bluuebunny/crossref_metadata_2025_split huggingface dataset:
Two new Brazilian patients with Gómez–López‐Hernández syndrome: Reviewing the expanded phenotype with molecular insights
Authors: Gomy Israel , Heck Benjamin , Santos Antônio Carlos , Figueiredo Maria Silvia L. , Martinelli Carlos E. , Nogueira Maria Priscila C. , Pina‐Neto João M. 
Published: February 2008

AbstractGómez–López‐Hernández (GLH) syndrome or cerebello‐trigeminal dysplasia is a neurocutaneous syndrome whose etiology is unknown at the present time. We report two additional Brazilian patients, including the oldest one known to date (age 29). Here, we review the expanded phenotype in four patients with new clinical, psychiatric, radiological, and molecular investigations. One patient may have hypomania within the bipolar spectrum disorder with onset in childhood and adolescence. Primary growth hormone (GH) deficiency was ruled out in all patients, although one of them might have developed secondary GH deficiency due to partial hypopituitarism following severe hydrocephalus. Brain magnetic resonance angiography disclosed no azygous anterior cerebral artery (ACA) but only normal variants. Molecular analysis of the lysosomal acid phosphatase gene (ACP2) was performed, but no pathogenic mutations were identified. We present an overview of the phenotypic features of all patients described to date. There are currently 12 unrelated patients reported in the literature, 5 of whom are Brazilian. We discuss new molecular insights and speculate about the pathogenesis of GLH syndrome. © 2008 Wiley‐Liss, Inc.
Second case of Beare–Stevenson syndrome with an FGFR2 Ser372Cys mutation
Authors: Fonseca Renata , Costa‐Lima Marcelo A. , Cosentino Viviana , Orioli Iêda M. 
Published: February 2008

AbstractBeare–Stevenson syndrome is characterized by cutis gyrata, acanthosis nigricans, skin furrows, skin tags, craniosynostosis, Crouzonoid‐like features in some cases and cloverleaf skull in others, anogenital anomalies, and prominent umbilical stump. Reported causes are an FGFR2 Tyr375Cys mutation in nine cases and an FGFR2 Ser372Cys mutation in one case. Here, we report on a second patient with the FGFR2 Ser372Cys mutation. © 2008 Wiley‐Liss, Inc.
Costello syndrome associated with novel germline HRAS mutations: An attenuated phenotype?
Authors: Gripp Karen W. , Innes A. Micheil , Axelrad Marni E. , Gillan Tanya L. , Parboosingh Jillian S. , Davies Christine , Leonard Norma J. , Lapointe Monique , Doyle Daniel , Catalano Sarah , Nicholson Linda , Stabley Deborah L. , Sol‐Church Katia 
Published: February 2008

AbstractCostello syndrome is a rare congenital disorder typically characterized by severe failure‐to‐thrive, cardiac abnormalities including tachyarrhythmia and hypertrophic cardiomyopathy, distinctive facial features, a predisposition to papillomata and malignant tumors, neurologic abnormalities, developmental delay, and mental retardation. Its underlying cause is de novo germline mutations in the oncogene HRAS. Almost all Costello syndrome mutations affect one of the glycine residues in position 12 or 13 of the protein product. More than 80% of patients with Costello syndrome share the same underlying mutation, resulting in a G12S amino acid change. We report on two patients with novel HRAS mutations affecting amino acids 58 (T58I) and 146 (A146V), respectively. Despite facial features that appear less coarse than those typically seen in Costello patients, both patients show many of the physical and developmental problems characteristic for Costello syndrome. These novel HRAS mutations may be less common than the frequently reported G12S change, or patients with these changes may be undiagnosed due to their less coarse facial features. In addition to the findings previously known to occur in Costello syndrome, one of our patients had hypertrophic pyloric stenosis. This led us to review the medical histories on a cohort of proven HRAS mutation positive Costello syndrome patients, and we found a statistically significantly (P < 0.001) increased frequency of pyloric stenosis in Costello syndrome (5/58) compared to the general population frequency of 2–3/1,000. Thus we add hypertrophic pyloric stenosis to the abnormalities seen with increased frequency in Costello syndrome. © 2008 Wiley‐Liss, Inc.
Effects of ultraviolet irradiation treatment on the surface properties and adhesion of moso bamboo (Phyllostachys pubescens)
Authors: Lu Kun‐Tsung , Fan Ssu‐Yuan 
Published: February 2008

