bluuebunny/crossref_metadata_2025_split huggingface dataset:
Immunoglobulin Replacement Therapy is critical and cost-effective in increasing life expectancy and quality of life in patients suffering from Common Variable Immunodeficiency Disorders (CVID): A health-economic assessment
Authors: van Wilder Philippe , Odnoletkova Irina , Mouline Mehdi , de Vries Esther 
Published: March 2021

BackgroundCommon variable immunodeficiency disorders (CVID), the most common form of primary antibody deficiency, are rare conditions associated with considerable morbidity and mortality. The clinical benefit of immunoglobulin replacement therapy (IgGRT) is substantial: timely treatment with appropriate doses significantly reduces mortality and the incidence of CVID-complications such as major infections and bronchiectasis. Unfortunately, CVID-patients still face a median diagnostic delay of 4 years. Their disease burden, expressed in annual loss of disability-adjusted life years, is 3-fold higher than in the general population. Hurdles to treatment access and reimbursement by healthcare payers may exist because the value of IgGRT is poorly documented. This paper aims to demonstrate cost-effectiveness and cost-utility (on life expectancy and quality) of IgGRT in CVID.Methods and findingsWith input from a literature search, we built a health-economic model for cost-effectiveness and cost-utility assessment of IgGRT in CVID. We compared a mean literature-based dose (≥450mg/kg/4wks) to a zero-or-low dose (0 to ≤100 mg/kg/4wks) in a simulated cohort of adult patients from time of diagnosis until death; we also estimated the economic impact of diagnostic delay in this simulated cohort.Compared to no or minimal treatment, IgGRT showed an incremental benefit of 17 life-years (LYs) and 11 quality-adjusted life-years (QALYs), resulting in an incremental cost-effectiveness ratio (ICER) of €29,296/LY and €46,717/QALY. These results were robust in a sensitivity analysis. Reducing diagnostic delay by 4 years provided an incremental benefit of six LYs and four QALYs compared to simulated patients with delayed IgGRT initiation, resulting in an ICER of €30,374/LY and €47,495/QALY.ConclusionsThe health-economic model suggests that early initiation of IgGRT compared to no or delayed IgGRT is highly cost-effective. CVID-patients’ access to IgGRT should be facilitated, not only because of proven clinical efficacy, but also due to the now demonstrated cost-effectiveness.
Ein neues Konzept für die Optimierung intelligenter Aktoren im Bereich beweglicher elektrischer Verbraucher
Authors: Manz Friedrich 
Published: March 2021

Zusammenfassung
Die Einbindung von mobilen Anwendungen in den Produktionsablauf von Fertigungs- und Produktionsanlagen gewinnt immer mehr an Bedeutung, um Produktionsprozesse flexibel und effizient abzubilden. Ausgehend von diesen Anforderungen wird in dieser Dissertation ein intelligenter Aktor für mobile Anwendungen entwickelt. Der Aufbau des Aktors selbst ist in drei Bereiche unterteilt. Der erste Bereich beschreibt die Datenkommunikation und Datensynchronisation zwischen den stationären und den beweglichen Kommunikationsteilnehmern. Im zweiten Teil ist der Aufbau einer Hardwareplattform für einen intelligenten Aktor und die Umsetzung einer Motorsteuerung realisiert. Der letzte Bereich zeigt die Einbindung eines prädiktiven Regelungsverfahrens im Vergleich zu einem klassischen Regelungskonzept.
Avoided motifs: short amino acid strings missing from protein datasets
Authors: Mier Pablo , Andrade-Navarro Miguel A. 
Published: March 2021

Abstract
According to the amino acid composition of natural proteins, it could be expected that all possible sequences of three or four amino acids will occur at least once in large protein datasets purely by chance. However, in some species or cellular context, specific short amino acid motifs are missing due to unknown reasons. We describe these as Avoided Motifs, short amino acid combinations missing from biological sequences. Here we identify 209 human and 154 bacterial Avoided Motifs of length four amino acids, and discuss their possible functionality according to their presence in other species. Furthermore, we determine two Avoided Motifs of length three amino acids in human proteins specifically located in the cytoplasm, and two more in secreted proteins. Our results support the hypothesis that the characterization of Avoided Motifs in particular contexts can provide us with information about functional motifs, pointing to a new approach in the use of molecular sequences for the discovery of protein function.
Land Policy and Food Prices: Evidence from a Land Consolidation Program in Rwanda
Authors: Nsabimana Aimable , Niyitanga Fidele , Weatherspoon Dave D. , Naseem Anwar 
Published: March 2021

