bluuebunny/crossref_metadata_2025_split huggingface dataset:
Successful treatment of lathosterolosis: A rare defect in cholesterol biosynthesis—A case report and review of literature
Authors: Yaplito‐Lee Joy , Pai Gautham , Hardikar Winita , Hong Kai M. , Pitt James , Marum Justine , Amor David J. 
Published: August 2020

AbstractLathosterolosis is a rare autosomal recessive disorder of cholesterol biosynthesis. It is caused by defects in the SC5D (sterol C5‐desaturase) gene which encodes for the 3‐beta‐hydroxysteroid‐delta‐5‐desaturase (also called sterol‐C5‐desaturase or lathosterol dehydrogenase). Only six cases have been described in the literature, but it is possible that a number of patients with milder forms of the condition might have been missed. Lathosterolosis manifests as microcephaly, bilateral cataracts, dysmorphism, limb anomalies, and developmental delay/intellectual disability. Liver involvement is variable and can range from normal liver function tests to portal fibrosis and cirrhosis. Diagnosis is made by demonstration of specific mutations in the SC5D gene and by plasma sterol analysis to confirm elevated lathosterol levels. In this report, we describe a girl with transaminitis in association with developmental delay/intellectual disability, facial dysmorphism, limb anomalies, and bilateral cataracts. Fibroscan showed severe liver fibrosis. Plasma sterol analysis and exome sequencing confirmed the diagnosis of lathosterolosis. Simvastatin treatment resulted in lowering of plasma lathosterol levels, improvement in transaminitis, and liver fibrosis grade, suggesting that children with this condition should be actively treated in order to prevent progression of liver disease.
Differentiation of COVID‐19 from seasonal influenza: A multicenter comparative study
Authors: Zhang Jianguo , Ding Daoyin , Huang Xing , Zhang Jinhui , Chen Deyu , Fu Peiwen , Shi Yinghong , Xu Wenrong , Tao Zhimin 
Published: August 2020

AbstractAs coronavirus disease 2019 (COVID‐19) crashed into the influenza season, clinical characteristics of both infectious diseases were compared to make a difference. We reported 211 COVID‐19 patients and 115 influenza patients as two separate cohorts at different locations. Demographic data, medical history, laboratory findings, and radiological characters were summarized and compared between two cohorts, as well as between patients at the intensive care unit (ICU) andnon‐ICU within the COVID‐19 cohort. For all 326 patients, the median age was 57.0 (interquartile range: 45.0–69.0) and 48.2% was male, while 43.9% had comorbidities that included hypertension, diabetes, bronchitis, and heart diseases. Patients had cough (75.5%), fever (69.3%), expectoration (41.1%), dyspnea (19.3%), chest pain (18.7%), and fatigue (16.0%), etc. Both viral infections caused substantial blood abnormality, whereas the COVID‐19 cohort showed a lower frequency of leukocytosis, neutrophilia, or lymphocytopenia, but a higher chance of creatine kinase elevation. A total of 7.7% of all patients possessed no abnormal sign in chest computed tomography (CT) scans. For both infections, pulmonary lesions in radiological findings did not show any difference in their location or distribution. Nevertheless, compared to the influenza cohort, the COVID‐19 cohort presented more diversity in CT features, where certain specific CT patterns showed significantly more frequency, including consolidation, crazy paving pattern, rounded opacities, air bronchogram, tree‐in‐bud sign, interlobular septal thickening, and bronchiolar wall thickening. Differentiable clinical manifestations and CT patterns may help diagnose COVID‐19 from influenza and gain a better understanding of both contagious respiratory illnesses.
Malignant peripheral nerve sheath tumors in children, adolescents, and young adults: Treatment results of five Cooperative Weichteilsarkom Studiengruppe (CWS) trials and one registry
Authors: Meister Michael T. , Scheer Monika , Hallmen Erika , Stegmaier Sabine , Vokuhl Christian , von Kalle Thekla , Fuchs Jörg , Münter Marc , Niggli Felix , Ladenstein Ruth , Kazanowska Bernarda , Ljungman Gustaf , Bielack Stefan , Koscielniak Ewa , Klingebiel Thomas ,   Cooperative Weichteilsarkom Studiengruppe [CWS]
Published: August 2020