AbstractThe poor adhesion of bamboo coatings is a serious issue in the bamboo industry. To overcome this problem it is necessary to modify the actual surface of the bamboo before finishing. A study on the surface properties and adhesion of moso bamboo (Phyllostachys pubescens) were investigated with various UV irradiation conditions including irradiation time and dose using different UV lamps. Two types of wood coatings, i.e., solvent‐borne nitrocellulose (NC) lacquer and waterborne polyurethane (PU) coating, were used in the study, and 180° peel strength and shear strength tests for measuring adhesion of films were conducted. The results revealed that the wettability and the carbonyl group concentration of the bamboo surface were increased. This was particularly apparent for an irradiation time less than 15 s with a mercury UV lamp (H‐lamp), rated at an intensity of 100 W/cm kept at a distance of 15 cm. In all the treatments, the greenish appearance of moso bamboo was retained and the adhesion was improved. Especially, using solvent‐borne NC lacquer finishing, the higher‐dose (under a mercury UV lamp combined with metal halide lamp; H + M‐lamps) irradiated bamboo had the best adhesion, while, for waterborne PU coating, the H‐lamp irradiated one showed the best improvement. © 2008 Wiley Periodicals, Inc. J Appl Polym Sci, 2008
Synthesis and characterization of novel semi‐interpenetrating polymer network electrolyte based on crosslinked P(GMA‐co‐AN)/PEO
Authors: Luo Dan , Li Yang , Li Weili , Yang Mujie 
Published: February 2008

AbstractA new solid polymer electrolyte based on semi‐interpenetrating polymer networks (semi‐IPN) of crosslinked poly(glycidyl methacrylate‐co‐acrylonitrile)/poly(ethylene oxide) (P(GMA‐co‐AN)/PEO) was synthesized with diethylenetriamine (DETA) as the crosslinking agent and characterized. Fourier transform infrared spectroscopy (FTIR) spectra suggested the formation of semi‐IPN structure by crosslinking and revealed the interactions of Li+ ions with both the ether oxygen in PEO chain and the nitrogen atom in AN segments. Differential scanning calorimetry (DSC) and X‐ray diffraction pattern (XRD) measurements showed that crystallization of the semi‐IPN polymer electrolyte was greatly impeded. Measurement of mechanical properties revealed that tensile strength of the polymer electrolyte was increased after crosslinking. Results of electrochemistry tests suggested that the new polymer electrolyte exhibited a high‐ionic conductivity (10−4 S/cm) at room temperature, and an Arrhenius‐like behavior of the conductivity was observed. And the semi‐IPN polymer electrolyte with less content of PEO exhibited lower ion conductivity. © 2008 Wiley Periodicals, Inc. J Appl Polym Sci, 2008
Preparation of organic/inorganic hybrid nanomaterials using aggregates of star block copolymer consisting of poly(stearyl methacrylate) and poly(3‐(trimethoxysilyl) propyl methacrylate) as precursor
Authors: Zhou Junfeng , Wang Li , Dong Xiaochen , Yang Qiang , Deng Libo , Huo Jia , Tan Qiaohua , Liu Qingquan 
Published: February 2008

