bluuebunny/crossref_metadata_2025_split huggingface dataset:
Market conditions of international VET providers: a comparative analysis of Australia, UK, USA, and Germany
Authors: Peters Susanne 
Published: December 2021

AbstractVET (vocational education and training) is a highly complex, multidimensional worldwide phenomenon with diverse structures. Additionally, very different actors define the functions of a national (or even a regional) VET system. The paper contributes to a better understanding of the policy frameworks and current states of such systems. Therefore, we focus on selected VET systems in order to understand their specifics and thus, their market conditions. A qualitative approach is used to answer the research question regarding which conditions create or support market-based opportunities for the provision of commercial vocational training services. We find that the liberalism and deregulation of the VET sectors, as well as the marketisation of VET practices, lead to incentives to internationalise VET offers. Thinking in terms of skills, the kind of education system does not play a role. This is the case in liberal market-driven VET approaches (here, the UK, the USA and Australia) and is mirrored in the micro-analysis categories of curricula, learning location, content, and learning process.
De novo pathogenic variant in SETX causes a rapidly progressive neurodegenerative disorder of early childhood-onset with severe axonal polyneuropathy
Authors: Hadjinicolaou Aristides , Ngo Kathie J. , Conway Daniel Y. , Provias John P. , Baker Steven K. , Brady Lauren I. , Bennett Craig L. , La Spada Albert R. , Fogel Brent L. , Yoon Grace 
Published: December 2021

AbstractPathogenic variants in SETX cause two distinct neurological diseases, a loss-of-function recessive disorder, ataxia with oculomotor apraxia type 2 (AOA2), and a dominant gain-of-function motor neuron disorder, amyotrophic lateral sclerosis type 4 (ALS4). We identified two unrelated patients with the same de novo c.23C > T (p.Thr8Met) variant in SETX presenting with an early-onset, severe polyneuropathy. As rare private gene variation is often difficult to link to genetic neurological disease by DNA sequence alone, we used transcriptional network analysis to functionally validate these patients with severe de novo SETX-related neurodegenerative disorder. Weighted gene co-expression network analysis (WGCNA) was used to identify disease-associated modules from two different ALS4 mouse models and compared to confirmed ALS4 patient data to derive an ALS4-specific transcriptional signature. WGCNA of whole blood RNA-sequencing data from a patient with the p.Thr8Met SETX variant was compared to ALS4 and control patients to determine if this signature could be used to identify affected patients. WGCNA identified overlapping disease-associated modules in ALS4 mouse model data and ALS4 patient data. Mouse ALS4 disease-associated modules were not associated with AOA2 disease modules, confirming distinct disease-specific signatures. The expression profile of a patient carrying the c.23C > T (p.Thr8Met) variant was significantly associated with the human and mouse ALS4 signature, confirming the relationship between this SETX variant and disease. The similar clinical presentations of the two unrelated patients with the same de novo p.Thr8Met variant and the functional data provide strong evidence that the p.Thr8Met variant is pathogenic. The distinct phenotype expands the clinical spectrum of SETX-related disorders.
Floral organ-specific proteome profiling of the floral ornamental orchid (Cymbidium goeringii) reveals candidate proteins related to floral organ development
Authors: Chen Yue , Xu Zihan , Shen Qi , Sun Chongbo 
Published: December 2021

Abstract
Background
Cymbidium goeringii, belonging to the Orchidaceae family, is an important ornamental plant with striking petals and lips. Extremely diversified floral patterns and morphologies make C. goeringii good research material to examine floral development of orchids. However, no floral organ-specific protein has been identified yet. To screen floral development associated proteins, four proteomes from petal (PE), lip (LI), gynostemium (GY), and sepal (SE) were analyzed using Tandem Mass Tag-based proteomic analysis.

Results
A total of 6626 unique peptides encoding 2331 proteins were identified in our study. Proteins in several primary metabolic pathways, including amino acid metabolism, energy metabolism, and lipid metabolism, were identified as differentially expressed proteins. Interestingly, most of the energy metabolism-related proteins highly expressed in SE, indicating that SE is an important photosynthetic organ of C. goeringii flower. Furthermore, a number of phytohormone-related proteins and transcription factors (TFs) were identified in C. goeringii flowers. Expression analysis showed that 1-aminocyclopropane-1-carboxylate oxidase highly expressed in GY, IAA-amino acid hydrolase ILR1-like 4 and gibberellin receptor 1 C greatly expressed in LI, and auxin-binding protein ABP20 significantly expressed in SE, suggesting a significant role of hormones in the regulation of flower morphogenesis and development. For TFs, GY-highly expressed bHLH13, PE-highly expressed WRKY33, and GY-highly expressed VIP1, were identified.

