bluuebunny/crossref_metadata_2025_split huggingface dataset:
Er3O2F5: Ein Erbiumoxidfluorid mit Vernier‐Struktur
Authors: Müller‐Bunz Helge , Janka Oliver , Schleid Thomas 
Published: December 2006

AbstractEr3O2F5: An Erbium Oxide Fluoride with Vernier‐Type StructureAttempts to synthesize multinary erbium‐trifluoride derivatives (e. g. Er3F[Si3O10], Er4F2[Si2O7][SiO4], CsEr2F7, and RbEr3F10) from mixtures of ErOF‐contaminated erbium trifluoride (ErF3) itself and appropriate other components (such as Er2O3 and SiO2 or CsF and RbF, respectively) frequently resulted in the formation of pale pink, transparent, lath‐shaped single crystals of Er3O2F5 (orthorhombic, Pnma; a = 562.48(5), b = 1710.16(14), c = 537.43(4) pm; Z = 4) as by‐product, typically after seven days at 800 °C and regardless of the applied reaction‐container material (evacuated torch‐sealed silica or silica‐jacketed arc‐welded tantalum capsules). Its crystal structure, often described as a vernier‐type arrangement consisting of two interpenetrating and almost misfitting lattices (ErOF and ErF3), contains two crystallographically different Er3+ cations in the eight‐ and seven‐plus‐one‐fold anionic coordination of bicapped trigonal prisms. Whereas (Er1)3+ carries four O2−
and F− anions each, (Er2)3+ resides in the neighbourhood of only two O2−, but five plus one F− anions. As the main structural feature, however, one can consider O2−‐centred (Er3+)4 tetrahedra which share common edges to form linear double strands of the composition $^{1}_{\infty}\rm \{[O_{2}Er_{3}]^{5+}\}(d(O^{2-}-Er^{3+}) = 220-234 pm, CN = 4)$. Running parallel to the [100] direction and assembling like a hexagonal closest rod‐packing, their electroneutralization and three‐dimensional interconnection is achieved by three crystallographically independent F− anions (d(F−−Er3+) = 221 − 251 plus 281 pm) in three‐ and two‐plus‐two‐fold coordination of the Er3+ cations, respectively.
Bis(2,4,6‐trimethylphenyl)tellurium(IV) Dicyanide: the First Crystal Structure for a Tellurium Cyanide of the Type R2Te(CN)2
Authors: Klapötke Thomas M. , Krumm Burkhard , Scherr Matthias , Schwab Ingo 
Published: December 2006

AbstractThe reaction of Mes2TeF2 (Mes = 2,4,6‐trimethylphenyl) with trimethylsilyl cyanide yields the corresponding tellurium(IV) dicyanide Mes2Te(CN)2. Isolation of suitable crystals allows the determination of the first crystal structure of a compound of the type R2Te(CN)2.
Elektrochemische Synthese von Ag5Pb2O6
Authors: Oberndorfer C. P. M. , Jansen M. 
Published: December 2006

AbstractElectrochemical Synthesis of Ag5Pb2O6Ag5Pb2O6 was prepared by anodic oxidation of silver in a PbO2 suspension in an aqueous KOH solution. Phase purity was checked by a Rietveld‐fit of the X‐ray powder pattern, thermal and chemical analysis. The compound exhibits ionic conductivity. It' anisotropy leads to an orientation of the crystallites on the silver anode.
Extracellular matrix turnover and disease severity in Anderson–Fabry disease
Authors: Shah J. S. , Hughes D. A. , Tayebjee M. H. , MacFadyen R. J. , Mehta A. B. , Elliott P. M. 
Published: December 2006