AbstractBackground and ObjectivesMalignant peripheral nerve sheath tumors (MPNST) are aggressive soft tissue sarcomas that present as large, invasive tumors. Our aim was to assess outcomes, identify prognostic factors, and analyze treatment strategies in a prospectively collected pediatric cohort.MethodsPatients less than 21 years with MPNST treated in the consecutive prospective European Cooperative Weichteilsarkom Studiengruppe (CWS)‐trials (1981‐2009) and the CWS‐SoTiSaR registry (2009‐2015) were analyzed.ResultsA total of 159 patients were analyzed. Neurofibromatosis type I (NF1) was reported in thirty‐eight patients (24%). Most were adolescents (67%) with large (>10 cm, 65%) tumors located at extremities (42%). Nodal involvement was documented in 15 (9%) and distant metastases in 15 (9%) upon diagnosis. Overall, event‐free survival (EFS) was 40.5% at 5 and 36.3% at 10 years, and overall survival (OS) was 54.6% at 5 and 47.1% at 10 years. Age, NF1 status, tumor site, tumor size, Intergroup Rhabdomyosarcoma Study (IRS) group, metastatic disease, and achieving first complete remission (CR1) were identified as prognostic factors for EFS and/or OS in the univariate analysis.ConclusionsPrognostic factors were identified and research questions for future clinical trials were addressed.
Sentinel lymph node biopsy predicts systemic recurrence in digital papillary adenocarcinoma
Authors: Bartelstein Meredith K. , Schwarzkopf Eugenia , Busam Klaus J. , Brady Mary Sue , Athanasian Edward A. 
Published: August 2020

AbstractBackground and ObjectivesDigital papillary adenocarcinoma (DPA) is a rare, aggressive neoplasm of sweat gland origin. It can recur at local, regional, or distant sites. There is limited knowledge about the role of sentinel lymph node biopsy (SLNB) in predicting recurrence in these patients. We present our experience with this uncommon tumor to evaluate the role of SLNB in predicting outcome.MethodsMedical records of all patients who underwent surgical treatment for biopsy‐proven upper extremity DPA at the study institution were reviewed. Descriptive statistics and Fisher's exact test were used to analyze data.ResultsTwenty‐one patients were identified. Most patients were male (71%), and the median age was 51 years. SLNB was performed in 18 patients; three were positive for nodal metastatic disease (17%). At a median follow‐up of 53 months, there were no local recurrences and two cases of systemic recurrence. No patient with a negative sentinel lymph node has evidence of metastasis or recurrence. Fisher's exact test demonstrated a significant association between a positive SLNB and recurrence (P = .02).ConclusionSLNB revealed metastatic disease in 17% of patients with DPA and appears to predict systemic recurrence in this small series.
Relationship between initial management strategy and survival in patients with gastric outlet obstruction due to gastric cancer
Authors: Blumenthaler Alisa N. , Ikoma Naruhiko , Blum Mariela , Das Prajnan , Minsky Bruce D. , Mansfield Paul F. , Ajani Jaffer A. , Badgwell Brian D. 
Published: August 2020

