bluuebunny/crossref_metadata_2025_split huggingface dataset:
Dream Enactment Behavior Disorder Associated with Pallido‐Nigro‐Luysian Degeneration and Tau Proteinopathy
Authors: Di Luca Daniel G. , Slow Elizabeth J. , Martinez‐Valbuena Ivan , Lang Anthony E. , Kovacs Gabor G. 
Published: March 2021

ABSTRACTBackgroundPallido‐nigro‐luysian atrophy (PNLA) is a rare neurodegenerative disorder with only a few cases reported to date. Although the clinical picture usually resembles progressive supranuclear palsy, pathological examination reveals more selective atrophy and loss of neurons in the globus pallidus, substantia nigra and subthalamic nucleus.ObjectivesTo describe the clinical features and pathological findings of a patient with PNLA.MethodsCase report with clinico‐pathological discussion.ResultsAn 83‐year‐old man presented to our clinic with a vertical supranuclear gaze palsy, parkinsonism, gait impairment and sleep abnormalities suggestive of REM‐sleep behavior disorder. Neuropathological examination 5 years after symptom onset revealed subcortical tau proteinopathy compatible with a PNLA pattern. There was also an associated mild degree of limbic/subcortical inflammatory response, Alzheimer's disease‐related changes, as well as argyrophilic grain disease.ConclusionsWe present a comprehensive clinico‐pathological discussion of a patient with PNLA. Besides parkinsonism and vertical supranuclear gaze palsy, the patient also had a sleep disorder, clinically suggestive of REM behavioral disorder, which has not been previously reported in PNLA. We expand the clinical phenotype of this rare condition and provide neuropathological evidence for the associated abnormalities.
The Effect of Education on Symptom Onset and Severity of Huntington's Disease
Authors: Cain Kristina K. , Flanigan Joseph L. , Dalrymple William Alex , Patrie James , Harrison Madaline B. , Barrett Matthew J. 
Published: March 2021

ABSTRACTBackgroundHuntington disease (HD) is an inherited neurodegenerative disorder characterized by motor, psychiatric, and cognitive symptoms. Little is known about the effects of environmental factors on HD symptom onset and severity.ObjectiveTo evaluate the relationship between education level and age of diagnosis, symptom onset, and symptom severity in HD.MethodsThis study evaluated 4537 adult‐onset, motor‐manifest HD participants from the Enroll‐HD global registry. Education level was assessed using International Standard Classification of Education categories, stratified into three education groups corresponding to pre‐secondary, secondary, and post‐secondary educational attainment. Motor and behavioral symptoms of HD, cognition, and functional capacity were measured using baseline Unified Huntington's Disease Rating Scale (UHDRS), Mini‐Mental State Exam (MMSE), Symbol Digit Modalities Test (SDMT), verbal fluency, and Stroop assessments.ResultsAfter adjusting for CAG repeats, higher level of education predicted lower age of onset of motor symptoms, depression, irritability, and cognitive impairment (all P‐values < 0.001). After adjusting for age of enrollment and CAG repeats, the highest education level predicted the lowest UHDRS motor scores, higher UHDRS total functional capacity and functional assessment scores, and higher SDMT, MMSE, verbal fluency, and Stroop assessment scores (all P‐values < 0.001).ConclusionsHD participants with higher education levels have earlier age of diagnosis and age of symptom onset, but lower motor exam scores and higher functional assessment scores. Earlier recognition of symptoms in more highly educated participants may explain earlier symptom onset and diagnosis. Better performance on motor and functional assessments may be explained by higher cognitive reserve in those with greater education.
Ex vivo model predicted in vivo efficacy of CFTR modulator therapy in a child with rare genotype
Authors: Terlizzi Vito , Amato Felice , Castellani Chiara , Ferrari Beatrice , Galietta Luis J. V. , Castaldo Giuseppe , Taccetti Giovanni 
Published: March 2021

