work hardening, and compressive residual stress by plastically deforming the workpiece surface. There have been very few studies concerning the effect of burnishing parameters on the fatigue life. The purpose of this work is to study the relationships between the fatigue life, the residual stress, and the ball burnishing process parameters. Experimental work was carried out on a lathe to establish the effect of three burnishing parameters, namely the burnishing speed, burnishing force, and burnishing feed, on the residual stress and fatigue life of aluminium alloy 6061-T6. The residual stress distribution in the surface region due to ball burnishing is determined using a deflectionetching technique. The number of cycles to failure is determined for each specimen using a universal-type fatigue machine, which is suitable for testing specimens requiring oscillatory or reciprocating motion. It was found that all input parameters have controlling effects, to different extents, on the residual stress and fatigue life. The results showed that the residual stress plays an important role in controlling the behaviour of fatigue. The burnishing force and burnishing feed are the most important parameters controlling the values of the compressive residual stress which, in turn, delay crack initiation, resulting in an increase in the fatigue life.
Evaluation of Austenitic-Ferritic Stainless Steel Wires for Orthodontic Applications
Authors: Itman Filho A. , Silva R. V. , Gouvêa C. A. R. , Casteletti L. C. 
Published: December 2007

Abstract
Several studies have shown that austenitic stainless steels are suitable for use in the final phases of orthodontic treatments, such as finishing and retention. These steels demonstrate appropriate mechanical properties, such as high ultimate tensile strength and good corrosion resistance. A new class of materials, the austenic-ferritic stainless steels, is substituting for austenitic stainless steels in several industrial applications where these properties are necessary. This work supports the hypothesis that orthodontic wires of austenic-ferritic stainless steels can replace austenitic stainless steels. The advantages are cost reduction and decrease of the nickel hypersensitivity effect in patients undergoing orthodontic treatments. The object of this study was to evaluate wires of austenitic-ferritic stainless steel SEW 410 Nr. 14517 (Cr26Ni6Mo3Cu3) produced by cold working through rolling and drawing processes. Tests were performed to evaluate the ultimate tensile strength, hardness, ductility, and formability. In accordance with technical standards the wires exhibited ultimate tensile strength and ductility suitable for orthodontic clinical applications. These austenitic-ferritic wires can be an alternative to substitute the common commercial wires of austenic stainless steels with the advantage of decreasing the nickel content.
Mechanical and Light Properties of Mangosteen Fruit as Related to Postharvest Quality
Authors: Jarimopas Bundit , Pushpariksha Phanida , Singh S. Paul , Singh Jay 
Published: December 2007

Abstract
During this study the postharvest damages of fresh mangosteen at wholesale level in Thailand were investigated from April to October 2004. Damages included fruit cracking, hard rind, rough surface, translucent flesh, gummosis, and decay. Total percentage of all damages that made fruit inedible was 43.3 % of production yield. Weight, volume, and dimension of large, medium, small, and undersized mangosteens were measured. The term of dimension ratio was introduced as a sizing parameter compared to the conventional trade parameter. The coefficient of static friction was also determined. This coefficient for gloss and rough surface mangosteens on plexiglass, plywood, and galvanized iron steel varied from 0.31 to 0.46. Color of sound and defective fruits was measured in terms of tristimulus values, X, Y, and Z. It was observed that the corresponding chromaticity coordinates, x and z for mangosteens depend on their maturity stage while y depends on the type of fruit surface. A color ratio term is proposed to be used as a nondestructive technique for detecting internal defects of mangosteens. The highest percentage of correct prediction was found at the color ratio X1/X2&gt; 1.25 at 67.4 %
Temperature effects on the interaction mechanisms between U(VI) and Eu(III) and ZrP2O7: experiment and modelling
Authors: Finck N. , Drot R. , Lagarde G. , Mercier-Bion Florence , Catalette H. , Simoni E. 
Published: December 2007