AbstractBackgroundThe optimal management of gastric outlet obstruction (GOO) due to gastric cancer (GC) is unclear. We examined the relationships between clinical and management variables and outcomes in patients with GC having GOO.MethodsThe GOO management and clinical course were reviewed in patients with GC and GOO. Cox regression and Kaplan‐Meier analyses were used to identify variables predictive of overall survival (OS).ResultsThe study included 59 patients. Eleven had imaging evidence of metastasis and 35 had pathologically confirmed peritoneal disease. Initial management included resection in 23 patients, feeding jejunostomy ± decompressive gastrostomy (JT/GT) in 25, surgical gastrojejunostomy in five, and endoscopic intervention in six. Seven patients with initial JT/GT underwent resection after neoadjuvant therapy. Median OS (95% confidence interval [CI]) was 21.4 (0.0‐45.1) months in the upfront resection group (median follow‐up, 14.7 months) and not reached in those with initial JT/GT, neoadjuvant therapy, and later resection (median follow‐up, 26.5 months) (P = .18). On multivariable analysis, clinically positive nodes (hazard ratio [HR]: 3.76; 95% CI, 1.17‐12.12; P = .03), metastasis on CT (HR: 3.97; 95% CI: 1.53‐10.26;P = .01), and resection (HR: 0.37; 95% CI: 0.17‐0.79;P = .01) independently predicted OS.ConclusionIn GOO due to GC, OS is similar after treatment with upfront resection compared with JT/GT, neoadjuvant therapy, and later resection. Upfront JT/GT may allow patients to tolerate chemotherapy and improve selection for gastrectomy.
Added Value of the Vascular Index on Superb Microvascular Imaging for the Evaluation of Breast Masses
Authors: Chae Eun Young , Yoon Ga Young , Cha Joo Hee , Shin Hee Jung , Choi Woo Jung , Kim Hak Hee 
Published: August 2020

ObjectivesTo evaluate the value of the vascular index (VI) on Superb Microvascular Imaging (SMI; Canon Medical Systems Corporation, Tokyo, Japan) in comparison with grayscale ultrasound (US) alone for assessing breast masses.MethodsWe evaluated a total of 70 breast masses (36 malignant and 34 benign lesions) in 70 consecutive patients using grayscale US and SMI. Two breast radiologists analyzed grayscale US alone and the combination of grayscale US and SMI. They also measured the VI based on SMI. The diagnostic performance of grayscale US alone and the combination of grayscale US and SMI was compared. The VI was compared between benign and malignant masses, and the optimal cutoff value was determined. In addition, the interobserver agreement in imaging analyses and the VI was assessed.ResultsThe interobserver agreements in imaging analyses and the VI were almost perfect. The VI of malignant breast masses was significantly higher than that of benign lesions (P < .001). The optimal cutoff value of the VI for differentiating between malignant and benign breast masses was 2.95, with sensitivity of 86.1% and specificity of 91.2%. The diagnostic performance values of grayscale US alone and the combination of grayscale US and SMI were 0.824 and 0.912, respectively, for reader 1 (P = .028) and 0.795 and 0.853 for reader 2 (P = .101).ConclusionsThe VI on SMI was significantly higher for malignant breast masses than for benign lesions, with high interobserver agreement. Our study suggests that the combination of grayscale US and SMI with the VI could improve the characterization of breast masses.
Low‐Density Lipoprotein Cholesterol/High‐Density Lipoprotein Cholesterol Ratio and Carotid Intima‐Media Thickness: A Cohort Study in China
Authors: Qin Pei , Shi Jing , Cao Liming , Li Xue , Lou Yanmei , Wang Changyi , Ma Jianping , Wang Li , Peng Xiaolin , Chen Hongen , Xu Shan , Zhao Ping , Hu Dongsheng , Hu Fulan 
Published: August 2020

