bluuebunny/crossref_metadata_2025_split huggingface dataset:
Mac-1 triggers neutrophil DNA extracellular trap formation to Aspergillus fumigatus independently of PAD4 histone citrullination
Authors: Silva Juliana C. , Rodrigues Najara C. , Thompson-Souza Glaucia A. , Muniz Valdirene de S. , Neves Josiane S. , Figueiredo Rodrigo T. 
Published: September 2019

Abstract
Aspergillus fumigatus (A. fumigatus) is an environmental fungus and a human pathogen. Neutrophils are critical effector cells during the fungal infections, and neutropenia is a risk factor for the development of pulmonary aspergillosis. Neutrophil extracellular traps (NETs) are released by neutrophils in response to A. fumigatus and inhibit the conidial germination. In this work, we observed that the receptors TLR2, TLR4, and Dectin-1 were dispensable for the A. fumigatus induced NET release. In contrast CD11b/CD18 was critical for the NET release in response to A. fumigatus conidia, and this required the CD11b I-domain-mediated recognition, whereas the blockade of the CD11b lectin domain did not affect the A. fumigatus induced NET release. A. fumigatus induced NET release relied on the activity of spleen tyrosine kinase (Syk), Src family kinase(s), and class IA PI3 kinase δ. Although A. fumigatus promoted histone citrullination, this process was dispensable for the NET release in response to A. fumigatus conidia. The A. fumigatus induced NET release required the reactive oxygen species generation by the NOX2 complex, in a downstream pathway requiring CD11b/CD18, Src kinase family activity, Syk and PI3K class IA δ. Our findings thus reveal the signaling pathways involved in the formation of NETs in response to A. fumigatus.
High diagnostic yield of direct Sanger sequencing in the diagnosis of neuronal ceroid lipofuscinoses
Authors: Jilani Abdulhakim , Matviychuk Diana , Blaser Susan , Dyack Sarah , Mathieu Jean , Prasad Asuri N. , Prasad Chitra , Kyriakopoulou Lianna , Mercimek‐Andrews Saadet 
Published: September 2019

AbstractBackgroundNeuronal ceroid lipofuscinoses are neurodegenerative disorders. To investigate the diagnostic yield of direct Sanger sequencing of the CLN genes, we reviewed Molecular Genetics Laboratory Database for molecular genetic test results of the CLN genes from a single clinical molecular diagnostic laboratory.MethodsWe reviewed electronic patient charts. We used consent forms and Research Electronic Data Capture questionnaires for the patients from outside of our Institution. We reclassified all variants in the CLN genes.ResultsSix hundred and ninety three individuals underwent the direct Sanger sequencing of the CLN genes for the diagnosis of neuronal ceroid lipofuscinoses. There were 343 symptomatic patients and 350 family members. Ninety‐one symptomatic patients had molecular genetic diagnosis of neuronal ceroid lipofuscinoses including CLN1 (PPT1) (n = 10), CLN2 (TPP1) (n = 33), CLN3 (n = 17), CLN5 (n = 7), CLN6 (n = 10), CLN7 (MFSD8) (n = 10), and CLN8 (n = 4) diseases. The diagnostic yield of direct Sanger sequencing of CLN genes was 27% in symptomatic patients. We report detailed clinical and investigation results of 33 NCL patients. Juvenile onset CLN1 (PPT1) and adult onset CLN6 diseases were nonclassical phenotypes.ConclusionIn our study, the diagnostic yield of direct Sanger sequencing was close to diagnostic yield of whole exome sequencing. Developmental regression, cognitive decline, visual impairment and cerebral and/or cerebellar atrophy in brain MRI are significant clinical and neuroimaging denominators to include NCL in the differential diagnosis.
Paediatric image‐guided radiation therapy: determining and evaluating appropriate kilovoltage planar exposure factors for the Varian on‐board imager
Authors: Ryan John , Willis David 
Published: September 2019

