Miracle-dz/UniFAR-FTUs huggingface dataset:
Creutzfeldt-Jakob Disease in Austria: An Autopsy-Controlled Study

Background: Definite diagnosis of prion diseases or transmissible spongiform encephalopathies (TSEs) requires neuropathology, usually at autopsy. Epidemiology of human TSEs has relied on definite as well as ‘probable’ cases in which neuropathological confirmation is lacking, usually because of low autopsy rates in most countries. Methods: In Austria, an active surveillance program for human prion diseases was established in 1996. Since then, more than 900 referrals were analyzed. Postmortem investigation of brain tissue is mandatory in every suspect case of TSE. Thus, epidemiological data on TSEs from Austria may serve as autopsy-controlled reference for countries with lower autopsy rates. Results: The total number of TSE cases in Austria since 1969 is 206. The average yearly mortality for the active surveillance period from 1996 to 30 June 2006 is 1.39 per million, with the highest rates for Vienna (2.37) compared with other provinces. Eighty-five percent of definite TSEs were classified as sporadic Creutzfeldt-Jakob disease (sCJD). We observed a significant linear increase in the mean age at death of 0.6 years per calendar year. Clinical diagnostic surveillance criteria had a sensitivity and specificity of 82.7 and 80.0% for probable CJD, respectively, and a positive predictive value of 80.5% for probable and 38.9% for ‘possible’ CJD. Alternative neuropathological diagnoses in suspect cases included Alzheimer’s disease with or without Lewy body pathology, vascular encephalopathy, metabolic encephalopathies and viral or limbic encephalitis. Conclusion: The steady increase in mortality rates, especially in old age groups, most likely reflects improved case ascertainment due to active surveillance causing higher awareness of the medical community. In comparison with other European countries, it is reassuring to note that the overall death rate of TSEs does not differ from the Austrian autopsy-controlled data, thus confirming the value of clinical surveillance criteria.
Levodopa and the progression of Parkinson's disease.

BACKGROUND Despite the known benefit of levodopa in reducing the symptoms of Parkinson's disease, concern has been expressed that its use might hasten neurodegeneration. This study assessed the effect of levodopa on the rate of progression of Parkinson's disease. METHODS In this randomized, double-blind, placebo-controlled trial, we evaluated 361 patients with early Parkinson's disease who were assigned to receive carbidopa-levodopa at a daily dose of 37.5 and 150 mg, 75 and 300 mg, or 150 and 600 mg, respectively, or a matching placebo for a period of 40 weeks, and then to undergo withdrawal of treatment for 2 weeks. The primary outcome was a change in scores on the Unified Parkinson's Disease Rating Scale (UPDRS) between baseline and 42 weeks. Neuroimaging studies of 142 subjects were performed at baseline and at week 40 to assess striatal dopamine-transporter density with the use of iodine-123-labeled 2-beta-carboxymethoxy-3-beta-(4-iodophenyl)tropane ([123I]beta-CIT) uptake. RESULTS The severity of parkinsonism increased more in the placebo group than in all the groups receiving levodopa: the mean difference between the total score on the UPDRS at baseline and at 42 weeks was 7.8 units in the placebo group, 1.9 units in the group receiving levodopa at a dose of 150 mg daily, 1.9 in those receiving 300 mg daily, and -1.4 in those receiving 600 mg daily (P<0.001). In contrast, in a substudy of 116 patients the mean percent decline in the [123I]beta-CIT uptake was significantly greater with levodopa than placebo (-6 percent among those receiving levodopa at 150 mg daily, -4 percent in those receiving it at 300 mg daily, and -7.2 percent among those receiving it at 600 mg daily, as compared with -1.4 percent among those receiving placebo; 19 patients with no dopaminergic deficits on the baseline scans were excluded from the analysis) (P=0.036). The subjects receiving the highest dose of levodopa had significantly more dyskinesia, hypertonia, infection, headache, and nausea than those receiving placebo. CONCLUSIONS The clinical data suggest that levodopa either slows the progression of Parkinson's disease or has a prolonged effect on the symptoms of the disease. In contrast, the neuroimaging data suggest either that levodopa accelerates the loss of nigrostriatal dopamine nerve terminals or that its pharmacologic effects modify the dopamine transporter. The potential long-term effects of levodopa on Parkinson's disease remain uncertain.
Cheese is a reliable alternative meal for solid-phase gastric emptying study

