bluuebunny/crossref_metadata_2025_split huggingface dataset:
Natural progression of cardiac features and long-term effects of enzyme replacement therapy in Taiwanese patients with mucopolysaccharidosis II
Authors: Lin Hsiang-Yu , Chen Ming-Ren , Lee Chung-Lin , Lin Shan-Miao , Hung Chung-Lieh , Niu Dau-Ming , Chang Tung-Ming , Chuang Chih-Kuang , Lin Shuan-Pei 
Published: February 2021

Abstract
Background
Cardiac abnormalities have been observed in patients with mucopolysaccharidosis type II (MPS II). The aim of this study was to investigate the cardiac features and natural progression of Taiwanese patients with MPS II, and evaluate the impact of enzyme replacement therapy (ERT) on cardiac structure and function.

Methods
The medical records and echocardiograms of 48 Taiwanese patients with MPS II (median age, 6.9 years; age range, 0.1–27.9 years) were reviewed. The relationships between age and each echocardiographic parameter were analyzed.

Results
The mean z-scores of left ventricular mass index (LVMI), interventricular septum diameter in diastole (IVSd), left ventricular posterior wall diameter in diastole (LVPWd), and aortic diameter were 1.10, 2.70, 0.95 and 1.91, respectively. Z scores > 2 were identified in 33%, 54%, 13%, and 46% for LVMI, IVSd, LVPWd, and aortic diameter, respectively. The most prevalent cardiac valve abnormality was mitral regurgitation (MR) (56%), followed by aortic regurgitation (AR) (33%). The severity of mitral stenosis (MS), MR, aortic stenosis (AS), AR, and the existence of valvular heart disease were all positively correlated with increasing age (p < 0.01). We also compared the echocardiographic parameters between two groups: (1) 12 patients who had up to 17 years of follow-up echocardiographic data without ERT, and (2) nine patients who had up to 12 years of follow-up data with ERT. The results showed that z-score changes of LVMI significantly improved in the patients who received ERT compared to those who did not receive ERT (0.05 versus 1.52, p < 0.05). However, the severity score changes of MS, MR, AS, and AR all showed gradual progression in both groups (p > 0.05).

Conclusions
High prevalence rates of valvular heart disease and cardiac hypertrophy were observed in the MPS II patients in this study. The existence and severity of cardiac hypertrophy and valvular heart disease in these patients worsened with increasing age, reinforcing the concept of the progressive nature of this disease. ERT for MPS II appeared to be effective in stabilizing or reducing the progression of cardiac hypertrophy, but it only had a limited effect on valvulopathy.
Use and impact of high intensity treatments in patients with traumatic brain injury across Europe: a CENTER-TBI analysis
Authors:   the CENTER-TBI investigators and participants, Huijben Jilske A. , Dixit Abhishek , Stocchetti Nino , Maas Andrew I. R. , Lingsma Hester F. , van der Jagt Mathieu , Nelson David , Citerio Giuseppe , Wilson Lindsay , Menon David K. , Ercole Ari 
Published: February 2021

Abstract
Purpose
To study variation in, and clinical impact of high Therapy Intensity Level (TIL) treatments for elevated intracranial pressure (ICP) in patients with traumatic brain injury (TBI) across European Intensive Care Units (ICUs).

Methods
We studied high TIL treatments (metabolic suppression, hypothermia (< 35 °C), intensive hyperventilation (PaCO2 < 4 kPa), and secondary decompressive craniectomy) in patients receiving ICP monitoring in the ICU stratum of the CENTER-TBI study. A random effect logistic regression model was used to determine between-centre variation in their use. A propensity score-matched model was used to study the impact on outcome (6-months Glasgow Outcome Score-extended (GOSE)), whilst adjusting for case-mix severity, signs of brain herniation on imaging, and ICP.

Results
313 of 758 patients from 52 European centres (41%) received at least one high TIL treatment with significant variation between centres (median odds ratio = 2.26). Patients often transiently received high TIL therapies without escalation from lower tier treatments. 38% of patients with high TIL treatment had favourable outcomes (GOSE ≥ 5). The use of high TIL treatment was not significantly associated with worse outcome (285 matched pairs, OR 1.4, 95% CI [1.0–2.0]). However, a sensitivity analysis excluding high TIL treatments at day 1 or use of metabolic suppression at any day did reveal a statistically significant association with worse outcome.

