bluuebunny/crossref_metadata_2025_split huggingface dataset:
Familial Hypocalciuric Hypercalcemia in Pregnancy: Diagnostic Pitfalls
Authors: Jones Alicia R , Hare Matthew JL , Brown Justin , Yang Jun , Meyer Caroline , Milat Frances , Allan Carolyn A 
Published: April 2020

ABSTRACTFamilial hypocalciuric hypercalcemia (FHH) is a group of autosomal dominant disorders caused by dysfunction of the calcium sensing receptor (CaSR) and its downstream signaling proteins, leading to generally asymptomatic hypercalcemia. During pregnancy, distinguishing FHH from primary hyperparathyroidism (PHPT) is important, as the latter is associated with adverse outcomes and can be treated surgically during pregnancy, whereas the former is benign. This case report highlights the difficulties in diagnosing FHH during pregnancy. A 32‐year‐old woman was found to have asymptomatic hypercalcemia at 14‐weeks’ gestation. Investigations showed a corrected calcium (cCa) of 2.61 mmol/L (2.10 to 2.60), ionized Ca (iCa) of 1.40 mmol/L (1.15 to 1.28), 25OHD of 33 nmol/L (75 to 250), and PTH of 9.5 pmol/L (1.5 to 7.0). The patient was treated with 2000 IU cholecalciferol daily with normalization of 25OHD. The urine calcium / creatinine clearance ratio (CCCR) was 0.0071, and neck US did not visualize a parathyroid adenoma. Upon a retrospective review of the patient's biochemistry from 2 years prior, hypercalcemia was found that was not investigated. The patient was monitored with serial iCa levels and obstetric US. She delivered a healthy boy at 38‐weeks’ gestation. Postnatal iCa was 1.48 mmol/L and remained elevated. Her son had elevated iCa at birth of 1.46 mmol/L (1.15 to 1.33), which rose to 1.81 mmol/L by 2 weeks. He was otherwise well. Given the familial hypercalcemia, a likely diagnosis of FHH was made. Genetic testing of the son revealed a missense mutation, NM_000388.3(CASR):c.2446A > G, in exon 7 of the CaSR, consistent with FHH type 1. To our knowledge, there are only three existing reports of FHH in pregnancy. When differentiating between FHH and PHPT in pregnancy, interpretation of biochemistry requires an understanding of changes in Ca physiology, and urine CCCR may be unreliable. If the decision is made to observe, clinical symptoms, calcium levels, and fetal US should be monitored, with biochemistry and urine CCCR performed postpartum, once lactation is completed © 2020 The Authors. JBMR Plus published by Wiley Periodicals, Inc. on behalf of American Society for Bone and Mineral Research.
Cover Image, Volume 100, Issue 6
Authors: Ye Mengliang , Zhang Chunhui , Zhu Lingyu , Jia Wei , Shen Qingshan 
Published: April 2020

The cover image is based on the Research Article Yak (Bos grunniens) bones collagen‐derived peptides stimulate osteoblastic proliferation and differentiation via the activation of Wnt/β‐catenin signaling pathway by Mengliang Ye et al., https://doi.org/10.1002/jsfa.10286.
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Nano‐vesicular formulation of propolis and cytotoxic effects in a 3D spheroid model of lung cancer
Authors: Ilhan‐Ayisigi Esra , Ulucan Fulden , Saygili Ecem , Saglam‐Metiner Pelin , Gulce‐Iz Sultan , Yesil‐Celiktas Ozlem 
Published: April 2020