ABSTRACTBackgroundNew drugs that target the basic defect in cystic fibrosis (CF) patients may now be used in a large number of patients carrying responsive mutations. Nevertheless, further research is needed to extend the benefit of these treatments to patients with rare mutations that are still uncharacterized in vitro and that are not included in clinical trials. For this purpose, ex vivo models are necessary to preliminary assessing the effect of CFTR modulators in these cases.MethodWe report the clinical effectiveness of lumacaftor/ivacaftor therapy prescribed to a CF child with a rare genetic profile (p.Phe508del/p.Gly970Asp) after testing the drug on nasal epithelial cells. We observed a significant drop of the sweat chloride value, as of the lung clearance index. A longer follow‐up period is needed to define the effects of therapy on pancreatic status, although an increase of the fecal elastase values was found.ConclusionDrug response obtained on nasal epithelial cells correlates with changes in vivo therapeutic endpoints and can be a predictor of clinical efficacy of novel drugs especially in pediatric patients.
Mode selective bandpass filter with high selectivity based on thirty‐second‐mode circular SIW resonator
Authors: Zhang Sheng , She Jin‐Chuan , Tong Meng‐Han , Hong Jia‐Sheng 
Published: March 2021

AbstractIn this paper, a thirty‐second‐mode circular substrate integrated waveguide resonator (TMCSIWR) is firstly investigated, which well retains the fundamental mode and high‐order modes (TM0n0 modes) in circular SIW resonator (CSIWR) for stable transmission. Considering miniaturization, high Q and multi frequency selection of TMCSIWR systematically, each TM0n0 mode in TMCSIWR can be effectively selected to achieve independent passband by adjusting feed position and coupling distance. Meanwhile, each TM0n0 mode and non‐resonant modes produce multiple transmission zeros by mode coupling. Furthermore, arc groove disturbance effectively adjust coupling to achieve high selectivity. Finally, a multi‐zero TM030 mode TMCSIW bandpass filter is designed and fabricated. The measured results are in good agreement with the simulated results.
Dual‐band impedance transformer with single shunt stub for complex impedance loads
Authors: Liu Wang , Liu Qiang , Du Guangxing , Li Guolin , Cheng Dong 
Published: March 2021

AbstractThis letter presents a novel impedance transformer that can transform two unequal frequency‐dependent complex loads (FDCLs) to a resistive load at two diverse bands. The proposed transformer contains four sections of transmission lines and one shunt stub. The design parameters of the transformer are obtained from the analytical formulas. The feasibility analysis of the transformer is discussed and an example based on microstrip technology is implemented and measured for verification. The good agreement between the simulated results and measured results proves the validity of the proposed configuration and design method. Compared with the reported works, the transformer not only features single shunt stub and shorter total electrical length at transmission direction, but also provides simpler analytic design equations and less likely to use the very high‐ or low‐impedance transmission lines.
Robust dose‐painting‐by‐numbers vs. nonselective dose escalation for non‐small cell lung cancer patients
Authors: Petit Steven F. , Breedveld Sebastiaan , Unkelbach Jan , den Hertog Dick , Balvert Marleen 
Published: March 2021

PurposeTheoretical studies have shown that dose‐painting‐by‐numbers (DPBN) could lead to large gains in tumor control probability (TCP) compared to conventional dose distributions. However, these gains may vary considerably among patients due to (a) variations in the overall radiosensitivity of the tumor, (b) variations in the 3D distribution of intra‐tumor radiosensitivity within the tumor in combination with patient anatomy, (c) uncertainties of the 3D radiosensitivity maps, (d) geometrical uncertainties, and (e) temporal changes in radiosensitivity. The goal of this study was to investigate how much of the theoretical gains of DPBN remain when accounting for these factors. DPBN was compared to both a homogeneous reference dose distribution and to nonselective dose escalation (NSDE), that uses the same dose constraints as DPBN, but does not require 3D radiosensitivity maps.MethodsA fully automated DPBN treatment planning strategy was developed and implemented in our in‐house developed treatment planning system (TPS) that is robust to uncertainties in radiosensitivity and patient positioning. The method optimized the expected TCP based on 3D maps of intra‐tumor radiosensitivity, while accounting for normal tissue constraints, uncertainties in radiosensitivity, and setup uncertainties. Based on FDG‐PETCT scans of 12 non‐small cell lung cancer (NSCLC) patients, data of 324 virtual patients were created synthetically with large variations in the aforementioned parameters. DPBN was compared to both a uniform dose distribution of 60 Gy, and NSDE. In total, 360 DPBN and 24 NSDE treatment plans were optimized.ResultsThe average gain in TCP over all patients and radiosensitivity maps of DPBN was 0.54 ± 0.20 (range 0–0.97) compared to the 60 Gy uniform reference dose distribution, but only 0.03 ± 0.03 (range 0–0.22) compared to NSDE. The gains varied per patient depending on the radiosensitivity of the entire tumor and the 3D radiosensitivity maps. Uncertainty in radiosensitivity led to a considerable loss in TCP gain, which could be recovered almost completely by accounting for the uncertainty directly in the optimization.ConclusionsOur results suggest that the gains of DPBN can be considerable compared to a 60 Gy uniform reference dose distribution, but small compared to NSDE for most patients. Using the robust DPBN treatment planning system developed in this work, the optimal DPBN treatment plan could be derived for any patient for whom 3D intra‐tumor radiosensitivity maps are known, and can be used to select patients that might benefit from DPBN. NSDE could be an effective strategy to increase TCP without requiring biological information of the tumor.
Aliasing layers for processing parallel imaging and EPI ghost artifacts efficiently in convolutional neural networks
Authors: Takeshima Hidenori 
Published: March 2021

