bluuebunny/crossref_metadata_2025_split huggingface dataset:
Kinetics of liquid‐phase hydrogenation of isooctenes on a Pd/γ‐alumina catalyst
Authors: Sarkar Amitava , Seth Deepyaman , Ng Flora T. T. , Rempel Garry L. 
Published: November 2005

AbstractThe kinetics of liquid‐phase hydrogenation of isooctenes [2,4,4‐trimethyl‐1‐pentene (1‐TMP) and 2,4,4‐trimethyl‐2‐pentene (2‐TMP)] was studied on a Pd/γ‐Al2O3 catalyst in a semibatch reactor. Experimental results revealed that palladium was highly active for the hydrogenation of isooctenes and did not show any significant deactivation like that of the nickel, cobalt, or platinum catalysts reported in the literature. The internal double bond of 2‐TMP was hydrogenated faster than the terminal double bond of 1‐TMP. In addition to the hydrogenation reactions, double‐bond isomerization was observed between 1‐TMP and 2‐TMP. A kinetic model was developed based on the Horiuti–Polanyi mechanism, involving a half‐hydrogenated surface intermediate and the rate‐limiting step was the first hydrogen addition. Isomerization was assumed to take place via the partly hydrogenated intermediates because no isomerization was observed in the absence of hydrogen. The dynamic reactor model consisted of material balances for the gas and the liquid phases, as well as for the porous catalyst particles. A resulting coupled ordinary differential equation–partial differential equation system was solved by Newton's method simultaneously with the minimization of the objective function from the kinetic model. The estimated model parameters with 95% confidence intervals accurately described the experimental data. The hydrogenation of both isomers was found to be strongly dependent on internal diffusion inside the catalyst particle. © 2005 American Institute of Chemical Engineers AIChE J, 2006
Unified conceptual approach to targeting and design of water and hydrogen networks
Authors: Agrawal Vibhor , Shenoy Uday V. 
Published: November 2005

AbstractAn algorithm is proposed to target the minimum freshwater for fixed flow rate (FF) problems in single‐contaminant water networks. The approach is based on an extension of the limiting composite curve concept proposed earlier for fixed contaminant‐load (FC) problems. The targeting method is elegant, noniterative, and can be applied to FF problems involving regeneration to minimize waste discharge. To design networks that achieve the minimum freshwater targets set for FF problems, an algorithm is presented based on the principle of nearest neighbors. The principle simply states that the sources to be chosen to satisfy a particular water demand must be the nearest available neighbors in terms of contaminant concentration. A significant advantage of the approach is that the same targeting concepts can be profitably used to determine the minimum makeup utility in hydrogen networks. Furthermore, hydrogen networks may be designed by the unified conceptual approach using the nearest neighbors algorithm. The hydrogen networks may then be evolved to account for the pressure constraints imposed by compressors or improved by regeneration through purification processes such as pressure‐swing adsorption. © 2005 American Institute of Chemical Engineers AIChE J, 2006
Fetal type IV glycogen storage disease: Clinical, enzymatic, and genetic data of a pure muscular form with variable and early antenatal manifestations in the same family
Authors: L'herminé‐Coulomb A. , Beuzen F. , Bouvier R. , Rolland M.O. , Froissart R. , Menez F. , Audibert F. , Labrune P. 
Published: November 2005

AbstractWe report on a family of three consecutive fetuses affected by type IV glycogen storage disease (GSD IV). In all cases, cervical cystic hygroma was observed on the 12‐week‐ultrasound examination. During the second trimester, fetal hydrops developed in the first pregnancy whereas fetal akinesia appeared in the second pregnancy. The diagnosis was suggested by microscopic examination of fetal tissues showing characteristic inclusions exclusively in striated fibers, then confirmed by enzymatic studies on frozen muscle. Antenatal diagnosis was performed on the third and fourth pregnancies: cervical cystic hygroma and low glycogen branching enzyme (GBE) activity on chorionic villi sample (CVS) were detected in the third pregnancy whereas ultrasound findings were normal and GBE activity within normal range on CVS in the fourth pregnancy. Molecular analysis showed that the mother was heterozygous for a c.1471G > C mutation in exon 12, leading to the replacement of an alanine by a tyrosine at codon 491 (p.A491T); the father was heterozygous for a c.895G > T mutation in exon 7, leading to the creation of a stop codon at position 299 (p.G299X). GSD IV has to be considered in a context of cervical cystic hygroma with normal karyotype, particularly when second trimester hydrops or akinesia develop. Enzymatic analysis of GBE must be performed on CVS or amniotic cells to confirm the diagnosis. Characteristic intracellular inclusions are specific to the disease and should be recognized, even in macerated tissues after fetal death. Genetic analysis of the GBE gene may help to shed some light on the puzzling diversity of GSD IV phenotypes. © 2005 Wiley‐Liss, Inc.
Neurofibromatosis von Recklinghausen type I phenotype and early onset of cancers in siblings compound heterozygous for mutations in MSH6
Authors: Østergaard John R. , Sunde Lone , Okkels Henrik 
Published: November 2005