AbstractIntroductionKilovoltage (kV) orthogonal imaging is commonly used for image‐guided radiation therapy (IGRT) in paediatrics. Paediatrics have an increased sensitivity to radiation. Exposure factors need to be optimised so that imaging dose is kept as low as reasonably achievable (ALARA).MethodsA table of low‐dose IGRT radiographic exposure factors for paediatric IGRT was determined through a phantom study. Four anatomical sites, head and neck, thorax, abdomen and pelvis, were investigated. The table was evaluated against standard manufacturer pre‐sets. Dose was evaluated in terms of system‐reported entrance surface air kerma (ESAK). Qualified participants volunteered to perform offline image matching in a further phantom study, recording misalignments detected and providing subjective assessments of image quality using an electronic survey tool. A statistical comparison of matching accuracy was conducted.ResultsTwelve radiation therapists or radiation oncologists completed the image matching task and survey. The low‐dose exposure table reduced imaging dose by 20–94% compared to manufacturer pre‐sets. No significant difference was observed in the accuracy of image matching (head and neck P = 0.82, thorax P = 0.15, abdomen P = 0.33, pelvis P = 0.59). Participant image exposure preference was largely equivocal.ConclusionsOptimising radiographic exposures in paediatric IGRT is feasible, logical and therefore reasonably achievable. Implementation of the low‐dose exposure table presented in this study should be considered by paediatric radiotherapy departments wishing to image gently without compromising the potential to detect set up errors. Further study using a contrast detail phantom and contrast to noise image analysis software is recommended.
Predictors of recurrence after pulmonary vein isolation in patients with normal left atrial diameter
Authors: Yano Masamichi , Egami Yasuyuki , Yanagawa Kyosuke , Matsuhiro Yutaka , Nakamura Hitoshi , Yasumoto Koji , Okamoto Naotaka , Tanaka Akihiro , Matsunaga‐Lee Yasuharu , Nakamura Daisuke , Yamato Masaki , Shutta Ryu , Nishino Masami , Tanouchi Jun 
Published: September 2019

AbstractBackgroundEnlarged left atrium (LA) is an established predictor of recurrence of atrial fibrillation (AF) after pulmonary vein isolation (PVI), but occasionally recurrences of AF/atrial tachycardia (AT) are experienced in patients with normal left atrial diameter. Therefore, the predictors of AF recurrence and AF triggers were evaluated in patients with normal LA.MethodsWe enrolled 168 patients with normal LA (<40 mm) who underwent PVI. Various predictors were compared, including age, gender, coronary risk factors, brain natriuretic peptide (BNP), medications, echocardiographic parameters, and procedure parameters, between recurrence and nonrecurrence groups.ResultsThe recurrence group consisted of 50 patients (29.8%). A univariate analysis demonstrated that the ratio of females, high BNP levels, severe tricuspid valve regurgitation (TR), and relapses of AF/AT during catheter ablation (CA) were significantly higher in the recurrence group. Multivariate analyses showed that a high BNP, severe TR, and AF/AT relapses during CA were independent factors associated with AF recurrence. During the second CA sessions, nonpulmonary vein (PV) triggers were therapeutic targets in 18 patients (46.2%), which was higher than that previously reported.ConclusionA high BNP, severe TR and AF/AT relapses during CA may be correlated with AF recurrence after PVI in the patients with normal LA.
In‐Vivo Efficacy of Recombinant Human Hyaluronidase (rHuPH20) Injection for Accelerated Healing of Murine Retrocalcaneal Bursitis and Tendinopathy
Authors: Rezvani Sabah N. , Chen Jinnan , Li Jun , Midura Ron , Cali Valbona , Sandy John D. , Plaas Anna , Wang Vincent M. 
Published: September 2019

