bluuebunny/crossref_metadata_2025_split huggingface dataset:
Pediatric Intestinal Failure: The Key Outcomes for the First 100 Patients Treated in a National Tertiary Referral Center During 1984–2017
Authors: Merras‐Salmio Laura , Mutanen Annika , Ylinen Elisa , Rintala Risto , Koivusalo Antti , Pakarinen Mikko P. 
Published: April 2018

AbstractBackground: Pediatric‐onset intestinal failure (IF) remains a severe illness with life‐threatening consequences. In this study, we analyzed a single center's outcomes of IF over 3 decades. Methods: All children with IF who required parenteral nutrition (PN) >2 months or small‐intestinal resection ≥50% managed since 1984 were included for retrospective outcome analyses. Results: In total, 100 patients with median PN duration of 1.2 (interquartile range, 0.4–3.5) years were identified. Causes of IF were short bowel syndrome (SBS; n = 78), primary intestinal motility disorders (n = 14), and congenital intestinopathies (n = 8). Patients with SBS had median 40 (25–60) cm of small bowel remaining. Overall, Kaplan‐Meier 5‐ and 10‐year weaning‐off estimates were 67% (95% CI, 57–77) and 73% (95% CI, 63–84), respectively. Weaning off PN was predicted by remaining bowel anatomy, multidisciplinary treatment era, and absence of immune deficiency. Catheter‐related bloodstream infections decreased from 1.4 to 0.6/1000 PN days (P = .0003) with systematic use of taurolidine locks. None had progressive liver disease. Thirty‐one percent of patients with SBS underwent autologous intestinal reconstructive surgery. Five patients received and 2 were listed for isolated intestinal transplantation. Eight patients died, and overall 15‐year survival rate estimate was 91% (95% CI, 85–98). Conclusions: Despite reassuring rates of survival and weaning off PN, long‐term PN failed in 14% of patients solely because of catheter complications in the recent era. Achievement of enteral autonomy in those with the shortest remaining small bowel and functional cause of IF remains challenging.
No Gut No Gain! Enteral Bile Acid Treatment Preserves Gut Growth but Not Parenteral Nutrition–Associated Liver Injury in a Novel Extensive Short Bowel Animal Model
Authors: Villalona Gustavo , Price Amber , Blomenkamp Keith , Manithody Chandrashekhara , Saxena Saurabh , Ratchford Thomas , Westrich Matthew , Kakarla Vindhya , Pochampally Shruthika , Phillips William , Heafner Nicole , Korremla Niraja , Greenspon Jose , Guzman Miguel A. , Kumar Jain Ajay 
Published: April 2018

AbstractBackgroundParenteral nutrition (PN) provides nutrition intravenously; however, this life‐saving therapy is associated with significant liver disease. Recent evidence indicates improvement in PN‐associated injury in animals with intact gut treated with enteral bile acid (BA), chenodeoxycholic acid (CDCA), and a gut farnesoid X receptor (FXR) agonist, which drives the gut–liver cross talk (GLCT). We hypothesized that similar improvement could be translated in animals with short bowel syndrome (SBS).MethodsUsing piglets, we developed a novel 90% gut‐resected SBS model. Fifteen SBS piglets receiving PN were given CDCA or control (vehicle control) for 2 weeks. Tissue and serum were analyzed posteuthanasia.ResultsCDCA increased gut FXR (quantitative polymerase chain reaction; P = .008), but not downstream FXR targets. No difference in gut fibroblast growth factor 19 (FGF19; P = .28) or hepatic FXR (P = .75), FGF19 (P = .86), FGFR4 (P = .53), or Cholesterol 7 α‐hydroxylase (P = .61) was noted. PN resulted in cholestasis; however, no improvement was noted with CDCA. Hepatic fibrosis or immunostaining for Ki67, CD3, or Cytokeratin 7 was not different with CDCA. PN resulted in gut atrophy. CDCA preserved (P = .04 vs control) gut mass and villous/crypt ratio. The median (interquartile range) for gut mass for control was 0.28 (0.17–0.34) and for CDCA was 0.33 (0.26–0.46).ConclusionsWe note that, unlike in animals with intact gut, in an SBS animal model there is inadequate CDCA‐induced activation of gut‐derived signaling to cause liver improvement. Thus, it appears that activation of GLCT is critically dependent on the presence of adequate gut. This is clinically relevant because it suggests that BA therapy may not be as effective for patients with SBS.
Synthesis, Characterization, and Applications of Some New Trimeric‐Type Cationic Surfactants
Authors: El‐Said Waleed A. , Moharram Ahmed S. , Hussein Essam M. , El‐Khawaga Ahmed M. 
Published: April 2018

