bluuebunny/crossref_metadata_2025_split huggingface dataset:
Strong increase of leukocyte apha‐galactosidase A activity in two male patients with Fabry disease following oral chaperone therapy
Authors: Lamari Foudil , Mauhin Wladimir , Koraichi Fairouz , Khrouf Walid , Bordet Celine , London Jonathan , Lidove Olivier , Charron Philippe 
Published: August 2019

AbstractBackgroundFabry disease (OMIM 301500) is an X‐linked disorder caused by alpha‐galactosidase A (α‐Gal A) deficiency. The administration of a pharmacologic chaperone (migalastat) in Fabry patients with amenable mutations has been reported to improve or stabilize organ damages and reduce lyso‐Gb3 plasma level. An increase of α‐Gal A activity has been observed in vitro in cells expressing amenable GLA mutations when incubated with migalastat. The impact of the drug on α‐Gal A in vivo activity has been poorly studied.MethodsWe conducted a retrospective analysis of two unrelated male Fabry patients with p.Asn215Ser (p.N215S) variant.ResultsWe report the important increase of α‐Gal A activity in blood leukocytes reaching normal ranges of activity after about 1 year of treatment with migalastat. Cardiac parameters improved or stabilized with the treatment.ConclusionWe confirm in vivo the effects of migalastat that have been observed in N215S carriers in vitro. The increase of α‐Gal A activity may be the strongest marker for biochemical efficacy. The normalization of enzyme activity could become the new therapeutic target to achieve.
Pharmacokinetic modeling of dynamic contrast‐enhanced MRI using a reference region and input function tail
Authors: Ahmed Zaki , Levesque Ives R. 
Published: August 2019

PurposeQuantitative analysis of dynamic contrast‐enhanced MRI (DCE‐MRI) requires an arterial input function (AIF) which is difficult to measure. We propose the reference region and input function tail (RRIFT) approach which uses a reference tissue and the washout portion of the AIF.MethodsRRIFT was evaluated in simulations with 100 parameter combinations at various temporal resolutions (5‐30 s) and noise levels (σ = 0.01‐0.05 mM). RRIFT was compared against the extended Tofts model (ETM) in 8 studies from patients with glioblastoma multiforme. Two versions of RRIFT were evaluated: one using measured patient‐specific AIF tails, and another assuming a literature‐based AIF tail.ResultsRRIFT estimated the transfer constant and interstitial volume with median errors within 20% across all simulations. RRIFT was more accurate and precise than the ETM at temporal resolutions slower than 10 s. The percentage error of had a median and interquartile range of −9 ± 45% with the ETM and −2 ± 17% with RRIFT at a temporal resolution of 30 s under noiseless conditions. RRIFT was in excellent agreement with the ETM in vivo, with concordance correlation coefficients (CCC) of 0.95 for , 0.96 for , and 0.73 for the plasma volume using a measured AIF tail. With the literature‐based AIF tail, the CCC was 0.89 for , 0.93 for and 0.78 for .ConclusionsQuantitative DCE‐MRI analysis using the input function tail and a reference tissue yields absolute kinetic parameters with the RRIFT method. This approach was viable in simulation and in vivo for temporal resolutions as low as 30 s.
Accurate identification of myocardial viability after myocardial infarction with novel manganese chelate‐based MR imaging
Authors: Wen Ling‐yi , Yang Zhi‐gang , Li Zhen‐lin , Ai Hua , Xia Chun‐chao , Zhang Li‐zhi , Lin Bin‐bin , Zhang Kun , Fu Hang , Wu Chang‐qiang , Yang Li , Fan Hai‐ming , Guo Ying‐kun 
Published: August 2019

