bluuebunny/crossref_metadata_2025_split huggingface dataset:
CHIP control degradation of mutant ETF:QO through ubiquitylation in late‐onset multiple acyl‐CoA dehydrogenase deficiency
Authors: Liu Xin‐Yi , Chen Xue‐Jiao , Zhao Miao , Wang Zhi‐qiang , Chen Hai‐zhu , Li Hong‐Fu , Wang Chen‐Ji , Wu Shi‐Fei , Peng Chao , Yin Yue , Fu Hong‐Xia , Lin Min‐Ting , Yu Long , Xiong Zhi‐Qi , Wu Zhi‐Ying , Wang Ning 
Published: January 2021

AbstractLate‐onset multiple acyl‐CoA dehydrogenase deficiency (MADD) is the most common form of lipid storage myopathy. The disease is mainly caused by mutations in electron‐transfer flavoprotein dehydrogenase gene (ETFDH), which leads to decreased levels of ETF:QO in skeletal muscle. However, the specific underlying mechanisms triggering such degradation remain unknown. We constructed expression plasmids containing wild type ETF:QO and mutants ETF:QO‐A84T, R175H, A215T, Y333C, and cultured patient‐derived fibroblasts containing the following mutations in ETFDH: c.250G>A (p.A84T), c.998A>G (p.Y333C), c.770A>G (p.Y257C), c.1254_1257delAACT (p. L418TfsX10), c.524G>A (p.R175H), c.380T>A (p.L127P), and c.892C>T (p.P298S). We used in vitro expression systems and patient‐derived fibroblasts to detect stability of ETF:QO mutants then evaluated their interaction with Hsp70 interacting protein CHIP with active/inactive ubiquitin E3 ligase carboxyl terminus using western blot and immunofluorescence staining. This interaction was confirmed in vitro and in vivo by co‐immunoprecipitation and immunofluorescence staining. We confirmed the existence two ubiquitination sites in mutant ETF:QO using mass spectrometry (MS) analysis. We found that mutant ETF:QO proteins were unstable and easily degraded in patient fibroblasts and in vitro expression systems by ubiquitin‐proteasome pathway, and identified the specific ubiquitin E3 ligase as CHIP, which forms complex to control mutant ETF:QO degradation through poly‐ubiquitination. CHIP‐dependent degradation of mutant ETF:QO proteins was confirmed by MS and site‐directed mutagenesis of ubiquitination sites. Hsp70 is directly involved in this process as molecular chaperone of CHIP. CHIP plays an important role in ubiquitin‐proteasome pathway dependent degradation of mutant ETF:QO by working as a chaperone‐assisted E3 ligase, which reveals CHIP's potential role in pathological mechanisms of late‐onset MADD.
Association between red blood cell distribution width and mortality and severity among patients with COVID‐19: A systematic review and meta‐analysis
Authors: Lee Jane J. , Montazerin Sahar M. , Jamil Adeel , Jamil Umer , Marszalek Jolanta , Chuang Michael L. , Chi Gerald 
Published: January 2021

AbstractEmerging evidence has underscored the potential usefulness of red blood cell distribution width (RDW) measurement in predicting the mortality and disease severity of COVID‐19. This study aimed to assess the association of the plasma RDW levels with adverse prognosis in COVID‐19 patients. A comprehensive literature search from inception to September 2020 was performed to harvest original studies reporting RDW on admission and clinical outcomes among patients hospitalized with COVID‐19. RDW levels were compared between cases (patients who died or developed more severe symptoms) and controls (patients who survived or developed less severe symptoms). A total of 14,866 subjects from 10 studies were included in the meta‐analysis. Higher levels of RDW were associated with adverse outcomes in COVID‐19 patients (mean differences = 0.72; 95% CI = 0.47–0.97; I2 = 89.51%). Deceased patients had higher levels of RDW compared to patients who survived (mean differences = 0.93; 95% CI = 0.63–1.23; I2 = 85.58%). Severely ill COVID‐19 patients showed higher levels of RDW, as opposed to patients classified to have milder symptoms (mean differences = 0.61; 95% CI = 0.28–0.94; I2 = 82.18%). Elevated RDW levels were associated with adverse outcomes in COVID‐19 patients. This finding warrants further research on whether RDW could be utilized as a simple and reliable biomarker for predicting COVID‐19 severity and whether RDW is mechanistically linked with COVID‐19 pathophysiology.
Detection of cardiac involvement in pulmonary sarcoidosis using high‐resolution Holter electrocardiogram
Authors: Maru Yujin , Yodogawa Kenji , Tanaka Toru , Kashiwada Takeru , Iwasaki Yu‐ki , Azuma Arata , Shimizu Wataru 
Published: January 2021