ABSTRACTThe deposition of aggrecan/hyaluronan (HA)‐rich matrix within the tendon body and surrounding peritenon impede tendon healing and result in compromised biomechanical properties. Hence, the development of novel strategies to achieve targeted removal of the aggrecan‐HA pericellular matrix may be effective in treating tendinopathy. The current study examined the therapeutic potential of a recombinant human hyaluronidase, rHuPH20 (FDA approved for reducing HA accumulation in tumors) for treating murine Achilles tendinopathy. The 12‐week‐old C57Bl/6 male mice were injected with two doses of rHuTGF‐β1 into the retrocalcaneal bursa (RCB) to induce a combined bursitis and tendinopathy. Twenty‐four hours following induction of injury, treatment groups were administered rHuPH20 Hyaluronidase (rHuPH20; Halozyme Therapeutics) into the RCB. At either 6 h (acute), 9 days, or 25 days following hyaluronidase treatment, Achilles tendons were analyzed for gene expression, histology and immunohistochemistry, fluorophore‐assisted carbohydrate electrophoresis, and biomechanical properties. The rHuPH20 treatment was effective, particularly at the acute and 9‐day time points, in (a) removing HA deposits from the Achilles tendon and surrounding tissues, (b) improving biomechanical properties of the healing tendon, and (c) eliciting targeted increases in expression of specific cell fate, extracellular matrix metabolism, and inflammatory genes. The potential of rHuPH20 to effectively clear the pro‐inflammatory, HA‐rich matrix within the RCB and tendon strongly supports the future refinement of injectable glycosidase preparations as potential treatments to protect or regenerate tendon tissue by reducing inflammation and scarring in the presence of bursitis or other inducers of damage such as mechanical overuse. © 2019 Orthopaedic Research Society. Published by Wiley Periodicals, Inc. J Orthop Res 38:59–69, 2020
Global travertine deposition modulated by oscillations in climate
Authors: Ricketts Jason W. , Ma Lin , Wagler Amy E. , Garcia Victor H. 
Published: September 2019

ABSTRACTTravertine deposits are important records of past fluid flow in the Earth's crust, and document fluid migration through both tectonic activity and changes in climate. While many studies hint at possible relationships between travertine formation and global climate, none have investigated these connections on a global scale. Here we compile 1649 published travertine ages from six continents to test the hypothesis that global and/or regional changes in climate regulate travertine deposition. Peaks in bedded travertine ages occur with main frequencies that correspond to 100‐kyr changes in global climate, where most peaks occur during glacial terminations or interglacial periods, including a large peak that coincides with the Early Holocene climatic optimum. Time–series analysis also suggests a possible connection with 41‐kyr obliquity cycles. At regional scales, many peaks also correspond with local times of high precipitation or wet conditions. This can be attributed to higher groundwater recharge rates, providing the necessary water to form travertine. Many bedded travertine‐depositing systems may therefore be water‐limiting and sufficient CO2 may be present even during times of no travertine deposition. Exceptions to this conclusion are banded vein travertine deposits, which typically form during times of dry climate when water tables are low. Copyright © 2019 John Wiley & Sons, Ltd.
Intraductal Salivary Gland Infusion With Botulinum Toxin
Authors: Schwalje Adam T. , Hoffman Henry T. 
Published: September 2019

BackgroundAdministration of botulinum toxin through intraductal salivary infusion may decrease the risks of percutaneous needle injection and improve delivery to permeate the entire gland parenchyma.MethodsThe safety of intraductal salivary gland infusion was tested with prospective evaluation of two patients using interviews, clinical examination, and pressure measurement during infusion. Retrospective chart review of two subsequently treated patients assessed treatment of a parotid‐cutaneous fistula and sialorrhea.ResultsNo complications were identified in the safety study. Pressure changes during infusion supported the concept of botulinum neurotoxin delivery to permeate the gland. Patient‐assessed success was subjectively reported as a reduction in the parotid‐cutaneous output “by 95%” and the sialorrhea “by 90%” at 2‐week follow‐up.ConclusionsThe intraductal route of botulinum toxin delivery to salivary glands was without complication and was effective in two patients treated therapeutically. Pressure measurements during infusion may be helpful to direct treatment.Level of Evidence4
ENAM mutations and digenic inheritance
Authors: Zhang Hong , Hu Yuanyuan , Seymen Figen , Koruyucu Mine , Kasimoglu Yelda , Wang Shih‐Kai , Wright John Timothy , Havel Michael W. , Zhang Chuhua , Kim Jung‐Wook , Simmer James P. , Hu Jan C.‐C. 
Published: September 2019