We developed a novel manganese (Mn2+) chelate for magnetic resonance imaging (MRI) assessment of myocardial viability in acute and chronic myocardial infarct (MI) models, and compared it with Gadolinium‐based delay enhancement MRI (Gd3+‐DEMRI) and histology. MI was induced in 14 rabbits by permanent occlusion of the left circumflex coronary artery. Gd3+‐DEMRI and Mn2+ chelate‐based delayed enhancement MRI (Mn2+ chelate‐DEMRI) were performed at 7 days (acute MI, n = 8) or 8 weeks (chronic MI, n = 6) after surgery with sequential injection of 0.15 mmol/kg Gd3+ and Mn2+ chelate. The biodistribution of Mn2+ in tissues and blood was measured at 1.5 and 24 h. Blood pressure, heart rate (HR), left ventricular (LV) function, and infarct fraction (IF) were analyzed, and IF was compared with the histology. The Mn2+ chelate group maintained a stable hemodynamic status during experiment. For acute and chronic MI, all rabbits survived without significant differences in HR or LV function before and after injection of Mn2+ chelate or Gd3+ (p > 0.05). Mn2+ chelate mainly accumulated in the kidney, liver, spleen, and heart at 1.5 h, with low tissue uptake and urine residue at 24 h after injection. In the acute MI group, there was no significant difference in IF between Mn2+ chelate‐DEMRI and histology (22.92 ± 2.21% vs. 21.79 ± 2.25%, respectively, p = 0.87), while Gd3+‐DEMRI overestimated IF, as compared with histology (24.54 ± 1.73%, p = 0.04). In the chronic MI group, there was no significant difference in IF between the Mn2+ chelate‐DEMRI, Gd3+‐DEMRI, and histology (29.50 ± 11.39%, 29.95 ± 9.40%, and 29.00 ± 10.44%, respectively, p > 0.05), and all three were well correlated (r = 0.92–0.96, p < 0.01). We conclude that the use of Mn2+ chelate‐DEMRI is reliable for MI visualization and identifies acute MI more accurately than Gd3+‐DEMRI.
Evaluation of the usefulness of saliva for DNA methylation analysis in cohort studies
Authors: Murata Yui , Fujii Ayaka , Kanata Sho , Fujikawa Shinya , Ikegame Tempei , Nakachi Yutaka , Zhao Zhilei , Jinde Seiichiro , Kasai Kiyoto , Bundo Miki , Iwamoto Kazuya 
Published: August 2019

AbstractIntroductionEpigenetic information such as DNA methylation is a useful biomarker that reflects complex gene‐environmental interaction. Peripheral tissues such as blood and saliva are commonly collected as the source of genomic DNA in cohort studies. Epigenetic studies mainly use blood, while a few studies have addressed the epigenetic characteristics of saliva.MethodsThe effects of methods for DNA extraction and purification from saliva on DNA methylation were surveyed using Illumina Infinium HumanMethylation450 BeadChip. Using 386 661 probes, DNA methylation differences between blood and saliva from 22 healthy volunteers, and their functional and structural characteristics were examined. CpG sites with DNA methylation levels showing large interindividual variations in blood were evaluated using saliva DNA methylation profiles.ResultsGenomic DNA prepared by simplified protocol from saliva showed a similar quality DNA methylation profile to that derived from the manufacturer provided protocol. Consistent with previous studies, the DNA methylation profiles of blood and saliva showed high correlations. Blood showed 1,514 hypomethylated and 2099 hypermethylated probes, suggesting source‐dependent DNA methylation patterns. CpG sites with large methylation difference between the two sources were underrepresented in the promoter regions and enriched within gene bodies. CpG sites with large interindividual methylation variations in blood also showed considerable variations in saliva.ConclusionIn addition to high correlation in DNA methylation profiles, CpG sites showing large interindividual DNA methylation differences were similar between blood and saliva, ensuring saliva could be a suitable alternative source for genomic DNA in cohort studies. Consideration of source‐dependent DNA methylation differences will, however, be necessary.
Multi‐input control design for a constrained bilinear biquadratic regulator with external excitation
Authors: Halperin Ido , Agranovich Grigory , Ribakov Yuri 
Published: August 2019

SummaryThis study addresses an optimal control problem that is defined for a type of bilinear excited plant, multiple control inputs, a biquadratic finite horizon performance index, and control trajectory constraints. A novel sequence of improving functions, which suits this problem, is derived, and the corresponding Krotov's algorithm is formulated. The significance of the results is illustrated by a structural control design for a structure that is subjected to a harmonic ground acceleration input and semi‐active control constraints.
Shared personal reflections on the need to broaden the scope of conservation social science
Authors: Moon Katie , Adams Vanessa M. , Cooke Benjamin 
Published: August 2019

Abstract

Social science has a more diverse and meaningful role to play in conservation science and ecology than is currently being published within this field.