AbstractBACKGROUNDPropolis exhibits therapeutic properties due to the presence of phenolic acids, esters, and flavonoids. The scope of this study was to develop a nano‐vesicular formulation and establish a three‐dimensional (3D) spheroid model in which lung cancer is recapitulated.RESULTSNiosome vesicles doped with galangin‐rich propolis extract were synthesized by the ether injection method using a cholesterol : surfactant mass ratio of 1 : 3 at 40 °C for 1 h. Formulated niosomes were administered to 3D lung cancer spheroid model and the cytotoxicity was compared with that of a two‐dimensional (2D) setting. The galangin content was determined as 86 μg mg–1 propolis extract by ultra‐performance liquid chromatography (UPLC). The particle size of loaded niosome was 151 ± 2.84 nm with a polydispersity index (PDI) of about 0.232, and an encapsulation efficiency of 70% was achieved.CONCLUSIONThe decrease in cell viability and the scattering in the 3D spheroids of A549 lung cancer cells treated with propolis‐loaded niosomes were notable, indicating a profound cytotoxic effect and suggesting that they can be utilized as an effective nano‐vesicle. © 2020 Society of Chemical Industry
Proteomic analysis reveals changes in energy metabolism of skeletal muscle in beef cattle supplemented with vitamin A
Authors: Campos Carolina F , Costa Thaís C , Rodrigues Rafael T S , Guimarães Simone E F , Moura Felipe H , Silva Walmir , Chizzotti Mario L , Paulino Pedro V R , Benedeti Pedro D B , Silva Fabyano F , Duarte Marcio S 
Published: April 2020

AbstractBACKGROUNDVitamin A has been reported as a factor influencing marbling deposition in meat from animals. Although the mechanisms by which vitamin A regulates lipid metabolism in mature adipocytes are already well‐established, information regarding molecular mechanisms underlying the effects of vitamin A on the regulation of intramuscular fat deposition in beef cattle still remains limited. The present study aimed to assess the molecular mechanisms involved in the intramuscular fat deposition in beef cattle supplemented with vitamin A during the fattening phase using a proteomic approach.RESULTSVitamin A supplementation during the fattening phase decreased intramuscular fat deposition in beef cattle. Proteome and phospho‐proteome analysis together with biological and networking analysis of the protein differentially abundant between treatments indicated that Vitamin A supplementation affects the overall energy metabolism of skeletal muscle, impairing lipid biosynthesis in skeletal muscle.CONCLUSIONVitamin A supplementation at fattening phase impairs intramuscular fat deposition in beef cattle likely by changing the energy metabolism of skeletal muscle. The interaction of retinoic acid and heat shock 70‐kDa protein may play a pivotal role in intramuscular fat deposition as a consequence of vitamin A supplementation by impairing de novo fatty acid synthesis as a result of a possible decrease in insulin sensitivity in the skeletal muscle. © 2020 Society of Chemical Industry
Optimal perfusion chemotherapy: A prospective comparison of mitomycin C and oxaliplatin for hyperthermic intraperitoneal chemotherapy in metastatic colon cancer
Authors: Woeste Matthew R. , Philips Prejesh , Egger Michael E. , Scoggins Charles R. , McMasters Kelly M. , Martin Robert C. G. 
Published: April 2020

AbstractBackgroundPeritoneal carcinomatosis of colorectal adenocarcinoma (CRC) origin is common and is the second‐most frequent cause of death in colorectal cancer. There is survival benefit to surgical resection plus hyperthermic intraperitoneal chemotherapy (HIPEC) for patients with metastatic CRC. However, there remains controversy between oxaliplatin (Oxali) and mitomycin C (MMC), as the agent of choice.MethodsA review of our 285 patients prospective HIPEC database from July 2007 to May 2018 identified 48 patients who underwent cytoreductive surgery plus HIPEC with MMC or Oxali. Patients were stratified based on preoperative and postoperative peritoneal cancer indices (PCI). The primary outcomes of survival and progression‐free survival were compared.ResultsType of HIPEC chemotherapy was not found to be predictive of overall survival. Preoperative PCI (P = .04), preoperative response to chemotherapy (P = .0001), and postoperative PCI (P = .05) were predictive for overall survival.ConclusionsMMC or Oxali based HIPEC chemotherapy are both safe and effective for the management of peritoneal only metastatic CRC. Both perfusion therapies should be considered with all patients receiving modern induction chemotherapy.
Carbon nanospheres as solid‐phase microextraction coating for the extraction of polycyclic aromatic hydrocarbons from water and soil samples
Authors: Chang Qingyun , Wang Mengting , Zhang Guijiang , Zang Xiaohuan , Li Hongda , Zhang Shuaihua , Wang Chun , Wang Zhi 
Published: April 2020