SummaryBackground: Anderson–Fabry Disease (AFD) is an inherited metabolic disease associated with premature death secondary to cardiovascular and renal disease. Patients with AFD develop progressive left ventricular (LV) remodelling and heart failure. We hypothesized that altered extracellular matrix (ECM) turnover contributes to the pathophysiology of cardiac disease in AFD. Methods and Results: Twenty‐nine consecutive patients (44.1 ± 11.7 years, 15 male) with AFD and 21 normal controls (39.7 ± 11.3 years, 10 male) had serum analysed for matrix metalloproteinase‐9 (MMP‐9), and tissue inhibitor of matrix metalloproteinase‐1 and ‐2 (TIMP‐1, TIMP‐2). All patients underwent clinical assessment, echocardiography and Mainz Severity Score Index (MSSI) measurement, a validated severity score in AFD. MMP‐9 levels were significantly higher in patients than controls (1003.8 ± 337.8 ng/ml vs 576.7 ± 276.3 ng/ml respectively, p < 0.001). There were no differences in TIMP levels between patients and controls. There was a positive correlation between MMP‐9 levels and MSSI (r = 0.5, p = 0.01). There was a negative correlation between MMP‐9 and endocardial fractional shortening (FS) (r = −0.5, p = 0.01) and mid‐wall FS (r = −0.6, p = 0.001). There was no correlation between other echocardiographic parameters and MMP‐9 levels. These relations were independent of age and sex using stepwise linear regression analysis. Conclusions: Patients with AFD have abnormal ECM turnover compared to normal controls. The correlation between MMP‐9 levels and systolic function suggests that altered ECM turnover is important in cardiac remodelling. The association between MMP‐9 and overall disease severity suggests that circulating levels of MMP‐9 may provide a useful marker for assessing the response of patients with AFD to enzyme replacement treatment.
The natural history of Niemann–Pick disease type C in the UK
Authors: Imrie J. , Dasgupta S. , Besley G. T. N. , Harris C. , Heptinstall L. , Knight S. , Vanier M. T. , Fensom A. H. , Ward C. , Jacklin E. , Whitehouse C. , Wraith J. E. 
Published: December 2006

SummaryNiemann–Pick disease type C (NPC) is an autosomal recessive, neurovisceral lipid storage disorder. Mutations in two genes (NPC1 and NPC2) produce indistinguishable clinical phenotypes by biochemical mechanisms that have not yet been entirely clarified. The wide spectrum of clinical presentations of NPC includes hepatic and pulmonary disease as well as a range of neuropsychiatric disorders. Late‐onset disease has been increasingly recognized as the biochemical diagnosis of NPC has been more widely applied in adult neurology clinics. The clinical presentation and follow‐up of 94 patients with NPC is described, 58 of whom were still alive at the time this report was prepared. The age at diagnosis ranged from the prenatal period (with hydrops fetalis) up to 51 years. This review of NPC patients in the UK confirms the phenotypic variability of this inherited lipid storage disorder reported elsewhere. Although a non‐neuronopathic variant has been described, most patients in this series who survived childhood inevitably suffered neurological and in some cases neuropsychiatric deterioration. While symptomatic treatment, such as anticholinergic and antiepileptic drugs, can alleviate some aspects of the disease, there is a clear need to develop a specific treatment for this progressively debilitating neurodegenerative disorder.
Neuropsychological profile of adult patients with Niemann–Pick C1 (NPC1) mutations
Authors: Klarner B. , Klünemann H. H. , Lürding R. , Aslanidis C. , Rupprecht R. 
Published: December 2006

SummaryNiemann–Pick type C disease is a fatal neurovisceral disorder linked to dysregulation in cholesterol processing. A medication for this disease is currently being tested in clinical trials. However, there is a lack of information on neuropsychological testing parameters for this disease. One aim of this pilot study was to evaluate a test battery that could be used to assess cognitive deficits in different stages of the disease. A second aim was to determine whether specific functional deficits are associated with certain disease stages. Eight men and two women (19–40 years of age) harbouring mutations in the gene coding for the cholesterol trafficking protein NPC1 were put through the same test battery independently of their disease stage. The external staging criterion was based on a five‐step clinical scale. Trail Making tests A & B and verbal fluency were sensitive indicators at early stages of NPC. Corsi Block‐Tapping, Mini Mental Status, Find Similarities and Clock Drawing showed abnormal results in patients with advanced disease. The Grooved Pegboard, Trail Making and Mosaic tests were unsuitable in advanced disease due to impaired fine motor skills. We observed that visuospatial working memory was less affected by the neurodegenerative process than verbal working memory. The series of tests used here could be supplemented by the severe impairment battery and Raven matrices tests for patients with advanced disease.
The course of life and quality of life of early and continuously treated Dutch patients with phenylketonuria
Authors: Bosch A. M. , Tybout W. , van Spronsen F. J. , de Valk H. W. , Wijburg F. A. , Grootenhuis M. A. 
Published: December 2006

