bluuebunny/crossref_metadata_2025_split huggingface dataset:
Heterogeneity in Phenotype of Usher-Congenital Hyperinsulinism Syndrome
Authors: Al Mutair Angham N. , Brusgaard Klaus , Bin-Abbas Bassam , Hussain Khalid , Felimban Naila , Al Shaikh Adnan , Christesen Henrik T. 
Published: November 2012

OBJECTIVE
To evaluate the phenotype of 15 children with congenital hyperinsulinism (CHI) and profound hearing loss, known as Homozygous 11p15-p14 Deletion syndrome (MIM #606528).

RESEARCH DESIGN AND METHODS
Prospective clinical follow-up and genetic analysis by direct sequencing, multiplex ligation-dependent probe amplification, and microsatellite markers.

RESULTS
Genetic testing identified the previous described homozygous deletion in 11p15, USH1C:c.(90+592)_ABCC8:c.(2694–528)del. Fourteen patients had severe CHI demanding near-total pancreatectomy. In one patient with mild, transient neonatal hypoglycemia and nonautoimmune diabetes at age 11 years, no additional mutations were found in HNF1A, HNF4A, GCK, INS, and INSR. Retinitis pigmentosa was found in two patients aged 9 and 13 years. No patients had enteropathy or renal tubular defects. Neuromotor development ranged from normal to severe delay with epilepsy.

CONCLUSIONS
The phenotype of Homozygous 11p15-p14 Deletion syndrome, or Usher-CHI syndrome, includes any severity of neonatal-onset CHI and severe, sensorineural hearing loss. Retinitis pigmentosa and nonautoimmune diabetes may occur in adolescence.
Modeling the effect of austenite deformation on the bainite structure parameters in low carbon microalloyed steels
Authors: Zolotorevsky Nikolay , Nesterova Elena , Titovets Yuri , Khlusova Elena 
Published: November 2012

Abstract
A modeling approach to calculate the kinetics of non-isothermal bainite formation in low carbon microalloyed steels is presented. The model development is based on experimental data obtained by means of deformation dilatometry and electron back scattering diffraction. It is suggested that the misorientations inherited from the plastically deformed austenite make a significant contribution to the misorientation distribution in the final microstructure. Taking the inheritance phenomenon into consideration, the model allows prediction of the transformation kinetics and the effective grain size of bainitic microstructure as a function of the austenite deformation level and the cooling rate.
Fabrication of micro/nano structured aluminum–nickel energetic composites by means of ultrasonic powder consolidation
Authors: Erdeniz Dinc , Ando Teiichi 
Published: November 2012

Abstract
Al–Ni energetic composites, applicable to localized heating, were fabricated from atomized powders and hammer-milled flakes by means of ultrasonic powder consolidation, a novel low-temperature rapid powder consolidation technique. Full-density consolidation was achieved in 1 s of consolidation time at temperatures as low as 573 K without causing appreciable reactions between Al and Ni. The consolidated composite structure consists of Ni powder particles or flakes uniformly distributed in a metallurgically consolidated Al matrix. Consolidated materials were evaluated using spark and continuous-heating ignition tests. Ultrasonic powder consolidation is potentially applicable to the fabrication of a wide range of metastable materials that cannot be processed at elevated temperatures.
Characterization of silicon-silicon carbide ceramic derived from carbon-carbon silicon carbide composites
Authors: Srivastava Vijay K. , Krenkel Walter 
Published: November 2012

Abstract
The main objective of the present work is to process porous silicon–silicon carbide (Si–SiC) ceramic by the oxidation of carbon–carbon silicon carbide (C/C–SiC) composites. Phase studies are performed on the oxidized porous composite to examine the changes due to the high temperature oxidation. Further, various characterization techniques are performed on Si–SiC ceramics in order to study the material's microstructure. The effects of various parameters such as fiber alignment (twill weave and short/chopped fiber) and phenolic resin type (resol and novolak) are characterized.
Solubility study of the copper-lead system
Authors: Vaajamo Iina , Johto Hannu , Taskinen Pekka 
Published: November 2012