PurposeThe purposes of this work are to develop a method for efficiently processing MR‐specific artifacts using a convolutional neural network (CNN), and to present its applications for the removal of the artifacts without suppressing actual signals. In MR images that are acquired using parallel imaging and/or EPI, the locations of aliasing artifacts and/or N‐half ghost artifacts can be analytically calculated. However, existing methods using CNNs do not take the structures of the artifacts into account, and therefore need a large number of convolution layers for processing the artifacts.MethodsFor processing the artifacts, a new layer that is named the aliasing layer (AL) is proposed. Because a CNN stands on the assumption that an image has spatial locality, a convolution layer is formulated as a linear function of neighbor locations. For processing the artifacts, the AL preprocesses MR images by moving the calculated locations to the locations accessible through summations over all channels in a standard convolution layer. To evaluate the application of ALs for the removal of parallel imaging and EPI artifacts, CNNs with ALs were compared with those without ALs.ResultsThe results showed that image‐quality metrics of a six‐layer CNN with ALs were better than those of a 12‐layer CNN without ALs. The results also showed that CNNs with ALs suppressed the artifacts selectively.ConclusionThe aliasing layer is proposed for processing MR‐specific artifacts efficiently. The experimental results demonstrated that the AL improved CNNs for removing artifacts from parallel imaging and EPI.
Pharmacosafety of fluoroquinolone and macrolide antibiotics in the clinical care of patients with myasthenia gravis
Authors: Pham Nguyen Thanh Phuong , Leonard Charles E. , Bird Shawn J. , Willis Allison W. , Hamedani Ali G. 
Published: March 2021

AbstractIntroduction/AimsAnecdotal case reports have suggested a potential association of fluoroquinolones and macrolides with myasthenia gravis (MG) exacerbation, prompting warnings against the use of these drugs in this population. However, large‐scale and reliable population‐based data that demonstrate this association are lacking. This study aims to examine the association between outpatient treatment with fluoroquinolones or macrolides and MG‐related hospitalization.MethodsA retrospective cohort study consisting of adult MG patients was conducted using a large de‐identified healthcare claims database. Antibiotic prescription claims were identified, and MG‐related hospitalizations were assessed at 15, 30, and 90 days after the date of prescription. We used mixed effects survival regression with log‐logistic distribution and independent covariance matrix to estimate odds ratios (ORs) of hospitalization for each potentially exacerbating antibiotic using beta‐lactam as the reference and adjusting for covariates.ResultsAmong 1556 MG patients receiving 894 fluoroquinolone prescriptions, 729 macrolide prescriptions, and 1608 beta‐lactam prescriptions during the study period, there was no difference in 15, 30, or 90‐day odds of MG‐related hospitalization between fluoroquinolone or macrolide users compared to prescribed beta‐lactams. However, estimates were higher for fluoroquinolones than macrolides, even after covariate adjustment (adjusted OR [aOR] 4.60, 95% confidence interval [CI] 0.55‐38.57 for fluoroquinolones and OR 0.56, 95% CI 0.32‐0.97 for macrolides, respectively, at 15 days).DiscussionFluoroquinolone and macrolide antibiotics are prescribed frequently to patients with MG. While statistical imprecision precludes a definitive conclusion, elevated ORs for fluoroquinolones raise the possibility of an underpowered association that merits further investigation.
Homeward Bound: In-River Movements of Adult Hatchery- and Natural-Origin Chinook Salmon in the Elk River, Oregon
Authors: Whitmore Margaret , Richardson Shannon , Huff Austin , Goodson Kevin , Quinn Thomas P. , Dittman Andrew H. , Johnson Marc A. , Kamran Maryam , Noakes David L. G. 
Published: March 2021

