bluuebunny/crossref_metadata_2025 huggingface dataset:
FLUTUAÇÕES CLIMÁTICAS DA PRECIPITAÇÃO NO VALE DO ITAJAÍ (SC)
Authors: Severo Dirceu Luis , Silva Hélio Dos Santos , Tachini Mário
Published: June 2019

A precipitação é uma componente climática de alta variabilidade espacial e temporal e, por isso estudos relacionados ao seu comportamento podem auxiliar nas práticas de gerenciamento de risco de diversas atividades econômicas. Este trabalho analisou as diferentes escalas temporais presentes na série temporal de precipitação registrada no posto pluviométrico da Agência Nacional de Águas (ANA) instalado no município de Pomerode, estado de Santa Catarina, no período de 1930 a 2015. A análise de múltiplas escalas foi realizada com a Transformada Wavelet enquanto que a análise de tendências foi feita através do teste de Mann-Kendall. As oscilações foram analisadas nas bandas intrasazonal, interanual, decadal e intermediárias. Na banda intrasazonal encontraram-se picos de alta variância significativos, principalmente em anos de El Niño.  Nas bandas interanual e decadal notou-se sobreposição dos picos significativos nas flutuações que apresentam períodos de 2,9, 8,2, 13,1 e 28,2 anos. Esta sobreposição de escalas temporais pode explicar a alta variabilidade temporal da precipitação no vale do Itajaí.
ESTUDO DE IMPACTO AMBIENTAL (EIA) APLICADO À AMPLIAÇÃO DA UNIVERSIDADE FEDERAL DE SANTA MARIA – CAMPUS FREDERICO WESTPHALEN/RS
Authors: Toniazzo Fabiane , Lavnitcki Laís , Ludwig Henika Marcia , Galvan Kelli Andreiza , Ferrão Custodio Passini Aline
Published: June 2019

Atualmente, as questões relativas ao ambiente natural têm preocupado a sociedade, tomando proporções universais. Por este motivo, normas e legislações vêm se tornando, cada vez mais, importantes e efetivas. Logo, licenciamentos e estudos de impactos ambientais estão sendo utilizados como ferramentas, tendo-se como objetivo a minimização de impactos socioambientais. Este trabalho objetivou prever possíveis impactos que possam ser causados durante uma possível ampliação da Universidade Federal de Santa Maria (UFSM), campus Frederico Westphalen/RS. O desenvolvimento do estudo abrangeu visitas a campo, pesquisas bibliográficas e análise de documentos e materiais disponibilizados pela instituição. Com isso, utilizou-se a matriz de Leopold para “quantificar” os graus de impacto de cada atividade relacionada, avaliando-se três possíveis formas de expansão territorial do campus. Tendo sido verificado que a segunda alternativa respeita os limites territoriais e explora de forma menos negativa os recursos naturais. Foi possível observar que através do EIA se analisou e levantou os impactos positivos e negativos mais importantes decorrentes desta ampliação, visando à construção de obras de infraestrutura e por meio destas informações foi possível indicar as medidas necessárias para a mitigação dos principais impactos negativos levantados.
ESTUDO COMPARATIVO DE TRÊS MECANISMOS DE REDUÇÃO DE EVAPORAÇÃO DE RESERVATÓRIOS
Authors: Helfer Fernanda , Louzada José Antônio , Zhang Hong , Lemckert Charles , Castro Nilza Maria dos Reis
Published: June 2019

O uso de tecnologias para redução da evaporação de reservatórios pode ajudar regiões áridas e semiáridas a superar a escassez hídrica. Alguns mecanismos, como filmes monomoleculares e estruturas flutuantes, podem apresentar efeitos negativos nos ambientes aquáticos e na qualidade da água. Este trabalho investigou a eficácia de três mecanismos de redução de evaporação que possuem alto desempenho ambiental: coberturas suspensas, destratificadores e quebra-ventos. A análise foi conduzida com o modelo DYRESM em um reservatório localizado no sudeste do estado de Queensland, Austrália. O modelo foi primeiramente calibrado e validado para o reservatório em estudo. Em seguida, as condições de contorno foram alteradas para refletir os efeitos dos mecanismos de redução de evaporação no comportamento térmico do reservatório. O método mais eficaz foi o uso de coberturas suspensas, que reduziu em 88% a evaporação. O uso de quebra-ventos reduziu em 27% a evaporação, e o uso de destratificadores não teve efeito significativo na redução da evaporação. Para cada um dos mecanismos estudados, uma análise compreensiva dos seus efeitos na temperatura da água e nas taxas de evaporação foi efetuada, inferindo sobre os potenciais resultados do uso destes mecanismos em reservatórios de outras condições climáticas.
Vulnerabilidades socioeconômicas e produtivas dos agricultores familiares pobres de Santa Catarina
Authors: Lovatel Marlise , Simonetti André Luiz , Gazolla Marcio
Published: June 2019

