Miracle-dz/UniFAR-FTUs huggingface dataset:
Variable morbidity in alagille syndrome: a review of 43 cases.

BACKGROUND Alagille syndrome is one of the most common inherited disorders that cause chronic liver disease in children. Early reports suggested a benign course in these patients. Subsequent reports showed significant morbidity and mortality. This study was designed to analyze the long-term clinical course in Alagille syndrome. METHODS The records of children with Alagille syndrome seen during a 20-year period were reviewed. RESULTS Forty-three patients were identified. Liver disease was diagnosed before 12 months of age in 95%. The frequencies of renal anomalies (50%) and intracranial hemorrhage (12%) were significant. The high incidence of chronic otitis media (35%) has not been reported previously. One patient had a renal transplant. Vascular compromise as a pathologic mechanism for some characteristics of the syndrome is also suggested by the presence of small bowel stenosis and atresia, tracheal and bronchial stenosis, renal artery stenosis, middle aortic syndrome, and avascular necrosis of the humeral and femoral heads. Twenty (47%) patients underwent liver transplantation. Five of six who underwent Kasai procedure required liver transplantation. Twelve died (28%), five after liver transplantation. One patient died of intracranial bleeding. Sixteen (37%) without liver transplantation and 15 (35%) who underwent liver transplantation are alive. CONCLUSIONS Some patients with early-onset and more severe liver disease can benefit from liver transplantation. Careful and complete assessment should be made of infants with a cholestatic syndrome, to avoid misdiagnosis and unnecessary Kasai procedures. Our observation of vascular compromise in various organ systems suggests that notch signaling pathway defects affect angiogenesis in Alagille syndrome.
Understanding training levies

This report develops a deeper understanding of training levies and focuses on two mandatory training levies and one voluntary training levy: (1) the construction levy in England, Scotland and Wales, operated by the Construction Industry Training Board - ConstructionSkills; (2) the engineering construction levy in England, Scotland and Wales, operated by the Engineering Construction Industry Training Board; and (3) the UK film industry levy, or Skills Investment Fund, which is operated by Creative Skillset. Training levies are one of a range of policy levers, or best market solutions, which the UK Commission has identified as having the potential to encourage employers to invest in skills, on a collective basis, where there is sub-optimal provision of training due to market failure of various kinds. A training levy is a mechanism whereby firms, within an industry or locality, come together with the express aim of increasing workforce skills, via a mandatory or voluntary agreement. The use of training levies to increase the demand for, and supply of, skills was previously considered in the UK Commission's Review of Employer Collective Measures. This report builds on that evidence base by providing further understanding of training levies, such as their benefits and their impacts on employer behaviour. Through qualitative research, including interviews conducted with employers, levy operators and other stakeholders, the research explores the following questions: (1) What does an effective training levy system look like? ; (2) What is the impact of current levy systems? ; (3) What lessons can be learned in order to encourage employers to participate in levy-systems more widely? ; and (4) What features are most attractive/persuasive to employers? These are addressed by examining the three training levies mentioned above. Their industry background, history, governance and management, and their relationship to training are explored. Before that the report begins by laying down theoretical considerations for introducing a levy. It then identifies different types of training levies and considers the design factors policy architects would have to take into account in order to establish one. Context is then added via an historical and comparative perspective of training levies in the UK.
A nitric oxide synthase inhibitor, NG‐nitro‐l‐arginine‐methyl‐ester, exerts potent antiangiogenic effects on plasmacytoma in a newly established multiple myeloma severe combined immunodeficient mouse model

