bluuebunny/crossref_metadata_2025_split huggingface dataset:
Biventricular Hypertrophic Cardiomyopathy in a Child with LEOPARD Syndrome: a Case Report
Authors: Blesneac Cristina , Șuteu Carmen-Corina , Bănescu Claudia , Benedek Theodora , Benedek Imre , Togănel Rodica 
Published: March 2018

Abstract

Background: LEOPARD syndrome is a complex dysmorphogenetic disorder of inconstant penetrance and various morphologic expressions. The syndrome is an autosomal dominant disease that features multiple lentigines, electrocardiographic changes, eye hypertelorism, pulmonary valve stenosis or hypertrophic cardiomyopathy, genital malformations, and a delayed constitutional growth hearing loss, which can be associated with rapidly progressive severe biventricular obstructive hypertrophic cardiomyopathy. No epidemiologic data are available on the real incidence of LEOPARD syndrome; however, this seems to be a rare disease, being often underdiagnosed, as many of its features are mild.

Case presentation: We report the case of a 10-year-old female pediatric patient, diagnosed with obstructive hypertrophic cardiomyopathy at the age of 3 months, and recently diagnosed with LEOPARD syndrome. The patient first presented for a cardiologic examination at the age of 3 months, due to a murmur. She presented failure to thrive and psychomotor retardation, and was diagnosed with biventricular obstructive hypertrophic cardiomyopathy for which she had received high-dose beta-blocker therapy. At the age of 7 years she underwent a biventricular myectomy for relief of outflow tract obstruction, completed with another myectomy after 2 years due to progressive increase of pressure gradient in the left ventricular outflow tract. Prior to the second surgical intervention, multiple lentigines appeared on her skin, and genetic testing revealed the presence of LEOPARD syndrome.

Conclusion: LEOPARD syndrome is a rare disease, which can be very difficult to diagnose, especially based on features other than lentigines. Cardiac involvement in LEOPARD syndrome can be progressive and requires multiple medical and surgical interventions.
First results of the Nordic and Baltic GNSS Analysis Centre
Authors: Lahtinen Sonja , Pasi Häkli , Jivall Lotti , Kempe Christina , Kollo Karin , Kosenko Ksenija , Pihlak Priit , Prizginiene Dalia , Tangen Oddvar , Weber Mette , Paršeliūnas Eimuntas , Baniulis Rimvydas , Galinauskas Karolis 
Published: March 2018

Abstract
The Nordic Geodetic Commission (NKG) has launched a joint NKG GNSS Analysis Centre that aims to routinely produce high qualityGNSS solutions for the common needs of the NKG and the Nordic and Baltic countries. A consistent and densified velocity field is needed for the constraining of the gla-cial isostatic adjustment (GIA) modelling that is a key component of maintaining the national reference frame realisations in the area. We described the methods of the NKG GNSS Analysis Centre including the defined processing setup for the local analysis centres (LAC) and for the combination centres.We analysed the results of the first 2.5 years (2014.5-2016). The results showed that different subnets were consistent with the combined solution within 1-2 mm level. We observed the so called network effect affecting our reference frame alignment. However, the accuracy of the reference frame alignment was on a few millimetre level in the area of the main interest (Nordic and Baltic Countries). TheNKGGNSS AC was declared fully operational in April 2017.
On the security of the Courtois-Finiasz-Sendrier signature
Authors: Morozov Kirill , Roy Partha Sarathi , Steinwandt Rainer , Xu Rui 
Published: March 2018

Abstract
We prove that a variant of the Courtois-Finiasz-Sendrier signature is strongly existentially unforgeable under chosen message attack in the random oracle model, assuming hardness of the Permuted Goppa Syndrome Decoding Problem (also known as the Niederreiter problem). In addition, we explicitly show that security against key substitution attacks can be arranged by a standard technique of Menezes and Smart, hashing the public key.
On the structure vector field of a real hypersurface in complex quadric
Authors: Dios Pérez Juan de 
Published: March 2018