O objetivo do trabalho é analisar as condições socioeconômicas e produtivas dos agricultores familiares pobres do estado de Santa Catarina, traçando um perfil dos mesmos para melhor compreender suas vulnerabilidades sociais. Para isso, utilizou-se dos dados do Censo Agropecuário do IBGE (2006), estratificando os agricultores em familiares e não familiares com base na Lei 11.326/2006. No grupo familiar, separou-se os do Grupo B do PRONAF, com base nos critérios do Plano Safra 2006/2007, sendo estes chamados de agricultores familiares pobres. Como resultado, evidencia-se que um a cada quatro agricultores é considerado pobre, não somente por possuir baixa renda econômica, mas por se caracterizar uma pobreza multidimensional, como Amartya Sen formulou em seus estudos. Os agricultores pobres apresentam vulnerabilidades sociais múltiplas, como limitação de terras, baixo nível tecnológico e de escolaridade, envelhecimento, pouca organização social coletiva, baixo valor de produção agropecuária e quase não recebem serviços de orientação técnica do Estado. O estudo identificou a necessidade de se construir estratégias para melhorar seus ativos, acessos, capacitações básicas e políticas públicas com vistas a promover ativamente o desenvolvimento rural e regional destes atores sociais.
A construção de um Eldorado? O projeto socialista do Orinoco como estratégia de desenvolvimento regional na Venezuela
Authors: Da Silva Robson Dias , De Matos Manuel Victor Martins
Published: June 2019

O principal objetivo do artigo é apresentar as linhas gerais da importância da indústria petrolífera para a Venezuela a partir do estudo da tentativa de expansão produtiva e urbana em direção à faixa centro-oriental do território nacional sob os objetivos do Projeto Socialista do Orinoco (PSO). Baseado em revisão da literatura e análise de dados secundários, o trabalho discute a proposta de adensamento urbano e diversificação produtiva comandada pela grande indústria de petróleo, assinalando seus objetivos, suas características e os desafios enfrentados. O texto conclui que a proposta de crescimento econômico e diversificação, baseada na indústria de petróleo, mostrou-se frágil pela alta dependência inicial à conjuntura internacional favorável e por não conseguir superar problemas estruturais crônicos de longa data que afetam o país, tal como a valorização cambial, que atua no desestímulo à substituição de importações.
Dinâmica do desenvolvimento regional brasileiro: uma discussão a partir de métodos de análise regional
Authors: Silva Almir Cléydison Joaquim da , Raposo Jaciara Gomes , Bagattolli Carolina
Published: June 2019

As experiências históricas têm demonstrado a importância da atuação estatal na condução de estratégias nacionais de desenvolvimento e na correção de desigualdades no interior dos países. No Brasil, essas estratégias estimularam o processo de concentração industrial e tem ocorrido via industrialização e políticas de estabilidade macroeconômica, variando conforme inclinação e orientação dos governos em políticas de corte mais ortodoxo ou mais heterodoxo. Aqui se objetiva discutir a trajetória recente das políticas de desenvolvimento regional no Brasil, a partir da identificação de padrões regionais de crescimento. Além de uma discussão teórica de aspectos do desenvolvimento regional, a pesquisa também se vale de uma análise quantitativa, com o cálculo de medidas de localização e especialização regional. Para tanto, utilizam-se dados de emprego para os anos de 2002, 2009 e 2016, presentes na matriz de informações de emprego por setores da economia e por áreas geográficas. Os resultados indicam a continuidade de problemas relativos à desigualdade regional no país. Embora as regiões Norte, Nordeste e Centro-Oeste se beneficiem de políticas e fundos constitucionais e de desenvolvimento para estimular suas economias, as regiões Sul e Sudeste concentram, ainda, uma estrutura produtiva mais dinâmica e de maior peso na composição setorial do país.
Development and Shelflife Study of Papaya-Black Grape Blend Fruit Rollups
Authors: Saranya V. , Uma Devi K. , Suneetha W. Jessie , Kumari B. Anila , Suneetha K. B.
Published: June 2019

The study aims to standardize papaya – black grape blend fruit rollups in proportions of 70:30, 60:40, 50:50 with experimental Ezidri food dehydrator and to compare them with 50:50 of control cabinet dryer rollups. Experimental Ezidri 50:50 ratio rollups were accepted by taste panel judges compared to other ratios and control sample. The results revealed that moisture content, TSS, thickness, pH, vitamin C, β – carotene, L*, a* and b* color values of fruit rollups decrease while, titrable acidity and microbial counts increase in storage period to 10 weeks at room temperature. It was concluded that Ezidri papaya – black grape sample showed superior qualities than control sample (cabinet) during storage.
Forum
Authors: Goodman Penelope J.
Published: June 2019

A forum was the monumental civic center of a city in the western Roman empire. Already common by the third century
bce
, fora became increasingly regularized and formalized over the succeeding centuries. Those in Rome always remained important archetypes, but new developments such as the double forum‐basilica complex flourished in the northwestern provinces. Typically an open rectangular area surrounded by public buildings, the forum was the metaphorical heart of urban life, offering essential space for communal activities such as markets, religious ceremonies, and political proceedings. It also became an important locus for elite display directed towards both residents and visitors.
Hinterland
Authors: Goodman Penelope J.
Published: June 2019

A hinterland is the primary short‐distance trading area for an urban central place. In antiquity, high transport costs meant that this area usually supplied the bulk of a city's food needs, making it expedient to control it politically. Nonetheless, long‐distance trade networks allowed some cities to cultivate economic hinterlands which extended well beyond their own directly‐controlled territory. City and hinterland have sometimes been seen as starkly distinct or even conflicting units, but in reality, they were intimately connected. New data generated by the growing discipline of landscape archaeology is increasingly clarifying the interdependent relationship between the two.
Calendar, Jewish
Authors: Stern Sacha
Published: June 2019

The function of Jewish calendars in antiquity was mainly to determine the dates of biblical and other Jewish festivals; in Judaea/Palestine, they were also used for more general purposes. Jewish calendars were always lunar, except for the short‐lived, schematic calendars of the Dead Sea Scrolls. The lunar calendars were diversely reckoned among Palestinian and Diaspora communities. In Late Antiquity, they gradually turned into fixed schemes.
Akhenaten (Amenhotep
IV
)
Authors: Dodson Aidan
Published: June 2019