Conclusions
Mining of floral organ differential expressed enzymes and TFs helps us to excavate candidate proteins related to floral organ development and to accelerate the breeding of Cymbidium plants.
A new theoretical understanding of big data analytics capabilities in organizations: a thematic analysis
Authors: Sabharwal Renu , Miah Shah Jahan 
Published: December 2021

AbstractBig Data Analytics (BDA) usage in the industry has been increased markedly in recent years. As a data-driven tool to facilitate informed decision-making, the need for BDA capability in organizations is recognized, but few studies have communicated an understanding of BDA capabilities in a way that can enhance our theoretical knowledge of using BDA in the organizational domain. Big Data has been defined in various ways and, the past literature about the classification of BDA and its capabilities is explored in this research. We conducted a literature review using PRISMA methodology and integrated a thematic analysis using NVIVO12. By adopting five steps of the PRISMA framework—70 sample articles, we generate five themes, which are informed through organization development theory, and develop a novel empirical research model, which we submit for validity assessment. Our findings improve effectiveness and enhance the usage of BDA applications in various Organizations.
Hydrocortisone, ascorbic acid, and thiamine (HAT) for sepsis and septic shock: a meta-analysis with sequential trial analysis
Authors: Na Weilan , Shen Huili , Li Yichu , Qu Dong 
Published: December 2021

AbstractBackgroundSepsis is a primary global health threat and costs a lot, requiring effective and affordable treatments. We performed this meta-analysis to explore the treatment of hydrocortisone, ascorbic acid, and thiamine (HAT) in sepsis and septic shock.MethodsWe searched Ovid MEDLINE, Embase, and the Cochrane Central Register of Controlled Trials from inception to August 14, 2021. We included randomized controlled trials (RCTs) that evaluated the HAT treatments in sepsis and septic shock. The primary outcome was the change in SOFA score over the 72 h. The second outcomes were the hospital, and 28-/30-day mortality, the duration of vasopressors, PCT clearance, hospital length of stay (LOS), and ICU LOS. We performed a subgroup analysis and a trial sequential analysis (TSA). The Der Simonian–Laird random-effects models were used to report the pooled risk ratios (RR) or mean difference (MD) with confidence intervals (CI).ResultsNine RCTs, enrolling 1427 patients of sepsis and septic shock treated with HAT (717) or only standard care (710), were included. There was a significant difference between the two groups in the change in SOFA score over the first 72 h (MD 0.65, 95% CI 0.30 to 1.00), the duration of vasopressors (MD − 18.16, 95% CI − 25.65 to − 10.68) and the PCT clearance (MD 14.54, 95% CI 0.64 to 28.43). In addition, there was no significant difference in the hospital mortality (RR 1.07, 95% CI 0.85 to 1.34), the 28-/30-day mortality (RR 0.96, 95% CI 0.80 to 1.15), the hospital LOS (MD 0.78, 95% CI − 0.30 to 1.86), and ICU LOS (MD 0.12, 95% CI − 0.53 to 0.78).ConclusionsThe HAT combination improves the SOFA score in the first 72 h and reduces the duration of vasopressors in patients with sepsis. Given the minor mean difference of the change in SOFA score, the mortality benefit has not been observed.Trial registrationPROSPERO, CRD42020203166.
Colonic metastasis of hepatocellular carcinoma with repeated retroperitoneal bleeding: a case report
Authors: Miyauchi Wataru , Yamamoto Manabu , Masahiro Makinoya , Shishido Yuji , Miyatani Kozo , Matsunaga Tomoyuki , Sakamoto Teruhisa , Fujiwara Yoshiyuki 
Published: December 2021

