Miracle-dz/UniFAR-FTUs huggingface dataset:
A boy with Prader-Willi syndrome: unmasking precocious puberty during growth hormone replacement therapy

SUMMARY Prader-Willi syndrome (PWS) is a genetic disorder frequently characterized by obesity, growth hormone deficiency, genital abnormalities, and hypogonadotropic hypogonadism. Incomplete or delayed pubertal development as well as premature adrenarche are usually found in PWS, whereas central precocious puberty (CPP) is very rare. This study aimed to report the clinical and biochemical follow-up of a PWS boy with CPP and to discuss the management of pubertal growth. By the age of 6, he had obesity, short stature, and many clinical criteria of PWS diagnosis, which was confirmed by DNA methylation test. Therapy with recombinant human growth hormone (rhGH) replacement (0.15 IU/kg/day) was started. Later, he presented psychomotor agitation, aggressive behavior, and increased testicular volume. Laboratory analyses were consistent with the diagnosis of CPP (gonadorelin-stimulated LH peak 15.8 IU/L, testosterone 54.7 ng/dL). The patient was then treated with gonadotropin-releasing hormone analog (GnRHa). Hypothalamic dysfunctions have been implicated in hormonal disturbances related to pubertal development, but no morphologic abnormalities were detected in the present case. Additional methylation analysis (MS-MLPA) of the chromosome 15q11 locus confirmed PWS diagnosis. We presented the fifth case of CPP in a genetically-confirmed PWS male. Combined therapy with GnRHa and rhGH may be beneficial in this rare condition of precocious pubertal development in PWS.
Arginase: The Emerging Therapeutic Target for Vascular Oxidative Stress and Inflammation

Oxidative stress and inflammation in the vascular wall are essential mechanisms of atherosclerosis and vascular dysfunctions associated with risk factors such as metabolic diseases, aging, hypertension, etc. Evidence has been provided that activation of the vascular endothelial cells in the presence of the risk factors promotes oxidative stress and vascular inflammatory responses, leading to acceleration of atherosclerotic vascular disease. Increasing number of studies from recent years demonstrates that uncoupling of endothelial nitric oxide synthase (eNOS), whereby the enzyme eNOS produces detrimental amount of superoxide anion O2− instead the vasoprotective nitric oxide (NO⋅), plays a critical role in vascular dysfunction under various pathophysiological conditions and in aging. The mechanisms of eNOS-uncoupling seem multiple and complex. Recent research provides emerging evidence supporting an essential role of increased activity of arginases including arginase-I and arginase-II in causing eNOS-uncoupling, which results in vascular oxidative stress and inflammatory responses, and ultimately leading to vascular diseases. This review article will summarize the most recent findings on the functional roles of arginases in vascular diseases and/or dysfunctions and the underlying mechanisms in relation to oxidative stress and inflammations. Moreover, regulatory mechanisms of arginases in the vasculature are reviewed and the future perspectives of targeting arginases as therapeutic options in vascular diseases are discussed.
Factors Influencing Office-Workers' Purchase Intention though Social Media: An Empirical Study

Social media (SM) are currently gaining rapid and widespread popularity. SM have been recognized as effective marketing tools that can influence customer behavior; however, the literature neglects this topic. This investigation proposed an integrated and modified framework derived from the technology acceptance model to systematically analyze and investigate the critical influences on the intention of office-workers to make a purchase via SM when SM are used for marketing communications. This study surveyed 400 office-workers from businesses in Taiwan. The findings demonstrate that factors such as perceived ease of use, SM experience, social influence, gender difference, and product/service information are important influences on SM shopping predisposition. Additionally, three clusters of shoppers are identified based on their purchase intention. Each cluster has different demographic and behavioral characteristics and exhibits significant concerns regarding the above factors. Specific marketing strategies are provided for each of the three clusters to help companies better target their customers. Factors Influencing OfficeWorkers’ Purchase Intention though Social Media: An Empirical Study
Brain Region–Specific Degeneration with Disease Progression in Late Infantile Neuronal Ceroid Lipofuscinosis (CLN2 Disease)