AbstractIn recent years, trimeric surfactants have created excitement in the surfactant field because of their properties, which have been found to be better than monomeric or dimeric homologues. Only a limited number of trimeric surfactants have been synthesized and studied so far, probably owing to the difficulty in synthesis. In this article, we synthesized some novel star‐shaped trimeric cationic surfactants based on the alkylation of the 3 hydroxyl groups of the phloroglucinol nuclei as a core (i.e., spacer) with 3 dodecyl or 3 octyl groups (as tails) for the surfactant. The chemical structures were confirmed by nuclear magnetic resonance, Fourier transform infrared, mass spectrometry, and elemental analysis; also the critical micelle concentration was determined by electrical conductivity measurements. These surfactants were used in the synthesis of mesoporous silica nanoparticles by the sol–gel method. The silica particles shape and size were determined using field emission scanning electron microscopy and high‐resolution transmission electron microscopy images. Furthermore, the corrosion inhibitor capability of these surfactants was investigated by monitoring the corrosion rate of iron sheets in 0.5 M hydrochloric acid in the presence and in the absence of different surfactants at 45°C based on the weight loss method. We have used cetyltrimethylammonium bromide (CTAB) as a positive control, the obtained results showed a high inhibition efficiency at very low concentrations, and the prepared trimeric surfactants exhibited a higher anticorrosion efficiency than the CTAB surfactants.
Study of image quality using event‐related potentials and eye tracking measurement
Authors: Jia Lixiu , Tu Yan , Wang Lili , Zhong Xuefei , Wang Ying 
Published: April 2018

AbstractThe image quality was investigated using electroencephalography and eye‐tracking measurements simultaneously. Different image qualities were obtained by changing σ values in the Gaussian kernel function. The experimental results illustrated that the measurable event‐related potentials and eye‐tracking parameters varied with the change of the image quality. The more blurred the image, the higher and earlier the P300 (event‐related potentials at latency about 300–550 ms) was elicited. Meanwhile, the visual fixation duration decreased and the detection rate increased with the increase of σ. The blurred image quality could be evaluated based on the synthetic results of the reciprocal of P300 latency and the visual fixation duration. The result showed a good correlation with the subjective rating scores, which proved the feasibility of utilizing electroencephalography and eye‐tracking technology to evaluate the blurred image quality.
Mortality of Calanus helgolandicus: Sources, differences between the sexes and consumptive and nonconsumptive processes
Authors: Maud Jacqueline L. , Hirst Andrew G. , Atkinson Angus , Lindeque Penelope K. , McEvoy Andrea J. 
Published: April 2018

AbstractWhile losses from mortality are as important as gains from reproduction in zooplankton population dynamics, the former are more challenging to quantify. We used two approaches to provide complementary insights into the mortality of a biomass‐dominant copepod, Calanus helgolandicus, at Station L4 in the English Channel. Using a neutral‐red staining method, we found that dead carcasses represented a mean of 9% of the C. helgolandicus copepodites sampled. The resulting nonconsumptive mortality rates are the first that have been derived for C. helgolandicus; and estimates suggest a contribution of 0–54% (median of 4.4%) to the total mortality rate. Consumptive mortality (i.e., that due to removal by predation), dominated for most of the year and contributed a mean of 89% to total mortality. Nonconsumptive mortality increased during summer and winter, and was positively related to maximum wind speed during the preceding 72 h, indicating that extreme weather events may lead to increased mortality. Using the Vertical Life Table approach, mortality rates across the CV‐adult male stage pair were on average ∼ 2.5 times greater than those of CV‐adult females. Adult male consumptive mortality rates were ∼ 6 times greater than those for females; adult male nonconsumptive rates were twice those of females, suggesting that predation is of greater significance to male loss rates. Summer CV‐adult mortality rates were positively correlated to temperature, and to the abundance of predatory chaetognaths and siphonophores, suggesting that the gelatinous predator assemblage is the dominant agent for population control of late stage copepodites of C. helgolandicus at L4.
Depolymerization of Trityl End‐Capped Poly(Ethyl Glyoxylate): Potential Applications in Smart Packaging
Authors: Fan Bo , Salazar Rómulo , Gillies Elizabeth R. 
Published: April 2018