AbstractWe report on a nonconsanguineous family in which two siblings with cutaneous manifestations leading to a diagnosis of neurofibromatosis type 1 (NF1) developed CNS tumors at an early age. In addition, one of them developed a T‐cell lymphoma. Neither parent had NF1. The mother was known to be heterozygous for a MSH6 mutation, and the father was found to be heterozygous for a different MSH6 mutation. Screening of MSH2, MLH1, MSH6, PMS1, PMS2, and MLH3 in the affected children disclosed that they both were compound heterozygote for the MSH6 mutations of their parents. Most recently, about a dozen other cases of inherited bi‐allelic deficiency of mismatch repair (MMR) genes associated with early onset CNS tumors, hematologic malignancy, gastrointestinal neoplasia, café‐au‐lait spots, and other NF1 features have been reported. In the present study, we summarize the clinical findings of 27 individuals homozygous or compound heterozygous for an MMR gene mutation reported in the medical literature. We suggest that biparentally inherited mutations of one of the MMR genes should be considered in children with multiple café‐au‐lait spots who have early‐onset CNS tumors, hematologic malignancies, or early onset gastrointestinal neoplasia. © 2005 Wiley‐Liss, Inc.
Promotor genotype of the platelet‐derived growth factor receptor‐α gene shows population stratification but not association with spina bifida meningomyelocele
Authors: Au K.‐S. , Northrup H. , Kirkpatrick T.J. , Volcik K.A. , Fletcher J.M. , Townsend I.T. , Blanton S.H. , Tyerman G.H. , Villarreal G. , King T.M. 
Published: November 2005

AbstractNeural tube defects (NTDs) constitute a major group of congenital malformations with an overall incidence of approximately 1–2 in 1,000 live births in the United States. Hispanic Americans have a 2.5 times higher risk than the Caucasian population. Spina bifida meningomyelocele (SBMM) is a major clinical presentation of NTDs resulting from lack of closure of the spinal cord caudal to the head. In a previous study of spina bifida (SB) patients of European Caucasian descent, it was suggested that specific haplotypes of the platelet‐derived growth factor receptor‐α (PDGFRA) gene P1 promoter strongly affected the rate of NTD genesis. In our study, we evaluated the association of PDGFRA P1 in a group of 407 parent–child triads (167 Caucasian, 240 Hispanics) and 164 unrelated controls (89 Caucasian, 75 Hispanic). To fully evaluate the association of PDGFRA P1, we performed both transmission‐disequilibrium test (TDT) and association analyses to test the hypotheses that PDGFRA P1 was (1) transmitted preferentially in SBMM affected children and (2) associated with the condition of SBMM comparing affected children to unaffected controls. We did find that there was a different allelic and genotypic distribution of PDGFRA P1 when comparing Hispanics and Caucasians. However, neither ethnic group showed strong association between SBMM and the PDGFRA P1 region. These findings suggest that PDGFRA P1 does not have a major role in the development of SBMM. © 2005 Wiley‐Liss, Inc.
Majewski osteodysplastic primordial dwarfism type II (MOPD II) complicated by stroke: Clinical report and review of cerebral vascular anomalies
Authors: Brancati Francesco , Castori Marco , Mingarelli Rita , Dallapiccola Bruno 
Published: November 2005

AbstractWe report on a 2 9/12‐year‐old boy with disproportionate short stature, microcephaly, subtle craniofacial dysmorphisms, and generalized skeletal dysplasia, who developed a left hemiparesis. Brain neuroimaging disclosed a complex cerebral vascular anomaly (CVA) with stenosis of the right anterior cerebral artery and telangiectatic collateral vessels supplying the cerebral cortex, consistent with moyamoya disease. Based on clinical and skeletal features, a diagnosis of Majewski osteodysplastic primordial dwarfism type II (MOPD II) was established. Review of 16 published patients with CVA affected by either Seckel syndrome or MOPD II suggested that CVA is preferentially associated to the latter subtype affecting about 1/4 of the patients. © 2005 Wiley‐Liss, Inc.
Epidemiology of hemimegalencephaly: A case series and review
Authors: Tinkle Brad T. , Schorry Elizabeth K. , Franz David N. , Crone Kerry R. , Saal Howard M. 
Published: November 2005