Akhenaten, originally known as Amenhotep IV, king of Egypt, 18th Egyptian Dynasty, later fourteenth century
bce
.
Co‐regency (Egypt)
Authors: Dodson Aidan
Published: June 2019

The institution within ancient Egypt whereby two co‐equal kings reign over the same territory at the same time. This is to be distinguished from an arrangement whereby a territory is subdivided, with the different parts of that territory ruled as separate entities by kings ruling in parallel.
Sety (Sethy, Seti) I–II
Authors: Dodson Aidan
Published: June 2019

Sety I and II, kings of Egypt, 19th Egyptian Dynasty, thirteenth to twelfth centuriesbce.
Tetisheri
Authors: Dodson Aidan
Published: June 2019

Tetisheri, queen of Egypt, sixteenth century
bce
. Mother of king Seqenenre Taa, grandmother of king Ahmose I, and probably wife of King Ahmose the Elder.
Tutankhamun
Authors: Dodson Aidan
Published: June 2019

Tutankhamun, king of Egypt, 18th Egyptian Dynasty, late fourteenth century
bce.
Cerveteri (Caere)
Authors: Pieraccini Lisa C.
Published: June 2019

The Etruscan metropolis of Caere, known today as Cerveteri and located some 45 km northwest of Rome, was situated on a tufa outcrop with a commanding view of the Tyrrhenian Sea. It is famous for its monumental tombs distributed over several necropoleis. Caere flourished in the eighth century
bce
, but reached its zenith by the seventh and sixth centuries
bce
, producing some of the most iconic works of Etruscan art (early bucchero, life‐size clay sculpture, gold jewelry, painted wall plaques and vases). The city's urban splendor was evident in its temples, sanctuaries, and shrines.
Conic sections
Authors: Fried Michael N.
Published: June 2019

Conic sections are curves obtained by cutting a cone with a plane which does not pass through the vertex of the cone. The principal conic sections are the parabola, the hyperbola, and ellipse. The circle can also be obtained as a conic section, but, in Greek mathematics, the circle is usually defined by simpler means. Conic sections were studied intensely throughout the Hellenistic period, but particularly by Apollonios of Perge, whose work
Conics
stands as one of the great works of all Greek mathematics.
Scribonius Largus
Authors: Oberhelman Steven M.
Published: June 2019

Scribonius Largus (
fl
. mid‐first century
ce
) was a physician who practiced at the court of the emperor Claudius. He was the author of the
Compositiones medicamentorum
, a compendium of medical recipes marked by polypharmacy
Melampus
Authors: Oberhelman Steven M.
Published: June 2019

Melampus was reputedly the first mortal to practice medicine through incantations and remedies based on animal and mineral substances. He was also the first to master all the arts of divination (portents, inspection of sacrificial victims, and inspiration). Melampus was said to introduce many cults into Greece, including the worship of Dionysus and of Demeter. Melampus’ descendants became a line of seers and prophets.
Paul of Aigina
Authors: Oberhelman Steven M.
Published: June 2019

Paul of Aigina (fl. seventh centuryce) was an important physician of the Byzantine period who wrote a compendium dealing with illness, surgery, and pharmacology. His medical handbook was intended to serve as an encyclopedic record of major ancient and early Byzantine Greek doctors and herbalists. Paul's work, through Syriac and Arabic translations, greatly influenced Islamic medicine and the development of Western medicine.
Burial clubs
Authors: Liu Jinyu
Published: June 2019

Very few ancient associations were founded for the sole purpose of performing burials. Nor was there a particular category of funerary associations that were authorized by law in the Roman imperial period, as scholars used to believe. Ancient associations’ involvement in funerary activities for their members may take a variety of forms.
Siblings
Authors: Harders Ann‐Cathrin
Published: June 2019

The relationship between siblings is special due to its longevity and its rather egalitarian character, especially between the sexes. It was seen as an intimate and affectionate bond; although brotherly solidarity was tested due to laws of inheritance and competition for honors and offices, brothers were expected to cooperate. In Greece as well as in Rome, they cared for their sisters and their offspring and acted on behalf of the father as a sister's guardian. The sibling bond thus proved to be a positive model to shape other social relationships as well, most notably those between members of early Christian communities.
Martial (Marcus Valerius Martialis)
Authors: Coleman Kathleen M.
Published: June 2019

The Spanish poet Martial, who moved to Rome ca. 64
ce
, composed fifteen books of epigrams between the reigns of Titus and Trajan. His reflection of contemporary social life is both vivid and stridently opinionated; his purpose is wit, not impartiality. He expresses contemporary prejudices against foreigners and other minorities, and is notorious for his obscenity. He flatters his many influential patrons, most prominently the emperor Domitian, while simultaneously mocking the patronage system. His inconsistencies are best ascribed to poetic opportunism, and it is now generally recognized that he adopts an authorial persona, rather than speaking in his own voice.
Sardinia, Nuraghe Sirai
Authors: Perra Carla
Published: June 2019

Nuraghe Sirai (southwest Sardinia) is a unique example of an integrated settlement pattern in the local Iron Age II between Nuragics and Phoenician settlers. Its importance lies in the evidence of a new phase in Nuragic civilization and in its original and mixed material culture of broadly mid‐sixth‐century
bce
date. Thanks to the large amount of data collected and research undertaken (a new site typology – the fortress – as well as new shapes in pottery and architectural artifacts), it is possible to suggest a
preliminary repertoire
of late Sardinian IAII material culture (625–550
bce
) and to document in more aspects the most intensive Phoenician presence in the Central Mediterranean.
Cassius (Gaius Cassius Longinus)
Authors: Gamauf Richard
Published: June 2019