SummaryPhenylketonuria (PKU; OMIM 261600) is an autosomal recessive disorder of phenylalanine metabolism caused by a deficiency of the enzyme phenylalanine hydroxylase (PAH; EC 1.14.16.1). Cognitive problems, neuropsychological abnormalities and psychosocial problems have been reported frequently in children and adolescents with PKU, even in those who are treated early and continuously. However, the developmental consequences in adulthood of growing up with PKU are not well known. The aim of this study was to assess the course of life, sociodemographic outcomes and health‐related quality of life in young adult patients with PKU identified on neonatal screening who were continuously on treatment. A total of 32 PKU patients 18 to 30 years old completed the Course of Life questionnaire, the RAND‐36 Health Survey, and the cognitive scale of the TNO‐AZL Adult Quality of Life (TAAQoL) questionnaire. The results of the Course of Life and Health‐Related Quality of Life questionnaires were comparable to controls, except that a higher percentage received special education in primary school. Their educational attainment, however, was comparable to that of their peers. The results of this study demonstrate that although PKU is a chronic disease with the burden of strict dietary control, early and continuously treated patients with PKU can have a normal health‐related quality of life and course of life.
Hyperlipidaemia due to carnitine palmitoyltransferase I deficiency
Authors: Worthington H. , Olpin S. E. , Blumenthal I. , Morris A. A. M. 
Published: December 2006

SummaryWe report a patient with carnitine palmitoyltransferase I (CPT I) deficiency, who presented with acute encephalopathy at 6 months of age. This was precipitated by an episode of gastroenteritis. No hypoglycaemia was documented, but there was hepatomegaly; blood tests revealed raised transaminases, a coagulopathy and severe hypertriglyceridaemia (48.8 mmol/L) and hypercholesterolaemia (9.5 mmol/L). The hyperlipidaemia resolved within 3 days of treatment and did not recur. At 2 years of age, the patient's liver function, growth and development are all normal. Hyperlipidaemia has been reported during acute illness in previous patients with CPT I deficiency but it is not a well‐recognized feature; it should alert metabolic specialists to this potential diagnosis.
Restricted upper extremity range of motion in mucopolysaccharidosis type I: no response to one year of enzyme replacement therapy
Authors: Cox‐Brinkman J. , Smeulders M. J. C. , Hollak C. E. M. , Wijburg F. A. 
Published: December 2006

SummaryBackground: Mucopolysaccharidosis type I (MPS I) results from deficiency of the lysosomal enzyme α‐L‐iduronidase (IDUA). Glycosaminoglycans (GAGs) accumulate in multiple organs and the storage in connective tissues results in restricted mobility. Previous studies provided evidence of an improvement in range of motion (ROM), as measured by goniometry, after supplementation of recombinant enzyme (ERT). Aim: The aim of the study was to evaluate the efficacy of ERT in improving ROM in patients with MPS I, using a blinded three‐dimensional (3D) video analysis of unrestricted movements of the upper extremities. Methods: In 6 patients with the attenuated phenotype of MPS I, the longitudinal changes in the upper extremity ROM were analysed by 3D video analysis during one year of ERT. Results: At baseline, all studied movements except for elbow flexion were restricted. No significant improvement of the restricted upper extremity ROM was observed after one year of ERT. Conclusion: Long‐standing restrictions in ROM probably cannot be reversed by ERT. Early initiation of treatment might be essential to prevent irreversible functional defects.
Spatio‐temporal dynamics of Scotinophara lurida (Hemiptera: Pentatomidae) in rice fields
Authors: Kim Hunsung , Kim Seung‐Tae , Jung Myung‐Pyo , Lee Joon‐Ho 
Published: December 2006