Abstract
Many adult hatchery-origin Pacific salmon Oncorhynchus spp. return to their natal river but do not enter the hatchery, instead spawning in the river, where they can have detrimental genetic and ecological effects on naturally reproducing wild populations. This phenomenon is especially well documented in Elk River (Oregon) Chinook Salmon O. tshawytscha based on previous analyses of coded wire tag recoveries. Here, we used radio tags to compare the movements of natural- and hatchery-origin Chinook Salmon (n = 11 and 15, respectively) to determine whether their behavior could explain the in-river straying patterns of hatchery salmon. Most of the tagged hatchery-origin (87%) and natural-origin (73%) adults initially moved upriver to the vicinity of the Elk River Hatchery. Most natural-origin adults then moved downstream of the hatchery entrance to spawn, but the movements of hatchery-origin adults were different. Hatchery-origin males and females had the highest and lowest variation in total distance moved, respectively. Our results are consistent with the hypothesis that while most hatchery-origin salmon return to the vicinity of the hatchery, some fail to enter, perhaps because the hatchery’s odors were not perceived as distinct from those of the river or (in the case of hatchery-origin males) because they stopped short of the hatchery after encountering spawning conspecifics. Thus, spawning distributions may not always reflect in-river movements of Chinook Salmon, and we encourage fisheries managers to continue investigating methods that reduce potentially harmful interactions between hatchery- and natural-origin fish.
Twin‐twin transfusion syndrome is associated with alterations in the metabolic profile of maternal plasma in early gestation: a pilot study
Authors: Yang Yang , Wen Li , Han Ting‐li , Zhang Lan , Fu Huijia , Gan Jie , Saffery Richard , Tong Chao , Li Junnan , Qi Hongbo , Baker Philip N. , Kilby Mark D. 
Published: March 2021

AbstractObjectiveTwin‐twin transfusion syndrome (TTTS) causes perinatal mortality and morbidity in monochorionic twins. The early recognition of and interventional therapy for TTTS is associated with a more favorable overall prognosis. However, the prediction by the use of ultrasound in the first trimester has relatively poor sensitivity and specificity. This study aimed to identify metabolic biomarkers to aid in ultrasound screening of TTTS.MethodsMaternal plasma was prospectively collected between 11 and 15 weeks of gestation in apparently uncomplicated monochorionic‐diamniotic twin pregnancies. This cohort was divided into: (i) patients who were subsequently diagnosed with TTTS by using ultrasound; (ii) uncomplicated matched controls. Metabolome was profiled by using gas chromatography‐mass spectrometry.ResultsThe levels of fatty acids, organic acids, oxaloacetic acid, and beta‐alanine were significantly lower in the TTTS maternal plasma at 11–15 weeks of gestation, and methionine and glycine were also higher (p < 0.05, FDR<0.12). Generally, in TTTS pregnancies, the metabolisms of amino acid, carbohydrate, cofactors, vitamins, and purine were “down‐regulated”; whereas bile secretion and pyrimidine metabolism were “upregulated.”ConclusionsThe metabolomics scanning of early gestation maternal plasma may identify those pregnancies that subsequently develop TTTS; in particular, downregulated fatty acid levels may be biologically plausible to be implicated in the pathogenesis of TTTS.
The da1 mutation in wheat increases grain size under ambient and elevated CO2 but not grain yield due to trade‐off between grain size and grain number
Authors: Mora‐Ramirez Isabel , Weichert Heiko , von Wirén Nicolaus , Frohberg Claus , de Bodt Stefanie , Schmidt Ralf‐Christian , Weber Hans 
Published: March 2021