We are better informed about the life and career of Cassius Longinus than of most jurists. He held various high public offices under the Julio‐Claudian dynasty and is perhaps best known for a speech he gave to the Senate in 61
ce
advocating a harsh line in response to the murder of the Urban Prefect by a member of his household. He was the leader for a time of the group of jurists known as the “Sabinian school,” which was at times called after him (“Cassians”).
Marcellus (Ulpius Marcellus)
Authors: Maragno Giorgia
Published: June 2019

Ulpius Marcellus (mid‐ to late second century
ce
) was a prominent Roman jurist of the age of Antoninus Pius and Marcus Aurelius who participated in their imperial councils. Little is known about his life; he was probably born about 120–5 and died around 185. Marcellus, well versed in rhetoric and an original legal thinker, is often represented in Justinian's
Digest
. His production is not vast but still significant. He wrote a
Commentary on the Lex Iulia et Papia
,
Notes on Pomponius's single book of rules
, a
Single book of answers
, a monograph about the
Duties of a consul
,
Notes on Julian's Digest
, and a
Digest
.
Vivianus
Authors: Procchi Federico
Published: June 2019

Active in the first century
ce
, Vivianus is known to us only through citations from subsequent authors (most of which are collected in Justinian's
Digest
), which invoke his work while often showing appreciation for and even sharing his views. The precise nature and even the titles of Vivianus’ works are also unknown.
Age differences in life stories and neuroticism mediate age differences in subjective well‐being
Authors: Jensen Rikke A.A. , Kirkegaard Thomsen Dorthe , O'Connor Maja , Mehlsen Mimi Y.
Published: June 2019

We examined whether age differences in life stories and personality traits mediated age differences in subjective well‐being. One hundred one young, 77 middle‐aged, and 81 older participants completed measures of subjective well‐being and personality traits. They described chapters and specific memories in their life stories and rated these on emotional tone and positive and negative self‐event connections. Older participants scored higher on subjective well‐being, rated their life stories as more positive, and scored lower on neuroticism compared with both young and middle‐aged participants. Age differences in subjective well‐being were mediated by life stories and neuroticism, with neuroticism being the strongest mediator. We suggest that changes in personality may enable older individuals to interpret events and themselves in a positive light, which help enhance their subjective well‐being.
High‐Throughput Scaffold System for Studying the Effect of Local Geometry and Topology on the Development and Orientation of Sprouting Blood Vessels
Authors: Szklanny Ariel A. , Debbi Lior , Merdler Uri , Neale Dylan , Muñiz Ayse , Kaplan Ben , Guo Shaowei , Lahann Joerg , Levenberg Shulamit
Published: June 2019

Live tissues require vascular networks for cell nourishing. Mimicking the complex structure of native vascular networks in vitro requires understanding the governing factors of early tubulogenesis. Current vascularization protocols allow for spontaneous formation of vascular networks; however, there is still a need to provide control over the defined network structure. Moreover, there is little understanding on sprouting decision and migration, especially within 3D environments. Here, tessellated polymer scaffolds with various compartment geometries and a novel two‐step seeding protocol are used to study vessel sprouting decisions. Endothelial cells first organize into hollow vessels tracing the shape contour with high fidelity. Subsequent sprouts emerge in specific directions, responding to compartment geometry. Time‐lapse imaging is used to track vessel migration, evidencing that sprouts frequently emerge from the side centers, mainly migrating toward opposing corners, where the density of support cells (SCs) is the highest, providing the highest levels of angiogenic factors. SCs distribution is quantified by smooth muscle actin expression, confirming the cells preference for curved compartment surfaces and corners. Displacements within the hydrogel correlate with SCs distribution during the initial tubulogenesis phase. This work provides new insight regarding vessel sprouting decisions that should be considered when designing scaffolds for vascularized engineered tissues.
A Dual‐Functional Photosensitizer for Ultraefficient Photodynamic Therapy and Synchronous Anticancer Efficacy Monitoring
Authors: Gao Yuting , Wang Xiuxia , He Xuewen , He Zhenyan , Yang Xiang , Tian Sidan , Meng Fanling , Ding Dan , Luo Liang , Tang Ben Zhong
Published: June 2019

A dual‐functional photosensitizer that demonstrates exceptional photodynamic therapy (PDT) efficacy while simultaneously self‐monitoring the therapeutic response in real time is reported here. Possessing an ultrahigh 1O2 quantum yield of 98.6% in water, the photosensitizer TPCI can efficiently induce cell death in a series of carcinoma cells (IC50 values less than 300 × 10−9 m) upon irradiation with an extremely low fluence (460 nm, 4 mW cm−2 for 10 min). In addition, TPCI can self‐monitor cell death in real time. It is weakly fluorescent in living cells before irradiation and lights up the nuclei concomitantly with cell death during PDT treatment by binding with chromatin to activate its aggregation‐induced emission, attributed to its strong binding affinity with DNA. In vivo studies using mouse models bearing H22 and B16F10 tumor cells validate the ultraefficient PDT efficacy of TPCI as well as the precise real‐time noninvasive readout of the tumor response from the beginning of cancer treatment. The dual‐functional TPCI serves as an excellent candidate for single‐agent photodynamic theranostics, and this work represents a new paradigm for the development of molecules with multiple intrinsic functions for future self‐reporting medical applications.
Spontaneous Phase Separation of Poly(3‐hexylthiophene)s with Different Regioregularity for a Stretchable Semiconducting Film
Authors: Gao Chun Yan , Pei Mingyuan , Choi Hyoung Jin , Yang Hoichang
Published: June 2019

