bluuebunny/crossref_metadata_2025 huggingface dataset:
Estudo da estabilidade térmica e oxidativa de misturas diesel-biodiesel utilizando a espectrofotometria UV-VIS
Authors: Tapanes Neyda de la Caridad Om , Silva Flaviane Roque Oliveira da , Salles João Bosco de , Santana Ana Isabel de Carvalho , Gaidzinski Roberta , Marinho Leonardo da Silva , Perez Rodolfo Salazar
Published: November 2024

Os combustíveis do petróleo contêm enxofre, ácidos naftênicos e metais que prejudicam sua qualidade e geram produtos de oxidação provocando a obstru-ção de filtros, a erosão de partes do sistema de injeção e a corrosão nos dutos e depósitos. Por estas razões, as técnicas para avaliar a estabilidade das mistu-ras diesel/biodiesel resultam determinantes para o cumprimento das especifica-ções de qualidade. Ante este cenário, foram desenvolvidos equipamentos es-pecíficos, contudo de altos custos e tempos de resposta. Dentre as técnicas al-ternativas para avaliar a oxidação destacam-se a espectrofotometria UV-Vis por apresentar robustez, custo relativamente baixo e aplicabilidade. Neste sentido, a presente pesquisa tem como objetivo avaliar a influência do teor de enxofre, ácidos e a temperatura sobre a estabilidade térmica e oxidativa das frações de diesel em misturas com biodiesel, aplicando espectrofotometria UV/Visível. Planejamentos fatoriais foram utilizados para avaliar a combinação dos fatores: teores de enxofre e ácidos graxos, temperatura, tempo de contato com aço microligado e teor de aditivo antioxidante adicionado. As variáveis de resposta foram: a taxa de corrosão em aço microligado e a estabilidade por espectrofotometria UV-VIS. Os resultados demostraram que a técnica de espec-trofotometria UV-VIS possui potencial para ser utilizado no estudo da estabili-dade térmica e oxidativa de misturas binárias base diesel.
MEMBANGUN KELAS KONDUSIF
Authors: Gea Amsal , Kurniastuti Septiana Feni
Published: November 2024

Penelitian kualitatif deskriptif ini dilakukan untuk mendeskripsikan peran guru Kristen dalam menerapkan lingkungan belajar yang kondusif. Lingkungan belajar merupakan bagian dari pengelolaan kelas yang bertujuan untuk mencapai tujuan pembelajaran. Untuk mencapai hal ini, guru merupakan salah satu faktor penting. Hal ini diteliti melalui peran yang dijalankan untuk mencapai tujuan tersebut. Pengumpulan data dilakukan pada Februari hingga April 2024. Data dalam penelitian ini diperoleh dari observasi dan wawancara seorang guru Kristen di kelas 2 di sebuah sekolah teologi Kristen di Surabaya. Hasil yang didapat dalam penelitian ini mendeskripsikan peran guru sebagai pendidik, pembimbing dan fasilitator dalam menerapkan lingkungan belajar yang kondusif. Secara khusus, peran guru Kristen ditumbuhkan dari identitas dan karakternya sebagai pendidik Kristen yang dipanggil untuk melayani murid.
PENGARUH GAME BASED LEARNING PADA KEMAMPUAN KOGNITIF DAN PERKEMBANGAN EMOSI MURID SEKOLAH DASAR DI PAPUA
Authors: Indriani Maria , Puspitasari Dani
Published: November 2024

Bahasa Indonesia merupakan bahasa kedua yang digunakan oleh para murid sekolah dasar yang terletak di pegunungan Papua. Mereka lebih fasih menggunakan bahasa daerah dibandingkan bahasa nasional. Kondisi ini membuat siswa kesulitan memahami penjelasan guru di sekolah. Penelitian ini dibuat untuk melihat pengaruh implementasi game based learning terhadap kemampuan kognitif dan perkembangan emosi di kelas Bahasa Indonesia. Penelitian ini menerapkan pendekatan campuran Kemampuan kognitif diukur dengan pretest-posttest sedangkan perkembangan emosi menggunakan observasi dan wawancara. Penelitian ini menyimpulkan bahwa, game based learning memberikan pengaruh positif dalam kemampuan kognitif dan perkembangan emosi murid di kelas Bahasa Indonesia.
PENGEMBANGAN ELEMEN BALOK LENGKUNG DENGAN PEDEKATAN DEGENERATED SOLID
Authors: Halim Sanjaya , Wong Foek Tjong
Published: November 2024

Salah satu teori untuk menganalisis balok lengkung dalam metode elemen hingga adalah degenerated solid beam. Fenomena yang umum terjadi pada elemen balok lengkung yaitu shear locking dan membrane locking. Koziey dan Mirza (1994) membuat perumusan baru untuk analisis belok lengkung berdasarkan third order shear deformation theory (TOSD). Pada perumusan degenerated solid beam element umumnya menerapkan first order shear deformation theory (FOSD) yang membutuhkan koefisien koreksi geser. Pada penelitian ini akan dikembangkan degenerated solid beam element dengan modifikasi metode least square smoothed assumed strain (LSSAS) untuk eleminasi locking. Selain itu, akan diteliti perbedaan elemen yang menggunakan FOSD dengan menggunakan TOSD. Hasil pengujian diperoleh bahwa metode LSSAS tidak efektif dalam membebaskan degenerated solid beam element dari locking dan consistent beam merupakan elemen yang belum stabil. Penggunaan perumusan TOSD memiliki performa yang sama dengan penggunaan perumusan FOSD.
Communication Strategy of Peduly Gotong Royong Foundation in Building Volunteer Re-participation in Surabaya
Authors: Wardana Ivana , Luik Jandy Edipson , Budiana Daniel
Published: November 2024