We evaluated the labeling stability of several alternative meals that could be used to perform solid-phase gastric emptying study. Cooked egg whites labeled with technetium-99m sulfur colloid served as a control. Packaged instant oatmeal and instant mashed potatoes were prepared by adding hot water. Cheddar cheese was melted. Peanut butter was added to bread. The different meals were mixed with technetium-99m sulfur colloid (2.2–3.7 MBq), chopped into small pieces and placed in a glass tube containing gastric juice. Four samples of each meal were analyzed after 1 and 4 h of agitation with a 3-D rotator (two samples per time point). The meal samples were washed with 2 ml of saline and filtered using a blood transfusion filter. The activity in each sample before and after filtering was assayed in a dose calibrator. The percentage of initial radioactivity remaining with the meal of admixture with gastric juice was measured and the average of the two samples was taken. The percentage of activity bound to the solid phase was 98.2±1.9, 95.6±1.1, 62.1±1.7, 41.8±0.6, and 74.5±3.8% at 1 h and 98.5±1.0, 95.8±2.6, 77.2±6.8, 55.5±3.4 and 40.2±22.1 at 4 h for egg whites, cheese, oatmeal, mashed potatoes and peanut butter respectively. For egg whites and cheese, there was no significant difference between the values at 1 and 4 h (P>0.8). Cheddar cheese provides an alternative meal for assessing solid gastric emptying in children comparable to egg whites. Oatmeal and mashed potatoes had low and variable labeling stability and are not recommended. In view of the significant proportion of pediatric patients who refuse to eat scrambled eggs or have allergy to eggs, the availability of other meal choices is essential. The versatility of cheddar cheese, which can be added to macaroni or as a topping on pizza, makes it a useful alternative to labeled eggs.
No evidence of telomere length attrition in newborns from high level natural background radiation areas in Kerala coast, south west India

Abstract Purpose: The tandemly repeated hexamers (TTAGGG)n present at the telomeric ends protect the human genome from a variety of environmental exposures including ionizing radiation. In order to find out the effect of chronic low dose radiation exposure, we have determined telomere length among newborns from high level natural radiation areas (HLNRA) of the Kerala coast in South west India. Methodology: Umbilical cord blood samples were collected from 128 newborns from HLNRA and 43 newborns from normal level natural radiation areas (NLNRA) and genomic DNA was isolated using a salt precipitation method. The mean telomere length was determined using SYBR green-based real time quantitative Polymerase chain reaction (qPCR), where telomere gene specific (T) and single copy gene specific (S) primers were used. The average of telomere versus single copy gene (T/S) ratio was calculated which was proportional to telomere length of each individual. Results: The mean relative telomere length was found to be 1.03 ± 0.01 (95% CI, 0.99–1.05) and 1.10 ± 0.03 (95% CI, 1.04–1.17) in HLNRA and NLNRA newborns, respectively (P > 0.05). Based on the level of background radiation dose, samples were divided into four groups, i.e., NLNRA: < 1.50 mGy/year and three HNLRA groups: 1.51–3.00 mGy/year, 3.01–5.00 mGy/year, and > 5.00 mGy/year. The mean relative telomere length in these groups were found to be 1.10 ± 0.03 (95% CI, 1.03–1.17), 0.98 ± 0.01 (95% CI, 0.95–1.01), 1.05 ± 0.02 (95% CI, 1.01–1.10) and 1.07 ± 0.03 (95% CI, 1.04–1.10), respectively. No significant difference was observed between the mean telomere length of male and female newborns. Conclusions: The elevated level of natural chronic background radiation prevailing in Kerala coast did not show any significant effect on telomere length of newborns. To our knowledge, this is the first report on newborn telomere length from a high level natural radiation area.
Anomalies in contingent valuation, plausible empirical explanations

