bluuebunny/crossref_metadata_2025_split huggingface dataset:
A newly recognized autosomal recessive syndrome with short stature and oculo‐skeletal involvement
Authors: Mégarbané André , Ghanem Ismat , Waked Naji , Dagher Fernand 
Published: June 2006

AbstractThis report describes a young girl and her cousin presenting with postnatal short stature, strabismus, photophobia, retinitis pigmentosa, short neck, rhizomelic shortening of the long bones, short and slightly bowed humeri with prominent deltoid tuberosities, short and wide ribs and clavicles, dorso‐lumbar scoliosis, biconcave vertebral bodies of the thoraco‐lumbar spine, and narrowed lumbar canal. In addition, in the girl there were amelogenesis imperfecta of the hypomaturation type, and the radiographs showed short distal ulnae, sloping epiphyses of the radii, short femoral necks, and slightly flat uncovered femoral heads. The children's parents are first cousins. Differential diagnoses are discussed and the possibility of a newly recognized oculo‐skeletal syndrome is raised. © 2006 Wiley‐Liss, Inc.
Osteocraniostenosis–hypomineralized skull with gracile long bones and splenic hypoplasia. Four new cases with distinctive chondro‐osseous morphology
Authors: Elliott Alison M. , Wilcox William R. , Spear Gerald S. , Field Fiona M. , Steffensen Thora S. , Friedman Barbara D. , Rimoin David L. , Lachman Ralph S. 
Published: June 2006

AbstractOsteocraniostenosis is a severe skeletal dysplasia characterized by a hypomineralized skull that has been previously described as kleeblattschädel (cloverleaf skull) and overtubulated long bones. Dysmorphic facial features include a short nose, short philtrum, and a small, inverted V‐shaped mouth. Splenic a/hypoplasia is a constant finding. We report four infants (two unrelated and two siblings) with osteocraniostenosis and describe the clinical, radiographic and chondro‐osseous morphology findings. The two siblings lack the moderate long‐bone shortening that is typically seen. The skull configuration is likely caused by severely hypoplastic cranial bones (parietal) rather than true craniosynostosis, making the term “osteocraniostenosis” misleading. Histological examination of bone in all cases showed an abnormal growth plate with short irregular columns. The resting cartilage showed pleomorphic chondrocytes with increased cellularity and unique pseudocolumn formation. There are some radiographic and chondro‐osseous morphologic similarities between osteocraniostenosis and severe Hallermann–Streiff syndrome (HSS), suggesting the two disorders may be pathogenetically related. © 2006 Wiley‐Liss, Inc.
Formtreue Makrocyclen: Strukturen und Synthesen aus Arylen‐ und Ethinylen‐Bausteinen
Authors: Zhang Wei , Moore Jeffrey S. 
Published: June 2006

AbstractFormtreue Arylenethinylen‐Makrocyclen mit ihren besonderen Strukturen und Eigenschaften werden für mögliche Anwendungen in der supramolekularen Chemie und den Materialwissenschaften intensiv erforscht. In diesem Aufsatz betrachten wir neuere Beispiele für die Synthese von Makrocyclen durch Kreuzkupplungen (Sonogashira: Arylacetylen‐Makrocyclen, Glaser: Aryldiacetylen‐Makrocyclen) und Methoden der dynamischen kovalenten Chemie. Ein Nachteil der Kupplungsmethoden ist die kinetisch kontrollierte Produktverteilung, da ein erheblicher Anteil der Oligomere über die erwünschte Cyclengröße hinauswächst (“Overshooting”). Bessere Ergebnisse wurden in jüngster Zeit mithilfe der dynamischen kovalenten Strategien auf der Grundlage reversibler Metathesereaktionen erzielt, die formtreue Makrocyclen in nur einem Reaktionsschritt liefern. Mechanistische Untersuchungen belegen, dass diese Form der Makrocyclenbildung ein thermodynamisch kontrollierter Prozess ist. Verbleibende präparative Probleme betreffen die Synthese von spezifisch funktionalisierten Strukturen sowie größeren und komplexeren zweidimensionalen und dreidimensionalen Molekülen.
Shape‐Persistent Macrocycles: Structures and Synthetic Approaches from Arylene and Ethynylene Building Blocks
Authors: Zhang Wei , Moore Jeffrey S. 
Published: June 2006