Highly regioregular (RR) poly(3‐hexylthiophene)s PHTs are known to exhibit excellent electrical properties in comparison to chemically identical but regiorandom (rr) PHTs. In this study, distinct RR (97% and 55%)‐graded PHTs are subjected to solution blending to spontaneously separate the high‐RR PHT chains from the low‐RR PHT media and develop highly conjugated nanodomains in both solution and film. In the spun‐cast blend films, the rr PHT matrix imparts sufficient deformability of the channel layer required for stretchable organic thin‐film transistors (OTFTs), compared to neat RR PHTs and blends with a deformable polymer. OTFTs including RR PHT/rr PHT blend films show excellent hole mobility (µ) values up to 0.13 cm2 V−1 s−1, surpassing that of the best RR PHT films (0.026 cm2 V−1 s−1) fabricated by ultrasound solution pretreatment. Furthermore, a 50% stretched RR PHT/rr PHT film maintains ≈55% of its µ value at no strain, while RR PHT films show a sudden decrease in µ even at 10% stretch. The simple blending approach imparts deformability to π‐conjugated polymer films for application in stretchable OTFTs.
Clinical features and mutational analysis in 114 young children with Wilson disease from South China
Authors: Li Xiuzhen , Lu Zhikun , Lin Yunting , Lu Xinshuo , Xu Yi , Cheng Jing , Shao Yongxian , Su Xueying , Liu Zongcai , Sheng Huiying , Cai Yanna , Li Taolin , Zhou Zhizi , Tan Jingwen , Liu Hongsheng , Huang Yonglan , Liu Li , Zeng Chunhua
Published: June 2019

Wilson disease (WD) is a rare autosomal recessive disorder caused by mutations in the ATP7B gene. Clinical features and mutational analysis of Chinese children with WD at early age were rarely described. Herein, we retrospectively examined 114 children with WD at the mean of 5.9 years old age at diagnosis. Eight patients developed acute liver failure at mean age of 9.7 years old, 4 of whom died. Among the 114 patients, 86.0% were presymptomatic with isolated elevation of transaminases at diagnosis, 99.1% had decreased ceruloplasmin, and 68.4% had urinary copper excretion over 100 μg/24 hr. Bi‐allele pathogenic ATP7B mutations were identified in all patients. Among the 60 mutations detected, 10 were novel, including 7 missense mutations (p.I566N, p.T704I, p.C980F, p.G1030 V, p.A1096Q, p.L1327P, and p.L1373F), 1 nonsense mutation (p.K866X), 1 small insertion (p.Y44LfsX2), and 1 small deletion (p.R1118PfsX10). The most frequent mutations were p.R778L, p.P992L, and p.I1148T, which affected 27.2, 25.4, and 20.2% of the 114 WD children, respectively. The patients carrying p.R778L presented a higher rate of acute liver failure than the patients without p.R778L (9.7% vs. 4.8%). These results will be helpful in establishing early diagnosis of WD at the gene level, offering beneficial information for genetic counseling and providing clues to genotype/phenotype correlation of ATP7B mutations.
Sensitive detection of FGFR1 N546K mosaic mutation in patient with encephalocraniocutaneous lipomatosis and pilocytic astrocytoma
Authors: Kordacka Joanna , Zakrzewski Krzysztof , Gruszka Renata , Witusik‐Perkowska Monika , Taha Joanna , Sikorska Beata , Liberski Paweł P. , Zakrzewska Magdalena
Published: June 2019

Encephalocraniocutaneous lipomatosis (ECCL) is a rare neurocutaneous disorder, with only about 100 cases reported worldwide. It is characterized by congenital lesions of the eye, skin, and central nervous system. Only recently, potential causative FGFR1 point mutations have been identified in brain tumors and cultured skin biopsies from patients with this condition. Here, we analyzed the molecular status of a patient with ECCL and a coexisting pilocytic astrocytoma with detected FGFR1 N546K mutation. The presence of the alteration in both affected and unaffected tissues has been evaluated using Sanger sequencing and droplet digital polymerase chain reaction (ddPCR) technique. The ddPCR analysis showed differential distribution of the alteration in all specimens, including unaffected and untreated samples. Therefore, we confirm that FGFR1 N546K is a plausible causative mutation of ECCL patients and could be associated with a risk of brain tumor development. We also show the usefulness of sensitive ddPCR method for detection of low levels of autosomal mosaic mutation in blood or swabs. We suggest that utilization of this method may improve the diagnostic process, especially when targeted therapies are considered.
Severe Noonan syndrome phenotype associated with a germline Q71R MRAS variant: a recurrent substitution in RAS homologs in various cancers
Authors: Suzuki Hisato , Takenouchi Toshiki , Uehara Tomoko , Takasago Satoshi , Ihara Satoshi , Yoshihashi Hiroshi , Kosaki Kenjiro
Published: June 2019

Activation of the RAS pathway through either the activation of genes that accelerate the pathway or the suppression of genes that inhibit the pathway leads to a group of disorders collectively referred to as RASopathies. The key molecules of the RAS pathway are KRAS, HRAS, and NRAS. Mutations in these three RAS homolog genes have been shown to be associated with RASopathies. Recently, two patients with a Noonan syndrome phenotype were shown to carry mutations in the yet another RASopathy gene, MRAS (muscle RAS oncogene homolog). Here, we report a patient with a severe Noonan syndrome phenotype associated with a germline Q71R MRAS variant, which represents a recurrent substitution in RAS homologs in various cancers. The patient's dysmorphic features included relative macrocephaly, a down‐slanted palpebral fissure, hypertelorism, a depressed nasal bridge, and low‐set ears with thick lobes; these facial features are strongly associated with RASopathy. We confirmed that the MRAS gene represents a causative gene for RASopathy.
An Enigmatic Small Neosuchian Crocodyliform from the Woodbine Formation of Texas
Authors: Noto Christopher R. , Drumheller Stephanie K. , Adams Thomas L. , Turner Alan H.
Published: June 2019