BACKGROUND AND PURPOSE: Late infantile neuronal ceroid lipofuscinosis (CLN2 disease) is a uniformly fatal lysosomal storage disease resulting from mutations in the CLN2 gene. Our hypothesis was that regional analysis of cortical brain degeneration may identify brain regions that are affected earliest and most severely by the disease. MATERIALS AND METHODS: Fifty-two high-resolution 3T MR imaging datasets were prospectively acquired on 38 subjects with CLN2. A retrospective cohort of 52 disease-free children served as a control population. The FreeSurfer software suite was used for calculation of cortical thickness. RESULTS: An increased rate of global cortical thinning in CLN2 versus control subjects was the primary finding in this study. Three distinct patterns were observed across brain regions. In the first, subjects with CLN2 exhibited differing rates of cortical thinning versus age. This was true in 22 and 26 of 34 regions in the left and right hemispheres, respectively, and was also clearly discernable when considering brain lobes as a whole and Brodmann regions. The second pattern exhibited a difference in thickness from healthy controls but with no discernable change with age (9 left hemispheres, 5 right hemispheres). In the third pattern, there was no difference in either the rate of cortical thinning or the mean cortical thickness between groups (3 left hemispheres, 3 right hemispheres). CONCLUSIONS: This study demonstrates that CLN2 causes differential rates of degeneration across the brain. Anatomic and functional regions that degenerate sooner and more severely than others compared with those in healthy controls may offer targets for directed therapies. The information gained may also provide neurobiologic insights regarding the mechanisms underlying disease progression.
The Hazards of Debt: Rollover Freezes, Incentives, and Bailouts

We investigate the trade-off between incentive provision and inefficient rollover freezes for a firm financed with short-term debt. First, debt maturity that is too short-term is inefficient, even with incentive provision. The optimal maturity is an interior solution that avoids excessive rollover risk while providing sufficient incentives for the manager to avoid risk-shifting when the firm is in good health. Second, allowing the manager to risk-shift during a freeze actually increases creditor confidence. Debt policy should not prevent the manager from holding what may appear to be otherwise low-mean strategies that have option value during a freeze. Third, a limited but not perfectly reliable form of emergency financing during a freeze--a "bailout"--may improve the terms of the trade-off and increase total ex ante value by instilling confidence in the creditor markets. Our conclusions highlight the endogenous interaction between risk from the asset and liability sides of the balance sheet. The Author 2011. Published by Oxford University Press on behalf of The Society for Financial Studies. All rights reserved. For Permissions, please e-mail: journals.permissions@oup.com. , Oxford University Press.
The Importance of Ignoring

Selective attention is often thought to entail an enhancement of some task-relevant stimulus or attribute. We discuss the perspective that ignoring irrelevant, distracting information plays a complementary role in information processing. Cortical oscillations within the alpha (8–14 Hz) frequency band have emerged as a marker of sensory suppression. This suppression is linked to selective attention for visual, auditory, somatic, and verbal stimuli. Inhibiting processing of irrelevant input makes responses more accurate and timely. It also helps protect material held in short-term memory against disruption. Furthermore, this selective process keeps irrelevant information from distorting the fidelity of memories. Memory is only as good as the perceptual representations on which it is based and on whose maintenance it depends. Modulation of alpha oscillations can be exploited as an active, purposeful mechanism to help people pay attention and remember the things that matter.
Performance and Cost Effectiveness of Caching in Mobile Access Networks

The paper considers the use of caching in mobile access networks and seeks to evaluate the optimal memory for bandwidth tradeoff at base station (BS), packet gateway (PGW) and a possible intermediate mobile cloud node (MCN). Formulas are derived for the hit rate under time varying popularity and for a novel cache insertion policy incorporating a pre-filter. The analytical model is applied first to demonstrate that reactive caching is not efficient for nodes with low demand due to the negative impact of content churn. This means BS or MCN caches must be managed proactively with popular content items pre-fetched under some centralized control. Quantifying the tradeoff at each level leads us to conclude that limited caching at BS and MCN levels brings significant savings while to store the vast majority of the content catalogue at the Internet edge at the PGW is clearly cost effective.
Function-Based Classification from 3D Data and Audio

