965 resultados para Pseudo-pelger-huet Anomaly


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The Atlas Mountains in Morocco are considered as type examples of intracontinental chains, with high topography that contrasts with moderate crustal shortening and thickening. Whereas recent geological studies and geodynamic modeling have suggested the existence of dynamic topography to explain this apparent contradiction, there is a lack of modern geophysical data at the crustal scale to corroborate this hypothesis. Newly-acquired magnetotelluric data image the electrical resistivity distribution of the crust from the Middle Atlas to the Anti-Atlas, crossing the tabular Moulouya Plain and the High Atlas. All the units show different and unique electrical signatures throughout the crust reflecting the tectonic history of development of each one. In the upper crust electrical resistivity values may be associated to sediment sequences in the Moulouya and Anti-Atlas and to crustal scale fault systems in the High Atlas developed during the Cenozoic times. In the lower crust the low resistivity anomaly found below the Mouluya plain, together with other geophysical (low velocity anomaly, lack of earthquakes and minimum Bouguer anomaly) and geochemical (Neogene-Quaternary intraplate alkaline volcanic fields) evidence, infer the existence of a small degree of partial melt at the base of the lower crust. The low resistivity anomaly found below the Anti-Atlas may be associated with a relict subduction of Precambrian oceanic sediments, or to precipitated minerals during the release of fluids from the mantle during the accretion of the Anti-Atlas to the West African Supercontinent during the Panafrican orogeny ca. 685 Ma).

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OBJECTIVE: To review the surgical outcomes of partial cricotracheal resection in children with severe congenital subglottic stenosis and define the effect of concomitant anomalies or syndromes affecting outcome. METHODS: Forty-one children with subglottic stenosis of congenital and mixed (acquired on congenital) etiologies who underwent partial cricotracheal resection were identified from a prospectively collected database. Children with congenital subglottic stenosis and concomitant anomalies/syndromes were compared to children with congenital subglottic stenosis with no syndromes or concomitant anomalies. Operation-specific decannulation rates and complication rates were the primary outcome measures. We performed a two-sample test of proportion using the STATA-10 software for categorical variables to detect differences in proportions. Significance was set at p value<0.05. RESULTS: Twenty-seven (66%) of 41 children had concomitant anomalies/syndromes and 14 (34%) had congenital subglottic stenosis without concomitant anomalies/syndromes. Four patients needed revision surgery in the concomitant anomaly group and two patients needed revision surgery in the non concomitant anomaly group before achieving decannulation. The operation-specific decannulation rate in the concomitant anomaly group was 85% and 86% in the non anomaly group. When compared to children without concomitant anomaly, children with concomitant anomalies were more likely to have delayed decannulation following partial cricotracheal resection. However, this difference was not found to be statistically significant. The complication and operation-specific decannulation rates after partial cricotracheal resection were comparable to children without concomitant anomalies. Mortality rate was 11% (three of 27 patients) in the group with associated congenital anomalies or syndromes. Two patients succumbed to the primary pathology and one patient died due to tracheostomy-tube obstruction. There was no post-operative death in the non anomaly group. CONCLUSION: Partial cricotracheal resection can be done safely and effectively in children with concomitant anomalies/syndromes to achieve decannulation. The post-operative course may be prolonged but the decannulation and the complication rates are comparable to those children with congenital subglottic stenosis without concomitant anomalies.

