bluuebunny/crossref_metadata_2025_split huggingface dataset:
Genetics and phenotypic heterogeneity of Dent disease: the dark side of the moon
Authors: Gianesello Lisa , Del Prete Dorella , Anglani Franca , Calò Lorenzo A. 
Published: August 2020

AbstractDent disease is a rare genetic proximal tubulopathy which is under-recognized. Its phenotypic heterogeneity has led to several different classifications of the same disorder, but it is now widely accepted that the triad of symptoms low-molecular-weight proteinuria, hypercalciuria and nephrocalcinosis/nephrolithiasis are pathognomonic of Dent disease. Although mutations on the CLCN5 and OCRL genes are known to cause Dent disease, no such mutations are found in about 25–35% of cases, making diagnosis more challenging. This review outlines current knowledge regarding Dent disease from another perspective. Starting from the history of Dent disease, and reviewing the clinical details of patients with and without a genetic characterization, we discuss the phenotypic and genetic heterogeneity that typifies this disease. We focus particularly on all those confounding clinical signs and symptoms that can lead to a misdiagnosis. We also try to shed light on a concealed aspect of Dent disease. Although it is a proximal tubulopathy, its misdiagnosis may lead to patients undergoing kidney biopsy. In fact, some individuals with Dent disease have high-grade proteinuria, with or without hematuria, as in the clinical setting of glomerulopathy, or chronic kidney disease of uncertain origin. Although glomerular damage is frequently documented in Dent disease patients’ biopsies, there is currently no reliable evidence of renal biopsy being of either diagnostic or prognostic value. We review published histopathology reports of tubular and glomerular damage in these patients, and discuss current knowledge regarding the role of CLCN5 and OCRL genes in glomerular function.
Space–Time Variational Multiscale Isogeometric Analysis of a tsunami-shelter vertical-axis wind turbine
Authors: Otoguro Yuto , Mochizuki Hiroki , Takizawa Kenji , Tezduyar Tayfun E. 
Published: August 2020

AbstractWe present computational flow analysis of a vertical-axis wind turbine (VAWT) that has been proposed to also serve as a tsunami shelter. In addition to the three-blade rotor, the turbine has four support columns at the periphery. The columns support the turbine rotor and the shelter. Computational challenges encountered in flow analysis of wind turbines in general include accurate representation of the turbine geometry, multiscale unsteady flow, and moving-boundary flow associated with the rotor motion. The tsunami-shelter VAWT, because of its rather high geometric complexity, poses the additional challenge of reaching high accuracy in turbine-geometry representation and flow solution when the geometry is so complex. We address the challenges with a space–time (ST) computational method that integrates three special ST methods around the core, ST Variational Multiscale (ST-VMS) method, and mesh generation and improvement methods. The three special methods are the ST Slip Interface (ST-SI) method, ST Isogeometric Analysis (ST-IGA), and the ST/NURBS Mesh Update Method (STNMUM). The ST-discretization feature of the integrated method provides higher-order accuracy compared to standard discretization methods. The VMS feature addresses the computational challenges associated with the multiscale nature of the unsteady flow. The moving-mesh feature of the ST framework enables high-resolution computation near the blades. The ST-SI enables moving-mesh computation of the spinning rotor. The mesh covering the rotor spins with it, and the SI between the spinning mesh and the rest of the mesh accurately connects the two sides of the solution. The ST-IGA enables more accurate representation of the blade and other turbine geometries and increased accuracy in the flow solution. The STNMUM enables exact representation of the mesh rotation. A general-purpose NURBS mesh generation method makes it easier to deal with the complex turbine geometry. The quality of the mesh generated with this method is improved with a mesh relaxation method based on fiber-reinforced hyperelasticity and optimized zero-stress state. We present computations for the 2D and 3D cases. The computations show the effectiveness of our ST and mesh generation and relaxation methods in flow analysis of the tsunami-shelter VAWT.
A new approach for the identification of gas hydrate in marine sediments
Authors: Tian Dongmei , Liu Xuewei 
Published: August 2020

