bluuebunny/crossref_metadata_2025_split huggingface dataset:
Modeling Enterprise Information and Enabling Access Using Information Sharing Server
Authors: Karinathi R. , Jagannathan V. , Montan V. , Petro J. , Sobolewski M. , Raman R. , Trapp G. , Deng Stephen , Almasi G. , Li Xi 
Published: March 2021

Abstract
The engineering data of a large enterprise is typically distributed over a wide area and archived in a variety of databases and file systems. Access to such information is crucial to a team member, particularly in a concurrent engineering setting. However, this is not easy, because (1) a model of the relevant information is not available, and (2) there is no simple way to access the information without being knowledgeable about various computer data formats, file systems, and networks. However, in a concurrent engineering environment, there is every need to be aware of the perspectives of the other members of the team. We have developed a system called the Information Sharing Server (ISS) to enable access to diverse and distributed information within a corporation. Such data could be stored in different repositories such as databases (relational, object oriented, etc.) and file systems including those that contain multiple media (text, graphics, audio, etc.). The ISS maintains an enterprise model that is visible to the user. The modeling of the enterprise is done in a language called EXPRESS developed by the STEP consortium as an international standard. The ISS also stores mappings from the model to the actual data residing in the repositories. The ISS accepts requests from the user and converts them into requests specific to a repository. The request is then communicated to the repository over the network and the results are fetched back to the user. The ISS is currently integrated with engineering data of two domains: electrical and mechanical. Our paper describes the methodology of the ISS, the details of the implementation and extensions planned for the future. We believe the transparency offered by the ISS will make it a very useful tool for an engineer and make it very convenient to integrate heterogeneous legacy databases.
Integration of Knowledge-Based Systems and Heterogeneous Databases in Design Information Systems
Authors: Yang Yong , Sims Williams Jon H. , McMahon Chris A. , Pitt David J. 
Published: March 2021

Abstract
The process of design depends on the availability of both a variety of data and many different types of knowledge drawn from specialists. Most of the data is naturally stored in various databases while the knowledge can often be organised in Knowledge-based Systems (KBSs). A design support system is thus required to comprise a number of elements such as databases and knowledge-based systems, as well as conventional analytical modelling systems. In common with many software environments that have been developed over several years these elements tend to form autonomous islands based on perhaps different computers or data servers between which exchange of data is difficult. Even in the more recent expert system field, communication is limited between different advisory programs, or between such programs and the databases which they use for inference. One central issue in designing such a support system is to identify means by which multiple systems can co-operate in an inter-connected environment and share data which is distributed over heterogeneous databases. This paper describes an Interprocess Communication (IPC) based integration framework which is designed to facilitate dynamic and smooth data communication between the cooperating KBSs and databases within a design support system.
Glaucoma diagnostic capabilities of macular vessel density on optical coherence tomography angiography: superficial versus deep layers
Authors: Lee Jin Yeong , Shin Joong Won , Song Min Kyung , Hong Ji Wook , Kook Michael S 
Published: March 2021

Background/aimsTo compare glaucoma diagnostic capabilities of superficial and deep macular vessel density (mVD) parameters in a series of healthy and open-angle glaucoma (OAG) eyes with central visual field (CVF) loss.MethodsWe consecutively enrolled 113 eyes of 113 patients with OAG and 47 eyes of 47 healthy participants in a retrospective manner. Superficial and deep mVDs were measured at foveal, parafoveal and perifoveal locations on optical coherence tomography (OCT) angiography. The macular ganglion cell-inner plexiform layer thickness (mGCIPLT) was measured on OCT as a reference standard. Glaucoma diagnostic capabilities of superficial and deep mVD parameters were assessed according to the glaucoma stage. Factors associated with the CVF mean sensitivity (MS) were evaluated using linear regression analyses in the OAG eyes.ResultsGlaucoma diagnostic capabilities of superficial perifoveal and parafoveal mVDs were significantly better than those of deep perifoveal and parafoveal mVDs, regardless of the glaucoma stage (both p<0.05). Both mGCIPLT and superficial parafoveal mVD were significantly associated with CVF MS (β-coefficients=10.567 and 21.147, respectively, both p<0.05), independent of age and glaucoma severity.ConclusionSuperficial mVD parameters showed significantly greater glaucoma diagnostic capabilities and better correlation with CVF MS compared with deep mVD parameters.
Economic burden of asthma in Singapore
Authors: Finkelstein Eric A , Lau Eden , Doble Brett , Ong Bennett , Koh Mariko Siyue 
Published: March 2021

