bluuebunny/crossref_metadata_2025_split huggingface dataset:
Distinct impacts of bi‐allelic WNT10A mutations on the permanent and primary dentitions in odonto‐onycho‐dermal dysplasia
Authors: Yu Miao , Liu Yang , Liu Haochen , Wong Sing‐Wai , He Huiying , Zhang Xiaoxia , Wang Yue , Han Dong , Feng Hailan 
Published: December 2018

AbstractOdonto‐onycho‐dermal dysplasia (OODD) is a rare autosomal recessive syndrome characterized by multiple ectodermal abnormalities. Mutations of the wingless‐type MMTV integration site family member 10A (WNT10A) gene have been associated with OODD. To date, only 11 OODD‐associated WNT10A mutations have been reported. In this report, we Characterized the clinical manifestations with focusing on dental phenotypes in four unrelated OODD patients. By Sanger sequencing, we identified five novel mutations in the WNT10A gene, including two homozygous nonsense mutations c.1176C>A (p.Cys392*) and c.742C>T (p.Arg248*), one homozygous frame‐shift mutation c.898‐899delAT (p.Ile300Profs*126), and a compound heterozygous mutation c.826T>A (p.Cys276Ser) and c.949delG (p.Ala317Hisfs*121). Our findings confirmed that bi‐allelic mutations of WNT10A were responsible for OODD and greatly expanded the mutation spectrum of OODD. For the first time, we demonstrated that bi‐allelic WNT10A mutations could lead to anodontia of permanent teeth, which enhanced the phenotypic spectrum of WNT10A mutations. Interestingly, we found that bi‐allelic mutations in the WNT10A gene preferentially affect the permanent dentition rather the primary dentition, suggesting that the molecular mechanisms regulated by WNT10A in the development of permanent teeth and deciduous teeth might be different.
Metal–Organic Framework (MOF) Template Based Efficient Pt/ZrO2@C Catalysts for Selective Catalytic Reduction of H2 Below 90 °C
Authors: Wang Qian , Sun Wei , Xie Tianying , Cao Limei , Yang Ji 
Published: December 2018

AbstractSelective catalytic reduction (SCR) of NOx with H2 as a reductant is the most promising denitration technology at low temperature. Achieving the conversion of NOx into N2 at ambient temperature not only prolongs the service life of the catalyst, but also provides more freedom for the arrangement of denitration units throughout the flue gas treatment equipment. However, the development of highly efficient, stable, and environmentally benign supported platinum‐based catalysts for H2‐SCR at ambient temperature is still a major challenge. Herein, a 0.5 wt % Pt/ZrO2@C catalyst, which was composed of carbon‐coated octahedral ZrO2 with highly dispersed Pt particles, was prepared by using a new stabilization strategy based on UiO‐66‐NH2 (a zirconium metal–organic framework) as a template. The catalytic performance of this Pt/ZrO2@C in H2‐SCR was tested and confirmed to achieve near 100 % NOx conversion at 90 °C. Also, 70 % N2 selectivity of the catalyst was achieved. The morphology, structure, and porous properties of the as‐synthesized nanocomposites were characterized by using data obtained from field‐emission SEM, TEM, XRD, Raman spectroscopy, thermogravimetric analysis, X‐ray photoelectron spectroscopy, and N2 adsorption–desorption isotherms. The results show that residual carbon formed by pyrolysis treatment is coated on octahedral ZrO2, and effectively prevents the agglomeration of platinum particles on the surface.
When consumers own their work: Psychological ownership and consumer citizenship on crowdsourcing platforms
Authors: Yuksel Mujde , Darmody Aron , Venkatraman Meera 
Published: December 2018

AbstractThe growth of crowdsourced platforms for consumer products has opened many interesting research questions on the impact of consumers participating in such crowdsourced work. In this paper, we focus on consumers' psychological ownership of the crowdsourced product and the role it plays in the relationship between consumer work and consumer citizenship. Further, drawing on social comparison theory, we show that consumers' perceived amount of work relative to others is a boundary condition for this explanation. Finally, we examine the theoretical contributions and managerial implications of our findings.
IL‐6 stimulation of DNA replication is JAK1/2 mediated in cross‐talk with hyperactivated ERK1/2 signaling
Authors: Subotički Tijana , Mitrović Ajtić Olivera , Beleslin‐Čokić Bojana B. , Bjelica Sunčica , Djikić Dragoslava , Diklić Miloš , Leković Danijela , Gotić Mirjana , Santibanez Juan F. , Noguchi Constance T. , Čokić Vladan P. 
Published: December 2018

