bluuebunny/crossref_metadata_2025_split huggingface dataset:
A teljesexom-szekvenálás jelentősége a ritka neurológiai betegségek diagnosztikájában – saját tapasztalatok egy ataxiás eset kapcsán
Authors: Balicza Péter , Grosz Zoltán , Bencsik Renáta , Illés Anett , Gál Anikó , Gézsi András , Molnár Mária Judit 
Published: July 2018

Abstract: Next generation sequencing (NGS) technologies reshape the diagnostics of rare neurological diseases. In the background of certain neurological symptoms, such as ataxia, many acquired and genetic causes may be present. Variations in a given gene can present with variable phenotypes, too. Because of this phenomenon, the conventional one gene sequencing approach often fails to identify the genetic background of a disease. Next generation sequencing panels allow to sequence 50–100 genes simultaneously, and if the disease stratification is not possible based on the clinical symptoms, whole exome sequencing can help in the diagnostic of genetic disorders with atypical presentation. This case study is about the exome sequencing of a patient with cerebellar ataxia. Genetic investigations identified rare variants in the SPG11 gene in association with the clinical phenotype, which gene was originally described in the background of hereditary spastic paraparesis. Our article highlights that in certain cases the variability of the leading presenting symptom makes it hard to select the correct gene panel. In our case the variants in the gene, formerly associated to hereditary spastic paraparesis, resulted in cerebellar ataxia initially, so even an ataxia NGS gene panel would not detect those. Orv Hetil. 2018; 159(28): 1163–1169.
A léböjtkúrák és az atípusos evészavarok lehetséges kapcsolata. Kvalitatív előtanulmány
Authors: Bóna Enikő , Forgács Attila , Túry Ferenc 
Published: July 2018

Abstract: Introduction and aim: There are two notable eating behaviors that are not far from having their own category as a mental disorder: the purging disorder, that is already among the DSM-5 non-specific eating disorders, and orthorexia nervosa, when a person is fixating too much on healthy foods. Our purpose is to describe how these can be observed in recreational juice cleanse camps, which are very popular today as an alternative health trend. Method: The first author recorded her data during multisited ethnographic observations in two Hungarian juice cleanse camps. Based on the diary logs, notes and interviews collected, we will present the motives of eating anomalies that the participants had shown. Results: The main motive of the camp is “detoxification”. The lack of solid food, drastically low calorie intake and lots of physical activity will bring an inevitable change in the body, that is interpreted as toxins leaving the body. Participants have also included deliberate use of laxatives in their everyday routines, with which they associate positive connotations and are linked to the spiritual processes of “letting it go” and “renewal” in the spirit of a holistic approach. The use of symbols in the physiological processes was highly noticeable. Rapid weight loss due to diuresis, the desire for “clean” meals, and “self-rewarding” borrowed from the esoteric-self-help culture are also common motives. Due to the refeeding complications, so far two deaths have been reported by camp organizers. Conclusions: Both purging disorder and orthorexia nervosa can be well-identified in our observations. This shows that also in the non-clinical environment, there is an institutionalization of eating habits that are dangerous to the health. This “detox” is not only physiologically harmful, but it is not proved to provide long-term help in mental health either. As a solution, we advocate developing an appropriate health communication plan for misconceptions about healthy lifestyle and eating, and also a promotion of psychotherapeutic opportunities. Orv Hetil. 2018; 159(28): 1153–1157.
A terhességet megelőzően alkalmazott hosszabb távú orális fogamzásgátlás mint a magzati 21-es triszómia lehetséges kockázatcsökkentő tényezője idős anyai életkorban vállalt terhességben
Authors: Horányi Dániel , Babay Lilla Éva , Győrffy Balázs , Nagy Gyula Richárd 
Published: July 2018

Abstract: Down syndrome is the most common autosomal chromosomal abnormality. According to the classical interpretation, it is the result of meiotic nondisjunction. Its occurrence is more common in advanced maternal age. Despite intensive research, pathophysiology of this genetic disorder is not fully understood. According to recent studies, a different kind of mechanism may be found in the background of trisomy 21 than was previously considered. Based on the ovarian mosaicism model, the cause of trisomy 21 (or any common trisomy) is a segregation error of a chromosome in premeiotic mitosis. The cell entering meiosis will be an oocyte with preexisting trisomy, where its (so-called “secondary”) nondisjunction is essential. Maturation of the trisomic oocytes appears to fall behind the disomic oocytes, resulting in their relative accumulation in the ovaries as time progresses. The ratio of trisomic/disomic cells becomes less favorable in maternal maturity. If ovulation is inhibited – although the number of oocytes will continue to decline due to apoptosis – it can be assumed that the trisomic/disomic oocyte ratio remains more favorable with the progression of age. In our summary report, presenting and updating our previous data, we would like to propose that – according to ovarian mosaicism model – long-term oral contraception in the anamnesis may be beneficial in pregnancies with advanced maternal age. Orv Hetil. 2018; 159(28): 1146–1152.
Practical aspects of treatment of kidney cancer in a modern hospital: the evolution of surgical approaches
Authors: Rakul S. A. , Pozdnyakov K. V. , Eloev R. A. , Pliskachevskiy N. A. 
Published: July 2018

