bluuebunny/crossref_metadata_2025_split huggingface dataset:
Functional and T Cell Receptor Repertoire Analyses of Peripheral Blood and Infrapatellar Fat Pad T Cells in Knee Osteoarthritis
Authors: Sae-jung Thitiya , Sengprasert Panjana , Apinun Jirun , Ngarmukos Srihatach , Yuktanandana Pongsak , Tanavalee Aree , Reantragoon Rangsima 
Published: October 2018

Objective.Osteoarthritis (OA) is a condition that features inflammation and immune responses of innate and adaptive immunity. The role of T cells in knee OA pathogenesis is still unclear. Our aim was to characterize T cell functions and their clonality in patients with knee OA in peripheral blood (PB) and infrapatellar fat pads (IPFP).Methods.We isolated T cells from PB and IPFP of patients with knee OA and PB of healthy individuals and determined soluble mediators produced from these cells. In addition, we performed a clonal analysis of activated CD8+ T cells and compared the T cell receptor β-variable gene chain (TRBV) usages between T cells in PB and IPFP of patients with knee OA.Results.Our results suggest that in patients with knee OA, circulating T cells possess a more “cytotoxic” profile or rather impaired cytokine production, but the knee microenvironment allows for these T cells to produce proinflammatory cytokines [interleukin (IL)-1β, IL-6, tumor necrosis factor], IL-17, and interferon-γ within IPFP. Activated CD8+ IPFP T cells carry different repertoire distribution from those present in PB of patients with knee OA. Shared TRBV usage of activated CD8+ IPFP T cells among the 3 patients with knee OA was also observed.Conclusion.Our study describes the nature of T cells in knee OA that may be due to “unhealthy” aging or other factors that drive healthy aging T cells into a state of imbalance, thus contributing to the pathogenesis of knee OA.
Development of a Preliminary Ultrasonographic Enthesitis Score in Psoriatic Arthritis — GRAPPA Ultrasound Working Group
Authors: Tom Stephanie , Zhong Yujie , Cook Richard , Aydin Sibel Zehra , Kaeley Gurjit , Eder Lihi 
Published: October 2018

Objective.To assess the performance of various sonographic elemental entheseal lesions in distinguishing between psoriatic arthritis (PsA) and controls to inform the development of a novel sonographic enthesitis score for PsA.Methods.A total of 100 age- and sex-matched individuals (50 PsA and 50 controls) were evaluated. Eleven entheseal sites were scanned bilaterally according to a standardized protocol by 2 sonographers. Based on the Outcome Measures in Rheumatology (OMERACT) definition of sonographic enthesitis, the following lesions were assessed: structural entheseal changes (hypoechogenicity), thickening, bone erosion, enthesophytes, calcification, and Doppler signal, in addition to bursitis and bone irregularities. The images were read by 2 readers blinded to the clinical information. A series of logistic regression models were used to find the optimal combination of entheseal sites and elementary lesions that distinguished PsA from controls.Results.Mean age was 55 ± 10 years (59% males). The optimal model that distinguished PsA from controls included 5 elementary lesions (enthesophytes, Doppler signal, erosions, thickening, and hypoechogenicity) and 6 entheseal sites (patellar ligament insertions into the distal patella and tibial tuberosity, Achilles tendon and plantar fascia insertions into the calcaneus, common extensor tendon insertion into lateral epicondyle, and supraspinatus insertion into the superior facet of the humerus). The area under the receiver-operating characteristic curve for this model was 0.93 (95% CI 0.88–0.98).Conclusion.We identified potential elemental ultrasonographic abnormalities and entheseal sites that could distinguish PsA and controls. This information will contribute to the development of a new sonographic score for assessment of enthesitis in patients with PsA.
Antimalarial-induced Cardiomyopathy in Systemic Lupus Erythematosus: As Rare as Considered?
Authors: Tselios Konstantinos , Deeb Mery , Gladman Dafna D. , Harvey Paula , Akhtari Shadi , Mak Susanna , Butany Jagdish , Urowitz Murray B. 
Published: October 2018

