bluuebunny/crossref_metadata_2025_split huggingface dataset:
Fabrication and Characterization of Two‐Photon Polymerized Features in Colloidal Crystals
Authors: Pruzinsky S. A. , Braun P. V. 
Published: November 2005

AbstractThe fabrication and characterization of two‐photon polymerized features written within and outside of colloidal crystals is presented. Two‐photon polymerization (TPP) response diagrams are introduced and developed to map the polymerization and damage thresholds for features written via modulated beam rastering. The use of tris[4‐(7‐benzothiazol‐2‐yl‐9,9‐diethylfluoren‐2‐yl)phenyl]amine (AF‐350) as an initiator for TPP is demonstrated for the first time and TPP response diagrams illustrate the polymerization window. These diagrams also demonstrate that the polymerization behavior within and outside of colloidal crystals is similar and electron microscopy reveals nearly identical resolution. Fluorescence confocal microscopy further enables visualization of non‐self‐supporting, three‐dimensional TPP features within self‐assembled photonic crystals. Finally, microspot spectroscopy is collected from a two‐photon feature written within a colloidal crystal and this is compared with simulation.
Cover Picture: Acceleration of Calcite Kinetics by Abalone Nacre Proteins (Adv. Mater. 22/2005)
Authors: Fu G. , Qiu S. R. , Orme C. A. , Morse D. E. , De Yoreo J. J. 
Published: November 2005

AbstractAbalone utilizes a system of macromolecular matrices and soluble proteins to produce beautiful and mechanically robust shells. The cover shows work by Qiu and co‐workers reported on p. 2678, in which AP8 proteins isolated from the shell of red abalone are shown to alter the growth of calcite both by accelerating the rate and modifying the shape from the simple rhombohedra seen in the upper left of the scheme to the more complex form seem in the lower right. The changes are made manifest at an atomic scale through alterations in the growth speed and shape of the atomic steps that form the growth hillocks (background).
Effect of steaming treatment in the structure and reactivity of FCC catalysts
Authors: Tonetto G. M. , Ferreira M. L. , Atias J. A. , de Lasa H. I. 
Published: November 2005

AbstractThe shape selectivity properties of USY zeolite crystallites is discussed, and is based on catalyst characterization, molecular simulation, catalytic experiments of model compounds and kinetic modeling. Typical FCC catalysts are prepared with different HY crystallite sizes (0.4 and 0.9 μm), and are structurally and chemically characterized with nitrogen and argon adsorption/desorption isotherms, temperature‐programmed desorption of ammonia and infrared spectroscopy. Catalyst characterization is carried out before and after the hydrothermal treatment (steaming) of the catalyst. Pore‐size distribution analysis demonstrates that the effect of the steaming treatment in the Y zeolite results in window enlargement. The influences of structural changes of steam treatment on reactivity is evaluated with the catalytic conversion of 1,2,4‐trimethylbenzene in a novel fluidized CREC riser simulator. It is proven that steaming enlarges zeolite windows and influence the 1,2,4‐TMB product distribution. A slight modification of the window diameter is proven to significantly affect the adsorbent‐adsorbate interactions. Focus is particularly given to the catalyst selectivity toward the tetramethylbenzene isomers, and the “transition‐state shape selectivity” is proven to be controlling the product distribution and is consistent with molecular mechanics calculations. © 2005 American Institute of Chemical Engineers AIChE J, 2006
Size distributions and stability of toluene diluted heavy oil emulsions
Authors: Angle Chandra W. , Hamza Hassan A. , Dabros Tadeusz 
Published: November 2005

AbstractThe sizes and stability of oil droplets created from various concentrations of heavy oil‐in‐toluene at a fixed oil:water ratio were investigated during turbulent flow in model process water. The Reynolds number (Re) ranged from 17,000 to 34,500 and was obtained by stirred tank mixing with a Rushton turbine. The droplet sizes were monitored using laser light scattering. Results showed that at high Re and low oil concentrations (that is, low drop‐surface coverage), breakage of the droplets was the dominant process, but as Re was reduced, coalescence was dominant. Droplets were less prone to breakage as the oil concentrations in toluene increased, and droplet sizes approached a steady state quickly during mixing. Their size distributions broadened and stability increased as heavy oil in toluene increased. Stability was attributed to a surface coverage by asphaltenes and the consequent interfacial elasticity that provided resilience to breakage. Equilibrium interfacial tension σE was determined by fitting a diffusion‐limited kinetic mathematical model to the data. The Gibbs adsorption model gave a monolayer surface coverage of 3 nm2/mol asphaltenes, consistent with other published results. High zeta potential of the droplets also hindered coalescence. © 2005 American Institute of Chemical Engineers AIChE J, 2006
Hybrid generalized modular/collocation framework for distillation column synthesis
Authors: Proios Petros , Pistikopoulos Efstratios N. 
Published: November 2005

