bluuebunny/crossref_metadata_2025 huggingface dataset:
Triple A Syndrome: Preliminary Response to the Antioxidant N-Acetylcysteine Treatment in a Child
Authors: Fragoso Maria Candida Barisson Villares , Albuquerque Edoarda Vasco de Albuquerque , Cardoso Ana Luiza de Almeida , da Rosa Paula Waki Lopes , de Paulo Rodrigo Bomeny , Schimizu Maria Heloisa Massola , Seguro Antonio Carlos , Passarelli Marisa , Koehler Katrin , Huebner Angela , Almeida Madson Q. , Latronico Ana Claudia , Arnhold Ivo Jorge Prado , Mendonca Berenice Bilharinho
Published: April 2017

Introduction: Triple A syndrome (AAAS) is a rare autosomal recessive disorder characterized by alacrima, achalasia, ACTH-resistant adrenal insufficiency, autonomic dysfunction, and progressive neurodegeneration. Increased oxidative stress, demonstrated in patients’ fibroblasts in vitro, may be a central disease mechanism. N-acetylcysteine protects renal function in patients with kidney injuries associated with increased oxidative stress and improves viability of AAAS-knockdown adrenal cells in vitro. Patient and Results: A boy diagnosed with AAAS presented with short stature and increased oxidative stress in vivo assessed by increased thiobarbituric acid reactive substances (TBARS), which are markers of lipid peroxidation, and by the susceptibility of LDL to oxidation and the capacity of HDL to prevent it. A homozygous missense germline mutation (c.523G>T, p.Val175Phe) in AAAS was identified. N-acetylcysteine (600 mg orally, twice daily) decreased oxidative stress but did not change the patient’s growth pattern. Conclusions: An increase in oxidative stress is reported for the first time in vivo in an AAAS patient. N-acetylcysteine was capable of decreasing TBARS levels, reducing the susceptibility of LDL to oxidation and improving the antioxidant role of HDL. The long-term effect of antioxidant treatment should be evaluated to determine the real benefit for the prevention of the degenerative process in AAAS.
The Spectrum of Ovotesticular Disorders of Sex Development in South Africa: A Single-Centre Experience
Authors: Ganie Yasmeen , Aldous Colleen , Balakrishna Yusentha , Wiersma Rinus
Published: April 2017

Objective: To describe the clinical characteristics, biochemistry, histopathology, and long-term outcomes in subjects with ovotesticular (OT) disorder of sex development (DSD). Study Design: This is a retrospective subset analysis of 64 cases of histologically confirmed OT DSD. Results: All subjects were South African; 97% (n = 62) were African and 92% (n = 59) were of Zulu ethnicity. The most common karyotype was 46,XX (88%; n = 56), followed by 46,XY (8%), 46,XY/45,X (3%), and 46,XX/46,XY (1%). The median age at presentation was 7 months (0.5 months to 5.1 years). Sixty-one of the subjects (95%) presented with DSD. The ovotestis was the most frequent gonad (56%), followed by the ovary (23%) and the testis (16%). Testes were more commonly located on the right and ovaries on the left (p < 0.0001). The male gender was the predominant sex of rearing in two-thirds of the subjects. Gender dysphoria was noted in 8 subjects (11%) at a median of 6.4 (4.3–9.3) years. Long-term follow-up (n = 14) revealed spontaneous puberty in 5 subjects, gender dysphoria in 2 subjects, and neuropsychiatric disorders in 4 subjects. Conclusion: OT DSD is an important differential diagnosis in Black South Africans with 46,XX DSD.
Distribution of Interstitial Telomeric Sequences in Primates and the Pygmy Tree Shrew (Scandentia)
Authors: Mazzoleni Sofia , Schillaci Odessa , Sineo Luca , Dumas Francesca
Published: April 2017

It has been hypothesized that interstitial telomeric sequences (ITSs), i.e., repeated telomeric DNA sequences found at intrachromosomal sites in many vertebrates, could be correlated to chromosomal rearrangements and plasticity. To test this hypothesis, we hybridized a telomeric PNA probe through FISH on representative species of 2 primate infraorders, Strepsirrhini (Lemur catta, Otolemur garnettii, Nycticebus coucang) and Catarrhini (Erythrocebus patas, Cercopithecus petaurista, Chlorocebus aethiops, Colobus guereza), as well as on 1 species of the order Scandentia, Tupaia minor, used as an outgroup for primates in phylogenetic reconstructions. In almost all primate species analyzed, we found a telomeric pattern only. In Tupaia, the hybridization revealed many bright ITSs on at least 11 chromosome pairs, both biarmed and acrocentric. These ITS signals in Tupaia correspond to fusion points of ancestral human syntenic associations, but are also present in other chromosomes showing synteny to only a single human chromosome. This distribution pattern was compared to that of the heterochromatin regions detected through sequential C-banding performed after FISH. Our results in the analyzed species, compared with literature data on ITSs in primates, allowed us to discuss different mechanisms responsible for the origin and distribution of ITSs, supporting the correlation between rearrangements and ITSs.
Innovative Treatment Concepts for Cutaneous T-Cell Lymphoma Based on Microenvironment Modulation
Authors: Leuchte Katharina , Schlaak Max , Stadler Rudolf , Theurich Sebastian , von Bergwelt-Baildon Michael
Published: April 2017