AbstractA series of star block copolymer consisting of poly(stearyl methacrylate) and poly(3‐(trimethoxysilyl)propyl methacrylate) was synthesized via atom transfer radical polymerization technique. The resultant polymers were characterized by gel permeation chromatography and 1H NMR. The copolymer can self‐assemble into the aggregates in selective solvents, the resulting aggregates can be used as precursor to prepare hybrid nanomaterials conveniently via the hydrolysis reaction of Si(OCH3)3 groups and the condensation reaction of Si(OH)3 groups. Moreover, the effect of the star copolymer‐solvent interaction on the aggregation behavior was also discussed. © 2008 Wiley Periodicals, Inc. J Appl Polym Sci, 2008
Effect of the nature of crosslinking agent on the unusual metal ion specificity and selectivity of N,N‐bis(2‐aminoethyl)polyacrylamide
Authors: Bindu M. , Mathew Beena 
Published: February 2008

AbstractMetal ion desorbed crosslinked N,N‐bis(2‐aminoethyl)polyacrylamides showed enhanced specificity for the desorbed metal ion, and these polymers selectively rebind the desorbed metal ion from a mixture of metal ions. For this, polyacrylamide with 8 mol % divinylbenzene (DVB) and N,N′‐methylene‐bisacrylamide (NNMBA) crosslinking were prepared by solution polymerization. Diethylenetriamino functions were incorporated into the polymers by polymer analogous reactions. The complexing ability of the amino polymers were investigated toward various transition metal ions like Co(II), Ni(II), Cu(II), and Zn(II). Polymeric ligand and metal complexes were characterized by various spectral methods. The removal of the metal ion from the polymer matrix resulted in a memory for the desorbed metal ion. On rebinding, these polymers specifically rebind the desorbed metal ion and from a mixture of metal ions, it showed selectivity to the desorbed metal ion. Thus, the Cu(II) desorbed polymer specifically and selectively rebind Cu(II) ion from a mixture of Cu(II) and other metal ion. This selectivity is higher in the rigid DVB‐crosslinked system, resulting from the high rigidity of the crosslinked matrix compared to the semirigid NNMBA‐crosslinked system. © 2008 Wiley Periodicals, Inc. J Appl Polym Sci, 2008
Benzimidazole derivatives and their complexes as accelerators for curing of epoxy resin
Authors: Reddy P. V. , Samuelson Sakuntala , Gowda N. M. Nanje , Sathyanarayana D. N. 
Published: February 2008

AbstractBisphenol‐A‐based epoxy resin was cured with an anhydride hardener in the presence of benzimidazoles and their complexes as accelerators. Investigations have revealed an appreciable reduction in the cure and gel times. The kinetic studies based on DSC showed that the rate of curing increases with enhanced concentration of accelerators. The coefficient of thermal expansion has been reduced considerably in the presence of the benzimidazole. The electrical properties of the cured epoxy resin system were unaffected by lower concentrations of benzimidazoles. A mechanism for curing has been proposed with 1,3‐bis(2‐benzimidazolyl)benzene as accelerator based on IR studies, which indicate the involvement of the secondary and tertiary nitrogen of the benzimidazole in the crosslinking of the resin system. © 2008 Wiley Periodicals, Inc. J Appl Polym Sci, 2008
Study of curing of layered silicate/trifunctional epoxy nanocomposites by means of FTIR spectroscopy
Authors: Pagès P. , Lacorte T. , Lipinska M. , Carrasco F. 
Published: February 2008

AbstractA study was conducted on the curing process of a nanocomposite consisting of a trifunctional epoxy resin, a hardener containing reactive primary amine groups, and montmorillonite (MMT) nanoparticles, previously treated with octadecyl ammonium. Three levels of MMT content were used: 2, 5, and 10%. The curing was carried out following the cycle: 4 h at 100°C, 2 h at 150°C, and 2 h at 200°C. Isothermal trials were also considered at three levels (120, 150, and 200°C) to conduct a kinetic study. The curing conversion was determined by FTIR spectroscopy by selecting the suitable bands for epoxide and primary amine functional groups. The study demonstrated that the MMT nanoparticles accelerate the curing process, especially at the initial stages of the thermal cycle, being this influence quasi negligible at the end of the cycle. Curing conversions were also evaluated by differential scanning calorimetry and compared to those obtained by FTIR spectroscopy. © 2008 Wiley Periodicals, Inc. J Appl Polym Sci, 2008
PMMA‐grafted‐silica/PVC nanocomposites: Mechanical performance and barrier properties
Authors: Zhu Aiping , Cai Aiyun , Zhang Jie , Jia Huawei , Wang Jingqing 
Published: February 2008