AbstractA solid‐phase microextraction with carbon nanospheres coated fiber coupled with gas chromatographic detection was established for the determination of eight polycyclic aromatic hydrocarbons (naphthalene, biphenyl, acenaphthene, fluorine, phenanthrene, anthracene, fluoranthene, and pyrene) in water and soil samples. The experimental parameters (extraction temperature, extraction time, stirring rate, headspace volume, salt content, and desorption temperature) which affect the extraction efficiency were studied. Under the optimized conditions, good linearity between the peak areas and the concentrations of the analytes was achieved in the concentration range of 0.5‐300 ng/mL for water samples, and in the concentration range of 6.0‐2700 ng/g for soil samples. The detection limits for the analytes were in the range of 0.12‐0.45 ng/mL for water samples, and in the range of 1.53‐2.70 ng/g for soil samples. The method recoveries of the polycyclic aromatic hydrocarbons for spiked water samples were 80.10‐120.1% with relative standard deviations less than 13.9%. The method recoveries of the analytes for spiked soil samples were 80.40‐119.6% with relative standard deviations less than 14.4%. The fiber was reused over 100 times without a significant loss of extraction efficiency.
Orofacial Strength and Voice Quality as Outcome of Levodopa Challenge Test in Parkinson Disease
Authors: Lechien Jérôme R. , Delsaut Bertil , Abderrakib Anissa , Huet Kathy , Delvaux Veronique , Piccaluga Myriam , Khalife Mohamad , Harmegnies Bernard , Saussez Sven , Blecic Serge 
Published: April 2020

ObjectiveTo assess the usefulness of orofacial strength and voice quality as assessment of response to levodopa challenge test (LCT) used in the diagnosis of early idiopathic Parkinson disease (IPD).Study DesignControlled Prospective Study.MethodsFrom January 2014 to April 2019, patients with early IPD and healthy individuals were recruited and evaluated for clinical findings (Hoehn and Yahr scale; Unified Parkinson's Disease Rating Scale); Voice Handicap Index (VHI); grade of dysphonia, roughness, breathiness, asthenia, and strain and instability (GRBASI); maximal phonation time; phonation quotient; acoustic parameters; and orofacial muscle strength Oral Performance Instrument (IOPI; IOPI Medical, Woodinville, WA, USA) t) at baseline and 45 minutes after the levodopa intake (LCT).ResultsA total of 32 IPD patients and 20 healthy individuals completed the study. Healthy individuals exhibited better VHI, grade of dysphonia, breathiness, asthenia, strain, instability, and acoustic measurements (noise‐related, tremor, F0 short‐ and mid‐term and intensity short‐term parameters) than healthy subjects. The mean values of muscle strength of lips, cheeks, fundamental frequency (F0), highest F0, and shimmer significantly improved from pre‐ to post‐LCT in IPD patients. Healthy individuals did not exhibit significant changes of orofacial strength and voice quality assessment from pre‐ to post‐LCT. Significant associations were found between clinical, orofacial strength, and some aerodynamic and acoustic measurements.ConclusionOrofacial strength and acoustic voice quality measurements may be used as objective outcomes of the LCT responsiveness in patients with early IPD.Level of Evidence3A. Laryngoscope, 2020
A novel heterozygous HTRA1 mutation is associated with autosomal dominant hereditary cerebral small vessel disease
Authors: Zhuo Zhong‐ling , Cong Lu , Zhang Jun , Zhao Xiao‐tao 
Published: April 2020