Peduly Surabaya is how people called Peduly Gotong Royong Foundation which islocated in Surabaya. Peduly Surabaya started volunteer activities involving external parties inJune 2023. In a month, there are around 3-5 volunteers who return more than twice in thesame month. This statement shows that the level of re-participation in Peduly Surabaya isrelatively high. With a case study method, the case that can be studied from Peduly Surabayais the strategy in attracting volunteers to return to Peduly Surabaya's new volunteer activities.Based on the result, there are six strategies. These six strategies include internal and externalstrategies that are communicated directly or through online media. First, choose amanagement team that is communicative and has high initiative in accordance with Peduly'svalue. Second, consistently communicating various activities online Third, convey the resultsof evaluations and learning from previous activities. Fourth, communication through manymedia. Fifth, familiarize volunteers in creating content about Peduly Surabaya. Sixth,transparency regarding the function of donation contribution
The Influence of “Every U Does Good Heroes” Public Relations Campaign Message on Followers Attitudes regarding Unilever Indonesia’s Vision and Mission on Instagram @unileveridn
Authors: Chikita Vienny , Lesmana Fanny , Indrayani Inri Inggrit
Published: November 2024

This research was intended to explain the influence of campaign message Every U Does GoodHeroes on the followers of @unileveridn. Unilever Indonesia has run a campaign of Every U DoesGood Heroes since August 2021 until now. The campaign invites individuals or companies toextend kindness to others in simple ways. To provide kindness for around, Indonesia Unileverguides on three pillars that are in line with this campaign. These pillars are building moresustainable planets, improving the health and well-being of the community, and contributing to ajust and inclusive society. The kind of research is explanatory quantitative, with online methodsof surveys and simple linear regression as data analysis techniques. The number of samples, asmany as 100 followers of @unileveridn based on purposive sampling. Research has shown thatcampaign messages influence the followers of @unilever. The highest influence is on the messagestructure sub-indicator, namely conclusion statement. The lowest influence is in the message'scontent sub-indicator, supporting material that contains the purpose of campaign message.Research shows that variable x: public relations campaign message also has strong correlation withvariable y: attitude. This was due to campaign messages consistent with three pillars of UnileverIndonesia.
Cooperative Enhancement of Two‐Photon‐Absorption‐Induced Photoluminescence from a 2D Perovskite‐Microsphere Hybrid Dielectric Structure
Authors: Liu Weiwei , Li Xiaohong , Song Yiling , Zhang Cong , Han Xiaobo , Long Hua , Wang Bing , Wang Kai , Lu Peixiang
Published: April 2018

Two‐photon‐absorption‐induced photoluminescence (TPL) from nanostructures is generally inefficient since it is a typical third‐order nonlinear optical process. Herein, a hybrid dielectric structure composed of dielectric microspheres (approximately micrometers in diameter) covering a 2D perovskite flake is reported, which provides a straightforward strategy for enhancing the TPL emission. The microspheres in the hybrid dielectric structure not only concentrate the pumping laser but also effectively increase the detection efficiency of the emitted TPL signal. The internal quantum efficiency of the 2D perovskite is also increased in the hybrid dielectric structure due to a reduced nonradiative rate. These effects cooperatively increase the TPL emission by two orders of magnitude in the hybrid dielectric structure. Moreover, the hybrid dielectric structure is proven to be useful for TPL‐based superresolution imaging at a relatively low excitation power of 0.05 mW. This work demonstrates great promise for developing low‐cost, high‐performance nonlinear nanodevices based on hybrid dielectric structures.
Clinical spectrum of KIAA2022 pathogenic variants in males: Case report of two boys with KIAA2022 pathogenic variants and review of the literature
Authors: Lorenzo Melissa , Stolte‐Dijkstra Irene , van Rheenen Patrick , Smith Ronald Garth , Scheers Tom , Walia Jagdeep S.
Published: April 2018

KIAA2022 is an X‐linked intellectual disability (XLID) syndrome affecting males more severely than females. Few males with KIAA2022 variants and XLID have been reported. We present a clinical report of two unrelated males, with two nonsense KIAA2022 pathogenic variants, with profound intellectual disabilities, limited language development, strikingly similar autistic behavior, delay in motor milestones, and postnatal growth restriction. Patient 1, 19‐years‐old, has long ears, deeply set eyes with keratoconus, strabismus, a narrow forehead, anteverted nares, café‐au‐lait spots, macroglossia, thick vermilion of the upper and lower lips, and prognathism. He has gastroesophageal reflux, constipation with delayed rectosigmoid colonic transit time, difficulty regulating temperature, several musculoskeletal issues, and a history of one grand mal seizure. Patient 2, 10‐years‐old, has mild dysmorphic features, therapy resistant vomiting with diminished motility of the stomach, mild constipation, cortical visual impairment with intermittent strabismus, axial hypotonia, difficulty regulating temperature, and cutaneous mastocytosis. Genetic testing identified KIAA2022 variant c.652C > T(p.Arg218*) in Patient 1, and a novel nonsense de novo variant c.2707G > T(p.Glu903*) in Patient 2. We also summarized features of all reported males with KIAA2022 variants to date. This report not only adds knowledge of a novel pathogenic variant to the KIAA2022 variant database, but also likely extends the spectrum by describing novel dysmorphic features and medical conditions including macroglossia, café‐au‐lait spots, keratoconus, severe cutaneous mastocytosis, and motility problems of the GI tract, which may help physicians involved in the care of patients with this syndrome. Lastly, we describe the power of social media in bringing families with rare medical conditions together.
U‐Shaped Association Between Serum Uric Acid Level and Risk of Mortality
Authors: Cho Sung Kweon , Chang Yoosoo , Kim Inah , Ryu Seungho
Published: April 2018

Objective

In addition to the controversy regarding the association of hyperuricemia with cardiovascular disease (CVD) mortality, few studies have examined the impact of a low uric acid level on mortality. We undertook the present study to evaluate the relationship between both low and high uric acid levels and the risk of all‐cause and cause‐specific mortality in a large sample of Korean adults over a full range of uric acid levels.