AbstractSystematic and detailed methods are proposed for the synthesis of simple, heat integrated, and complex distillation column sequences for the separation of non‐azeotropic mixtures based on formal superstructure optimization techniques. The Generalized Modular Framework (GMF) developed in previous works for the conceptual synthesis of the aforementioned sequencing problems is enhanced through the incorporation of principles of Collocation techniques for distillation order reduction. The proposed GMF/Collocation methods overcome structural complexities through systematically composed structural models and provide accurate and detailed guidelines for the optimal design and operation of the represented distillation sequences. The synthesis methods are applied for a number of sequencing case studies, generating substantial economic savings by finding systematically, accurately, and in a unified way the most cost efficient distillation column sequence. © 2005 American Institute of Chemical Engineers AIChE J, 2006
Multiobjective molecular design for integrated process‐solvent systems synthesis
Authors: Papadopoulos Athanasios I. , Linke Patrick 
Published: November 2005

AbstractThe limitations are addressed that emerge from the computer aided molecular design (CAMD) applications in the optimal synthesis of solvents through a novel approach of designing solvents built upon multiple objective optimization technology. This technology has certain advantages over single objective optimization approaches that have been used for the design of optimal solvent molecules in the past. The different synthesis drives, described by the various objective functions, are treated independently without being submitted to unnecessary fixed assumptions that bias the designs. As a result, all the underlying trends and trade‐offs among the properties of the candidate optimal molecules, as well as the structure – property relations are allowed to be revealed. The ability of the presented synthesis framework to embed highly inclusive solutions is elaborated, and the trends are revealed that dominate the relations between the utilized objective functions. It is also shown that solvent molecules, designed in the event that process performance feedback is accounted for, are existent in the Pareto optimal front. The proposed methodology is illustrated with two comprehensive examples in the design of solvents for liquid‐liquid extraction and gas‐absorption processes. © 2005 American Institute of Chemical Engineers AIChE J, 2006
A numerical study of combustion stability in emergency oxygen generators
Authors: Diakov Victor , Shafirovich Evgeny , Varma Arvind 
Published: November 2005

AbstractCombustion in sodium chlorate based emergency oxygen generators is accompanied by significant oscillations of the product oxygen flow rate, which decrease the efficiency of these devices. Reactant melting and convective heat transfer from the generated gas are key steps in the process. In this work, the effect of reactant melting on filtration combustion front propagation stability in gas generating mixtures is investigated numerically. The formation of melting regions is demonstrated in cases with significant convective gas‐to‐core heat transfer. Numerical simulations exhibit the oscillatory behavior of oxygen generation. By decreasing the effective heat of reaction, reactant melting is detrimental to combustion front stability. The simulations show growth of pulsations with increasing reaction activation energy and decreasing convective heat transfer. A strong stabilization of front propagation is observed when combustion temperature reaches the melting point of reaction product. These results identify the important factors responsible for pulsating behavior of chemical oxygen generators. © 2005 American Institute of Chemical Engineers AIChE J, 2006
Ebstein anomaly and duplication of the distal arm of chromosome 15: Report of two patients
Authors: Miller Michelle S. , Rao P. Nagesh , Dudovitz Rebecca N. , Falk Rena E. 
Published: November 2005

AbstractEbstein anomaly of the tricuspid valve is an uncommon congenital heart defect. We report two unrelated patients with Ebstein anomaly and duplication of the distal long arm of chromosome 15 (15q22 → qter and 15q24 → qter). Duplication of 15q is a well‐described phenotype that includes congenital heart defects, and these are the first cases with Ebstein anomaly. Duplication of 15q likely affects the early morphogenesis of cardiac structures, including the normal formation of the tricuspid valve. © 2005 Wiley‐Liss, Inc.
Molecular cytogenetic characterization of a familial der(1)del(1)(p36.33)dup(1)(p36.33p36.22) with variable phenotype
Authors: Tonk Vijay S. , Wilson Golder N. , Yatsenko Svetlana A. , Stankiewicz Pawel , Lupski James R. , Schutt Robert C. , Northup J.K. , Velagaleti Gopalrao V.N. 
Published: November 2005