AbstractIt has always been the focus of researchers to accurately identify gas hydrate location. Geophysical prospecting is a widely used method for gas hydrate exploration, which has high credibility, especially seismic exploration technology is most generally used. In our study, we analyze the different physical properties of gas hydrate and other minerals bearing in unconsolidated marine sediments based on the effective medium theory. Then, a new attribute is put forward to discriminate gas hydrate. The logging data in the Dongsha area of the South China Sea and the Hydrate Ridge along the Oregon continental margin are applied to validate this method. Our test results are basically in line with actual situation, which provides a new approach in hydrate identification.
Does entrepreneurship ecosystem influence business re-entries after failure?
Authors: Guerrero Maribel , Espinoza-Benavides Jorge 
Published: August 2020

AbstractPrevious studies have found a close relationship between exit/failure decisions and entrepreneurial/organisational characteristics. In the same line, entrepreneurship literature has recognised that the context matters in any entrepreneurial process, including “exit,” “failure” or “re-entry.” This manuscript proposes a conceptual framework to identify the elements of the entrepreneurial ecosystem that foster or impede the re-entry into entrepreneurship after a business failure. By reviewing the accumulation of knowledge, we identified the individual, the organisational, and the contextual conditions that influence the trajectory of an individual who decides to re-enter after a business failure. This manuscript provides a better understanding of the critical role of agents involved in the entrepreneurial ecosystem. A provocative discussion and implications emerge for this study in order to reduce individual barriers and unfavourable social norms towards business failure.
Specific Patterns of Spinal Metabolite Ratio Underlying α-Me-5-HT-evoked Pruritus Compared with Compound 48/80 Based on Proton Nuclear Magnetic Resonance Spectroscopy
Authors: Chen Ying-le , He Zhi-gang , Wang Qian , Xiang Hong-bing , Fan Li , Xiong Jun 
Published: August 2020

SummaryMechanisms of pruritus are implicated in the dysregulation of the metabolites in the spinal cord. We investigated pruritus behavioral testing in three groups of young adult male C57Bl/6 mice, including one group treated with normal saline, while the other groups intradermally injected with α-Me-5-HT (histamine-independent pruritogen), compound 48/80 (histamine-dependent pruritogen) at the nape skin of the neck, respectively. Proton nuclear magnetic resonance spectroscopy (MRS) was used to compare spinal metabolites from the vertebral cervical among three groups, and to study the association of spinal metabolite ratio and pruritus intensity. The MRS-measured N-acetylaspartate-to-myoinositol ratio (NAA/Ins) was significantly correlated with the number of scratches between normal saline group and 48/80 group or α-Me-5-HT group (both P<0.0001), indicating that NAA/Ins may be a robust surrogate marker of histamine-independent/dependent pruritogen. There was significant difference in Glu/Ins between normal saline group and 48/80 group (P=0.017), indicating that Glu/Ins may be a surrogate marker of histamine-dependent pruritogen, while GABA/Ins was highly significantly different between normal saline group and α-Me-5-HT group (P=0.008), suggesting that GABA/Ins may be a surrogate marker of histamine-independent pruritogen. MRS may reflect the extent of pruritus intensity elicited by α-Me-5-HT and compound 48/80 with sensitivity similar to the number of scratches, and above potential markers need to be further validated in pre-clinical and clinical treatment trials.
Emerging Treatments for Advanced/Metastatic Pheochromocytoma and Paraganglioma
Authors: Ilanchezhian Maran , Jha Abhishek , Pacak Karel , Del Rivero Jaydira 
Published: August 2020