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In dieser Thèse "Studien zu einer Poetik des Engels" wird anhand verschiedener Texte aus verschiedenen Epochen, jedoch mit Akzent auf der zweiten Hàlfte des 20. Jahrhunderts, dem Erscheinen von Engeln in literarischen Texten nachgegangen, mit besonderem Augenmerk auf der poetologischen Dimension, die mit der Darstellung von Engelfiguren zusammenhàngt, und mit besonderer Beriicksichtigung der Konsequenzen fur die Texte, in denen sie erscheinen.¦Literarische Texte bieten nàmlich einen geeigneten Immanenzort fur diese Art Epi-phanie an, eine Erscheinungsmoglichkeit fur Engel. Die Art und Weise, wie Dichterinnen und Dichter sie in ihren Texten erscheinen lassen, steht im Zentrum des Interesses dieser Studien. Wie verkorpern sich Engel in diesen Texten? Wie sprechen sie und wie kann mit ihnen und von ihnen gesprochen werden? Welche poetischen Funktionen ubernehmen diese Figuren, von denen die Dichter sprechen und die sie sprechen lassen? Welche poetischen Dynamiken entstehen in diesen Texten durch die Engelserscheinungen?¦Der Engel, der traditionell als Bote (gr. angelos), als Vermittler oder Obermittler gilt, hat im Medium Literatur (oder Text) selbst auch eine mediale Funktion inne, sodass Engel und Literatur einander widerspiegeln und Engeltexte zu Orten werden, an denen eine sprachliche und poetologische Auseinandersetzung stattfinden kann, die sowohl der Lite¬ratur (und auch etwa dem Erzàhler) als auch dem Engel inhàrent ist und es mit dem Ver- mittlungs(un)vermogen zu tun hat.¦In diesen Studien geht es darum, die produktive und dynamische Leistung des Engels (oder der Engel) im Schreib- und im Leseprozess zu erfahren und nachzuzeichnen. Durch ihre Pràsenz im Text bringen Engel einen Perspektivenwechsel ins Spiel, der die Auseinandersetzung mit dem Text pràgt und den Text selbst beeinflusst, sodass die Engel diese Texte zur Schreib- und Leseerfahrung werden lassen.¦Engelsdichtungen erweisen sich als durch und durch relational, sie involvieren den Dichter sowie auch den Leser, der ihnen - lesend - begegnet, und beeinflussen den ganzen Text und seine Dynamik, da diese Figuren unmoglich von ihm abstrahiert werden konnen. Engel erweisen sich auch als àusserst subversiv, wenn sie dem nicht entsprechen, was man von ihnen erwartet, wenn sie sich den geltenden (sozialen, politischen, aber auch - im Medium Literatur - sprachlichen oder dichterischen) Regeln widersetzen.¦In einem einleitenden Kapitel wird die Fragestellung skizziert und anhand vieler Textbeispiele verfeinert, sowie auch die Methode veranschaulicht. Darin werden Texte von Wallace Stevens (The Necessary Angel), Jean Paul (Der Tod eines Engels), Wim Wenders und Peter Handke (Himmel über Berlin) betrachtet, sowie auch etwa von Goethe, Morike, Andersen, Tolstoi (Der Engel Gottes oder Wovon die Menschen leben), Pseudo- Strieker (Der König im Bade), Baudelaire.¦Die weiteren Kapitel sind als Fallstudien zu verstehen und setzen sich mit einzelnen Texten auseinander, die von verschiedenen Autoren und zu verschiedenen Zeiten geschrieben wurden und sich von der Gattung und der Lange her stark unterscheiden:¦- "Zwischen Lichterscheinung und Holzfigur": Zu einem Tagebucheintrag von Franz Kafka (25. Juni 1914).¦- "Schweigende Boten": Zu Heinrich Bolls Roman Der Engel schwieg [1949-1951 niedergeschrieben, erst 1992 posthum erschienen).¦- "Ein subversiver Engel - wider Willen": Zu Fridrich Dürrenmatts Komödie Ein Engel kommt nach Babylon (1. Fassung 1954, 2. Fassung 1957, Neufassung 1980).¦- Zu drei Engel-Texten von Ilse Aichinger:¦- "Ein errungenes Weihnachten - im Spiel": Zum Romankapitel Das grosse Spiel (6. Kapitel aus dem Roman Die grössere Hoffnung, 1948),¦- "»Habt ihr den Engel gesehen?« - Wenn sich mit den Engeln auf einmal jeder Sinn entzieht": Zur Erzählung Engel in der Nacht (1949; aus dem Band Der Gefesselte),¦- "Ober Verwirrung hinweg - Zur subversiven Poetik eines Engels": Zur Erzâhlung Der Engel (1963; aus dem Band Eliza Eliza).¦Im Schlusskapitel, in dem vergleichende Beobachtungen zu diesen Texten und ihren Analysen, Hervorhebungen der Gemeinsamkeiten und entscheidende poetische Merkmale dieser Texte erwähnt werden, wird nicht das Ziel angestrebt, eine Synthese aus dieser Vielfältigkeit zu machen, die in den verschiedenen Kapiteln zum Vorschein kommt. Vielmehr wird gezeigt, wie fruchtbar die Auseinandersetzung mit Engeln in diesen Texten ist und wie diese Texte sich von ihrem 'Gegenstand' berühren lassen, je auf unterschiedliche Weise.

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La Unión Europea se encuentra actualmente inmersa en un proceso de cambio de su sistema de gobernanza económica. Este artículo analiza por qué los Estados miembros están siendo capaces de cooperar en temas que en los años 1990s eran tabú en la arena comunitaria. En base a una metodología cualitativa y un marco teórico nutrido por los enfoques neofuncionalista e intergubernamental, concluimos que los acuerdos sobre gobernanza económica a los que se está llegando en la actualidad responden más a la necesidad que a la voluntad de hacer avanzar la integración.