Abstract
From the notion of Jacobi type vector fields for a real hypersurface in complex quadric Qm
we prove that if the structure vector field is of Jacobi type it is Killing when the real hypersurface is either Hopf or compact. In such cases we classify real hypersurfaces whose structure vector field is of Jacobi type.
Effect of pulsed magnetron sputtering process for the deposition of thin layers of nickel and nickel oxide
Authors: Posadowski Witold , Wiatrowski Artur , Kapka Grzegorz 
Published: March 2018

Abstract
Magnetron sputtered nickel and nickel oxide films have been studied for various applications. We may find, among others, these films in electrochromic display devices, in resistive type gas sensors, as metal electrodes in electronic devices, in solar thermal absorbers. Pure nickel films deposited using PVD technique possess good corrosion and wear resistant properties. Magnetron sputtering has several advantages in film deposition (in comparison to other methods) such as relatively low heating temperature of the deposited substrate during sputtering process, high energy of sputtered atoms (about 10 eV) at the substrate, which influences positively the films adhesion. From application point of view, the most valuable feature of these films is the possibility of scaling target dimensions, which makes feasible the deposition on a several square meter surfaces. The improvement of magnetron sputtering devices design may influence positively the optimization of the deposition technology and its efficiency. The thin nickel and nickel oxide films were prepared by pulsed magnetron sputtering using original type WMK magnetron device. Ni (99.9 %) has been used as a sputtering target of 100 mm in diameter and different thicknesses (3 mm, 5 mm, and 6 mm). The distance between the substrate and target was the same in all experiments and equal to 120 mm. Argon and oxygen gases were introduced during the reactive process through needle gas valves at a total pressure of 0.4 Pa. The sputtering power, sputtering pressure and oxygen partial pressure have been used as technological knobs for deposition processes. The helpful tool for controlling the pulsed magnetron sputtering process was the original parameter of supply (so called circulating power). Results from our experiments showed that the deposition of Ni films is possible even from targets of 6 mm thickness. Deposition rate increased proportionally with the sputtering power. The aim of this work is to use the acquired expertise to develop an efficient technology of thin nickel oxide layers for electrochromic systems.
Thin SixNyCz films deposited from hexamethyldisilazane by RF PECVD technique for optical filter applications
Authors: Oleśko Katarzyna , Szymanowski Hieronim , Gazicki-Lipman Maciej , Balcerzak Jacek , Ski Witold Szymań , Pawlak Wojciech , Sobczyk-Guzenda Anna 
Published: March 2018

Abstract
This work initiates a series of reports aimed at a construction of rugate optical filters based on silicon rich materials of alternating gradients of refractive index n with the help of plasma enhanced chemical vapor deposition (PECVD) technique, The idea is to start deposition of high refractive index SixNy type of material using hexamethyldisilazane (HMDSN) vapor and nitrogen rich atmosphere, and then to gradually replace nitrogen with oxygen in that atmosphere in order to lower n down to a minimum characteristic of SixOy type of material. A return to initial gas composition should increase the index back to its maximum. In the present work, thin SixNyCz films were synthesized from a mixture of HMDSN vapor with gaseous NH3 and N2. The effect of NH3/N2 ratio on the coating morphology, its elemental composition, chemical bonding and optical properties was studied using scanning electron microscopy, X-ray photoelectron spectroscopy, Fourier transform infrared spectroscopy, ultra-violet absorption spectroscopy and variable angle spectroscopic ellipsometry. The results show that films of the highest index of refraction and the lowest extinction coefficient have been deposited from the gas mixture containing 90 % of ammonia. These coatings are also characterized by the lowest carbon and the highest nitrogen contents.
Das Verbot der deutschen Luftfahrtindustrie und die Erfindung ihrer Geschichte, 1945 bis 1953
Authors: Budrass Lutz 
Published: March 2018

Abstract:

The ban on German aviation and the creation/fabrication of its history The article traces the origins of two central features of the historiography of the German aircraft industry: while its contribution to technical progress by the end of World War II tends to be grossly exaggerated – particularly in the case of the so-called Wunderwaffen, the jet fighters of Messerschmitt and Heinkel – its industrial basis und its role in the German war economy are played down to an impression that, until 1945, the industry consisted of tiny workshops of mere handicraft character. It is shown that this narrative was carefully constructed between 1945 and 1953, highlighted by memoirs of former military leaders of the Luftwaffe like Adolf Galland and Werner Baumbach, but predominantely through the memoirs of Ernst Heinkel, the leading industrialist during the Nazi period. These memioirs appeared at a time when it seemed unlikely – due to a total ban on German aviation under the Allied occupation – that a German aircraft industry would ever rise again. By exaggerating the German technological lead in 1945 they eased the idea that the industry was outdated when the chances grew for its return to the international market in 1953. Meanwhile, the outright denial of the size and importance of the industry during the war indirectly provided a chance to gloss over the participation of the industrialists in the Nazi crimes, highlighted by the use of concentration camp inmates and slave labourers who formed the bulk of the workforce in the production of the Wunderwaffen.
Inter-replicon Gene Flow Contributes to Transcriptional Integration in the Sinorhizobium meliloti Multipartite Genome
Authors: diCenzo George C , Wellappili Deelaka , Golding G Brian , Finan Turlough M 
Published: March 2018

Abstract
Integration of newly acquired genes into existing regulatory networks is necessary for successful horizontal gene transfer (HGT). Ten percent of bacterial species contain at least two DNA replicons over 300 kilobases in size, with the secondary replicons derived predominately through HGT. The Sinorhizobium meliloti genome is split between a 3.7 Mb chromosome, a 1.7 Mb chromid consisting largely of genes acquired through ancient HGT, and a 1.4 Mb megaplasmid consisting primarily of recently acquired genes. Here, RNA-sequencing is used to examine the transcriptional consequences of massive, synthetic genome reduction produced through the removal of the megaplasmid and/or the chromid. Removal of the pSymA megaplasmid influenced the transcription of only six genes. In contrast, removal of the chromid influenced expression of ∼8% of chromosomal genes and ∼4% of megaplasmid genes. This was mediated in part by the loss of the ETR DNA region whose presence on pSymB is due to a translocation from the chromosome. No obvious functional bias among the up-regulated genes was detected, although genes with putative homologs on the chromid were enriched. Down-regulated genes were enriched in motility and sensory transduction pathways. Four transcripts were examined further, and in each case the transcriptional change could be traced to loss of specific pSymB regions. In particularly, a chromosomal transporter was induced due to deletion of bdhA likely mediated through 3-hydroxybutyrate accumulation. These data provide new insights into the evolution of the multipartite bacterial genome, and more generally into the integration of horizontally acquired genes into the transcriptome.
Grafikatörténeti érdekességek manierista Krisztus-típusok fényében
Authors: Szilárdfy Zoltán 
Published: March 2018

St John of God is the patron saint of booksellers and bookbinders. An engraving by Joseph Anton Schmidt of Augsburg depicts him, still in civilian clothes, in a baroque printing office of the engraver’s time, around 1770. Johann Andreas Pfeffel jr. made an engraved portrait of his father with a German text of 8 lines under it. My collection has two engravings from around 1670 showing engraving workshops.
Hereafter I am going to list types of Christ. In Johann Andreas Pfeffel sr.’s composition the triumphant Saviour is standing on the instruments of Passion on top of Golgotha, with the flag of Easter in his right hand. His favourite disciple is holding to a rope lowered by the Heavenly Father, his feet treading on the column of the flagellation. The meaning of the allegorical picture is illumined by a quotation from St John’s Gospel (6,44). The Lord Triumphing over Death is reminiscent of a painting by Giovanni Battista Tinti: the blood flowing from Jesus’s heart is gathered by an angel in a cup. Christ’s foot is treading on a skull, he is holding his cross with the wreath of thorns. The mannerist painter of Parma drew inspiration from Michelangelo’s Risen Christ in Rome’s S. Maria sopra Minerva. In a book illustration Pfeffel depicts the blood and water from the side of the transfigured Saviour as the material of the Eucharist, adoring angels gathering it in a chalice and a pitcher.
In Buda’s Víziváros district, on the first side altar on the right in the former Franciscan church (later belonging to the sisters of St Elizabeth) a painted version of the votive statue of Vir Dolorum in Matrei in Tyrol, of which János Fülöp Binder made an engraving, was venerated.
Two monumental works by Michelangelo Buonarroti convey the mystery of Easter. The statue of Jesus in BasBassano Romano was made by Michelangelo earlier (1514- 16) and can thus be taken as precedent to the sculpture of a similar theme in S. Maria sopra Minerva (1521). The dominant attribute is the cross. In the earlier sculpture, in addition to the ropes, sponge and loincloth, the robe of mockery is dropped by Christ’s left hand onto the column of his flogging, which also serves as support.
There is a short red jasper column in the middle of a recess opening from the St Zeno chapel in Rome’s Basilica di S Prassede. Cardinal Giovanni Colonna, the commander of the papal army of the fifth crusade (1219) brought it home from the Holy Land and set it up in 1223. It is allegedly the column of Christ’s flagellation. The Greek emperor Alexios I Komnenos listed the relics kept in Constantinople in 1092: he already mentioned the purple robe and the reed. A register of 1200 includes the sponge, the purple chlamys and the reed in the sanctuary of Hagia Sophia. After the transfer of the relics to Rome, the reed with the sponge could be found in the reliquary of the Sancta Sanctorum in the Lateran. The Lord’s loincloth was preserved in the cathedral of Aachen visited by pilgrims for plenary indulgence as late as the 16th century.
Use of feracrylum in tonsillectomy
Authors: D. C. Sathyaki , Roy Mereen Susan , G. Vishnu Kumar , R. Jyothiswarup 
Published: March 2018