In the field of nuclear waste management, prediction of the radionuclides migration through the geosphere has to take into account the effects of temperature. The chosen substrate, zirconium diphosphate, was previously described concerning its surface acidity properties for several temperatures (25 °C, 50 °C, 75 °C, 90 °C). Edges for uranyl sorption were obtained in NaClO4medium, and the europium(III) sorption edges were realized in KNO3medium to evaluate the influence of the complexing nitrate ions. Influence of the temperature, the nature of the cation and the nature of the substrate were pointed out by comparing the edges for the different systems. A structural investigation (TRLFS) was carried out to define experimentally, at the microscopic level, the sorption equilibria. Only UO22+and Eu3+were sorbed on both ZrP2O7surface sites (≡Zr–O and ≡P–O), the nitrate medium having no influence on the sorbed species. Furthermore, temperature did not change the sorption mechanisms. The surface complexation modelling of both investigated systems was realized using i) the constant capacitance model ii) the results of the substrate characterizations iii) uranyl sorbed complex determinations. The formation of bidentate surface complexes onto the ≡Zr–O site is not accompanied by a proton release, whereas the formation of bidentate surface complexes onto the ≡P–O site is followed by a release of 2 protons, for both cations and at all temperatures studied. The determined sorption constants increase with temperature, as was expected from the analysis of the sorption edge positions.
2006 National Clinical Pharmacy Services Survey: Clinical Pharmacy Services, Collaborative Drug Management, Medication Errors, and Pharmacy Technology
Authors: Bond C. A. , Raehl Cynthia L. 
Published: December 2007

Study Objective. To determine the extent of 15 hospital‐based clinical pharmacy services, 51 different drugs managed under protocol by pharmacists, medication errors, and pharmacy technology in United States hospitals.Design. A survey was mailed, as well as sent electronically, to pharmacists in 2893 hospitals.Results. A total of 1125 surveys were returned (38.9% response rate). The 1125 hospitals had 14,315,506 patients admitted, which represented 45.7% of the 31,324,496 admissions to all U.S. hospitals in 2006. The proportion of clinical pharmacy services provided by Veterans Affairs (VA) hospitals was higher compared with non‐VA hospitals. In all hospitals, the clinical pharmacy services with the greatest growth from 1989–2006 were pharmacist‐provided admission drug histories (300% increase), pharmacist participation on medical rounds (292.3% increase), drug protocol management (208% increase), pharmacist‐conducted clinical research (166.7% increase), pharmacist‐provided drug information (150% increase), and pharmacist‐provided pharmacokinetic consultation (117.5% increase). A total of 864 hospitals (76.8%) had pharmacists providing drug protocol management (collaborative drug management). Pharmacists managed a mean ± SD of 9.18 ± 10.23 different drugs/hospital (7932 protocols). Drugs commonly managed included aminoglycosides (64.4% of hospitals), vancomycin (63.8%), warfarin (37.8%), low‐molecular‐weight heparins (32.7%), unfractionated heparin (30.0%), fluoroquinolones (30.0%), antiparkinsonian drugs (22.8%), proton pump inhibitors (22.7%), human immunodeficiency virus drugs (21.9%), and cephalosporins (19.7%). The mean number of medication errors reported/hospital increased by 151.4% between 1995 and 2006. The percentage of patients who experienced a medication error increased from 4.7% to 6.5% between 1995 and 2006 (a 38.3% increase). A total of 220 hospitals (19.6%) had computerized prescriber order entry systems, 263 (23.4%) had bar coding for drug administration, and 439 (39.0%) used robotics for dispensing.Conclusion. This study provides continuing evidence of the growth and value of clinical pharmacy services and clinical pharmacists in our nation's hospitals. These data will guide hospital pharmacy directors and clinical coordinators in allocating resources to optimally meet their patients' needs.
Use of a Combination of Class III and Class Ic Antiarrhythmic Agents in Patients with Electrical Storm
Authors: Fuchs Therese , Groysman Rima , Meilichov Ilia 
Published: December 2007

Study Objective. To determine the efficacy of the combination of class III and class Ic antiarrhythmic agents in suppressing an electrical storm in patients with and without a transvenous implantable cardioverter‐defibrillator (ICD).Design. Retrospective medical record review.Setting. Arrhythmia service of an academic medical center in Zerifin, Israel.Patients. Ten patients who experienced an electrical storm that was not effectively treated with amiodarone or sotalol monotherapy between December 15, 1999, and June 13, 2007.Measurements and Main Results. The medical records of 152 patients who received an ICD during the study period were reviewed. Twenty patients experienced an electrical storm, an arrhythmia defined as more than two episodes of hemodynamically unstable ventricular tachycardia during a 24‐hour period. Ten of the 20 patients responded favorably to amiodarone or sotalol monotherapy (class III antiarrhythmics), but in 10 patients, the combination of a class III and a class Ic antiarrhythmic agent was needed to effectively eliminate the electrical storm. Of the 10 patients who required both agents, two (20%) developed an electrical storm before implantation of their ICD. In another patient who had ongoing ischemia, ventricular tachycardia recurred, but the drug combination decreased the number of ventricular arrhythmia episodes. One patient with dilated cardiomyopathy had one recurrence of ventricular tachycardia, which was terminated with antitachycardia pacing. Three patients died during a mean ± SD follow‐up of 8.7 ± 9.9 months.Conclusion. Electrical storm can be acutely treated with the combination of a class III and a class Ic antiarrhythmic agent when a class III agent alone is insufficient and when radiofrequency ablation is not an option. Patients receiving this drug combination can be discharged from the hospital only if they have an ICD.
Association of Galactose Single‐Point Test Levels and Phenytoin Metabolic Polymorphisms with Gingival Hyperplasia in Patients Receiving Long‐Term Phenytoin Therapy
Authors: Lin Chun‐Jung , Yen Ming‐Fang , Hu Oliver Yoa‐Pu , Lin Min‐Shung , Hsiong Cheng‐Huei , Hung Chin‐Chuan , Liou Horng‐Huei 
Published: December 2007