Environmental valuation is an important analytical tool for policy decisions concerning the environment. The Contingent Valuation (CV) method is a widely employed tool for assessing non use values of natural resources. The method is however subject to critics concerning anomalies. The most important anomaly is insensitivity to scope. As a consequence, a great effort has been made in the literature for explaining this controversy. Based on this extensive work, I provide new alternative explanations not previously explored that help us understanding motives causing insensitivity to scope. With this aim, empirical approaches which explore determining reasons for insensitivity to scope are conducted here. A meta-analysis on willingness to pay estimates for wildlife and ecosystem goods provides evidence of the effect information about the amount of the good has in sensitivity to scope. Moreover, use and non use values are not valued the same way. Thee case studies concerning a high use value species and a high non use value species are conducted where high use values species are sensitive to scope. Additionally, other factors such as the uniqueness of the good, ethical motivations, respondent"s roles or the expected values associated to a recovery level are seen to affect scope test results.
Acute Hemorrhagic Leukoencephalitis: Recovery and Reversal of Magnetic Resonance Imaging Findings in a Child

A case of acute hemorrhagic leukoencephalitis (AHLE) in a 6-year-old girl is reported. The presentation was typical for acute hemorrhagic leukoencephalitis, with acute onset of a rapidly progressive neurologic disorder with asymmetric involvement of brain, with polymorphonuclear predominant peripheral leukocytosis and cerebrospinal fluid pleocytosis. Cerebrospinal fluid findings not previously reported included elevation of IgG and the presence of myelin basic protein. Additional previously unreported findings were striking abnormalities on magnetic resonance imaging (MRI) of the brain, in contrast to normal findings on computed tomography (CT). The child was treated with high dose intravenous steroids and made a full recovery, with a parallel disappearance of all of her cerebrospinal fluid abnormalities and almost all of her abnormalities on MRI. Detailed examination of cerebrospinal fluid and MRI of brain should facilitate early diagnosis in other cases of suspected acute hemorrhagic leukoencephalitis and high-dose steroid therapy may lead to improved clinical outcomes. (J Child Neurol 1997;12:448-454).
Elucidating the role of conformity in innovative smartphones

Adoption of innovative products such as smartphones may be due to a mixture of experienced considerations and psychological perceptions. By synthesising adoption theories through reinforcement of the role of conformity. This study explores user intention to adopt innovative smartphones in the Taiwan telecommunication market. The survey data is analysed using structural equation modelling which is based on the constructs of ease of use, compatibility, and relative advantage, analysis results indicate that informational conformity and normative conformity are positively related to the intention to use smartphones. Additionally, early adopters and early majority users have a higher likelihood of conformity driven by informational influence, while the late majority users display a higher tendency towards normative conformity. The results of this study significantly contribute to efforts of mobile communication operators to anticipate and predict a customer's intention to adopt smartphones, thus provides further insight into the development of marketing strategies for smartphones.
Nursing work environment in Saudi Arabia.

AIM The purpose of this study was to assess the work environment as perceived by nurses in a large tertiary hospital in Saudi Arabia. BACKGROUND The quality of patient care services has been associated with the quality of work environment of nurses. It is therefore important to assess the work environment in order to acquire baseline data and enable the institution to benchmark their status from established quality standards. METHOD This study used a descriptive survey with 1007 staff nurses across service units of a 1000-bed government-operated hospital. The American Association of Critical-Care Nurses (AACN) Healthy Work Environment Assessment Questionnaire was used for data collection. Scores were aggregated and interpreted. RESULT Effective decision making, authentic leadership, appropriate staffing, true collaboration, skilled communication and meaningful recognition were rated as good (mean range 3.53-3.76). CONCLUSION Healthy work environments mutually benefit patients, nurses, nurse managers, health care providers, the health team, administration, the institution and the community at large. IMPLICATIONS FOR NURSING MANAGEMENT Valuable baseline data on the status of the work environment in this setting were generated. This should allow administrators and staff to work together in improving weaknesses and strengthening further whatever gains that are attained to ensure consistent provision of safe and quality patient care.
Milk Fat Globule Epidermal Growth Factor-Factor VIII Is Down-Regulated in Sepsis via the Lipopolysaccharide-CD14 Pathway1