AbstractBackgroundENAM mutations cause autosomal dominant or recessive amelogenesis imperfecta (AI) and show a dose effect: enamel malformations are more severe or only penetrant when both ENAM alleles are defective.MethodsWhole exome sequences of recruited AI probands were initially screened for mutations in known AI candidate genes. Sanger sequencing was used to confirm sequence variations and their segregation with the disease phenotype. The co‐occurrence of ENAM and LAMA3 mutations in one family raised the possibility of digenic inheritance. Enamel formed in Enam+/+Ambn+/+, Enam+/−, Ambn+/−, and Enam+/−Ambn+/− mice was characterized by dissection and backscattered scanning electron microscopy (bSEM).ResultsENAM mutations segregating with AI in five families were identified. Two novel ENAM frameshift mutations were identified. A single‐nucleotide duplication (c.395dupA/p.Pro133Alafs*13) replaced amino acids 133‐1142 with a 12 amino acid (ATTKAAFEAAIT*) sequence, and a single‐nucleotide deletion (c.2763delT/p.Asp921Glufs*32) replaced amino acids 921‐1142 with 31 amino acids (ESSPQQASYQAKETAQRRGKAKTLLEMMCPR*). Three families were heterozygous for a previously reported single‐nucleotide ENAM deletion (c.588+1delG/p.Asn197Ilefs*81). One of these families also harbored a heterozygous LAMA3 mutation (c.1559G>A/p.Cys520Tyr) that cosegregated with both the AI phenotype and the ENAM mutation. In mice, Ambn+/− maxillary incisors were normal. Ambn+/− molars were also normal, except for minor surface roughness. Ambn+/− mandibular incisors were sometimes chalky and showed minor chipping. Enam+/− incisor enamel was thinner than normal with ectopic mineral deposited laterally. Enam+/− molars were sometimes chalky and rough surfaced. Enam+/−Ambn+/− enamel was thin and rough, in part due to ectopic mineralization, but also underwent accelerated attrition.ConclusionNovel ENAM mutations causing AI were identified, raising to 22 the number of ENAM variations known to cause AI. The severity of the enamel phenotype in Enam+/−Ambn+/− double heterozygous mice is caused by composite digenic effects. Digenic inheritance should be explored as a cause of AI in humans.
Case Report: Transitioning to a Mixed‐Oil Intravenous Lipid Emulsion in an Adult Patient Receiving Home Parenteral Nutrition
Authors: Bathgate Jennifer R. , Matarese Laura , Ziegler Jane , Touger‐Decker Riva 
Published: September 2019

AbstractA potential risk of long‐term parenteral nutrition (PN) is intestinal failure–associated liver disease (IFALD). One recommendation for mitigating risk is limiting the fat dose to reduce the harmful effects of the ω‐6 fatty acids, which are the main ingredient in traditional fats. SMOFlipid (SMOF) (Kabi‐Fresenius, Bad Homburg, Germany) is a combination of soybean oil, medium‐chain triglycerides, olive oil, and fish oil emulsion. This fat source may alleviate the risk of IFALD and improve liver function tests. A patient with a long history of PN reliance and IFALD is presented in this case report. After 4 months on SMOF, total and direct bilirubin levels improved.
Surgery in Patients with Super Obesity: Medium‐Term Follow‐Up Outcomes at a High‐Volume Center
Authors: Bhandari Mohit , Ponce de Leon‐Ballesteros Guillermo , Kosta Susmit , Bhandari Mahak , Humes Terrel , Mathur Winni , Fobi Mathias 
Published: September 2019