<p class="abstract"><strong>Background:</strong> Haemorrhage is the most common complication which is encountered in tonsillectomy. Various methods have been employed to minimise it. One such method has been employed in this study. The objective of the study was to study the efficacy of feracrylum in reducing post tonsillectomy haemorrhage with that of tranexemic acid.</p><p class="abstract"><strong>Methods:</strong> 40 patients who underwent tonsillectomy in a tertiary care centre were involved in this study. They were classified into two groups with 20 patients in each group. </p><p class="abstract"><strong>Results:</strong> Use of feracrylum reduced the haemorrhage significantly. Only one patient required ligation of vessels.</p><p class="abstract"><strong>Conclusions:</strong> Use of feracrylum reduces haemorrhage significantly as compared to that of intra venous tranexemic acid.</p><p class="abstract"> </p>
El rol de las humanidades en la formación de los trabajadores sociales
Authors: Arrese  Igor Héctor Óscar 
Published: March 2018

En este trabajo intento mostrar la importancia de las Humanidades para la formación de trabajadores sociales. En este contexto, considero las teorías de la justicia, a fin de determinar el rol de la Universidad en la construcción de una sociedad justa. Como consecuencia, planteo la importancia de promover las relaciones de reconocimiento entre los ciudadanos, desde el enfoque de Axel Honneth. A fin de evitar las relaciones de menosprecio, considero que es importante no tratar al otro como un mero objeto, sino más bien ponerse en su lugar. Por eso, considero relevante el enfoque de Martha Nussbaum, basado en el desarrollo de la empatía pormedio de la educación humanística y artística.Nussbaum sostiene que la experiencia estética nos permite ponernos en contacto con nuestro mundo emocional, para luego poder captar la interioridad del otro. Por otro lado, el estudio de la filosofía nos permite reconstruir la posición del otro y mirar el mundo desde el punto de vista del otro. En este contexto, considero la necesidad de desarrollar un modelo de desarrollo humano en la formación de los trabajadores sociales, orientado no sólo al entrenamiento técnico, sino también ampliado al desarrollo de la creatividad y el pensamiento crítico, que son fundamentales para el logro de una ciudadanía plena.
The Effect of Human Resources Competencies on a Firm Performance: A Marketing Perspective (With Reference to Firms Located in UAE)
Authors: Awad Ashraf 
Published: March 2018