Objective.Antimalarials (AM) are recommended for all systemic lupus erythematosus (SLE) patients without specific contraindications. Their main adverse effect is retinal damage; however, heart disease has been described in isolated cases. The aim of our study is to describe 8 patients with AM-induced cardiomyopathy (AMIC) in a defined SLE cohort.Methods.Patients attending the Toronto Lupus Clinic and diagnosed with definite (based on endomyocardial biopsy; EMB) and possible AMIC were included [based on cardiac magnetic resonance imaging (cMRI) and other investigations].Results.Eight female patients (median age 62.5 yrs, disease duration 35 yrs, AM use duration 22 yrs) were diagnosed with AMIC in the past 2 years. Diagnosis was based on EMB in 3 (extensive cardiomyocyte vacuolation, intracytoplasmic myelinoid, and curvilinear bodies). In 4 patients, cMRI was highly suggestive of AMIC (ventricular hypertrophy and/or atrial enlargement and late gadolinium enhancement in a nonvascular pattern). Another patient was diagnosed with complete atrioventricular block, left ventricular and septal hypertrophy, along with concomitant ocular toxicity. All patients had abnormal cardiac troponin I (cTnI) and brain natriuretic peptide (BNP), whereas 7/8 also had chronically elevated creatine phosphokinase. During followup, 1 patient died from refractory heart failure. In the remaining patients, hypertrophy regression and a steady decrease of heart biomarkers were observed after AM cessation.Conclusion.Once considered extremely rare, AMIC seems to be underrecognized, probably because of the false attribution of heart failure or hypertrophy to other causes. Certain biomarkers (cTnI, BNP) and imaging findings may lead to early diagnosis and enhance survival.
Differentiation of Langerhans Cells from Monocytes and Their Specific Function in Inducing IL-22–Specific Th Cells
Authors: Otsuka Yohei , Watanabe Eri , Shinya Eiji , Okura Sadayuki , Saeki Hidehisa , Geijtenbeek Teunis B. H. , Takahashi Hidemi 
Published: October 2018

Abstract
Human mucosal tissues and skin contain two distinct types of dendritic cell (DC) subsets, epidermal Langerhans cells (LCs) and dermal DCs, which can be distinguished by the expression of C-type lectin receptors, Langerin and DC-SIGN, respectively. Although peripheral blood monocytes differentiate into these distinct subsets, monocyte-derived LCs (moLCs) induced by coculture with GM-CSF, IL-4, and TGF-β1 coexpress both Langerin and DC-SIGN, suggesting that the environmental cues remain unclear. In this study, we show that LC differentiation is TGF-β1 dependent and that cofactors such as IL-4 and TNF-α promote TGF-β1–dependent LC differentiation into Langerin+DC-SIGN− moLCs but continuous exposure to IL-4 blocks differentiation. Steroids such as dexamethasone greatly enhanced TNF-α–induced moLC differentiation and blocked DC-SIGN expression. Consistent with primary LCs, dexamethasone-treated moLCs express CD1a, whereas monocyte-derived DCs (moDCs) express CD1b, CD1c, and CD1d. moDCs but not moLCs produced inflammatory cytokines after stimulation with CD1b and CD1d ligands mycolic acid and α-galactosylceramide, respectively. Strikingly, CD1a triggering with squalene on moLCs but not moDCs induced strong IL-22-producing CD4+ helper T cell responses. As IL-22 is an important cytokine in the maintenance of skin homeostasis, these data suggest that CD1a on LCs is involved in maintaining the immune barrier in the skin.
Differential Activation of Hepatic Invariant NKT Cell Subsets Plays a Key Role in Progression of Nonalcoholic Steatohepatitis
Authors: Maricic Igor , Marrero Idania , Eguchi Akiko , Nakamura Ryota , Johnson Casey D. , Dasgupta Suryasarathi , Hernandez Carolyn D. , Nguyen Phirum Sam , Swafford Austin D. , Knight Rob , Feldstein Ariel E. , Loomba Rohit , Kumar Vipin 
Published: October 2018