Conclusion
Substantial between-centre variation in use of high TIL treatments for TBI was found and treatment escalation to higher TIL treatments were often not preceded by more conventional lower TIL treatments. The significant association between high TIL treatments after day 1 and worse outcomes may reflect aggressive use or unmeasured confounders or inappropriate escalation strategies.

Take home message
Substantial variation was found in the use of highly intensive ICP-lowering treatments across European ICUs and a stepwise escalation strategy from lower to higher intensity level therapy is often lacking. Further research is necessary to study the impact of high therapy intensity treatments.

Trial registration

The core study was registered with ClinicalTrials.gov, number NCT02210221, registered 08/06/2014, https://clinicaltrials.gov/ct2/show/NCT02210221?id=NCT02210221&draw=1&rank=1 and with Resource Identification Portal (RRID: SCR_015582).
MEDALT: single-cell copy number lineage tracing enabling gene discovery
Authors: Wang Fang , Wang Qihan , Mohanty Vakul , Liang Shaoheng , Dou Jinzhuang , Han Jincheng , Minussi Darlan Conterno , Gao Ruli , Ding Li , Navin Nicholas , Chen Ken 
Published: February 2021

AbstractWe present a Minimal Event Distance Aneuploidy Lineage Tree (MEDALT) algorithm that infers the evolution history of a cell population based on single-cell copy number (SCCN) profiles, and a statistical routine named lineage speciation analysis (LSA), whichty facilitates discovery of fitness-associated alterations and genes from SCCN lineage trees. MEDALT appears more accurate than phylogenetics approaches in reconstructing copy number lineage. From data from 20 triple-negative breast cancer patients, our approaches effectively prioritize genes that are essential for breast cancer cell fitness and predict patient survival, including those implicating convergent evolution.The source code of our study is available at https://github.com/KChen-lab/MEDALT.
Study protocol: a core outcome set for perinatal interventions for congenital diaphragmatic hernia
Authors: Vergote Simen , De Bie Felix , Bosteels Jan , Hedrick Holly , Duffy James , Power Beverley , Benachi Alexandra , De Coppi Paolo , Fernandes Caraciolo , Lally Kevin , Reiss Irwin , Deprest Jan 
Published: February 2021

Abstract
Background
Congenital diaphragmatic hernia (CDH) is, depending of the severity, a birth defect associated with significant mortality and morbidity. Prenatal screening by ultrasound may detect this condition and comprehensive assessment of severity is possible, allowing for in utero referral to an experienced centre for planned delivery. In an effort to improve outcomes, prenatal interventions to stimulate lung development were proposed. Along the same lines, new postnatal management strategies are being developed. In order to enable proper comparison of novel perinatal interventions as well as outcomes, a set of uniform and relevant outcome measures is required. Core outcome sets (COS) are agreed, clearly defined sets of outcomes to be measured in a standardised manner and reported consistently. Herein we aim to describe the methodology we will use to define a COS for perinatal and neonatal outcomes of foetuses and newborns with congenital diaphragmatic hernia and to draft a dissemination and implementation plan.

Methods
We will use the methodology described in the Core Outcome Measures in Effectiveness Trials (COMET) Initiative Handbook. An international steering group will be created to guide the development of the COS. We are systematically reviewing the literature to identify all potential relevant pre- and neonatal outcomes previously used in studies on perinatal interventions for CDH. We will build a consensus on these core outcomes in a stakeholder group using the Delphi method. After completion, a stakeholder meeting will decide on a final COS, using a modified Nominal Group Technique. Thereafter, we will review potential definitions and measurements of these outcomes, and again a consensus meeting will be organised, to finalise the COS before dissemination.

Discussion
We have started a procedure to develop a COS for studies on perinatal interventions for congenital diaphragmatic hernia, with the purpose of improving the quality of research, guide clinical practice and improve patient care and eventual use in future clinical trials, systematic reviews and clinical practice guidelines.