AbstractThe temperature‐dependent depolymerization of self‐immolative poly(ethyl glyoxylate) (PEtG) capped with triphenylmethyl (trityl) groups is studied and its potential application for smart packaging is explored. PEtGs with four different trityl end‐caps are prepared and found to undergo depolymerization to volatile products from the solid state at different rates depending on temperature and the electron‐donating substituents on the trityl aromatic rings. Through the incorporation of hydrophobic dyes including Nile red and IR‐780, the depolymerization is visualized as a color change of the dye as it changes from a dispersed to aggregated state. The ability of this platform to provide information on thermal history through an easily readable signal makes it promising in smart packaging applications for sensitive products such a food and other cargo that is susceptible to degradation.
Preliminary development of porous aluminum via powder metallurgy technique
Authors: Jamal N.A. , Maizatul O. , Anuar H. , Yusof F. , Nor Y. Ahmad , Khalid K. , Zakaria M.N. 
Published: April 2018

AbstractPorous aluminum has been extensively studied, particularly in the field in which lightweight and high stiffness properties are essential. In this study, a preliminary investigation is performed to determine the optimum sintering temperature to develop porous aluminium by a powder metallurgy technique, using polymethylmethacrylate as a space holder. The effects of the sintering temperatures on the physical characteristics, oxidation level, microstructure and sintered density of the porous specimen are systematically evaluated. Based on the results, an increase in the sintering temperature from 580 °C to 600 °C changes the colour of the porous aluminum body from a silver‐like colour to a gold‐like colour, with some of the specimens encountering severe cracking, spalling and even collapsing. As such, the oxygen content is significantly increased from 0.45 wt.% to 2.14 wt. %, suggesting the oxidation phenomenon. In line with this, an obvious appearance of particle boundaries with less macro‐pores formation is also observed. Additionally, the sintered density of the porous specimen is found to reduce from 1.305 g/cm3 to 0.908 g/cm3. Therefore, fabrication of the resultant porous aluminium at 580 °C is an ideal condition in this study, owing to the ideal combination of physical characteristics, microstructure, oxidation level and sintered density.
Exogenous fatty acids alter phospholipid composition, membrane permeability, capacity for biofilm formation, and antimicrobial peptide susceptibility in Klebsiella pneumoniae
Authors: Hobby Chelsea R. , Herndon Joshua L. , Morrow Colton A. , Peters Rachel E. , Symes Steven J. K. , Giles David K. 
Published: April 2018

AbstractKlebsiella pneumoniae represents a major threat to human health due to a combination of its nosocomial emergence and a propensity for acquiring antibiotic resistance. Dissemination of the bacteria from its native intestinal location creates severe, complicated infections that are particularly problematic in healthcare settings. Thus, there is an urgency for identifying novel treatment regimens as the incidence of highly antibiotic‐resistant bacteria rises. Recent findings have highlighted the ability of some Gram‐negative bacteria to utilize exogenous fatty acids in ways that modify membrane phospholipids and influence virulence phenotypes, such as biofilm formation and antibiotic resistance. This study explores the ability of K. pneumoniae to assimilate and respond to exogenous fatty acids. The combination of thin‐layer chromatography liquid chromatography‐mass spectrometry confirmed adoption of numerous exogenous polyunsaturated fatty acids (PUFAs) into the phospholipid species of K. pneumoniae. Membrane permeability was variably affected as determined by two dye uptake assays. Furthermore, the availability of many PUFAs lowered the MICs to the antimicrobial peptides polymyxin B and colistin. Biofilm formation was significantly affected depending upon the supplemented fatty acid.
Evaluation of smartphone‐based testing to generate exploratory outcome measures in a phase 1 Parkinson's disease clinical trial
Authors: Lipsmeier Florian , Taylor Kirsten I. , Kilchenmann Timothy , Wolf Detlef , Scotland Alf , Schjodt‐Eriksen Jens , Cheng Wei‐Yi , Fernandez‐Garcia Ignacio , Siebourg‐Polster Juliane , Jin Liping , Soto Jay , Verselis Lynne , Boess Frank , Koller Martin , Grundman Michael , Monsch Andreas U. , Postuma Ronald B. , Ghosh Anirvan , Kremer Thomas , Czech Christian , Gossens Christian , Lindemann Michael 
Published: April 2018