New discoveries at the Arlington Archosaur Site (AAS), a Cenomanian (Late Cretaceous) locality in north‐central Texas, are filling gaps in our knowledge of mid‐Cretaceous Appalachian ecosystems, which remain poorly characterized. The AAS is notable because it preserves a diverse crocodyliform record. As seen in other sites that preserve four or more crocodyliform taxa, the species present at the AAS exhibit different snout shapes and body sizes, indicating that this high diversity of sympatric species was likely sustainable due to niche partitioning. Here we describe Scolomastax sahlsteini gen. et sp. nov., a new species of crocodyliform from the AAS, currently known from a partial right mandibular ramus. This species differs from other crocodyliforms in possessing features associated with durophagy or omnivory, including a shortened mandible, reduced tooth count, heterodonty, a dorsally expanded surangular, and enlarged attachments for jaw adductor muscles. Our phylogenetic analysis places this new taxon within Eusuchia as a member of Paralligatoridae and sister taxon to Paralligator gradilifrons. Scolomastax sahlsteini extends the record of paralligatorids into the Late Cretaceous of North America. This discovery represents the first appearance of this clade on the poorly known landmass of Appalachia, supporting a biogeographic connection between North America and Asia in the Early Cretaceous prior to completion of the Western Interior Seaway. However, relationships among other endemic crocodyliforms and tree instability within Paralligatoridae suggest further analysis is needed to resolve phylogenetic and biogeographic relationships (http://zoobank.org/urn:lsid:zoobank.org:pub:DC114471‐6687‐4BB5‐8FAE‐96F7278B1DAF). Anat Rec, 303:801–812, 2020. © 2019 Wiley Periodicals, Inc.
Fernando de Castro: Cajal's Man on the Peripheral Nervous System
Authors: Ros‐Bernal Francisco , de Castro Fernando
Published: June 2019

Santiago Ramón y Cajal developed his initial scientific career working alone. After the publication of his opus magna (“Textura del sistema nervioso del hombre y los vertebrados”) and the general recognition of the scientific environments that crystallized with the concession of the International Moscow Prize (1900), the Spanish Government decided to officially support Cajal with a laboratory and the first salaries to pay collaborators. Is then when the Spanish Neurological School births: in 1902, Francisco Tello is the first one to be incorporated. With new additions, Cajal's work is complimented in new aspects, including Neuropathologies. Fernando de Castro is one of his youngest direct disciples, one of the closest and more beloved. Fernando de Castro worked from 1916 in Cajal's lab, until the death of El Maestro. He was specially committed by Cajal to unravel different aspects of the structure of the peripheral ganglia: sensitive and vegetative. Afterward, Fernando de Castro described by first time the nature of arterial chemoreceptors in the carotid body. While trying to confirm his anatomical description with physiological demonstrations, and accumulating delays because of scientific decision and the sociopolitical circumstances in Spain, Corneille Heymans was awarded with the Nobel Prize in Physiology or Medicine 1938 for his contributions to the knowledge of cardiorespiratory reflexes. The Karolinska Institutet forgot Heinrich Hering and Fernando de Castro in their decision. Undoubtedly, Fernando de Castro was the most important disciple of Cajal working in the different structures of the peripheral nervous system, and this work is now reviewed here. Anat Rec, 303:1206–1214, 2020. © 2019 American Association for Anatomy
Layer‐by‐Layer Assembly: Recent Progress from Layered Assemblies to Layered Nanoarchitectonics
Authors: Ariga Katsuhiko , Ahn Eungjin , Park Minju , Kim Byeong‐Su
Published: June 2019

As an emerging concept for the development of new materials with nanoscale features, nanoarchitectonics has received significant recent attention. Among the various approaches that have been developed in this area, the fixed‐direction construction of functional materials, such as layered fabrication, offers a helpful starting point to demonstrate the huge potential of nanoarchitectonics. In particular, the combination of nanoarchitectonics with layer‐by‐layer (LbL) assembly and a large degree of freedom in component availability and technical applicability would offer significant benefits to the fabrication of functional materials. In this Minireview, recent progress in LbL assembly is briefly summarized. After introducing the basics of LbL assembly, recent advances in LbL research are discussed, categorized according to physical, chemical, and biological innovations, along with the fabrication of hierarchical structures. Examples of LbL assemblies with graphene oxide are also described to demonstrate the broad applicability of LbL assembly, even with a fixed material.
β‐Selective Glycosylation by Using O‐Aryl‐Protected Glycosyl Donors
Authors: Otsuka Yuji , Yamamoto Toshihiro , Fukase Koichi
Published: June 2019

New glycosyl donors have been developed that contained several para‐substituted O‐aryl protecting groups and their stereoselectivity for the glycosylation reaction was evaluated. A highly β‐selective glycosylation reaction was achieved by using thioglycosides that were protected by 4‐nitrophenyl (NP) groups, which were introduced by using the corresponding diaryliodonium triflate. Analysis of the stereoselectivities of several glycosyl donors indicated that the β‐glycosides were obtained through an SN2‐type displacement from the corresponding α‐glycosyl triflate. The NP group could be removed by reduction of the nitro group and acylation, followed by oxidation with ceric ammonium nitrate (CAN).
Outcome of patients aged 60‐75 years with newly diagnosed secondary acute myeloid leukemia: A single‐institution experience
Authors: Bertoli Sarah , Tavitian Suzanne , Bories Pierre , Luquet Isabelle , Delabesse Eric , Comont Thibault , Sarry Audrey , Huguet Françoise , Bérard Emilie , Récher Christian
Published: June 2019