Trial registration
We prospectively registered this study in the International Prospective Register of Systematic Reviews (PROSPERO) (registration number: CRD42019124399) and The Core Outcome Measures in Effectiveness Trials (COMET) Initiative (registration number:1296).
Synthesis, antibiotic structure–activity relationships, and cellulose dissolution studies of new room-temperature ionic liquids derived from lignin
Authors: Liu Shihong , Gonzalez Michael , Kong Celine , Weir Scott , Socha Aaron M. 
Published: February 2021

Abstract
Background
Ionic liquids (ILs) are promising pretreatment solvents for lignocellulosic biomass, but are largely prepared from petroleum precursors. Benzaldehydes from depolymerized lignin, such as vanillin, syringaldehyde, and 4-methoxy benzaldehyde, represent renewable feedstocks for the synthesis of ionic liquids. We herein report syntheses of novel lignin-derived ionic liquids, with extended N-alkyl chains, and examine their melting points, cellulose dissolution capacities, and toxicity profiles against Daphnia magna and E. coli strain 1A1. The latter organism has been engineered to produce isoprenol, a drop-in biofuel and precursor for commodity chemicals.

Results
The new N,N-diethyl and N,N-dipropyl methyl benzylammonium ILs were liquids at room temperature, showing 75–100 °C decreased melting points as compared to their N,N,N-trimethyl benzylammonium analog. Extension of N-alkyl chains also increased antibacterial activity threefold, while ionic liquids prepared from vanillin showed 2- to 4-fold lower toxicity as compared to those prepared from syringaldehyde and 4-methoxybenzaldehyde. The trend of antibacterial activity for anions of lignin-derived ILs was found to be methanesulfonate < acetate < hydroxide. Microcrystalline cellulose dissolution, from 2 to 4 wt% after 20 min at 100 °C, was observed in all new ILs using light microscopy and IR spectroscopy.

Conclusions
Ionic liquids prepared from H-, S- and G-lignin oxidation products provided differential cytotoxic activity against E. coli and D. magna, suggesting these compounds could be tailored for application specificity within a biorefinery.
HDL cholesterol efflux capacity and lipid profile in patients with systemic sclerosis
Authors: Ferraz-Amaro Iván , Delgado-Frías Esmeralda , Hernández-Hernández Vanesa , Sánchez-Pérez Hiurma , de Armas-Rillo Laura , Armas-González Estefanía , Machado José David , Diaz-González Federico 
Published: February 2021

Abstract
Objective
It is well established that patients with systemic sclerosis (SSc) have a disrupted lipid profile and an increased cardiovascular risk. Cholesterol efflux capacity (CEC), the ability of high-density lipoprotein (HDL)-cholesterol to accept cholesterol from macrophages, has been linked to cardiovascular events. The aim of this study was to establish whether CEC and lipid profile were impaired in SSc patients with respect to controls and whether these changes were associated with disease-related data.

Methods
Cross-sectional study encompassed 188 individuals: 73 SSc patients and 115 controls. CEC, using an in vitro assay, and lipoprotein serum concentrations were assessed in patients and controls. A multivariable analysis was performed to study the differences in CEC between patients and controls, and if SSc-related data could explain such differences.

Results
The multivariable analysis adjusted for demographic characteristics, cardiovascular risk factors, and lipid-related molecules showed that total cholesterol (beta coefficient: − 22 [95%CI – 37 to – 7], p = 0.004), triglycerides (beta coefficient: 24 [95%CI 2–47], p = 0.033), lipoprotein A (beta coefficient: 22 [95%CI 2–43], p = 0.033), and CEC (beta coefficient: – 6 [95%CI − 10 to – 2]%,p = 0.002) were significantly different between patients and controls. Skin thickness, as assessed by modified Rodnan skin score, was independently associated with a lower CEC (beta coefficient: – 0.21 [95%CI – 0.37 to – 0.05]%, p = 0.011) after multivariable adjustment.

Conclusion
SSc patients show an abnormal lipid profile with respect to controls including CEC. Skin thickness is independent and inversely associated with CEC in SSc patients.
CRBP-TS - evaluation of a home-based training and health care program for colorectal, breast, and prostate cancer using telemonitoring and self-management: study protocol for a randomized controlled trial
Authors: Falz Roberto , Thieme René , Tegtbur Uwe , Bischoff Christian , Leps Christian , Hillemanns Peter , Kohlhaw Kay , Klempnauer Jürgen , Lordick Florian , Stolzenburg Jens-Uwe , Aktas Bahriye , Weitz Jürgen , Bork Ulrich , Wimberger Pauline , Thomas Christian , Biemann Ronald , Jansen-Winkeln Boris , Schulze Antina , Gockel Ines , Busse Martin 
Published: February 2021

Abstract
Background
Physical training is recommended in various national and international guidelines for patients with cancer. Observational studies have shown that physical activity leads to reduced recurrence and mortality rates by 20–40% in colorectal, breast, and prostate cancer. Despite existing evidence, a systematic care structure is still lacking. The primary aim of this study is to implement and evaluate an online training platform to strengthen physical performance and patient empowerment after cancer surgery.