AbstractHemimegalencephaly (HME) is a congenital brain malformation characterized by unilateral enlargement of the cerebral hemisphere. Clinically, HME is typically associated with hemiparesis, psychomotor retardation, and intractable seizures usually apparent soon after birth. HME is often an isolated finding, but it has been described as an occasional feature of a large number of syndromes, many of which may not be readily identified at birth. There are a multitude of case series and reports of HME in the English literature; however, there is no comprehensive, unbiased, detailed survey characterizing the proportion of cases of HME that are associated with a syndrome. We performed a retrospective study of all cases of HME seen at our institution from 1990 to 2003. Of the 15 cases of HME identified, 53% (8/15) were non‐syndromic and 47% (7/15) of the cases were associated with a known or suspected genetic syndrome. In patients with syndromic HME, many of the syndromic features were not readily discernible at birth or in early infancy. It is, therefore, imperative to continually evaluate any infant with HME for signs and symptoms of these and other syndromes. Knowing the relative differential diagnosis will lead to a more comprehensive evaluation, improvement in expectant management, and appropriate counseling of families before considering radical surgical options such as hemispherectomy. © 2005 Wiley‐Liss, Inc.
Prion genotypes in Central America suggest selection for the V129 allele
Authors: Hardy John , Scholz Sonja , Evans Whitney , Goldfarb Lev , Singleton Andrew 
Published: November 2005

AbstractUsing the well‐characterized, human diversity sample series, we show that the V129 prion allele has a very high frequency in South American populations relative to the East Asian populations from which it arose. We suggest there has been selection at the prion locus, possibly mediated by Kuru‐like diseases, which has influenced its allele frequency. © 2005 Wiley‐Liss, Inc.
A genetic approach to fracture epidemiology in childhood
Authors: Tinkle Brad T. , Wenstrup Richard J. 
Published: November 2005

AbstractThe purpose of this report is to provide a review of both childhood fracture epidemiology and known heritable causes for fracture predisposition to the Medical Geneticist, who is frequently consulted to assess children with multiple or unexplained fractures for a physiologic etiology. A detailed knowledge of the clinical and laboratory evaluation for osteogenesis imperfecta (OI) and other single‐gene disorders is obviously essential to complete a useful evaluation of such children. The experienced clinician will immediately recognize that single gene disorders represent only a small fraction of these patients. In infants, non‐accidental trauma (NAT) unfortunately is the likely explanation for the fracture pattern, but in some infants, and certainly in older children with recurrent fractures, no medical explanations can be found. Recent studies in which bone mineral density (BMD) has been associated with genetic variation at a number of candidate genes are promising but these studies are too premature yet to be used clinically. Nonetheless, we do expect that in the future whole‐genome approaches in conjunction with key clinical and epidemiological variables may be combined through an informatics approach to create better predictors of fracture susceptibility for these populations of patients. © 2005 Wiley‐Liss, Inc.
Noise‐enhanced balance control in patients with diabetes and patients with stroke
Authors: Priplata Attila A. , Patritti Benjamin L. , Niemi James B. , Hughes Richard , Gravelle Denise C. , Lipsitz Lewis A. , Veves Aristidis , Stein Joel , Bonato Paolo , Collins James J. 
Published: November 2005

AbstractObjectiveSomatosensory function declines with diabetic neuropathy and often with stroke, resulting in diminished motor performance. Recently, it has been shown that input noise can enhance human sensorimotor function. The goal of this study was to investigate whether subsensory mechanical noise applied to the soles of the feet via vibrating insoles can be used to improve quiet‐standing balance control in 15 patients with diabetic neuropathy and 15 patients with stroke. Sway data of 12 healthy elderly subjects from a previous study on vibrating insoles were added for comparison.MethodsFive traditional sway parameters and three sway parameters from random‐walk analysis were computed for each trial (no noise or noise).ResultsApplication of noise resulted in a statistically significant reduction in each of the eight sway parameters in the subjects with diabetic neuropathy, the subjects with stroke, and the elderly subjects. We also found that higher levels of baseline postural sway in sensory‐impaired individuals was correlated with greater improvements in balance control with input noise.InterpretationThis work indicates that noise‐based devices could ameliorate diabetic and stroke impairments in balance control. Ann Neurol 2006
Rapid eye movement sleep behavior disorder and potassium channel antibody–associated limbic encephalitis
Authors: Iranzo Alex , Graus Francesc , Clover Linda , Morera Jaume , Bruna Jordi , Vilar Carlos , Martínez‐Rodriguez José Enrique , Vincent Angela , Santamaría Joan 
Published: November 2005