Abstract
In this study isothermal equilibration experiments were done at 866–1 166 K for the copper–lead (Cu-Pb) binary system. The experiments, conducted in a quartz ampoule, enabled the solubility limits at each end of the phase diagram to be obtained. In addition to the experiments, a literature survey and compilation of the experimental data of the solid solubilities was done. Critically evaluated and selected literature data and our own experimental values were used to optimise the fcc phase interaction parameters. According to the experiments the lead solubility in solid copper at the copper-rich end is smaller than previously reported. The liquidus curve at the lead-rich end was validated with our own experimental results, which agree well with previous experimental data. The thermodynamic assessment of the Cu-Pb system, in the area of fcc_A1 solid solutions, has been improved.
Preparation and magnetic characterization of Fe/metal oxide nanocomposite particles by means of hydrogen reduction assisted ultrasonic spray pyrolysis (USP-HR)
Authors: Ebin Burçak , Toparlı Çiğdem , Gürmen Sebahattin 
Published: November 2012

Abstract
Fe/metal oxide nanocomposite particles were produced by means of hydrogen reduction assisted ultrasonic spray pyrolysis. Fe/Fe0.761Mg0.239O and Fe/MgO nanocomposite particles were obtained at 600 and 800 °C, respectively. The thermodynamics of the formation reactions were investigated. Increasing the reaction temperature allowed efficient reduction of the precursor to metallic iron that induces the formation of pure MgO phase. The crystallite sizes of the Fe in the composite structures slightly increased, and also the crystallite sizes of the oxides decreased with elevating temperature. The nanocomposite particles exhibited spherical morphology and their particle sizes were slightly different. All of the samples showed ferromagnetic characteristics and the results indicate that the amount of metal and metal oxide phases most affected the saturation magnetizations of the composite particles which were lower than pure iron.
Synthesis of Ir1-xRex (0.15 ≤ x ≤ 0.40) solid solutions under high-pressure and high-temperature
Authors: Gromilov Sergey A. , Dyachkova Tatiyana V. , Bykova Elena A. , Tarakina Nadezda V. , Zaynulin Yurii G. , Yusenko Kirill V. 
Published: November 2012

Abstract
Ir1–xRex (0.15 ≤ x ≤ 0.40) phases prepared under high-pressure, high-temperature conditions from nanopowders of iridium and rhenium were characterized using powder X-ray diffraction and scanning electron microscopy. Structural characteristics of the phases obtained were identical with the corresponding parameters for the solid solutions prepared by means of melting and thermal decomposition of the precursors. The data obtained make it possible to improve solid-state solubility limits in the binary Ir–Re phase diagram. As revealed, the maximum solid solubility of Ir in Re is 68 at.%, and that of Re in Ir is 20 at.%.
The effect of nitrogen on the coarsening rate of precipitate phases in iron-based alloys with chromium and vanadium: experimental and theoretical investigations
Authors: Lindwall Greta , Frisk Karin 
Published: November 2012

Abstract
A comparison of the coarsening of nitrogen-rich vanadium precipitates and the coarsening of carbon-rich vanadium precipitates is presented. The precipitate phases are studied experimentally, via fabrication of model alloys, and theoretically, via simulations utilizing the DICTRA software. The experimental investigations indicate that the nitrogen-rich precipitates exhibit a slower coarsening behaviour than the carbon-rich precipitates. Analysis using thermodynamic and kinetic modelling shows that this can be explained by the higher thermodynamic stability of the nitrogen-rich precipitate compared to the carbon-rich precipitate. The calculated coarsening rates are compared with the measured rates, and found to be in satisfactory agreement using reasonable values for the interfacial energies. The investigations are motivated by the fine precipitate size distribution of nitrides and carbonitrides characteristic for high nitrogen alloyed tool steels produced by means of powder metallurgy.
Phase diagram investigation of the Sn-In
x
Ag
y
Cu
z
(x:y:z = 7:2:1) section in the Ag-In-Sn-Cu system
Authors: Milosavljević Aleksandra , Živković Dragana , Manasijević Dragan , Du Yong , Talijan Nadežda , Bu Mengjie , Kostov Ana 
Published: November 2012

