Miracle-dz/UniFAR-FTUs huggingface dataset:
Clinical symptoms of adult metachromatic leukodystrophy and arylsulfatase A pseudodeficiency.

OBJECTIVE To determine the clinical symptoms in adult metachromatic leukodystrophy and in adult pseudodeficiency for arylsulfatase A. DESIGN Case series. SETTING University hospital. PATIENTS Twenty-five adult patients with very low arylsulfatase A activity. RESULTS In 13 patients, a diagnosis of adult metachromatic leukodystrophy was made. The main symptoms were dementia, behavioral abnormalities, ataxia, and polyneuropathy. In 12 patients, a diagnosis of arylsulfatase A pseudodeficiency was made. No characteristic clinical syndrome could be detected in these patients. CONCLUSIONS Adult metachromatic leukodystrophy is a progressive metabolic disease with symptoms of demyelination of the central and peripheral nervous systems. Diagnosis must be confirmed by determination of arylsulfatase A activity and accumulation of sulfatides. Pseudodeficiency for arylsulfatase A can be confirmed or excluded by means of DNA analysis.
Developing a theory of auditing behavior in the electronic business environment

The rapid adoption of e-business technology has led to the disappearance of paper documents. Auditors now receive electronic information from their clients instead of the traditional paper-based information. Current auditing literature acknowledges that auditors have concerns about the competency and sufficiency of electronic information. Hence, this study endeavors to develop a theory of auditing behavior in the e-business environment by exploring those factors that may influence the assessment of electronic evidence as “sufficient competent evidential matter. ” The recommended solutions, presented in the literature, to alleviate the auditing concerns are technology-focused solutions. While the technology-focused solutions may address some of the concerns, they are inadequate because e-business technology does not function independently of the humans who interact with it. Therefore, the consideration of certain social, behavioral and contextual factors in seeking resolution to the auditing concerns is essential. This study seeks to answer the question of what factors influence auditors' concerns regarding evidential matter in the e-business environment, as well as the questions of how and why these factors exert influence in the ways that they do. A grounded theory research design is utilized to collect and evaluate research data to formulate a theory of auditing behavior. Three types of auditing behavior emerge from the data; these behaviors are consistent with the technological imperative, social/organizational imperative and socio-technical imperative schools of thought. The study identifies six factors that influence auditors' behavior toward electronic information as sufficient, competent evidential matter. Consequently, the study theorizes that the auditor's behavior toward electronic information as audit evidential matter is influenced by his or her perceived risk associated with the institutional factors of audit objective, professional orientation (training), professional standards, professional responsibility, professional reputation, and legal liability.
Photo‐degradation of melatonin: influence of argon, hydrogen peroxide, and ethanol

Abstract: When organic compounds are irradiated with UV light at 254 nm, part of their covalent bonds can dissociate if the compound absorbs light at that wavelength. Therefore, photo‐degradation depends strongly on the wavelength used. The energy of a light quanta at 254 nm amounts to approximately 110 kcal/mol quanta, which is in many cases higher than the binding energy of a variety of covalent bonds. As a consequence, the absorbing molecule is degraded. As melatonin absorbs light at 254 nm, this compound is vulnerable to UV light. In order to minimize undesired effects of other absorbing substances, we used as solvent mostly pure water and analyzed the influence of λirr = 254 nm on the disappearance of the educt (melatonin) as well as on the appearance of products derived from melatonin in the presence of oxygen, argon, hydrogen peroxide, and ethanol by UV–VIS spectroscopy and high‐performance liquid chromatography (HPLC) technique. N1‐acetyl‐N2‐formyl‐5‐methoxykynuramine (AFMK) appears to be the main, but obviously not the only product of photo‐degradation of melatonin, independently of whether the system contains oxygen or not. If the system contains additionally hydrogen peroxide, a very strong oxidant, the hydroxyl radical (•OH), is formed. Under such conditions, melatonin is not solely photo‐degraded but also attacked by the formed •OH which interact similarly with the formed main photo‐product AFMK. Ethanol, as a potent scavenger of •OH, efficiently blocks the effect of this aggressive radical even at low concentrations of that scavenger (0.1% v/v) but is less effective in preventing photo‐degradation of melatonin.
Conscientiousness, Agreeableness, Medication Beliefs and Locus of Control in Chronic Kidney Disease Treatment Adherence