AbstractBackgroundColonic metastasis is uncommon in patients with hepatocellular carcinoma (HCC). In the past, extrahepatic metastasis of HCC was not treated aggressively because of its poor prognosis. Herein, we describe the case of a patient with HCC who survived for 30 months following resection of a metastatic tumor in the ascending colon.Case presentationAn 80-year-old man presented at our hospital with symptoms of abdominal pain on the right side and fever. He had undergone transcatheter arterial chemoembolization and posterior segment resection of the liver because of HCC, followed by radiofrequency ablation for a recurrent intrahepatic lesion 5 and 3 years, respectively, prior to the visit. He was diagnosed with retroperitoneal hematoma, which was thought to be associated with diverticulitis and an extramural tumor in the ascending colon. A definitive diagnosis could not be reached; however, a right hemicolectomy of the colon was performed because of progression to anemia. A pathological examination revealed a metastatic tumor in the ascending colon extending from the subserosal layer to the muscularis propria layer. The patient was treated with lenvatinib after surgery, but presented with intrahepatic recurrence, lymph node metastasis, and peritoneal dissemination metastasis 15 months later. The progression of the disease could not be controlled and his postoperative survival time was 30 months.ConclusionResection of metastasis of HCC might contribute to prolonged survival in cases, where radical resection is possible.
Temporary worsening of mitral regurgitation due to conduction disturbance after transcatheter aortic valve implantation
Authors: Sajima Takeyuki , Onimaru Taichi , Sawamura Shigehito 
Published: December 2021

Abstract
Background
Mitral regurgitation after transcatheter aortic valve implantation (TAVI) can be caused by various etiologies.

Case presentation
An 81-year-old woman with mild mitral regurgitation and complete right bundle branch block was scheduled to undergo TAVI under general anesthesia. After the deployment of the prosthetic valve, electrocardiography depicted a wide QRS wave and bradycardia, suggestive of complete atrioventricular block. Although there was no lesion indicative of tissue injury to the valve itself, worsening of mitral regurgitation was identified on transesophageal echocardiography. The hemodynamic condition was stable, and no additional procedure was performed. Electrocardiography depicted a return to a narrow QRS wave 3 days after surgery, and the mitral regurgitation appeared comparable to the preoperative grade. We assumed that the worsening of mitral regurgitation was caused by dyssynchrony in the left ventricle due to the conduction disorder.

Conclusions
Mitral regurgitation after TAVI needs observation, including the determination of the etiology and treatment principle.
Altered microRNAs in C3H10T1/2 cells induced by p.E95K mutant IHH signaling
Authors: Zhou Wei , Chen Luan , Wu Hao , Wang Ting , Ma Gang , Wang Baocheng , Wang Cong , Zhang Na , Zhang Yingtian , He Lin , Qin Shengying , Sun Xiaofang , Zhang Hai , Shen Lu 
Published: December 2021

Abstract
Background
Indian Hedgehog (IHH), an important cell signaling protein, plays a key regulatory role in development of cartilage and chondrogenesis. Earlier studies have shown that heterozygous missense mutations in IHH gene may cause brachydactyly type A1 (BDA1), an autosomal dominant inheritance disease characterized by apparent shortness or absence of the middle phalanges of all digits. MicroRNAs (miRNAs) have been found to be significant post-transcriptional regulators of gene expression and significantly influence the process of bone-development. Therefore, it is possible that miRNAs are involved in the mechanism underlying the development of BDA1. However, the relationship between miRNAs and the pathogenesis of BDA1 remains unclear.

Methods
In this study, we used microarray-based miRNA profiling to investigate the role of miRNAs in BDA1 by characterization of differentially expressed miRNAs in C3H10T1/2 cell line induced by wild type (WT) and p.E95K mutant (MT) IHH signaling.

Results
Our results identified 6 differentially expressed miRNAs between WT and control (CT) group and 5 differentially expressed miRNAs between MT and CT groups. In particular, miR-135a-1-3p was found to be a significantly differentially expressed miRNA between WT and CT group. Results of dual-luciferase reporter gene experiment successfully discovered Hoxd10 was one of the target gene of miR-135a-1-3p. Additionally, our pathway analysis revealed that the targets of these miRNAs of interest were highly involved with Runx1/2, Notch and collagen-related pathways.

Conclusions
Taken together, our findings provided important clue for future study of the process of miRNA-regulation in IHH signaling and novel insights into the regulatory role of miRNA in pathogenesis of BDA1.
Residence registration to cope with homelessness: evidence from a qualitative research study in Milan
Authors: Pasqualini M. , Bazzani G. 
Published: December 2021