AbstractOf six patients registered in our center with nonparaneoplastic limbic encephalitis associated with antibodies to voltage‐gated potassium channels, the five men had rapid eye movement sleep behavior disorder (RBD) coincident with voltage‐gated potassium channel antibody–associated limbic encephalitis onset. In three patients, immunosuppression resulted in resolution of RBD in parallel with remission of the limbic syndrome. RBD persisted in two patients with partial resolution of the limbic syndrome. Our findings suggest that RBD is frequent in the setting of voltage‐gated potassium channel antibody–associated limbic encephalitis and can be related to autoimmune‐mediated mechanisms. In addition, these observations suggest that impairment of the limbic system may play a role in the pathogenesis of RBD. Ann neurol 2006
APOE ε4 is not associated with Alzheimer's disease in elderly Nigerians
Authors: Gureje Oye , Ogunniyi Adesola , Baiyewu Olusegun , Price Brandon , Unverzagt Frederick W. , Evans Rebecca M. , Smith‐Gamble Valerie , Lane Kathleen A. , Gao Sujuan , Hall Kathleen S. , Hendrie Hugh C. , Murrell Jill R. 
Published: November 2005

AbstractSince 1992, research teams from Indiana University and the University of Ibadan have been collecting and comparing data from two diverse, elderly populations to identify risk factors for dementia and Alzheimer's disease. Apolipoprotein E (APOE) was genotyped in 2,245 Nigerian samples. Of these, 830 had a diagnosis: 459 were normal, and 140 had dementia including 123 diagnosed with Alzheimer's disease. In contrast with other populations, the APOE ε4 allele was not significantly associated with Alzheimer's disease or dementia. This lack of association in the Yoruba might reflect genetic variation, environmental factors, as well as genetic/environmental interactions. Ann neurol 2006
Clinical and biochemical spectrum of D‐bifunctional protein deficiency
Authors: Ferdinandusse Sacha , Denis Simone , Mooyer Petra A. W. , Dekker Conny , Duran Marinus , Soorani‐Lunsing Roelineke J. , Boltshauser Eugen , Macaya Alfons , Gärtner Jutta , Majoie Charles B. L. M. , Barth Peter G. , Wanders Ronald J. A. , Poll‐The Bwee Tien 
Published: November 2005

AbstractObjectiveD‐bifunctional protein deficiency is an autosomal recessive inborn error of peroxisomal fatty acid oxidation. Although case reports and small series of patients have been published, these do not give a complete and balanced picture of the clinical and biochemical spectrum associated with this disorder.MethodsTo improve early recognition, diagnosis, prognosis, and management of this disorder and to provide markers for life expectancy, we performed extensive biochemical studies in a large cohort of D‐bifunctional protein–deficient patients and sent out questionnaires about clinical signs and symptoms to the responsible physicians.ResultsVirtually all children presented with neonatal hypotonia and seizures and died within the first 2 years of life without achieving any developmental milestones. However, within our cohort, 12 patients survived beyond the age of 2 years, and detailed information on 5 patients with prolonged survival (≥7.5 years) is provided.InterpretationBiochemical analyses showed that there is a clear correlation between several biochemical parameters and survival of the patient, with C26:0 β‐oxidation activity in cultured skin fibroblasts being the best predictive marker for life expectancy. Remarkably, three patients were identified without biochemical abnormalities in plasma, stressing that D‐bifunctional protein deficiency cannot be excluded when all peroxisomal parameters in plasma are normal. Ann Neurol 2006
Rates of cerebral atrophy in autopsy‐confirmed progressive supranuclear palsy
Authors: Josephs Keith A. , Whitwell Jennifer L. , Boeve Bradley F. , Shiung Maria M. , Gunter Jeffrey L. , Parisi Joseph E. , Dickson Dennis W. , Jack Clifford R. 
Published: November 2005

AbstractObjectiveTo determine the rates of cerebral atrophy and ventricular expansion in six patients with autopsy confirmed progressive supranuclear palsy (PSP) and multiple antemortem volumetric head MRI scans.MethodsThis study uses the boundary shift integral to determine rates of cerebral atrophy and ventricular expansion in six patients with pathologically confirmed PSP compared with six age‐ and sex‐matched control subjects. Results: Patients with PSP had a rate of cerebral atrophy and ventricular expansion of 1.3 and 7.0% per year compared with 0.4 and 1.8% in control subjects.InterpretationThese rates provide a benchmark that can be used to monitor future treatment response in PSP. Ann Neurol 2006
Reaktionen in micellaren Systemen
Authors: Dwars Torsten , Paetzold Eckhard , Oehme Günther 
Published: November 2005