Adherence to treatment regimens were investigated in eighty-three individuals with Chronic Kidney Disease. Personality traits of conscientiousness and agreeableness, necessity and concern medication beliefs and health locus of control were examined. This quasiexperimental, cross-sectional, correlational, quantitative and qualitative design consisted of an anonymous online survey with measures including Medication Adherence Questionnaire, two dimensions of the Big Five Inventory, the Belief about Medicines Questionnaire and the Multidimensional Health Locus of Control. Results demonstrated poor adherence rates and personality traits can be associated with adherence behaviours. Higher medication concern beliefs were related to lower medication adherence. No significant findings were reported for internal locus of control, differences were reported for the chance dimension of external locus of control concurring with previous research. No associations were found between medication adherence and lifestyle adherence. Personality traits, medication beliefs and personal control beliefs are important for assessments, interventions and developing coping behaviours for adherence.
Determinants of online shopping intention

The internet has become a key medium for the purchase of products and services in virtual markets and has effectively linked all countries and business. It has been estimated that the internet market is worth $300 billion in 1995. Through the internet, electronic commerce offers a tremendously wide variety of electronic business opportunities. One of them is online shopping which has become the third most popular internet activity after e-mail or instant messaging and web browsing. Malaysia is ranked 17th among 27 countries across Europe, Asia-Pacific and North America in terms of the percentage of internet users shopping online. Even though this method has started to win hearts of Malaysian consumers, the factors influencing the willingness to shop online are still unknown. Thus, the general objective of the study is to examine the factors that influence consumer's online shopping intention (dependent variable). The main independent variables are demographic, trusts, quality and loyalty to website visited. A 100 percent response rate was obtained from students sampled randomly. Findings indicate that quality and loyalty contributed 26.8 percent (R2 = 0.268) and 6.1 percent (R2 = 0.061) respectively to the variance in online shopping intention. Implications of the study are discussed.
Theoretical and Empirical Analysis of Common Factors in a Term Structure Model

This paper studies dynamical and cross-sectional structures of bonds, typically used as riskfreeassets in mathematical finance. After reviewing a mathematical theory on commonfactors, also known as principal components, we compute empirical common factors for 10US government bonds (3month, 6month, 1year, 2year, 3year, 5year, 7year, 10year, 20year,and 30year) from the daily data for the period 1993-2006 (data for earlier period is notcomplete) obtained from the official web site www.treas. gov. We find that the principalcommon factor contains 91% of total variance and the first two common-factors contain 99.4%of total variance. Regarding the first three common factors as stochastic processes, we findthat the simple AR(1) models produce sample paths that look almost indistinguishable (incharacteristic) from the empirical ones, although the AR(1) models do not seem to pass thenormality based Portmanteau statistical test. Slightly more complicated ARMA(1,1) modelspass the test. To see the independence of the first two common factors, we calculate theempirical copula (the joint distribution of transformed random variables by their marginaldistribution functions) of the first two common-factors. Among many commonly used copulas(Gaussian, Frank, Clayton, FGM, Gumbel), the copula that corresponds to independentrandom variables is found to fit the best to our empirical copula. Loading coefficients (that ofthe linear combinations of common factors for various individual bonds) are briefly discussed.We conclude from our empirical analysis that yield-to-maturity curves of US governmentbonds from 1993 to 2006 can be simply modelled by two independent common factors which,in turn, can be modelled by ARMA(1,1) processes.
Hot Press Joining Optimization of Polyethylene to Aluminium Alloy AA6061-T6 Lap Joint Using Design of Experiments