Phagocytosis prevents the release of potentially harmful or immunogenic materials from dying cells. Milk fat globule epidermal growth factor (EGF)-factor VIII (MFG-E8) mediates the clearance of apoptotic cells. We have previously shown that the administration of MFG-E8-rich exosomes from immature dendritic cells promotes the phagocytosis of apoptotic cells and improves survival in sepsis. Because endotoxin is elevated in polymicrobial sepsis, we hypothesized that down-regulation of MFG-E8 is mediated via the LPS-CD14 pathway, eventually leading to the accruement of apoptotic cells. Polymicrobial sepsis was induced by cecal ligation and puncture (CLP) in CD14-deficient (CD14−/−), TLR4-mutated and wild-type (WT) mice. In addition, endotoxemia was elicited by i.p. injection of LPS. LPS was also neutralized by pretreating CLP-induced WT mice with polymyxin B. Splenic MFG-E8 expression, phagocytic activity, and apoptosis were assessed 5 and 20 h after CLP or 5 h after LPS administration. In septic WT mice, MFG-E8 mRNA and protein levels were suppressed by 49 and 33%, respectively. Endotoxemia reduced MFG-E8 mRNA expression in a dose dependent manner and the down-regulation of MFG-E8 mRNA expression in CLP-induced sepsis was attenuated by polymyxin B. This CLP-induced suppression was not observed in both CD14−/− and TLR4-mutated mice. CLP significantly decreased phagocytic activity of peritoneal macrophages in WT (by 30%), but not in CD14−/− mice. CLP also induced significant apoptosis in the spleen of WT (by 61%), but less in CD14−/− mice. Thus, MFG-E8 production is down-regulated in sepsis by LPS-CD14 dependent fashion, leading to a reduction of phagocytosis of apoptotic cells.
Subject Descriptions and Single-Subject Research

In this manuscript, we respond to the minimum standards for describing research subjects as proposed by the Council for Learning Disabilities (CLD) Research Committee. Three issues are raised about the standards: (a) whether justification exists for the recommended standards, (b) whether a relationship exists between the description of subjects and the probability of replication, and (c) whether all types of research require the same subject description standards. These issues are discussed, and three recommendations to investigators who use single-subject research designs are made: (1) Describe variables that control subjects' performance in baseline conditions and the contexts of that performance, (2) identify subject selection criteria, and (3) report status variables as recommended by the CLD Research Committee.
The Impact of Customer Experience on Relationship Quality with Travel Agencies in a Multichannel Environment

This article seeks to examine the impact of the customer experience (concept of THINK, FEEL, SENSE, ACT and RELATE developed by Schmitt in 1999) on relationship quality with travel agencies in a multichannel environment (in-store and online). A self-administered Web questionnaire was answered by 289 adult respondents from a panel of individuals residing in Canada, not working for a travel agency and having visited both the physical agency (in-store) and website (online) of the travel agency with which they generally conducted business. Results were analyzed using structural modeling techniques (EQS 6.2). For both the in-store and online contexts, results pointed to the THINK and FEEL dimensions as the key factors positively impacting relationship quality. The ACT (in-store) and SENSE (online) dimensions were also found to influence relationship quality positively.
The perception of global hierarchies: South-Eastern European patterns in comparative perspectives

In this paper we examine the views of ordinary people in Hungary and Romania about developmental trajectories and developmental hierarchies. Our work extends research on perception of global hierarchies as we include the views of ordinary people in the countries of Hungary and Romania. In addition, our research makes a unique contribution to the development and developmental hierarchy literature by examining, for the first time, how individuals define development. Although the main focus of this paper is the developmental views of Hungarians and Romanians, at times we add to our discussion results from a survey in Bulgaria and another from Albania. In this paper we conclude that developmental models are widespread among ordinary people in Hungary and Romania; we also find that the South-East European region does show some specificities in terms of the over- and under-positioning of certain countries relative to the dominant international rating system. China and, to a lesser degree, Russia were over-positioned by respondents relative to the developmental index scores of those countries. Within the region, unlike Bulgarians and Albanians, who severely underrated themselves, Hungarian and Romanian respondents put themselves in a middle position on the developmental scale. Concerning developmental items with some differences between the two countries, our data show that the economy is the most important development criterion in the minds of Hungarians and Romanians, followed by democracy, science and technology, and education.
Evaluation of the PANBIO Brucella Immunoglobulin G (IgG) and IgM Enzyme-Linked Immunosorbent Assays for Diagnosis of Human Brucellosis