AbstractPoly(vinyl chloride) (PVC)/SiO2 nanocomposites were prepared via melt mixture using a twin‐screw mixing method. To improve the dispersion degree of the nanoparticles and endow the compatibility between polymeric matrix and nanosilica, SiO2 surface was grafted with polymethyl methacrylate (PMMA). The interfacial adhesion was enhanced with filling the resulting PMMA‐grafted‐SiO2 hybrid nanoparticles characterized by scanning electron microscopy. Both storage modulus and glass transition temperature of prepared nanocomposites measured by dynamic mechanical thermal analysis were increased compared with untreated nanosilica‐treated PVC composite. A much more efficient transfer of stresses was permitted from the polymer matrix to the hybrid silica nanoparticles. The filling of the hybrid nanoparticles caused the improved mechanical properties (tensile strength, notched impact strength, and rigidity) when the filler content was not more than 3 wt %. Permeability rates of O2 and H2O through films of PMMA‐grafted‐SiO2/PVC were also measured. Lower rates were observed when compared with that of neat PVC. This was attributed to the more tortuous path which must be covered by the gas molecules, since SiO2 nanoparticles are considered impenetrable by gas molecules. © 2008 Wiley Periodicals, Inc. J Appl Polym Sci, 2008
Synthesis, characterization, and properties for Hg2+ of new chelating resin containing sulfoxide and 3‐aminopyridine
Authors: Ji Chunnuan , Qu Rongjun , Wang Chunhua , Sun Changmei , Chen Hou , Sun Yanzhi , Liu Jie 
Published: February 2008

AbstractA new chelating resin (PVESO‐AP) containing sulfoxide and 3‐aminopyridine (AP) was synthesized by using chloromethylated polystyrene‐co‐divinylbenzene (PS‐Cl) as material. Its structure was characterized by infrared spectra and elemental analysis. The adsorption capacities of the newly formed resin for Hg2+, Ag+, Cu2+, Zn2+, Cd2+, and Ni2+ were determined at pH 2 and 5. In addition, the adsorption selective coefficients for Hg2+ in several binary mixture systems were investigated by batch experiment method. The results showed that this resin had high adsorption selectivity for Hg2+. The adsorption kinetics and isothermal of PVESO‐AP for Hg2+ at different temperatures were also investigated. The adsorption kinetics showed that the apparent activation energy Ea was 13.71 kJ/mol. The results also showed that the Langmuir model was better than the Frundlich model to describe the isothermal process of PVESO‐AP resin for Hg2+. © 2008 Wiley Periodicals, Inc. J Appl Polym Sci, 2008
Effect of solvent on the properties of thermoplastic polyurethane/clay nanocomposites prepared by solution mixing
Authors: Dan Cheol Ho , Kim Young Doo , Lee Minho , Min Byong Hun , Kim Jeong Ho 
Published: February 2008

AbstractEffect of different solvents on the clay dispersion and the final properties of the thermoplastic polyurethanes (TPUs)/clay nanocomposites prepared via solution mixing was studied. The polyether‐ and polyester‐based TPUs were used along with organically modified clays (C30B, C25A, and C15A) and the pristine montmorillonite (PM). Dimethylacetamide (DMAc) and tetrahydrofuran (THF) were used as solvents for solution mixing. Nanocomposites containing C30B prepared from DMAc solution showed the better clay dispersion than the ones from THF solution, while THF gave the better dispersion of clays for nanocomposites containing C15A. Morphologies of the nanocomposites were observed to be determined not only by the state of clay dispersion in different solvents but also by the interaction between the polymer and the specific clay. Affinity between solvents and clays becomes important when there is no specific interaction between the clay and the polymer of interest, or when the interaction between the two is rather weak. The compatibility between clays and polymers becomes dominating if there exists a specific interaction between the two. FTIR analysis was conducted to study the interactions involved in the nanocomposites. Dynamic mechanical properties measurement was also carried out to see the effect of solvents. © 2008 Wiley Periodicals, Inc. J Appl Polym Sci, 2008
Rendering synthetic fabrics acid printable using chitosan and binder
Authors: Hakeim O. A. , El‐Gabry L. , Abou‐Okeil A. 
Published: February 2008