AbstractDie Vorstellung einer “grünen Chemie” hat selbst den präparativ arbeitenden Organiker ermutigt, Wasser als Lösungsmittel in Betracht zu ziehen. Durch Zusätze von Amphiphilen bestimmter Struktur kann man erstaunliche Aktivitäten und Selektivitäten erzielen. Die von den Tensiden gebildeten Assoziate variieren in ihrer Morphologie je nach Temperatur und Konzentration. In der Regel arbeitet man im Bereich einfacher sphärischer Aggregate, der nanometergroßen Micellen. Da in Micellen ein starker Polaritätsgradient von der hydrophilen Oberfläche zum hydrophoben Kern vorliegt, können sowohl unpolare als auch polare Reaktanten solubilisiert werden. Dadurch kommt es oft im Vergleich zur umgebenden Wasserphase zu einer Konzentrierung der Reaktionspartner und damit zu einer Beschleunigung der Reaktion. Darüber hinaus treten selektive Effekte auf. Micellare Systeme können kinetisch als mikroheterogene Zweiphasensysteme behandelt werden.
Soft Nanotechnology with Soft Nanoparticles
Authors: Nayak Satish , Lyon L. Andrew 
Published: November 2005

AbstractThe last decade of research in the physical sciences has seen a dramatic increase in the study of nanoscale materials. Today, “nanoscience” has emerged as a multidisciplinary effort, wherein obtaining a fundamental understanding of the optical, electrical, magnetic, and mechanical properties of nanostructures promises to deliver the next generation of functional materials for a wide range of applications. While this range of efforts is extremely broad, much of the work has focused on “hard” materials, such as Buckyballs, carbon nanotubes, metals, semiconductors, and organic or inorganic dielectrics. Meanwhile, the soft materials of current interest typically include conducting or emissive polymers for “plastic electronics” applications. Despite the continued interest in these established areas of nanoscience, new classes of soft nanomaterials are being developed from more traditional polymeric constructs. Specifically, nanostructured hydrogels are emerging as a promising group of materials for multiple biotechnology applications as the need for advanced materials in the post‐genomic era grows. This review will present some of the recent advances in the marriage between water‐swellable networks and nanoscience.
Reaction of the N‐heterocyclic carbene, 1,3‐dimesityl‐ imidazol‐2‐ylidene, with a uranyl triflate complex, UO2(OTf)2(thf)3
Authors: Oldham Susan M. , Scott Brian L. , Oldham Warren J. 
Published: November 2005

AbstractThe N‐heterocyclic carbene, 1,3‐dimesityl‐imidazol‐2‐ylidene (IMes) reacts with tetrahydrofuran (THF) in the presence of an oxidizing uranyl triflate complex, UO2(OTf)2(thf)3 (−OTf = −OSO2CF3), to give 1,4‐bis(1,3‐dimesityl‐2‐imidazolium)‐1,3‐butadiene bis(trifluoromethanesulfonate), formally understood as the coupling product of two equivalents of IMes with [CHCHCHCH](OTf)2. Copyright © 2005 John Wiley & Sons, Ltd.
Synthesis of two luminescent coordination polymers based on self‐assembly of Zn(II) with polycarboxylic acids ligands and heteroaromatic N‐donor
Authors: Song Yi‐Shan , Yan Bing , Chen Zhen‐Xia 
Published: November 2005

AbstractUsing the principles of molecular self‐assembly, two novel zinc complexes {[Zn(phth)(bipy)(H2O)][Zn(phth)(bipy)]·H2O}n (1) and [Zn(1,2,4‐btc)(bipy)(H2O)·2H2O]n (2) were obtained by hydrothermal reaction of Zn(CH3COO)2·2H2O with phthalic acid (phth), 1,2,4‐benzenetricarboxylic acid (1,2,4‐btc) and 2,2′‐bipyridine (bipy) respectively, and characterized by single‐crystal X‐ray diffraction. The crystal structures reveal that both complexes form one‐dimensional chain structures, and the zinc ions are five‐coordinated; there are two types of metal environment in the structure of the complex 1. The photophysical properties have been investigated with fluorescence excitation and emission spectra. Copyright © 2005 John Wiley & Sons, Ltd.