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[spa] La Unión Europea se encuentra actualmente inmersa en un proceso de cambio de su sistema de gobernanza económica. Este artículo analiza por qué los Estados miembros están siendo capaces de cooperar en temas que en los años 1990s eran tabú en la arena comunitaria. En base a una metodología cualitativa y un marco teórico nutrido por los enfoques neofuncionalista e intergubernamental, concluimos que los acuerdos sobre gobernanza económica a los que se está llegando en la actualidad responden más a la necesidad que a la voluntad de hacer avanzar la integración.

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BACKGROUND: The use of valproic acid in the first trimester of pregnancy is associated with an increased risk of spina bifida, but data on the risks of other congenital malformations are limited. METHODS: We first combined data from eight published cohort studies (1565 pregnancies in which the women were exposed to valproic acid, among which 118 major malformations were observed) and identified 14 malformations that were significantly more common among the offspring of women who had received valproic acid during the first trimester. We then assessed the associations between use of valproic acid during the first trimester and these 14 malformations by performing a case-control study with the use of the European Surveillance of Congenital Anomalies (EUROCAT) antiepileptic-study database, which is derived from population-based congenital-anomaly registries. Registrations (i.e., pregnancy outcomes with malformations included in EUROCAT) with any of these 14 malformations were compared with two control groups, one consisting of infants with malformations not previously linked to valproic acid use (control group 1), and one consisting of infants with chromosomal abnormalities (control group 2). The data set included 98,075 live births, stillbirths, or terminations with malformations among 3.8 million births in 14 European countries from 1995 through 2005. RESULTS: Exposure to valproic acid monotherapy was recorded for a total of 180 registrations, with 122 registrations in the case group, 45 in control group 1, and 13 in control group 2. As compared with no use of an antiepileptic drug during the first trimester (control group 1), use of valproic acid monotherapy was associated with significantly increased risks for 6 of the 14 malformations under consideration; the adjusted odds ratios were as follows: spina bifida, 12.7 (95% confidence interval [CI], 7.7 to 20.7); atrial septal defect, 2.5 (95% CI, 1.4 to 4.4); cleft palate, 5.2 (95% CI, 2.8 to 9.9); hypospadias, 4.8 (95% CI, 2.9 to 8.1); polydactyly, 2.2 (95% CI, 1.0 to 4.5); and craniosynostosis, 6.8 (95% CI, 1.8 to 18.8). Results for exposure to valproic acid were similar to results for exposure to other antiepileptic drugs. CONCLUSIONS: The use of valproic acid monotherapy in the first trimester was associated with significantly increased risks of several congenital malformations, as compared with no use of antiepileptic drugs or with use of other antiepileptic drugs.

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Oculo-auriculo-vertebral spectrum is a complex developmental disorder characterised mainly by anomalies of the ear, hemifacial microsomia, epibulbar dermoids and vertebral anomalies. The aetiology is largely unknown, and the epidemiological data are limited and inconsistent. We present the largest population-based epidemiological study to date, using data provided by the large network of congenital anomalies registries in Europe. The study population included infants diagnosed with oculo-auriculo-vertebral spectrum during the 1990-2009 period from 34 registries active in 16 European countries. Of the 355 infants diagnosed with oculo-auriculo-vertebral spectrum, there were 95.8% (340/355) live born, 0.8% (3/355) fetal deaths, 3.4% (12/355) terminations of pregnancy for fetal anomaly and 1.5% (5/340) neonatal deaths. In 18.9%, there was prenatal detection of anomaly/anomalies associated with oculo-auriculo-vertebral spectrum, 69.7% were diagnosed at birth, 3.9% in the first week of life and 6.1% within 1 year of life. Microtia (88.8%), hemifacial microsomia (49.0%) and ear tags (44.4%) were the most frequent anomalies, followed by atresia/stenosis of external auditory canal (25.1%), diverse vertebral (24.3%) and eye (24.3%) anomalies. There was a high rate (69.5%) of associated anomalies of other organs/systems. The most common were congenital heart defects present in 27.8% of patients. The prevalence of oculo-auriculo-vertebral spectrum, defined as microtia/ear anomalies and at least one major characteristic anomaly, was 3.8 per 100,000 births. Twinning, assisted reproductive techniques and maternal pre-pregnancy diabetes were confirmed as risk factors. The high rate of different associated anomalies points to the need of performing an early ultrasound screening in all infants born with this disorder.