Cutaneous T-cell lymphomas (CTCL) are a rare and biologically heterogeneous malignant entity comprising mycosis fungoides and Sézary syndrome as the most common subtypes. The current treatment outcome is characterized by high rates of relapse, but survival is usually not significantly shortened in low-stage disease. This is different in tumor-stage disease or aggressive CTCL subtypes where survival is significantly reduced. Recent advances have been made on several levels of tumor biology: Whole genome sequencing has resulted in novel strategies of NF-κB or JAK inhibition, ongoing translational research has revealed new targets like CD30 or CCR4, and, finally, research focusing on impaired immunosurveillance has gained more importance. Based on the growing knowledge about the functional roles of the tumor microenvironment and non-malignant infiltrating cells, current research aims to develop novel CTCL treatment strategies. This review focuses on the mounting evidence for efficiently targeting tumor-infiltrating immune cells and thereby modulating the tumor microenvironment and restoring immunosurveillance.
Uso de contraceptivos e fatores associados entre adolescentes de 15 a 18 anos de idade em Unidade de Saúde da Família
Authors: Zanini Marcela , Selvante Joana D. S. , Quagliato Fabio Franchi
Published: April 2017

O início da atividade sexual em mulheres jovens pode causar uma série de consequências devido ao uso inapropriado ou à falta de acesso aos métodos anticoncepcionais. Considerando aquelas que fazem uso de contraceptivos, em muitos casos o uso é incorreto ou inconsistente. O objetivo deste estudo é avaliar o conhecimento das mulheres de 15 a 18 anos sobre diferentes métodos contraceptivos, por meio de questionário. 25 mulheres foram entrevistadas e 55,52% relataram ter sido sexualmente ativas, das quais 44% relataram que usam regularmente algum tipo de método contraceptivo. O preservativo masculino é o método mais conhecido (84%) em todas as idades. A fonte de informação mais citada em relação a cada idade foi a família (15 e 16 anos), a escola (17 anos) e profissionais de saúde (18 anos). 36% das entrevistadas admitiram nunca ter ido a uma consulta com um ginecologista ou o médico de família. Concluímos que, mesmo tendo acesso a diversos meios de informação, as jovens não adotam uma vida sexual segura, deixando de usar ou usando de forma inadequada os diversos métodos anticoncepcionais disponíveis.
Análise temporal do aleitamento materno em Unidade Básica de Saúde de Pelotas, Brasil
Authors: Dupont Erick , Dietrich Martina Vitória Flach , Frizzo Rafael Augusto , Ribeiro Leonardo Elpídio , Vitória Ângela Moreira
Published: April 2017

O conjunto de ações de atenção à saúde da criança implementado no eixo da assistência básica integral foi uma das determinantes da redução da mortalidade infantil no Brasil. Uma dessas ações foi o estímulo ao aleitamento materno que foi relacionado à prevenção de diversas doenças em lactantes e crianças. Por esse motivo, foi realizada uma pesquisa com método transversal descritivo com utilização de dados secundários em UBS no município de Pelotas – RS, com o objetivo de analisar temporalmente o aleitamento materno nos três primeiros meses de vida das crianças usuárias do serviço. Os dados foram obtidos das fichas espelho de puericultura da unidade e posteriormente agrupados em tabela confeccionada em Microsoft Excel 2013. A amostra foi composta por 246 crianças, nascidas entre 2011 e 2015. Não foi possível evidenciar um padrão temporal de melhora ou piora nos índices de aleitamento, uma vez que se verificou a inadequação de registros, contudo, inferiu-se que nos períodos de melhor registro, o percentual de aleitamento materno exclusivo também apresentou maiores valores. A média de aleitamento materno encontrada no período foi consonante com outros estudos realizados na Bahia, Florianópolis e Itapira (SP). No entanto, tais resultados ainda estão aquém do preconizado pela OMS, sendo verificada a necessidade de se instituir medidas que estimulem o aleitamento materno, além da realização de um melhor controle e avaliação dos serviços de saúde prestados pelo SUS na atenção primária.
Pregnancy-induced hypertension: a review about management
Authors: Rocha Karina Silva , Farias Tayná Fregoneze , Silvestre Alessandro , Pereira Maria Monica
Published: April 2017

Introdução: Estima-se que os distúrbios hipertensivos na gravidez afetam 6-8% das gestantes nos Estados Unidos e são considerados um dos principais fatores de mortalidade materna. No Brasil, de acordo com o DATASUS, se considerarmos as categorias O13 a O16 do CID-10, os distúrbios hipertensivos representaram cerca de 20,22% das mortes maternas no período de 2004 a 2014. Objetivos: O objetivo da revisão é demonstrar os tratamentos mais atuais para o tratamento de distúrbios hipertensivos durante a gravidez. Métodos: Foram consultadas as bases de dados Medline/Pubmed, LILACS/SciELO, Cochrane e Scopus, procurando artigos nacionais e internacionais publicados entre 2006 e 2016, em inglês e português, bem como os consensos estabelecidos pela Organização Mundial da Saúde e as estatísticas do DATASUS. Resultados: As medidas recomendadas para prevenir a pré-eclâmpsia (PE) foram a suplementação de cálcio para gestantes com baixa ingestão dietética e a administração de baixas doses de aspirina. Na PE, se acontecer no termo, o tratamento é induzir o parto, mas se ocorrer pré-termo, é monitorar, administrar sulfato de magnésio (SM), anti-hipertensivos e corticóide. Em caso de eclâmpsia, a SM é muito eficaz para reduzir as convulsões eclâmpticas. Os fármacos utilizados no tratamento da hipertensão crônica grave são metildopa em associação ou não, com nifedipina ou hidralazina. A crise hipertensiva aguda é tratada com fármacos de primeira linha, como nifedipina e hidralazina, e alternativamente com nitroprussiato de sódio. Conclusão: Os principais protocolos mundiais seguem basicamente a mesma orientação, mudando apenas detalhes específicos de conduta de acordo com os recursos do sistema de saúde de cada país. O controle da pressão arterial materna apresenta resultados satisfatórios para o binômio materno-fetal, pois reduz o risco de PE e eclâmpsia. O tratamento definitivo para PE e eclâmpsia é a indução de parto, sendo utilizado apenas medidas terapêuticas para controle, já que não têm cura.
Mechanisms of subcutaneous adipose tissue formation in experimental lymphedema model
Authors: Cuadrado Guilherme de Arruda , Ariga Suely K. , Andrade Mauro F. C. de , Souza Heraldo P. de
Published: April 2017

no abstract
Minerais do grupo da crichtonita em veios de quartzo da Serra do Espinhaço (Minas Gerais e Bahia)
Authors: Chaves Mario Luiz de Sá Carneiro , Menezes Filho Luiz Alberto Dias
Published: April 2017