AbstractMyeloproliferative neoplasms (MPNs) are developing resistance to therapy by JAK1/2 inhibitor ruxolitinib. To explore the mechanism of ruxolitinib's limited effect, we examined the JAK1/2 mediated induction of proliferation related ERK1/2 and AKT signaling by proinflammatory interleukin‐6 (IL‐6) in MPN granulocytes and JAK2V617F mutated human erythroleukemia (HEL) cells. We found that JAK1/2 or JAK2 inhibition prevented the IL‐6 activation of STAT3 and AKT pathways in polycythemia vera and HEL cells. Further, we showed that these inhibitors also blocked the IL‐6 activation of the AKT pathway in primary myelofibrosis (PMF). Only JAK1/2 inhibitor ruxolitinib largely activated ERK1/2 signaling in essential thrombocythemia and PMF (up to 4.6 fold), with a more prominent activation in JAK2V617F positive granulocytes. Regarding a cell cycle, we found that IL‐6 reduction of HEL cells percentage in G2M phase was reversed by ruxolitinib (2.6 fold). Moreover, ruxolitinib potentiated apoptosis of PMF granulocytes (1.6 fold). Regarding DNA replication, we found that ruxolitinib prevented the IL‐6 augmentation of MPN granulocytes frequency in the S phase of the cell cycle (up to 2.9 fold). The inflammatory stimulation induces a cross‐talk between the proliferation linked pathways, where JAK1/2 inhibition is compensated by the activation of the ERK1/2 pathway during IL‐6 stimulation of DNA replication.
A sex stratified outcome analysis from the OPEN‐CTO registry
Authors: Pershad Ashish , Gulati Martha , Karmpaliotis Dimitrios , Moses Jeffery , Nicholson William J. , Nugent Karen , Tang Yuanyuan , Sapontis James , Lombardi William , Grantham James A. ,   on behalf of the OPEN CTO Subgroup
Published: December 2018

AbstractIntroductionWomen have been under‐represented in trials. Due to the dearth of information about CTO‐PCI in women and discordance of previous results, sex differences in outcomes in the OPEN‐CTO Trial were investigated.MethodsOPEN‐CTO is an investigator‐initiated, multicenter, prospective observational registry of consecutive CTO patients undergoing PCI at 12 U.S. centers. The one‐year outcomes of this trial stratified by sex were examined. Optimal propensity matching was performed to compare outcomes between sexes. Multivariate conditional logistic regression modeling for predictors of procedural success was performed.ResultsWomen represented 19.6% of the cohort (196/1,000 patients). Women were more likely to report dyspnea as their predominant symptom. Women reported statistically worse physical limitation and poorer quality of life as compared to men. J‐CTO scores were similar in males and females. Technical, procedural success and MACE rates were similar in both sexes. Contrast and radiation doses were however significantly lower in women. The SAQ‐ summary score, RDS, EQ‐5D VAS, PHQ‐8 scores were all improved to the same degree at 1 year in women as compared to men. Predictors of procedural success revealed that younger age, lower J‐CTO score and absence of prior CABG were predictors of procedural success. Sex did not predict procedural success or 1‐year MACE in this regression model.ConclusionThis real‐world registry revealed that women derive the same benefit from CTO‐PCI as men without additional complications and with favorable health status outcomes at 1 year. Consideration of revascularization by PCI in symptomatic women should be considered as part of the treatment when appropriate.
Safety and feasibility of simultaneous endovascular therapy for supra‐arch multivessel stenosis in 256 Chinese patients
Authors: Dong Hui , Chen Yang , Jiang Xiongjing , Zou Yubao , Che Wuqiang , Xiong Hongliang , Xu Bo , Yang Yuejin , Gao Runlin 
Published: December 2018