We reflect on our personal research experiences to demonstrate how our in‐field learning has provided us with shared understandings of the importance of a broader engagement with social science methods, methodologies and philosophy.

We focus on the value that comes from understanding that social science is not just answers, but stories; not just data, but meaning; and that place is a critical part of understanding socio‐ecological phenomena and processes.

We engage in a shared process of reflexivity of our doctoral research experiences to show the potential of social science beyond its predominant positivist applications in the conservation science and ecology literature. We each discuss our experiences of our social science research endeavours in the context of private land conservation. We then distil our experiences into three themes that aim to advance social science engagement for conservation scientists: the partiality of knowledge, situating research within socio‐ecological context and researcher positionality.

We conclude by acknowledging that as researchers we are not the exclusive authority on knowledge; that a deep understanding of conservation and ecology challenges might not provide simple or reducible answers that can be abstracted and applied universally; and that we must reflect on the possibilities for a more plural and diverse research practice for conservation and ecology through a wider engagement with the social sciences.

A free Plain Language Summary can be found within the Supporting Information of this article.
Double‐conditioning regimen consisting of high‐dose thiotepa and melphalan with autologous stem cell rescue for high‐risk pediatric solid tumors: A second report
Authors: Okada Keiko , Yamasaki Kai , Nitani Chika , Fujisaki Hiroyuki , Osugi Yuko , Hara Junichi 
Published: August 2019

AbstractBackgroundPediatric patients with high‐risk, relapsed, or refractory solid tumors have a poor prognosis. We have previously reported a dose‐finding experience of high‐dose chemotherapy consisting of thiotepa and melphalan (“double‐conditioning regimen”). Using doses derived from that study, we have treated patients since 2005. We now report a retrospective review of patients treated by this fixed dose.ProcedureWe reviewed 50 patients (median 4 years; range 0–15 years) with high‐risk or relapsed/refractory solid tumors treated by this dose‐fixed, double‐conditioning regimen from April 2005 to May 2014. Doses were thiotepa 800 mg/m2 and melphalan 280 mg/m2 for children ≥2 years of age, and 32 mg/kg and 6 mg/kg, respectively, for children <2 years of age. Further, doses were reduced according to creatinine clearance with poor renal function.ResultsNonhematological toxicity was mainly gastrointestinal—grade 3 mucositis (n = 41) and grade 3–4 diarrhea (n = 10). Neurological, renal, and endothelial cell toxicity and sinusoidal obstruction syndrome were not observed. There were two toxic deaths (interstitial viral pneumonia). This regimen demonstrated antitumor activity against several types of tumors. Although the frequency of gastrointestinal toxicity was high, other severe toxicity was not observed.ConclusionsOur double‐conditioning regimen was very well tolerated and demonstrated antitumor activity. We are moving forward with multi‐institutional trials now.
Pregnant women with confirmed neoplasms should not have noninvasive prenatal testing
Authors: Lenaerts Liesbeth , Van Calsteren Kristel , Che Huiwen , Vermeesch Joris Robert , Amant Frédéric 
Published: August 2019

What's already known about this topic?

Incidental diagnoses of an occult maternal malignancy have been reported upon aberrant routine noninvasive prenatal testing (NIPT).
The presence of tumor‐derived cell‐free DNA in the maternal circulation can skew the NIPT profile.
What does this study add?

Pregnant women with a confirmed neoplastic disease should not have NIPT testing for fetal aneuploidy screening since NIPT results cannot accurately be applied to assess the fetal chromosomal constitution in this condition.
Nasal continuous positive airway pressure (NCPAP) or noninvasive neurally adjusted ventilatory assist (NIV‐NAVA) for preterm infants with respiratory distress after birth: A randomized controlled trial
Authors: Yagui Ana C. , Meneses Jucille , Zólio Bianca A. , Brito Gabriela M. G. , da Silva Rafael Justino , Rebello Celso M. 
Published: August 2019