AbstractIn this study chitosan samples of different nature, prepared by hydrolysis with HCL and modification by reaction with dimethylaminobenzaldehyde in methanol were applied to acrylic and polyester fabrics by pad‐dry‐cure method in presence and absence of binder (acrylate based copolymer). The pretreated samples were printed with acid dye. The effect of acid hydrolysis, modification, and concentration of chitosan on the color strength of the prints was investigated. The printed fabrics were evaluated for color yield and fastness properties. The color strength of the prints in presence of binder was three times higher than in its absence. The enhancment in color strength was relies mainly on N% of chitosan. © 2008 Wiley Periodicals, Inc. J Appl Polym Sci, 2008
Quantifying Scholarly Impact: IQp Versus the Hirsch h
Authors: Antonakis John , Lalive Rafael 
Published: February 2008

AbstractHirsch's (2005) h index of scholarly output has generated substantial interest and wide acceptance because of its apparent ability to quantify scholarly impact simply and accurately. We show that the excitement surrounding h is premature for three reasons: h stagnates with increasing scientific age; it is highly dependent on publication quantity; and it is highly dependent on field‐specific citation rates. Thus, it is not useful for comparing scholars across disciplines. We propose the scholarly “index of quality and productivity” (IQp) as an alternative to h. The new index takes into account a scholar's total impact and also corrects for field‐specific citation rates, scholarly productivity, and scientific age. The IQp accurately predicts group membership on a common metric, as tested on a sample of 80 scholars from three populations: (a) Nobel winners in physics (n = 10), chemistry (n = 10), medicine (n = 10), and economics (n = 10), and towering psychologists (n = 10); and scholars who have made more modest contributions to science including randomly selected (b) fellows (n = 15) and (c) members (n = 15) of the Society of Industrial and Organizational Psychology. The IQp also correlates better with expert ratings of greatness than does the h index.
Tragsicherheitsprüfung von Granitsäulen in der Katholischen Kirche “St. Joseph” in Mannheim
Authors: Bißwurm Axel , Steffens Klaus , Steiner Josef 
Published: February 2008

AbstractDie Erkundung und Beurteilung von Schäden an Bauwerken und Bauteilen sind zentrale und verantwortungsvolle Aufgaben des Tragwerksplaners. Viel zu oft werden Entscheidungen ohne ausreichende Voruntersuchungen getroffen. Ein nicht genau definierbarer Tragwerkszustand, der ein mögliches Versagen nicht mit ausreichender Sicherheit ausschließen kann, ist nicht akzeptabel, auch wenn dieser Zustand schon über viele Jahre hinweg Bestand hat. Der hier geschilderte Fall der St. Josephskirche in Mannheim beschreibt den Zustand von Granitsäulen, die infolge von Kriegseinwirkungen geschädigt wurden. Erst jetzt, über 60 Jahre nach Kriegsende, wurde das Ausmaß der Schäden als Folge von Brandeinwirkungen und der möglichen Beeinträchtigung der Standsicherheit in vollem Umfang erkannt, so dass die Kirche vorübergehend nicht genutzt werden durfte. Da die Beurteilung der Standsicherheit der Säulen mit rechnerischen Methoden nicht möglich war, wurde der Nachweis unter Zuhilfenahme von Bauteilversuchen geführt.
Restaurierung antiker Tempel: Experimentelle Untersuchungen zum Ausziehverhalten von Verankerungen im Marmor
Authors: Kourkoulis Stavros , Papanicolopulos Stefanos‐Aldo , Marinelli Aikaterini , Vayas Ioannis 
Published: February 2008