AbstractChromosome deletions involving 1p36 are the most common known terminal rearrangements occurring at a frequency of ∼1 in 5,000 live births. In contrast, duplications of the same region have been reported rarely. We describe a familial rearrangement der(1)del(1)(p36.33)dup(1)(p36.33p36.22) identified in a mother, daughter, and son. These individuals help define a syndrome with variable mental disability, attention deficit‐hyperactivity disorder, and a distinctive facial appearance with wide palpebral fissures, broad nasal root, macrostomia, ear malformations, and prominent incisors. Based on our results we suggest that the complex rearrangement seen in our family could be the result of the breakage‐fusion‐bridge (BFB) cycles model of formation. © 2005 Wiley‐Liss, Inc.
Myhre syndrome in a female with previously undescribed symptoms: Further delineation of the phenotype
Authors: van Steensel M.A.M. , Vreeburg M. , Steijlen P.M. , de Die‐Smulders C. 
Published: November 2005

AbstractMyhre syndrome is a rare connective tissue disease characterized by nonprogressive stiffness of the large joints, short stature with a peculiar build, and a distinctive facial phenotype. Developmental delay is common. Three female patients have so far been described. Here, we report on a 16‐year‐old female with Myhre syndrome. She has vertebral defects, hypertrophic scar formation, and a stiff skin in addition to the features that have previously been reported in association with Myhre syndrome. © 2005 Wiley‐Liss, Inc.
Perlman syndrome: Clinical report and nine‐year follow‐up
Authors: Piccione Maria , Cecconi Massimiliano , Giuffrè Mario , Curto Margherita Lo , Malacarne Michela , Piro Ettore , Riccio Andrea , Corsello Giovanni 
Published: November 2005

AbstractWe present the clinical and follow‐up data of a female infant with Perlman syndrome from birth to the age of 9 years. Main features of Perlman syndrome include polyhydramnios, fetal overgrowth, neonatal macrosomia, macrocephaly, dysmorphic facial features, visceromegaly, nephroblastomatosis, and a predisposition for Wilm's tumor. In our patient, the nephromegaly with nephroblastomatosis was not present at birth or during the neonatal period; it became evident in the first months of postnatal life. A Wilm's tumor was diagnosed when she was about 1 year old. Long term follow‐up documents the natural history of Perlman syndrome and allows us to establish the long‐term prognosis of the affected individuals. © 2005 Wiley‐Liss, Inc.
A paternally derived inverted duplication of distal 14q with a terminal 14q deletion
Authors: Chen Chih‐Ping , Chern Schu‐Rern , Lin Shuan‐Pei , Lin Chyi‐Chyang , Li Yueh‐Chun , Wang Tzu‐Hao , Lee Chen‐Chi , Pan Chen‐Wen , Hsieh Lie‐Jiau , Wang Wayseen 
Published: November 2005

AbstractA girl presented with a phenotype including neonatal hypotonia, psychomotor retardation, mental retardation, short stature, and facial dysmorphism. She demonstrated common features of both 14q31‐qter duplication and terminal 14q deletion. She had undergone surgery for patent ductus arteriosus and pyloric stenosis in infancy. Her karyotype was 46,XX,der(14) dup(14)(q32.3 q31.3)del(14)(q32.3). Molecular cytogenetic analysis showed a paternally derived 14q31.3‐q32.3 duplication and a terminal 14q deletion and led to the correlations between a particular genotype and phenotype. This is the first description of a deletion and inverted duplication of 14q, and adds 14q to the growing list of the inverted duplication associated with a terminal deletion. © 2005 Wiley‐Liss, Inc.
Severe bilateral panlobular emphysema and pulmonary arterial hypoplasia: Unusual manifestations of Menkes disease
Authors: Grange Dorothy K. , Kaler Stephen G. , Albers Gary M. , Petterchak James A. , Thorpe Connie M. , deMello Daphne E. 
Published: November 2005