O trabalho descreve quatro minerais do grupo da crichtonita encontrados em veios hidrotermais de quartzo, que cortam diversas formações geológicas da Serra do Espinhaço (Supergrupo Espinhaço). Esses minerais – senaíta, crichtonita, almeidaíta e gramaccioliíta-(Y) – foram coletados em duas regiões: Presidente Kubitschek, ao sul de Diamantina (MG), e Novo Horizonte, na Chapada Diamantina Ocidental (BA). A idade de tais veios é relacionada ao final do Ciclo Brasiliano, em ca. 490 Ma. A senaíta é um mineral descoberto no Brasil e classicamente identificado nos aluviões diamantíferos da região de Diamantina; sua primeira ocorrência primária é aqui descrita. A crichtonita, apesar de conhecida desde longa data, teve seu primeiro depósito no país reportado nessa mesma região. A almeidaíta é o 61º e um dos mais recentes dos “minerais brasileiros”, sendo encontrada na região de Novo Horizonte. A gramaccioliíta-(Y), uma espécie descoberta na última década (Itália), tem sua primeira ocorrência brasileira descrita também nessa região. Os depósitos e as composições químicas desses minerais são caracterizados, bem como as problemáticas envolvidas até suas definições finais.
Associations Between Left Ventricular Dysfunction and Brain Structure and Function: Findings From the SABRE (Southall and Brent Revisited) Study
Authors: Park Chloe M. , Williams Emily D. , Chaturvedi Nish , Tillin Therese , Stewart Robert J. , Richards Marcus , Shibata Dean , Mayet Jamil , Hughes Alun D.
Published: April 2017

Background

Subclinical left ventricular (
LV
) dysfunction has been inconsistently associated with early cognitive impairment, and mechanistic pathways have been poorly considered. We investigated the cross‐sectional relationship between
LV
dysfunction and structural/functional measures of the brain and explored the role of potential mechanisms.

Method and Results

A total of 1338 individuals (69±6 years) from the Southall and Brent Revisited study underwent echocardiography for systolic (tissue Doppler imaging peak systolic wave) and diastolic (left atrial diameter) assessment. Cognitive function was assessed and total and hippocampal brain volumes were measured by magnetic resonance imaging. Global
LV
function was assessed by circulating N‐terminal pro–brain natriuretic peptide. The role of potential mechanistic pathways of arterial stiffness, atherosclerosis, microvascular disease, and inflammation were explored. After adjusting for age, sex, and ethnicity, lower systolic function was associated with lower total brain (beta±standard error, 14.9±3.2 cm
3
;
P
<0.0001) and hippocampal volumes (0.05±0.02 cm
3
,
P
=0.01). Reduced diastolic function was associated with poorer working memory (−0.21±0.07,
P
=0.004) and fluency scores (−0.18±0.08,
P
=0.02). Reduced global
LV
function was associated with smaller hippocampal volume (−0.10±0.03 cm
3
,
P
=0.004) and adverse visual memory (−0.076±0.03,
P
=0.02) and processing speed (0.063±0.02,
P
=0.006) scores. Separate adjustment for concomitant cardiovascular risk factors attenuated associations with hippocampal volume and fluency only. Further adjustment for the alternative pathways of microvascular disease or arterial stiffness attenuated the relationship between global
LV
function and visual memory.

Conclusions

In a community‐based sample of older people, measures of
LV
function were associated with structural/functional measures of the brain. These associations were not wholly explained by concomitant risk factors or potential mechanistic pathways.
Argumentation, Impact, and Normative Change: Responsibility to Protect after the Commission of Inquiry Report into Human Rights in North Korea
Authors: Kim Jawoon , Bloomfield Alan
Published: April 2017

In 2014 a unhrc report found North Korea was practicing mass atrocities, contravening the Responsibility to Protect (R2P) norm, and it called for sanctions and referral of the matter to the International Criminal Court. This paper assesses the report’s impact upon ‘the matter of human rights in North Korea’ and upon the R2P norm itself. We find that when a relatively ‘demanding’ standard is applied, whereby R2P is judged to be effective only if it affects the actual human rights situation, the report has had little impact. But judging impact according to a more ‘forgiving’ standard suggests the report has substantially affected international debates about human rights in North Korea. We also argue that the report affected the R2P norm itself: specifically, Pillars i and ii are now considered relevant in cases of ‘chronic’ abuse, although strong opposition to the position that Pillar iii measures are also relevant remains strong.
Treatises on the Salvation of Abū Ṭālib
Authors: Husayn Nebil A.
Published: April 2017