Abstract
Innate immune mechanisms play an important role in inflammatory chronic liver diseases. In this study, we investigated the role of type I or invariant NKT (iNKT) cell subsets in the progression of nonalcoholic steatohepatitis (NASH). We used α-galactosylceramide/CD1d tetramers and clonotypic mAb together with intracytoplasmic cytokine staining to analyze iNKT cells in choline-deficient l-amino acid–defined (CDAA)-induced murine NASH model and in human PBMCs, respectively. Cytokine secretion of hepatic iNKT cells in CDAA-fed C57BL/6 mice altered from predominantly IL-17+ to IFN-γ+ and IL-4+ during NASH progression along with the downmodulation of TCR and NK1.1 expression. Importantly, steatosis, steatohepatitis, and fibrosis were dependent upon the presence of iNKT cells. Hepatic stellate cell activation and infiltration of neutrophils, Kupffer cells, and CD8+ T cells as well as expression of key proinflammatory and fibrogenic genes were significantly blunted in Jα18−/− mice and in C57BL/6 mice treated with an iNKT-inhibitory RAR-γ agonist. Gut microbial diversity was significantly impacted in Jα18−/− and in CDAA diet–fed mice. An increased frequency of CXCR3+IFN-γ+T-bet+ and IL-17A+ iNKT cells was found in PBMC from NASH patients in comparison with nonalcoholic fatty liver patients or healthy controls. Consistent with their in vivo activation, iNKT cells from NASH patients remained hyporesponsive to ex-vivo stimulation with α-galactosylceramide. Accumulation of plasmacytoid dendritic cells in both mice and NASH patients suggest their role in activation of iNKT cells. In summary, our findings indicate that the differential activation of iNKT cells play a key role in mediating diet-induced hepatic steatosis and fibrosis in mice and its potential involvement in NASH progression in humans.
Computational evidence on the distributive properties of monetary policy
Authors: Chen Siyan , Desiderio Saul 
Published: October 2018

AbstractEmpirical studies have pointed out that monetary policy may significantly affect income and wealth inequality. To investigate the distributive properties of monetary policy the authors resort to an agent-based macroeconomic model where firms, households and one bank interact on the basis of limited information and adaptive rules-of-thumb. Simulations show that the model can replicate fairly well a number of stylized facts, especially those relative to the business cycle. The authors address the issue using three types of computational experiments, including a global sensitivity analysis carried out through a novel methodology which greatly reduces the computational burden of simulations. The result emerges that a more restrictive monetary policy increases inequality, even though this effect may differ across groups of households. In addition, it appears to be attenuated if the bank’s willingness to lend is lower. The overall analysis suggests that inequality can constitute valuable information also for central banks.
Empirical high-resolution wind field and gust model in mountainous and hilly terrain based on the dense WegenerNet station networks
Authors: Schlager Christoph , Kirchengast Gottfried , Fuchsberger Juergen 
Published: October 2018

Abstract. A weather diagnostic application for automatic generation of gridded wind fields in near-real-time, recently developed by the authors Schlager et al. (2017), is applied to the WegenerNet Johnsbachtal (JBT) meteorological station network. This station network contains 11 meteorological stations at elevations from about 600 to 2200 m in a mountainous region in the north of Styria, Austria. The application generates, based on meteorological observations with a temporal resolution of 10 min from the WegenerNet JBT, mean wind and wind gust fields at 10 and 50 m height levels with a high spatial resolution of 100 m × 100 m and a temporal resolution of 30 min. These wind field products are automatically stored to the WegenerNet data archives, which also include long-term averaged weather and climate datasets from post-processing. The main purpose of these empirically modeled products is the evaluation of convection-permitting dynamical climate models as well as investigating weather and climate variability on a local scale. The application's performance is evaluated against the observations from meteorological stations for representative weather conditions, for a month including mainly thermally induced wind events (July 2014) and a month with frequently occurring strong wind events (December 2013). The overall statistical agreement, estimated for the vector-mean wind speed, shows a reasonably good modeling performance. Due to the spatially more homogeneous wind speeds and directions for strong wind events in this mountainous region, the results show somewhat better performance for these events. The difference between modeled and observed wind directions depends on the station location, where locations along mountain slopes are particularly challenging. Furthermore, the seasonal statistical agreement was investigated from 5-year climate data of the WegenerNet JBT in comparison to 9-year climate data from the high-density WegenerNet meteorological station network Feldbach Region (FBR) analyzed by Schlager et al. (2017). In general, the 5-year statistical evaluation for the JBT indicates similar performance as the shorter-term evaluations of the two representative months. Because of the denser WegenerNet FBR network, the statistical results show better performance for this station network. The application can now serve as a valuable tool for intercomparison with, and evaluation of, wind fields from high-resolution dynamical climate models in both the WegenerNet FBR and JBT regions.
The importance of small artificial water bodies as sources of methane emissions in Queensland, Australia
Authors: Grinham Alistair , Albert Simon , Deering Nathaniel , Dunbabin Matthew , Bastviken David , Sherman Bradford , Lovelock Catherine E. , Evans Christopher D. 
Published: October 2018