We propose a novel scheme for fusion between two types of modalities to support function-based classification. While the first modality targets functional classification from sounds registered at impact, the second one aims classification of objects in 3D images. Using audio one can answer functional questions such as what is the material the analyzed objects are built of, if the objects are full or hollow, if they are heavy, and if they are rigidly linked to their supports. Audio based signatures are used to label parts of the object under analysis. Different parts of any object can be partitioned in generic multi-level hierarchical descriptions of functional components. Functionality, in the visual modality reasoning scheme, is derived from a large set of geometric attributes and relationships between object parts. These geometric properties represent labeling signatures to the primitive and functional parts of the analyzed classes. The fusion between both of the modalities relies on a shared cooperation among audio and visual signatures of the functional and primitive parts. The scheme does not require a-priori knowledge about any class. We tested the proposed scheme on a database of about one thousand different 3D objects. The results show high accuracy in classification
Towards the automatic generation of biomedical sources schema

Biologists and physicians need to access biological and medical data for their experimentations and researches. This information is available on the Internet and is scattered over many heterogeneous data sources. Collecting information is consequently tedious, time consuming and must be improved. To cope with this difficulty, our overall objective is to realize a mediator-based system to integrate heterogeneous biomedical data sources. This requires first an automatic generation of source schema, which is the goal of this work. For that, we describe an algorithm which is based on information extraction. It consists of the extraction of meta-information from each source to infer their schema. Our system enables users to access relevant and specific data, which are up-to-date. To solve the semantic heterogeneity of data sources, we are considering the creation of an ontology. Finally, the management of source evolution is discussed
Up-regulation of the Wnt, Estrogen Receptor, Insulin-like Growth Factor-I, and Bone Morphogenetic Protein Pathways in C57BL/6J Osteoblasts as Opposed to C3H/HeJ Osteoblasts in Part Contributes to the Differential Anabolic Response to Fluid Shear*

C57BL/6J (B6), but not C3H/HeJ (C3H), mice responded to mechanical loading with an increase in bone formation. A 30-min steady fluid shear of 20 dynes/cm2 increased [3H]thymidine incorporation and alkaline phosphatase activity and up-regulated the expression of early mechanoresponsive genes (integrin β1 (Igtb1) and cyclooxygenase-2 (Cox-2)) in B6 but not C3H osteoblasts, indicating that the differential mechanosensitivity was intrinsic to osteoblasts. In-house microarray analysis with 5,500 gene fragments revealed that the expression of 669 genes in B6 osteoblasts and 474 genes in C3H osteoblasts was altered 4 h after the fluid shear. Several genes associated with the insulin-like growth factor (IGF)-I, the estrogen receptor (ER), the bone morphogenetic protein (BMP)/transforming growth factor-β, and Wnt pathways were differentially up-regulated in B6 osteoblasts. In vitro mechanical loading also led to up-regulation of these genes in the bones of B6 but not C3H mice. Pretreatment of B6 osteoblasts with inhibitors of the Wnt pathway (endostatin), the BMP pathway (Noggin), or the ER pathway (ICI182780) blocked the fluid shear-induced proliferation. Inhibition of integrin and Cox-2 activation by echistatin and indomethacin, respectively, each blocked the fluid shear-induced up-regulation of genes associated with these four pathways. In summary, up-regulation of the IGF-I, ER, BMP, and Wnt pathways is involved in mechanotransduction. These four pathways are downstream to the early mechanoresponsive genes, i.e. Igtb1 and Cox-2. In conclusion, differential up-regulation of these anabolic pathways may in part contribute to the good and poor response, respectively, in the B6 and C3H mice to mechanical loading.
Interferon Regulatory Factor 3 Is a Cellular Partner of Rotavirus NSP1