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The objective of this work was to estimate the genetic diversity of improved banana diploids using data from quantitative analysis and from simple sequence repeats (SSR) marker, simultaneously. The experiment was carried out with 33 diploids, in an augmented block design with 30 regular treatments and three common ones. Eighteen agronomic characteristics and 20 SSR primers were used. The agronomic characteristics and the SSR were analyzed simultaneously by the Ward-MLM, cluster, and IML procedures. The Ward clustering method considered the combined matrix obtained by the Gower algorithm. The Ward-MLM procedure identified three ideal groups (G1, G2, and G3) based on pseudo-F and pseudo-t² statistics. The dendrogram showed relative similarity between the G1 genotypes, justified by genealogy. In G2, 'Calcutta 4' appears in 62% of the genealogies. Similar behavior was observed in G3, in which the 028003-01 diploid is the male parent of the 086079-10 and 042079-06 genotypes. The method with canonical variables had greater discriminatory power than Ward-MLM. Although reduced, the genetic variability available is sufficient to be used in the development of new hybrids.

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We analysed the relationship between changes in land cover patterns and the Eurasian otter occurrence over the course of about 20 years (1985-2006) using multi-temporal Species Distribution Models (SDMs). The study area includes five river catchments covering most of the otter's Italian range. Land cover and topographic data were used as proxies of the ecological requirements of the otter within a 300-m buffer around river courses. We used species presence, pseudo-absence data, and environmental predictors to build past (1985) and current (2006) SDMs by applying an ensemble procedure through the BIOMOD modelling package. The performance of each model was evaluated by measuring the area under the curve (AUC) of the receiver-operating characteristic (ROC). Multi-temporal analyses of species distribution and land cover maps were performed by comparing the maps produced for 1985 and 2006. The ensemble procedure provided a good overall modelling accuracy, revealing that elevation and slope affected the otter's distribution in the past; in contrast, land cover predictors, such as cultivations and forests, were more important in the present period. During the transition period, 20.5% of the area became suitable, with 76% of the new otter presence data being located in these newly available areas. The multi-temporal analysis suggested that the quality of otter habitat improved in the last 20 years owing to the expansion of forests and to the reduction of cultivated fields in riparian belts. The evidence presented here stresses the great potential of riverine habitat restoration and environmental management for the future expansion of the otter in Italy

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Manet security has a lot of open issues. Due to its character-istics, this kind of network needs preventive and corrective protection. Inthis paper, we focus on corrective protection proposing an anomaly IDSmodel for Manet. The design and development of the IDS are consideredin our 3 main stages: normal behavior construction, anomaly detectionand model update. A parametrical mixture model is used for behav-ior modeling from reference data. The associated Bayesian classi¯cationleads to the detection algorithm. MIB variables are used to provide IDSneeded information. Experiments of DoS and scanner attacks validatingthe model are presented as well.