AbstractMenkes disease is an X‐linked recessive disorder of copper transport characterized by neurological deterioration, connective tissue, and vascular defects, abnormal hair, and death in early childhood. We report on a patient with Menkes disease in whom severe diffuse emphysema caused respiratory failure and death at 14 months of age. He had severe growth and developmental delays and other typical clinical manifestations of Menkes disease. He developed respiratory problems requiring continuous supplemental oxygen and a progressively enlarging soft tissue mass appeared on the neck. Imaging studies revealed cystic spaces in multiple lobes of the lung consistent with bullous emphysema. The neck mass was determined to be an internal jugular venous aneurysm. At autopsy, extensive emphysematous change was evident. Post‐mortem barium injections of the pulmonary arterial system revealed marked dilatation and tortuosity of the preacinar pulmonary arteries and reduced numbers of intra‐acinar arteries. Severe emphysema, presumably caused by abnormal elastin due to deficiency of the copper‐dependent enzyme lysyl oxidase, may represent an underestimated clinical complication of Menkes disease and should be considered in the differential diagnosis of chronic respiratory disease in these patients. © 2005 Wiley‐Liss, Inc.
Costello syndrome and hyperinsulinemic hypoglycemia
Authors: Alexander Saji , Ramadan Dina , Alkhayyat Haya , Al‐Sharkawi Ibrahim , Backer K.C. Aboo , El‐Sabban Fatma , Hussain Khalid 
Published: November 2005

AbstractCostello syndrome is characterized by mental retardation, loose skin, coarse facies, skeletal abnormalities, cardiovascular abnormalities (congenital heart defects, cardiomyopathy, rhythm disturbances), and predisposition to neoplasia. Endocrine abnormalities including growth hormone deficiency, adrenal insufficiency, glucose intolerance, parathyroid adenoma with hyperprolactinemia and hypoglycemia have been described. Hypoglycemia has been documented due to growth hormone and cortisol deficiency. We report on two patients with Costello syndrome and persistent hyperinsulinemic hypoglycemia and review the endocrine manifestations of Costello syndrome. Both patients required diazoxide therapy to stop the unregulated insulin secretion and maintain normoglycemia. The mechanism of persistent hyperinsulinism in patients with Costello syndrome is unclear. © 2005 Wiley‐Liss, Inc.
A patient with monosomy 1p36, atypical features and phenotypic similarities with Cantu syndrome
Authors: Tan Tiong Yang , Bankier Agnes , Slater Howard R. , Northrop Emma L. , Zacharin Margaret , Savarirayan Ravi 
Published: November 2005

AbstractWe report on a 16‐year‐old boy with a distal 1p36 deletion with some clinical features consistent with Cantu syndrome (OMIM#239850). He also has hypercholesterolemia, type II diabetes, recurrent bony fractures, and non‐alcoholic steatohepatitis, not previously described in either condition. The 1p36 deletion was detected in a screen of all chromosome subtelomeres using multiplex ligation‐dependent probe amplification and was verified using FISH with a region‐specific BAC clone. We suggest that patients suspected of having Cantu syndrome, especially those with unusual or more severe manifestations be analyzed for distal 1p36 deletions. © 2005 Wiley‐Liss, Inc.
Submicroscopic deletions and duplications in individuals with intellectual disability detected by array‐CGH
Authors: Tyson C. , Harvard C. , Locker R. , Friedman J.M. , Langlois S. , Lewis M.E.S. , Van Allen M. , Somerville M. , Arbour L. , Clarke L. , McGilivray B. , Yong S.L. , Siegel‐Bartel J. , Rajcan‐Separovic E. 
Published: November 2005