AbstractStudying the spatial pattern of insect pests and the temporal stability of their patterns is important in understanding underlying ecological mechanisms and in developing pest management programs in cultivated crop systems. To elucidate the spatio‐temporal pattern of the black rice bug, Scotinophara lurida, in rice fields, samplings were conducted in two rice fields over 2 years. Using spatial analysis by distance indices, the spatial pattern of each developmental stage of S. lurida and their temporal stability of the spatial pattern were identified. Most of the Ia (the index of aggregation) values for overwintered adults and eggs of S. lurda were close to 1, indicating random distribution pattern while nymphs and new adults mainly had Ia values >1, indicating an aggregated distribution pattern. According to spatial association analysis between successive samples using X (the index of spatial association), the spatial pattern of S. lurida showed strong temporal stability throughout the season. Also, there was strong association between the spatial patterns of developmental stages, indicating the great effect of the spatial pattern of the previous developmental stage on that of later developmental stage. Factors influencing the spatial pattern and spatial stability of S. lurida are discussed.
Structure and regeneration patterns of the Juniperus excelsa Bieb. stands in the central part of the Nestos valley in the northeast of Greece, in the context of anthropogenic disturbances and nurse plant facilitation
Authors: Milios Elias , Pipinis Elias , Petrou Petros , Akritidou Sofia , Smiris Paulos , Aslanidou Maria 
Published: December 2006

AbstractIn order to analyse the structure and regeneration patterns of the Juniperus excelsa Bieb. stands on the slopes of the Nestos valley, 40 sample plots were established and more than 120 J. excelsa trees and seedlings were cut and collected. The main results of this research indicate that stand structure, species mixture, age structure and stand development patterns were strongly determined by the interaction of anthropogenic disturbances such as grazing and illegal cuttings, and the facilitation of adult J. excelsa trees in the establishment of the J. excelsa seedlings. Their effect was influenced by site quality and J. excelsa together with other species ecology. Under present conditions, almost all J. excelsa seedlings, except for a small number of plants, are found under the facilitation of nurse plants that belong to the same species. It seems that the layer of litter underneath the nurse plants combined with the protection of seedlings against grazing play an important role in the establishment and growth of J. excelsa seedlings.
Influence of body size on coexistence of bird species
Authors: Leyequién Eurídice , de Boer Willem F. , Cleef Antoine 
Published: December 2006

AbstractTheory suggests that body size is an important factor in determining interspecific competition and, ultimately, in structuring ecological communities. However, there is a lack of pragmatic studies linking body size and interspecific competition to patterns in ecological communities. The objective of the present study was to determine the effect of body size (mass) on competitive interactions between bird pairs and to investigate the influence of food guilds. Point‐counts were carried out in nine sites every month from November 2002 to November 2003 in the Cuetzalan Region, Mexico, and we used presence/absence and abundance data for the analyses. To calculate the strength of competition we used the Angle Frequency Method to extract form factors from 20 pairwise interactions. A prototype competition interaction and random pairs were also constructed. We used clustering techniques (PCA) to calculate the dissimilarity scores (distances,D) of each of the pairwise interactions to the prototype competition and random pairs and one‐way ANOVA to test for differences between the means of the random and competitive pairs. The ratio in body mass (lnBM) for each of the interacting pairs was calculated, and the association between the lnBM ratio and the strength of competition (D) was tested using a Pearson product‐moment correlation coefficient. To test for the influence of foraging guilds we used a univariate general linear model. Our results demonstrate a significant negative relationship between bird body mass ratio and competition strength – i.e. competition strength increased when the body masses of the birds became more similar. We did not find a significant influence of foraging guild on the relationship between body mass ratio and competition strength. On the basis of these results, we suggest that high variation in body sizes amongst sympatric species promotes coexistence in communities.
Fatty Acid Composition of the Oil from Developing Seeds of Different Varieties of Safflower (Carthamus tinctorius L.)
Authors: Gecgel Umit , Demirci Mehmet , Esendal Enver , Tasan Murat 
Published: December 2006

AbstractFatty acid composition and moisture and oil content were determined for Montola‐2001 and Centennial safflower varieties at three different harvest dates from flowering to maturity, which were grown as autumn and spring crops in two different locations in 2001–2002 and 2002–2003. The experiment was carried out using split–split plots in a randomized complete block design with three replicates. Sowing dates affected oil content and fatty acid compositions significantly (P < 0.01), whereas moisture content in both years was not significantly affected. Moisture content declined 15 days from flowering period to maturity, while oil content increased. The rate of the palmitic acid formation decreased in both varieties 15 days from flowering period to maturity, whereas formation rates of the oleic and linoleic acids increased in Montola‐2001 and Centennial varieties, respectively.
Physical Properties of Polyurethanes Produced from Polyols from Seed Oils: II. Foams
Authors: Narine Suresh S. , Kong Xiaohua , Bouzidi Laziz , Sporns Peter 
Published: December 2006