Objective: analysis of results of the outcomes of surgical treatment for renal tumors in the Saint Petersburg City Hospital No. 40 over the last 5 years and determination the trends.Materials and methods. The study included 293 patients that underwent 296 surgeries for renal tumors. The majority of patients (87.84 %) were diagnosed with localized cancer, whereas locally advanced and metastatic forms were detected in 4.39 and 7.77 % of cases respectively. We performed radical nephrectomy (RNE) or nephron sparing (NS) via open or minimally invasive route (videoendoscopic or robot-assisted surgery using the da Vinci Surgical System).Results. Organ-preserving surgeries were performed in 52.36 % of patients, RNE – in 47.64 % of patients; minimally invasive and open surgeries were conducted in 95.95 and 4.05 % respectively. In individuals with stage cT1a cancer, NS and RNE were performed in 87 and 13 % of cases respectively; in patients with stage cT1b cancer, NS and RNE were performed in 50.82 and 49.18 % of cases respectively. The majority (90.2 %) of patients with renal tumors >7 cm underwent minimally invasive surgeries, primarily RNE. The incidence of severe postoperative complications after NS and RNE was comparable: 5.75–8.06 and 1.67–15.38 % respectively (р = 0.64).Conclusion. Minimally invasive NS is the method of choice for stage сT1 tumors; however, in some cases, we should also consider it for tumors >7 cm. Videoendoscopic surgery is the most preferable option for these patients, whereas robot-assisted techniques should be used for organpreserving surgeries and RNE in difficult cases.
LATE KROTOVO (CHERNO-OZERYE) BURIALS WITH CASTING MOLDS FROM TARTAS-1, BARABA FOREST-STEPPE
Authors: Molodin V. I. , Durakov I. A. 
Published: July 2018

We describe fi ve burials with casting molds from the Late Krotovo (Cherno-Ozerye) cemetery Tartas-1 in the Baraba foreststeppe. Three of them form a distinct group belonging to two parallel rows of graves. One of the reconstructed molds was destined for casting Seima-Turbino-type celts, the other, for manufacturing thin rods, round in section. One kernel was used for making a hollow in a tiny chisel-like tool. All molds are made of clay and bear traces of prolonged use. They were made using a bottom board; molding mixture was smoothed and tamped, excessive material was removed with a narrow-bladed cutting tool, and lines were drawn on the raw surface. Certain graves with molding tools were single, others were collective. All basic age and sex groups are represented: adults, adolescents, women and children, apparently suggesting that all were involved in manufacture, and the skills were transmitted from one generation to another. Because children were involved too, status was heritable. Emphasis on bronze casting in the funerary rite, virtually without traces of other specializations, indicates a separate social stratum, whose share was no higher than 4 %. Obviously, not all its members were professional casters; some may have participated occasionally.
OFFERINGS OF HUNNIC-TYPE ARTIFACTS IN STONE ENCLOSURES AT ALTYNKAZGAN, THE EASTERN CASPIAN REGION
Authors: Astafyev A. E. , Bogdanov E. S. 
Published: July 2018

In 2014–2015, nine enclosures built of stone slabs were excavated at Altynkazgan on the Mangyshlak Peninsula, Republic of Kazakhstan. Inside them, remains of offering ceremonies were found: vessels dug into the ground, altars made of limestone blocks, and pits for offerings. In one of these, we found a richly decorated bridle, in another, a belt set of inlaid golden plaques, and in the third, remains of a saddle (silver plates and other items). The entire assemblage has numerous parallels among the 5th and 6th century fi nds from the northern Black Sea area, North Caucasus, and the Volga basin. Ritual burial of a “golden” belt, a bridle, and a ceremonial saddle indicate an advanced cult that included offerings of prestigious belongings of a horseman. These rituals were introduced by Iranian-speaking nomads who had migrated to the eastern Caspian region during the Hunnic raids to Iran in the 5th century. At that time, owing to the regressive phase of the Caspian Sea, the semi-desert northern Caspian coast was connected with Mangyshlak by a land bridge. Our hypotheses are supported by both historical records and modern geomorphological studies of the Caspian Sea.
SILVER VESSELS OF THE GOLDEN HORDE ERA FROM THE MIDDLE OB BASIN
Authors: Fedorova N. V. 
Published: July 2018