The following article surveys a few treatises regarding the salvation of the Prophet Muḥammad’s uncle, Abū Ṭālib b. ʿAbd al-Muṭṭalib (d. circa 619ce). The controversy concerning Abū Ṭālib’s place in the hereafter stems from a wealth of reports condemning him to hell due to his refusal to convert to Islam and others which testify to his lifelong belief in God and the prophethood of Muḥammad. The first group of reports was canonized in the collections of Bukhārī and Muslim, while the second group largely appeared insīraand Shīʿīḥadīthliterature. Although Shīʿī thinkers have upheld the faith and salvation of Abū Ṭālib from the earliest periods of Islamic history, very few Sunnīs shared this opinion despite transmitting some of the same proof-texts cited in Shīʿī works. According to most Sunnīs, these proof-texts were either inconclusive or insufficient in proving Abū Ṭālib’s conversion to Islam or his salvation. However, there is a remarkable shift in the sensibilities of some Sunnīs after the ninth centuryhijrī(fifteenth centuryce). In contrast to early Sunnīs who considered such a possibility to be unlikely or flatly denied it, a few Sunnīs over the past five centuries have joined their Shīʿī co-religionists in their commitment to the salvation of Abū Ṭālib. This article introduces the relevant proof-texts and theological arguments that classical Shīʿī and modern Sunnī writers have utilized to advocate the belief in Abū Ṭālib’s salvation.
Philosophical Theology among Sixth/Twelfth-Century Twelver Shīʿites: From Naṣīr al-Dīn al-Ṭūsī (d. after 599/1201-2 or 600/1202-3) to Naṣīr al-Dīn al-Ṭūsī (d. 672/1274)
Authors: Ansari Hassan , Schmidtke Sabine
Published: April 2017

msYale, Landberg 510, a multitext volume, contains unique copies of two doctrinal works composed as it seems by an Imāmī scholar of the sixth/twelfth century, ʿAbd Allāh b. Ḥamza b. ʿAbd Allah b. Ḥamza al-Ṭūsī al-Mashhadī al-Shāriḥīal-maʿrūf bi-Naṣīr al-Dīn al-Ṭūsī,al-Wāfī bi-kalām al-muthbit wa-l-nāfīandal-Masʾala fī tanzīh dhāt al-khāliq ʿan al-talawwuth bi-waṣmat al-fanāʾ wa-l-ʿadam. Both tracts show the author’s close familiarity with the doctrinal systems of the followers of Abū Hāshim al-Jubbāʾī (d. 321/933) on the one hand and Abū l-Ḥusayn al-Baṣrī (d. 436/1044) on the other. The discussions revolve around the contested Bahshamite notion that the “non-existent” (maʿdūm) is a “thing” (shayʾ), as well as the related doctrine that essences (dhawāt, sg.dhāt) are “real” or “actual” (thābit) in the state of non-existence (ʿadam). The paper introduces the author and the two tracts and provides critical editions.
Speolabeo, a new genus name for the cave fish Bangana musaei (Teleostei: Cyprinidae)
Authors: KOTTELAT MAURICE
Published: April 2017

Speolabeo, new genus, is established for Bangana musaei, a fish from caves in the Khammouan Karst in Laos. It was originally tentatively placed in the genus Bangana s.l. Besides characters related to hypogean life (absence of eyes, absence of pigment resulting in a whitish body), it is distinguished from all species of Bangana s.l. in having only 7–8½ branched dorsal-fin rays, the pelvic-fin at the vertical between the first unbranched and first branched dorsal-fin rays, and details of mouth morphology.
The Dependence of the Predictability of Mesoscale Convective Systems on the Horizontal Scale and Amplitude of Initial Errors in Idealized Simulations
Authors: Weyn Jonathan A. , Durran Dale R.
Published: April 2017

Recent work has suggested that modest initial relative errors on scales of O(100) km in a numerical weather forecast may exert more control on the predictability of mesoscale convective systems at lead times beyond about 5 h than 100% relative errors at smaller scales. Using an idealized model, the predictability of deep convection organized by several different profiles of environmental vertical wind shear is investigated as a function of the horizontal scale and amplitude of initial errors in the low-level moisture field. Small- and large-scale initial errors are found to have virtually identical impacts on predictability at lead times of 4–5 h for all wind shear profiles. Both small- and large-scale errors grow primarily up in amplitude at all scales rather than through an upscale cascade between adjacent scales. Reducing the amplitude of the initial errors improves predictability lead times, but this improvement diminishes with further reductions in the error amplitude, suggesting a limit to the intrinsic predictability in these simulations of slightly more than 6 h at scales less than 20 km. Additionally, all the simulated convective systems produce a k−5/3 spectrum of kinetic energy, providing evidence of the importance of the unbalanced, divergent gravity wave component of the flow produced by thunderstorms in generating the observed atmospheric kinetic energy spectrum.
In Search of Fingerprints of the Recent Intensification of the Ocean Water Cycle
Authors: Vinogradova Nadya T. , Ponte Rui M.
Published: April 2017

Unprecedented changes in Earth’s water budget and a recent boom in salinity observations prompted the use of long-term salinity trends to fingerprint the amount of freshwater entering and leaving the oceans (the ocean water cycle). Here changes in the ocean water cycle in the past two decades are examined to evaluate whether the rain-gauge notion can be extended to shorter time scales. Using a novel framework it is demonstrated that there have been persistent changes (defined as significant trends) in both salinity and the ocean water cycle in many ocean regions, including the subtropical gyres in both hemispheres, low latitudes of the tropical Pacific, the North Atlantic Subpolar Gyre, and the Arctic Ocean. On average, the ocean water cycle has amplified by approximately 5% since 1993, but strong regional variations exist (as well as dependency on the surface freshwater flux products chosen). Despite an intensified ocean water cycle in the last two decades, changes in surface salinity do not follow expected patterns of amplified salinity contrasts, challenging the perception that if it rains more the seas always get fresher and if it evaporates more the seas always get saltier. These findings imply a time of emergence of anthropogenic hydrological signals shorter in surface freshwater fluxes than in surface salinity and point to the importance of ocean circulation, salt transports, and natural climate variability in shaping patterns of decadal change in surface salinity. Therefore, the use of salinity measurements in conjunction with ocean salt fluxes can provide a more meaningful way of fingerprinting changes in the global water cycle on decadal time scales.
Evaluation of Precipitable Water Vapor from Four Satellite Products and Four Reanalysis Datasets against GPS Measurements on the Southern Tibetan Plateau
Authors: Wang Yan , Yang Kun , Pan Zhengyang , Qin Jun , Chen Deliang , Lin Changgui , Chen Yingying , Lazhu  , Tang Wenjun , Han Menglei , Lu Ning , Wu Hui
Published: April 2017