Background and objectiveUp-to-date economic burden of asthma in Singapore is currently unknown.MethodsWe quantify the per capita and total annual costs of asthma for adults and children by level of symptom control (uncontrolled, partly controlled, and well controlled) via a cross-sectional online survey administered to a national web panel. Participants were asked about healthcare utilisation, days missed from work, and reduced productivity due to their symptoms. These values were then monetised and multiplied by prevalence estimates of adult and child asthmatics to generate total costs.ResultsA total of 300 adults and 221 parents of children with asthma were included in analysis. The total annual cost of adult asthma was estimated to be SGD 1.74 billion (US$1.25 billion) with 42% coming from the uncontrolled group, 45% from the partly controlled group, and 13% from the well-controlled group. For children, the total cost is SGD 0.35 billion (US$0.25 billion), with 64%, 26% and 10% coming from each group respectively. Combined, the annual economic burden of asthma in Singapore is SGD 2.09 billion (US$1.50 billion) with 79% due to productivity losses.ConclusionPoorly controlled asthma imposes a significant economic burden. Therefore, better control of disease has the potential to generate not only health improvements, but also medical expenditure savings and productivity gains.
Disentangling molecular and clinical stratification patterns in beta-galactosidase deficiency
Authors: Tebani Abdellah , Sudrié-Arnaud Bénédicte , Dabaj Ivana , Torre Stéphanie , Domitille Laur , Snanoudj Sarah , Heron Benedicte , Levade Thierry , Caillaud Catherine , Vergnaud Sabrina , Saugier-Veber Pascale , Coutant Sophie , Dranguet Hélène , Froissart Roseline , Al Khouri Majed , Alembik Yves , Baruteau Julien , Arnoux Jean-Baptiste , Brassier Anais , Brehin Anne-Claire , Busa Tiffany , Cano Aline , Chabrol Brigitte , Coubes Christine , Desguerre Isabelle , Doco-Fenzy Martine , Drenou Bernard , Elcioglu Nursel H , Elsayed Solaf , Fouilhoux Alain , Poirsier Céline , Goldenberg Alice , Jouvencel Philippe , Kuster Alice , Labarthe François , Lazaro Leila , Pichard Samia , Rivera Serge , Roche Sandrine , Roggerone Stéphanie , Roubertie Agathe , Sigaudy Sabine , Spodenkiewicz Marta , Tardieu Marine , Vanhulle Catherine , Marret Stéphane , Bekri Soumeya 
Published: March 2021

IntroductionThis study aims to define the phenotypic and molecular spectrum of the two clinical forms of β-galactosidase (β-GAL) deficiency, GM1-gangliosidosis and mucopolysaccharidosis IVB (Morquio disease type B, MPSIVB).MethodsClinical and genetic data of 52 probands, 47 patients with GM1-gangliosidosis and 5 patients with MPSIVB were analysed.ResultsThe clinical presentations in patients with GM1-gangliosidosis are consistent with a phenotypic continuum ranging from a severe antenatal form with hydrops fetalis to an adult form with an extrapyramidal syndrome. Molecular studies evidenced 47 variants located throughout the sequence of the GLB1 gene, in all exons except 7, 11 and 12. Eighteen novel variants (15 substitutions and 3 deletions) were identified. Several variants were linked specifically to early-onset GM1-gangliosidosis, late-onset GM1-gangliosidosis or MPSIVB phenotypes. This integrative molecular and clinical stratification suggests a variant-driven patient assignment to a given clinical and severity group.ConclusionThis study reports one of the largest series of b-GAL deficiency with an integrative patient stratification combining molecular and clinical features. This work contributes to expand the community knowledge regarding the molecular and clinical landscapes of b-GAL deficiency for a better patient management.
Comparing the Corticosteroid Injection and A1 Pulley Percutaneous Release in Treatment of Trigger Finger: A Clinical Trial
Authors: Abdoli Abbas , Hashemizadeh Aghda Seyed Masoud , Jalil Abrisham Seyed Mohammad 
Published: March 2021