Study Objective.To evaluate whether the occurrence or severity of gingival hyperplasia is associated with liver function test results or phenytoin metabolism.Design.Prospective analysis.Setting.University‐affiliated medical center in Taipei, Taiwan.Patients.Sixty‐six patients (mean age 37.9 yrs) with epilepsy who were receiving phenytoin for more than 1 year.Intervention.Four blood samples were drawn from each patient for liver function testing, concentrations of phenytoin and its metabolites R‐5‐(4′‐hydroxyphenyl)‐5‐phenylhydantoin (R‐HPPH) and S‐HPPH, and genotyping of cytochrome P450 (CYP) 2C9 and 2C19.Measurements and Main Results.Plasma concentrations of phenytoin and its metabolites were determined by a high‐performance liquid chromatography method. The CYP2C9 and CYP2C19 genotypes were analyzed by polymerase chain reaction‐restriction fragment length polymorphism analysis. Conventional liver function assays and a quantitative liver function test—galactose single‐point (GSP) measurement—were performed. Statistical analyses were performed to evaluate the association between liver function test results as well as metabolic phenotype and the occurrence and severity of gingival hyperplasia. Among liver function tests, only GSP levels showed a significant difference between patients with and those without gingival hyperplasia. Patients with an elevated GSP level (> 280 μg/ml) had a significantly higher odds ratio (OR 4.51) for the occurrence of gingival hyperplasia. In addition, increased R‐HPPH (OR 1.02) and phenytoin (OR 1.09) concentrations were associated with an increased occurrence of gingival hyperplasia. However, only increased GSP and R‐HPPH concentrations had significantly higher ORs (2.84 and 1.02, respectively) associated with the severity of gingival hyperplasia. Although mean ± SD plasma R‐HPPH concentration was significantly lower in CYP2C19 poor metabolizers compared with CYP2C9 and CYP2C19 extensive metabolizers and CYP2C9 poor metabolizers (30.38 ± 16.73 vs 68.22 ± 44.75 and 78.95 ± 51.67 μg/ml, respectively), no significant association between genotype and gingival hyperplasia was found.Conclusion.Increased GSP, phenytoin, and R‐HPPH concentrations were associated with increased occurrence of phenytoin‐induced gingival hyperplasia; only increased GSP and R‐HPPH concentrations were associated with increased severity of this adverse effect.
Oral Voriconazole for Empiric Antifungal Treatment in Patients with Uncomplicated Febrile Neutropenia
Authors: Przepiorka Donna , Buadi Francis K. , McClune Brian 
Published: December 2007

Study Objective. To determine the success rate and toxicity profile of oral voriconazole when used as empiric antifungal therapy in patients with uncomplicated persistent neutropenic fever.Design. Retrospective medical record review.Setting. University hospital.Patients. Twenty‐seven patients who received oral voriconazole as empiric antifungal treatment during 31 episodes of persistent neutropenic fever between August 1, 2003, and April 1, 2006.Measurements and Main Results. Data were collected on patient demographics, diagnosis, cancer treatment plan, voriconazole regimen, dates of voriconazole treatment, other antibiotics administered during the episode of fever, and adverse events. Patient survival data from at least 3 months of follow‐up were also collected. Treatment success, which was defined as survival to the resolution of fever without a need to change antibiotics and without new fungal infections, was also determined. Median duration of therapy was 11 days (range 2–54 days). No patient discontinued the drug because of toxicity. The success rate was 55% (95% confidence interval [CI] 36–73%). For all patients, 90‐day survival from the start of voriconazole treatment was 81% (95% CI 63–93%). Neither treatment success nor 90‐day survival rates differed significantly when they were compared by the route used for loading doses or by the presence of a possible invasive fungal infection at the start of voriconazole therapy.Conclusion. Oral voriconazole appears to be a safe empiric antifungal treatment with an encouraging rate of activity for patients with neutropenia and uncomplicated persistent fever. Further research, including randomized controlled trials comparing the efficacy of intravenous versus oral voriconazole, is needed.
The Stem Cell Marker Prominin-1/CD133 on Membrane Particles in Human Cerebrospinal Fluid Offers Novel Approaches for Studying Central Nervous System Disease
Authors: Huttner Hagen B. , Janich Peggy , Köhrmann Martin , Jászai József , Siebzehnrubl Florian , Blümcke Ingmar , Suttorp Meinolf , Gahr Manfred , Kuhnt Daniela , Nimsky Christopher , Krex Dietmar , Schackert Gabriele , Löwenbrück Kai , Reichmann Heinz , Jüttler Eric , Hacke Werner , Schellinger Peter D. , Schwab Stefan , Wilsch-Bräuninger Michaela , Marzesco Anne-Marie , Corbeil Denis 
Published: December 2007