AbstractLow‐density lipoprotein cholesterol to high‐density lipoprotein cholesterol (LDL‐C/HDL‐C) ratio may carry additional information and has been suggested as a better predictor for atherosclerosis progression and cardiovascular disease (CVD) than LDL‐C and HDL‐C alone. Therefore, we aim to explore the association between LDL‐C/HDL‐C ratio and high carotid intima‐media thickness (CIMT) risk in a large Cohort in Beijing, China. This cohort study included 13,612 adults without high CIMT at first entry and who attended the baseline examination and at least one follow‐up annual examination between 2009 and 2016. We used multivariable Cox regression to estimate hazard ratios (HR) with their 95% confidence intervals (CI) for the association between LDL‐C/HDL‐C ratio and risk of high CIMT. During 37,912 person‐years of follow‐up, 1996 (1268 men and 728 women) developed high CIMT. Compared with the first quartile of LDL‐C/HDL‐C ratio, the risk of high CIMT was significantly increased for the fourth quartile of LDL‐C/HDL‐C ratio (HR = 1.51, 95% CI: 1.29–1.78). We observed a significant association between LDL‐C/HDL‐C ratio and high CIMT risk comparing LDL‐C/HDL‐C ratio >2.78 with LDL‐C/HDL‐C ratio ≤2.78 and significant dose–response relationship between LDL‐C/HDL‐C ratio and risk of high CIMT. The restricted cubic spline showed a significant nonlinear association between LDL‐C/HDL‐C ratio and the risk of high CIMT (pnon‐linearity = 0.009). We identify a significant association between LDL‐C/HDL‐C ratio and the risk of high CIMT in the Chinese Cohort study. Future studies are needed to evaluate the effectiveness of reducing LDL‐C/HDL‐C ratio treatment on the development of high CIMT.
How online trust evolves over time: The role of social perception
Authors: Ye Christine , Hofacker Charles F. , Peloza John , Allen Alexis 
Published: August 2020

AbstractUsing customer service scenarios in an online retail context, the current study examines how cognitive and affective trust develop over time and how service failure negatively impacts trust, along with the trust restoration opportunities provided by recovery. Study 1 findings reveal that the relationship between Web site social perception and affective trust is stronger for repeat visitors than for first‐time visitors. Study 2 findings indicate that failure timing and recovery duration play important roles in service failure situations. Overall, the results demonstrate that consumer cognitive and affective trust develop through the number of interactions with a retail Web site over time and that increased Web site social perceptions facilitate the trust‐building process.
A Facile Synthesis of Poly(3‐octyl thiophene):Ni0.4Sr0.6TiO3 Hybrid Nanocomposites for Solar Cell Applications
Authors: Adimule Vinayak , Suryavanshi Anusha , BC Yallur , Nandi Santosh S. 
Published: August 2020

AbstractThe solar cell properties of poly(3‐octyl thiophene) (P3OT) doped with Ni0.4Sr0.6TiO3 nanocomposite (NCs) as a function of P3OT in different concentrations are studied for photovoltaic characteristics using Hg probe instrumental technique without any fabrication. Nickel nanoparticles (Ni+) loaded with SrTiO3 nanoparticles are attempted to be synthesized by co‐precipitation method, cetyl trimethyl ammonium bromide is used as capping agent and is successfully converted into Ni‐SrTiO3 hybrid nanoparticles. The thin films are made by using different solvent systems in spin coating method, for low concentration (20–30%) of P3OT the device performance is poor compared to pure Ni‐SrTiO3. Significant improvements are obtained with the increased doping (50%) of P3OT. The maximum absorbance in the thin film of UV spectrum lies between 600 and 800 nm spectral region. Increased addition of P3OT results UV band shifts in visible spectral region while power conversion efficiency increases approximately ten times than the existing. The increased solar cell efficiency is strongly dependent not only on the doping of P3OT concentration but also on the solvent used, changes in the film morphology. The optimal fabrication the external quantum efficiency of 10% and power conversion efficiency of 17.12% were obtained.
Synthesis, Impedance, and Current–Voltage Characteristics of Strontium‐Manganese Titanate Hybrid Nanoparticles
Authors: Suryavanshi Anusha , Adimule Vinayak , Nandi Santosh S. 
Published: August 2020