Abstract. The hot press method is used to join a poly-ethylene plate of thickness 4 mm with an aluminium alloy AA6061-T6 plate of thickness 1.5 mm. A preanodizing process is crucial for the success of such joint. The anodizing was performed under a current density of 200 A/m2, temperature 24°C, sulphuric acid 5 wt. % and for a 60 minutes. Three period parameters were considered in the hot pressing process; temperature: 115 and 135°C, pressure: 2, 6, and 10 bar and pressing time: 1, 2, 3, 4 and 5 minutes. The joint specimens are tested by shear tensile test, microstructure, energy dispersive spectrometry, differential scanning calorimetric and thermo grvanometric analysis. The results of shear tensile were analyzed using design of experiments. Minimum and maximum shear tensile force were found at temperature T = 115°C where; F min = 680N and F max = 2800N. From Pareto chart, the applied pressure had the most significant effect on the shear tensile force of the joint specimens compared to temperature and joining time. The main effect plot showed that optimum value of shear tensile force was obtained at joining conditions of T = 135°C, P = 6 bar and t = 3 minutes. The tested specimens exhibited an interfacial shear failure, extension and necking in the region of polymer with a ductile fracture. The mechanical interlocking of polymer element in the anodizing surface is approximately 15 μm. The melting point and glass transmission temperature of PE are decreased after the hot press.
Long-Acting Reversible Contraception in the Pediatric Emergency Department: Clinical Implications and Common Challenges

Abstract Long-acting reversible contraception (LARC) is recommended as first-line contraception for adolescents and young adults. As the use of LARC increases, pediatric emergency medicine clinicians should be able to recognize different types of LARC and address their common adverse effects, adverse reactions, and complications. This continuing medical education activity provides an overview of LARC and will assist clinicians in the evaluation and management of patients with LARC-associated complaints.
Predictors of Well Developed Coronary Collateral Circulation in Patients with Stable Angina Pectoris

Objective: We aimed to demonstrate the predictors of Coronary Collateral Development (CCD) in patients with Stable Angina Pectoris (SAP). Methods: We prospectively enrolled 52 patients with at least 90% coronary stenosis. Their demographic, anthropometric and clinical data as well as hematological and biochemical parameters, medications, Ejection Fraction (EF) and perfusion index values and angiographic findings were used for determining the predictors for CCD. The Rentrop score (between 0 and 3) was used for the angiographic categorization and patients in Rentrop grades 0 and 1 were classified as poor CCD, and patients in Rentrop grades 2 and 3 were classified as well CCD. Results: We found moderate negative correlation between the Rentrop score and ejection fraction (r=-0,469, p<0.001). There was also positive correlation between the Gensini score (GS) and the Rentrop score (r=0.627, p<0.001). We made Classification and Regression Tree Model (C&RT) to define best predictors of CCD and found that EF (cut off 55%) and GS (cut off 41%) together constitute a useful prediction algorithm. Furthermore an advanced research was conducted to develop another algorithm to forecast coronary collateral development prior to angiography. The analysis was repeated after the extraction of angiographic data from the first C&RT model. It was concluded that EF (cut off 55%) and mean platelet volume (MPV) (cut off 9 fl) can be used in the second algorithm. Conclusions: CCD is negatively related to EF and positively related to GS. EF and MPV together constitute a simple and cost effective algorithm to predict the CCD before the angiography. However, GS and EF seem to be the best predictors of CCD among the whole variables.
Flavobacterium johnsoniae PorV Is Required for Secretion of a Subset of Proteins Targeted to the Type IX Secretion System

ABSTRACT Flavobacterium johnsoniae exhibits gliding motility and digests many polysaccharides, including chitin. A novel protein secretion system, the type IX secretion system (T9SS), is required for gliding and chitin utilization. The T9SS secretes the cell surface motility adhesins SprB and RemA and the chitinase ChiA. Proteins involved in secretion by the T9SS include GldK, GldL, GldM, GldN, SprA, SprE, and SprT. Porphyromonas gingivalis has orthologs for each of these that are required for secretion of gingipain protease virulence factors by its T9SS. P. gingivalis porU and porV have also been linked to T9SS-mediated secretion, and F. johnsoniae has orthologs of these. Mutations in F. johnsoniae porU and porV were constructed to determine if they function in secretion. Cells of a porV deletion mutant were deficient in chitin utilization and failed to secrete ChiA. They were also deficient in secretion of the motility adhesin RemA but retained the ability to secrete SprB. SprB is involved in gliding motility and is needed for formation of spreading colonies on agar, and the porV mutant exhibited gliding motility and formed spreading colonies. However, the porV mutant was partially deficient in attachment to glass, apparently because of the absence of RemA and other adhesins on the cell surface. The porV mutant also appeared to be deficient in secretion of numerous other proteins that have carboxy-terminal domains associated with targeting to the T9SS. PorU was not required for secretion of ChiA, RemA, or SprB, indicating that it does not play an essential role in the F. johnsoniae T9SS.
Interferon‐lambda (IFN‐λ) induces signal transduction and gene expression in human hepatocytes, but not in lymphocytes or monocytes