ObjectiveThere is a need to determine which bariatric operations are the most effective for patients with super obesity and super‐super obesity.MethodsA retrospective cohort study was performed on patients with super obesity and super‐super obesity at Mohak Bariatrics and Robotics Surgery Center in Indore, India.ResultsFive hundred fourteen patients with super obesity and super‐super obesity had surgery at our center from January 2010 through December 2013. The baseline characteristics were different in different operations. The initial average age, weight, and BMI were 44.4 (SD 11.9) years, 145.4 (SD 24.2) kg, and 55.48 (SD 5.32) kg/m2, respectively. Sleeve gastrectomy (SG) (227 [44.2%]) was the most common procedure, followed by one‐anastomosis gastric bypass (OAGB) (124 [24.1%]), Roux‐en‐Y gastric bypass (RYGB) (102 [19.8%]), banded sleeve gastrectomy (BSG) (33 [6.4%]), and banded Roux‐en‐Y gastric bypass (BRYGB) (28 [5.4%]). After 3 years, the percentages of excess body weight loss (%EBWL) for SG, OAGB, RYGB, BSG, and BRYGB were 62.38%, 78.59%, 69.55%, 85.11%, and 75.77% (P < 0.0001), respectively. Failure to achieve BMI < 35 kg/m2 was more frequent in the group who underwent SG (67.9%), followed by RYGB (29.16%), BRYGB (22.2%), OAGB (9.87%), and none in the BSG group.ConclusionsBSG, OAGB, and BRYGB have very good to excellent midterm outcomes for patients with super obesity and super‐super obesity, whereas RYGB and SG have average outcomes at 3 years. There is a need for multicenter, long‐term, and prospective studies to be performed to confirm these findings.
Helmholtz decomposition of compressible flow data at Mach 0.8
Authors: Schoder Stefan , Freidhager Clemens , Kaltenbacher Manfred 
Published: September 2019

AbstractThe Helmholtz decomposition separates a compressible velocity field into vortical and compressible structures. Based on the original Helmholtz theorem for an unbounded domain, we use a modified theorem to take bounded domains into account. Furthermore, we present two different calculation procedures of the obtained equations and show results of an application to a compressible flow field. For this purpose the Helmholtz decomposition is applied to a Mach 0.8 flow over a rectangular cavity.
Sugar palm (Arenga pinnata [Wurmb.] Merr) starch films containing sugar palm nanofibrillated cellulose as reinforcement: Water barrier properties
Authors: Ilyas R. A. , Sapuan S. M. , Atiqah A. , Ibrahim Rushdan , Abral Hairul , Ishak M. R. , Zainudin E. S. , Nurazzi N. M. , Atikah M. S. N. , Ansari M. N. M. , Asyraf M. R. M. , Supian A. B. M. , Ya Hamdan 
Published: September 2019

AbstractSugar palm fiber (SPF) is an agro‐waste plant that can be used as potential source of biomass for various biomaterial applications. In this study, sugar palm nanofibrillated cellulose (SPNFC) that was isolated from SPF was used as a nanofiller to reinforce sugar palm starch (SPS) to produce bionanocomposites. To attain SPNFCs, SPF was undergo strong acid and alkaline treatments. Later, the SPNFCs were prepared from SPFs via high pressurized homogenization process. The reinforcement of SPNFCs (0‐1.0 wt%) and SPS is done by using solution casting methods. The films were characterized in terms of physical properties such as light transmittance, moisture content, water solubility, and water absorption. The resulting nanocomposites permitted better water resistance, low moisture absorption, and low light transmittance as compared to control SPS film. Adding 1 wt% SPNFCs loading significantly improved the water absorption and water solubility of the composite film by 24.13% and 18.60%, respectively, compared with the control SPS film. This was attributed to the high compatibility between the SPNFCs and SPS matrixes, which composed of the multi‐hydroxyl polymer having three hydroxyl groups per monomer. Thus, this study is to show the potential of SPS/SPNFCs nanocomposite films in packaging industries.
Impact of a New Consumer Form on the Quantity and Quality of Adverse Event Reports Submitted to the United States Food and Drug Administration
Authors: Muñoz Monica A. , Delcher Chris , Dal Pan Gerald J. , Kortepeter Cindy M. , Wu Eileen , Wei Yu‐Jung Jenny , Xiao Hong , Winterstein Almut G 
Published: September 2019