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Une fois déposé, un sédiment est affecté au cours de son enfouissement par un ensemble de processus, regroupé sous le terme diagenèse, le transformant parfois légèrement ou bien suffisamment pour le rendre méconnaissable. Ces modifications ont des conséquences sur les propriétés pétrophysiques qui peuvent être positives ou négatives, c'est-à-dire les améliorer ou bien les détériorer. Une voie alternative de représentation numérique des processus, affranchie de l'utilisation des réactions physico-chimiques, a été adoptée et développée en mimant le déplacement du ou des fluides diagénétiques. Cette méthode s'appuie sur le principe d'un automate cellulaire et permet de simplifier les phénomènes sans sacrifier le résultat et permet de représenter les phénomènes diagénétiques à une échelle fine. Les paramètres sont essentiellement numériques ou mathématiques et nécessitent d'être mieux compris et renseignés à partir de données réelles issues d'études d'affleurements et du travail analytique effectué. La représentation des phénomènes de dolomitisation de faible profondeur suivie d'une phase de dédolomitisation a été dans un premier temps effectuée. Le secteur concerne une portion de la série carbonatée de l'Urgonien (Barrémien-Aptien), localisée dans le massif du Vercors en France. Ce travail a été réalisé à l'échelle de la section afin de reproduire les géométries complexes associées aux phénomènes diagénétiques et de respecter les proportions mesurées en dolomite. De plus, la dolomitisation a été simulée selon trois modèles d'écoulement. En effet, la dédolomitisation étant omniprésente, plusieurs hypothèses sur le mécanisme de dolomitisation ont été énoncées et testées. Plusieurs phases de dolomitisation per ascensum ont été également simulées sur des séries du Lias appartenant aux formations du groupe des Calcaire Gris, localisées au nord-est de l'Italie. Ces fluides diagénétiques empruntent le réseau de fracturation comme vecteur et affectent préférentiellement les lithologies les plus micritisées. Cette étude a permis de mettre en évidence la propagation des phénomènes à l'échelle de l'affleurement. - Once deposited, sediment is affected by diagenetic processes during their burial history. These diagenetic processes are able to affect the petrophysical properties of the sedimentary rocks and also improve as such their reservoir capacity. The modelling of diagenetic processes in carbonate reservoirs is still a challenge as far as neither stochastic nor physicochemical simulations can correctly reproduce the complexity of features and the reservoir heterogeneity generated by these processes. An alternative way to reach this objective deals with process-like methods, which simplify the algorithms while preserving all geological concepts in the modelling process. The aim of the methodology is to conceive a consistent and realistic 3D model of diagenetic overprints on initial facies resulting in petrophysical properties at a reservoir scale. The principle of the method used here is related to a lattice gas automata used to mimic diagenetic fluid flows and to reproduce the diagenetic effects through the evolution of mineralogical composition and petrophysical properties. This method developed in a research group is well adapted to handle dolomite reservoirs through the propagation of dolomitising fluids and has been applied on two case studies. The first study concerns a mid-Cretaceous rudist and granular platform of carbonate succession (Urgonian Fm., Les Gorges du Nan, Vercors, SE France), in which several main diagenetic stages have been identified. The modelling in 2D is focused on dolomitisation followed by a dédolomitisation stage. For the second study, data collected from outcrops on the Venetian platform (Lias, Mont Compomolon NE Italy), in which several diagenetic stages have been identified. The main one is related to per ascensum dolomitisation along fractures. In both examples, the evolution of the effects of the mimetic diagenetic fluid on mineralogical composition can be followed through space and numerical time and help to understand the heterogeneity in reservoir properties. Carbonates, dolomitisation, dédolomitisation, process-like modelling, lattice gas automata, random walk, memory effect.

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Complications related to the neck-stem junction of modular stems used for total hip arthroplasty (THA) are generating increasing concern. A 74-year-old male had increasing pain and a cutaneous reaction around the scar 1 year after THA with a modular neck-stem. Imaging revealed osteolysis of the calcar and a pseudo-tumour adjacent to the neck-stem junction. Serum cobalt levels were elevated. Revision surgery to exchange the stem and liner and to resect the pseudo-tumour was performed. Analysis of the stem by scanning electron microscopy and by energy dispersive X-ray and white light interferometry showed fretting corrosion at the neck-stem junction contrasting with minimal changes at the head-neck junction. Thus, despite dry assembly of the neck and stem on the back table at primary THA, full neck-stem contact was not achieved, and the resulting micromotion at the interface led to fretting corrosion. This case highlights the mechanism of fretting corrosion at the neck-stem interface responsible for adverse local tissue reactions. Clinical and radiological follow-up is mandatory in patients with dual-modular stems.

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Les anomalies vasculaires constituent des affections rares présentes dès la naissance. Elles sont classées selon leur histologie en tumeurs ou malformations vasculaires. La connaissance de ces anomalies par le médecin de famille est importante, permettant ainsi leur diagnostic, qui repose essentiellement sur la clinique. Les caractéristiques anatomiques et hémodynamiques de l'anomalie sont précisées par l'angiologue grâce à l'écho-Doppler. Cet examen permet un suivi de la lésion et le diagnostic d'éventuelles complications. Un bilan radiologique peut compléter cette évaluation avant discussion multidisciplinaire en vue d'un traitement parfois difficile. Dans cet article, un bref aperçu des malformations vasculaires et de leur prise en charge multidisciplinaire est discuté, en particulier dans le cas du syndrome de Klippel-Trenaunay. Vascular anomalies are rare conditions that could be observed at all ages. They are classified, according to their histology, in vascular tumors or vascular malformations. The general practitioner plays a significant role in diagnosis and patient management, diagnosis being suspected on clinical history. In case of vascular anomaly, ultrasound-Doppler assessment is helpful to characterize morphologic and hemodynamic changes of the lesion and permits to monitor the evolution and to detect complications. Further investigations are often necessary prior to multidisciplinary management. In this article, a brief overview of vascular anomalies, their multidisciplinary management and the exemple of Klippel-Trenaunay syndrome are presented.