This study compared the ability of IFN‐α and IFN‐λ to induce signal transduction and gene expression in primary human hepatocytes, PBLs, and monocytes. IFN‐α drug products are widely used to treat chronic HCV infection; however, IFN‐α therapy often induces hematologic toxicities as a result of the broad expression of IFNARs on many cell types, including most leukocytes. rIFN‐λ1 is currently being tested as a potential alternative to IFN‐α for treating chronic HCV. Although IFN‐λ has been shown to be active on hepatoma cell lines, such as HepG2 and Huh‐7, its ability to induce responses in primary human hepatocytes or leukocytes has not been examined. We found that IFN‐λ induces activation of Jak/STAT signaling in mouse and human hepatocytes, and the ability of IFN‐λ to induce STAT activation correlates with induction of numerous ISGs. Although the magnitude of ISG expression induced by IFN‐λ in hepatocytes was generally lower than that induced by IFN‐α, the repertoire of regulated genes was quite similar. Our findings demonstrate that although IFN‐α and IFN‐λ signal through distinct receptors, they induce expression of a common set of ISGs in hepatocytes. However, unlike IFN‐α, IFN‐λ did not induce STAT activation or ISG expression by purified lymphocytes or monocytes. This important functional difference may provide a clinical advantage for IFN‐λ as a treatment for chronic HCV infection, as it is less likely to induce the leukopenias that are often associated with IFN‐α therapy.
Cutting Edge: Lectin-Like Transcript 1 Is a Ligand for the CD161 Receptor1

Human NK cells and subsets of T cells or NKT cells express the orphan C-type lectin receptor CD161 (NKR-P1A) of unknown function. In contrast to rodents that possess several NKR-P1 genes coding for either activating or inhibitory receptors, the nature of signals delivered by the single human NKR-P1A receptor is still to be clarified. In this article, we show that the lectin-like transcript 1 (LLT1) molecule is a ligand for the CD161 receptor. Engagement of CD161 on NK cells with LLT1 expressed on target cells inhibited NK cell-mediated cytotoxicity and IFN-γ secretion. Conversely, LLT1/CD161 interaction in the presence of a TCR signal enhanced IFN-γ production by T cells. These findings identify a novel ligand/receptor pair that differentially regulate NK and T cell functions.
An individual patient data meta-analysis of adjuvant therapy with carmofur in patients with curatively resected colon cancer.

BACKGROUND Oral carmofur, either as a single or in combination with other chemotherapeutic agents, has been used as adjuvant chemotherapy for curatively resected colon cancer patients. Past trials and meta-analyses indicate that it is somewhat effective in extending survival of patients with this cancer. The objective of this study was to perform a reappraisal of randomized clinical trials conducted in this regard. METHODS We designed an individual patient-based meta-analysis of relevant clinical trials to examine the benefit of oral carmofur for curatively resected colon cancer in terms of overall survival (OS) and disease-free survival (DFS). RESULTS We analyzed individual patient data of three randomized clinical trials, which met the predetermined inclusion criteria. These three trials had a combined total of 2152 patients, carmofur as adjuvant chemotherapy compared with surgery-alone, 5 years follow-up, intention-to-treat-based analytic strategy and similar end points (OS and DFS). In a pooled analysis, 5 year OS rates were 80.4 and 76.4%, and 5 year DFS rates 76.9 and 71.0%, respectively, in carmofur and surgery-alone group. Oral carmofur had significant advantage over surgery-alone in terms of both OS [pooled hazard ratio, 0.82; 95% confidence interval (CI) = 0.68-0.99; P = 0.043] and DFS (pooled hazard ratio, 0.77; 95% CI = 0.65-0.91; P = 0.003). CONCLUSIONS This individual patient-based meta-analysis demonstrated that oral carmofur significantly improves both OS and DFS in patients with curatively resected colon cancers.
Increased Risk of Dementia Among Patients With Pulmonary Tuberculosis

