bluuebunny/crossref_metadata_2025_split huggingface dataset:
Patterns of Arterial Disease in Takayasu Arteritis and Giant Cell Arteritis
Authors: Gribbons K. Bates , Ponte Cristina , Carette Simon , Craven Anthea , Cuthbertson David , Hoffman Gary S. , Khalidi Nader A. , Koening Curry L. , Langford Carol A. , Maksimowicz-McKinnon Kathleen , McAlear Carol A. , Monach Paul A. , Moreland Larry W. , Pagnoux Christian , Quinn Kaitlin A. , Robson Joanna C. , Seo Philip , Sreih Antoine G. , Suppiah Ravi , Warrington Kenneth J. , Ytterberg Steven R. , Luqmani Raashid , Watts Richard , Merkel Peter A. , Grayson Peter C. 
Published: August 2019

ObjectiveTo identify and validate, using computer‐driven methods, patterns of arterial disease in Takayasu arteritis (TAK) and giant cell arteritis (GCA).MethodsPatients with TAK or GCA were studied from the Diagnostic and Classification Criteria for Vasculitis (DCVAS) cohort and a combined North American cohort. Case inclusion required evidence of large‐vessel involvement, defined as stenosis, occlusion, or aneurysm by angiography/ultrasonography, or increased18F‐fluorodeoxyglucose (FDG) uptake by positron emission tomography (PET) in at least 1 of 11 specified arterial territories. K‐means cluster analysis identified groups of patients based on the pattern of arterial involvement. Cluster groups were identified in the DCVAS cohort and independently validated in the North American cohort.ResultsA total of 1,068 patients were included (DCVAS cohort: TAK = 461, GCA = 217; North American cohort: TAK = 225, GCA = 165). Six distinct clusters of patients were identified in DCVAS and validated in the North American cohort. Patients with TAK were more likely to have disease in the abdominal vasculature, bilateral disease of the subclavian and carotid arteries, or focal disease limited to the left subclavian artery than GCA (P< 0.01). Patients with GCA were more likely to have diffuse disease, involvement of bilateral axillary/subclavian arteries, or minimal disease without a definable pattern than TAK (P< 0.01). Patients with TAK were more likely to have damage by angiography, and patients with GCA were more likely to have arterial FDG uptake by PET without associated vascular damage.ConclusionArterial patterns of disease highlight both shared and divergent vascular patterns between TAK and GCA and should be incorporated into classification criteria for large‐vessel vasculitis.
Clinical Presentation, Management, and Long‐Term Outcome of Pars Planitis, Panuveitis, and Vogt‐Koyanagi‐Harada Disease in Children and Adolescents
Authors: Reiff Andreas 
Published: August 2019

ObjectiveChronic uveitis is a common manifestation of pediatric rheumatologic conditions and may result in irreversible blindness and long‐term disability. While chronic anterior uveitis is the most commonly encountered ocular manifestation of rheumatic disease, little is known about the clinical presentation, management, and long‐term outcome of more complex eye conditions such as pars planitis (PP), panuveitis (PU), and Vogt‐Koyanagi‐Harada disease (VKH). The present study was undertaken to comprehensively assess the long‐term safety and efficacy of disease‐modifying antirheumatic drugs (DMARDs) and biologics for the treatment of pediatric and adolescent patients with PP, PU, and VKH.MethodsWe retrospectively reviewed a cohort of 75 children and adolescents with idiopathic PP (n = 50), PU (n = 12), and VKH (n = 14) followed by the Pediatric Rheumatology Core at Children’s Hospital Los Angeles and evaluated referral patterns, clinical presentation, treatment response, and long‐term clinical outcome.ResultsPatients were followed for an average of 52 months. Their mean age at disease onset was 10 years. Bilateral eye involvement was seen in 87% of the patients. At first presentation to an ophthalmologist, glaucoma was noted in 21% of patients and vision loss (<20/40) in 87% of patients, while legal blindness (≤20/200 in the better‐seeing eye) was diagnosed in 18 of 75 (24%) of patients (PP 22%, PU 36%, and VKH 21%). The average referral time to a pediatric rheumatologist was 13 months (range 1–96 months). Topical steroids were used in all patients, but 98% of patients required additional DMARDs, and 73% required therapy with biologics. After a mean of 52 months, 35% of patients across all disease groups had significant vision loss or were blind, and only 28% were in clinical remission without medications. The worst outcome was observed in children with PU. Regression analysis, young age at onset, delayed referral to a pediatric rheumatologist, and chronic disease were strong predictors for the risk of long‐term blindness.ConclusionPP, PU, and VKH involve a high risk of permanent vision loss and should be managed by a skilled rheumatologist as early and as aggressively as possible.
Real‐Time Imaging of Ammonia Release from Single Live Cells via Liquid Crystal Droplets Immobilized on the Cell Membrane
Authors: Khan Mashooq , Li Weiwei , Mao Sifeng , Shah Syed Niaz Ali , Lin Jin‐Ming 
Published: August 2019