Abstract
The results of phase diagram investigation and characterization of the Ag-In-Sn-Cu system are presented in this paper. Samples at the In : Ag : Cu = 7 : 2 : 1 vertical section were measured with differential scanning calorimetry, and the obtained transition temperatures were compared with calculated values. From Calphad-type calculation, the phase diagram for Sn-In
x
Ag
y
Cu
z
(molar ratio x:y:z = 7:2:1) section is constructed. In order to obtain the microstructure of investigated alloys, scanning electron microscopy with energy dispersive X-ray spectroscopy was used.
Characterization of electrospun fibrous scaffold produced from Indian eri silk fibroin
Authors: Andiyappan Muthumanickam , Sundaramoorthy Subramanian , Vidyasekar Prasanna , Srinivasan Natarajan Tirupattur , Verma Rama Shanker 
Published: November 2012

Abstract
A scaffold, synthesized from bio-degradable polymers and Bombyx
mori silk fibroin in the form of films and fibrous assemblies, has been used as the bio-material for in-vivo applications. In the present work, the scaffold was prepared from the fibroin of Indian eri silk via the electrospinning method. The diameter of the fibre produced was in the range of 300 to 900 nm. The scaffold was subjected to ethanol treatment to improve its dimensional stability, as there was the problem of curling and shrinking when it was treated with solutions used for the cell culture. The scaffold was characterized for surface, thermal and tensile properties. The dimensional stability of the scaffold improved and the porosity reduced, due to the treatment of the scaffold with ethanol. The average failure stress of the raw and ethanol treated scaffold was 2.34 MPa and 4.91 MPa respectively and the mean strain was 13.63 % and 7.91 % respectively. Bone marrow stromal cells were isolated from the bone marrow of Swiss albino mice, and cultured on the ethanol treated electrospun fibrous scaffold. Scanning electron microscopy of the culture was carried out to evaluate the attachment and growth of cells on the scaffold at different incubation periods. Mouse bone marrow stromal cells adhered and grew on the electrospun fibrous scaffold prepared from eri silk fibroin, and the cell density increased with increasing incubation periods.
Thermophysical properties of solid phase palladium over a wide temperature range
Authors: Milošević Nenad , Babić Marijana 
Published: November 2012

AbstractThis paper presents experimental results of a range of thermophysical properties of pure polycrystalline palladium samples over a wide temperature range. Specific heat capacity was measured from 250 to 1 770 K, specific electrical resistivity from 250 to 1 800 K, hemispherical total emissivity from 1 100 to 1 800 K, normal spectral emissivity from 1 340 to 1 720 K, and thermal diffusivity in the range from 200 to 1 540 K. From the measured data, the thermal conductivity and Lorentz function were computed in the range from 250 to 1 540 K. Specific heat capacity, electrical resistivity, and both emissivities were measured by means of subsecond pulse calorimetry, while the thermal diffusivity was obtained using the laser flash technique. The first method used specimens in the form of a thin rod, of about 2 mm in diameter and 200 mm in length, and the second disk specimens of about 3 mm in thickness and 10 mm in diameter. For necessary corrections, literature data on thermal linear expansion have been used. All the results are compared with available literature data and discussed.
Analysis of size effect and anisotropy of 6H – SiC thermal conductivity
Authors: Wang Z. H. , Zhang L. Y. , Meng X. 
Published: November 2012

Abstract
Silicon carbide has been used in refractories, ceramics, and numerous high-performance applications with very good mechanical properties and high thermal conductivity. Silicon carbide composites have excellent potential as a low-activation structural material for fusion and industrial applications. Thermal properties of silicon carbide ceramics are important for the design and safe operation in these situations. To obtain the thermal properties of silicon carbide ceramics, based on the nonequilibrium molecular dynamics simulation method, the size effect on thermal conductivity in different directions is described. 6H – SiC thermal conductivities in the normal and tangential directions are predicted in a nanoscale cuboid system. It is found that there is an obvious size effect and anisotropy of thermal conductivity in different directions because of the impact of the boundary scattering. The normal and tangential thermal conductivity equations have been obtained as good power function expressions. The results show that boundary scattering is strong in phonon transport in different directions within thin film silicon carbide.
Influence of Twin-Screw Processing Conditions on Structure and Properties of Polypropylene – Organoclay Nanocomposites
Authors: Domenech T. , Peuvrel-Disdier E. , Vergnes B. 
Published: November 2012