AbstractBackgroundEarly detection of cardiac involvement in patients with sarcoidosis is important but currently unresolved. The aim of this study was to elucidate the utility of frequency domain microvolt T‐wave alternans (TWA), signal‐averaged ECG (SAECG), and heart rate turbulence (HRT) using 24‐hour Holter ECG for detecting cardiac involvement in patients with pulmonary sarcoidosis.MethodsThis study consisted of consecutive 40 pulmonary sarcoidosis patients (11 males, 62 ± 13 years) who underwent 24‐hour Holter monitoring with and without cardiac involvement. All patients underwent frequency domain TWA, SAECG, and HRT using 24‐hour Holter monitoring. Patients with atrial fibrillation pacing or wide QRS electrocardiogram were excluded.ResultsAfter 14 patients were excluded, a total of 26 patients (six males, 59 ± 14 years) were evaluated. Seven patients had cardiac involvement (cardiac sarcoidosis [CS] group). On the Holter SAECG, duration of low‐amplitude signals <40 μV in the terminal filtered QRS complex (LAS40) was significantly higher, and root mean square voltage of the terminal 40 ms of the filtered QRS complex (RMS40) was significantly lower in the CS group compared with the non‐CS group (LAS40: 61.4 ± 35.9 vs 37.6 ± 9.2 ms; P = .018, RMS40: 11.4 ± 10.3 vs 23.6 ± 13.2 ms; P = .023). Prevalence of positive late potential (LP) was also significantly higher in the CS group than that in the non‐CS group (85.7% vs 31.5%; P = .026). The sensitivity, specificity, positive, and negative predictive values of LP for identifying patients with cardiac involvement were 85.7%, 68.4%, 50.0%, and 92.8%, respectively.ConclusionHolter SAECG may be useful for detecting cardiac involvement in patients with pulmonary sarcoidosis.
Detection and attribution of precipitation trends associated with the poleward shift of the South Atlantic Convergence Zone using CMIP5 simulations
Authors: Zilli Marcia T. , Carvalho Leila M. V. 
Published: January 2021

AbstractObservational studies demonstrate a poleward shift in the South Atlantic Convergence Zone (SACZ) in recent decades with regional impacts on extreme precipitation. However, the relative contribution of anthropogenic forcing and natural variability to the observed trends is presently unknown. To evaluate the main forcings associated with the recent trends in precipitation rate consistent with the poleward shift of the SACZ, this study examines the last 40 years of the historical scenario of 20 global climate models participating in the fifth phase of the Coupled Model Intercomparison Project (CMIP5). The relative contribution of natural variability and anthropogenic‐related forcings to precipitation in the 20th century is assessed based on historical, natural, anthropogenic, and pre‐industrial simulations from CMIP5 models. Over the study area, precipitation trends simulated by the CMIP5 historical scenario are small, with large disagreement among models and members. Despite the small magnitude of the trends, the spatial pattern emerging is coherent with a poleward displacement of the SACZ, as indicated by a reduction (increase) in the precipitation rate over Southeastern Brazil (Southeastern South America). Even with large discrepancies among models, the attribution analysis suggests that precipitation trends could partially result from land‐use change, followed by changes in ozone concentrations, with more significant influence over Southeastern Brazil. The large uncertainty in the simulated precipitation suggests that not all mechanisms related to the position and intensity of the SACZ events are well captured by the CMIP5 models considered here.
Attitudes and Perceptions of Blenderized Tube Feed Use Among Physicians and Advanced Practice Providers
Authors: Eustace Kathleen , Cole Lillian , Hollaway Lauren 
Published: January 2021