AbstractTumor cells exhibit prominent metabolic alterations through which they acclimatize to their stressful microenvironment. These cells have a high rate of glutaminolysis and release ammonia (NH3) as a byproduct, which may function as a diffusible signal among cancer cells and can reveal cellular heterogeneity. E7, a nematic liquid crystal (LC), is doped with 4‐pentyl‐4′‐biphenyl carboxylic acid (PBA) and encapsulated in polymeric microcapsules (P‐E7PBA), which are then immobilized on cells in a microfluidic channel. Normal human umbilical vein endothelial cells (HUVECs) and myeloma, human primary glioblastoma (U87), human colon carcinoma (Caco‐2), and human breast adenocarcinoma (MCF‐7) cells are investigated for the release of NH3. The P‐E7PBA is able to visualize NH3 release from the cell via a radial‐to‐bipolar (R‐B) orientation change, observed through a polarized optical microscope. The various cell lines significantly differ in their response time required for an R‐B change. The mean response times for Caco‐2, U87, and MCF‐7 cells are 277, 155, and 121 s, respectively. NH3 release from a single cell captured in a microwell flow chip shows a similar R‐B change. The P‐E7PBA droplets technology could be applied to other multiple targets by functionalizing LCs with different probes.
Physical activity and the association between the FTO rs9939609 polymorphism and obesity in Portuguese children aged 3 to 11 years
Authors: Manco Licínio , Pinho Simão , Albuquerque David , Machado‐Rodrigues Aristides M. , Padez Cristina 
Published: August 2019

AbstractObjectivesTo investigate whether objectively measured physical activity (PA) modulates the association between the FTO rs9939609 polymorphism and obesity variables in a sample of Portuguese children.MethodsA total of 440 children (213 girls and 227 boys) aged 3 to 11 years were observed. Genotyping was performed using TaqMan assay. Body mass index (BMI), BMI Z scores, waist circumference (WC), and waist‐to‐height ratio (WHtR) were calculated. PA was estimated in 399 children by accelerometry.ResultsLinear regression, in the additive model, showed that the rs9939609 minor A‐allele significantly associated with BMI (P = .029), BMI Z score (P = .017), WC (P = .016), and WHtR (P = .019). Logistic regression, in the additive model, showed a marginally significant association between the A‐allele and overweight/obesity (odds ratio [OR]: 1.372; P = .049). When stratified by sex, rs9939609 showed marginal or significant associations with BMI (P = .08), BMI Z score (P = .07), WC (P = .005), WHtR (P = .02), and overweight/obesity (OR: 1.529; P = .064) in girls but not in boys (P > .05). Significant interactions were not found between the FTO polymorphism and PA (inactive vs active groups of children) for BMI (P = .461), BMI Z score (P = .387), WC (P = .757), or WHtR (P = .621).ConclusionsFindings of the present study highlight the association between FTO rs9939609 and obesity or body fat indices in girls but not in boys. PA was not found to mediate the impact of FTO genetic variation on risk of obesity.
Associated anomalies in cases with agenesis of the corpus callosum
Authors: Stoll Claude , Dott Beatrice , Roth Marie‐Paule 
Published: August 2019