The southern Tibetan Plateau (STP) is the region in which water vapor passes from South Asia into the Tibetan Plateau (TP). The accuracy of precipitable water vapor (PWV) modeling for this region depends strongly on the quality of the available estimates of water vapor advection and the parameterization of land evaporation models. While climate simulation is frequently improved by assimilating relevant satellite and reanalysis products, this requires an understanding of the accuracy of these products. In this study, PWV data from MODIS infrared and near-infrared measurements, AIRS Level-2 and Level-3, MERRA, ERA-Interim, JRA-55, and NCEP final reanalysis (NCEP-Final) are evaluated against ground-based GPS measurements at nine stations over the STP, which covers the summer monsoon season from 2007 to 2013. The MODIS infrared product is shown to underestimate water vapor levels by more than 20% (1.84 mm), while the MODIS near-infrared product overestimates them by over 40% (3.52 mm). The AIRS PWV product appears to be most useful for constructing high-resolution and high-quality PWV datasets over the TP; particularly the AIRS Level-2 product has a relatively low bias (0.48 mm) and RMSE (1.83 mm) and correlates strongly with the GPS measurements (R = 0.90). The four reanalysis datasets exhibit similar performance in terms of their correlation coefficients (R = 0.87–0.90), bias (0.72–1.49 mm), and RMSE (2.19–2.35 mm). The key finding is that all the reanalyses have positive biases along the PWV seasonal cycle, which is linked to the well-known wet bias over the TP of current climate models.
Effect of Mechanical Ventilation on the Thermal Offset of Pyranometers during Cloud-Free Summer Conditions
Authors: Sanchez G. , Serrano A. , Cancillo M. L.
Published: April 2017

Thermal offset is a significant source of uncertainty for solar radiation measurements. This study assesses the influence of mechanical ventilation on the daytime thermal offset of pyranometers. Toward this goal, an intensive unprecedented campaign of measurements was conducted in Badajoz, Spain, during four selected summer days under cloud-free conditions, covering a large range of solar zenith angle, irradiance, and temperature. Three leading manufacturers participated in the campaign, providing secondary standard pyranometers and compatible ventilation units. The thermal offset was experimentally measured following the capping methodology. A total of 372 capping events were conducted, the largest number ever reported in the literature. Each pyranometer was tested under different operational conditions (with/without ventilation and measuring global/diffuse irradiance). Results show that mechanical ventilation generally reduces the thermal offset. The magnitude of this reduction is different for each pyranometer model and depends on whether the instrument is shadowed (for measuring diffuse irradiance) or not (for measuring global irradiance). Mechanical ventilation tends to homogenize the temperature around the pyranometer and therefore reduces the impact of environmental conditions on the thermal offset. CMP11 and SPP pyranometers show notable tendencies in the thermal offset even when mechanical ventilation is applied. The Dutton et al. model, which aimed to correct the daytime thermal offset, is evaluated. Results show this model performs well for the SPP pyranometer but underestimates the absolute value of thermal offset for the CMP11 and SR20 pyranometers.
How School and District Leaders Access, Perceive, and Use Research
Authors: Penuel William R. , Briggs Derek C. , Davidson Kristen L. , Herlihy Corinne , Sherer David , Hill Heather C. , Farrell Caitlin , Allen Anna-Ruth
Published: April 2017

This study examined how school and district leaders access, value, and use research. From a representative sample of school districts across the United States, we surveyed 733 school and district leaders as part of an effort to develop understanding of the prevalence of research use, the nature of leaders’ attitudes toward research, and individual and organizational correlates of research use. School and district leaders alike reported frequent use of research use and generally positive attitudes toward research. Leaders reported accessing research primarily through their professional networks. Those in certain roles, those pursuing or holding an advanced degree, and those who reported a strong organizational culture of evidence use reported higher levels of research use. These findings suggest that policy efforts to promote evidence use among education leaders will be welcomed but that policy makers need to take into account the prevalence of various types of research use in designing supports for evidence use.
Imaging Features of Renal Capsule Leiomyoma on Contrast-Enhanced Ultrasonography: A Case Report
Authors: Moura Neto José Andrade , Perpétuo de Souza Ana Flavia , Rocco Suassuna José Hermógenes , Aguiar Vaz Luiz Carlos , Araújo Nordeval Cavalcante
Published: April 2017

Leiomyomas of the kidney are rare, mostly occurring in adults as incidental findings, and are a diagnostic challenge. In a renal transplant recipient population, an increased risk of tumors is observed due to the effect of immunosuppressive drugs. A differential diagnosis between leiomyomas and other malignant lesions is not possible with current imaging methods. This report presents a case of a native kidney capsular leiomyoma in a kidney transplant recipient and highlights the features of computed tomography and contrast-enhanced ultrasound (CEUS), along with the histopathologic analysis. The CEUS and quantification studies are helpful in demonstrating the vascularization pattern of the renal capsular leiomyoma. This entity appearing with chronic kidney disease can resemble a renal cell carcinoma, in an otherwise healthy kidney. In this case, CEUS was useful for diagnosing the vascularization of the renal mass, although the pattern of intense vascularization could be diagnostically misleading, implying a malignant lesion. However, the pattern of enhancement due to CEUS showed no peculiarities that indicated histopathologic diagnosis.
Serologic characterization of anti-protamine/heparin and anti-PF4/heparin antibodies
Authors: Lee Grace M. , Joglekar Manali , Kuchibhatla Maragatha , Khandelwal Sanjay , Qi Rui , Rauova Lubica , Arepally Gowthami M.
Published: April 2017