Abstract
Rwanda’s “Crop Intensification Program (CIP)” is primarily a land consolidation program aimed at improving agricultural productivity and food security. The program, which began in 2007, focuses on monocropping and commercialization of six priority crops: maize, wheat, rice, white potato, beans, and cassava. CIP has facilitated easy access to improved seed stocks, fertilizer, extension services, and postharvest handling and storage services. Although studies have documented the impact of CIP on changes in farm yield, incomes, and productivity, less is known about its impact on food prices. In this study, we examine the crop-food price differences in intensive monocropped CIP and non-intensive monocropped CIP zones in Rwanda. Specifically, the study evaluates price variations of beans and maize along with complementary food crops in intensive and non-intensive monocropped zones before and after the introduction of the CIP policy. We find that the CIP policy is not associated with differences in CIP crop prices between the intensive and non-intensive monocropped zones. Over time, prices increased for CIP crops but generally, the crop prices in the two zones were cointegrated. Prices for non-CIP crops in the two different zones did show price differentials prior to the implementation of CIP, with the prices in intensive monocropped zones being greater than in the non-intensive monocropped zones. Moreover, the prices in intensive areas are cointegrated with prices in non-intensive areas for maize and beans and these prices are converging. This indicates that farmers who intensively produced one CIP crop were able to go to the market and purchase other food crops and that price differences between zones have decreased over time, potentially making the CIP intensive farmers better off.
Highly birefringent photonic crystal fiber with D-shaped air holes for terahertz (THz) application
Authors: Wang Biao , Jia Chunrong , Yang Jiantan , Di Zhigang , Yao Jianquan 
Published: March 2021

Abstract
A new type of high birefringence terahertz (THz) band photonic crystal fiber (PCF) is proposed. D-shaped air holes are introduced into the core of the PCF and the cladding near the core. Some important parameters of this waveguide were researched in the range of 0.6–1.2 THz. The simulation results show that the proposed fiber has a high birefringence of 0.0425 at 0.7 THz. In addition, the confinement loss and the effective mode area are respectively 6.63 × 10−7 dB/m and 0.77 × 105 μm2, when the operating frequency is 1 THz and the high birefringence of 0.04 is maintained. At the same time, very low bending loss and near zero flat positive dispersion are obtained. And has single-mode operating characteristics in the entire research frequency range. The PCF structure will provide new solutions for the application of THz waves and the design of THz waveguides.
Epileptic phenotype in late-onset hyperinsulinemic hypoglycemia successfully treated by diazoxide
Authors: Descamps Justine , Ruello Cyril , Perge Kevin , de Bellescize Julitta , Saint-Martin Cécile , Nicolino Marc 
Published: March 2021

Abstract

Objectives
Serious hyperinsulinemic hypoglycemia (HH) is generally the main initial symptom of hyperinsulinism. Epilepsy, without any overt feature of hypoglycemia, might be a very rare initial presentation of late-onset isolated hyperinsulinism.

Case presentation
We describe a case of late-onset HH in a 15-year-old boy with a history of idiopathic generalized epilepsy, now named genetic generalized epilepsy (IGE/GGE), beginning with a tonic–clonic seizure at the age of 11 years. Subsequently, absences with rare eyelid myoclonia were recorded on electroencephalogram (EEG), followed by episodes of impaired consciousness with facial myoclonia. Neurological status was normal except attention-deficit hyperactivity disorder (ADHD). At the age of 15 years, an episode of slight alteration of consciousness with neurovegetative signs could be recorded, which did not correspond to an absence status. Hypoglycemia due to hyperinsulinism was documented (clinically, biologically, and genetically). Diazoxide treatment resolved the glycopenic symptoms, the non-hypoglycemic seizures and normalized brain electrical activity allowing complete withdrawal of antiepileptic medication.

Conclusions
Epilepsy can be a very rare initial feature of HH starting in childhood. The occurrence of atypical features in the context of GGE as “absence statuses” with unusual vegetative symptoms and facial myoclonia might be suggestive for HH. Careful assessment and specific treatment are necessary to prevent hyperinsulinism related brain damage. Our case showed that diazoxide might also resolve seizures and normalize EEG.
Establishment of analysis methodology of RADTRAD for Maanshan PWR ATWS
Authors: Lin H.-T. 
Published: March 2021