AbstractAgenesis of corpus callosum (ACC) is an uncommon congenital anomaly, its etiology is unclear and its pathogenesis is controversial. Cases with ACC often have other non‐ACC associated congenital anomalies. The purpose of this study was to assess the prevalence and the types of these associated anomalies in a defined population. The associated anomalies in cases with ACC were collected in all live births, stillbirths, and terminations of pregnancy during 29 years in 387,067 consecutive births in the area covered by our population‐based registry of congenital malformations. Of the 99 cases with ACC, representing a prevalence of 2.56 per 10,000, 73 (73.7%) had associated anomalies. There were 16 (16.2%) cases with chromosomal abnormalities, and 13 (13.2%) nonchromosomal recognized dysmorphic conditions including syndromes two each: Aicardi, Dandy–Walker, and fetal alcoholism. Forty‐four (44.4%) of the cases had nonsyndromic multiple congenital anomalies (MCA). Anomalies in the musculoskeletal, the urogenital, the central nervous, the cardiovascular, and the digestive systems were the most common other anomalies in the cases with MCA. The anomalies associated with ACC could be classified into a recognizable malformation syndrome in 29 out of the 73 cases (39.7%) with associated anomalies. This study included special strengths: it is population‐based, each affected child was examined by a geneticist, all elective terminations were ascertained, and the surveillance for anomalies was continued until 2 years of age. In conclusion the overall prevalence of associated anomalies, three of four cases, emphasizes the need for a screening for other anomalies in cases with ACC.
Novel homozygous ENPP1 mutation causes generalized arterial calcifications of infancy, thrombocytopenia, and cardiovascular and central nervous system syndrome
Authors: Staretz‐Chacham Orna , Shukrun Rachel , Barel Ortal , Pode‐Shakked Ben , Pleniceanu Oren , Anikster Yair , Shalva Nechama , Ferreira Carlos R. , Ben‐Haim Kadosh Admit , Richardson Justin , Mane Shrikant M. , Hildebrandt Friedhelm , Vivante Asaf 
Published: August 2019

AbstractGeneralized arterial calcifications of infancy (GACI) is caused by mutations in ENPP1. Other ENPP1‐related phenotypes include pseudoxanthoma elasticum, hypophosphatemic rickets, and Cole disease. We studied four children from two Bedouin consanguineous families who presented with severe clinical phenotype including thrombocytopenia, hypoglycemia, hepatic, and neurologic manifestations. Initial working diagnosis included congenital infection; however, patients remained without a definitive diagnosis despite extensive workup. Consequently, we investigated a potential genetic etiology. Whole exome sequencing (WES) was performed for affected children and their parents. Following the identification of a novel mutation in the ENPP1 gene, we characterized this novel multisystemic presentation and revised relevant imaging studies. Using WES, we identified a novel homozygous mutation (c.556G > C; p.Gly186Arg) in ENPP1 which affects a highly conserved protein domain (somatomedin B2). ENPP1‐associated genetic diseases exhibit phenotypic heterogeneity depending on mutation type and location. Follow‐up clinical characterization of these families allowed us to revise and detect new features of systemic calcifications, which established the diagnosis of GACI, expanding the phenotypic spectrum associated with ENPP1 mutations. Our findings demonstrate that this novel ENPP1 founder mutation can cause a fatal multisystemic phenotype, mimicking severe congenital infection. This also represents the first reported mutation affecting the SMB2 domain, associated with GACI.
Maternal environmental risk factors and the development of internalizing and externalizing problems in childhood: The complex role of genetic factors
Authors: Ensink Judith B. M. , de Moor Marleen H. M. , Zafarmand Mohammad Hadi , de Laat Sanne , Uitterlinden André , Vrijkotte Tanja G. M. , Lindauer Ramón , Middeldorp Christel M. 
Published: August 2019

AbstractThe development of problem behavior in children is associated with exposure to environmental factors, including the maternal environment. Both are influenced by genetic factors, which may also be correlated, that is, environmental risk and problem behavior in children might be influenced by partly the same genetic factors. In addition, environmental and genetic factors could interact with each other increasing the risk of problem behavior in children. To date, limited research investigated these mechanisms in a genome‐wide approach. Therefore, the goal of this study was to investigate the association between genetic risk for psychiatric and related traits, as indicated by polygenetic risk scores (PRSs), exposure to previously identified maternal risk factors, and problem behavior in a sample of 1,154 children from the Amsterdam Born Children and their Development study at ages 5–6 and 11–12 years old. The PRSs were derived from genome‐wide association studies (GWASs) on schizophrenia, major depressive disorder, neuroticism, and wellbeing. Regression analysis showed that the PRSs were associated with exposure to multiple environmental risk factors, suggesting passive gene–environment correlation. In addition, the PRS based on the schizophrenia GWAS was associated with externalizing behavior problems in children at age 5–6. We did not find any association with problem behavior for the other PRSs. Our results indicate that genetic predispositions for psychiatric disorders and wellbeing are associated with early environmental risk factors for children's problem behavior.
P‐LME polymer nanocapsules stimulate naïve macrophages and protect them from oxidative damage during controlled drug release
Authors: Yamala Anil K. , Nadella Vinod , Mastai Yitzhak , Prakash Hridayesh , Paik Pradip 
Published: August 2019