AbstractA polycrystalline hybrid nanocomposite of strontium‐manganese titanate nanocomposites (NCs) is prepared by co‐precipitation technique using cetyl trimethyl ammonium bromide as capping compound. Formation of the NC powders in desired phase is confirmed by powdered X‐ray diffraction characterization. The barrier oxidation and reduction potentials and band gap of the crystalline nanocomposites are measured by cyclic voltammetry, scanning electron microscopy. Images of the materials show irregular grains with an average size of 50 nm. The absorption maxima of the NCs is shown at 580 nm using UV–vis spectrophotometer. Electrical properties impedance and current–voltage characteristics of Sr‐MnTiO3 powders are investigated. The real part of impedance increases linearly with the filler loading of the strontium (obtained for each uplift of 0.2 V). The IV characteristics show variable conductivity with increase in the impedance of the nanocomposites (nonlinear relationship). The filler loading up to 50% IV characteristics and also 40% are nonlinear which show the ohmic nature of the NCs and the electrodes. The impedance increases linearly as the applied voltage is increased. The IV behavior of NCs depends largely on the Sr content in the NCs of titanate. The NCs of 50% filler loaded TiO3 show better efficiency in IV related characteristics (conductance, admittance, and power dissipation factor) than the rest.
Synthesis and Characterization of New Organosoluble and Thermally Stable Aromatic Polyamides Containing Flexible Ether, Ketone, and Methylene Linkages
Authors: Diwate Arati , Ghodake Shivaji , Tamboli Asalam , Patil Kiran , Gurame Mahesh , Maldar Noormahammad 
Published: August 2020

AbstractA new aromatic diacid monomer, namely, 1,4‐phenylene‐bis‐[[4′‐(4″‐carboxy methylene) phenoxy phenyl] methanone] (PBCPPM) is successfully synthesized and characterized by physical constant, FT‐IR, NMR (1H and 13C) spectroscopy, and mass spectrometry. Synthesis of new series of polyamides with high thermal stability and improved solubility are performed by Yamazaki's direct phosphorylative polycondensation method from new PBCPPM with different aromatic diamines. The inherent viscosity of these polyamides is in the range 0.47–0.81 dL g−1. All these polyamides are amorphous or partly crystalline and reveal good solubility in polar aprotic solvents and exhibit glass transition temperatures between 201 and 232 °C. Initial decomposition temperatures are in the range 300–367 °C and T10 is in the range of 376–414 °C indicating good thermal stability of these polyamides. The present observations suggest that these polyamides can find potential applications as high performance polymers.
Bitter melon juice intake with gemcitabine intervention circumvents resistance to gemcitabine in pancreatic patient‐derived xenograft tumors
Authors: Dhar Deepanshi , Raina Komal , Kumar Dileep , Wempe Michael F. , Bagby Stacey M. , Pitts Todd M. , Orlicky David J. , Agarwal Chapla , Messersmith Wells A. , Agarwal Rajesh 
Published: August 2020

AbstractChemoresistance to gemcitabine (GEM)—a frontline chemotherapeutic, resulting from its dysfunctional uptake and metabolism in cancer cells, is a major contributing factor for failed therapy in pancreatic cancer (PanC) patients. Therefore, there is an urgent need for agents that could reverse GEM resistance and allow continued chemosensitivity to the drug. We employed natural nontoxic agent (with anti‐PanC potential) bitter melon juice (BMJ) and GEM to examine their combinatorial benefits against tumorigenesis of PanC patient‐derived xenograft (PDX)—pancreatic ductal adenocarcinomas explants PDX272 (wild‐type KRAS), PDX271 (mutant KRAS and SMAD4), and PDX266 (mutant KRAS). Anti‐PanC efficacy of single agents vs combination in the three tumor explants, both at the end of active dosing regimen and following a drug‐washout phase were compared. In animal studies, GEM alone treatment significantly inhibited PDX tumor growth, but effects were not sustained, as GEM‐treated tumors exhibited regrowth posttreatment termination. However, combination‐regimen displayed enhanced and sustained efficacy. Mechanistic assessments revealed that overcoming GEM resistance by coadministration with BMJ was possibly due to modulation of GEM transport/metabolism pathway molecules (ribonucleotide reductase regulatory subunit M1, human equilibrative nucleoside transporter 1, and deoxycytidine kinase). Study outcomes, highlighting significantly higher and sustained efficacy of GEM in combination with BMJ, make a compelling case for a clinical trial in PanC patients, wherein BMJ could be combined with GEM to target and overcome GEM resistance. In addition, given their specific effectiveness against KRAS‐mutant tumors, this combination could be potentially beneficial to a broader PanC patient population.
Interference of miR‐107 with Atg12 is inhibited by HULC to promote metastasis of hepatocellular carcinoma
Authors: Zhang Haiming , Li Shipeng , Xu Haixu , Sun Liying , Zhu Zhijun , Yao Zhi 
Published: August 2020