Methods
The evaluation will be conducted as a prospective multicenter randomized controlled trial with three subgroups (colorectal-, breast-, and prostate cancer). Each group will include 100 patients (total 300 patients including dropouts; clinical stages T1–3 and/or N+; M0 after surgery intervention) and the primary endpoint (13% increase in the maximal oxygen consumption during exercise) will be examined. The intervention group will receive a 6-month home-based online training (2–3 times per week strength-endurance training using video presentations), bidirectional activity feedback information, online communication, and online counseling. The control group (usual care) will be advised lifestyle improvement. In-hospital testing will be performed before, during, and after the intervention. In addition to cardiopulmonary capacity, tumor specific diagnostics (liquid biopsy, depression and fatigue assessment, metabolic and endothelial screening) will be applied.

Discussion
Due to the increasing incidence of cancer, associated with considerable mortality, morbidity and impaired quality of life, there is an imperative requirement for improved cancer care, of which structured physical training may become an integral component.

Trial registration
DRKS-ID: DRKS00020499; Registered 17 March 2020.
Mechanism of mesenchymal stem cells in spinal cord injury repair through macrophage polarization
Authors: An Nan , Yang Jiaxu , Wang Hequn , Sun Shengfeng , Wu Hao , Li Lisha , Li Meiying 
Published: February 2021

AbstractTreatment and rehabilitation of spinal cord injury (SCI) is a major problem in clinical medicine. Modern medicine has achieved minimal progress in improving the functions of injured nerves in patients with SCI, mainly due to the complex pathophysiological changes that present after injury. Inflammatory reactions occurring after SCI are related to various functions of immune cells over time at different injury sites. Macrophages are important mediators of inflammatory reactions and are divided into two different subtypes (M1 and M2), which play important roles at different times after SCI. Mesenchymal stem cells (MSCs) are characterized by multi-differentiation and immunoregulatory potentials, and different treatments can have different effects on macrophage polarization. MSC transplantation has become a promising method for eliminating nerve injury caused by SCI and can help repair injured nerve tissues. Therapeutic effects are related to the induced formation of specific immune microenvironments, caused by influencing macrophage polarization, controlling the consequences of secondary injury after SCI, and assisting with function recovery. Herein, we review the mechanisms whereby MSCs affect macrophage-induced specific immune microenvironments, and discuss potential avenues of investigation for improving SCI treatment.
Local well-posedness of the inertial Qian–Sheng’s Q-tensor dynamical model near uniaxial equilibrium
Authors: Wang Xiaoyuan , Li Sirui , Wang Tingting 
Published: February 2021

AbstractWe consider the inertial Qian–Sheng’s Q-tensor dynamical model for the nematic liquid crystal flow, which can be viewed as a system coupling the hyperbolic-type equations for the Q-tensor parameter with the incompressible Navier–Stokes equations for the fluid velocity. We prove the existence and uniqueness of local in time strong solutions to the system with the initial data near uniaxial equilibrium. The proof is mainly based on the classical Friedrich method to construct approximate solutions and the closed energy estimate.
New trapezium type inequalities of coordinated distance-disturbed convex type functions of higher orders via extended Katugampola fractional integrals
Authors: Kashuri Artion , Iqbal Sajid , Liko Rozana , Samraiz Muhammad , Abdeljawad Thabet 
Published: February 2021

AbstractIn this paper we establish some new results on trapezium type inequalities of coordinated distance-disturbed$(\ell _{1},h_{1})$(ℓ1,h1)–$(\ell _{2},h_{2})$(ℓ2,h2)-convex functions of higher orders$(\sigma _{1},\sigma _{2})$(σ1,σ2)by using the Katugampola$(k_{1},k_{2})$(k1,k2)-fractional integrals. As special cases of our general results, we recapture some earlier proved results.
New discrete inequalities of Hermite–Hadamard type for convex functions
Authors: Mohammed Pshtiwan Othman , Abdeljawad Thabet , Alqudah Manar A. , Jarad Fahd 
Published: February 2021