Key Points

Monoclonal and polyclonal anti-PRT/heparin antibodies are serologically distinct from anti-PF4/heparin antibodies. Binding of anti-PRT/heparin antibodies to PRT/dextran complexes correlates with PRT/GAG reactivity.
Hepatic Insulin Resistance and Altered Gluconeogenic Pathway in Premature Baboons
Authors: McGill-Vargas Lisa , Gastaldelli Amalia , Liang Hanyu , Anzueto Guerra Diana , Johnson-Pais Teresa , Seidner Steven , McCurnin Donald , Muscogiuri Giovanna , DeFronzo Ralph , Musi Nicolas , Blanco Cynthia
Published: April 2017

Premature infants have altered glucose regulation early in life and increased risk for diabetes in adulthood. Although prematurity leads to an increased risk of diabetes and metabolic syndrome in adult life, the role of hepatic glucose regulation and adaptation to an early extrauterine environment in preterm infants remain unknown. The purpose of this study was to investigate developmental differences in glucose metabolism, hepatic protein content, and gene expression of key insulin-signaling/gluconeogenic molecules. Fetal baboons were delivered at 67%, 75%, and term gestational age and euthanized at birth. Neonatal baboons were delivered prematurely (67% gestation), survived for two weeks, and compared with similar postnatal term animals and underwent serial hyperinsulinemic-euglycemic clamp studies. Premature baboons had decreased endogenous glucose production (EGP) compared with term animals. Consistent with these results, the gluconeogenic molecule, phosphoenolpyruvate carboxykinase messenger RNA, was decreased in preterm baboons compared with terms. Hepatic insulin signaling was altered by preterm birth as evidenced by decreased insulin receptor–β, p85 subunit of phosphoinositide 3-kinase, phosphorylated insulin receptor substrate 1, and Akt-1 under insulin-stimulated conditions. Furthermore, preterm baboons failed to have the normal increase in glycogen synthase kinase-α from fetal to postnatal life. The blunted responses in hepatic insulin signaling may contribute to the hyperglycemia of prematurity, while impaired EGP leads to hypoglycemia of prematurity.
A Thyroid Hormone–Based Strategy for Correcting the Biochemical Abnormality in X-Linked Adrenoleukodystrophy
Authors: Hartley Meredith D. , Kirkemo Lisa L. , Banerji Tapasree , Scanlan Thomas S.
Published: April 2017

X-linked adrenoleukodystrophy (X-ALD) is a rare, genetic disorder characterized by adrenal insufficiency and central nervous system (CNS) demyelination. All patients with X-ALD have the biochemical abnormality of elevated blood and tissue levels of very long chain fatty acids (VLCFAs), saturated fatty acids with 24 to 26 carbons. X-ALD results from loss of function mutations in the gene encoding the peroxisomal transporter ABCD1, which is responsible for uptake of VLCFAs into peroxisomes for degradation by oxidation. One proposed therapeutic strategy for genetic complementation of ABCD1 is pharmacologic upregulation of ABCD2, a gene encoding a homologous peroxisomal transporter. Here, we show that thyroid hormone or sobetirome, a clinical-stage selective thyroid hormone receptor agonist, increases cerebral Abcd2 and lowers VLCFAs in blood, peripheral organs, and brains of mice with defective Abcd1. These results support an approach to treating X-ALD that involves a thyromimetic agent that reactivates VLCFA disposal both in the periphery and the CNS.
Cardiorespiratory Fitness and Adiposity as Determinants of Metabolic Health—Pooled Analysis of Two Twin Cohorts
Authors: Jukarainen Sakari , Holst René , Dalgård Christine , Piirilä Päivi , Lundbom Jesper , Hakkarainen Antti , Lundbom Nina , Rissanen Aila , Kaprio Jaakko , Kyvik Kirsten Ohm , Sørensen Thorkild I.A. , Pietiläinen Kirsi H.
Published: April 2017

Context:

The joint effects of cardiorespiratory fitness (CRF) and body composition on metabolic health are not well known.

Objective:

To examine the associations of CRF, fat-free mass index (FFMI), and fat mass index (FMI) with metabolic health in individual twins and controlling for genetic and shared environmental effects by studying monozygotic intrapair differences.

Design, Setting, and Participants:

Two cross-sectional samples of healthy adult monozygotic and dizygotic twins were drawn from population-based Danish and Finnish national twin registries (n = 996 and n = 309).

Main Measures:

CRF was defined as VO2max divided by fat-free mass. Insulin sensitivity and acute insulin response indices were derived from an oral glucose tolerance test. A continuous metabolic syndrome score was calculated. Visceral and liver fat were measured in the Finnish sample. Associations were analyzed separately in both cohorts with multivariate linear regression and aggregated with meta-analytic methods.

Results:

Insulin sensitivity, acute insulin response, metabolic syndrome score, visceral, and liver fat amount had strong and statistically significant associations with FMI (|β| 0.53 to 0.79), whereas their associations with CRF and FFMI were at most weak (|β| 0.02 to 0.15). The results of the monozygotic intrapair differences analysis showed the same pattern.