Methods

A cohort study was performed in 375,163 South Korean men and women who underwent health check‐ups from 2002 to 2012. Vital status and cause of death were ascertained from the national death records. Hazard ratios (HRs) and 95% confidence intervals (95% CIs) for mortality outcomes were estimated using Cox proportional hazards regression analysis.

Results

During a total of 2,060,721.9 person‐years of follow‐up, 2,020 participants died, with 287 CVD deaths and 963 cancer deaths. Low and high uric acid levels were associated with increased all‐cause, CVD, and cancer mortality. The multivariable‐adjusted HRs for all‐cause mortality in the lowest uric acid categories (<3.5 mg/dl for men and <2.5 mg/dl for women) compared with the sex‐specific reference category were 1.58 (95% CI 1.18–2.10) and 1.80 (95% CI 1.10–2.93), respectively. Corresponding HRs in the highest uric acid categories (≥9.5 mg/dl for men and ≥8.5 mg/dl for women) were 2.39 (95% CI 1.57–3.66) and 3.77 (95% CI 1.17–12.17), respectively.

Conclusion

In this large cohort study of men and women, both low and high uric acid levels were predictive of increased mortality, supporting a U‐shaped association between serum uric acid levels and adverse health outcomes.
Triphenylamine–Thienothiophene Organic Charge‐Transport Molecular Materials: Effect of Substitution Pattern on their Thermal, Photoelectrochemical, and Photovoltaic Properties
Authors: Le Thi Huong , Dao Quang‐Duy , Nghiêm Mai‐Phuong , Péralta Sébastien , Guillot Regis , Pham Quoc Nghi , Fujii Akihiko , Ozaki Masanori , Goubard Fabrice , Bui Thanh‐Tuân
Published: April 2018

Two readily accessible thienothiophene–triphenylamine charge‐transport materials have been synthesized by simply varying the substitution pattern of the triphenylamine groups on a central thienothiophene π‐linker. The impact of the substitution pattern on the thermal, photoelectrochemical, and photovoltaic properties of these materials was evaluated and, based on theoretical and experimental studies, we found that the isomer in which the triphenylamine groups were located at the 2,5‐positions of the thienothiophene core (TT‐2,5‐TPA) had better π‐conjugation than the 3,6‐isomer (TT‐3,6‐TPA). Whilst the thermal, morphological, and hydrophobic properties of the two materials were similar, their optoelectrochemical and photovoltaic properties were noticeably impacted. When applied as hole‐transport materials in hybrid perovskite solar cells, the 2,5‐isomer exhibited a power‐conversion efficiency of 13.6 %, much higher than that of its 3,6‐counterpart (0.7 %) under the same standard conditions.
Evaluating Options for Regenerant Brine Reuse in Magnetic Ion Exchange Systems
Authors: Medina Beverly B. , Boyer Treavor , Indarawis Katrina
Published: April 2018

The purpose of this work was to provide an empirical foundation for decisions regarding regenerant brine management in anion exchange systems for the removal of compounds that absorb ultraviolet (UV) light at a wavelength of 254 nm (UV254) in drinking water treatment. Regenerant performance was assessed for fresh regenerant brine, reused regenerant brine, and used brine that had been treated with aluminum sulfate (alum) before reuse. Jar tests were conducted to examine the UV254 reduction achieved by resins treated with each of these regenerants. Over three regeneration cycles, resin treated with continuously reused regenerant did not display a significant decline in UV254 reduction. While treatment with alum was effective for removing UV254‐absorbing compounds from used regenerant brine, this treatment did not improve the brine's performance as a regenerant. These results demonstrate that regenerant brine may be reused several times without any intermediate treatment, which provides a cost‐saving solution for treatment plants seeking to install anion exchange systems.
Gene expression profiles for a prognostic immunoscore in gastric cancer
Authors: Zeng D , Zhou R , Yu Y , Luo Y , Zhang J , Sun H , Bin J , Liao Y , Rao J , Zhang Y , Liao W
Published: April 2018

Background

Increasing evidence has indicated an association between immune infiltration in gastric cancer and clinical outcome. However, reliable prognostic signatures, based on systematic assessments of the immune landscape inferred from bulk tumour transcriptomes, have not been established. The aim was to develop an immune signature, based on the cellular composition of the immune infiltrate inferred from bulk tumour transcriptomes, to improve the prognostic predictions of gastric cancer.

Methods

Twenty-two types of immune cell fraction were estimated based on large public gastric cancer cohorts from the Gene Expression Omnibus using CIBERSORT. An immunoscore based on the fraction of immune cell types was then constructed using a least absolute shrinkage and selection operator (LASSO) Cox regression model.

Results

Using the LASSO model, an immunoscore was established consisting of 11 types of immune cell fraction. In the training cohort (490 patients), significant differences were found between high- and low-immunoscore groups in overall survival across and within subpopulations with an identical TNM stage. Multivariable analysis revealed that the immunoscore was an independent prognostic factor (hazard ratio 1·92, 95 per cent c.i. 1·54 to 2·40). The prognostic value of the immunoscore was also confirmed in the validation (210) and entire (700) cohorts.