AbstractObjectivesTo compare rates of treatment failure between the use of nasal continuous positive airway pressure (NCPAP) and noninvasive neurally adjusted ventilatory assist (NIV‐NAVA) in infants with respiratory distress after birth.MethodsA randomized, unblinded, double‐center trial was conducted in infants with birth weights (BWs) less than or equal to 1500 g and respiratory distress receiving noninvasive respiratory support for less than or equal to 48 hours of life; some infants were initially treated with minimally invasive surfactant therapy as the standard of care. Primary outcome: need for endotracheal intubation with use of mechanical ventilation (MV) at less than or equal to 72 hours of life using prespecified failure criteria. Secondary outcomes: use of surfactant, duration of noninvasive support, duration of MV, bronchopulmonary dysplasia (BPD) and death.ResultsA total of 123 infants were included (NCPAP group = 64 and NIV‐NAVA group = 59). Population characteristics were similar between groups. No difference in the primary outcome was observed: NCPAP = 10 (15.6%) and NIV‐NAVA = 12 (20.3%), P = .65. Groups were also similar in the use of surfactant (19 vs 17), duration of noninvasive support (147 ± 181 hours vs 127 ± 137 hours), BPD incidence and death. However, duration of MV was significantly longer in NCPAP group (95.6 ± 45.8 hours vs 28.25 ± 34.1 hour), P = .01.ConclusionIn infants with respiratory distress after birth, no differences in treatment failures were observed between NIV‐NAVA and NCPAP. These results require further evaluation in a larger study.
Liquid chromatography–tandem mass spectrometry analysis of aristolochic acids in soil samples collected from Serbia: Link to Balkan endemic nephropathy
Authors: Chan Chi‐Kong , Chan K.K. Jason , Pavlović Nikola M. , Chan Wan 
Published: August 2019

RationaleOver the past six decades, residents of farming villages in multiple countries of the Balkan peninsula have been suffering from a unique type of chronic renal disease, Balkan endemic nephropathy (BEN). It was speculated that environmental pollution by aristolochic acids (AAs) produced naturally by Aristolochia clematitis L., a weed that grows in the area, was causing the disease. However, the human exposure pathway to this class of phytotoxin remains obscure. Knowledge of the sink and stability of AAs in the environment would assist in the formulation of policy reducing exposure risk.MethodsUsing our newly developed liquid chromatography/tandem mass spectrometry method of high sensitivity and selectivity, we analysed over 130 soil samples collected from cultivation fields in southern Serbia for the presence of AAs. The environmental stability of AAs was also investigated by incubating soil samples spiked with AAs at various temperatures.ResultsThe analysis detected AA‐I in over two‐fifths of the tested samples at sub‐μg/kg to μg/kg levels, with higher concentrations observed in more acidic farmland soil. Furthermore, analysis of soil samples incubated at various temperatures revealed half‐lives of over 2 months, indicating that AAs are relatively resistant to degradation.ConclusionsCultivation soil in southern Serbia is being extensively contaminated with AAs released from the decomposition of A. clematitis weeds. Since AAs are resistant to degradation, it is possible that AAs could have been taken up by root absorption and transported to the edible part of food crops. Prolonged exposure to AA‐contaminated food grown from polluted soil could be one of the main aetiological mechanisms of BEN observed in the area.
Stereoselective Reducing Complexes Supported in Solid Phase: Preparation and Reactivity Study
Authors: Vargas‐Durazo Judas , Aguilar‐Martínez Milagros , Ochoa‐Terán Adrian , Santacruz‐Ortega Hisila , Alonzo Fernando Rocha , Meza Nohemi Gamez , Galvez‐Ruiz Juan Carlos 
Published: August 2019

AbstractTwelve new chiral chelating resins have been prepared using solid phase organic synthesis with the Merrifield and Wang resins and the bidentate amine 1,2‐ethylenediamine, followed by the coupling of asymmetric ketones such as 1‐phenyletanone, 4‐methyl‐2‐pentanone 4‐nitroacetophenone and 4‐methoxyacetophenone to form prochiral imines. The enantioselective reduction with borane and (R)‐(+)‐2‐Methyl‐CBS‐oxazaborolidine leads to the chiral resins with yields greater than 86%. We used these chiral resins to prepare 24 reducing complexes with alkali tetrahydroborates in a very simple way giving yields greater than 87%. Finally, we achieved a preliminary enantioselective reduction of acetophenone employing the Merrifield's chiral chelating resins obtaining the corresponding S alcohol in a 70:30 ratio with the R analog alcohol.
Reducing renewable power curtailment and CO2 emissions in China through district heating storage
Authors: Zheng Wandong , Hennessy Jay J. , Li Hailong 
Published: August 2019