Conclusions:

Although FMI is strongly associated with worsening of metabolic health traits, even after controlling for genetic and shared environmental factors, there was little evidence for the effects of CRF or FFMI on metabolic health. This suggests that changing FMI rather than CRF or FFMI may affect metabolic health irrespective of genetic or early environmental determinants.
A Novel APOC2 Missense Mutation Causing Apolipoprotein C-II Deficiency With Severe Triglyceridemia and Pancreatitis
Authors: Ueda Masako , Dunbar Richard L. , Wolska Anna , Sikora Tracey U. , Escobar Maria del Rosario , Seliktar Naomi , deGoma Emil , DerOhannessian Stephanie , Morrell Linda , McIntyre Adam D. , Burke Frances , Sviridov Denis , Amar Marcelo , Shamburek Robert D. , Freeman Lita , Hegele Robert A. , Remaley Alan T. , Rader Daniel J.
Published: April 2017

Context:

Familial chylomicronemia syndrome (FCS) is a rare heritable disorder associated with severe hypertriglyceridemia and recurrent pancreatitis. Lipoprotein lipase deficiency and apolipoprotein C-II deficiency are two well-characterized autosomal recessive causes of FCS, and three other genes have been described to cause FCS. Because therapeutic approaches can vary according to the underlying etiology, it is important to establish the molecular etiology of FCS.

Case Description:

A man originally from North Africa was referred to the University of Pennsylvania Lipid Clinic for severe hypertriglyceridemia and recurrent pancreatitis, consistent with the clinical diagnosis of FCS. Molecular analyses of FCS-associated genes revealed a homozygous missense variant R72T in APOC2. Molecular modeling of the variant predicted that the apolipoprotein C-II R72T peptide has reduced lipid binding affinity. In vitro studies of the patient’s plasma confirmed the lack of functional apoC-II activity. Moreover, the apoC-II protein was undetectable in the patient’s plasma, quantitatively as well as qualitatively.

Conclusions:

We identified a missense APOC2 variant causing apoC-II deficiency in a patient with severe hypertriglyceridemia and recurrent pancreatitis. Beyond dietary management and usual pharmacologic therapies, an apoC-II mimetic peptide may become an optional therapy in patients with apoC-II deficiency in the future.
A Material-Documentary Literacy
Authors: Kosciejew Marc
Published: April 2017

The document is one of the oldest material objects of all recorded civilizations, one upon which we are, in many ways, still dependent today. Documentation — that is, documents and their associated practices, institutions, and histories — plays an important role in helping to materialize information, transforming it from something seemingly intangible into something tangible. Indeed, one of the main effects of documentation is information's materialization. This article contributes to new materialism by introducing a documentary approach to analyzing and understanding information's materiality. The article calls for a more materialist reorientation in the library and information science (LIS) field, specifically, and for considerations of information generally, by drawing attention to the important role played by documentation in the materialization of information. This reorientation also serves as a response to Ann-Sophie Lehmann's call for greater material literacy to help us better learn and understand more about our material surroundings. Lehmann argues that we need to have more awareness of and appreciation for the basic materials of our daily life and world. She explains that to uncover the richness of the material world, including how it affects us and its implications for our lives, we need to know what it is made of; in other words, what actually makes up the objects and things that we need and use? Documentation science complements and supports Lehmann's call for material literacy by drawing attention to documents and our practices with them. It explores how documents relate to the material world and vice versa. One places a specific document, or documents, at the center of observation, study, and analysis and thereby develops documentary dialogues about and for it, uses the document to better illuminate its context, and integrates the document in teaching and researching information.
3D motion of vesicles along microtubules helps them to circumvent obstacles in cells
Authors: Verdeny-Vilanova Ione , Wehnekamp Fabian , Mohan Nitin , Sandoval Álvarez Ángel , Borbely Joseph Steven , Otterstrom Jason John , Lamb Don C. , Lakadamyali Melike
Published: April 2017

Vesicle transport is regulated at multiple levels, including regulation by scaffolding proteins and the cytoskeleton. This tight regulation is essential, since slowing or stoppage of transport can cause accumulation of obstacles and has been linked to diseases. Understanding the mechanisms by which transport is regulated as well as how motor proteins overcome obstacles can give important clues as to how these mechanisms break down in disease states. Here, we describe that the cytoskeleton architecture impacts transport in a vesicle-size-dependent manner, leading to pausing of vesicles larger than the separation of the microtubules. We further develop methods capable of following 3D transport processes in living cells. Using these methods, we show that vesicles move using two different modes along the microtubule. Off-axis motion, which leads to repositioning of the vesicle in 3D along the microtubule, correlates with the presence of steric obstacles and may help in circumventing them.
Muscle biochemistry of a pelagic delphinid (Stenella longirostris longirostris): insight into fishery-induced separation of mothers and calves
Authors: Noren Shawn R. , West Kristi
Published: April 2017

The length of time required for postnatal maturation of the locomotor muscle (longissimus dorsi) biochemistry [myoglobin (Mb) content and buffering capacity] in marine mammals typically varies with nursing duration, but it can be accelerated by species-specific behavioral demands, such as deep-diving and sub-ice transit. We examined how the swimming demands of a pelagic lifestyle influence postnatal maturation of Mb and buffering capacity in spinner dolphins (Stenella longirostris longirostris). Mb content of newborn (1.16±0.07 g Mb per 100 g wet muscle mass, n=6) and juvenile (2.77±0.22 g per 100 g, n=4) spinner dolphins were only 19% and 46% of adult levels (6.00±0.74 g per 100 g, n=6), respectively. At birth, buffering capacity was 52.70±4.48 slykes (n=6) and increased to 78.53±1.91 slykes (n=6) once a body length of 141 cm was achieved, representing 1.6- to 2.0-year-old dolphins. Based on the age of weaning (1.3–1.6 years post-partum), muscle maturation occurred just after weaning as described for coastal bottlenose dolphins (Tursiops truncatus). Thus, a pelagic lifestyle does not promote rapid maturation of muscle biochemistry. Rather, it promotes enhanced muscle biochemistry: newborn and adult spinner dolphins had four- and two-times greater Mb contents than newborn and adult bottlenose dolphins, respectively. Indeed, adult levels rivaled those of deep-diving cetaceans. Nonetheless, the relatively underdeveloped muscle biochemistry of calves likely contributes to documented mother–calf separations for spinner dolphins chased by the tuna purse-seine fishery.
Integrative analysis unveils new functions for the Drosophila Cutoff protein in noncoding RNA biogenesis and gene regulation
Authors: Pritykin Yuri , Brito Tarcisio , Schupbach Trudi , Singh Mona , Pane Attilio
Published: April 2017