AbstractObjectivesTo evaluate the safety and feasibility of simultaneous endovascular therapy for supra‐arch multivessel stenosis.BackgroundLimited data are currently available on simultaneous intervention for supra‐arch multivessel stenosis because of concerns regarding the high incidence of procedure‐related complications.MethodsFrom January 2005 to December 2012, the clinical data of 256 consecutive inpatients who underwent simultaneous intervention for supra‐arch multivessel stenosis were retrospectively analyzed. The primary end point was a composite of any type of stroke or neurological death within 30 days.ResultsIn total, 535 supra‐arch vessels underwent endovascular therapy. In total, 234, 21, and 1 patient underwent simultaneous intervention of 2, 3, and 4 supra‐arch vessels, respectively. The lesions involved only the anterior circulation in 40.2% (103/256), only the posterior circulation in 10.5% (27/256), and the combined circulations in 49.2% (126/256) patients. The overall procedural success rate was 99.6% (533/535). The incidence of the primary end point was 3.5%. According to the lesion location, the incidence of the primary end point was 3.9% (4/103), 0% (0/27), and 4.0% (5/126) in the patients who underwent endovascular treatment for the anterior circulation alone, posterior circulation alone, and combined circulations, respectively. According to the number of supra‐arch lesions, the incidence of the primary end point was 3.4% (8/234), 4.8% (1/21), and 0% (0/1) in the patients who underwent simultaneous intervention of 2, 3, and 4 vessels, respectively.ConclusionsThe study showed that simultaneous intervention for supra‐arch multivessel stenosis was safe and technically feasible.
In‐hospital outcomes of transcatheter versus surgical aortic valve replacement for nonagenarians
Authors: Elgendy Islam Y. , Mahmoud Ahmed N. , Elbadawi Ayman , Elgendy Akram Y. , Omer Mohamed A. , Megaly Michael , Mojadidi Mohammad K. , Jneid Hani 
Published: December 2018

AbstractObjectivesTo compare the in‐hospital outcomes of transcatheter aortic valve replacement (TAVR) versus surgical aortic valve replacement (SAVR) in nonagenarians.BackgroundData comparing the outcomes of TAVR versus SAVR in nonagenarians are limited.MethodsUsing the National Inpatient Sample years 2012–2014, hospitalization data were retrieved for subjects aged ≥90 years who underwent TAVR or SAVR for severe aortic stenosis. The incidence of in‐hospital mortality and peri‐procedural outcomes were compared using unadjusted, multivariate logistic regression, and propensity score matched analyses.ResultsThe final cohort included 6,680 records of nonagenarians undergoing aortic valve replacement, among which 5,840 (87.4%) underwent TAVR. There was no difference in the incidence of in‐hospital mortality between both groups in the unadjusted (5.8% versus 6.0% P = 0.95), multivariate (odds ratio [OR] 0.78, 95% confidence interval [CI] 0.35–1.74), and propensity score matched (OR 1.07, 95% CI 0.75–1.51) analyses. In the propensity‐matched analysis, TAVR was associated with a lower incidence of acute kidney injury (OR 0.58, 95% CI 0.47–0.72), post‐operative blood transfusion (OR 0.51, 95% CI 0.43–0.61), a higher likelihood of discharge to home (OR 4.71, 95% 3.44–5.06), and a similar incidence of pacemaker placement (OR 1.16, 95% 0.89–1.53) and stroke (OR 1.34, 0.90–1.99).ConclusionsIn this nationwide analysis, TAVR was associated with an overall similar incidence of in‐hospital mortality and less morbidity compared with SAVR. These findings suggest that TAVR is effective and safe in nonagenarians.
Electrochemical Performance of Borate‐Doped Nickel Sulfide: Enhancement of the Bifunctional Activity for Total Water Splitting
Authors: Zhang Zhao , Zhang Tianran , Lee Jim Yang 
Published: December 2018

AbstractElectrolytic water splitting with solar electricity is one of the few technologies which can produce hydrogen with nearly zero carbon emission. The viability of the technology depends on the availability of low‐cost alternatives to the platinum group metals (PGMs); which are the best known electrocatalysts for both the hydrogen evolution reaction (HER) and the oxygen evolution reaction (OER). In this study, borate doping of nickel sulfide was found to yield catalysts which are effective for total water electrolysis. For the borate‐doped nickel sulfides ((BO3)x−Ni3S4, x=0, 0.57, 0.78 and 1.08), the strong affinity of borate for hydroxyl groups facilitates the dissociation of adsorbed water molecules to oxygen. Consequently, the borate‐doped nickel sulfide catalyst with the best performance in this study, (BO3)1.08−Ni3S4, requires only a small overpotential of 306 mV to support a current density of 10 mA cm−2 in OER. This, together with a small Tafel slope of 36.5 mV decade−1 and a stable extended operation; places (BO3)1.08−Ni3S4 among the top non‐PGM OER electrocatalysts reported to date. At the same time (BO3)1.08−Ni3S4 also shows good HER activity and stability for it to be used as a standalone catalyst for efficient total water splitting in alkaline electrolyte (1.69 V at 10 mA cm−2).
The lipoxygenase‐hepoxilin pathway is activated in cutaneous plaque lesions of psoriasis
Authors: Takeichi Takuya , Kinoshita Fumie , Tanaka Hirotaka , Fujita Setsuko , Kobayashi Yumiko , Nakatochi Masahiro , Sugiura Kazumitsu , Akiyama Masashi 
Published: December 2018