AbstractHomeless people are one of the most vulnerable and marginalized groups in developed countries, and their homelessness situation often persists over the long term. However, so far, no studies have explained the specific role played by residence registration as it relates to deprivation amongst the homeless population and its contribution to improving the lives of homeless people. This paper investigates the paths homeless people in Milan use to access residence registration, via a case study in the city of Milan. Home to Italy’s largest homeless population, the city of Milan has implemented the innovative ResidenzaMi project to improve access to residence registration for homeless people. The study considers official statistics and individual interviews with service providers involved in the registration process. It further investigates the main factors impeding the registration process and outlines the consequences of the COVID-19 pandemic. Results from our study indicate that a residence certificate plays a critical role in helping homeless people exercise their rights and access the services they need to escape homelessness. Our findings suggest the importance of a holistic, multidimensional approach to ensure access to residence registration for homeless persons.
Eosinophilic granulomatosis with polyangiitis (Churg–Strauss syndrome) imitating Guillain–Barre syndrome (GBS): a case report
Authors: Khandelwal Dinesh , Singh Mridula , Jagota Ruchi , Mathur Vaibhav 
Published: December 2021

Abstract
Background
Eosinophilic granulomatosis with polyangiitis (EGPA) is associated with vasculitic neuropathy and being rare can present as subacute symmetric sensorimotor quadriparesis mimicking Guillain–Barre syndrome (GBS). It warrants timely diagnosis as treatment for both conditions is different and vasculitic neuropathy needs long-term immunosuppression. Nerve biopsy of our patient showed eosinophilic infiltration along with mononuclear infiltrate. Typical histopathological presentations of EGPA are different among different organs and eosinophilic infiltration is rarely observed in peripheral nerve and kidney involvements.

Case presentation
A 49-year-old female with a history of asthma with 3-week duration of acute onset ascending weakness, preceded by severe pain and burning in glove and stocking pattern. Nerve conduction studies could not rule out Guillain–Barre syndrome initially, but subsequent studies show axonal affection and she received intravenous immunoglobulin (IVIg) but her weakness progressed after slight improvement. Her bloodwork revealed marked eosinophilia (> 50%) with computed tomography (CT) paranasal sinuses showing pansinusitis with background history of asthma led us towards eosinophilic granulomatosis with polyangiitis and later antineutrophil cytoplasmic antibodies came out positive with nerve biopsy showing perivascular mononuclear inflammation with eosinophils. She was started on steroids immediately and then received intravenous rituximab in view of long-term immunosuppression with maintenance steroids and on follow-up she improved.

Conclusion
Eosinophilic granulomatosis with polyangiitis is a small-vessel vasculitis associated with antineutrophil cytoplasmic antibodies with significant paranasal sinuses involvement. Mononeuritis multiplex is the most common presentation of vasculitic neuropathy of eosinophilic granulomatosis with polyangiitis, but they can mimic Guillain–Barre syndrome and should always be considered in the differential diagnosis, since the treatment strategies for these conditions are radically different.
Treatment of Short Stature in Aggrecan-deficient Patients With Recombinant Human Growth Hormone: 1-Year Response
Authors: Muthuvel Gajanthan , Dauber Andrew , Alexandrou Eirene , Tyzinski Leah , Andrew Melissa , Hwa Vivian , Backeljauw Philippe 
Published: December 2021

Abstract

Context
Patients with aggrecan (ACAN) deficiency present with dominantly inherited short stature, often with advanced skeletal maturation and premature growth cessation. There is a paucity of information on the effects of growth-promoting interventions.

Objective
The aim of this study was to evaluate the efficacy and safety of recombinant human growth hormone (rhGH) therapy on linear growth in children with ACAN deficiency.

Methods
Open-label, single-arm, prospective study at Cincinnati Children’s Hospital Medical Center. Ten treatment-naïve patients were recruited. Inclusion criteria were a confirmed heterozygous mutation in ACAN, age ≥2 years, prepubertal, bone age (BA) ≥chronological age (CA), and normal insulin-like growth factor I concentration. Treatment was with rhGH (50 µg/kg/day) over 1 year. Main outcomes measured were height velocity (HV) and change in (Δ) height SD score (HtSDS).

Results
Ten patients (6 females) were enrolled with median CA of 5.6 years (range 2.4-9.7). Baseline median HtSDS was –2.5 (range –4.3 to –1.1). Median baseline BA was 6.9 years (range 2.5-10.0), with median BA/CA of 1.2 (range 0.9-1.5). Median pretreatment HV was 5.2 cm/year (range 3.8-7.1), increased to 8.3 cm/year (range 7.3-11.2) after 1 year of therapy (P = .004). Median ΔHtSDS after 1 year was +0.62 (range +0.35 to +1.39) (P = .002). Skeletal maturation did not advance inappropriately (median ΔBA/CA –0.1, P = .09). No adverse events related to rhGH were observed.