AbstractIntellectual disability (ID) affects about 3% of the population (IQ < 70), and in about 40% of moderate (IQ 35–49) to severe ID (IQ < 34), and 70% of cases of mild ID (IQ 50–70), the etiology of the disease remains unknown. It has long been suspected that chromosomal gains and losses undetectable by routine cytogenetic analysis (i.e., less than 5–10 Mb in size) are implicated in ID of unknown etiology. Array CGH has recently been used to perform a genome‐wide screen for submicroscopic gains and losses in individuals with a normal karyotype but with features suggestive of a chromosome abnormality. In two recent studies, the technique has demonstrated a ∼15% detection rate for de novo copy number changes of individual clones or groups of clones. Here, we describe a study of 22 individuals with mild to moderate ID and nonsyndromic pattern of dysmorphic features suspicious of an underlying chromosome abnormality, using the 3 Mb and 1 Mb commercial arrays (Spectral Genomics). Deletions and duplications of 16 clones, previously described to show copy number variability in normal individuals [Iafrate et al., 2004; Lapierre et al., 2004; Schoumans et al., 2004; Vermeesch et al., 2005] were seen in 21/22 subjects and were considered polymorphisms. In addition, three subjects showed submicroscopic deletions and duplications not previously reported as normal variants. Two of these submicroscopic changes were of de novo origin (microdeletions at 7q36.3 and a microduplication at 11q12.3‐13.1) and one was of unknown origin as parental testing of origin could not be performed (microduplication of Xp22.3). The clinical description of the three subjects with submicroscopic chromosomal changes at 7q36.3, 11q12.3‐13.1, Xp22.3 is provided. © 2005 Wiley‐Liss, Inc.
Cardiovascular malformations in Fryns syndrome: Is there a pathogenic role for neural crest cells?
Authors: Lin Angela E. , Pober Barbara R. , Mullen Mary P. , Slavotinek Anne M. 
Published: November 2005

AbstractWe performed a comprehensive literature and case report review to characterize the cardiovascular malformations (CVMs) associated with Fryns syndrome (OMIM #229850), a multiple congenital anomaly/mental retardation syndrome consisting of diaphragmatic defects, significant pulmonary hypoplasia, distinctive facial appearance, distal digital hypoplasia, and numerous other external and internal anomalies. A total of 112 patients meeting diagnostic guidelines for Fryns syndrome were identified, of whom 82 met narrowly defined criteria (Group I) and 30 met broader diagnostic criteria (Group II). Twelve patients reported as having Fryns syndrome with atypical features (Group III) were also analyzed. A CVM was reported in 51% (42 of 82) of Group I patients, most commonly an atrial or ventricular septal defect (VSD) (23 of 42, 55%). Conotruncal and aortic arch CVMs were common (11 of 42, 26%), but not significantly so compared to the general population of infants to age 1 year [Ferencz et al., 1997]. Recognizing that minor septal defects associated with congenital diaphragmatic hernia (CDH) may occur in response to altered hemodynamics (instead of being a bonafide CVM), we excluded four patients reported as having hemodynamically insignificant VSDs. Following these exclusions, conotruncal CVMs were found more commonly than in the general population (11 of 38, 29%, P ≤ 0.025). In Group II, 9 of 30 (30%) had a CVM with no predominant type among the small number of cases reviewed. Among the atypical Fryns syndrome patients in Group III, half (6 of 12, 50%) had a CVM; most (4 of 6, 67%) were conotruncal, in particular, type B interrupted aortic arch (3 of 4). Patients with Fryns syndrome have a high rate of CVMs, warranting thorough cardiac evaluation including echocardiogram (fetal and/or postnatal) in all patients, similar to the evaluation for other patients with diaphragmatic hernia. The possible association between conotruncal CVMs and Fryns syndrome may provide additional support for an etiologic role of genes related to neural crest cell development in the pathogenesis of Fryns syndrome and hence, congenital diaphragmatic hernia. © 2005 Wiley‐Liss, Inc.
A preliminary comparison of the hopes of researchers, clinicians, and families for the future ethical use of genetic findings on schizophrenia
Authors: DeLisi Lynn E. , Bertisch Hilary 
Published: November 2005

AbstractA written questionnaire about genetic testing was distributed to all registrants at The 2004 World Congress of Psychiatric Genetics, mailed to clinical psychiatrists obtained from a directory of clinicians practicing in New York City, and mailed to members of families who have multiple affected family members with schizophrenia. A total of 274 individuals responded (162 researchers, 64 clinicians, and 48 family members). This survey shows that the majority of family members who completed the questionnaire (83.3%) would want to be tested if a genetic test were to become available. Over half of the family members (56.2%) would want prenatal testing. Similarly, over half of the clinicians (56.3%) would recommend it, despite only 25% of the researchers reporting that it would be a future useful tool. All of the clinicians surveyed thought adoption agencies should inform families about a family history of schizophrenia, while only half of the researchers thought this should be done (51.9%). These differences in opinions between consumers, their clinicians, and researchers could be based on a lack of understanding of the amount of risk conferred to family members by reported gene variants. Providing public discussions for placing these risks in perspective should be the responsibility of researchers. Open public discussion of the ethical and social uses of the information gained from psychiatric genetic research and its limitations is encouraged. © 2005 Wiley‐Liss, Inc.