AbstractShape‐persistent arylene ethynylene macrocycles have attracted much attention in supramolecular chemistry and materials science because of their unique structures and novel properties. In this Review we describe recent examples of macrocycle synthesis by cross‐coupling (Sonogashira: aryl acetylene macrocycle or Glaser: aryl diacetylene macrocycle) and dynamic covalent chemistry. The primary disadvantage of the coupling methods is the kinetically determined product distribution, since a significant portion of oligomers grow beyond the length of the cyclic targets (“overshooting”). Better results have been obtained recently by a dynamic covalent approach involving reversible metathesis reactions that afford macrocycles in one step. Mechanistic studies demonstrate that macrocycle formation is thermodynamically controlled by this route. Remaining synthetic challenges include the efficient preparation of site‐specifically functionalized structures and larger, more complex two‐ and three‐dimensional molecules.
The aminergic control of cockroach salivary glands
Authors: Walz Bernd , Baumann Otto , Krach Christian , Baumann Arnd , Blenau Wolfgang 
Published: June 2006

AbstractThe acinar salivary glands of cockroaches receive a dual innervation from the subesophageal ganglion and the stomatogastric nervous system. Acinar cells are surrounded by a plexus of dopaminergic and serotonergic varicose fibers. In addition, serotonergic terminals lie deep in the extracellular spaces between acinar cells. Excitation‐secretion coupling in cockroach salivary glands is stimulated by both dopamine and serotonin. These monoamines cause increases in the intracellular concentrations of cAMP and Ca2+. Stimulation of the glands by serotonin results in the production of a protein‐rich saliva, whereas stimulation by dopamine results in saliva that is protein‐free. Thus, two elementary secretory processes, namely electrolyte/water secretion and protein secretion, are triggered by different aminergic transmitters. Because of its simplicity and experimental accessibility, cockroach salivary glands have been used extensively as a model system to study the cellular actions of biogenic amines and to examine the pharmacological properties of biogenic amine receptors. In this review, we summarize current knowledge concerning the aminergic control of cockroach salivary glands and discuss our efforts to characterize Periplaneta biogenic amine receptors molecularly. Arch Insect Biochem Physiol 62:141–152, 2006. © 2006 Wiley‐Liss, Inc.
PKC‐mediated USP phosphorylation is required for 20E‐induced gene expression in the salivary glands of Drosophila melanogaster
Authors: Sun Xiaoping , Song Qisheng 
Published: June 2006

AbstractEcdysone receptor (EcR) and its heterodimer, ultraspiracle protein (USP), are ligand‐dependent transcriptional factors that mediate the action of molting hormone 20‐hydroxyecdysone. The activities of transcriptional factors are subjected to regulation not only by transcriptional/translational mechanisms, but also by posttranslational mechanisms such as phosphorylation. Protein kinase consensus recognition sequence analysis of Drosophila EcR and USP reveals multiple phosphorylation sites for protein kinase C (PKC) and casein kinase II (CKII) on EcR and USP sequence. By using specific protein kinase inhibitors, we have shown that PKC, not CKII, is responsible for USP phosphorylation. Inhibition of PKC activity by protein kinase inhibitors blocked USP phosphorylation, resulting in inhibition of 20E‐induced gene expression at both transcriptional and translational levels. The composite data suggest that PKC‐mediated USP phosphorylation is required for 20E‐induced gene expression in the salivary glands of Drosophila melanogaster. Arch Insect Biochem Physiol 62:116–127, 2006. © 2006 Wiley‐Liss, Inc.
A geophysical approach to buried site detection using down‐hole susceptibility and soil magnetic techniques
Authors: Dalan Rinita A. 
Published: June 2006

AbstractRecently developed down‐hole magnetic capabilities and laboratory soil magnetic techniques are combined in a geophysical approach to identify buried archaeological sites. Down‐hole magnetic susceptibility measurements are used to locate palaeosols and soil magnetic studies are applied to evaluate whether an associated human occupation is likely. This geophysical approach has the potential to locate and explore buried archaeological deposits across varied environments in a cost‐effective, efficient, and relatively non‐invasive manner.This approach was developed and tested on sites in Minnesota and North Dakota, USA. Prototype Bartington Instruments MS2H down‐hole sensors allowed identification of major stratigraphical units, buried occupation layers and non‐cultural palaeosols at these sites. Buried soils correlated with enhanced susceptibilities.Buried cultural soils produced, in some cases, a signal over twice the magnitude of non‐archaeological palaeosols. Although this magnitude shift suggested a possible avenue for determining if ancient land surfaces had been occupied by humans, laboratory soil magnetic techniques, which allow an understanding of changes in composition, concentration and/or grain size of the magnetic carrier that produce this contrast in magnitude, were found to be much more effective in making this determination. These soil magnetic tests were applied to sediments collected from the down‐hole locations.An increase in magnetic remanence carried in the coarse soil fraction was found to consistently and reliably distinguish those soils associated with human occupation. Investigations into the origin of the magnetic signature have suggested this remanence resides in nodules of burnt clay found in the archaeological soils. As it is suspected that domestic fires produced these clay aggregations, this phenomenon may extend to a wide range of sites and thus provide a technique for buried site identification that is broadly applicable. Copyright © 2006 John Wiley & Sons, Ltd.
Flexible Designs by Adaptive Plans of Generalized Pocock‐ and O'Brien‐Fleming‐Type and by Self‐Designing Clinical Trials
Authors: Hartung Joachim 
Published: June 2006