Piwi-interacting RNAs (piRNAs) are central components of the piRNA pathway, which directs transposon silencing and guarantees genome integrity in the germ cells of several metazoans. In Drosophila, piRNAs are produced from discrete regions of the genome termed piRNA clusters, whose expression relies on the RDC complex comprised of the core proteins Rhino, Deadlock, and Cutoff. To date, the RDC complex has been exclusively implicated in the regulation of the piRNA loci. Here we further elucidate the function of Cutoff and the RDC complex by performing genome-wide ChIP-seq and RNA-seq assays in the Drosophila ovaries and analyzing these data together with other publicly available data sets. In agreement with previous studies, we confirm that Cutoff is involved in the transcriptional regulation of piRNA clusters and in the repression of transposable elements in germ cells. Surprisingly, however, we find that Cutoff is enriched at and affects the expression of other noncoding RNAs, including spliceosomal RNAs (snRNAs) and small nucleolar RNAs (snoRNAs). At least in some instances, Cutoff appears to act at a transcriptional level in concert with Rhino and perhaps Deadlock. Finally, we show that mutations in Cutoff result in the deregulation of hundreds of protein-coding genes in germ cells. Our study uncovers a broader function for the RDC complex in the Drosophila germline development.
Diagnostic Imaging of Pregnant Women – The Role of Magnetic Resonance Imaging
Authors: Bekiesińska-Figatowska Monika , Romaniuk-Doroszewska Anna , Szkudlińska-Pawlak Sylwia , Duczkowska Agnieszka , Mądzik Jarosław , Szopa-Krupińska Martyna , Maciejewski Tomasz M.
Published: April 2017

Background: Presentation of magnetic resonance imaging (MRI) findings in pregnant women in the Department of Diagnostic Imaging, Institute of Mother and Child, Warsaw, Poland.

Material/Methods: Forty-three symptomatic pregnant women underwent MRI between 9 and 33 weeks of gestation (mean of 23 weeks). Moreover, we included 2 pregnant women who underwent fetal MRI and had incidental abnormalities.

Results: In 9 cases, we excluded the suspected brain abnormalities. In 4 cases, we found unremarkable changes in the brain without clinical significance. One patient was diagnosed with multiple sclerosis, one with cortical dysplasia, one with pineal hemorrhage and one with a brain tumor. On abdominal MRI, 2 patients had normal findings, one patient had colon cancer with a hepatic metastasis, one patient had a hepatic angioma, one patient had an extraadrenal pheochromocytoma, one patient had an abscess in the iliopsoas muscle, 9 patients had myomas, two patients had ovarian simple cysts, two endometrial cysts, three dermoid cysts, one patient had sacrococcygeal teratoma, one patient had a cystadenofibroma (partial borderline tumor), one patient had an androgenic ovarian tumor and two patients had hyperreactio luteinalis. One patient was diagnosed with transient osteoporosis of the hip and one with a stress fracture of the sacral bone.

Conclusions: Magnetic resonance imaging is the best imaging modality for pregnant women. Although ultrasonography is the method of choice, doubtful cases as well as structures that cannot be examined with ultrasonography can be non-invasively evaluated with MRI.
The Evolutionary Game of Pressure (or Interference), Resistance and Collaboration
Authors: Kolokoltsov Vassili
Published: April 2017

In this paper we extend the framework of the evolutionary inspection game put forward recently by the author and coworkers to a large class of conflict interactions to address the pressure executed by the major player (or principal) on the large group of small players who can resist this pressure or collaborate with the major player. We prove rigorous results on the convergence of various Markov decision models of interacting small agents (including evolutionary growth), i.e., pairwise, in groups and by coalition formation, to a deterministic evolution on the distributions of the state spaces of small players paying main attention to situations with an infinite state-space of small players. We supply precise rates of convergence. The theoretical results of the paper are applied to the analysis of the processes of inspection, corruption, cyber-security, counter-terrorism, banks and firms merging, strategically enhanced preferential attachment, and many other.
Unbound: Beyond the codex, the book as process/experience/event
Authors: Calvert Sheena
Published: April 2017

This article argues that the book as ‘process’ and ‘event’ requires a different understanding of ‘book’: one that acknowledges the impossibility of meaning residing in the final object alone. The finitude of the codex form is made a mockery of by the infinite dimensions of language, interpretation and time: this article extends that notion by exploring the process of making artists books as experience(s) that engage spatial/temporal/sensory attributes of making as being fully involved in their knowledge and meaning. Such books pose knowledge as an ‘event’: foregrounding the making and not the outcome. By example, the bookwork, Skinful (2003), questions the role of the materiality of the bound codex as object and examines how time and process operate in the book as a metaphor for emotional encounters. To complement this, The Cruelty of the Classical Canon (2014), expands the paradox of the material book as a container of knowledge explored through ‘brute’ encounters with making and experiencing such an object. Both books explore the relationship between the body/embodied thought in the book-as-process.