Opinion statementThe incidence of metastatic pheochromocytoma (PHEO) and paraganglioma (PGL) may occur in as many as 35% of patients particularly with PGL and even more frequently in those with specific mutations. Biochemical, morphological, and molecular markers have been investigated for use in the distinction of benign from malignant PHEO/PGL. PHEO/PGL metastasizes via hematogenous or lymphatic routes and shows differences based on mutational status. The most common sites of involvement in patients that have an SDHB mutation are the bone (78%), lungs (45%), lymph nodes (36%), and liver (35%). In patients with sporadic PHEO/PGL, the most common sites of metastasis are the bones (64%), lungs (47%), lymph nodes (36%), and liver (32%). Metastases may be present at presentation or may occur later. Metastases to the liver and lungs are associated with a shorter survival. Overall, the estimated 5-year survival rates are between 34 and 74%. Currently, treatments for metastatic PHEO/PGL are essentially palliative. Surgery is potentially curative; however, tumor dissemination limits the chance for a curative resection. When surgical intervention is not amenable, the therapeutic options include radiolabeled MIBG (Azedra®—iobenguane 131 was recently FDA-approved for patients > 12 years and older with iobenguane scan positive) or systemic chemotherapy with cyclophosphamide, vincristine, and dacarbazine (CVD) with an overall objective response rate (ORR) of less than 40%; however, it is not clear if the administration of CVD impacts overall survival, as nearly all patients develop progressive and ultimately fatal disease. Other treatment modalities under investigation include cytoreductive techniques, novel radiopharmaceuticals, chemotherapy, radiotherapy, immunotherapy, and experimental therapies. Here we are discussing emerging treatment for advanced/metastatic PHEO/PGL.
Rock physics attribute analysis for hydrocarbon prospectivity in the Eva field onshore Niger Delta Basin
Authors: Ogbamikhumi Alexander , Igbinigie Nosa Samuel 
Published: August 2020

AbstractDirect hydrocarbon indicator (DHI) expressions observed on seismic could arise due to various geological conditions. Such expression could lead to misinterpretation as hydrocarbon presence if not properly analyzed. This study employs rock physics attributes analysis to evaluate an identified prospect in the undrilled area of the studied reservoir. Prospect identification was actualized by analyzing structural and amplitude maps of the reservoir, which revealed a possible roll over anticline at both the exploited and prospective zone, with a very good amplitude support that conforms to structure. Well-based cross-plot analysis adopted four cross-plot techniques for feasibility study to test the applicability of rock physics for prospect evaluation in the field; Lambda-Rho versus Lambda-Rho/Mu-Rho ratio; Mu-Rho versus Lambda-Rho; and Poisson Ratio versus P-impedance. The result presented Poisson ratio, Lambda-Rho and Lambda/Mu-Rho ratio as good fluid indicator and Mu-Rho as a viable lithology indicator. As such, they were selected for seismic-based attribute and cross-plot analysis to validate the identified prospect. The results from seismic-based analysis showed consistency in the expression of the analyzed attribute at both the exploited and prospective zone. The seismic-based cross-plot analysis result was similar to the well-based analysis and was able to confirm that the observed amplitude expression in the exploited zone is an indication of hydrocarbon-bearing sand.
Some algebraic structures on the generalization general products of monoids and semigroups
Authors: Wazzan Suha Ahmad , Cevik Ahmet Sinan , Ates Firat 
Published: August 2020

AbstractFor arbitrary monoidsAandB, in Cevik et al. (Hacet J Math Stat 2019:1–11, 2019), it has been recently defined an extended version of the general product under the name ofa higher version of Zappa products for monoids(orgeneralized general product)$$A^{\oplus B}$$A⊕B$$_{\delta }\bowtie _{\psi }B^{\oplus A}$$δ⋈ψB⊕Aand has been introduced an implicit presentation as well as some theories in terms of finite and infinite cases for this product. The goals of this paper are to present some algebraic structures such as regularity, inverse property, Green’s relations over this new generalization, and to investigate some other properties and the product obtained by a left restriction semigroup and a semilattice.
Quality of life in Klinefelter patients on testosterone replacement therapy compared to healthy controls: an observational study on the impact of psychological distress, personality traits, and coping strategies
Authors: Fabrazzo M. , Accardo G. , Abbondandolo I. , Goglia G. , Esposito D. , Sampogna G. , Catapano F. , Giugliano D. , Pasquali D. 
Published: August 2020

Abstract
Purpose
We aimed to verify if 1 year-testosterone-replacement therapy could produce a psychopathological recovery and a satisfactory quality of life in Klinefelter syndrome (KS) patients compared to matched healthy controls. Further, we analyzed personality traits and coping strategies, an issue not yet examined in androgen-treated KS patients. We also enquired whether any of the sociodemographic and psychological variables might predict a patient’s general and sexual life satisfaction.