AbstractBackgroundWe investigated whether a heterozygous mutation that we newly identified in HTRA1 (high‐temperature requirement serine protease A1 gene) in a pedigree with autosomal dominant hereditary cerebral small vessel disease (SVD) reduces the function of HTRA1 and affects the transforming growth factor‐β1 (TGF‐β1)/Smad signaling.MethodsWhole‐exome sequence from the proband and her two sisters was examined using whole‐exome enrichment and sequencing. Expression of HTRA1 and TGF‐β1/Smad and HTRA1 activity were assayed using sodium dodecyl sulfate‐polyacrylamide gel electrophoresis and western blotting analyses after transfecting wild‐type and mutant HTRA1 genes into HEK293 cells.ResultsA new heterozygous mutation (c.614C>G:p.Ser205Cys) in HTRA1 was identified in the sequence encoding the trypsin‐like serine protease domain. The mutation was predicted to be deleterious by in silico tools. Moreover, in vitro activity and protein analyses revealed a loss‐of‐function effect of the mutation: the proteolytic activity of mutant HTRA1 was decreased, and, notably, this was accompanied by an increase in TGF‐β1/Smad protein levels.ConclusionsThe heterozygous mutation HTRA1 S205C causing diminished protease activity is associated with—and could represent a cause of—autosomal dominant hereditary cerebral SVD. Our results also indicate a relationship between HTRA1 and TGF‐β1/Smad signaling.
How young patients with cancer perceive the COVID‐19 (coronavirus) epidemic in Milan, Italy: Is there room for other fears?
Authors: Casanova Michela , Pagani Bagliacca Elena , Silva Matteo , Patriarca Carlo , Veneroni Laura , Clerici Carlo Alfredo , Spreafico Filippo , Luksch Roberto , Terenziani Monica , Meazza Cristina , Podda Marta , Biassoni Veronica , Schiavello Elisabetta , Chiaravalli Stefano , Puma Nadia , Bergamaschi Luca , Gattuso Giovanna , Sironi Giovanna , Massimino Maura , Ferrari Andrea 
Published: April 2020

AbstractThe rapid spread of coronavirus disease 2019 epidemic in Italy, in particular in the Milan focal point, required drastic measures and led to panic in the population. While in our center we did not change our approach to the treatment of our young patients with cancer, we developed a qualitative survey to assess their perception of the risk and level of stress. The survey showed that a relatively large proportion of young patients felt personally at risk of severe complications. We believe that we need to adequately inform our patients, focusing on hygienic measures and personal protection and prompt reporting of any suspicious symptoms.
Integrative structure and function of the yeast exocyst complex
Authors: Ganesan Sai J. , Feyder Michael J. , Chemmama Ilan E. , Fang Fei , Rout Michael P. , Chait Brian T. , Shi Yi , Munson Mary , Sali Andrej 
Published: April 2020

AbstractExocyst is an evolutionarily conserved hetero‐octameric tethering complex that plays a variety of roles in membrane trafficking, including exocytosis, endocytosis, autophagy, cell polarization, cytokinesis, pathogen invasion, and metastasis. Exocyst serves as a platform for interactions between the Rab, Rho, and Ral small GTPases, SNARE proteins, and Sec1/Munc18 regulators that coordinate spatial and temporal fidelity of membrane fusion. However, its mechanism is poorly described at the molecular level. Here, we determine the molecular architecture of the yeast exocyst complex by an integrative approach, based on a 3D density map from negative‐stain electron microscopy (EM) at ~16 Å resolution, 434 disuccinimidyl suberate and 1‐ethyl‐3‐(3‐dimethylaminopropyl)carbodiimide hydrochloride cross‐links from chemical‐crosslinking mass spectrometry, and partial atomic models of the eight subunits. The integrative structure is validated by a previously determined cryo‐EM structure, cross‐links, and distances from in vivo fluorescence microscopy. Our subunit configuration is consistent with the cryo‐EM structure, except for Sec5. While not observed in the cryo‐EM map, the integrative model localizes the N‐terminal half of Sec3 near the Sec6 subunit. Limited proteolysis experiments suggest that the conformation of Exo70 is dynamic, which may have functional implications for SNARE and membrane interactions. This study illustrates how integrative modeling based on varied low‐resolution structural data can inform biologically relevant hypotheses, even in the absence of high‐resolution data.
Structural, mechanical, electronic, and thermodynamic properties of pure tungsten metal under different pressures: A first‐principles study
Authors: Jiang Diyou , Zhong Shuying , Xiao Wenbo , Liu Desheng , Wu Musheng , Liu Sanqiu 
Published: April 2020