Abstract. Emissions from flooded land represent a direct source of anthropogenic
greenhouse gas (GHG) emissions. Methane emissions from large, artificial water
bodies have previously been considered, with numerous studies assessing
emission rates and relatively simple procedures available to determine their
surface area and generate upscaled emissions estimates. In contrast, the role
of small artificial water bodies (ponds) is very poorly quantified, and
estimation of emissions is constrained both by a lack of data on their
spatial extent and a scarcity of direct flux measurements. In this study, we
quantified the total surface area of water bodies
< 105 m2 across Queensland, Australia, and emission rates
from a variety of water body types and size classes. We found that the
omission of small ponds from current official land use data has led to an
underestimate of total flooded land area by 24 %, of small artificial
water body surface area by 57 % and of the total number of artificial
water bodies by 1 order of magnitude. All studied ponds were significant
hotspots of methane production, dominated by ebullition (bubble) emissions.
Two scaling approaches were developed with one based on pond primary use
(stock watering, irrigation and urban lakes) and the other using size class.
Both approaches indicated that ponds in Queensland alone emit over 1.6 Mt
CO2 eq. yr−1, equivalent to 10 % of the state's entire
land use, land use change and forestry sector emissions. With limited data
from other regions suggesting similarly large numbers of ponds, high
emissions per unit area and under-reporting of spatial extent, we conclude
that small artificial water bodies may be a globally important missing
source of anthropogenic greenhouse gas emissions.
Robinson Jeffers's Presence in Cormac McCarthy's Imagination
Authors: Graham Don 
Published: October 2018

Abstract
One of the literary influences in No Country for Old Men is Robinson Jeffers's poem “Hurt Hawks.” A passage involving Sheriff Bell's discovery of a dead hawk on the desert highway points clearly to echoes of Jeffers's moving account of a wounded hawk in his poem first published in 1928. McCarthy's literary models are impeccable.
Orthorexia nervosa and obsessive-compulsive behavior among college students in the United States
Authors: Costa Christine B. , Hardan-Khalil Kholoud 
Published: October 2018

Background/Objective: Orthorexia nervosa (ON) is a disordered eating pattern and obsessive-compulsive disorder (OCD) is an anxiety condition. Although ON shares many similarities with OCD, their relationship remains unclear. The objective of this study was to investigate the prevalence of both ON behaviors and OCD behaviors and whether or not an association between ON behaviors and OCD behaviors exist among college/university students in the United States.Methods: Using a cross-sectional, descriptive research design, a convenience sample of 270 college undergraduate students provided data using self-administered questionnaires: ORTO-15 to detect orthorexia behaviors and Obsessive-Compulsive Inventory-Revised (OCI-R) to detect obsessive-compulsive behaviors. Additionally, the participants completed a demographic questionnaire designed by the authors. Chi square, Pearson’s correlation coefficient and analysis of variance evaluated the variables of interest using a significance level of .05.Results: The prevalence rate for orthorexia nervosa behaviors was 37% and for obsessive-compulsive behaviors was 38.5%. There was a strong negative correlation (p < .001) between the scores of the ORTO-15 and the scores of the OCI-R. As orthorexia behaviors increased, obsessive-compulsive behaviors like-wise increased.Conclusions: It was concluded that college/university students are a high-risk group for orthorexia nervosa and obsessive-compulsive behaviors. Mental health professionals who encounter clients with orthorexic tendencies are encouraged to also screen for obsessive compulsive symptomology as a comorbid problem.
The Effect of Slice Thickness on the Volume Estimations Performed by Using Cone Beam CT
Authors: Bayrak Seval , Sezgin Omer Said , Kayıpmaz Saadettin , Çan Gamze 
Published: October 2018

Objective: The purpose of this
study is to investigate possible effects of the slice thickness on volume
estimations with CBCT.

Materials
and Methods: Intraosseous cavities representing bone defects on femoral
condyles of bovines were scanned by CBCT. Consecutive slices at 0.1 mm, 0.2 mm,
0.3 mm, 0.4 mm, 0.5 mm, 1 mm, 2 mm, 3 mm, 4 mm, and 5 mm thickness were used to
estimate the volumes of the cavities using Cavalieri principle of stereological
methods then compared with the volumes obtained by Archimedean principle.

Results: The volumes estimated
by Cavalieri principle in 0.1, 0.2, and 0.3 mm thickness slices were consistent
with the volumes obtained by Archimedean principle (p&gt;0.05). For all the
defects on the CBCT images, the volumes of the defects which were calculated
with Cavalieri principle in 0.1, 0.2, and 0.3mm slice thickness were found to
be consistent with the actual volumes, however, the volumes that were
calculated in 0.4 mm, 0.5 mm, 1 mm, 2 mm, 3 mm, 4mm, and 5 mm slice thickness
were found to differ from the actual volumes.