Abstract
In this research, the analysis methodology for Maanshan PWR nuclear power plant (NPP) ATWS (Anticipated Transient Without Scram) is established by using RADTRAD code. This methodology can evaluate the dose at Exclusion Area Boundary (EAB), Low Population Zone (LPZ), and control room for the Maanshan ATWS. In addition, there are four leakage pathways in the Maanshan ATWS that are containment leakage, containment low volume purge, steam generator leakage, and ESF leakage. Therefore, for these leakage pathways, the RADTRAD models are established. Finally, according to the analysis results, they are below the criteria of 10 CFR 100.11 for the EAB and LPZ and Standard Review Plan (SRP) for the control room. Additionally, the steam generator leakage gives the maximum dose in these leakage pathways.
Risk factors of left atrial appendage thrombus in patients with non-valvular atrial fibrillation
Authors: Liu Yaowu , Zhu Didi , Xiao Yunyun , Zhu Yeqian , Zhou Qianxing , Ren Liqun , Chen Long 
Published: March 2021

Abstract

Objective
To investigate the risk factors of left atrial appendage thrombus (LAAT) in patients with non-valvular atrial fibrillation (AF).

Methods
We collected the clinical data of patients with non-valvular AF who underwent transesophageal echocardiography (TEE) at the Zhongda Hospital of Southeast University between January 2016 and June 2019. The patients were divided into two groups, LAAT and non-LAAT. We performed comparative analysis, receiver operating characteristic (ROC) curve analysis and logistic regression analysis to estimate the risk factors of LAAT.

Results
A total of 442 patients with non-valvular AF were enrolled in the study. LAAT was detected by TEE in 20 cases (4.7%). Compared with patients without LAAT, patients with LAAT had higher CHA2DS2-VASc scores (3 vs 2, p = 0.001), higher values of D-dimer (180.0 vs 90.0 μg/L, p = 0.003), larger LA anteroposterior diameters (50.5 vs 41.0 mm, p < 0.001) and higher ratios of non-paroxysmal AF (85.0% vs 23.6%, p < 0.001). ROC curve analysis revealed that the cutoff value of LA anteroposterior diameter was 49.5 mm. After adjusting for other confounders, logistic regression analysis showed that enlarged LA (anteroposterior diameter ≥49.5 mm) and non-paroxysmal AF were independently associated with higher risks of LAAT (OR = 7.28, 95% CI: 2.36–22.47; OR = 8.89, 95% CI: 2.33–33.99, respectively). The proportions of LAAT in patients with larger LA (anteroposterior diameter ≥49.5 mm), non-paroxysmal AF and both larger LA and non-paroxysmal AF were 30% (12/40), 15.2% (17/112) and 39.1% (9/23), respectively.

Conclusion
Enlarged LA (anteroposterior diameter ≥49.5 mm) and non-paroxysmal AF were independent risk factors of LAAT in non-valvular AF patients.
Synergistic strengthening mechanism of copper matrix composite reinforced with nano-Al2O3 particles and micro-SiC whiskers
Authors: Lin Huanran , Guo Xiuhua , Song Kexing , Feng Jiang , Li Shaolin , Zhang Xiangfeng 
Published: March 2021

Abstract
Although Cu–Al2O3 composites have good comprehensive performance, higher mechanical properties and arc erosion resistance are still required to meet heavy-duty applications such as electromagnetic railguns. In this work, a novel hybrid SiCw/Cu–Al2O3 composite was successfully prepared by combining powder metallurgy and internal oxidation. The microstructure and mechanical behavior of the SiCw/Cu–Al2O3 composite were studied. The results show that nano-Al2O3 particles and micro-SiCw are introduced into the copper matrix simultaneously. Well-bonded interfaces between copper matrix and Al2O3 particles or SiCw are obtained with improved mechanical and arc erosion resistance of SiCw/Cu–Al2O3 composite. The ultimate tensile strength of the SiCw/Cu–Al2O3 composite is 508.9 MPa, which is 7.9 and 56.1% higher than that of the Cu–Al2O3 composite and SiCw/Cu composite, respectively. The strengthening mechanism calculation shows that Orowan strengthening is the main strengthening mechanism of the SiCw/Cu–Al2O3 composite. Compared with Cu–Al2O3 composite, the hybrid SiCw/Cu–Al2O3 composite has lower arc time and energy and better arc stability.
Integrated weed management practices and sustainable food production among farmers in Kwara State, Nigeria
Authors: Imoloame Emmanuel Oyamedan , Ayanda Ibrahim Folorunsho , Yusuf Olayinka Jelili 
Published: March 2021