AbstractWe introduce new time scales on$\mathbb{Z}$Z. Based on this, we investigate the discrete inequality of Hermite–Hadamard type for discrete convex functions. Finally, we improve our result to investigate the discrete fractional inequality of Hermite–Hadamard type for the discrete convex functions involving the left nabla and right delta fractional sums.
Using Kano diagrams to display the most cited article types, affiliated countries, authors and MeSH terms on spinal surgery in recent 12 years
Authors: Chou Po-Hsin , Yeh Yu-Tsen , Kan Wei-Chih , Chien Tsair-Wei , Kuo Shu-Chun 
Published: February 2021

AbstractBackgroundCitation analysis has been increasingly applied to assess the quantity and quality of scientific research in various fields worldwide. However, these analyses on spinal surgery do not provide visualization of results. This study aims (1) to evaluate the worldwide research citations and publications on spinal surgery and (2) to provide visual representations using Kano diagrams onto the research analysis for spinal surgeons and researchers.MethodsArticle abstracts published between 2007 and 2018 were downloaded from PubMed Central (PMC) in 5 journals, includingSpine, European Spine Journal, The Spine Journal, Journal of Neurosurgery: Spine, and Journal of Spinal Disorders and Techniques. The article types, affiliated countries, authors, and Medical subject headings (MeSH terms) were analyzed by the number of article citations using x-index. Choropleth maps and Kano diagrams were applied to present these results. The trends of MeSH terms over the years were plotted and analyzed.ResultsA total of 18,808 publications were extracted from the PMC database, and 17,245 were affiliated to countries/areas. The 12-year impact factor for the five spine journals is 5.758. We observed that (1) the largest number of articles on spinal surgery was from North America (6417, 37.21%).Spineearns the highest x-index (= 82.96). Comparative Study has the highest x-index (= 66.74) among all article types. (2) The United States performed exceptionally in x-indexes (= 56.86 and 44.5) on both analyses done on the total 18,808 and the top 100 most cited articles, respectively. The most influential author whose x-index reaches 15.11 was Simon Dagenais from the US. (3) The most cited MeSH term with an x-index of 23.05 was surgery based on the top 100 most cited articles. The most cited article (PMID = 18164449) was written by Dagenais and his colleagues in 2008. The most productive author was Michael G. Fehlings, whose x-index and the author's impact factor are 13.57(= √(13.16*14)) and 9.86(= 331.57/33.64), respectively.ConclusionsThere was a rapidly increasing scientific productivity in the field of spinal surgery in the past 12 years. The US has extraordinary contributions to the publications. Furthermore, China and Japan have increasing numbers of publications on spinal surgery. This study with Kano diagrams provides an insight into the research for spinal surgeons and researchers.
Highly sensitive terahertz non‐destructive testing technology for stone relics deterioration prediction using SVM-based machine learning models
Authors: Meng Tianhua , Huang Rong , Lu Yuhe , Liu Hongmei , Ren Jianguang , Zhao Guozhong , Hu Weidong 
Published: February 2021

Abstract
The hollowing deterioration of stone relics required effective non-destructive testing (NDT) methods for their timely restoration and maintenance. To this end, a new NDT method based on terahertz (THz) technology by using support vector machine (SVM)-based machine learning models was developed to assess and diagnose the hollowing deterioration of the Yungang Grottoes. According to experiment design, a series of hollowing deterioration samples with various thicknesses of hollowing deterioration were prepared and then measured by using THz time-domain spectroscopy (THz-TDS). Based on the THz-TDS results of 30 randomly selected samples, a SVM-based hollowing deterioration prediction model (SVM-HDPM) was established by analyzing the relationship between the hollowing samples and the THz spectral information. The reliability and accuracy of the model was further proved by verified and compared with using the THz spectral data of the remaining 10 samples. The experimental results with the linear kernel function greatly demonstrated that the SVM-HDPM can have superior prediction accuracy, implying that the model is feasible for the prediction the hollowing deterioration of the stone relics. Moreover, one data preprocess was introduced into SVM-HDPM to meet the needs of field-based test. The predicted results of five different hollowing deterioration with different flaked stone thickness revealed good performance with very low mean square error (MSE) value. Therefore, it is believed that the proposed method can be regarded as an effective NDT technique with practical applications in analyzing cultural relics and have promising future prospects in inspection stone relics-like ancient heritage for hidden flaws.