Abstract
Cerebrospinal fluid (CSF) is routinely used for diagnosing and monitoring neurological diseases. The CSF proteins used so far for diagnostic purposes (except for those associated with whole cells) are soluble. Here, we show that human CSF contains specific membrane particles that carry prominin-1/CD133, a neural stem cell marker implicated in brain tumors, notably glioblastoma. Differential and equilibrium centrifugation and detergent solubility analyses showed that these membrane particles were similar in physical properties and microdomain organization to small membrane vesicles previously shown to be released from neural stem cells in the mouse embryo. The levels of membrane particle-associated prominin-1/CD133 declined during childhood and remained constant thereafter, with a remarkably narrow range in healthy adults. Glioblastoma patients showed elevated levels of membrane particle-associated prominin-1/CD133, which decreased dramatically in the final stage of the disease. Hence, analysis of CSF for membrane particles carrying the somatic stem cell marker prominin-1/CD133 offers a novel approach for studying human central nervous system disease.
Disclosure of potential conflicts of interest is found at the end of this article.
Application of Coiled-Tubing Fracturing Method Improves Field Production
Authors: Fussell L. D. , Redfearn J. R. , Marshall E. J. 
Published: December 2007

Abstract
Application of a new coiled tubing assisted fracturing method (CTFM) has been implemented in the Elm Grove Field in Bossier Parish, Louisiana. The new method has allowed all potential pay intervals to be efficiently stimulated. As compared with traditional methods of stimulation, the new method has resulted in better sustained production.
On the Development of a Three-Dimensional Model for a Coiled-Tubing/Riser System
Authors: Adnan S. , Zheng A. S. , Chen Y. 
Published: December 2007

Abstract
This paper establishes a three-dimensional mathematical model for a tubing/riser system subjected to external loadings. Effects such as mud weights, internal pressure in the tubing and annulus, and cable forces are all included in the model. The derivations of the mathematical model are based on principals of beam-column stability theory, which lead to a set of ordinary differential equations with the loading and displacements as functions of the axial location of the riser. With the system equations established, the rest of the paper is devoted to the devise of a numerical algorithm to solve the equations with boundary conditions imposed. The problem is re-casted as two points boundary value problem (TPVBP). Modified Quasilinearization Algorithms (MQA) together with Method of Particular Solution (MPS) are employed in the search of numerical solution for the TPBVP. Results from these algorithms are compared to those obtained from commercial finite element program to verify the validity of the mathematical model and accuracy of the numerical algorithm.
Operability Challenges During Coiled-Tubing (CT) Well Intervention: Panna Field Case Histories From Western India Offshore
Authors: Kale S. V. , Proctor R. J. , Paliwal S. K. , Nayak S. K. 
Published: December 2007

Abstract
Coiled Tubing (CT) applications are performed to intervene in completions installed in the lateral section of Panna wells in Western India Offshore.
The paper discusses the analysis process and the challenges during CT well interventions. Work done to improve CT entry in the lateral section is also discussed in the paper. The paper compares simulated CT entry against the actual reach in each well. Illustrative examples of the actual field data are presented as case histories.
The process is elaborated in this paper. The paper also discusses specific examples where application of tools to excite the BHA was attempted with success, along with the resources needed to accomplish the analysis process effectively.
To describe the challenges this paper groups Panna completions in three categories; open hole, open hole with tail pipe and cased hole with swell packers. Sliding sleeves to operate selective sections of the well are installed in the completion and CT entry to operate the sliding sleeve is imperative.
The operational challenges are grouped as either logistical or environmental.
The paper focuses on technical design challenges and analysis processes to determine the feasibility of future well intervention.
To improve operational success the authors recommend that each intervention campaign be engineered utilizing the results from the previous operations. This increases the likelihood of successful interventions.