AbstractBackgroundBlenderized tube feeds (BTFs) have increased in popularity among enteral patients and their caregivers as an alternative to commercial formula. Motivations include ingredient flexibility, increased tolerance, and inclusion in family meals. Research has explored the attitudes and perceptions of patients, caregivers, and registered dietitian nutritionists but not those of physicians and advanced practice providers (APPs). The purpose of this study was to evaluate their attitudes and perceptions of BTFs.MethodsA survey created via Research Electronic Data Capture tools was distributed to physicians and APPs to evaluate clinical practice characteristics, experience, knowledge, and perceptions of BTFs.ResultsSurvey response rate was 17.3% (n = 206). Half of respondents reported familiarity with BTFs, but 95% of respondents had received no formal training regarding BTFs. Physicians reported higher levels of confidence in BTF knowledge than APPs. However, the overall level of confidence in BTF knowledge was poor, with a majority of respondents (73.3% [n = 151]) reporting either “not very confident” or “not confident at all.” The most perceived benefits included tailoring diet to better fit the patients’ needs (70.9% [146]) and psychological benefits (59.2% [122]). Barriers that most concerned respondents included tube occlusions (59.7% [123]) and nutrition inadequacy (47.6% [98]). χ2 Analysis revealed no relationship between type of provider and perceived benefits or barriers.ConclusionA majority of physicians and APPs are willing to support BTF use but lack education or guidance, resulting in poor confidence. Increased familiarity with BTF use and awareness of available education materials are required to help physicians and APPs support patients utilizing BTFs.
Nitrogen nutritional status in perennial grasses under defoliation: Do stubble height and mixed cultivation matter?
Authors: Américo Luana Fidelis , Duchini Paulo Gonçalves , Schmitt Daniel , Guzatti Gabriela Cristina , Lattanzi Fernando Alfredo , Sbrissia André Fischer 
Published: January 2021

AbstractBackground and aims: The concepts of critical nitrogen (N) concentration and nitrogen nutrition index (NNI) provide effective tools for assessing the N nutritional status of pastoral systems. However, no conclusive data exist concerning the effects of stubble height at harvest on the N dilution curve [N concentration (N%) = a(biomass)−b] and mixed species cultivation on N nutritional status of forage plants with contrasting growth strategies. To evaluate these effects, three cool‐season perennial grasses (exploitative, Arrhenatherum elatius L.; moderately exploitative, Dactylis glomerata L.; conservative, Festuca arundinacea Scherb.) were grown under different cultivation methods either as pure stands or as a mixture and conducted two experiments.Methods: In experiment I, canopies of different grass species pure stands and mixtures were subjected to defoliation managements aimed to not exceed the critical LAI at harvest. Forage samples were collected from October 2015 to August 2016 to determine the N nutritional status throughout the seasons. In experiment II, half of each pure stand was mowed to 10 cm in height, and the other half to ground level. Thereafter, herbage samples were taken during regrowth to determine N dilution curves in both scenarios (from ground level and from stubble height of 10 cm).Results: We verified an NNI above 0.8 during autumn and winter for A. elatius, D. glomerata, and the mixture but only in winter for F. arundinacea. Dactylis glomerata grown in the mixture had a lower N% than its pure stand during autumn and winter, whereas A. elatius and F. arundinacea showed no differences irrespective of cultivation method throughout the year. The three forage species presented classical dilution curves when regrowth was initiated from ground level, but quadratic trends were observed when regrowth was initiated from stubble heights of 10 cm.Conclusions: Overall the N nutritional status of cool‐season perennial grasses was not affected by cultivation method when pastures were managed in conditions of non‐limiting N supply and with pre‐cut/grazing height targets derived from critical LAI measured at the full vegetative growth stage. Moreover, the N nutrition status in pastures managed under moderate/lenient defoliation should not be evaluated during periods of less intense canopy renewal and/or right after the cut/grazing events.
Effects of combined application of high‐pressure processing and active coatings on phenolic compounds and microbiological and physicochemical quality of apple cubes
Authors: Bambace María Florencia , del Rosario Moreira María , Sánchez‐Moreno Concepción , De Ancos Begoña 
Published: January 2021