Conclusion

The proposed immunoscore represents a promising signature for estimating overall survival in patients with gastric cancer.
Effect of Catalyst Pore Size on the Performance of Non‐Precious Fe/N/C‐Based Electrocatalysts for High‐Temperature Polymer Electrolyte Membrane Fuel Cells
Authors: Byeon Ayeong , Lee Kyung Jin , Lee Min Jae , Lee Ju Sung , Lee In Hyuk , Park Hee‐Young , Lee So Young , Yoo Sung Jong , Jang Jong Hyun , Kim Hyoung‐Juhn , Kim Jin Young
Published: April 2018

Owing to the high cost of technologies utilizing the oxygen reduction reaction (ORR) in fuel cell applications, considerable efforts have been recently made to develop non‐precious metal electrocatalysts for the commercialization of the low‐temperature polymer electrolyte membrane fuel cells (LT‐PEMFCs). By extension, non‐precious‐metal electrocatalysts can be a cost‐effective solution for the commercialization of high‐temperature PEMFCs (HT‐PEMFCs). However, no previous reports have been published regarding the applicability. Herein, we demonstrate that a hierarchical mesoporous iron and nitrogen co‐doped carbon (Fe/N/C) electrocatalyst is able to efficiently electrocatalyze the ORR in a phosphoric acid electrolyte with a half‐wave potential of 0.72 V (only 170 mV deviation from conventional Pt/C) and high selectivity (electron‐transfer number >3.95) with a mild overpotential. Furthermore, we fabricated HT‐PEMFC devices on the basis of hierarchical mesoporous Fe/N/C electrocatalysts and non‐aqueous electrolytes, which exhibited a high performance, over 20 mW/cm2. Besides, the optimal hierarchical porosity of the mesoporous Fe/N/C catalyst contributes to its high ORR activity. It is considered that it provides a large surface area for electrocatalytic ORR, and improves the proton and oxygen transport properties.
Allergic contact dermatitis caused by Solvent Orange 60 dye in the temple tips of eyeglasses and a review of cases of eyeglass allergic contact dermatitis
Authors: Nishihara Seiko , Kozuka Takehito , Sasaki Kazumi
Published: April 2018

Background

A 57‐year‐old Japanese woman developed eczematous skin lesions behind both ears 10 days after she began using a new pair of glasses. The skin lesions resolved two weeks after she stopped wearing the glasses.

Methods

Patch testing for material scraped from the temple tips of the glasses was performed. A +2 reaction to the scraped material was observed on D2 and D3. Chemical analyses were performed to determine the causative allergen. A second patch test was performed with 6 fractions from the temple tips and Solvent Orange 60. We retrieved literature addressing eyeglass allergic contact dermatitis from MEDLINE (1997‐2017).

Results

Patch tests and chemical analyses of the temple tips of the glasses revealed that Solvent Orange 60 dye was a causative allergen.

Conclusions

We report a case of contact dermatitis caused by Solvent Orange 60 dye in the temple tips of eyeglasses. Overall, causative allergens of eyeglass allergic contact dermatitis include metals, plastics, plasticizers, solvents, UV stabilizers, antioxidants, dyes, waxes and preservatives; metals and dyes have been the most common such allergens in recent years.
Underwater Superaerophobic and Superaerophilic Nanoneedles‐Structured Meshes for Water/Bubbles Separation: Removing or Collecting Gas Bubbles in Water
Authors: Yong Jiale , Chen Feng , Li Wentao , Huo Jinglan , Fang Yao , Yang Qing , Bian Hao , Hou Xun
Published: April 2018

Water/bubbles separation has great practical significance, which can avoid the harm caused by underwater bubbles or cleverly collect useful gas bubbles in water. Here, a Cu(OH)2‐nanoneedles‐structured rough copper mesh is fabricated by a one‐step chemical reaction. The original rough mesh shows superhydrophilicity in air and superaerophobicity in water. The underwater superaerophobic mesh has great anti/removing‐bubbles ability in water. In contrast, the rough mesh switches to superhydrophobicity in air and superaerophilicity in water after further being modified with fluoroalkylsilane. The underwater superaerophilic mesh can absorb bubbles and allow bubbles to pass through the mesh. Based on the superhydrophilic/superaerophobic mesh and the superhydrophobic/superaerophilic mesh, a strategy to remove gas bubbles from the water pipe is proposed, and an in‐water bubbles‐collection device is also designed. It is believed that these two kinds of mesh will have more applications in controlling the behavior of underwater bubbles.
Protein phosphatase 1 regulatory subunit 3B gene variation protects against hepatic fat accumulation and fibrosis in individuals at high risk of nonalcoholic fatty liver disease
Authors: Dongiovanni Paola , Meroni Marica , Mancina Rosellina M. , Baselli Guido , Rametta Raffaela , Pelusi Serena , Männistö Ville , Fracanzani Anna L. , Badiali Sara , Miele Luca , Grimaudo Stefania , Petta Salvatore , Bugianesi Elisabetta , Soardo Giorgio , Fargion Silvia , Pihlajamäki Jussi , Romeo Stefano , Valenti Luca
Published: April 2018

Nonalcoholic fatty liver disease (NAFLD) is a major cause of liver damage and has a strong genetic component. The rs4841132 G>A variant, modulating the expression of protein phosphatase 1 regulatory subunit 3B (PPP1R3B), which is involved in glycogen synthesis, has been reported to reduce the risk of NAFLD but at the same time may favor liver disease by facilitating glycogen accumulation. The aim of this study was to assess the impact of rs4841132 on development of histologic steatosis and fibrosis in 1,388 European individuals in a liver biopsy cohort, on NAFLD hepatocellular carcinoma in a cross‐sectional Italian cohort (n = 132 cases), and on liver disease at the population level in the United Kingdom Biobank cohort. We investigated the underlying mechanism by examining the impact of the variant on gene expression profiles. In the liver biopsy cohort, the rs4841132 minor A allele was associated with protection against steatosis (odds ratio [OR], 0.63; 95% confidence interval [CI], 0.42‐0.95; P = 0.03) and clinically significant fibrosis (OR, 0.35; 95% CI, 0.14‐0.87; P = 0.02) and with reduced circulating cholesterol (P = 0.02). This translated into protection against hepatocellular carcinoma development (OR, 0.22; 95% CI, 0.07‐0.70; P = 0.01). At the population level, the rs4841132 variation was not associated with nonalcoholic or nonviral diseases of the liver but was associated with lower cholesterol (P = 1.7 × 10–8). In individuals with obesity, the A allele protecting against steatosis was associated with increased PPP1R3B messenger RNA expression and activation of lipid oxidation and with down‐regulation of pathways related to lipid metabolism, inflammation, and cell cycle. Conclusion: The rs4841132 A allele is associated with protection against hepatic steatosis and fibrosis in individuals at high risk of NAFLD but not in the general population and against dyslipidemia. The mechanism may be related to modulation of PPP1R3B expression and hepatic lipid metabolism. (Hepatology Communications 2018;2:666‐675)
Reasoning on the edge or in the cloud?
Authors: Seydoux Nicolas , Drira Khalil , Hernandez Nathalie , Monteil Thierry
Published: April 2018