Abstract
A survey was conducted in the Kwara State of Nigeria to study the integrated weed management (IWM) practices by farmers. This was in view of the poor weed management practices adopted by farmers, which is a major factor responsible for low yields of many arable crops in Kwara State. A multi-stage sampling technique was used to select a sample size of 480 respondents, and a structured interview schedule was used to elicit information from them. Data obtained were analyzed using descriptive statistics. Factor analysis was also carried out to examine the perception of farmers’ benefits of IWM. Results showed that the majority of farmers (29.4%) were youths, married (89.1%), and involved in medium-scale farming (47.2%). Furthermore, 50.8% of the farmers had primary or secondary education. Although farmers use different weed control methods, more than half of them (54.7%) use herbicides. Most farmers (92.6%) are engaged in the use of IWM, However, 73.5% of them use a combination of herbicides and hoe weeding. Although not properly practiced, farmers perceived IWM as having socio-environmental (29.229%) and techno-efficacious (23.495%) benefits over either hoe weeding or herbicides used alone. The findings suggest a need to train farmers on all aspects of IWM to achieve self-sufficiency in food production in Kwara State.
W-Chirped optical solitons and modulation instability analysis of Chen–Lee–Liu equation in optical monomode fibres
Authors: Inc Mustafa , Yakada Salathiel , Bienvenu Depelair , Betchewe Gambo , Chu Yu-Ming 
Published: March 2021

Abstract
This work was devoted to unearth W-chirped to the famous Chen–Lee–Liu equation (CLLE) in optical monomode fibres. The results obtained will be useful to explain wave propagating with the chirp component. To attempt the main goal, we have used the new sub-ordinary differential equation (ODE) technique which was upgraded recently by Zayed EME, Mohamed EMA. Application of newly proposed sub-ODE method to locate chirped optical solutions to the Triki–Biswas equation equation. Optik. 2020;207:164360. On the other hand, we have used the modulation analysis to study the steady state of the obtained chirped soliton solutions in optical monomode fibres.
Sounds and gestures of linguistic reference: the endurance of reality in the poetry of Wallace Stevens
Authors: Levi Melih 
Published: March 2021

AbstractThe article seeks a rapprochement between pragmatic and semantic theories of language by returning to a breaking point in the history of philosophy, the middle of the twentieth century, when these theoretical models began to evolve into distinct schools of thought. Philosophical accounts of this period explore various and intertwined dependencies between semantics and context; however, they only implicitly examine the potential of sounds and bodily gestures in bringing descriptive clarity to the modes and limits of such dependencies. The article first investigates the way W. V. Quine conceptualized linguistic reference by combining behavioral models of language acquisition with more systematic explorations of syntax. It then turns to P. F. Strawson’s revisionary accounts of Kantian philosophy which reassess the silent strains of empiricism in Kant’s framework in order to identify possible grounds of reconciliation between pragmatic and semantic theories of language. Sound and gesture, two aspects that supplement language and give it an embodied feeling, are suggested as possible devices for formalizing intersections between context and semantic-oriented approaches. Poems by Wallace Stevens are used to think about the endurance of reference and how this endurance can find more compelling demonstration through an investigation of language as an embodied phenomenon.
Integration Analysis of m6A Regulators and m6A-Related Genes in Hepatocellular Carcinoma
Authors: Xie Jingdun , Qi Zhenhua , Luo Xiaolin , Yan Fang , Xing Wei , Zeng Weian , Chen Dongtai , Li Qiang 
Published: March 2021

Abstract

Background: N6-Methyladenosine (m6A) RNA methylation of eukaryotic mRNA is involved in the progression of various tumors. We aimed to investigate m6A-related genes and m6A regulators in hepatocellular carcinoma (HCC) and their association with prognosis in HCC.

Methods: We downloaded liver cancer sample data from The Cancer Genome Atlas (TCGA) and the International Cancer Genome Consortium database. A total of 21 m6A regulators and 1258 m6A-related genes were then analyzed by consensus clustering, Spearman’s correlation, GO, KEGG, LASSO Cox regression, and univariate Cox regression analyses. Finally, we constructed a risk prognostic model.

Results: We obtained 192 candidate m6A-related genes and 3 m6A regulators, including YTHDF1, YTHDF2, and YTHDC1. The expression of these genes and regulators differed significantly in different stages of HCC. Based on Cox regression analysis, 19 of 98 m6A-related prognostic genes were obtained to construct a risk score model. The 1- and 3-year area under the curves (AUCs) among HCC patients were greater than 0.7. Finally, based on analysis of mutation differences between high- and low-risk score groups, we determined that TP53 had the highest mutation frequency in the high-risk HCC patient group, whereas titin (TTN) had the highest mutation frequency in the low-risk HCC patient group.