AbstractBACKGROUNDIn recent years the use of high‐pressure processing (HPP) of fruit products has steadily increased due to its antimicrobial effectiveness and the retention of nutritional and quality attributes compared to conventional thermal technologies. Edible coatings are already being used to enhance the quality of minimally processed fruits. Thus, apple cubes (AC) and alginate–vanillin‐coated apple cubes (AVAC) were subjected to HPP (400 MPa/5 min/35 °C). The microbiological and physicochemical parameters were evaluated and the bioactive compounds were monitored before and after HPP of apple cubes. Also, an in vitro gastrointestinal digestion (GID) was conducted.RESULTSHPP left L. monocytogenes counts below the detection limit (2 log UFC g−1), regardless of the presence of coating. For E. coli, HPP + active coating showed a synergism affording the greatest reduction (>5 log) for AVAC‐HPP. Firmness was maintained in AVAC‐HPP samples, while AC‐HPP samples suffered reductions of 35%. Colour attributes were also better retained in AVAC‐HPP samples. In general, HPP led to a decrease in phenolic compounds. Regarding the effects of GID, vanillin‐based active coating exerted a protective effect on some phenolics. Thus, p‐coumaroylquinic acid concentration was maintained for AVAC and AVAC‐HPP during GID. Epigallocatechin, the compound with the highest concentration in apple cubes, increased for AVAC (106%) and AVAC‐HPP (57%). Also, phloridzin concentration increased for AVAC‐HPP (17%). At the end of GID, procyanidin B1 and epigallocatechin were the main phenolic compounds for all samples, AVAC showing the highest concentration.CONCLUSIONSThis work demonstrates that the combined application of HPP and active coatings on apple cubes could be used to obtain a safe and good‐quality product. © 2021 Society of Chemical Industry
Effect of ultrasonic pretreatment monitored by real‐time online technologies on dried preparation time and yield during extraction process of okra pectin
Authors: Zhang Lei , Hu Yang , Wang Bei , Xu Xin , Yagoub Abu ElGasim A , Fakayode Olugbenga Abiola , Ma Haile , Zhou Cunshan 
Published: January 2021

AbstractBACKGROUNDUltrasonic pretreatment is a novel physical method that can be used in the extraction process of okra pectin. Real‐time online monitoring technologies were introduced in time and frequency domains when okra was pretreated. Preparation time of dried okra and yield of okra pectin were studied; and physicochemical properties of okra pectin were analyzed at the optimum ultrasonic parameter.RESULTSResults showed that ultrasonic intensity of sweeping‐frequency ultrasonic (SFU) pretreatment was stronger than that of fixed‐frequency ultrasonic pretreatment (FFU). SFU pretreatment (60 ± 1 kHz) at 30 min had a strong ultrasonic voltage peak of 0.05387 V and signal power peak of −6.62 dBm. The preparation time of dried okra was 160 ± 14.14 min in the pretreated group, 44.83% lower than control without SFU pretreatment. The intercellular space was 56.03% higher than control. Water diffusion coefficient increased from 1.41 × 10−9 to 2.14 × 10−9 m2 s−1. Monobasic quadratic equations were developed for the monitored ultrasonic intensity and pectin yield. Compared to control, extraction yield (16.70%), pectin content (0.564 mg mg−1), solubility (0.8187 g g−1) and gel strength (30.91 g) were improved in the pretreated group. Viscosity decreased, and values of G′ and G″ crossing at 63 rad s−1 revealed the viscoelastic behavior and the beginning of viscous behavior with a sol state.CONCLUSIONDecrement of dried preparation time and increment of yield were achieved by ultrasonic pretreatment during the extraction process of okra pectin, and the relationship of ultrasonic intensity monitored by real‐time online technologies and yield was given. © 2021 Society of Chemical Industry
Blood testis barrier revisited—Analysis of post‐chemotherapy germ cell tumor orchidectomy and retroperitoneal lymph node dissection specimens
Authors: Gupta Amit , Noronha Jarin , Bakshi Ganesh , Menon Santosh , Pal Mahendra , Joshi Amit , Prabash Kumar , Noronha Vanita , Murthy Vedang , Krishnattry Rahul , Patil Akshay , Prakash Gagan J. 
Published: January 2021