The emergence of the Internet of Things (IoT) and the Semantic Web of Things is leading to intensive cloud processing executing reasoning rules over large volumes of enriched data. However, the role of the cloud in existing approaches as a central point for both data processing and provisioning reduces scalability and introduces latency. This paper sketches a new approach for rule‐based reasoning, enabling distributed rule evaluation on edge nodes, and reducing the latency for IoT applications while avoiding the total dependence on a central node. This approach is evaluated in a simulated smart building.
What Are Your Moral Duties as a Reader and as a Teacher of Reading?
Authors: Harrison Colin
Published: April 2018

This department column is a venue for thoughtful discussions of contemporary issues dealing with policy and practice, remixed in ways that generate new insights into enduring dilemmas, debates, and controversies.
How Could It Happen? Learning From Adolescents as They Engage Critical Literacy Skills of History
Authors: Schall‐Leckrone Laura , Barron Debra , Konuk Joan , Kain Nick
Published: April 2018

Often coauthored by university‐ and school‐based educators, preservice teachers, and youths, this department column considers how literacies are best developed through context‐crossing partnerships among university, school, and community constituents.
The Politics of Reading, Agency, and Participation
Authors: Garcia Antero
Published: April 2018

This forum reviews high‐interest young adult literature and adult texts. Exploring both print‐based and multimodal works, this column considers all kinds of texts that are encountered by contemporary readers, considering how the texts challenge and are challenged by readers.
Genetic and Epigenetic Defects at the GNAS Locus Lead to Distinct Patterns of Skeletal Growth but Similar Early-Onset Obesity
Authors: Hanna Patrick , Grybek Virginie , Perez de Nanclares Guiomar , Tran Léa C , de Sanctis Luisa , Elli Francesca , Errea Javier , Francou Bruno , Kamenicky Peter , Linglart Léa , Pereda Arrate , Rothenbuhler Anya , Tessaris Daniele , Thiele Susanne , Usardi Alessia , Shoemaker Ashley H , Kottler Marie-Laure , Jüppner Harald , Mantovani Giovanna , Linglart Agnès
Published: April 2018

Pseudohypoparathyroidism type 1A (PHP1A), pseudoPHP (PPHP), and PHP type 1B (PHP1B) are caused by maternal and paternal GNAS mutations and abnormal methylation at maternal GNAS promoter(s), respectively. Adult PHP1A patients are reportedly obese and short, whereas most PPHP patients are born small. In addition to parathyroid hormone (PTH) resistance, PHP1A and PHP1B patients may display early-onset obesity. Because early-onset and severe obesity and short stature are daily burdens for PHP1A patients, we aimed at improving knowledge on the contribution of the GNAS transcripts to fetal and postnatal growth and fat storage. Through an international collaboration, we collected growth and weight data from birth until adulthood for 306 PHP1A/PPHP and 220 PHP1B patients. PHP1A/PPHP patients were smaller at birth than healthy controls, especially PPHP (length Z-score: PHP1A –1.1 ± 1.8; PPHP –3.0 ± 1.5). Short stature is observed in 64% and 59% of adult PHP1A and PPHP patients. PHP1B patients displayed early postnatal overgrowth (height Z-score at 1 year: 2.2 ± 1.3 and 1.3 ± 1.5 in autosomal dominant and sporadic PHP1B) followed by a gradual decrease in growth velocity resulting in normal adult height (Z-score for both: –0.4 ± 1.1). Early-onset obesity characterizes GNAS alterations and is associated with significant overweight and obesity in adults (bodey mass index [BMI] Z-score: 1.4 ± 2.6, 2.1 ± 2.0, and 1.4 ± 1.9 in PPHP, PHP1A, and PHP1B, respectively), indicating that reduced Gsα expression is a contributing factor. The growth impairment in PHP1A/PPHP may be due to Gsα haploinsufficiency in the growth plates; the paternal XLαs transcript likely contributes to prenatal growth; for all disease variants, a reduced pubertal growth spurt may be due to accelerated growth plate closure. Consequently, early diagnosis and close follow-up is needed in patients with GNAS defects to screen and intervene in case of early-onset obesity and decreased growth velocity. © 2018 The Authors. Journal of Bone and Mineral Research Published by Wiley Periodicals, Inc. on behalf of American Society for Bone and Mineral Research (ASBMR).
Prediction of Fractures in Men Using Bone Microarchitectural Parameters Assessed by High-Resolution Peripheral Quantitative Computed Tomography—The Prospective STRAMBO Study
Authors: Szulc Pawel , Boutroy Stéphanie , Chapurlat Roland
Published: April 2018