AbstractFlexible designs are provided by adaptive planning of sample sizes as well as by introducing the weighted inverse normal combining method and the generalized inverse chi‐square combining method in the context of conducting trials consecutively step by step. These general combining methods allow quite different weighting of sequential study parts, also in a completely adaptive way, based on full information from unblinded data in previously performed stages. So, in reviewing some basic developments of flexible designing, we consider a generalizing approach to group sequentially performed clinical trials of Pocock‐type, of O'Brien‐Fleming‐type, and of Self‐designing‐type. A clinical trial may be originally planned either to show non‐inferiority or superiority. The proposed flexible designs, however, allow in each interim analysis to change the planning from showing non‐inferiority to showing superiority and vice versa.Several examples of clinical trials with normal and binary outcomes are worked out in detail. We demonstrate the practicable performance of the discussed approaches, confirmed in an extensive simulation study.Our flexible designing is a useful tool, provided that a priori information about parameters involved in the trial is not available or subject to uncertainty. (© 2006 WILEY‐VCH Verlag GmbH & Co. KGaA, Weinheim)
A Regulatory View on Adaptive/Flexible Clinical Trial Design
Authors: Hung H. M. James , O'Neill Robert T. , Wang Sue‐Jane , Lawrence John 
Published: June 2006

AbstractRecently there is growing interest in use of adaptive or flexible designs for development of pharmaceutical products. Statistical methodology has been greatly advanced in the literature. However, there are still some important issues with the methodology and application. In addition, there are many other challenges with these designs, including efficiency of these designs in the entire development program, trial conduct and logistics, the infrastructure of an adaptive trial, the regulatory evaluation of trial results and trial conduct, etc. Up till now, regulatory experience in these designs is very limited. We share some of the challenges. (© 2006 WILEY‐VCH Verlag GmbH & Co. KGaA, Weinheim)
Systemic inflammatory response syndrome
Authors: Davies M G , Hagen P-O 
Published: June 2006

Abstract

Background
Localized inflammation is a physiological protective response which is generally tightly controlled by the body at the site of injury. Loss of this local control or an overly activated response results in an exaggerated systemic response which is clinically identified as systemic inflammatory response syndrome (SIRS). Compensatory mechanisms are initiated in concert with SIRS and outcome (resolution, multiple organ dysfunction syndrome or death) is dependent on the balance of SIRS and such compensatory mechanisms. No directed therapies have been successful to date in influencing outcome.

Method
This review examines the current spectrum and pathophysiology of SIRS.

Results and conclusion
Further clinical and basic scientific research is required to develop the global picture of SIRS, its associated family of syndromes and their natural histories.
Delusion in general and forensic psychiatry—historical and contemporary aspects
Authors: Hoff Paul 
Published: June 2006

AbstractDelusion has always been a central topic for psychiatric research with regard to etiology and pathogenesis and to diagnosis, treatment, and forensic relevance. Throughout the history of psychiatry as a scientific discipline, there has been dissent on the issue of whether chronic delusion is a nosological entity of its own or just a specific type of another mental disorder, e.g. schizophrenia, mania, or personality disorder, and there already is a considerable literature on this. This article seeks to elucidate the central lines of thought that have governed the scientific debate on delusions and delusion‐associated phenomena since the early 19th century. Special attention is given to the practical relevance of these theoretical considerations for forensic questions and psychiatric research. Due to the complex features of delusions, research in this area may well become paradigmatic for many other complicated psycho(patho)logical phenomena, e.g. consciousness, hallucinations and psychotic depression. Copyright © 2006 John Wiley & Sons, Ltd.
Psychological processes and paranoia: implications for forensic behavioural science
Authors: Bentall Richard P. , Taylor Jayne L. 
Published: June 2006