ABSTRACTBackground: Ubiquitous digital technologies such as smartphone sensors promise to fundamentally change biomedical research and treatment monitoring in neurological diseases such as PD, creating a new domain of digital biomarkers.Objectives: The present study assessed the feasibility, reliability, and validity of smartphone‐based digital biomarkers of PD in a clinical trial setting.Methods: During a 6‐month, phase 1b clinical trial with 44 Parkinson participants, and an independent, 45‐day study in 35 age‐matched healthy controls, participants completed six daily motor active tests (sustained phonation, rest tremor, postural tremor, finger‐tapping, balance, and gait), then carried the smartphone during the day (passive monitoring), enabling assessment of, for example, time spent walking and sit‐to‐stand transitions by gyroscopic and accelerometer data.Results: Adherence was acceptable: Patients completed active testing on average 3.5 of 7 times/week. Sensor‐based features showed moderate‐to‐excellent test‐retest reliability (average intraclass correlation coefficient = 0.84). All active and passive features significantly differentiated PD from controls with P < 0.005. All active test features except sustained phonation were significantly related to corresponding International Parkinson and Movement Disorder Society–Sponsored UPRDS clinical severity ratings. On passive monitoring, time spent walking had a significant (P = 0.005) relationship with average postural instability and gait disturbance scores. Of note, for all smartphone active and passive features except postural tremor, the monitoring procedure detected abnormalities even in those Parkinson participants scored as having no signs in the corresponding International Parkinson and Movement Disorder Society–Sponsored UPRDS items at the site visit.Conclusions: These findings demonstrate the feasibility of smartphone‐based digital biomarkers and indicate that smartphone‐sensor technologies provide reliable, valid, clinically meaningful, and highly sensitive phenotypic data in Parkinson's disease. © 2018 The Authors. Movement Disorders published by Wiley Periodicals, Inc. on behalf of International Parkinson and Movement Disorder Society.
Protein molecular modeling techniques investigating novel TAB2 variant R347X causing cardiomyopathy and congenital heart defects in multigenerational family
Authors: Caulfield Thomas R. , Richter John E. , Brown Emily E. , Mohammad Ahmed N. , Judge Daniel P. , Atwal Paldeep S. 
Published: April 2018

AbstractBackgroundHaploinsufficiency of TAB2 is known to cause congenital heart defects and cardiomyopathy due to its important roles in cardiovascular tissue, both during development and through adult life. We report a sibling pair displaying adult‐onset cardiomyopathy, hypermobility, and mild myopia. Our proband, a 39‐year‐old male, presents only with the above symptoms, while his 36‐year‐old sister was also notable for a ventricular septal defect in her infancy.MethodsWhole‐exome sequencing was utilized to identify the molecular basis of the phenotype found in two siblings. A molecular modeling technique that takes advantage of conformational sampling advances (Maxwell's demon molecular dynamics and Monte Carlo) were used to make a model of the mutant variant for comparative analytics to the wild‐type.ResultsExome sequencing revealed a novel, heterogeneous pathogenic variant in TAB2, c.1039 C>T (p.R347X), that was present in both individuals. This pathogenic variant removes just over half the residues from the TAB2 protein and severely impacts its functional ability, which we describe in detail.ConclusionsAnalysis of the proband's family showed a history of cardiomyopathy, but no congenital heart defects or connective tissue disease. We highlight the heterogeneity in phenotype of TAB2 pathogenic variants and confirm the pathogenicity of this new variant through neoteric protein modeling techniques.
Impact of miR‐140 Deficiency on Non‐Alcoholic Fatty Liver Disease
Authors: Wolfson Benjamin , Lo Pang‐Kuo , Yao Yuan , Li Linhao , Wang Hongbing , Zhou Qun 
Published: April 2018

ScopeWe have previously shown that loss of miR‐140 has a pro‐fibrotic effect in the mammary gland. This study aims to investigate whether miR‐140 loss and obesity act synergistically to promote non‐alcoholic fatty liver disease (NAFLD), and to identify the underlying mechanisms.Methods and ResultsLiver tissues were isolated from lean‐fat‐diet and high‐fat‐diet fed wild‐type and miR‐140 knockout mice. Using molecular staining and immunohistochemistry techniques, increased development of NAFLD and fibrotic indicators in miR‐140 knockout mice were identified. Utilizing an in vitro model system, miR‐140 was demonstrated to target TLR‐4, and miR‐140 overexpression was shown to be sufficient to inhibit palmitic acid signaling through the TLR‐4/NFκB pathway.ConclusionThese findings demonstrate that loss of miR‐140 results in increased expression of TLR‐4, sensitizing cells to palmitic acid signaling and in increased inflammatory activity through the TLR4/NFκB pathway. This signaling axis promotes NAFLD development in a high‐fat diet context and indicates the potential utility of miR‐140 rescue as a therapeutic strategy in NAFLD.
The Leiden Family Lab study on Social Anxiety Disorder: A multiplex, multigenerational family study on neurocognitive endophenotypes
Authors: Bas‐Hoogendam Janna Marie , Harrewijn Anita , Tissier Renaud L.M. , van der Molen Melle J.W. , van Steenbergen Henk , van Vliet Irene M. , Reichart Catrien G. , Houwing‐Duistermaat Jeanine J. , Slagboom P. Eline , van der Wee Nic J.A. , Westenberg P. Michiel 
Published: April 2018