ABSTRACTUsing polymer‐based nanocapsules as novel drug delivery systems have gained significant importance. In this work, we present poly‐(N‐Acryloyl L‐Leucine methyl ester [pLME]) nanocapsules with hollow core (pLME) of size approximately ~160 nm (dia.) synthesized for the first time through the miniemulsion approach. Our study reveals that pLME are biocompatible and immune stimulatory in nature on macrophages, which are decisive for host immunity. These nanocapsules are effective for encapsulating and sustainable release of drug (SNP; nitric oxide donor) at a high extent ~1.04 μM mg‐. pLME also demonstrated their potential of stimulating naïve macrophages toward the M1 phenotype for extended period of time indicating their adjuvant activity on immune system. Our data also demonstrated their antioxidant potential toward H2O2‐induced oxidative stress in macrophages. These results suggest pLME as an effective drug/vaccine delivery material with immune adjuvant and cytoprotective efficacy warranting its pharmacological application. © 2019 Wiley Periodicals, Inc. J. Appl. Polym. Sci. 2020, 137, 48363.
Retracted: Alpinumisoflavone Exhibits Anticancer Activities in Glioblastoma Multiforme by Suppressing Glycolysis
Authors: Zhao Xingzhi , Zhang Ting , Jiang Kewei , Gao Heng 
Published: August 2019

ABSTRACT“Alpinumisoflavone exhibits anticancer activities in glioblastoma multiforme by suppressing glycolysis” by Xingzhi ZHAO, Ting ZHANG, Kewei JIANG, and Henggao GAO. The above article, published in The Anatomical Record on Early View on Wiley Online Library on 24/Aug/2019 (DOI: 10.1002/ar.24242) is retracted by agreement among the authors and the Editor‐in‐Chief, Dr. Kurt H. Albertine, with notification to the American Association for Anatomy and Wiley Periodicals LLC. The decision is the result of an investigation that revealed issues of scientific integrity in the data that compromise the findings of the paper
An All‐Inclusive and Straightway Laboratory Activity to Solve the Three‐Dimensional Crystal Structure of a Protein
Authors: Klinke Sebastián , Rinaldi Jimena , Goldbaum Fernando A. , Suarez Sebastián , Otero Lisandro H. 
Published: August 2019

AbstractX‐ray crystallography provides structural information of molecules at the atomic level, being a central technique at the forefront of science and technology. However, crystallography teaching is not usually implemented in biochemistry lab classes due to its complex execution by nonexpert users. Here, we report the basic step‐by‐step workflow performed by crystallographers in order to solve the three‐dimensional structure of a protein. All these activities were executed in a course for Latin‐American graduate students with no previous knowledge on X‐ray crystallography entitled “Crystallography in Structural Biology: why do we need a protein crystal, and how do we get it?.” We would like to share our experience with the educational research community, with the main purpose being to enrich teaching in biochemistry and structural molecular biology by performing a series of interesting laboratory and computer experiments. © 2019 International Union of Biochemistry and Molecular Biology, 47(6):700–707, 2019.
Determination of phenamacril residues in flour and rice based on Z‐Sep+ using ultra‐high‐performance liquid chromatography–tandem mass spectrometry
Authors: Sun MingNa , Yu Lu , Tong Zhou , Dong Xu , Chu Yue , Wang Mei , Gao TongChun , Duan JinSheng 
Published: August 2019

AbstractPhenamacril is a new broad‐spectrum fungicide that is commonly used for the control of fungal diseases in wheat and rice. In this study, ultra‐high‐performance liquid chromatography–tandem mass spectrometry was used to establish a method for analyzing the residual phenamacril in flour and rice based on the improved QuEChERS (quick, easy, cheap, effective, rugged and safe) method using Z‐Sep+ as the adsorbent in the pre‐treatment process. The average recovery of phenamacril in flour and rice was 82.2–96.0%, the relative standard deviation was 2.1–5.6% and the limit of quantification was 0.5 μg/kg. The accuracy and sensitivity of this method meet the requirements for residue analysis. The method was applied to commercially available flour and rice samples, and the detected concentrations of phenamacril were 0.005–0.033 mg/kg. This method provides technical support for the safety evaluation of phenamacril.
Differences in perceptual masking between humans and rats
Authors: Dell Katrina L. , Arabzadeh Ehsan , Price Nicholas S. C. 
Published: August 2019