AbstractPsoriasis is a common skin disorder that is characterized by hyperkeratosis, epidermal hyperplasia, T‐cell and neutrophil recruitment, and changes in the vascular endothelial system. The several upregulated variable lipid mediators, including the eicosanoids, in psoriatic keratinocytes were described previously, although some results were contradictory. Since hepoxilin is less stable than other eicosanoids such as hydroxyeicosatetraenoic acid (HETE), the measurement of hepoxilin is difficult. In this study, to clarify the relationship between hepoxilin/hepoxilin‐related fatty acid derivatives and psoriasis vulgaris, we evaluated the amount of production of lipid mediators including hepoxilins in skin tissues of patients with psoriasis vulgaris in comparison with normal controls by high‐performance liquid chromatography/mass spectrometry. Most of the hepoxilins and related lipids evaluated in this study tended to be more abundant in the lesional skin from the psoriasis patient group (PPG) than in the skin from controls. Interestingly, 12R‐HETE was markedly higher in the skin samples from the PPG. In addition, the lipids produced in the lipoxygenase pathway, including hepoxilin, were elevated in the skin lesions of the PPG compared with those of normal controls, whereas the lipids produced in the cyclooxygenase pathway were decreased in the skin lesions from the PPG. Our results suggest that an imbalance between the lipoxygenase and cyclooxygenase pathways may contribute to psoriatic pathomechanisms.
Simultaneous removal of SO2 and NO by CO reduction over prevulcanized Fe2O3/AC catalysts
Authors: Song Yongji , Wang Ting , Cheng Liang , Li Cuiqing , Wang Hong , Wang Xincheng 
Published: December 2018

AbstractLow‐cost Fe2O3 modified activated carbon catalysts have been prepared by a facile incipient‐wetness impregnation method and used for the low‐temperature simultaneous catalytic reduction of SO2 and NO by CO. The effect of CO, NO, and SO2 was studied experimentally by transient response method. Removal efficiencies of 95 % for NO and 100 % for SO2 were obtained, respectively, for the optimum catalyst, in which the amount of Fe2O3 was 10 wt%. It was found that the catalyst was robust enough under SO2 and NO circumstances; moreover, the prevulcanization of Fe atoms was found to be essential for high efficiency. The reaction mechanism of the simultaneous desulphurization and denitrification reactions of Fe2O3/AC was proposed to follow both redox and COS mechanisms. These results make activated carbon supported Fe2O3 highly promising for the simultaneous abatement of SO2 and NO emissions generated during the combustion processes.
Mocetinostat for patients with previously treated, locally advanced/metastatic urothelial carcinoma and inactivating alterations of acetyltransferase genes
Authors: Grivas Petros , Mortazavi Amir , Picus Joel , Hahn Noah M. , Milowsky Matthew I. , Hart Lowell L. , Alva Ajjai , Bellmunt Joaquim , Pal Sumanta K. , Bambury Richard M. , O’Donnell Peter H. , Gupta Sumati , Guancial Elizabeth A. , Sonpavde Guru P. , Faltaos Demiana , Potvin Diane , Christensen James G. , Chao Richard C. , Rosenberg Jonathan E. 
Published: December 2018