Background: Primary treatment of trigger digits is conservative including stretching, night splinting and combination of heat and ice. When these methods fail, invasive methods such as corticosteroid injection, percutaneous release and open surgery are used. The purpose of this study is to compare the efficacy of two outpatient methods of percutaneous trigger finger release (PTFR) and corticosteroid injection (CI). Methods: This study is a randomized clinical trial that was performed with 6-month follow up. A total of 83 patients with trigger finger treated either with corticosteroid injection (n:40) or percutaneous release of the A1 pulley (n:43) were enrolled in this study. Demographic data were recorded before intervention. Pain score (VAS criterion), disease stage (Quinnell criteria), patient satisfaction and complications such as paresthesia, scarring, and stiffness (decrease in the range of motion) were recorded after the intervention. We used SPSS program (statistical package for the social science SPSS version 16) to perform the analysis. Results: There were 18 male (21.7%) and 65 female (78.3%) patients, whose mean age was 52.54 ± 11.45 (28–85) years. There was a significant difference between the degree of pain at the time of the third, sixth weeks and sixth months in two groups. The degree of pain was lower in the CI group in the third and sixth weeks but it was lower in the PTFR group in the sixth month. Satisfaction of the patients in the sixth month was significantly higher in the PTFR group. The incidence of stiffness was also significantly lower in the PTFR group in the sixth month. Conclusions: Patients in PTFR group had greater recovery and satisfaction level and lower recurrence rate and pain. Therefore PTFR may be used as a substitute for CI in the treatment of trigger finger from the beginning especially in patients who do not want to have open surgery.
Epidemiology of Dupuytren’s Disease in Japanese General Population
Authors: Sasaki Norihiro , Uesato Ryoko , Yamauchi Taisei , Ishibashi Yasuyuki , Nakaji Shigeyuki 
Published: March 2021

Background: Dupuytren’s disease (DD) is well known as a fibromatous disorder of the palmar aponeurosis. Although there is a large body of literature on the etiology of DD in Europe, there have been few studies in Japan. The purpose of our study was to investigate the etiology and risk factors of DD in a large population in Japan. Methods: The subjects were voluntary participants from the 2014 Health Promotion Project, and 1,112 individuals were included (421 men and 691 women; mean age = 54.2 ± 15.3 years) in this study. The severity of DD was assessed using the Meyerding classification. All participants completed a questionnaire on age, sex, lifestyle, and occupations. All participants also completed the Short Form Health Survey (SF-36) to investigate the QOL. Fasting venous blood specimens were taken for biochemical analysis. Results: DD was found in 44 participants (3.9%). The prevalence was 8.3% among the men and 1.3% among the women. The prevalence in these older than 60 years was 7.7% (men; 18.5%, women; 2.4%). There were 17 participants that had DD of both hands. Multiple digits were affected in 9 participants. The ring finger was the most affected finger (71.2%), followed by the little finger (16.4%), and middle finger (12.3%). In Meyerding classification, 39 cases were stage 0, one case was stage 1, and four cases were stage 2. The multivariate logistic regression analysis revealed that age, sex, smoking, and occupation were significantly associated with DD. There was no significant association between DD and other parameters. When associations between DD and the SF-36 subscales were analyzed, there were significant associations with physical functioning, physical role functioning, and mental health. Conclusions: Our study is the largest in Japan to date. These results will provide very useful data to aid understanding of DD.
Surgery for Trapeziometacarpal Joint Osteoarthritis: A Meta-Analysis on Efficacy and Safety
Authors: Knightly Nauar , Sullivan Paul 
Published: March 2021