AbstractObjectiveTo assess the response of chemotherapy on the primary tumor, compare it with the response in retroperitoneal disease, and study factors associated with pathological complete response.MethodsWe conducted a retrospective audit of all high inguinal orchidectomies (HIOs) performed after chemotherapy between 2012 and 2019 at a tertiary cancer center in India. Patient characteristics and histopathological response were extracted from electronic medical records, and predictors of testicular disease response were assessed.ResultsOf the 260 retroperitoneal lymph node dissections (RPLNDs) performed in the study period, 37 HIOs (14.23%) were carried out after chemotherapy. The median age of presentation was 28 years (16–41). Histopathology was divided into a viable tumor, mature teratoma, and necrosis/scarring. Residual disease was seen in 17 RPLND (46.0%) and 18 HIO (48.6%) specimens respectively. Of these 18, three patients had a residual viable tumor in the testis, and the remaining had a mature teratoma. Clinico‐radiological assessment showed an average reduction of 61% in testicular disease size following chemotherapy. On orchidectomy histopathological assessment, the median tumor size was 9, 4, and 1.5 cm in specimens with a viable tumor, mature teratoma, and necrosis/scarring, respectively.ConclusionsA low threshold for upfront chemotherapy in patients with a high disease burden may be considered as tumors within the testis respond to chemotherapy in more than half of the patients. Discordance rates of residual cancer in RPLND and HIO specimens exist but post‐chemotherapy tumor size in testis correlates with the presence of a residual viable tumor.
Cucurbituril and zwitterionic surfactant‐based matrix solid‐phase dispersion microextraction to simultaneously determine terpenoids from Radix Curcumae
Authors: Chen Yan , Zhen Xiao‐Ting , Yu Ya‐Ling , Shi Min‐Zhen , Cao Jun , Zheng Hui , Ye Li‐Hong 
Published: January 2021

AbstractA rapid, efficient, and environmentally friendly matrix solid‐phase dispersion microextraction was established to determine and quantify terpenoids in Radix Curcumae using ultra‐high‐performance liquid chromatography with a diode array detector. Various parameters affecting the extraction were investigated in detail, such as the grinding time, amount of adsorbent, type and concentration of elution solvent, and pH. The optimization of single‐factor and response surface methodology was performed to confirm the best conditions in this procedure. The final optimized conditions were obtained by applying 70 mg of cucurbituril as adsorbent, 149 s as the optimum grinding time, and 228 mM of 3‐(N,N‐dimethylpalmitylammonio)propanesulfonate aqueous solution (pH 6.5) as the optimal elution solvent. The validated method showed a satisfactory linear range of 0.10–10 µg/mL for curdione and furanodiene, 0.01–10 µg/mL for isocurcumenol and germacrone, and 0.05–10 µg/mL for furanodienone, while the correlation coefficients ranged from 0.9945 to 0.9970. The recoveries of the investigated analytes at two spiked concentration levels (0.1 and 1.0 µg/mL) ranged from 96.53 to 104.60%. In addition, this method displayed acceptable reproducibility (relative standard deviation ≤ 3.66%). The results showed that the newly proposed matrix solid‐phase dispersion microextraction method was successfully applied to analyze curdione, isocurcumenol, furanodienone, germacrone and furanodiene in Radix Curcumae samples.
The Significance of Fetal Brain Ventricular Asymmetry Without Dilation
Authors: Meyer Raanan , Bar‐Yosef Omer , Barzilay Eran , Radinsky Igal , Segal Chen Key , Ziv‐Baran Tomer , Shani Hagit , Levin Gabriel , Katorza Eldad 
Published: January 2021