AbstractParanoid delusions have recently become the focus of empirical research. In this article, we review studies of the psychological mechanisms that might be involved in paranoid thinking and discuss their implications for forensic behaviour science. Paranoia has not been consistently associated with any specific neuropsychological abnormality. However, evidence supports three broad types of mechanism that might be involved in delusional thinking in general and paranoia in particular: anomalous perceptual experiences, abnormal reasoning, and motivational factors. There is some evidence that paranoia may be associated with hearing loss, and good evidence that paranoid patients attend excessively to threatening information. Although general reasoning ability seems to be unaffected, there is strong evidence that a jumping‐ to‐conclusions style of reasoning about data is implicated in delusions in general, but less consistent evidence specifically linking paranoia to impaired theory of mind. Finally, there appears to be a strong association between paranoia and negative self‐esteem, and some evidence that attempts to protect self‐esteem by attributing negative events to external causes are implicated. Some of these processes have recently been implicated in violent behaviour, and they therefore have the potential to explain the apparent association between paranoid delusions and offending. Copyright © 2006 John Wiley & Sons, Ltd.
Unexpected Aggregation of Neutral, Xylene‐Cored Dinuclear GdIII Chelates in Aqueous Solution
Authors: Costa Jérôme , Balogh Edina , Turcry Véronique , Tripier Raphaël , Le Baccon Michel , Chuburu Françoise , Handel Henri , Helm Lothar , Tóth Éva , Merbach André E. 
Published: June 2006

AbstractWe have synthesized ditopic ligands L1, L2, and L3 that contain two DO3A3− metal‐chelating units with a xylene core as a noncoordinating linker (DO3A3− = 1,4,7,10‐tetraazacyclododecane‐1,4,7‐triacetate; L1 = 1,4‐bis{[4,7,10‐tris(carboxymethyl)‐1,4,7,10‐tetraazacyclododecane‐1‐yl]methyl}benzene; L2 = 1,3‐bis{[4,7,10‐tris(carboxymethyl)‐1,4,7,10‐tetraazacyclododecane‐1‐yl]methyl}benzene; L3 = 3,5‐bis{[4,7,10‐tris(carboxymethyl)‐1,4,7,10‐tetraazacyclododecane‐1‐yl]methyl}benzoic acid). Aqueous solutions of the dinuclear GdIII complexes formed with the three ligands have been investigated in a variable‐temperature, multiple‐field 17O NMR and 1H relaxivity study. The 17O longitudinal relaxation rates measured for the [Gd2L1–3(H2O)2] complexes show strong field dependence (2.35–9.4 T), which unambiguously proves the presence of slowly tumbling entities in solution. The proton relaxivities of the complexes, which are unexpectedly high for their molecular weight, and in particular the relaxivity peaks observed at 40–50 MHz also constitute experimental evidences of slow rotational motion. This was explained in terms of self‐aggregation related to hydrophobic interactions, π stacking between the aromatic linkers, or possible hydrogen bonding between the chelates. The longitudinal 17O relaxation rates of the [Gd2L1–3(H2O)2] complexes have been analysed with the Lipari–Szabo approach, leading to local rotational correlation times ${\tau {{298\hfill \atop {\rm l}\hfill}}}$ of 150–250 ps and global rotational correlation times ${\tau {{298\hfill \atop {\rm g}\hfill}}}$ of 1.6–3.4 ns (cGd: 20–50 mM), where ${\tau {{298\hfill \atop {\rm l}\hfill}}}$ is attributed to local motions of the Gd segments, while ${\tau {{298\hfill \atop {\rm g}\hfill}}}$ describes the overall motion of the aggregates. The aggregates can be partially disrupted by phosphate addition; however, at high concentrations phosphate interferes in the first coordination sphere by replacing the coordinated water. In contrast to the parent [Gd(DO3A)(H2O)1.9], which presents a hydration equilibrium between mono‐ and dihydrated species, a hydration number of q = 1 was established for the [Ln2L1–3(H2O)2] chelates by 17O chemical shift measurements on Ln = Gd and UV/Vis spectrophotometry for Ln = Eu. The exchange rate of the coordinated water is higher for [Gd2L1–3(H2O)2] complexes (${k{{298\hfill \atop {\rm ex}\hfill}}}$ = 7.5–12.0×106 s−1) than for [Gd(DOTA)(H2O)]−. The proton relaxivity of the [Gd2L1–3(H2O)2] complexes strongly decreases with increasing pH. This is related to the deprotonation of the inner‐sphere water, which has also been characterized by pH potentiometry. The protonation constants determined for this process are logKOH = 9.50 and 10.37 for [Gd2L1(H2O)2] and [Gd2L3(H2O)2], respectively.
Synthesis and Properties of the First Möbius Annulenes
Authors: Ajami Dariush , Hess Kirsten , Köhler Felix , Näther Christian , Oeckler Oliver , Simon Arndt , Yamamoto Chiyo , Okamoto Yoshio , Herges Rainer 
Published: June 2006