Conclusion:
When
volume calculations were made by Cavalieri principle, the thinnest slice section
should be chosen to make calculations consistent with actual volumes.
Molecular Genetics of the α‐Thalassaemias
Authors: Aguilar‐Martinez Patricia , Gulbis Béatrice 
Published: October 2018

Abstractα‐Thalassaemias are widespread disorders worldwide, with the exception of rare non‐α‐globin gene related forms and of acquired forms in patients with myelodysplasia. However, the most severe forms are found among the populations of Asian and Mediterranean ancestry. Clinical manifestations of α‐thalassaemia are highly variable, ranging from asymptomatic subjects to a more or less severe haemolytic anaemia. Haemoglobin Bart'shydrops foetalis(absence of functional α‐globin genes) leads to deathin uteroor at birth. Antenatal screening is available for couples at risk for this severe form, that is carriers of an α zero thalassaemia allele (absence of any functional α gene on one chromosome 16). Carrier detection is first based on red blood cell indices and confirmed by molecular analysis. Selective advantage against severePlasmodium falciparuminfection has been demonstrated for individuals with α‐thalassaemia. α‐Thalassaemia is also a genetic modifier in sickle cell syndromes and β‐thalassaemia.Key Conceptsα‐Thalassaemia has become a widespread genetic disorder worldwide due to the relative protection it conferred against life‐threatening malarial anaemia.There are four main forms of α‐thalassaemia depending on the number of remaining functional α globin gene(s) in the individual.α‐Thalassaemia can be a severe condition: absence of three functional α‐globin genes results in chronic haemolytic anaemia of variable severity (haemoglobin H disease), whereas the total absence of functional α‐globin gene leads to haemoglobin Bart'shydrops foetalis, a usually lethal condition.In uterotransfusion therapy and stem cell transplantation may improve the outcome of foetuses and infants with Hb Bart'shydrops foetalisin very rare cases.α‐Thalassaemia carriers are usually clinically asymptomatic; however, detection of carriers of an α zero thalassaemia allele is of utmost importance to allow preventinghydrops foetalis.α‐Thalassaemia carriers may benefit from the diagnosis of their condition, as this may avoid unnecessary iron therapy due to the presence of microcytosis.α‐Thalassaemia is a genetic modifier of sickle cell disease severity through the reduction of the haemolytic rate and higher haemoglobin levels.
Genetics of Human Congenital Heart Disease
Authors: Wilsdon Anna 
Published: October 2018

Abstract

Congenital heart disease (CHD) affects 0.9% of babies born in the United Kingdom. With improvements in medical and surgical treatments, individuals are surviving longer, and there are now more adults surviving with CHD than children. Prospective parents may ask about the chances of any children they have being affected. Unfortunately, the rate of genetic diagnosis in CHD is relatively low. CHD is likely to result from a complex interaction of genes and the environment. Recent sequencing studies in large cohorts of individuals with CHD have started to decipher the genetic contribution. There is a role for
de novo
and inherited mutations in CHD, in addition to chromosomal abnormalities, copy number variation, somatic mutations and epigenetics. Whole exome sequencing studies have identified novel CHD genes and expanded the phenotype of known CHD genes, but researchers and clinicians must also consider how to deal with secondary findings which are unrelated to the primary research aim.

Key Concepts

Congenital heart disease is the most common birth defect. Some individuals have complex health needs requiring lifelong follow‐up.

Mendelian inheritance is rare in CHD, and it is likely that CHD results from a complex interaction between genes and the environment.

Reduced penetrance and variable expressivity are common in CHD.

Most individuals have CHD in isolation (nonsyndromic CHD). Syndromic CHD affects a smaller proportion of people, and is the combination of CHD with another extracardiac congenital abnormality and/or neurodevelopmental disability.

A genetic diagnosis is more likely to be identified in syndromic CHD, but the majority of individuals have nonsyndromic CHD.

Untargeted sequencing in large cohorts with CHD can identify novel genes and expand the phenotypic spectrum of known genes.

There may be a shared pathogenesis between CHD and neurodevelopmental disability.

Collaboration between researchers will be required to fully understand the genetic causes of CHD.

Sequencing will generate secondary findings that are not related to the primary aim of the research. Clinicians and researchers have a duty of care to their research participants and should consider the methods to deal with these.