Summary. Angiogenesis is one of critical factors in sustaining the growth, invasion and metastasis of certain solid tumours and haematological malignancies such as multiple myeloma (MM). In the present study, we examined the anticancer potential of an inhibitor of nitric oxide synthase (NOS), NG‐nitro‐l‐arginine methyl ester (L‐NAME), in a novel severe combined immunodeficient mouse model (KHM mouse) that harbours the highly sanguineous plasmacytoma cell line KHM‐4, derived from a patient with highly chemoresistant MM. KHM mice were intraperitoneally administered with either L‐NAME, doxorubicin, melphalan, or paclitaxel. A significant reduction in tumour sizes was noted in L‐NAME‐administered KHM mice while no significant reduction was observed in melphalan‐ or doxorubicin‐administered mice. A profound decrease in angiogenesis was observed in tumour tissues from L‐NAME‐ and paclitaxel‐administered KHM mice. A marked decrease in human vascular endothelial cell growth factor (VEGF) levels was identified in tumour tissues from L‐NAME‐administered KHM mice, strongly suggesting that L‐NAME suppressed VEGF production by tumour cells through its inhibition of NOS in tumour cells, resulting in reduced neovasculization in mice leading to the regression of tumour sizes. The present data represent the first observations that certain NOS inhibitors potentially serve as experimental agents for the treatment of chemoresistant MM and plasmacytoma.
Reform of the Global Financial Architecture

This paper examines the implications of the global financial crisis of 2007-10 for reform of the global financial architecture, in particular the International Monetary Fund and the Financial Stability Board and their interaction. These two institutions are not fully comparable, but they must work more closely in the future to help prevent global financial crises. To this end, the paper identifies institutional and substantive reforms separately and in their joint work that would be desirable and appropriate.
A Disulfide Bond-forming Machine Is Linked to the Sortase-mediated Pilus Assembly Pathway in the Gram-positive Bacterium Actinomyces oris*

Background: Gram-positive bacteria secrete pilins through the Sec translocon in unfolded states. Results: Disruption of pilus disulfide bonds or genetic disruption of oxidoreductase-encoding genes mdbA and vkor abrogates pilus assembly in Actinomyces oris. Conclusion: MdbA and VKOR constitute a disulfide bond-forming machine in A. oris. Significance: Oxidative protein folding may be common in Actinobacteria and an attractive target for antimicrobials. Export of cell surface pilins in Gram-positive bacteria likely occurs by the translocation of unfolded precursor polypeptides; however, how the unfolded pilins gain their native conformation is presently unknown. Here, we present physiological studies to demonstrate that the FimA pilin of Actinomyces oris contains two disulfide bonds. Alanine substitution of cysteine residues forming the C-terminal disulfide bridge abrogates pilus assembly, in turn eliminating biofilm formation and polymicrobial interaction. Transposon mutagenesis of A. oris yielded a mutant defective in adherence to Streptococcus oralis, and revealed the essential role of a vitamin K epoxide reductase (VKOR) gene in pilus assembly. Targeted deletion of vkor results in the same defects, which are rescued by ectopic expression of VKOR, but not a mutant containing an alanine substitution in its conserved CXXC motif. Depletion of mdbA, which encodes a membrane-bound thiol-disulfide oxidoreductase, abrogates pilus assembly and alters cell morphology. Remarkably, overexpression of MdbA or a counterpart from Corynebacterium diphtheriae, rescues the Δvkor mutant. By alkylation assays, we demonstrate that VKOR is required for MdbA reoxidation. Furthermore, crystallographic studies reveal that A. oris MdbA harbors a thioredoxin-like fold with the conserved CXXC active site. Consistently, each MdbA enzyme catalyzes proper disulfide bond formation within FimA in vitro that requires the catalytic CXXC motif. Because the majority of signal peptide-containing proteins encoded by A. oris possess multiple Cys residues, we propose that MdbA and VKOR constitute a major folding machine for the secretome of this organism. This oxidative protein folding pathway may be a common feature in Actinobacteria.
Review of 3‐year outcomes of a very‐low‐energy diet‐based outpatient obesity treatment programme