Study ObjectiveConsumers and healthcare professionals can voluntarily report adverse experiences associated with drug products to the United States Food and Drug Administration's (FDA) Adverse Event Reporting System (FAERS). Consumers and healthcare professionals used the same general voluntary reporting form (GVR) until mid‐2013, when a consumer voluntary reporting form (ConVR), written in plain language, was implemented. The objective of this study was to examine the effect of the ConVR on the quality and quantity of consumer reports submitted directly to FAERS.DesignDescriptive; quasi‐experimental.Data SourceFAERS database.Measurements and Main ResultsWe identified all consumer and healthcare professional reports received directly by the FDA from January 1, 2011, through December 31, 2015. Report quality was defined by the completeness of 15 individual data fields and a structured tool measuring clinical documentation. An interrupted time series design was used to evaluate the impact on the quantity of consumer reports. Consumer reports submitted on the ConVR generally included more patient, product, and event data in the structured data fields than those submitted on the GVR. Fields with the greatest absolute percentage difference after the ConVR was introduced included race/ethnicity (+77.2%), product start and stop dates (+43% and +40.3%, respectively), dechallenge and rechallenge information (+19.1% and +29.4%, respectively), and medical history (+27%). Our structured assessment also classified more reports received on the ConVR as well documented relative to the GVR consumer reports (64.9% vs 37.8%, p<0.01). The time series model demonstrated an immediate increase of 499 consumer reports in the month following the ConVR's implementation (p<0.01).ConclusionOur findings suggest that the ConVR has contributed positively to both the quality and quantity of consumer reports in FAERS.
Native ion mobility mass spectrometry reveals that small organic acid fragments impart gas‐phase stability to carbonic anhydrase II
Authors: Veale Clinton G.L. , Mateos Jimenez Maria , Mackay C. Logan , Clarke David J. 
Published: September 2019

RationaleA key element of studies that utilise ion mobility mass spectrometry (IM‐MS) under native electrospray conditions for the analysis of protein–ligand binding is the maintenance of the native conformation of a protein during the removal of bulk solvent. Ruotolo and co‐workers have demonstrated that the binding and subsequent dissociation of the anionic component of inorganic salts stabilise native protein conformations in the gas phase. In this study, we investigated the effect that organic acid fragments identified from a fragment‐based drug discovery (FBDD) campaign might have on the gas‐phase stability of carbonic anhydrase II (CA II).MethodsWe utilised native IM‐MS to monitor changes in the conformation of CA II in the absence and presence of four acidic fragments. By performing a series of collision‐induced unfolding (CIU) experiments we determined the effect of fragment binding on the gas‐phase stability of CA II.ResultsBinding and dissociation of acidic fragments result in increased gas‐phase stability of CA II. CFU experiments revealed that the native‐like compact gas‐phase conformation of the protein is stable with higher degree of pre‐activation when bound to a series of acidic fragments. Importantly, although acetate was present in high concentrations, the stabilising effect was not observed without the addition of the acidic fragments.ConclusionsBinding and subsequent dissociation of acidic fragments from CA II significantly delayed CIU in a manner which is probably analogous to the effect of inorganic anions. Furthermore, we saw a slightly altered stabilising effect between the different fragments investigated in this study. This suggests that the prevention of CIU by organic acids may be tuneable to specific properties of a bound ligand. These observations may open avenues to exploit IM‐MS as a screening platform in FBDD.