AbstractHeilbronner in 1964 predicted that annulenes with “…︁ a planar perimeter of N=4r AO's, which would yield an open shell configuration when occupied by 4r electrons, can be twisted into a closed shell Möbius strip perimeter without loss in π electron energy”. We have been able to synthesize the first [4n]annulene with such a Möbius topology and now present further Möbius isomers and the details of their preparation as stable compounds. To address the question whether the twist in the π system has an effect on the properties we systematically investigate energy, geometry and magnetic parameters of a large number isomers of [16]annulenes. The Möbius twisted annulenes are consistently more aromatic than the non‐twisted isomers. This is true for the parent as well as our benzoannelated systems. Our results are in contrast to those published recently by C. Castro, W. L. Karney, P. von R. Schleyer et al.
The reliability of fine‐needle aspiration biopsy as the initial diagnostic procedure for palpable masses
Authors: Florentine Barbara D. , Staymates Barry , Rabadi Munif , Barstis John , Black Alexander ,   Cancer Committee of the Henry Mayo Newhall Memorial Hospital
Published: June 2006

AbstractBACKGROUNDFine‐needle aspiration biopsy (FNAB) is a reliable, rapid, minimally invasive alternative to surgical biopsy when it is performed by physician specialists for the diagnosis of palpable masses. FNAB may be under‐utilized in community hospitals in the U.S. because physicians without specialty training commonly provide the service, resulting in less reliable results.METHODSRecords were reviewed retrospectively from 730 consecutive FNAB cases that were performed and interpreted by expert cytopathologists practicing in an outpatient community hospital setting between 2000 and 2004. Data concerning patient demographics, referring physician specialty type, body sites, diagnoses, specimen adequacy, accuracy of diagnosis, and follow‐up were examined and analyzed.RESULTSFNAB was diagnostic in 93% of patients and was 95% accurate. There were 5 false‐negative results and no false‐positive results for the diagnosis of malignancy. The overall sensitivity of FNAB was 93%, and the specificity was 100%. Using either histology or clinical follow‐up, the positive predictive value was 100%, and the negative predictive value was 99%.CONCLUSIONSHighly reliable results can be obtained when patients are referred to specialty‐trained cytopathologists practicing in the community for FNAB of palpable mass lesions. Clinicians are encouraged to seek out and support specialized FNAB services in their own communities. Cancer 2006. © 2006 American Cancer Society.
Parallel four‐dimensional Haralick texture analysis for disk‐resident image datasets
Authors: Woods Brent , Clymer Bradley , Heverhagen Johannes , Knopp Michael , Saltz Joel , Kurc Tahsin 
Published: June 2006

AbstractTexture analysis is one possible method of detecting features in biomedical images. During texture analysis, texture‐related information is found by examining local variations in image brightness. Four‐dimensional (4D) Haralick texture analysis is a method that extracts local variations along space and time dimensions and represents them as a collection of 14 statistical parameters. However, application of the 4D Haralick method on large time‐dependent image datasets is hindered by data retrieval, computation, and memory requirements. This paper describes a parallel implementation using a distributed component‐based framework of 4D Haralick texture analysis on PC clusters. The experimental performance results show that good performance can be achieved for this application via combined use of task‐ and data‐parallelism. In addition, we show that our 4D texture analysis implementation can be used to classify imaged tissues. Copyright © 2006 John Wiley & Sons, Ltd.
Grid benchmarking: vision, challenges, and current status
Authors: Dikaiakos Marios D. 
Published: June 2006

AbstractGrid benchmarking is an important and challenging topic of Grid computing research. In this paper, we present an overview of the key challenges that need to be addressed for the integration of benchmarking practices, techniques, and tools in emerging Grid computing infrastructures. We discuss the problems of performance representation, measurement, and interpretation in the context of Grid benchmarking, and propose the use of ontologies for organizing and describing benchmarking metrics. Finally, we present a survey of ongoing research efforts that develop benchmarks and benchmarking tools for the Grid. Copyright © 2006 John Wiley & Sons, Ltd.