Areal bone mineral density (aBMD) poorly identifies men at high fracture risk. Our aim was to assess prediction of fractures in men by bone microarchitectural measures. At baseline, 825 men aged 60 to 87 years had the assessment of bone microarchitecture at distal radius and distal tibia by high-resolution peripheral QCT (HR-pQCT; XtremeCT-I, Scanco Medical, Brüttisellen, Switzerland). Bone strength was estimated by micro-finite element analysis. During the prospective 8-year follow-up, 105 men sustained fractures (59 vertebral fractures in 49 men and 70 nonvertebral fractures in 68 men). After adjustment for age, body mass index (BMI), prior falls, and fractures, most HR-pQCT measures at both skeletal sites predicted fractures. After further adjustment for aBMD, low distal radius trabecular number (Tb.N) was most strongly associated with higher fracture risk (hazard ratio [HR] = 1.63 per SD, 95% confidence interval [CI] 1.31–2.03, p < 0.001). In similar models, low Tb.N was associated with higher risk of major osteoporotic fracture (HR = 1.80 per SD, p < 0.001), vertebral fracture (HR = 1.78 per SD, p < 0.01) and nonvertebral fracture (HR = 1.46 per SD, p < 0.01). In comparison with the reference model (age, BMI, falls, fractures, aBMD), the adjustment for distal radius Tb.N increased the estimated fracture probability in men who sustained fractures versus those who did not have ones (difference = 4.1%, 95% CI 1.9–6.3%, p < 0.001). However, the adjustment for distal radius Tb.N did not increase the area under the curve (AUC, p = 0.37). Similar results were found for distal radius trabecular separation (Tb.Sp) and connectivity density (Conn. D). They were predictive of all fracture types and increased the estimated fracture risk, but not AUC, in men who had incident fractures. Thus, poor distal radius trabecular microarchitecture is predictive of fracture after adjustment for age, BMI, falls, fractures, and aBMD. Although distal radius Tb.N, Conn. D, and Tb.Sp improve the discrimination between men who will or who will not have fracture, they do not provide clinically relevant improvement of fracture prediction in older men. © 2018 American Society for Bone and Mineral Research.
Sclerostin Neutralizing Antibody Treatment Enhances Bone Formation but Does Not Rescue Mechanically Induced Delayed Healing
Authors: Kruck Bettina , Zimmermann Elizabeth A , Damerow Sophie , Figge Christine , Julien Catherine , Wulsten Dag , Thiele Tobias , Martin Madge , Hamdy Reggie , Reumann Marie K , Duda Georg N , Checa Sara , Willie Bettina M
Published: April 2018

During bone healing, tissue formation processes are governed by mechanical strain. Sost/sclerostin, a key Wnt signaling inhibitor and mechano-sensitive pathway, is downregulated in response to mechanical loading. Sclerostin neutralizing antibody (SclAb) increases bone formation. Nevertheless, it remains unclear whether sclerostin inhibition can rescue bone healing in situations of mechanical instability, which otherwise delay healing. We investigated SclAb's influence on tissue formation in a mouse femoral osteotomy, stabilized with rigid or semirigid external fixation. The different fixations allowed different magnitudes of interfragmentary movement during weight bearing, thereby influencing healing outcome. SclAb or vehicle (veh) was administeredand bone healing was assessed at multiple time points up to day 21 postoperatively by in vivo micro-computed tomography, histomorphometry, biomechanical testing, immunohistochemistry, and gene expression. Our results show that SclAb treatment caused a greater bone volume than veh. However, SclAb could not overcome the characteristic delayed healing of semirigid fixation. Indeed, semirigid fixation resulted in delayed healing with a prolonged endochondral ossification phase characterized by increased cartilage, lower bone volume fraction, and less bony bridging across the osteotomy gap than rigid fixation. In a control setting, SclAb negatively affected later stages of healing under rigid fixation, evidenced by the high degree of endosteal bridging at 21 days in the rigid-SclAb group compared with rigid-veh, indicating delayed fracture callus remodeling and bone marrow reconstitution. Under rigid fixation, Sost and sclerostin expression at the gene and protein level, respectively, were increased in SclAb compared with veh-treated bones, suggesting a negative feedback mechanism. Our results suggest that SclAb could be used to enhance overall bone mass but should be carefully considered in bone healing. SclAb may help to increase bone formation early in the healing process but not during advanced stages of fracture callus remodeling and not to overcome delayed healing in semirigid fixation. © 2018 American Society for Bone and Mineral Research.
Respiratory disease, behavior, and survival of mountain goat kids
Authors: Blanchong Julie A. , Anderson Christopher A. , Clark Nicholas J. , Klaver Robert W. , Plummer Paul J. , Cox Mike , McAdoo Caleb , Wolff Peregrine L.
Published: April 2018

Bacterial pneumonia is a threat to bighorn sheep (Ovis canadensis) populations. Bighorn sheep in the East Humboldt Mountain Range (EHR), Nevada, USA, experienced a pneumonia epizootic in 2009–2010. Testing of mountain goats (Oreamnos americanus) that were captured or found dead on this range during and after the epizootic detected bacteria commonly associated with bighorn sheep pneumonia die‐offs. Additionally, in years subsequent to the bighorn sheep epizootic, the mountain goat population had low kid:adult ratios, a common outcome for bighorn sheep populations that have experienced a pneumonia epizootic. We hypothesized that pneumonia was present and negatively affecting mountain goat kids in the EHR. From June–August 2013–2015, we attempted to observe mountain goat kids with marked adult females in the EHR at least once per week to document signs of respiratory disease; identify associations between respiratory disease, activity levels, and subsequent disappearance (i.e., death); and estimate weekly survival. Each time we observed a kid with a marked adult female, we recorded any signs of respiratory disease and collected behavior data that we fit to a 3‐state discrete hidden Markov model (HMM) to predict a kid's state (active vs. sedentary) and its probability of disappearing. We first observed clinical signs of respiratory disease in kids in late July–early August each summer. We observed 8 of 31 kids with marked adult females with signs of respiratory disease on 13 occasions. On 11 of these occasions, the HMM predicted that kids were in the sedentary state, which was associated with increased probability of subsequent death. We estimated overall probability of kid survival from June–August to be 0.19 (95% CI = 0.08–0.38), which was lower than has been reported in other mountain goat populations. We concluded that respiratory disease was present in the mountain goat kids in the EHR and negatively affected their activity levels and survival. Our results raise concerns about potential effects of pneumonia to mountain goat populations and the potential for disease transmission between mountain goats and bighorn sheep where the species are sympatric. © 2018 This article is a U.S. Government work and is in the public domain in the USA.
Strategies That Reduce 90‐Day Readmissions and Inpatient Costs After Liver Transplantation
Authors: Zeidan Joseph H. , Levi David M. , Pierce Ruth , Russo Mark W.
Published: April 2018