AbstractIntroductionThe perception of a target stimulus can be impaired by a subsequent mask stimulus, even if they do not overlap temporally or spatially. This “backward masking” is commonly used to modulate a subject's awareness of a target and to characterize the temporal dynamics of vision. Masking is most apparent with brief, low‐contrast targets, making detection difficult even in the absence of a mask. Although necessary to investigate the underlying neural mechanisms, evaluating masking phenomena in animal models is particularly challenging, as the task structure and critical stimulus features to be attended must be learned incrementally through rewards and feedback. Despite the increasing popularity of rodents in vision research, it is unclear if they are susceptible to masking illusions.MethodsWe characterized how spatially surrounding masks affected the detection of sine‐wave grating targets.ResultsIn humans (n = 5) and rats (n = 7), target detection improved with contrast and was reduced by the presence of a mask. After controlling for biases to respond induced by the presence of the mask, a clear reduction in detectability was caused by masks. This reduction was evident when data were averaged across all animals, but was only individually significant in three animals.ConclusionsWhile perceptual masking occurs in rats, it may be difficult to observe consistently in individual animals because the complexity of the requisite task pushes the limits of their behavioral capabilities. We suggest methods to ensure that masking, and similarly subtle effects, can be reliably characterized in future experiments.
Decompression of the thoracic duct: A novel transcatheter approach
Authors: Smith Christopher L. , Hoffman Timothy M. , Dori Yoav , Rome Jonathan J. 
Published: August 2019

AbstractIn patients with total cavopulmonary connections, elevated central venous pressures (CVP) have detrimental effects on the lymphatic system causing an imbalance in fluid production and drainage of the interstitium. This combination may result in life‐threatening lymphatic complications including plastic bronchitis (PB), protein losing enteropathy (PLE), chylothorax, and ascites. While embolization of the abnormal lymphatics has greatly improved outcomes from these complications, alternative treatment strategies have been proposed that would result in improved lymphatic drainage while leaving the lymphatic system intact. We report two novel transcatheter approaches for thoracic duct (TD) decompression in two patients who developed PLE after completion of the Fontan procedure as part of staged palliation for congenital heart disease. In addition, one patient had severe concurrent PB. In both patients, a connection was created between a left superior vena cava (LSVC) to the left atrium allowing for a nonsurgical method to decompress the TD. This procedure resulted in significant clinical and laboratory improvement of both patients' PLE and other symptoms of lymphatic dysfunction.
Photocatalytic degradation of dimethyl sulphoxide by CdS/TiO2 core/shell catalyst: A novel measurement method
Authors: Alizadeh Sajad , Fallah Narges , Nikazar Manouchehr 
Published: August 2019

AbstractIn the present research, the photocatalytic degradation of dimethyl sulphoxide (DMSO) by means of CdS/TiO2 core/shell nanocomposite was investigated and modelled for the direct measurement of degradation rate. Instead of common measurement methods, liquid freezing point measurement was used in order to determine the rate of DMSO decomposition. To model the photocatalytic behaviour, two empirical‐statistical equations based on photocatalytic retention time, amount of catalyst and pollutant were introduced. The effect of parameters such as retention time, amount of catalyst and pollutant were studied by statistical methods. The design of experiments, acquisition and optimization of statistical models was performed by response surface methodology (RSM) through central composite design (CCD). The rates of disappearance fitted the Langmuir‐Hinshelwood kinetics model and the parameter k was determined to be up to 0.0105 per minute for a low concentration and 0.0073 per minute for a high concentration of DMSO. In addition, more than 85% degradation of 1% DMSO was attained by 8% catalyst in 150 minutes. Finally, the accuracy and consistency of the statistical models was verified by the HPLC method and ~ ± 2.7% difference was observed. The other results indicate that the CdS/TiO2 nano photocatalyst can efficiently remove dimethyl sulphoxide from wastewater under visible light irradiation.