A recent phase 3 trial showed that outcome of older patients with secondary acute myeloid leukemia (AML) may be improved by a liposomal encapsulation of cytarabine and daunorubicin (CPX‐351). This phase 3 study represents a unique example of prospective data in this rare subgroup providing basis for comparison with real life data. Here, we retrospectively assessed characteristics and outcome of patients aged 60‐75 years with secondary or therapy‐related AML in real life. Out of 218 patients that fulfilled CPX‐351 study criteria, 181 patients (83.0%) received antileukemic treatment either intensive chemotherapy (n = 121) or hypomethylating agents (HMA, n = 60). As compared with patients treated by chemotherapy, HMA‐treated patients were older, had lower WBC, more often AML with antecedent myelodysplastic syndrome and adverse cytogenetic risk. In chemotherapy‐treated patients, the complete response rate was 69%, median overall survival (OS) was 11 months whereas 3‐year and 5‐year OS was 21% and 17%, respectively. In HMA‐treated patients, the complete response rate was 15%, median OS was 11 months whereas 3‐year and 5‐year OS was 15% and 2%, respectively. In conclusion, although outcome of older patients with high‐risk AML is very poor, a significant proportion of patients treated by standard intensive chemotherapy but not HMA are long‐term survivors.
Anal adenocarcinoma: Treatment outcomes and trends in a rare disease entity
Authors: Wegner Rodney E. , White Richard J. , Hasan Shaakir , Raj Moses , Monga Dulabh , Finley Gene , Kirichenko Alexander V. , McCormick James
Published: June 2019

Importance

Primary Adenocarcinoma of the anus is a rare disease with a poor prognosis and thus tends to have a more aggressive treatment algorithm, typically involving a surgical approach. Prior to 2001, a few retrospective studies outlined improved outcomes with the incorporation of surgery with chemoradiation. However, since the publication of these studies, advancement in radiotherapy modalities and imaging have left the question of improved outcomes while reserving surgery for salvage.

Objective

We conducted this National Cancer Database (NCDB)‐driven retrospective study to analyze treatment trends and outcomes in the current time from 2004 to 2015 with respect to chemoradiation and surgery.

Design

Retrospective NCDB tumor registry data review—using propensity score‐adjusted multivariable analyses for survival.

Setting

Database review.

Participants

We selected for patients listed in the NCDB with AJCC stage 1‐3 anal adenocarcinoma diagnosed between 2004 and 2015 and selected out patients with undocumented/stage 4 disease, those with radiation outside the pelvis, not treated with systemic therapy and patients lost to follow‐up.

Exposure(s)

None.

Main outcomes and measures

Overall survival and use of surgery in the up‐front management of anal adenocarcinoma.

Results

Of the 1729 patients eligible in this study, 1028 were treated with surgery as up‐front management and 701 had definitive chemoradiation. Median overall survival for all patients was 55 months with a 5‐year survival rate of 55%. Patients treated without surgery had worse overall survival, median survival of 45 months compared to 87 months (P < 0.0001) with 5‐year survival rates of 42% and 55% in favor of incorporation of surgery. Analysis across patients treated with surgery alone, surgery followed by adjuvant chemoradiation, neoadjuvant chemoradiation followed by surgery, and chemoradiation alone had median survival rates of 78, 83, 92, and 46 months, respectively. Propensity score‐adjusted multivariable analysis identified older age, grade 3, high comorbidity score, and lack of surgery as predictive of worse outcome.

Conclusions and Relevance

The results of the NCDB analysis indicate improved overall survival with the incorporation of surgery into the initial management of anal adenocarcinoma when compared to chemoradiation alone, despite the omission of surgery in up to 50% of the cases logged. Our results corroborate earlier studies published prior to the year 2000 for surgery to be included in the definitive management of anal adenocarcinoma.
Second primary malignancies in postpolycythemia vera and postessential thrombocythemia myelofibrosis: A study on 2233 patients
Authors: Mora Barbara , Rumi Elisa , Guglielmelli Paola , Barraco Daniela , Maffioli Margherita , Rambaldi Alessandro , Caramella Marianna , Komrokji Rami , Gotlib Jason , Kiladjian Jean Jacques , Cervantes Francisco , Devos Timothy , Palandri Francesca , De Stefano Valerio , Ruggeri Marco , Silver Richard T. , Benevolo Giulia , Albano Francesco , Cavalloni Chiara , Pietra Daniela , Barbui Tiziano , Rotunno Giada , Cazzola Mario , Vannucchi Alessandro Maria , Giorgino Toni , Passamonti Francesco
Published: June 2019

Patients with myeloproliferative neoplasms (MPN) are known to have higher incidence of nonhematological second primary malignancies (SPM) compared to general population. In the MYSEC study on 781 secondary myelofibrosis (SMF) patients, the incidence of SPM after SMF diagnosis resulted 0.98/100 patient‐years. When including non‐melanoma skin cancers (NMSC), the incidence arose to 1.56/100 patient‐years. In SMF, JAK inhibitor treatment was associated only with NMSC occurrence. Then, we merged the MYSEC cohort with a large dataset of PV and ET not evolving into SMF. In this subanalysis, we did not find any correlation between SPM and SMF occurrence. These findings highlight the need of studies aimed at identifying MPN patients at higher risk of SPM.
GDF15 induced apoptosis and cytotoxicity in A549 cells depends on TGFBR2 expression
Authors: Tarfiei Ghorban Ali , Shadboorestan Amir , Montazeri Hamed , Rahmanian Narges , Tavosi Gholamreza , Ghahremani Mohammad Hossein
Published: June 2019