Background: Osteoarthritis of the thumb base is the second most prevalent arthritis of the hand. Management is primarily conservative, consisting of analgesia, splinting, physiotherapy, and steroid injections. Surgery is considered when conservative measures fail. Methods: The primary objective was to assess the safety and efficacy of the surgical interventions and therein, evaluate whether any superiority exists among the available interventions. Efficacy was evaluated by examining four parameters: pain, function, range of movement and strength of the joint postoperatively. Safety was determined by comparing the rate and severity of postoperative complications. A systematic search of MEDLINE (2014–2019), EMBASE (2014–2019), CINAHL (2014–2019) and CENTRAL (2014–2019) databases was carried out. Abstracts were screened for relevant studies. Randomised controlled trials were only considered. Results: Eight studies were included in the quantitative synthesis. The procedures evaluated are: Trapeziectomy (T), trapeziectomy with ligament reconstruction (T + LR), trapeziectomy with ligament reconstruction and tendon interposition (T + LRTI), trapeziectomy with allograft suspension (T + ALS) and joint arthrodesis (A). Low-moderate quality evidence suggests that T + LRTI yields better range of movement (palmar abduction) when compared with (T) alone; (SMD 0.61, 95% CI 0.22 to 1.00, random-effects, p = 0.002). Comparing adverse events showed that arthrodesis carries a greater risk of adverse events when compared with T + LRTI; (RR 0.33, 95% CI 0.17 to 0.61, random-effects, p = 0.0005). In addition, T + LRTI is preferred over arthrodesis by patients (OR 0.29 95% CI 0.09 to 0.95; p = 0.04). This difference was no seen in the other comparison groups. Conclusions: It is difficult to declare with any degree of certainty which procedure offers the best functional outcome and safety profile. Results suggest T + LRTI yields good postoperative range of movement. Arthrodesis demonstrated an unacceptably high rate of moderate-severe complications and should be considered with careful consideration.
Disparities in Hepatocellular Carcinoma Incidence, Stage, and Survival: A Large Population-Based Study
Authors: Flores Yvonne N. , Datta Geetanjali D. , Yang Liu , Corona Edgar , Devineni Divya , Glenn Beth A. , Bastani Roshan , May Folasade P. 
Published: March 2021

Abstract

Background:
Liver cancer is one of the most rapidly increasing cancers in the United States, and hepatocellular carcinoma (HCC) is its most common form. Disease burden and risk factors differ by sex and race/ethnicity, but a comprehensive analysis of disparities by socioeconomic status (SES) is lacking. We examined the relative impact of race/ethnicity, sex, and SES on HCC incidence, stage, and survival.

Methods:
We used Surveillance, Epidemiology, and End Results (SEER) 18 data to identify histologically confirmed cases of HCC diagnosed between January 1, 2000 and December 31, 2015. We calculated age-adjusted HCC incidence, stage at diagnosis (local, regional, distant, unstaged), and 5-year survival, by race/ethnicity, SES and sex, using SEER*Stat version 8.3.5.

Results:
We identified 45,789 cases of HCC. Incidence was highest among low-SES Asian/Pacific Islanders (API; 12.1) and lowest in high-SES Whites (3.2). Incidence was significantly higher among those with low-SES compared with high-SES for each racial/ethnic group (P &lt; 0.001), except American Indian/Alaska Natives (AI/AN). High-SES API had the highest percentage of HCC diagnosed at the local stage. Of all race/ethnicities, Blacks had the highest proportion of distant stage disease in the low- and high-SES groups. Survival was greater in all high-SES racial/ethnic groups compared with low-SES (P &lt; 0.001), except among AI/ANs. Black, low-SES males had the lowest 5-year survival.

Conclusions:
With few exceptions, HCC incidence, distant stage at diagnosis, and poor survival were highest among the low-SES groups for all race/ethnicities in this national sample.

Impact:
HCC prevention and control efforts should target low SES populations, in addition to specific racial/ethnic groups.
Relevance of Molecular Groups in Children with Newly Diagnosed Atypical Teratoid Rhabdoid Tumor: Results from Prospective St. Jude Multi-institutional Trials
Authors: Upadhyaya Santhosh A. , Robinson Giles W. , Onar-Thomas Arzu , Orr Brent A. , Johann Pascal , Wu Gang , Billups Catherine A. , Tatevossian Ruth G. , Dhanda Sandeep Kumar , Srinivasan Ashok , Broniscer Alberto , Qaddoumi Ibrahim , Vinitsky Anna , Armstrong Gregory T. , Bendel Anne E. , Hassall Tim , Partap Sonia , Fisher Paul G. , Crawford John R. , Chintagumpala Murali , Bouffet Eric , Gururangan Sridharan , Mostafavi Roya , Sanders Robert P. , Klimo Paul , Patay Zoltan , Indelicato Daniel J. , Nichols Kim E. , Boop Frederick A. , Merchant Thomas E. , Kool Marcel , Ellison David W. , Gajjar Amar 
Published: March 2021

Abstract

Purpose:
Report relevance of molecular groups to clinicopathologic features, germline SMARCB1/SMARCA4 alterations (GLA), and survival of children with atypical teratoid rhabdoid tumor (ATRT) treated in two multi-institutional clinical trials.