Till date numerous theories in the field of marking has recognized both the need for improved marketing skills and the literature which provide in-depth analysis and details about such marketing skills and competencies for the help of marketing managers.For the current work, a questionnaire was served over the managers of 162 companies, constituting 52 percent of industrial firms in Abu Dhabi. A pre-tested questionnaire having details about three prominent dimensions of leadership management, marketing and personal attributes, as measures of company performance were sent to them to analyse and to add to existing stock of knowledge on marketing skills and competency. The questionnaire turnover ratio was 77 percent, which was suitable for statistically significant analysis.The current research has been carried out with aim to find out how much fund multi-national companies (MNCs) should allocate to Human Resource building, also to bring out competencies issues across industries, along with that to develop nexus between employee competencies and firm performance and finally to devise an appropriate mechanisms through which these relationship in companies may operate. Findings of the current work revealed that human resource competencies on the firm performance from marketing perspectives paly its role through different channels namely organizational cultural, communication system, managing changes, knowledge sharing, employees inspiration by leaders, global completion, empowering employees, leadership competency and taking initiatives by employees.It has been observed that performance perception, which is based on manager individual perception and a managerial style, has a negative impact on perceived benefits of creativity negligence factors. Out of nine motivating factors, only five factors were found having statistically significant impact on the competencies of firms in the selected sample of manufacturing and service industries. Analysis shows that management initiatives highlight the fact that not all of them are necessarily successful.
CRONOLOGIA DE AZULEJOS HISTÓRICOS: Danos e Caracterização Química dos Revestimentos das Fachadas do Museu da Abolição no Recife, Pernambuco
Authors: Muniz Eduardo De Freitas , Monteiro Gabriela De Andrade , Lavalle Henry , Souto Maior Paulo Martin , Souza Ravena Barbosa de , Lucena Renata Alves 
Published: March 2018

A pesquisa que resultou neste relatório partiu de uma série de fotografias das décadas de 1970-80, do acervo do Iphan em Pernambuco, nas quais se registra a intervenção ocorrida no antigo Sobrado Grande da Madalena como motivo de sua adequação e reforma para abrigar o Museu da Abolição. A partir dessas imagens e de documentos administrativos da instituição indagou-se quais azulejos eram antigos e quais eram mais recentes, pois não se localizou nenhum registro técnico da intervenção nas fachadas dessa edificação. Embora os azulejos mantenham o padrão estético do século XIX as fotografias denotam intervenções nas quais alguns elementos foram substituídos por réplicas. Mapear o estado de conservação, caracterizar quimicamente e identificar quais azulejos são antigos e quais são recentes foram os objetivos principais desta pesquisa. HISTORICAL TILES CHRONOLOGY: Damage and Chemical Characterization of the Facade Cladding of the Abolition Museum in Recife, Pernambuco ABSTRACTThe research that resulted in this report came from a series of photographs from the 1970s and 1980s, from the Iphan collection in Pernambuco, in which one was registered the intervention in the old Sobrado Grande of Madalena for its adaptation and renovation to the Museum of Abolition. From these images and administrative documents of the institution, we inquired which tiles were old and which were more recent, because no technical record of the intervention in the façades of this building was found. Although all the tiles retain the aesthetic pattern of the nineteenth century, photographs indicate interventions in some tile’s facades that have been replaced by replicas. Mapping the state of conservation, chemical characterizing and identifying which tiles are old and which are recent were the main objectives of this research.Keywords: Historical tiles, chemical and mineralogical characterizations, restoration,
Qualidade de vida e condições para se viver na pesca artesanal em Itanhaém-SP
Authors: PICCOLO Natali Isabela Pierin , MACHADO Ingrid Cabral , HENRIQUES Marcelo Barbosa 
Published: March 2018

Novas contribuições í­Â gestão pesqueira vêm sendo discutidas a partir de abordagens sobre bem-estar e qualidade de vida. No Brasil, a qualidade de vida (QV) e condições para se viver (QCV) foram avaliadas para comunidades rurais e aquicultores, com resultados objetivos que poderiam contribuir também í­Â avaliação da atividade pesqueira de pequena escala. O presente trabalho buscou conhecer o distanciamento entre os olhares de pescadores e técnicos do municí­­pio de Itanhaém/SP sobre a atividade pesqueira em quatro dimensões: social, ambiental, econômica e governança, a partir de uma abordagem qualiquantitativa. A dimensão governança foi a mais crí­­tica e insatisfatória, implicando no menor indicador de qualidade de vida (IQV) e condições para se viver (IQLC), ressaltando a discussão sobre a necessidade de novos estudos sobre como os processos institucionais interferem no bem-estar da comunidade pesqueira da região.