AbstractThe structural, mechanical, electronic, and thermodynamic properties of pure W metal under different pressures have been investigated using the first‐principles method. Our calculated structural parameters are in good agreement with experimental and previous theoretical results. The obtained elastic constants show that pure W metal is mechanically stable. Elastic properties such as the bulk modulus (B), shear modulus (G), Young's modulus (E), Poisson's ratio (ν), Cauchy pressure (C′), and anisotropy coefficients (A) are calculated by the Voigt‐Reuss‐Hill method. The results show that the pressure can improve the strength of pure tungsten and has little effect on the ductility. In addition, the total density of states as a function of pressure is analyzed. Thermodynamic properties such as the Debye temperature, phonon dispersion spectrum, free energy, entropy, enthalpy, and heat capacity are also discussed.
Experimental and DFT Studies on the FT‐IR, NMR and UV/Vis Spectra of a Xanthene Derivative: The Case of 9‐benzoyl‐3,4,5,6,7,9‐hexahydro‐1h‐xanthene‐1,8(2h)‐dione
Authors: Sima Abdollahi Mrs. , Nemati‐Kande Ebrahim , Poursattar Marjani Ahmad 
Published: April 2020

Abstract9‐benzoyl‐3,4,5,6,7,9‐hexahydro‐1H‐xanthene‐1,8(2H)‐dione was synthesized and studied uing FT‐IR, NMR and UV/Vis spectra both experimentally and using density functional theory (DFT) methods. The absoulte average deviation (AAD) between experimental NMR chemical shifts and DFT results calculated at B3LYP/6‐311+g(d,p) level was 3.9 % and 4.8 % for 1H and 13C‐NMR, respectively. Scaled quantum mechanical (SQM) method was implemented to scale the calculated harmonic frequencies, and it was found that, the experimental and SQM frequencies are in acceptable agreement with AAD of ∼7 cm−1. UV/Vis spectra in water, ethanol, acetonitrile and n‐hexane solvents measured experimentally and calculated using time‐dependent CAM‐B3LYP/6‐311+g(d,p) method. Bathochromic shift of some electronic excitations are also discussed based on the linear solvation energy relationships. Molecular orbital, natural transition orbital and hole‐electron analyses were used to further analyze the nature of the electron transitions. In Uv/Vis spectra, only S0→S19 excitation is a charge transfer transition, where the other excitations are local electron transitions.
Synergistic Effect of CdS and NH2‐UiO‐66 on Photocatalytic Reduction of CO2 under Visible Light Irradiation
Authors: Xie Xia‐Fei , Dao Xiao‐Yao , Guo Fan , Zhang Xiao‐Yu , Wang Fang‐Ming , Sun Wei‐Yin 
Published: April 2020

AbstractPhotocatalytic reduction of CO2 has drawn much attention as a promising way that can effectively alleviate the issue of global warming and energy crisis. In this work, we fabricated a series of CdS/NH2‐UiO‐66 composites with varied CdS amounts and tested their capacity for photocatalytic reduction of CO2. The reactions were performed in a solvent‐free system and displayed excellent selectivity for CO production without liquid phase products. When the content of CdS in the composite is up to 40 %, the hybrid catalyst produces 105.41 μmol/g within 6 h, exhibiting remarkable photocatalytic performance, which is much higher than that catalyzed by mechanically mixed CdS and NH2‐UiO‐66. The brilliant catalytic performance of the composite catalyst is attributed to the formation of heterostructure between the well‐dispersed CdS and NH2‐UiO‐66, which promotes the enhancement of electron transfer during photocatalysis.
Tailoring Hierarchically Porous Nitrogen‐, Sulfur‐Codoped Carbon for High‐Performance Supercapacitors and Oxygen Reduction
Authors: Lu Huihang , Yang Chao , Chen Jing , Li Jun , Jin Huile , Wang Jichang , Wang Shun 
Published: April 2020