Liver transplantation (LT) is hospital‐resource intensive and associated with high rates of readmission. We have previously shown a reduction in 30‐day readmission rates by implementing a specifically designed protocol to increase access to outpatient care. The aim of this work is to determine if the strategies that reduce 30‐day readmission after LT were effective in also reducing 90‐day readmission rates and costs. A protocol was developed to reduce inpatient readmissions after LT that expanded outpatient services and provided alternatives to readmission. The 90‐day readmission rates and costs were compared before and after implementing strategies outlined in the protocol. Multivariable analysis was used to control for potential confounding factors. Over the study period, 304 adult primary LTs were performed on patients with a median biological Model for End‐Stage Liver Disease of 22. There were 112 (37%) patients who were readmitted within 90 days of transplant. The readmission rates before and after implementation of the protocol were 53% and 26%, respectively (
P
< 0.001). The most common reason for readmission was elevated liver tests/rejection (24%). In multivariable analysis, the protocol remained associated with avoiding readmission (odds ratio, 0.33; 95% confidence interval, 0.20‐0.55;
P
< 0.001). The median length of stay after transplant before and after protocol implementation was 8 days and 7 days, respectively. A greater proportion of patients were discharged to hospital lodging after protocol implementation (10% versus 19%;
P
= 0.03). The 90‐day readmission costs were reduced by 55%, but the total 90‐day costs were reduced by only 2.7% because of higher outpatient costs and index admission costs. In conclusion, 90‐day readmission rates and readmission costs can be reduced by improving access to outpatient services and hospital‐local lodging. Total 90‐day costs were similar between the 2 groups because of higher outpatient costs after the protocol was introduced.
Phenotypes in siblings with homozygous mutations of TRAPPC9 and/or MCPH1 support a bifunctional model of MCPH1
Authors: Duerinckx Sarah , Meuwissen Marije , Perazzolo Camille , Desmyter Laurence , Pirson Isabelle , Abramowicz Marc
Published: April 2018

Background

Autosomal recessive intellectual disability (ARID) is vastly heterogeneous. Truncating mutations of TRAPPC9 were reported in 8 ARID families. Autosomal recessive primary microcephaly (MCPH) represents another subgroup of ARID, itself very heterogeneous, where the size of the brain is very small since birth. MCPH1 plays a role at the centrosome via a BRCT1 domain, and in DNA Damage Repair (DDR) via BRCT2 and BRCT3, and it is not clear which of these two mechanisms causes MCPH in man.

Methods

We studied the phenotype and sequenced the exome in two siblings with MCPH and their unaffected sister.

Results

Homozygous mutations of TRAPPC9 (p.Leu178Pro) and of MCPH1 (p.Arg741X) were found in both affected siblings. Brain MRI showed anomalies previously associated with TRAPPC9 defects, supporting the implication of TRAPPC9 in the phenotype. Importantly, the asymptomatic sister with normal head size was homozygous for the MCPH1 truncating mutation and heterozygous for the TRAPPC9 mutation.

Conclusion

The affected siblings represent the first ARID cases with a TRAPPC9 missense mutation and with microcephaly of prenatal onset of. Furthermore, their unaffected sister represents strong evidence that the lack of MCPH1 BRCT3 domain does not cause MCPH in man, supporting a bifunctional model of MCPH1 where the centrosomal function is involved in brain volumic development and not the DDR function.
Meta‐analysis of the incidence and patterns of second neoplasms after photon craniospinal irradiation in children with medulloblastoma
Authors: Bavle Abhishek , Tewari Sayani , Sisson Amy , Chintagumpala Murali , Anderson Michael , Paulino Arnold C.
Published: April 2018

Background

Second neoplasms (SNs) are a well‐established long‐term adverse effect of radiation therapy (RT), but there are limited data regarding their incidence and location relative to the radiation field, specific to medulloblastoma (MB) survivors after craniospinal irradiation (CSI).

Methods

A systematic literature review, per Preferred Reporting Items for Systematic Reviews and Meta‐Analyses, identified six studies reporting the incidence and locations of SNs for 1,114 patients with MB, after CSI, with a median follow‐up of ∼9 years (7.6–15.4 years). The study‐specific cumulative incidence (CI) of SNs, second benign neoplasms (SBNs), and second malignant neoplasms (SMNs) were standardized to a 10‐year time frame. Meta‐analysis was performed using random effects models, with pooled data from selected studies and an institutional cohort of 55 patients.

Results

The 10‐year CI was 6.1% for all SNs (excluding skin cancer and leukemia), 3.1% for SBNs, and 3.7% for SMNs. Fifty‐eight percent of SNs were malignant; high‐grade glioma was the most common SMN (15/33; 45%) and meningioma, the most common SBN (16/24; 67%). Forty percent of SNs occurred outside the target central nervous system (CNS) field, with a majority in areas of exit RT dose. Seventy‐four percent of extra‐CNS tumors (17/23) were malignant, most commonly thyroid carcinoma (7/17; 41%) and bone and soft‐tissue tumors (6/17, 35%).