AbstractRigid polyurethane (PU) foams were prepared using three North American seed oil starting materials. Polyol with terminal primary hydroxyl groups synthesized from canola oil by ozonolysis and hydrogenation based technology, commercially available soybean based polyol and crude castor oil were reacted with aromatic diphenylmethane diisocyanate to prepare the foams. Their physical and thermal properties were studied and compared using dynamic mechanical analysis and thermogravimetric analysis techniques, and their cellular structures were investigated by scanning electron microscope. The chemical diversity of the starting materials allowed the evaluation of the effect of dangling chain on the properties of the foams. The reactivity of soybean oil‐derived polyols and of unrefined crude castor oil were found to be lower than that of the canola based polyol as shown by their processing parameters (cream, rising and gel times) and FTIR. Canola‐PU foam demonstrated better compressive properties than Soybean‐PU foam but less than Castor‐PU foam. The differences in performance were found to be related to the differences in the number and position of OH‐groups and dangling chains in the starting materials, and to the differences in cellular structure.
Time, Temperature, and Concentration Dependence of Ricinelaidic Acid–Canola Oil Organogelation
Authors: Wright Amanda J. , Marangoni Alejandro G. 
Published: December 2006

AbstractCanola oil (CO) gels were formed using ricinelaidic acid (REA), a hydroxylated fatty acid, and the time, temperature, and concentration dependence of the resulting gel structure was studied using small‐deformation rheology, light microscopy, and powder X‐ray diffraction (XRD). Between 5 °C and 30 °C, REA concentration had a significant influence on gel elasticity (P < 0.05), whereas temperature had a relatively lesser influence on gel rheology. Differences were observed in the scaling exponent of G′LVR with concentration above 20 °C, which were also correlated with significant differences in gelation time at 20 °C. However, the 5% gels at 5 °C, 20 °C, and 35 °C displayed similar microstructures and behaved like weak gels stabilized by junction zones. Most of the gels studied (i.e., the 2%, 3%, 4%, and 5% gels at 15 °C, 20 °C, and 25 °C) consisted of long, thin, fibrous REA strands, although at 25 °C, the 2% gel was characterized by more transient and circular entities. During 28 days’ storage, there were no apparent changes detected in gel microstructure by microscopy or XRD, despite increases in the gel's opacity.
CHANGES IN PERCEPTIONS OF QUALITY OF, AND ACCESS TO, SERVICES AMONG CLIENTS OF A FRACTIONAL FRANCHISE NETWORK IN NEPAL
Authors: AGHA SOHAIL , GAGE ANASTASIA , BALAL ASMA 
Published: December 2006

Summary.With declining levels of international donor funding for financing reproductive health programmes, developing country governments and international donors are looking towards private sector strategies to expand the supply of quality reproductive health services. One of the challenges of a health franchise is to improve the quality of services provided by independent private practitioners. Private providers are more likely to abide by the quality standards set by a franchiser if they see a financial benefit resulting from franchise participation. This study was conducted to measure whether (a) there were improvements in perceived quality of care and perceived access to health facilities once these facilities became part of a franchise and (b) improvements in perceived quality and perceived access were associated with increased client loyalty to franchised clinics. Franchisees were given basic reproductive health training for seven days and services marketing training for two days. Exit interviews were conducted with male and female clients at health facilities. A pre-test measurement was taken in April 2001, prior to the start of project activities. A post-test measurement was taken in February/March 2002, about 9 months after the pre-test. Multilevel regression analysis, which takes the hierarchical structure of the data into account, was used for the analysis. After taking provider-level variation into account and controlling for client characteristics, the analyses showed significant improvements in perceived quality of care and perceived access to services. Private provider participation in a franchise network helps improve client perceptions of quality of, and access to, services. Improvements in client perceptions of quality and access contribute to increased client loyalty to franchised clinics. Once increased client loyalty translates into higher client volumes, providers are likely to see the benefits of franchise participation. In turn, this should lead to increased provider willingness to remain part of the franchise and to abide by the standards of quality set by the franchiser.