Obesity is a complex disorder that requires a multidisciplinary treatment approach. This review evaluated 3‐year outcomes of a very‐low‐energy diet (VLED)‐based programme at a tertiary hospital multidisciplinary weight management clinic. Medical records of all patients who agreed to undertake the VLED programme and who did not undergo bariatric surgery during the 3‐year follow‐up period were examined. Baseline data collection included demographic and anthropometric characteristics, childhood onset of obesity and co‐existing medical conditions. Weight was modelled using a linear mixed effects analysis. Logistic regression analyses were used to model the probability of continuing to attend the clinic and to identify pre‐treatment factors associated with longer duration of attendance. Data from 1109 patients were included. A total of 231 patients (19.2%) were still attending the clinic 3 years after their initial appointment. Mean weight loss among patients who attended the clinic for 3 years was 6.4 kg (3.5%, 95% confidence interval [CI] 2.8, 4.2%). People who were prescribed pharmacotherapy maintained greater weight loss at 3 years (7.7% vs. 2.3% without pharmacotherapy, 95% CI for difference 3.9, 7.0%). People who had an onset of obesity in childhood, who had co‐existing hypertension or coronary artery disease, and who did not currently smoke were more likely to continue to attend the clinic for up to 3 years. In summary, in an outpatient weight management clinic, patients who undertook a VLED‐based programme and continued in follow‐up achieved a clinically significant weight loss at 3 years, particularly if pharmacotherapy was used for weight loss maintenance.
Social Marketing and Falls Prevention: Market Segmentation and Product Positioning

The present study sets out to better understand how falls prevention can be marketed and positioned in order to increase participation rates in seniors. Market segmentation was used to create unique marketing mixes for individual groups of seniors. The senior market was segmented into frequent falling males and females, and non-falling males and females. Frequent fallers were defined by having experienced 2 or more falls in the past year, where non-fallers had experienced 1 or less falls. Twelve focus groups were conducted with thirty-five participants (N=35), 65 years or older. Differences were mainly found between number of falls experienced and gender. Risk perception was found to be associated with number of falls as well as participation. Specific marketing mixes are presented for each segment, and compared with a pre-existing falls prevention pamphlet. Future research should investigate the use of risk perception and age as potential segmentation criteria.
Robust Head-shoulder Detection using Deformable Part-based Models

Conventional person detection algorithms lack of robustness, especially when the person is partially occluded. We propose thereby a robust head-shoulder detector in 2-D images using deformable part-based models. This detector can be used in a variety of applications such as people counting and person dwell time measurements. In experiments, we compare the head-shoulder detector with the full body detector quantitatively and analyze the robustness of the detector in realistic scenarios. In the results, we show that the model learned with our method outperforms other methods proposed in related work on an ambient assisted living application.
Meridional reorganizations of marine and terrestrial productivity during Heinrich events

[1] To study the response of the global carbon cycle to a weakening of the Atlantic Meridional Overturning Circulation (AMOC), a series of freshwater perturbation experiments is conducted both under preindustrial and glacial conditions using the earth system model of intermediate complexity LOVECLIM. A shutdown of the AMOC leads to substantial cooling of the North Atlantic, a weak warming of the Southern Hemisphere, intensification of the northeasterly trade winds, and a southward shift of the Intertropical Convergence Zone (ITCZ). Trade wind anomalies change upwelling in the tropical oceans and hence marine productivity. Furthermore, hydrological changes associated with a southward displacement of the ITCZ lead to a reduction of terrestrial carbon stocks mainly in northern Africa and northern South America in agreement with paleoproxy data. In the freshwater perturbation experiments the ocean acts as a sink of CO2, primarily through increased solubility. The net atmospheric CO2 anomaly induced by a shutdown of the AMOC amounts to about +15 ppmv and � 10 ppmv for preindustrial and glacial conditions, respectively. This background state dependence can be explained by the fact that the glacial climate is drier and the terrestrial vegetation therefore releases a smaller amount of carbon to the atmosphere. This study demonstrates that the net CO2 response to large-scale ocean circulation changes has significant contributions both from the terrestrial and marine carbon cycle.
Voluntary Pension Funding in Germany and the Effect of International Accounting