Methods
The Quality of Life Enjoyment and Satisfaction Questionnaire and the Temperament and Character Inventory-Revised were administered to both 23 KS patients and matched healthy subjects. Psychopathology was investigated by the Symptom Checklist-90-Revised (SCL-90-R) and the Mini-mental State Examination. The COPE Inventory was used to identify cognitive and behavioral strategies to manage disease-related distress.

Results
In testosterone-treated KS patients, when compared with controls, SCL-90-R subscales analysis evidenced high psychological distress, mainly presented as obsessive thoughts, hanger-hostility, phobias, and psychoticism. Self-directedness and self-transcendence, along with the prevalent use of emotion-focused coping strategies, outlined the personality of our KS patients. Depression and somatization proved to be predictors of general life dissatisfaction. Depression, anger-hostility, and paranoid ideation, instead, emerged as predictors of sexual life dissatisfaction.

Conclusion
Endocrinologists should cooperate with mental health providers to foster a better outcome of the disease in KS patients.
Multitasking scheduling with batch distribution and due date assignment
Authors: Xu Xinrui , Yin Guangqiang , Wang Chunyu 
Published: August 2020

AbstractThis study addresses the multitasking scheduling problems with batch distribution and due date assignment (DDA). Compared with classical scheduling problems with due date-related optimization functions, the job due dates are decision variables rather than given parameters. The jobs completed are distributed in batches, and the sizes of all batches are identical, which may be bounded or unbounded. The jobs in every batch are scheduled one by one. Each batch incurs a fixed cost. Under multitasking environment, it allows the machine to put an uncompleted job on hold and turn to another uncompleted job. The goal is to identify the optimal primary job sequence, the optimal job due dates, and the optimal batch production and distribution strategy so that one of the following two optimization functions is minimised: the total cost composed of the earliness penalty, DDA cost, tardiness penalty and batch distribution cost, and the total cost composed of the earliness penalty, weighted number of late jobs, DDA cost and batch distribution cost. We devise efficient exact algorithms for the problems we consider, and perform numerical experiments to check how multitasking affects the scheduling cost or value, the results of which can assist decision-makers to justify the extent to put to use or refrain from multitasking.
Adsorption control of a pipeline robot based on improved PSO algorithm
Authors: Yu Yilin , Xu Yanli , Wang Fusheng , Li Wensheng , Mai Xiaoming , Wu Hao 
Published: August 2020

AbstractParticle swarm optimization (PSO) is a widely used method that can provide good parameters for the motion controller of mobile robots. In this paper, an improved PSO algorithm that optimize the control PID parameters of a specific robot have been proposed. This paper first presents a brief review of recently proposed PSO methods, and then presents a detailed analysis of the PID optimization algorithm, which uses H∞ theory to reduce the search space and fuses the information entropy to ensure the diversity of particles. Simulations in Matlab show that the algorithm can improve the convergence speed and get a better global optimization ability than the standard PSO algorithm. Experimental results present a sound effects for the control of the negative pressure adsorption motor in the power grid pipeline robot during its adsorption along the circular movements, which verifies the effectiveness of the proposed method.
Role of oxidative stress in epileptogenesis and potential implications for therapy
Authors: Borowicz-Reutt Kinga K. , Czuczwar Stanisław J. 
Published: August 2020

AbstractIn a state of balance between oxidants and antioxidants, free radicals play an advantageous role of “redox messengers”. In a state of oxidative stress, they trigger a cascade of events leading to epileptogenesis. During this latent, free of seizures period, a cascade of neurological changes takes place and finally leads to spontaneous recurrent seizures. The main processes involved in seizure generation are: neuroinflammation, neurodegeneration with anomalous neuroregeneration and lowering seizure threshold. Time of epileptogenesis offers a unique therapeutic window to prevent or at least attenuate seizure development. Animal data indicate that some antioxidants (for instance, resveratrol) may bear an anti-epileptogenic potential.
A Survey of Usage of Penile Prosthesis
Authors: Knoll Paul , Rai Samarpit , Talluri Sriharsha , Bezinque Adam , Micciche Ross , Rao Ganesh , Ankem Murali K. 
Published: August 2020

Abstract

Background
Implantation of an inflatable penile prosthesis (IPP) has high success and satisfaction rates, but there remains a paucity of evidence examining non-usage of IPP and reasons for discontinuation.