Abstract
This study looks at the influence of extrusion parameters such as screw speed, feed rate and barrel temperature on the nanocomposite structure (size of agglomerates, level of intercalation and exfoliation) and its consequences on final mechanical properties. Nanocomposites of polypropylene, maleated polypropylene and organomodified montmorillonite, with respective mass fractions of 85/10/5, were prepared in a co-rotating twin-screw extruder using a masterbatch dilution method. The nanocomposites structure was quantified by scanning and transmission electron microscopy, X-ray diffraction and dynamic rheometry. Relationships between the microstructure at different levels (size and number of agglomerates, interlayer distance, melt yield stress to quantify the exfoliation level) and the processing conditions were established, revealing that specific mechanical energy received during extrusion was the key parameter controlling this microstructure. Mechanical properties in uniaxial tension (apparent Young's modulus) were measured and related to the microstructural parameters resulting from extrusion conditions.
Design and Validation of a Cable Extrusion Tip with Spiral Channels
Authors: Haller D. , Gonnet J.-M. , Lankes C. 
Published: November 2012

Abstract
Traditional cable extrusion dies employ a side-fed flow distribution system. In this case, the flow is split into two streams, resulting in the formation of a weld line. The weld line runs as a radial line through the cross section of the cable coating and has the least favorable orientation when the cable coating is exposed to circumferential stresses. Therefore, a new cable extrusion tip based on the principle of spiral mandrel dies was developed to change the orientation of the weld line from radial, that takes place when side-fed mandrel dies are used, to circumferential, when melt distribution resulting from the layering of the leakage flow in spiral mandrel dies occurs. The circumferential orientation of the weld line reduces the exposure of the weld line to circumferential stresses and thus improves the mechanical properties of the cable coating. This paper first outlines the design strategy for the new cable extrusion tip and further presents experimental results. The design strategy is based on two-dimensional flow networks. The network model is applied to calculate the flow channel geometry for a wanted distribution of the flow. Further, it should generate a better understanding of the correlation between the flow, pressure drop, and the flow channel geometry to facilitate the design of the tip. Three dimensional FEM simulations were used in this work to validate the flow network model in terms of pressure drop and velocity profile at the exit of the tip. The results of the calculations with the network model show good accordance with three dimensional FEM-simulations. For the experimental part a tensile test was conducted to compare the tensile strength in circumferential direction of a cable extruded with the new tip with a cable extruded with a conventional cable extrusion tip. The measurements showed an increase of the tensile strength in circumferential direction of 10% to 15% for the cable extruded with the new tip compared to the tensile strength at the location of the weld line in the cable extruded with the conventional tip.
Mechanical Properties and Fracture Morphology of Blends of PC with PMMA
Authors: Lee S.-K. , Cho S.-S. , Kang L.-S. , Lee C. G. , Hong H. S. 
Published: November 2012

Abstract
The poly methyl methacrylate (PMMA) and polycarbonate (PC) residues were collected from waste LCD panels for use as raw materials to prepare PC/PMMA polymer blends (1/99, 3/97, 5/95, 10/90), which were extruded in a twin screw extruder. The extrudates were subsequently pelletized and then dried in an oven before injection molding into tensile bars of ASTM D638-91, type I. The tensile bars were placed in an Instron-type tensile tester and the load-elongation curves of tested 5/95 specimens demonstrated distinctively varied yielding before final brittle fracture but 10/90 showed some sign of ductile behavior, peak in the load-elongation curve attributable to formation of a neck. The tensile strength increased from 569.6 to 633.3 kgf/cm2 with PC concentration in the range of 1 to 5 wt% while it decreased at 10 wt% PC. On the contrary, transverse rupture strength increased with added amount of PC. Addition of PC could have toughened recycled PMMA matrix under impact test conditions. Finally, the fractured surfaces were examined by scanning electron microscopy and numerous fine particles dispersed in the matrix of 10/90 are regarded as PC phase, in reasonable agreement with literature results. On the other hand, fracture surface of 5/95 showed nearly homogeneous matrix phase of PC/PMMA.
Effect of Prepolymerization Time on Morphology and Properties of Epoxy-modified Bismaleimide Foams
Authors: Liu X. , Lu H. , Xing L. 
Published: November 2012