Conclusions

Survivors of MB are at risk of SNs both within and outside the CNS. A significant proportion of SNs occur in areas of exit RT dose. Studies are needed to determine whether the use of proton therapy, which has no exit RT dose, is associated with a lower incidence of SNs.
Unravel the Active Site in Nitrogen‐Doped Double‐Walled Carbon Nanotubes for Nitrogen Dioxide Gas Sensor
Authors: Muangrat Worawut , Wongwiriyapan Winadda , Yordsri Visittapong , Chobsilp Thanattha , Inpaeng Saowaluk , Issro Chaisak , Domanov Oleg , Ayala Paola , Pichler Thomas , Shi Lei
Published: April 2018

In this paper, a nitrogen dioxide (NO2) gas sensor using nitrogen‐doped double‐walled carbon nanotubes (N‐DWCNTs) with different types of nitrogen is demonstrated, and the sensor performance to the pyridinic nitrogen is related. The ratio of nitrogen is controlled by the temperature applied for the synthesis. It is found that the fabricated sensor from N‐DWCNTs enable an approximately threefold improvement in NO2 detection compared to the sensor from DWCNTs. Also, the improvement of sensor response of N‐DWCNTs more depends on the pyridinic site than the other types of nitrogen, because it can strongly interact with the NO2 molecule. The sensing mechanism is attributed to the charge transfer between the NO2 molecule and the sensing materials (especially with pyridinic site), which shifts the Fermi level, resulting in a decrease of the electrical resistance. Furthermore, the relation between the sensor response and the concentration of NO2 is derived based on Langmuir adsorption isotherm, and the calculated detection limit can be down to 0.14 ppm, which suggests that the N‐DWCNTs‐based sensor is a promising approach for low concentration NO2 detection at room temperature.
Electrochemical Fabrication of Flat, Polymer‐Embedded Porous Silicon 1D Gradient Refractive Index Microlens Arrays
Authors: Krueger Neil A. , Holsteen Aaron L. , Zhao Qiujie , Kang Seung‐Kyun , Ocier Christian R. , Zhou Weijun , Mensing Glennys , Rogers John A. , Brongersma Mark L. , Braun Paul V.
Published: April 2018

Gradient refractive index (GRIN) optics has attracted considerable interest due to the ability to decouple optical performance from optical element shape. However, despite the utility of GRIN optical components, it remains challenging to fabricate arbitrary GRIN profiles and the available refractive index contrast remains small, particularly at the microscale. Here, using mathematical transformations that the authors developed, the electrochemical waveform required to electrochemically etch arrays of bulk Si microstructures into 1D porous Si (PSi) GRIN microlens arrays (MLAs) is determined. This waveform is then used to form high refractive index contrast MLAs containing precisely‐defined, arbitrary refractive index profiles. The MLAs are then embedded in a transparent optical polymer, mechanically detached from the host Si substrate, and planarized via simple polishing. Cylindrical microlenses and 1D axicons are demonstrated and characterized, and the optical behavior is found to be in agreement with theory. These MLAs could find applications in displays, photodetectors, and optical microscopy.
Addressing policy challenges in implementing Sustainable Development Goals through an adaptive governance approach: A view from transitional China
Authors: Xue Lan , Weng Lingfei , Yu Hanzhi
Published: April 2018

In 2015, the 17 Sustainable Development Goals (SDGs) were adopted by all member states of the United Nations. This paper argues that actually implementing the SDGs is far more challenging than reaching agreement to adopt them. To overcome these challenges, a Four‐Step Adaptive Governance Framework was set up to facilitate implementation of these goals. Two‐level policy tools are presented to illustrate the policy evolution of China's development transitions, within the framework of “adaptive governance.” This study found that the Four‐Step Adaptive Governance Approach is deeply embedded in China's policy process, and it has contributed significantly to China's development during the past four decades. The Adaptive Governance Framework and this example of its application in China suggests it could be a useful tool for developing countries that are formulating their national strategies to achieve their SDGs by 2030.
Graphene‐Oxide‐Supported SO3H‐Functionalized Imidazolium‐Based Ionic Liquid: Efficient and Recyclable Heterogeneous Catalyst for Alcoholysis and Aminolysis Reactions
Authors: Nakhate Akhil V. , Yadav Ganapati D.
Published: April 2018

Ionic liquids have been established as solvents and catalysts in a number of reactions due to their unique properties. In this study, we have synthesized sulfonic acid functionalized imidazolium based strongly acidic ionic liquid supported on graphene oxide (PTS−Im‐3@GO). PTS−Im‐3@GO was synthesized by grafting 1‐(4‐sulfobutyl)‐3‐(3‐propyltriethoxysilyl)imidazolium hydrogen sulfate onto graphene oxide (GO) by covalent bonds. The prepared PTS−Im‐3@GO was used for regio‐selective ring opening reaction of styrene oxide with isopropyl alcohol giving 95% conversion and 100% regio‐selectivity towards 2‐isopropoxy‐2‐phenylethan‐1‐ol at 50 °C. The activity of the said catalyst was compared with various heterogeneous catalysts such as GO, PTS−Im‐1@GO, PTS−Im‐2@GO, PTS−Im‐1, PTS−Im‐2, PTS−Im‐3, Hβ‐zeolite, and montmorillonite K‐10. Among all catalysts, PTS−Im‐3@GO showed good conversion towards the desired product. PTS−Im‐3@GO was characterized by FTIR, SEM, EDS, TGA, XRD, 29Si NMR, XPS and CHNS analysis. Systematic studies demonstrated that PTS−Im‐3@GO had excellent catalytic activity and regio‐selectivity for the alcoholysis of epoxides rendering β‐alkoxy alcohols and aminolysis of epoxide giving β‐amino alcohols in very good yields. The protocol is clean and green.