Materials and Methods:
Seventy-four participants with newly diagnosed ATRT were treated in two trials: infants (SJYC07: age &lt; 3 years; n = 52) and children (SJMB03: age 3–21 years; n = 22), using surgery, conventional chemotherapy (infants), or dose-dense chemotherapy with autologous stem cell rescue (children), and age- and risk-adapted radiotherapy [focal (infants) and craniospinal (CSI; children)]. Molecular groups ATRT-MYC (MYC), ATRT-SHH (SHH), and ATRT-TYR (TYR) were determined from tumor DNA methylation profiles.

Results:
Twenty-four participants (32%) were alive at time of analysis at a median follow-up of 8.4 years (range, 3.1–14.1 years). Methylation profiling classified 64 ATRTs as TYR (n = 21), SHH (n = 30), and MYC (n = 13), SHH group being associated with metastatic disease. Among infants, TYR group had the best overall survival (OS; P = 0.02). However, outcomes did not differ by molecular groups among infants with nonmetastatic (M0) disease. Children with M0 disease and &lt;1.5 cm2 residual tumor had a 5-year progression-free survival (PFS) of 72.7 ± 12.7% and OS of 81.8 ± 11%. Infants with M0 disease had a 5-year PFS of 39.1 ± 11.5% and OS of 51.8 ± 12%. Those with metastases fared poorly [5-year OS 25 ± 12.5% (children) and 0% (infants)]. SMARCB1 GLAs were not associated with PFS.

Conclusions:
Among infants, those with ATRT-TYR had the best OS. ATRT-SHH was associated with metastases and consequently with inferior outcomes. Children with nonmetastatic ATRT benefit from postoperative CSI and adjuvant chemotherapy.
VEGF-Induced Endothelial Podosomes via ROCK2-Dependent Thrombomodulin Expression Initiate Sprouting Angiogenesis
Authors: Kuo Cheng-Hsiang , Huang Yi-Hsun , Chen Po-Ku , Lee Gang-Hui , Tang Ming-Jer , Conway Edward M. , Shi Guey-Yueh , Wu Hua-Lin 
Published: March 2021

Objective:
VEGF (vascular endothelial growth factor) plays a critical role in physiological and pathological angiogenesis. Endothelial 3D podosomes (3DPs) are a type of F-actin-rich membrane microdomain, predominantly found in endothelial tip cells controlled by VEGF signaling during sprouting angiogenesis, such as occurs in retinal vasculature development. The molecular mechanisms governing 3DP formation have not been completely elucidated.

Approach and Results:
By using in vitro cell models and in vivo mouse models, we study the role of TM (thrombomodulin) in VEGF-induced endothelial 3DPs. Here, we report that VEGF can induce the expression of TM via ROCK2 (Rho-associated coiled-coil kinase 2). Furthermore, ROCK2 can catalyze the phosphorylated activation of ezrin to promote the association of the cytoplasmic domain of TM with F-actin in 3DPs and thereby promote the formation of 3DPs. We used endothelial cells transfected with different TM mutants as models to verify the role of TM domains in 3DPs and angiogenic activity. TM expression in endothelial cells augments angiogenic activity, a response that is dependent on the interaction of the cytoplasmic tail of TM with ezrin, and the integrity of the lectin-like domain of TM. Thus, as compared with wild-type counterparts, mice lacking the lectin-like domain of TM exhibit reduced neovascularization of granulation tissues during cutaneous wound healing and less retinal neovascularization in a model of oxygen-induced retinopathy.

Conclusions:
VEGF-ROCK2-ezrin-TM-F-actin axis promotes the formation of the lipid raft membrane-associated complex configuration, 3DP, which plays a critical role in mediating tube formation and cell migration of endothelial cells in sprouting angiogenesis.