AbstractEmissions reductions are often achieved through the increased share of renewable energy sources (RES) and China is the leader in the growth of RES in the power sector. This growth has led to high levels of curtailment of RES power due to insufficient reinforcement of the electricity grid to support such growth and due to competition with other power sources. Although the problem of curtailment has been alleviated in recent years, large amounts of power are still discarded, and it is important to consider how to address this problem in the short term and how much CO2e emissions could be avoided as a result. The use of district heating systems to reduce the curtailment of renewable power has seen increasing interest in recent years. Based on a review of potential energy storage in district heating, the current paper assesses the capability to use the national storage potential of district heating systems in China to reduce curtailment and to determine what effects that may have on avoiding CO2e emissions. The distribution networks and the thermal inertia of buildings connected to district heating are considered as two major forms of storage that can be “charged” using power that would otherwise be curtailed. The results show that there may be sufficient storage available to absorb all renewable power that is currently curtailed in those provinces using district heating during the heating season, resulting in avoided emissions of up to 14 MtCO2e/annum.This article is categorized under:
Energy and Climate > Economics and Policy
Wind Power > Climate and Environment
Energy Infrastructure > Climate and Environment
Energy and Urban Design > Climate and Environment
Pancreatectomy with Hepatic Artery Resection for Pancreatic Head Cancer
Authors: Yang Feng , Wang Xiaoyi , Jin Chen , He Hang , Fu Deliang 
Published: August 2019

AbstractBackgroundTo report our experiences and outcome of pancreatectomy with hepatic artery resection (PT‐HAR) for advanced pancreatic head cancer.MethodsA retrospective study of clinical data from 14 patients with advanced pancreatic ductal adenocarcinoma undergoing PT‐HAR in a tertiary academic center between March 2010 and June 2017 was performed. Furthermore, a comparison in a match‐pair analysis (1:3) with patients received standard pancreatectomy during the same period was conducted to evaluate the clinical outcome.ResultsThe PT‐HAR cohort included pancreaticoduodenectomy (n = 11) and total pancreatectomy (n = 3). Of them, six underwent portal/superior mesenteric vein resection and reconstruction and three underwent hepatic artery reconstruction. Four patients without arterial reconstruction developed liver perfusion failure. No perioperative mortality occurred, with a median postoperative hospital stay of 10.5 days (range 6–39). The median overall survival was 30 months (95% confidence interval 9.8–50.2 months), with the 1‐, 2‐, and 3‐year survival rates of 81.8%, 63.6%, and 42.4%, respectively. The matched‐pair data analysis showed no significant differences between PT‐HAR and standard pancreatectomy, except that liver perfusion failure occurred more frequently after PT‐HAR.ConclusionsPT‐HAR can be performed with acceptable morbidity, mortality, and survival for advanced pancreatic head cancer. Considering the potential risk of liver perfusion failure, only highly selected patients are eligible for PT‐HAR without reconstruction.
Detection and Removal of Motion Artifacts in PPG Signals
Authors: Pollreisz David , TaheriNejad Nima 
Published: August 2019

AbstractWith the rise of wearable devices, which integrate myriad of health-care and fitness procedures into daily life, a reliable method for measuring various bio-signals in a daily setup is more desired than ever. Many of these physiological parameters, such as Heart rate (HR) and Respiratory Rate (RR), are extracted indirectly and using other signals such as Photoplethysmograph (PPG). Part of the reason is that in some cases, such as RR measurements, the devices which directly measure them are cumbersome to wear and thus, rather impractical. On the other hand, signals, such as PPG from which the RR can be extracted, are not very clean. This poses a challenge on reliable extraction of these metrics. The most important problem is that they are corrupted by motion artifacts. In this paper, we review the state of the art algorithms which are used to detect and filter motion artifacts in PPG signals and compare them in terms of their performance. The insight provided by this paper can help the scientists and engineers to obtain a better understanding of the field and be able to use the most suitable technique for their work, or come up with innovative solutions based on existing ones.