AbstractEpoxy-modified bismaleimide (BMI) foams were prepared through prepolymerization and foaming. Density-adjustable BMI foams with closed-cell structures were produced. The influence of prepolymerization time on bubble growth and morphology, as well as the compressive and heat resistant properties of the foams, were thoroughly investigated. BMI prepolymerization was an effective method for controlling the bubble growth rate, the bubble size and its distribution, and the foam density. As the prepolymerization time increased, the matrix viscosity, the number of cells per unit volume and the foam density increased, whereas, the bubble growth rate and cell size decreased, and the cell size distribution became narrower. The compressive strength and modulus increased with increasing prepolymerization time. The dimensional stability temperatures of the BMI foams were all above 200°C, as determined by their base materials, and were unaffected by prepolymerization time.
Effects of a Multifunctional Polymeric Chain Extender on the Properties of Polylactide and Polylactide/Clay Nanocomposites
Authors: Meng Q.-K. , Heuzey M.-C. , Carreau P. J. 
Published: November 2012

Abstract
A multifunctional polymeric chain extender (Joncryl) was used in the melt processing of a neat polylactide and polylactide/clay nanocomposites. The effects of Joncryl on morphology, rheology, thermal and mechanical properties, barrier properties and biodegradability were investigated. Three Joncryl loadings (0.1, 0.3 and 0.5 wt%) were used in this study, and the 0.5 wt% loading induced a long-chain branched structure in the PLA matrix, as indicated by the melt rheology results. It is believed that the property variations are all related to the long-chain branched structure as well as on the molecular weight recovery. The use of Joncryl did not change the intercalated and partially exfoliated clay structures in the PLA/clay nanocomposites, as observed by X-ray diffraction (XRD) and transmission electron microscopy (TEM). The tensile modulus and maximum strength slightly increased with Joncryl loading. The oxygen barrier properties were also improved by adding Joncryl. However, the addition of Joncryl prevented the formation of large spherulitic crystals and decreased the creep resistance at low stress level. Joncryl could not only control the thermal degradation of PLA during processing, but also affected its biodegradation in compost: higher Joncryl loading led to slower biodegradation and less molecular weight reduction with time.
Grain-size evolution of polar firn: a new empirical grain growth parameterization based on X-ray microcomputer tomography measurements
Authors: Linow Stefanie , Hörhold Maria W. , Freitag Johannes 
Published: November 2012

AbstractFirn microstructure properties from six different sites in Greenland and Antarctica are investigated by means of X-ray microcomputer tomography. The optical effective radius is calculated from the specific surface area (SSA) and used as a measure of grain size. It is shown that the recently introduced spherical approximation of firn grains using the effective radius Reff is representative of grain size in the microwave frequency region. The measured profiles show the well-known increase of grain size with depth at all sites, where the increase is largest at near-surface depths. A large variability in grain size on the decimeter-to-centimeter scale as a result of different grain properties of single layers is superimposed on the overall trend at each site. A simple empirical parameterization of grain-size evolution is developed which allows the rapid grain growth in the uppermost layers of the firn to be predicted. The growth is driven by strong seasonal and diurnal temperature gradients. The model can be used to simulate grain-size profiles required by models of firn/microwave interaction (e.g. for retrieval of accumulation rates from satellite microwave sensors) in a more realistic fashion.
Ice-shelf basal channels in a coupled ice/ocean model
Authors: Gladish Carl V. , Holland David M. , Holland Paul R. , Price Stephen F. 
Published: November 2012

AbstractA numerical model for an interacting ice shelf and ocean is presented in which the ice- shelf base exhibits a channelized morphology similar to that observed beneath Petermann Gletscher’s (Greenland) floating ice shelf. Channels are initiated by irregularities in the ice along the grounding line and then enlarged by ocean melting. To a first approximation, spatially variable basal melting seaward of the grounding line acts as a steel-rule die or a stencil, imparting a channelized form to the ice base as it passes by. Ocean circulation in the region of high melt is inertial in the along-channel direction and geostrophically balanced in the transverse direction. Melt rates depend on the wavelength of imposed variations in ice thickness where it enters the shelf, with shorter wavelengths reducing overall melting. Petermann Gletscher’s narrow basal channels may therefore act to preserve the ice shelf against excessive melting. Overall melting in the model increases for a warming of the subsurface water. The same sensitivity holds for very slight cooling, but for cooling of a few tenths of a degree a reorganization of the spatial pattern of melting leads, surprisingly, to catastrophic thinning of the ice shelf 12 km from the grounding line. Subglacial discharge of fresh water along the grounding line increases overall melting. The eventual steady state depends on when discharge is initiated in the transient history of the ice, showing that multiple steady states of the coupled system exist in general.