This article addresses one of the most informative archaeological sources for reconstructing medieval history––imported silverware, which is abundant in northwestern Siberia. Based on three newly described silver vessels of the Golden Horde Age from the Saigatino I sanctuary in the Surgut region of the Ob basin, the possible attribution of these and similar specimens from Siberia and Ural is discussed. Locating the places where such items had been made is difficult for reasons relating to the functioning of individual workshops and of the silversmiths’ schools in general. Nevertheless, a suggestion is made that the workshops were located in the Bulgar Ulus––a successor of the Volga Bulgaria, with its rich tradition of manufacturing jewelry and transporting it along the trade routes directed northward to western Ural and western Siberia. The time and the routes whereby the Golden Horde toreutics was brought to western Siberia are assessed. All finds in this region cluster within a small area in the Surgut region of the Ob basin, while the possible trade routes are marked by similar but isolated finds in the Perm area and in northern Ural. The question is raised whether the finds of imported silverware in western Siberia fall within the territory of medieval Ob principalities––Koda, Bardakov, and Kunovatsky. The appearance of personal hoards may indicate the emergence of new elite. Generally, the imported silverware of the Golden Horde age is highly relevant to social developments in medieval western Siberia.
ANIMAL BONES IN RITUAL ACCUMULATIONS AT MONKYS URIY
Authors: Lobanova T. V. , Kardash O. V. 
Published: July 2018

At Monkys Uriy, a late 16th–17th century fort on the Bolshoy Yugan River, in the taiga zone of the Ob basin, Western Siberia, bones of wild animals (reindeer, elks, brown bears, and wolves) and those of domestic dogs were found in residential areas together with artifacts. We describe ten ritual accumulations of bones, species composition, that of skeletal elements, fragmentation types, and the age of animals. Seven accumulations of bones were found at residential quarters. Six of them contained complete or partial skeletons of reindeer and cranial bones of an elk. These accumulations may indicate construction sacrifi ces and those marking childbirth. Three bone accumulations found outside the residence area include bones of a dog and a brown bear, evidently sacrifi ced during funerary rites. Ethnographic and folkloric evidence suggests that such sacrifi ces were practiced by the Yugan Khanty as late as the 19th and 20th centuries and had been rooted in ancient traditions of Ugrians and Samoyeds.
MANIFESTATION OF WORLDVIEWS IN TRADITIONAL NARRATIVES: RECONSTRUCTION OF GLOBAL TENDENCIES IN THE SPREAD AND THE CHRONOLOGY OF EMERGENCE OF MYTHOLOGICAL MOTIFS
Authors: Berezkin Y. E. 
Published: July 2018

Data on the areal distribution of motifs extracted from ca 25,000 traditional narratives were computed with the purpose of revealing a chronology of the emergence of particular mythological themes. The statistical processing of this material allowed selection of sets of motifs that probably correspond to the routes of major prehistoric migrations known thanks to archaeology and population genetics. Our conclusions are largely based on the comparison of similar sets of motifs in the Old and New Worlds, the time of the peopling of America and its particular episodes being more or less known (initial peopling by Pacifi c and then by continental Siberian groups). Thanks to the methods applied, the epochal dynamics of the development of mythology were for the fi rst time reconstructed by using systematized data, and not by proceeding from general assumptions. The earliest complex, which is related to the explanation of the mortal nature of man and the loss of the easy life, corresponds to the southern route by which humans of the modern type moved from Africa to the Indo-Pacifi c borderlands of Asia. These motifs are abundant in sub-Saharan Africa, the southern part of Eurasia, Oceania and America (especially South America), but rare in northern Eurasia and the American Arctic and Subarctic. Motifs relating to the origin of man, human anatomy, and relations between the sexes are most typical of the CircumPacifi c world. This theme probably fi rst developed in Southeast Asia among the people who came from Africa, but before the time when their earliest groups reached America. The geographic distribution of motifs relating to cosmogony and cosmology, and to the etiology of natural phenomena, plants, and animals suggests that many of the corresponding motifs initially appeared in southern Eurasia, were then brought to Siberia, and from there brought to the New World (this movement could be explained by the gradual northward displacement of population after the Late Glacial Maximum). The ideas relating to the interpretation of celestial objects were the last to develop. Corresponding motifs are only abundant in Northern Eurasia, from where many of them were brought to North America but not to South America. Interpretations of celestial objects in European cosmonymy mostly date to the Bronze Age, if not to Iron Age technology, while some are related to the spread of world religions.
Terrorismo: recurrencia, causalidad y expansión
Authors: Molano Rojas Andrés 
Published: July 2018