This paper empirically analyzes voluntary pension funding. Using data of German company pension plans from 1998 to 2006, it documents that traditionally unfunded plans have been increasingly replaced, especially by funding Contractual Trust Arrangements (CTAs). International influences such as pension funding abroad are a major determinant of this shift in corporate financial policy but financial factors such as profitability are not found to have an important effect. We also investigate the consequences of applying IAS/IFRS or US GAAP instead of German GAAP and find evidence that supports the hypothesis that international accounting has a positive effect on voluntary pension funding in Germany.
Does Hedging Increase Firm Value

In an uncertain financial world, corporate risk management has become an important element of a firm’s overall business strategy. The ability to manage risk will help companies act more confidently on future business decisions. Their knowledge of the risks they are facing will give them various options on how to deal with potential problems. One of the most popular risk management programs that firm adopts is to hedge against the future’s fluctuations of income due to the changes in currency exchange rate or interest rate risk. Despite an increasing interest in developing theoretical studies about the reasons why firms involved in risk management, however, only a handful of studies that address the issue whether risk management can enhance the firm value. The purpose of this project research is to fill this gap and investigate the impact of hedging on firm value. Using Tobin’s Q as an approximation for firm market value and hedging as a control variable, I examined 90 Swedish firms listed on the Stockholm Stock Exchange, having the total assets exceeds at least 1 billion Euros. The results of the regression analysis show insignificant indication that the usage of hedging impacts firm value positively. The findings of this research imply that there is no evidence that support the hypothesis that hedging causes an increase in firm value. (Less)
Ulcerative colitis: effect on fertility and pregnancy

__________________________________________________________________________________________ Introduction: Ulcerative colitis is one of the most interesting nosological entities of gastroenterology and internal medicine. This chronic autoimmune disorder of the digestive system, along with Crohn's disease, is a type of idiopathic inflammatory bowel disease. It mainly affects people during their peak reproductive age, i.e., 15 to 30 years old. It is characterized by diffuse mucosal inflammation of the large intestine, which is limited to the colon and shows alternating periods of exacerbations and remissions. Purpose: To review the world literature regarding the impact of the disease on fertility, the course of pregnancy, and consecutively, the outcome of pregnancy. Materials and methods: An extensive review of the recent national and international literature in electronic databases (Pubmed, Google Scholar) and in scientific journals was accomplished through the use of appropriate keywords. Results: The majority of women will have a chance of conceiving. For about 25% of them, conception will be achieved in the course of the disease and it will progress normally, resulting in a healthy fetal outcome. Infertility only occurs among men while they are taking medicines, whereas among women it occurs after surgery for ileoanal anastomosis. During periods of flare-ups, there is a possibility of spontaneous abortion, premature labor and birth of an infant with low birth weight. The majority of pharmaceutical formulations can be used safely both before and after pregnancy without causing any birth defects.
Desing of a coded aperture base computed tomography architecture with two X-ray rotating sources

Computed tomography (CT) is a non-destructive technique that allows estimation and visualization of the internal structure of an object. Traditionally, CT images are captured by a CT scanner. However, different factors reduce the quality of the acquired images. To obtain a high quality CT images is necessary increase the number of sensors or oversample the object. The number of projections needed for sensing a CT scene is determined by the Nyquist limit, however, in some cases the imposed projections number is excessive. Coded aperture are elements that can block or allow the passing of X-rays and is one approach that can overcome these limitations. Compressive sensing (CS) has emerged as a sampling technique requiring fewer projections than those specified by the Nyquist criterion. CS is a theory to acquire and to reconstruct a signal efficiently by the search of a sparse solution to an indeterminate system of linear equations. A strategy to introduce CS theory in a CT configuration is to include elements into the system that allow coding the measurements to get compressed samples. This paper describes a CS system for CT based on coded apertures using two sources and a two-dimensional array that rotate around the object. An optimized value of transmittance and an aperture distribution are selected such that the quality of reconstruction is efficient. In order to compare the performance of the proposed method, two real CT images and two synthetic CT image were used. Simulations indicate that CT architecture provides comparable results to those achieved with traditional CT architectures. The simulation results show that the proposed method allows more diversity coding. This allows up to 2 dB improvement in terms of PSNR than the results obtained using traditional architecture cone beam.
A new semantic segmentation model for remote sensing images