Conclusion: This study comprehensively analyzed m6A regulators and m6A-related genes through an integrated bioinformatic analysis, including expression, clustering, protein–protein interaction, and prognosis, thus providing novel insights into the roles of m6A regulators and m6A-related genes in HCC.
MANAJEMEN KEARSIPAN DALAM MENUNJANG KEGIATAN ADMINISTRASI PELAYANAN PUBLIK PADA SEKOLAH USAHA PERIKANAN MENENGAH SORONG DI LINGKUNGAN KEMENTERIAN KELAUTAN DAN PERIKANAN
Authors: Fakaubun Arfa 
Published: March 2021

Dilihat dari perkembangan ilmu pengetahuan dan teknologi yang sangat pesat dewasa ini membawa pengaruh terhadap seluruh kegiatan yang dilakukan oleh setiap instansi, oleh sebab itu perlu ditingkatkan manajemen kearsipan dalam menunjang kegiatan administarsi pengelolaan arsip yang baik dan benar agar menjadi sumber informasi publik. karena sangat menunjang kecepatan dan ketepatan penyajian informasi, serta dapat membantu semua pihak dalam rangka melancarkan penyelesaian tugas, guna mencapai tujuan yang telah ditentukan. maka perlu dipahami tentang seluk beluk kearsipan sehingga dapat mengatasi masalah yang dihadapi oleh instansi Pemerintah maupun swasta, seperti pada Sekolah Usaha Perikanan Menengah Sorong yang notabenya sebagai Sekolah Kejuruan di bidang Perikanan, maka perlu di dukung dengan Sarana dan Prasarana Pendidikan yang disebut dengan manajemen kearsipan, sehingga dapat mewujudkan informasi pelayanan administrasi yang baik dan benar, yang diamanatkan dalam Undang-Undang Nomor 43 Tahun 2009 tentang Kearsipan dan Undang-Undang Nomor 20 Tahun 2003 tentang Sistem Pendidikan Nasional. Judging from the rapid development of science and technology today under the influence of all activities carried out by each agency, therefore it is necessary to improve the management of archives in supporting the administration of good and correct records management in order to become a source of public information. because it greatly supports the speed and accuracy of the presentation of information, and can help all parties in order to expedite the completion of the task, in order to achieve the specified goals. then it needs to be understood about the ins and outs of archiving so as to overcome the problems faced by government and private agencies, such as the Sorong Middle Fisheries Business School which in fact is a Vocational School in the field of Fisheries, it needs to be supported with Educational Facilities and Infrastructure called archival management, so that it can realize good and correct administrative service information, which is mandated in Act Number 43 of 2009 concerning Archives and Act Number 20 of 2003 concerning the National Education System.
Doenças lisossômicas de depósito em pacientes com possíveis diagnóstico de erros inatos do metabolismo
Authors: Martins Cíntia Costa Medeiros , Lima Thomas Werland , Cé Jaqueline , Coelho Janice Carneiro , Mello Alexandre Silva de 
Published: March 2021

As Doenças Lisossômicas de Depósito (DLDs) são distúrbios genéticos, clinicamente heterogêneos, causados principalmente por defeitos em genes que codificam enzimas lisossômicas que degradam macromoléculas. Este estudo tem o objetivo de verificar o índice de DLDs na coleta de sanguede 4986 pacientes, com possíveis diagnósticosde uma destas doenças de um laboratório de referêncialocalizado no Sul do Brasil, com amostras de todos os Estados do País.Trata-se de um estudo quantitativo, transversal de caráter descritivo e exploratório, com dados secundários coletados pela pesquisa realizada no Laboratório de Bioquímica da Universidade Federal do Rio Grande do Sul- UFRGS,entre os anos de 2010 a 2015, para a pesquisa, foi utilizado um banco de dados que já estavam arquivados em uma planilha de Excel. Nesse estudo, avaliamos protocolos para o diagnóstico confirmatório de casos. Inicialmente, revisamoscasos triados positivos para Doença de Fabry, Doença de Gaucher, Doença de Pompe, MucopolissacaridoseeNiemann-Pick. Esse estudo discute também as limitações das análises enzimáticas e moleculares na confirmação do diagnóstico, os desafios que podem ser encontrados durante a interpretação dos resultados e a necessidade de um protocolo abrangente para a investigação precisa de casos em suspeita, identificados por estudos para DLDs. Esse estudo representa uma base para avaliações mais abrangentes, em vista de uma possível implementação diagnóstica em grande escala.