Why this research MattersGrain size is potentially yield determining in wheat, controlled by the ubiquitin pathway and negatively regulated by ubiquitin receptor DA1. We analyzed whether increased thousand grain weight in wheat da1 mutant is translated into higher grain yield and whether additional carbon provided by elevated (e)CO2 can be better used by the da1, displaying higher grain sink strength and size. Yield‐related, biomass, grain quality traits, and grain dimensions were analyzed by two‐factorial mixed‐model analysis, regarding genotype and eCO2. da1 increased grain size but reduced spikes and grains per plant, grains per spike, and spikelets per spike, independent of eCO2 treatment, leaving total grain yield unchanged. eCO2 increased yield and grain number additively and independently of da1 but did not overcome the trade‐off between grain size and number observed for da1. eCO2 but not da1 impaired grain quality, strongly decreasing concentrations of several macroelement and microelement. In conclusion, intrinsic stimulation of grain sink strength and grain size, achieved by da1, is not benefitting total yield unless trade‐offs between grain size and numbers can be overcome. The results reveal interactions of yield components in da1‐wheat under ambient and eCO2, thereby uncovering limitations enhancing wheat yield potential.
Malononitrile‐involved Michael addition polymerization: An efficient and facile route for cyano‐rich polyesters with programmable thermal and mechanical properties
Authors: Yang Xiaoxia , Xie Hongyan , Xu Zhiguang , Feng Jiabing , Fu Qiwei , Li Haidong , Jia Yongtang 
Published: March 2021

AbstractAn efficient, atom‐economic, oxygen‐tolerant, and water‐tolerant strategy has been established to synthesize cyano‐rich polyesters. Four kinds of organic bases, 1,1,3,3‐tetramethylguanidine (TMG), 4‐dimethylaminopyridine, triethylamine, and 1,5,7‐triazabicyclo[4.4.0]dec‐5‐ene (TBD) were explored for accelerating Michael addition polymerization of malononitrile and 1,4‐butandiol diacrylate. TMG can promote the polymerization efficiently under mild conditions to quantitatively afford polyester with high‐molecular weight and moderate polydispersity. The comparison of the kinetic studies of TMG and TBD reveals that TMG shows better catalytic performance, while the catalysis of TBD brings about oligomers in spite of the higher efficiency at early age of the polymerization. Moreover, other diacrylate compounds could also be quantitatively polymerized to afford polyesters with high molecular weight. When dimethacrylate is chose as the monomer, the polymerization becomes sluggish. All the afforded polyesters display programmable thermal and mechanical properties that are closely related to their chemical structures.
COVID‐19 in childhood: Transmission, clinical presentation, complications and risk factors
Authors: Siebach Melissa K. , Piedimonte Giovanni , Ley Sylvia H. 
Published: March 2021

AbstractChildren less than 18 years of age account for an estimated 2%–5% of reported severe acute respiratory syndrome coronavirus 2 (SARS‐CoV‐2) cases globally. Lower prevalence of coronavirus disease 2019 (COVID‐19) among children, in addition to higher numbers of mild and asymptomatic cases, continues to provide challenges in determining appropriate prevention and treatment courses. Here, we summarize the current evidence on the transmission, clinical presentation, complications and risk factors in regard to SARS‐CoV‐2 in children, and highlight crucial gaps in knowledge going forward. Based on current evidence, children are rarely the primary source of secondary transmission in the household or in child care and school settings and are more likely to contract the virus from an adult household member. Higher transmission rates are observed in older children (10–19 years old) compared with younger children ( <10 years old). While increasing incidence of COVID‐19 in neonates raises the suspicion of vertical transmission, it is unlikely that breast milk is a vehicle for transmission from mother to infant. The vast majority of clinical cases of COVID‐19 in children are mild, but there are rare cases that have developed complications such as multisystem inflammatory syndrome in children, which often presents with severe cardiac symptoms requiring intensive care. Childhood obesity is associated with a higher risk of infection and a more severe clinical presentation. Although immediate mortality rates among children are low, long‐term respiratory, and developmental implications of the disease remain unknown in this young and vulnerable population.