Aim
To identify how frequent patients use their prosthesis and their personal reasons for no longer using it.

Methods
We conducted a survey of all patients who underwent an IPP implantation by a single surgeon over a 6-year period, between 2012 and 2018. After application of inclusion and exclusion criteria, a total of 114 patients formed the final cohort. Patients were initially surveyed via mail with a questionnaire; those who did not respond were surveyed via telephone. The factors determining patient selection for IPP implantation included suitability for general anesthesia, manual dexterity to use the device by the patient or their partner on a demonstration device, and presence of refractory erectile dysfunction, HbA1C lower than 8.5, or need for a revision of a previously placed IPP. Universally, a 3-piece AMS 700 Series implant was placed via the penoscrotal approach. Data were analyzed with Pearson chi square test, and survivability of the device was assessed with Kaplan-Meier survival curve.

Outcomes
The main outcomes of this study are the frequency of IPP usage and reasons for discontinuation.

Results
The survey participation rate was 97%. The mean age of patient was 64 years (range 34–83 years), and the mean time between surgery and completion of survey was 2.98 years (range 0.25–7.4 years). Kaplan-Meier curve demonstrated that 68% of the patients were using the IPP at 5 years after implantation. Using the age 70 years as a cutoff, 18 (22%) patients younger than 70 years and 14 (42%) patients older than 70 years discontinued using the IPP (P = .029). The commonest reasons for discontinuation were poor health to engage in sexual activity (2.6%), loss of companion (19%), loss of interest in sex (2.6%), and device malfunction with no further interest in revision of prosthesis (14%).

Clinical Implications
The clinical implication of this study was improved patient selection for device implantation.

Strengths and Limitations
To our knowledge, no other study has investigated reasons for patients no longer using their prosthesis. Our study has several limitations including that it is a cross-sectional analysis, our survey is not validated, this is a single-surgeon experience, we have a small sample size, and we did not differentiate between virgin implant and reimplantation.

Conclusion
Our study shows a high rate (28%) of non-usage of IPP, more so in men older than the age of 70 years in the first 5 years of implantation.
Synthesizing the Vertical Structure of Tropical Cirrus by Combining CloudSat Radar Reflectivity With In Situ Microphysical Measurements Using Bayesian Monte Carlo Integration
Authors: Liu Yuli , Mace Gerald G. 
Published: August 2020

AbstractCreating synthetic ice cloud profiles that are distributed according to realistic probability density functions is fundamentally essential for submillimeter wave radiometer studies. In this paper, we develop an algorithm to create synthetic ice cloud profiles by combing in situ microphysics measurements and spaceborne radar reflectivity observations using Bayesian Monte Carlo Integration (MCI). We first conduct comprehensive studies of the in situ data from the Tropical Composition, Cloud and Climate Coupling (TC4) experiment based on a bimodal particle size distribution (PSD) scheme and the realistic particle scattering properties for different crystal habits. Ice water content and radar reflectivity simulations from the in situ PSD are compared with the in situ measurements qualitatively and quantitatively. We then evaluate the Bayesian MCI by applying the algorithm to the Cloud Radar System (CRS) during the TC4 campaign and compare the brightness temperature simulations for the retrievals with the Compact Scanning Submillimeter Imaging Radiometer (CoSSIR) measurements. Finally, we apply the Bayesian MCI to the Cloud Profiling Radar (CPR) measurements on CloudSat, and we use the cumulative distribution functions (CDFs) and empirical orthogonal functions (EOFs) to preserve the one‐point statistics and the two‐point statistics that allow for the creation of any number of synthetic ice cloud profiles that are statistically consistent with the Bayesian retrieval results. The synthesized profiles are a useful characterization of the ice cloud vertical structure that combines two disparate data sources, and the synthesis algorithm constitutes an essential initial step for producing critical information in submillimeter radiometry studies.