Conclusion
Treatment with rhGH improved linear growth in a cohort of patients with short stature due to ACAN deficiency.
An Indirect Treatment Comparison of Semaglutide 2.0 mg vs Dulaglutide 3.0 mg and 4.5 mg Using Multilevel Network Meta-regression
Authors: Lingvay Ildiko , Bauer Robert , Baker-Knight James , Lawson Jack , Pratley Richard 
Published: December 2021

Abstract

Aims
Currently, no head-to-head data are available comparing semaglutide 2.0 mg with dulaglutide 3.0 mg or 4.5 mg. We conducted an indirect treatment comparison (ITC) of their effects on glycated hemoglobin (HbA1c) and body weight in patients with type 2 diabetes.

Materials and methods
Multilevel network meta-regression was conducted, based on a connected evidence network of published results from the A Study of the Efficacy and Safety of Dulaglutide (LY2189265) in Participants With Type 2 Diabetes 11 trial and individual patient data from the A Research Study to Compare Two Doses of Semaglutide Taken Once Weekly in People With Type 2 Diabetes (SUSTAIN) and SUSTAIN 7 trials.

Results
Semaglutide 2.0 mg significantly reduced HbA1c vs dulaglutide 3.0 mg and 4.5 mg, with estimated treatment differences (ETDs) of –0.44% points (95% credible interval [CrI], –0.68 to –0.19) and –0.28% points (95% CrI, –0.52 to –0.03), respectively. Semaglutide 2.0 mg also significantly reduced body weight vs dulaglutide 3.0 mg and 4.5 mg with ETDs of –3.29 kg (95% CrI, –4.62 to −1.96) and –2.57 kg (95% CrI, –3.90 to –1.24), respectively. Odds of achieving HbA1c &lt; 7.0% were significantly greater for semaglutide 2.0 vs dulaglutide 3.0 mg (odds ratio [OR]: 2.23 [95% CrI, 1.15-3.90]), whereas this did not reach significance for semaglutide 2.0 mg vs dulaglutide 4.5 mg (OR: 1.58 [95% CrI, 0.82-2.78]). Sensitivity analyses supported the main analysis findings.

Conclusions
This ITC demonstrated significantly greater reductions from baseline in HbA1c and body weight with semaglutide 2.0 mg vs dulaglutide 3.0 mg and 4.5 mg. The findings of this study provide important comparative effectiveness information until randomized head-to-head studies become available.
Mendelian Randomization Analysis Reveals Causal Effects of Plasma Proteome on Body Composition Traits
Authors: Han Bai-Xue , Yan Shan-Shan , Xu Qian , Ni Jing-Jing , Wei Xin-Tong , Feng Gui-Juan , Zhang Hong , Li Bin , Zhang Lei , Pei Yu-Fang 
Published: December 2021

Abstract

Context
Observational studies have demonstrated associations between plasma proteins and obesity, but evidence of causal relationship remains to be studied.

Objective
We aimed to evaluate the causal relationship between plasma proteins and body composition.

Methods
We conducted a 2-sample Mendelian randomization (MR) analysis based on the genome-wide association study (GWAS) summary statistics of 23 body composition traits and 2656 plasma proteins. We then performed hierarchical cluster analysis to evaluate the structure and pattern of the identified causal associations, and we performed gene ontology enrichment analysis to explore the functional relevance of the identified proteins.

Results
We identified 430 putatively causal effects of 96 plasma proteins on 22 body composition traits (except obesity status) with strong MR evidence (P &lt; 2.53 × 10 − 6, at a Bonferroni-corrected threshold). The top 3 causal associations are follistatin (FST) on trunk fat-free mass (Beta = −0.63, SE = 0.04, P = 2.00 × 10−63), insulin-like growth factor-binding protein 1 (IGFBP1) on trunk fat-free mass (Beta = −0.54, SE = 0.03, P = 1.79 × 10−57) and r-spondin-3 (RSPO3) on WHR (waist circumference/hip circumference) (Beta = 0.01, SE = 4.47 × 10−4, P = 5.45 × 10−60), respectively. Further clustering analysis and pathway analysis demonstrated that the pattern of causal effect to fat mass and fat-free mass may be different.

Conclusion
Our findings may provide evidence for causal relationships from plasma proteins to various body composition traits and provide basis for further targeted functional studies.