AbstractBackgroundThe authors evaluated mocetinostat (a class I/IV histone deacetylase inhibitor) in patients with urothelial carcinoma harboring inactivating mutations or deletions in CREB binding protein [CREBBP] and/or E1A binding protein p300 [EP300] histone acetyltransferase genes in a single‐arm, open‐label phase 2 study.MethodsEligible patients with platinum‐treated, advanced/metastatic disease received oral mocetinostat (at a dose of 70 mg 3 times per week [TIW] escalating to 90 mg TIW) in 28‐day cycles in a 3‐stage study (ClinicalTrials.gov identifier NCT02236195). The primary endpoint was the objective response rate.ResultsGenomic testing was feasible in 155 of 175 patients (89%). Qualifying tumor mutations were CREBBP (15%), EP300 (8%), and both CREBBP and EP300 (1%). A total of 17 patients were enrolled into stage 1 (the intent‐to‐treat population); no patients were enrolled in subsequent stages. One partial response was observed (11% [1 of 9 patients; the population that was evaluable for efficacy comprised 9 of the 15 planned patients]); activity was deemed insufficient to progress to stage 2 (null hypothesis: objective response rate of ≤15%). All patients experienced ≥1 adverse event, most commonly nausea (13 of 17 patients; 77%) and fatigue (12 of 17 patients; 71%). The median duration of treatment was 46 days; treatment interruptions (14 of 17 patients; 82%) and dose reductions (5 of 17 patients; 29%) were common. Mocetinostat exposure was lower than anticipated (dose‐normalized maximum serum concentration [Cmax] after TIW dosing of 0.2 ng/mL/mg).ConclusionsTo the authors’ knowledge, the current study represents the first clinical trial using genomic‐based selection to identify patients with urothelial cancer who are likely to benefit from selective histone deacetylase inhibition. Mocetinostat was associated with significant toxicities that impacted drug exposure and may have contributed to modest clinical activity in these pretreated patients. The efficacy observed was considered insufficient to warrant further investigation of mocetinostat as a single agent in this setting.
Effect of Rifampicin on the Pharmacokinetics of a Single Dose of Vemurafenib in Patients With BRAFV600 Mutation–Positive Metastatic Malignancy
Authors: Zhang Weijiang , McIntyre Christine , Forbes Harper , Gaafar Rabab , Kohail Hanaa , Beck J. Thaddeus , Plestina Stjepko , Bertran Enric , Riehl Todd 
Published: December 2018

AbstractVemurafenib prolongs survival in patients with BRAFV600‐mutated advanced melanoma. In vitro studies show cytochrome P450 (CYP) 3A4 is involved in vemurafenib metabolism, but the effect of strong inducers or inhibitors of CYP3A4 on vemurafenib exposure in vivo is unknown. This phase 1, open‐label, multicenter study evaluated the effect of rifampicin, a CYP3A4 inducer, on the pharmacokinetics of single‐dose vemurafenib in 27 patients with BRAFV600 mutation–positive metastatic malignancy. Patients received a single oral dose of vemurafenib 960 mg on day 1, oral rifampicin 600 mg daily on days 8–16 (period B), and a single oral dose of vemurafenib 960 mg on day 17 and rifampicin 600 mg daily for days 17–23 (period C), with plasma samples obtained up to 168 hours after vemurafenib dosing. The geometric mean ratio (period C/period A) of area under the concentration‐time curve from time zero to last measurable concentration time point and area under the concentration‐time curve from time zero to infinity for vemurafenib (n = 23 for the pharmacokinetic analysis) was 0.61 (90% confidence interval, 0.48–0.78) and 0.60 (90% confidence interval, 0.47–0.76), respectively, indicating rifampicin significantly decreased vemurafenib plasma exposure by approximately 40%. The geometric mean ratio of the maximum concentration for vemurafenib was 1.1; this slight increase is likely owing to one outlier in period C. Adverse events were consistent with those previously seen for rifampicin and for vemurafenib monotherapy. Caution is advised when dosing vemurafenib concurrently with CYP3A4 inducers.
Utility of intraoperative squash cytology in diagnosis of paediatric central nervous system lesions
Authors: Maity Priyanka , Sengupta Moumita , Jain Kavita , Chaudhuri Shubhamitra , Chatterjee Uttara , Datta Chhanda , Chatterjee Sandip 
Published: December 2018

BackgroundRole of squash cytology in intraoperative diagnosis of central nervous system lesions has been well established. Intraoperative diagnosis is especially important in paediatric CNS lesions as decision regarding gross total resection or near total resection or subtotal resection is crucial and radiotherapy and chemotherapy are best avoided in this age group. The aim of this study was to evaluate the utility of squash cytology of CNS lesions in paediatric age group and to assess its diagnostic accuracy, sensitivity, specificity, positive predictive value and negative predictive value.Materials and methodsA prospective study was conducted on 42 paediatric patients with clinico‐radiologically diagnosed CNS lesions. Intraoperative squash smears were stained with haematoxylin and eosin (H&E) stain. Diagnosis made subsequently on paraffin sections was taken as gold standard.ResultsThe overall diagnostic accuracy of intraoperative squash cytology of CNS lesions in paediatric age group was 73.80%. The sensitivity, specificity, positive predictive value and negative predictive value of this modality were 92.31%, 87.50%, 96.00% and 77.78%, respectively.ConclusionsThe spectrum of CNS lesions in paediatric age group is different from that in adults. Though the sensitivity and specificity of squash cytology in paediatric tumours are less than that of adults, it is an important tool for intraoperative diagnosis that guides regarding the extent of resection.