Para efectos de este trabajo, el terrorismo se entiende como un método de acción política violenta, que tiende a articularse en procesos de larga duración (campañas terroristas), con el fin de compensar asimetrías en el contexto de un conflicto. Como método, el terrorismo opera provocando una destrucción o caos suntuario, según un modelo eminentemente transitivo y cuyo efecto psicológico es superior a sus efectos materiales (por cuanto elige objetivos con alto valor simbólico), a efectos de transmitir un mensaje y afectar grandes audiencias, en aras de la promoción (principal aunque no exclusivamente) de determinadas pretensiones políticas (Molano-Rojas, 2010). Una de las características más llamativas del método terrorista es su tendencia a presentarse en oleadas de cobertura global. Un investigador pionero (Rapoport, 2004: 47) definió estas oleadas como “un ciclo de actividad en un periodo determinado –un ciclo caracterizado por fases de expansión y contracción… (cuyo) rasgo fundamental es su carácter internacional; actividades similares que ocurren en varios países, orientadas por una fuerza común predominante que determina las características y relaciones mutuas entre los grupos participantes”. Siguiendo esta hipótesis, se ha sugerido en otra parte (Molano-Rojas, 2009b) la existencia de tres ciclos globales de terrorismo, es decir, momenta (más que periodos) durante los cuales se intensificó y extendió el uso del terrorismo como método predilecto para la acción política por diversos grupos en todo el mundo: un “ciclo revolucionario” (que va desde 1870 hasta 1914, aproximadamente), uno “emancipatorio” (que se produciría entre 1948 y 1980), y uno más reciente, el “milenarista”, que data de la década de 1990 y actualmente estaría en plena expansión.
Projeto musicalização quilombola: música e reflexões
Authors: Oliveira Ilton Luiz Fonseca de 
Published: July 2018

<p>O presente artigo tem como objetivo publicizar as atividades desenvolvidas no Projeto de Extensão Musicalização Quilombola desenvolvido pelo Instituto Federal de Educação, Ciência e Tecnologia da Paraíba Campus Catolé do Rocha no ano de 2017 . Este projeto teve como objetivo a formação musical básica e a construção de uma banda rítmica voltada para o repertório afro-brasileiro envolvendo crianças e adolescentes da comunidade quilombola Lagoa Rara - localizada na zona rural da cidade de Catolé do Rocha PB . Essas ações visaram contribuir com perpetuação da cultura Quilombola . Além disso, esse projeto destinou a fomentar a reflexão a respeito da identidade quilombola, da discriminação racial e da inclusão</p>
Escritório modelo Projetart: projetando sonhos, garantindo qualidade de habitat
Authors: Dantas Anna Aline Roque Santana , Dantas Niara Fernandes Barbosa Formiga , Dos Santos Natan Patrick Lima , Alves Caroline Dos Santos , Moreira Luccas Brito Nunes , Lima Esther Almeida , Dantas Bisneto Pedro Tomaz , De Araújo Luís Filipe França 
Published: July 2018

<p>O escritório modelo “Projetart” é um projeto extensionista, que leva à população da cidade de Picuí e adjacências,consultoria em atividades da construção civil. O projeto está em funcionamento no Instituto Federal de Educação, Ciência e Tecnologia da Paraíba (IFPB) - <em>campus</em> Picuí desde o ano de 2013 e tem como integrantes docentes e discentes do curso técnico em edificações integrado ao ensino médio. No escritório modelo, os alunos envolvidos lidam com o desenvolvimento de projetos arquitetônicos, com necessidades e dificuldades próximas da realidade do mercado de trabalho que irão enfrentar com o término do curso técnico nesta instituição. Durante o projeto, os alunos tem a liberdade de criação dos projetos, sendo supervisionados por professores qualificados para tal atividade. Os membros do escritório modelo também se aprofundaram em temas da arquitetura bioclimática, tornando-se cada vez mais capazes de elaborar projetos eficientes, que colaborem com o meio ambiente no qual estão inseridos e que possam melhorar as condições de habitabilidade para seus usuários, independente das dificuldades climáticas impostas. Dessa forma, a "Projetart" oferece serviços de qualidade e sem custos à sociedade.</p>
Isogravando no Cariri Paraibano
Authors: Ferreira Felipe Louise Pereira 
Published: July 2018