AbstractBei der Restaurierung antiker griechischer Tempel werden einzelne Marmorblöcke zu einem Bauteil, beispielsweise einem Architrav, zusammengebaut. Wegen der Forderung nach Reversibilität der Eingriffe erfolgt dies an der Akropolis von Athen durch Anwendung von in Zementmörtel eingelassenen Titanstäben mit Gewinde in vorgebohrten Löchern. Diese Art der Restaurierung entspricht einer Teil sanierung der Architrave. Im hier vorliegenden Beitrag werden experimentelle Untersuchungen zum Ausziehverhalten dieser Verankerungsstäbe vorgestellt. Die Versuche werden an einer speziell entworfenen Versuchsanordnung durchgeführt. Das Versagen erfolgt bei allen Versuchen an der Grenzfläche zwischen Mörtel und Bohrlochwand. Es zeigt sich, dass die Gewindeform das Tragverhalten entscheidend beeinflusst. Ferner werden mögliche Grenzzustände definiert.
Quercetin pharmacokinetics in humans
Authors: Moon Young J. , Wang Liang , DiCenzo Robert , Morris Marilyn E. 
Published: February 2008

AbstractThe purpose of this study was to examine the pharmacokinetics of quercetin aglycone as well as its conjugated metabolites and to develop a population pharmacokinetic model for quercetin that incorporates enterohepatic recirculation. The stability of quercetin in different matrices at various temperatures and pH, and the quercetin content of six capsules of the herbal preparation Quercetin‐500 Plus® were determined by HPLC. Subjects received quercetin 500 mg three times daily and blood and urine samples were obtained. The concentration of quercetin aglycone and conjugated metabolites were assayed using a liquid chromatography‐tandem mass spectrometry assay. Pharmacokinetic parameters were determined using noncompartmental analysis with WinNonlin. A population compartment model incorporating input from the gallbladder was developed to account for the enterohepatic recirculation observed with quercetin. The oral clearance (CL/F) was high (3.5 × 104l/h) with an average terminal half‐life of 3.5 h for quercetin. The plasma concentration versus time curves exhibited re‐entry peaks. A one‐compartment model that included enterohepatic recirculation best described the plasma data. This represents the first comprehensive evaluation of the pharmacokinetics and enterohepatic recirculation of quercetin in humans. Population pharmacokinetic models adapted for enterohepatic recirculation allowed an assessment of the magnitude and frequency of the enterohepatic recirculation process. Copyright © 2008 John Wiley & Sons, Ltd.
Setup and dosimetry for exposing anaesthetised pigs in vivo to 900 MHz GSM mobile phone fields
Authors: Toivonen Tommi , Toivo Tim , Pitkäaho Risto , Puranen Lauri , Silfverhuth Minna , Mennander Ari , Hannula Markus , Hyttinen Jari , Jokela Kari 
Published: February 2008

AbstractThe aim of this study was a dosimetrical analysis of the setup used in the exposure of the heads of domestic pigs to GSM‐modulated radio frequency electromagnetic fields (RF‐EMF) at 900 MHz. The heads of pigs were irradiated with a half wave dipole using three different exposure routines; short bursts of 1–3 s at two different exposure levels and a continuous 10‐min exposure. The electroencephalogram (EEG) was registered continuously during the exposures to search for RF‐EMF originated changes. The dosimetry was based on simulations with the anatomical heterogeneous numerical model of the pig head. The simulation results were validated by experimental measurements with the exposure dipole and a homogeneous liquid phantom resembling the pig head. The specific absorption rate (SAR), defined as a maximum average over 10 g tissue mass (SAR10g), was 7.3 W/kg for the first set of short bursts and 31 W/kg for the second set of short bursts. The SAR10g in the continuous 10‐min exposure was 31 W/kg. The estimated uncertainty for the dosimetry was ±25% (K = 2). Bioelectromagnetics 29:363–370, 2008. © 2008 Wiley‐Liss, Inc.