ABSTRACT PANBIO Brucella immunoglobulin G (IgG) and IgM enzyme-linked immunosorbent assays (ELISAs) were assessed against Brucella standard agglutination tube and Coombs tests. The sensitivities of ELISA IgG and IgM were 91% and 100%, respectively, while the specificity was 100% for both. These ELISAs are simple, rapid, and reliable for the diagnosis of human brucellosis.
Radiation-Hardening Technique for Voltage Reference Circuit in a Standard 130 nm CMOS Technology

A radiation-hardening technique for a CMOS voltage reference circuit is proposed. Its operation principle consists in combining linearly two different NMOS threshold voltages and a Proportional-To-Absolute-Temperature (PTAT) voltage, which allows the compensation of both temperature-induced and radiation-induced discrepancies. This circuit was implemented in a standard 130 nm CMOS technology and designed in two different layouts. Measurements show a good operation with a minimal supply voltage of 2.5 V, a PSRR of 80 dB at 3.3 V. The voltage output shift is around 0.5% under irradiation up to 40 krad(Si). The active area of the circuit is about 0.04 mm2.
Lymphocyte recruitment to the lung.

Lymphocyte recruitment in lymphoid tissues and inflammatory sites occurs in response to two events. The first is adherence of lymphocytes to specialized molecules expressed on the surface of appropriately stimulated vascular endothelial cells known as vascular addressins. The interaction occurs via specialized lymphocyte surface molecules known as homing receptors. There is considerable diversity among these molecules. At least three, and possibly four, different addressin-homing receptor pairs exist, regulating entry into peripheral lymph nodes, gut lymphoid tissue, BALT and intrathoracic lymphoid tissue, and inflamed synovium. Vascular addressins are expressed by specialized endothelial cells known as HEV. HEV are not found in normal lung parenchyma but may be induced to appear during an immune response. The mechanism for induction of HEV is unknown, although it may involve the action of inflammatory cytokines. It is not known whether separate endothelial cells exist with a propensity to develop into HEV or if any endothelial cells will develop into HEV if stimulated in the proper manner. Other accessory, lymphocyte-endothelium adhesion molecule pairs have been described, including LFA-1-ICAM-1 and CD4-HLA-DR. These molecules are induced by exposure of the endothelium to inflammatory cytokines, chiefly IFN-gamma. Thus, local humoral influences present during inflammation can alter the possibility of lymphocyte traffic through the endothelium by regulating the presence of lymphocyte adherence molecules. These processes have been documented to occur in the lung in normal homeostasis (e.g., BALT) and in disease (e.g., immunization with SRBC). After adherence, lymphocytes exit the circulation via amoeboid motility. This motility can be altered and enhanced through chemoattractant substances that act via surface receptors. The biochemical basis of cell motility is not entirely clear but appears to involve a link between the second messengers of receptor signaling and changes in the cytoskeleton, particularly actin filaments and microtubules. Like fibroblasts and smooth muscle cells, lymphocytes appear to respond to a number of "mitoattractants," substances that cause cell cycle entry and/or progression as well as enhanced motility. This relationship illustrates the integral relationship between cell motility and proliferation and suggests that the process of cell recruitment might also prime the recruitment cells to become activated to proliferate and perform effector function. Studies of lymphocyte-mediated lung disease confirm that antigen-specific as well as antigen-nonspecific lymphocytes are selectively recruited to the lung from the circulation during an inflammatory reaction in the lung. (ABSTRACT TRUNCATED AT 400 WORDS)