ABSTRACT The rotavirus nonstructural protein NSP1 is the least conserved protein in the rotavirus genome, and its function in the replication cycle is not known. We employed NSP1 as bait in the yeast two-hybrid interaction trap to identify candidate cellular partners of NSP1 that may provide clues to its function. Interferon regulatory factor 3 (IRF-3) was identified as an NSP1 interactor. NSP1 synthesized in rotavirus-infected cells bound IRF-3 in a glutathione S-transferase pull-down assay, indicating that the interaction was not unique to the two-hybrid system. NSP1 of murine rotavirus strain EW also interacted with IRF-3. NSP1 deletion and point mutants were constructed to map domains important in the interaction between NSP1 and IRF-3. The data suggest that a binding domain resides in the C terminus of NSP1 and that the N-terminal conserved zinc finger is important but not sufficient to mediate binding to IRF-3. We predict that a role for NSP1 in rotavirus-infected cells is to inhibit activation of IRF-3 and diminish the cellular interferon response.
Delineation of Groundwater Prospect Zones in Hard Rocks Using Remote Sensing and GIS - A Case Study from Rajasthan

Groundwater resources are extremely important in Rajasthan as almost the entlre region lies in arid/semi-arid tropics especially in the hard rock areas. The recharge being less due to scanty rainfall. The study has revealed the advantages of remotely sensed data in identifying the prospects of groundwater in geologically and structurally complex terrain. Remote Sensing (RS) and Geographic Information System (GIS) is a dynamic tool for facilitating the generation and use of thematic information; has been applied to know the groundwater potentiality of the Upper Kali Sindh Chauli watershed in the Jhalawar district of Rajasthan, India. The role of different parameters namely geology, geomorphology, structures, lineaments, slope, land use and land cover, digital elevation model, etc. have been emphasized for delineation of the groundwater potential Zones. The groundwater potentia1 zone map was prepared based on the visual interpretation of satellite Geocoded data of IRS-ID, LISS III, FCC and the topographic maps on 1:50,00 scale. In thc study area major lineaments are identified from the satellite data interpretation, which are surface manifestation of some structural features in the bedrocks as fractures and joints. The study area is divided into mint-watershed and micro-watershed on the basis of area and stream channels. Groundwater prospects are controlled by the lineaments/joints, as the lithology is not much suitable for groundwater development. Ultimate groundwater prospecting map indicatea variable groundwater potentiality in the area i.e. good, moderate, limited , and poor and based on these categorization the depth of wells are recommended for drilling.
A Comparison between International Obesity Task Force and Center for Disease Control References in Assessment of Overweight and Obesity Among Adolescents in Babol, Northern Iran

Objective: This study compares the Center for Disease Control (CDC) and International Obesity Task Force (IOTF) references in assessment of overweight and obesity among Iranian adolescents. Methods: The data of this study was drawn from a cross sectional study of a representative sample of 1200 adolescents aged 12-17 years in Babol, northern Iran. A standard procedure was used to measure height and weight and the body mass index was calculated. Each subject was classified as overweight and obese based on IOTF cut off values of BMI and CDC references BMI percentile sets by age and sex. The kappa coefficients were estimated for the degree of agreement. Results: In assessment of obesity/overweight prevalence, the CDC and IOTF references produced a similar estimate by age group and sex. The maximum differences was about 1% and the kappa coefficients was 0.96 to 1 (P = 0.001). While for assessment of obesity, the CDC reference produced slightly a higher rate of obesity and the difference in prevalence between the two sets of references was ranged from 1.4% to 3.2% with kappa coefficients: 0.90 to 0.70 (P = 0.001) depending on the age group and sex and a greater difference was observed among younger age group. Conclusion: The findings suggest an excellent agreement between the TOTF and CDC references in assessment of overall overweight/obesity prevalence among adolescent boys and girls. While in assessment of obesity prevalence alone the degree of agreement between the two sets of references slightly diminished. Overall, the two references are comparable and the agreement varies a little with respect to age and sex.
Association Between Folate, Vitamin B12 and Cognitive Performance in Demented Elderly.