AbstractHighly upregulated in liver cancer (HULC) had a significant predictive effect on tumor growth and metastasis of hepatocellular carcinoma (HCC); however, the mechanisms of HULC on HCC still need to be clarified. We attempted to determine the roles of HULC and miR‐107 in autophagy and invasion of HCC. HULC siRNA reduced the level of autophagy. The impact of HULC siRNA on invasion can be reversed by activating autophagy in HCC cell lines. Further studies on HULC and autophagy were conducted. An interacting sequence between HULC and miR‐107, as well as miR‐107 and Atg12, was predicted by software. The relationship of each pair of molecules was confirmed by luciferase reporter assays. The negative impacts of miR‐107 on autophagy and invasion were proved in HCC cell lines. The inhibitor of miR‐107‐promoted invasion can also be reversed by Atg12 siRNA. The changes of miR‐107, Atg12, epithelial‐mesenchymal transition, and autophagy in transplanted tumors of mouse models also confirmed the results in HCC cell lines. Finally, we find that HULC acts as an endogenous sponge, which abolishes the binding of miR‐107 on the Atg12 3′‐UTR and promotes autophagy and metastasis of HCC.
Can Autonomic Testing and Imaging Contribute to the Early Diagnosis of Multiple System Atrophy? A Systematic Review and Recommendations by the Movement Disorder Society Multiple System Atrophy Study Group
Authors: Pellecchia Maria Teresa , Stankovic Iva , Fanciulli Alessandra , Krismer Florian , Meissner Wassilios G. , Palma Jose‐Alberto , Panicker Jalesh N. , Seppi Klaus , Wenning Gregor K. ,   Members of the Movement Disorder Society Multiple System Atrophy Study Group
Published: August 2020

ABSTRACTBackgroundIn the current consensus diagnostic criteria, the diagnosis of probable multiple system atrophy (MSA) is based solely on clinical findings, whereas neuroimaging findings are listed as aid for the diagnosis of possible MSA. There are overlapping phenotypes between MSA‐parkinsonian type and Parkinson's disease, progressive supranuclear palsy, and dementia with Lewy bodies, and between MSA‐cerebellar type and sporadic adult‐onset ataxia resulting in a significant diagnostic delay and misdiagnosis of MSA during life.ObjectivesIn light of an ongoing effort to revise the current consensus criteria for MSA, the Movement Disorders Society Multiple System Atrophy Study Group performed a systematic review of original articles published before August 2019.MethodsWe included articles that studied at least 10 patients with MSA as well as participants with another disorder or control group for comparison purposes. MSA was defined by neuropathological confirmation, or as clinically probable, or clinically probable plus possible according to consensus diagnostic criteria.ResultsWe discuss the pitfalls and benefits of each diagnostic test and provide specific recommendations on how to evaluate patients in whom MSA is suspected.ConclusionsThis systematic review of relevant studies indicates that imaging and autonomic function tests significantly contribute to increasing the accuracy of a diagnosis of MSA.