GDF15 plays a paradoxical role during carcinogenesis; it inhibits tumour growth in the early stages and promotes tumour cell proliferation in the late stages of cancer. Besides, GDF15 can induce apoptosis in some cancer cells including A549 but not in some others. Moreover, as a potential receptor for GDF15, TGFBR2 is inactivated during carcinogenesis in many types of cancers, and it is not present in cells with no GDF15 induced apoptosis. Thus, we tested whether GDF15 overexpression and/or TGFBR2 silencing can affect the GDF15 induced apoptosis in A549 cells. The full and mature forms of GDF15 were cloned and overexpressed in A549 cells. The TGFBR2 was silenced using specific siRNA and confirmed by real‐time PCR. Results indicated that overexpression of full and mature forms of GDF15 as well as TGFBR2 knocked down reduced A549 cell viability in 24 and 48 hours. Flow cytometric analysis of annexin V/PI indicated induction of apoptosis in A549 cells by overexpression of GDF15 or silencing TGFBR2. Interestingly, the silencing of TGFBR2 inhibited the GDF15 induced cytotoxicity and apoptosis in A549 cells. Overexpression of GDF15 activated caspase‐9 and caspase‐3 and inhibited ERK1/2 and p38 phosphorylation in A549 cells. TGFBR2 knocked down inhibited GDF15 effects on caspases, ERK1/2, and p38MAPK activation. Our results indicated that the effect of GDF15 on apoptosis and activation of MAPK in A549 cells depends on TGFBR2 expression. These findings may point to mechanisms in which GDF15 exerts dual effect during carcinogenesis with regard to TGFBR2 expression.

Significance of the study

GDF15 plays a tumour suppressor or promotor roles during carcinogenesis. The expression of GDF15 induced cytotoxicity, apoptosis, and inhibition of MAPK in A549 cells. All these effects were blocked by silencing TGFBR2 expression. These findings may point to mechanisms in which GDF15 exerts dual effect during carcinogenesis with regard to TGFBR2 expression.
Isobutene from Ethanol: Describing the Synergy between In2O3 and m‐ZrO2
Authors: Zonetti Priscila C. , Bridi Vivian L. , Gonzalez Guilherme G. , Moreira Carla R. , Alves Odivaldo C. , de Avillez Roberto R. , Appel Lucia G.
Published: June 2019

The performances of In2O3/ZrO2, ZrO2 and In2O3 were evaluated in the synthesis of isobutene from ethanol in one‐step. These catalysts were characterized by XRD, EPR, TPD‐H2O, TPD‐NH3, TPD‐ethanol and pyridine adsorption. The In2O3/ZrO2 catalyst is a promising catalytic system for the synthesis of isobutene from ethanol. The catalytic tests showed that acetone, hydrogen and carbon dioxide are the main co‐products of this synthesis. The In2O3/ZrO2 catalyst can promote the synthesis of isobutene due to the inter‐diffusion process between In2O3 and ZrO2. This phenomenon generates, among others, the Zr insertion and a high concentration of anionic vacancies in the In2O3 lattice. This increases the In2O3/ZrO2 redox properties leading to a high activity of this catalyst for the acetone generation, a key intermediate of the isobutene synthesis. These changes also promote the acid and basic properties of In2O3/ZrO2, which are in charge of the acetaldehyde synthesis, acetone condensation and, finally, dehydration of the intermediates of the isobutene synthesis.
Self‐Focused Attention and Career Anxiety: The Mediating Role of Career Adaptability
Authors: Shin Yun‐Jeong , Lee Ji‐Yeon
Published: June 2019

The purpose of this study was to examine how 2 forms of self‐focused attention, self‐reflection and self‐rumination, influence career anxiety. The authors hypothesized that the association between these 2 types of self‐focused attention and career anxiety would be mediated by career adaptability on the basis of a career construction model of adaptation. The participants were 326 undergraduate students in South Korea. The results of this study supported the hypothesized mediation model by indicating significant indirect effects of self‐reflection and self‐rumination on career anxiety via career adaptability. The direct effect of self‐rumination on career anxiety was significant (B = .44, p < .01), but the direct effect of self‐reflection on career anxiety was not significant (B = –.05, p > .05). The authors discuss implications for counselors to help college students manage career anxiety by encouraging and supporting increased career adaptability. Future research to examine the cross‐cultural validity of the current findings is needed.
Construction and Initial Validation of the Career Maximizing Scale
Authors: Voss Nathaniel M. , Lake Christopher J. , Chlevin‐Thiele Cassandra
Published: June 2019

Applying the concept of maximizing—careful evaluation of options in pursuit of optimal goals—to career decisions, the authors developed the Career Maximizing Scale (CMS). The measure was administered to samples of working adults and university students across 3 studies. Factor analysis indicated that the measure is unidimensional and has favorable psychometric properties. Career maximizing was related to but distinct from general maximizing. Career maximizing was positively related to indicators of decision confidence (e.g., career decision‐making self‐efficacy) and positively related to desirable career outcomes (e.g., career satisfaction). Career maximizing was also modestly related to certain desirable academic outcomes (e.g., commitment to university major). Use of the CMS may facilitate effective career counseling.