Semantic segmentation for remote sensing images is a critical process in the workflow of object-based image analysis. Recently, convolutional neural networks(CNNs) are powerful visual models that yield hierarchies of features. In this paper, we propose a deep convolutional encoder-decoder model for remote sensing images segmentation. Specifically, we rely on the encoder network to extract the high-level semantic feature of ultra-high resolution images and the decoder network is employed to map the low resolution encoder feature maps to full input resolution feature maps for pixel-wise labeling. Also the fully connected conditional random field (CRF) is integrated into the model so that the network can be trained end-to-end. Experiments on the Vaihingen dataset demonstrate that our model can make promising performance.
Differential Intrinsic Response Dynamics Determine Synaptic Signal Processing in Frog Vestibular Neurons

Central vestibular neurons process head movement-related sensory signals over a wide dynamic range. In the isolated frog whole brain, second-order vestibular neurons were identified by monosynaptic responses after electrical stimulation of individual semicircular canal nerve branches. Neurons were classified as tonic or phasic vestibular neurons based on their different discharge patterns in response to positive current steps. With increasing frequency of sinusoidally modulated current injections, up to 100 Hz, there was a concomitant decrease in the impedance of tonic vestibular neurons. Subthreshold responses as well as spike discharge showed classical low-pass filter-like characteristics with corner frequencies ranging from 5 to 20 Hz. In contrast, the impedance of phasic vestibular neurons was relatively constant over a wider range of frequencies or showed a resonance at ∼40 Hz. Above spike threshold, single spikes of phasic neurons were synchronized with the sinusoidal stimulation between ∼20 and 50 Hz, thus showing characteristic bandpass filter-like properties. Both the subthreshold resonance and bandpass filter-like discharge pattern depend on the activation of an ID potassium conductance. External current or synaptic stimulation that produced impedance increases (i.e., depolarization in tonic or hyperpolarization in phasic neurons) had opposite and complementary effects on the responses of the two types of neurons. Thus, membrane depolarization by current steps or repetitive synaptic excitation amplified synaptic inputs in tonic vestibular neurons and reduced them in phasic neurons. These differential, opposite membrane response properties render the two neuronal types particularly suitable for either integration (tonic neurons) or signal detection (phasic neurons), respectively, and dampens variations of the resting membrane potential in the latter.
An Efficient Metropolitan WDM Ring Architecture for a Slotted Transmission Technique

In this paper, we present a wavelength division multiplexing multiring metropolitan area network architecture using a separate ring as control channel and a finite number of access nodes. Each access node is equipped with a fixed tuned transmitter and a fixed tuned receiver to exchange control information over the control wavelength. Also, each access node has a tunable transmitter to efficiently exploit all data wavelengths for data transmission. The set of data wavelengths is divided into wavelength bands. This allows a flexible node design with a number of parallel tunable receivers per node, each operating in a specific wavelength band and providing limited tuning time. On the one hand, our network strategy confronts more efficiently the scalability and maintenance problems comparatively with most of the access ring protocols. On the other hand, the proposed access algorithm avoids both the data wavelengths and the receiver collisions, improving even more the network utilization. An analytic model is developed for the performance measures evaluation. Also, we develop another analysis approach using discrete event simulation model based on self-similar statistics. Analysis is accomplished studying various numbers of access nodes, data wavelengths, and buffer size.