<p>Este artigo tem como objetivo relatar as experiências do Projeto de Extensão intitulado “Isogravando no Cariri Paraibano”, desenvolvido por servidores e discentes do Instituto Federal de Educação, Ciência e Tecnologia da Paraíba (IFPB) - Campus Monteiro na região do Cariri Ocidental Paraibano. O objetivo do projeto foi contribuir para a utilização da isogravura como meio pedagógico lúdico, agradável e criativo de ensinar e aprender arte, disseminando através de oficinas a prática da isogravura nas escolas de Monteiro e região, bem como em espaços coletivos de vivência, incentivando a prática, a criatividade, a livre expressão e a utilização de meios de fácil aquisição e manuseio para produzir arte. A metodologia utilizada para o cumprimento desse objetivo foi a realização de oficinas, metodologia de trabalho que se baseia na formação coletiva. Participaram das oficinas pessoas com idade compreendida entre 5 e 90 anos. Como resultado, o projeto proporcionou a difusão da arte da isogravura entre o maior número de pessoas possível, ajudando na formação do conhecimento mútuo e individual, além de levar a produção de arte, a livre expressão e criação artística para fora da sala de aula, onde pessoas do povo podem valorizá-la e apropriar-se dela como instrumento de soma de vivências.</p>
Aperfeiçoamento instrumental: relato de experiência
Authors: Lima Marlon Barros de , Santos Carlos Victor Silva dos , Nascimento Isabelle Melo do 
Published: July 2018

<p>O projeto Aperfeiçoamento Instrumental, foi realizado no Instituto Federal da Paraíba Campus Monteiro dentre os meses de maio e novembro de 2017 contemplado com taxa de bancada e bolsas para aluno através do Edital de Extensão nº 01/2017 – PROBEXC PROJETO. Dentre os objetivos, o projeto buscou oferecer aulas de saxofone, trompete, trombone, tuba, bateria e percussão para alunos da região do cariri paraibano. Assim, foi possível oferecer aulas de instrumento com professores específicos, além da realização de um evento voltado para instrumentos de metais, o I Encontro de Metais do Cariri. Portanto, tivemos a participação de 16 alunos ativos do projeto, e cerca de 80 através do evento, oriundo de 15 cidades do estado da Paraíba e Pernambuco.</p>
Alimentação saudável e reeducação alimentar na Escola Municipal “Irmã Iraídes” na Cidade de Sousa-Pb
Authors: Barreto Heloiza Carneiro , Andrade Maria Edileuza Leite de , Souza Lane Maria de Oliveira Gadelha , Lins Francisca Bivania de Araujo , Fernandes Maria Do Socorro Abrantes , Pereira Laisa Rodrigues , Silva Maria Wanessa de Oliveira , Candida Josefa Josydeth Santana 
Published: July 2018

<p>Este estudo teve como objetivos promover uma reeducação alimentar com o consumo de alimentos saudáveis, manter hábitos de higiene e a consciência de sua contribuição para a promoção da saúde de uma forma lúdica e educativa. A A escola tem 104 alunos matriculados, sendo quatro turmas no turno da manhã com 86 alunos cursando Educação Infantil 1, 2 e 3 na faixa etária de 03 a 05 anos e 1º ano Fundamental I de 06 a 08 anos. No turno da tarde consta de 30 alunos no 2º e 3º do Fundamental I, na faixa etária de 07 a 11 anos. As atividades foram desenvolvidas em etapas semanalmente na própria escola e no turno da tarde. Em comemoração ao dia 12 de outubro, foi realizada a VII Festa em homenagem ao dia da criança com brincadeiras e lanches saudáveis. De acordo com os resultados obtidos nesse estudo, em relação ao consumo de bolo de cascas de abacaxi a aceitabilidade foi de 98% enquanto que na salada de frutas, Milk Shake, e pão com ervas a aceitabilidade foi de 100%. Este resultado pode favorecer a inserção destes lanches no cardápio da merenda escolar. </p>
Modelos tridimensionais de papel como ferramenta de aguçamento sensorial por deficientes visuais
Authors: Nunes Ângela Araújo 
Published: July 2018

<p>Artigo expõe a experiência do projeto de extensão MODELOS DE PAPEL COMO FERRAMENTAS DE PERCEPÇÃO SENSORIAL PARA ENSINO DE GEOMETRIA PLANA E ESPACIAL PARA DEFICIENTES VISUAIS, realizado no ano de 2017. Surgiu como derivação de projeto de pesquisa realizado a partir de 2016 no IFPB – Campus Patos, que propunha a utilização de sólidos geométricos físicos de papel como auxiliares metodológicos no ensino da disciplina de Desenho Técnico, para os cursos de Edificações e Eletrotécnica. </p>