AbstractObjectivesSocial anxiety disorder (SAD) is a serious and prevalent psychiatric condition, with a heritable component. However, little is known about the characteristics that are associated with the genetic component of SAD, the so‐called “endophenotypes”. These endophenotypes could advance our insight in the genetic susceptibility to SAD, as they are on the pathway from genotype to phenotype. The Leiden Family Lab study on Social Anxiety Disorder (LFLSAD) is the first multiplex, multigenerational study aimed to identify neurocognitive endophenotypes of social anxiety.MethodsThe LFLSAD is characterized by a multidisciplinary approach and encompasses a variety of measurements, including a clinical interview, functional and structural magnetic resonance imaging and an electroencephalography experiment. Participants are family members from 2 generations, from families genetically enriched for SAD.ResultsThe sample (n = 132 participants, from 9 families) was characterized by a high prevalence of SAD, in both generations (prevalence (sub)clinical SAD: 38.3%). Furthermore, (sub)clinical SAD was positively related to self‐reported social anxiety, fear of negative evaluation, trait anxiety, behavioral inhibition, negative affect, and the level of depressive symptoms.ConclusionsBy the multidimensional character of the measurements and thorough characterization of the sample, the LFLSAD offers unique opportunities to investigate candidate neurocognitive endophenotypes of SAD.
Therapeutic experience with hepatoblastoma associated with trisomy 18
Authors: Inoue Akiko , Suzuki Ryo , Urabe Keisuke , Kawamura Yutaro , Masuda Midori , Kishi Kanta , Takitani Kimitaka , Katayama Hiroshi , Tomiyama Hideki , Hayashi Michihiro , Nemoto Shintaro , Uchiyama Kazuhisa , Tamai Hiroshi 
Published: April 2018

AbstractTrisomy 18 is often fatal, but patients with this disease can now have longer survival due to proactive treatment intervention. However, hepatoblastomas may develop in these patients. In this study, we report four cases of hepatoblastoma associated with trisomy 18. All of the patients had congenital heart disease and three had undergone intracardiac surgical repair. Tumor growth was relatively slow in all cases, and there were no problems with chemotherapy tolerability and surgical resection. Three of the patients are currently disease‐free and the fourth is alive with remaining of the tumor. These cases suggest that combined chemotherapy and surgical resection may be an option to treat hepatoblastoma associated with trisomy 18 when cardiac pulmonary function is relatively stable.
Polyurethane/doxorubicin nanoparticles based on electrostatic interactions as pH‐sensitive drug delivery carriers
Authors: Huang Dengcheng , Zhou Yu , Xiang Yuan , Shu Meijie , Chen Hongxiang , Yang Bing , Liao Xinghua 
Published: April 2018

AbstractIn order to obtain a pH‐sensitive delivery carrier for doxorubicin (DOX), DOX‐loaded polyurethane (PU·DOX) nanoparticles were readily prepared in water by electrostatic interactions between amphiphilic polyurethane with carboxyl pendent groups (PU‐COOH) and doxorubicin hydrochloride (DOX·HCl). The structures of the products obtained were characterized by Fourier transform infrared spectroscopy, 1H NMR spectroscopy, gel permeation chromatography, UV–visible spectroscopy, dynamic light scattering and transmission electron microscopy. The average hydrodynamic size of the PU·DOX nanoparticles was around 182 nm with negative surface charge (−1.1 mV) and a spherical or rodlike shape. PU·DOX nanoparticles had a higher drug‐loading content of 14.1 wt%. The in vitro drug release properties of PU·DOX nanoparticles were investigated at pH 4.0, 5.0 and 7.4, respectively. PU·DOX nanoparticles exhibited a good pH‐sensitive drug release property, but there was almost no release of DOX from PU·DOX nanoparticles at pH 7.4. The in vitro cellular uptake assay and the Cell Counting Kit‐8 assay demonstrated that PU·DOX nanoparticles had a higher level of cellular internalization and higher inhibitory effects on the proliferation of human breast cancer (MCF‐7) cells than pure DOX. The enhancement of the inhibition effects resulted from increasing apoptosis‐inducing effects on MCF‐7 cells, which was related to the enhancement of Bax expression and the reduction of Bcl‐2 expression confirmed by terminal deoxynucleotidyl transferase mediated dUTP nick end labeling (TUNEL) assay, real‐time polymerase chain reaction (PCR) assay and western blot assay. © 2018 Society of Chemical Industry