Objective: We investigated and compared the risk of dementia development in a cohort of patients with tuberculosis (TB). Methods: The study involved 6473 patient with newly diagnosed TB, and each patient was randomly frequency matched with 4 people without TB based on age, sex, and index year. The risk of dementia development was analyzed using Cox proportional hazards regression. Results: Among the patients with TB, the overall risk of developing dementia was 1.21-fold significantly higher than the non-TB cohort. In the stratified analysis of dementia risks, only the patients with TB who were male or 50 to 64 years of age exhibited a significantly higher risk of dementia development compared with those without TB. An analysis of the follow-up duration revealed that patients with TB had a 1.78-fold increased risk within 1 year of follow-up. Conclusion: Patients with TB have a significantly higher risk of developing dementia than that of the general population.
Health effects of radon: A review of the literature

Purpose: Radon is natural radioactive noble gas that can be found in soil, water, outdoor and indoor air. Exposure to radon accounts for more that 50% of the annual effective dose of natural radioactivity. The purpose of the current review is to summarize recent literature and evaluate the weight of evidence on the adverse health effects of radon. Conclusions: Radon is an established human lung carcinogen based on human epidemiological data supported by experimental evidence of mutagenesis studies in cell culture and laboratory animals. Extrapolation from cohort studies on miners suggested that radon is the second leading cause of lung cancer death after tobacco smoke. The majority of studies on the relationship between radon and other types of cancers showed weak or no association. Low levels of radon can be found in drinking water; however, radon released during water usage adds small quantities to indoor radon concentration. Studies showed that the risk of stomach cancer and other gastrointestinal malignancies from radon in drinking water is small. Studies of the genetic and cytogenetic effects of indoor radon yielded equivocal results; while radon exposure in miners induces gene mutations and chromosomal aberrations. Numerous in vitro cytogenetic studies demonstrated that radon induces different types of genetic and cytogenetic damage that is likely to play a role in radon lung carcinogenesis.
Qualitative Analysis and Implications of Chinese Expatriates' Food Consumption Behavior

Abstract A nationwide web-based survey of Chinese students' food consumption behavior in the U.S. is conducted to investigate how food consumption in China may change as Western influences and product availability increase over time. Qualitative data on consumption of milk, beef, chicken, pork, bread, rice, pizza, hamburger, juice, and vegetables is collected. Factors that affect Chinese expatriates' consumption changes are determined for each product category. Perceived taste differences between the U.S. and China, perceived health and nutrition benefits, consumption frequency, price and age have a significant effect on the consumption of most food products. A marginal effect analysis reveals that factors affect different food products to different degrees. For example, length of stay in the U.S. (i.e., time) affects consumption changes of pizza, hamburgers, and beef more than that of other products. Of the 10 product categories investigated, fruit juice and milk appear to be the most promising short-term opportunities in Chinese urban areas.
Views on potential methods for raising environmental awareness in developing countries: a study on social responsibility engagement in Liberia

Maintaining environmental standards is a challenging task that requires knowledge and an understanding of the impacts of unethical decisions on human well-being, especially in developing countries. Lack of knowledge, the pursuit of profits, and other factors are preventing leaders from making ethical decisions, which could have a direct and indirect influence on health, technology, and the economy – problems that are particularly acute in poor and developing countries. Some progress can act as a smokescreen for managers or leaders, who fail to see the general environmental issues that still exist and that should be of primary concern for governments, policymakers, and local communities. Climate change and some of the other major environmental challenges that have emerged over the past few decades represent major social responsibility concerns for developing communities. Abuses of power by key decision-makers have been and continue to be an endemic problem in many developing countries and beyond. The present study seeks to understand what affects the thought processes of leaders who make environmental management decisions, using Liberia as an example.