Dementia is prevalent among elderly people. As the world population ages, it is projected that the number of people affected by dementia may triple in the next 50 years. Over the last two decades, research has focused on identifying potentially modifiable risk factors in development and progression of dementia, such as vitamin B12 and folate. Results concerning the effects of low folate and vitamin B12 on cognitive performance are mixed. The main objective of the present study was to investigate the effects of vitamin deficiency on cognitive functioning in a clinical sample of elderly individuals with cognitive problems using a comprehensive neuropsychological assessment. A retrospective chart-review was performed on the 102 records of patients from the Geriatrics Clinic at the University of North Texas Health Science Center who presented with cognitive deficits. Charts were reviewed to obtain data on vitamin supplementation, vitamin status, history of chronic conditions and other biochemical data. The available database was used to obtain data on neuropsychological assessment. The study demonstrated mild association between vitamin B12 and folate status and cognitive deficits. There appeared to be a higher cut-off level that is above the traditionally used levels for vitamin B12 and folate deficiency concentrations at which cognitive deficits became more pronounced. Clinical applications, limitations and suggestions for future research were discussed.
Efficacy and safety of the Ahmed glaucoma valve implant in Chinese eyes with complicated glaucoma

AIMS To evaluate the efficacy and safety of the Ahmed glaucoma valve implant in Chinese eyes with complicated glaucomas. METHODS This retrospective study reviewed the final intraocular pressure, visual outcome, and incidence of complications in all patients with the Ahmed glaucoma valve implant performed at the Prince of Wales Hospital, Hong Kong, between June 1996 and November 1998. RESULTS A total of 65 eyes from 60 patients were treated with the Ahmed glaucoma implant. At a mean follow up (SD, median) of 21.8 (9.2, 28.0) months (range 6–37 months), the mean intraocular pressure was reduced from 37.0 (SD 12.1) mm Hg before the implant surgery to 16.1 (12.4) mm Hg at the last follow up after surgery. The success rate of intraocular pressure control of <22 mm Hg was achieved in 73.8% of operated eyes. Transient postoperative hypotony with shallow anterior chamber occurred in 10.8% of cases. The most common postoperative complication was the formation of encapsulated bleb (24.6%). CONCLUSIONS The Ahmed glaucoma valve implant appears to be effective and relatively safe for treating complicated glaucomas in Chinese eyes. The success rate is comparable with those reported in non-Asian eyes. Formation of postoperative encapsulated bleb is, however, more commonly encountered.
Relating solute and gas dispersion in granite at different transport velocities

Dispersion is a key process controlling gas and solute transport in porous media. Performing dispersion tracer tests for solutes is generally much more time consuming than for gases. Ability to estimate solute dispersion based on gas dispersion data is therefore advantageous. The aim of this study was to investigate the relationship between solute and gas dispersion in selected porous media to develop a procedure for estimating solute dispersion from gas dispersion. Solute (NaCl) and gas (O2) breakthrough curves were measured at different fluid (water and gas) velocities (at identical Reynolds numbers for the two fluids). A commercial crushed granite, available in multiple particle sizes, was used as porous medium. NaCl and O2 dispersion coefficients were determined from measured breakthrough data and used to calculate solute and gas dispersivity as a function of particle size (Dm) and particle size range (R). Both solute and gas dispersivity increased with increasing R, and decreasing Dm. A simple relationship between dispersivities was found.
Cross-talk between Language Processes and Overt Motor Behavior in the First 200 msec of Processing

A recently emerging view sees language understanding as closely linked to sensory and motor processes. The present study investigates this issue by examining the influence of processing action verbs and concrete nouns on the execution of a reaching movement. Fine-grained analyses of movement kinematics revealed that relative to nouns, processing action verbs significantly affects overt motor performance. Within 200 msec after onset, processing action verbs interferes with a concurrent reaching movement. By contrast, the same words assist reaching movement when processed before movement onset. The cross-talk between language processes and overt motor behavior provides unambiguous evidence that action words and motor action share common cortical representations and could thus suggest that cortical motor regions are indeed involved in action word retrieval.