ObjectivesFetal brain non‐dilated ventricular asymmetry (NDVA) is a common finding on prenatal ultrasound exams. However, the optimal prenatal management in these cases remains unknown. We aimed to evaluate the benefit of prenatal genetic and magnetic resonance imaging (MRI) exams performed in cases of fetal NDVA detected on ultrasound.MethodsA historical cohort study from a tertiary medical center. Singleton pregnancies with fetal brain NDVA diagnosed on ultrasound were included. We defined ventricular asymmetry as a difference of ≥2.0 mm between the lateral ventricles and ventricular dilation as ventricular width of >10.0 mm. Outcomes were evaluated with genetic exams (karyotype and chromosomal microarray analysis [CMA]) and fetal brain MRI.ResultsDuring the study period, there were 145 cases diagnosed with NDVA on ultrasound that comprised the cohort study. The rate of abnormal karyotype was 1.8% (1/56) and of abnormal CMA was 10% (3/30). The rate of minor additional CNS findings did not differ between ultrasound and MRI (3.4 versus 2.8%, respectively, p = .74). No major additional fetal brain findings were detected on MRI performed after ultrasound.ConclusionsIn cases diagnosed with NDVA on ultrasound, no significant additional anomalies were detected on fetal brain MRI. The rate of abnormal genetic tests was relatively high and warrants further studies.
Fetal‐TAPSE for Surveillance of Cardiac Function in Growth‐Restricted Fetuses With a Portosystemic Shunt
Authors: Bardin Ron , Perlman Sharon , Hadar Eran , Mozer Glassberg Yael , Bruckheimer Elchanan , Silber Michal , Gilboa Yinon 
Published: January 2021

ObjectivesCongenital portosystemic shunt (CPSS) in a growth‐restricted fetus may lead to cardiac overload and ultimately hemodynamic imbalance. The aim of the study was to describe the application of tricuspid annular plane systolic excursion (TAPSE) for surveillance of cardiac function in growth‐restricted fetuses diagnosed with CPSS.MethodsThe study group consisted of 7 fetuses with growth restriction diagnosed with CPSS between 2018 and 2020. Patients were followed longitudinally every 2 weeks. Sonographic fetal‐TAPSE (f‐TAPSE) was performed every 2 weeks. At each visit, the following parameters were recorded: estimated fetal weight, biophysical profile, nonstress test, Doppler flow indices, and fetal cardiothoracic index. Postnatal laboratory and imaging tests were retrieved from the medical files.ResultsMean gestational age at diagnosis of CPSS was 32 + 1 weeks. Cardiomegaly was observed in all cases. All portosystemic shunts were classified as intrahepatic. Values of f‐TAPSE were above the 95th percentile in 6/7 fetuses at presentation and throughout follow‐up. Gestational age at delivery ranged between 36 + 5 and 38 + 5 weeks. Postnatally, spontaneous closure of the shunt was noted in 2 infants. Transient hyperammonemia was diagnosed in 2 neonates, with no signs of the characteristic complication.ConclusionsIn growth‐restricted fetuses diagnosed concomitantly with CPSS, f‐TAPSE offers a practical sonographic tool for assessment of cardiac function and may serve as an additional clinical marker for follow‐up. The appearance of cardiomegaly in growth‐restricted fetuses should prompt a dedicated sonographic evaluation of the fetal portal system.