AbstractHeteroatom‐doped carbon materials are intensively studied in supercapacitors and fuel cells, because of their great potential for sustainably bearing on the energy crisis and environmental pollution. Although enormous efforts are put in material perfection with a hierarchically porous microstructure, the simultaneous optimization of both porous structures and surface functionalities is hard to achieve due to inevitable concurrent dopant leaching effect and structural collapse under required high pyrolysis temperature. In this study, an in situ dehalogenation polymerization and activation protocol is introduced to synthesize nitrogen‐ and sulfur‐codoped carbon materials (NS‐PCMs) with hierarchical pore distribution and abundant surface doping, which endows them with good conductivity, abundant accessible active sites, and efficient mass transport. As a result, the as‐prepared carbon materials (NS‐a‐PCM‐1000) show an excellent mass specific capacitance of 461.5 F g−1 at a current density of 0.1 A g−1, long cycle life (>23 k, 10 A g−1), and high device energy and power density (17.3 Wh kg−1, 250 W kg−1). Significantly, NS‐a‐PCM‐1000 also exhibits one of the highest oxygen reduction reaction activities (onset potential of 1.0 V vs reversible hydrogen electrode) in alkaline media among all reported metal‐free catalysts.
Mitigating off-target effects in CRISPR/Cas9-mediated in vivo gene editing
Authors: Han Hua Alexander , Pang Jeremy Kah Sheng , Soh Boon-Seng 
Published: April 2020

AbstractThe rapid advancement of genome editing technologies has opened up new possibilities in the field of medicine. Nuclease-based techniques such as the CRISPR/Cas9 system are now used to target genetically linked disorders that were previously hard-to-treat. The CRISPR/Cas9 gene editing approach wields several advantages over its contemporary editing systems, notably in the ease of component design, implementation and the option of multiplex genome editing. While results from the early phase clinical trials have been encouraging, the small patient population recruited into these trials hinders a conclusive assessment on the safety aspects of the CRISPR/Cas9 therapy. Potential safety concerns include the lack of fidelity in the CRISPR/Cas9 system which may lead to unintended DNA modifications at non-targeted gene loci. This review focuses modifications to the CRISPR/Cas9 components that can mitigate off-target effects in in vitro and preclinical models and its translatability to gene therapy in patient populations.
Implementation Analysis of a Perioperative Patient Safety Program in Guatemala
Authors: Sico Isabelle P. , Hall Bria J. , Aguilar‐González Angie , Orozco Monica , Ramirez Carla , Baumgartner Joy Noel , Boyd David , Bolaños Javier , Calgua Erwin , Lou‐Meda Randall , Rice Henry E. 
Published: April 2020

AbstractBackgroundThe implementation of programs to improve patient safety remains challenging in low‐ and middle‐income countries. The goal of our study was to define the barriers and facilitators to implementation of a perioperative patient safety program in Guatemala.Material and methodsWe conducted semi‐structured interviews with 16 staff pre‐intervention and a follow‐up focus group discussion 1 year later in the perioperative department at the Roosevelt Hospital in Guatemala. We performed qualitative thematic analysis to identify barriers and facilitators to the implementation process, with analysis guided by the Consolidated Framework for Implementation Research.ResultsWe found several dominant themes affecting implementation of a patient safety program. Implementation facilitators included strong prioritization of patient needs, program compatibility with existing workflow, and staff attributes. Barriers included a lack of knowledge about patient safety, limited resources, limited leadership engagement, and lack of formal implementation leaders. Several program modifications were made to enhance successful implementation iteratively during the implementation process.DiscussionOur analysis highlights several dominant themes which affect the implementation of a perioperative safety program in Guatemala. Understanding the barriers and facilitators to implementation during program deployment allows for program modification and improvement of the implementation process itself.