Miracle-dz/UniFAR-FTUs huggingface dataset:
Neuronal intranuclear hyaline inclusion disease showing motor-sensory and autonomic neuropathy

Background: Neuronal intranuclear hyaline inclusion disease (NIHID), a rare neurodegenerative disease in which eosinophilic intranuclear inclusions develop mainly in neurons, has not yet been described to present as hereditary motor-sensory and autonomic neuropathy. Methods: Patients in two NIHID families showing peripheral neuropathy were evaluated clinically, electrophysiologically, and histopathologically. Results: In both families, patients had severe muscle atrophy and weakness in limbs, limb girdle, and face; sensory impairment in the distal limbs; dysphagia, episodic intestinal pseudoobstruction with vomiting attacks; and urinary and fecal incontinence. No patients developed symptoms suggesting CNS involvement. Electrophysiologic study showed the reduced motor and sensory nerve conduction velocities and amplitudes, and also extensive denervation potentials. In sural nerve specimens, numbers of myelinated and unmyelinated fibers were decreased. In two autopsy cases, eosinophilic intranuclear inclusions were widespread, particularly in sympathetic and myenteric ganglion neurons, dorsal root ganglion neurons, and spinal motor neurons. These neurons also were decreased in number. Conclusion: Patients with neuronal intranuclear hyaline inclusion disease (NIHID) can manifest symptoms limited to those of peripheral neuropathy. NIHID therefore is part of the differential diagnosis of hereditary motor-sensory neuropathy associated with autonomic symptoms. Intranuclear hyaline inclusions in Schwann cells and in the myenteric plexus may permit antemortem diagnosis of NIHID.
Evaluation of hemostatic and fibrinolytic markers in dogs with ascites attributable to right-sided congestive heart failure.

OBJECTIVE To determine whether dogs with ascites secondary to right-sided congestive heart failure (CHF) have bleeding disorders associated with hypofibrinogenemia and discordant plasma fibrin-fibrinogen degradation products (FDPs) and D-dimer assay results (ie, a circulating concentration of FDPs higher than the reference range and a circulating concentration of D-dimer within the reference range). DESIGN Retrospective case-control study. ANIMALS 80 client-owned dogs. PROCEDURES Dogs with ascites secondary to right-sided CHF (group 1; n = 20), unhealthy dogs without cardiac disease (group 2; 40), and dogs with left-sided CHF (group 3; 20) were included in the study. Urine bile acids-to-creatinine concentration ratios were calculated as a marker of liver function. Differences among groups regarding coagulation profile analysis results and prevalence of discordant FDPs and D-dimer assay results were determined. RESULTS No significant differences were detected among the 3 groups regarding urine bile acids-to-creatinine concentration ratios. Plasma fibrinogen concentration was significantly lower for group 1 versus groups 2 or 3. Prevalence of discordant FDPs and D-dimer assay results was significantly higher for group 1 versus groups 2 or 3. Eighteen group 1 dogs had discordant FDPs and D-dimer assay results. Ten of these dogs had concurrent hypofibrinogenemia, 2 of which had clinical signs of bleeding. Only 10 dogs in groups 2 or 3 had discordant FDPs and D-dimer assay results; none of these dogs had hypofibrinogenemia or clinical signs of bleeding. CONCLUSIONS AND CLINICAL RELEVANCE Dogs with right-sided CHF and ascites may be at increased risk for primary hyperfibrinogenolysis (ie, hypofibrinogenemia and discordant FDPs and D-dimer assay results).
Tweets I've seen: analysing factors influencing re-finding frustration on Twitter

While social networking and microblogging platforms have received considerable research attention, little work has been done to understand how users preserve, manage and reaccess content they acquire from these sources. In this paper we present initial analyses of a large-scale survey (n=606) to understand Personal Information Management (PIM) practices with social networking and microblogging systems, and Twitter in particular. Our results indicate that re-finding information in tweets is a common Twitter activity and can be frustrating. Using questionnaire responses, we investigate the influence of several factors, including how the user tends to preserve tweets of interest, and the level of frustration involved in re-finding such tweets when they are required later.
Why Did Socialism Fail? The Role of Factor Inputs Reconsidered

We present revised growth accounts for three socialist economies between 1950 and 1989. Government statistics reported distorted measures for both the rate and trajectory of productivity growth in Czechoslovakia, Hungary, and Poland. Researchers have benefited from revised output data, but continued to use official statistics on capital input, or estimated capital stock from official investment data. Investment levels and rates of capital accumulations were, in fact, much lower than officially claimed and over-reporting worsened over time. Sluggish factor accumulation, specifically declining equipment investment and labor input, contributed much more to the socialist growth failure of the 1980s than previously thought.
Suppression of serum insulin by diazoxide reduces serum testosterone levels in obese women with polycystic ovary syndrome.

To test the hypothesis that insulin plays a role in the hyperandrogenism of obese women with polycystic ovary syndrome, we conducted a prospective study in which the androgen status of five obese women with polycystic ovary syndrome was assessed on two occasions: before and after 10 days of oral diazoxide (100 mg, three times daily) administration. Fasting serum insulin levels decreased from 177 +/- 45 (+/- SE) to 123 +/- 43 pmol/L (P less than 0.01) and insulin release in response to 100 g oral glucose administration decreased from 223.0 +/- 29.2 to 55.6 +/- 7.9 nmol.min/L (P less than 0.002) after diazoxide administration. At the same time, serum total testosterone fell from 2.5 +/- 0.4 to 2.1 +/- 0.3 nmol/L (P less than 0.007), serum testosterone not bound to sex hormone-binding globulin fell from 1.9 +/- 0.3 to 1.4 +/- 0.2 nmol/L (P less than 0.01), and the molar ratio of serum androstenedione to serum estrone fell from 25.7 +/- 7.7 to 16.6 +/- 5.5 (P less than 0.04). Serum sex hormone-binding globulin levels increased slightly but not significantly from 13.2 +/- 1.0 to 21.7 +/- 4.1 nmol/L. Serum androstenedione, dehydroepiandrosterone sulfate, estradiol, estrone, and progesterone concentrations did not change, nor did basal or GnRH-stimulated serum LH and FSH concentrations. These results suggest that hyperinsulinemia in obese women with polycystic ovary syndrome may directly increase serum testosterone levels.
The discriminating power of a risk score for postoperative vomiting in adults undergoing various types of surgery

Background: Recently, we have demonstrated that the probability of postoperative vomiting (PV) following ENT surgery with inhalational anaesthetics can be predicted using a risk score. This score is based on gender, age, smoking status, history of motion sickness or postoperative nausea and vomiting and the duration of anaesthesia. Therefore, it is of interest whether this score is also accurate in predicting PV in patients undergoing different types of surgery.
Functional Analysis and Regulation of the Divergent spuABCDEFGH-spuI Operons for Polyamine Uptake and Utilization in Pseudomonas aeruginosa PAO1

ABSTRACT A multiple-gene locus for polyamine uptake and utilization was discovered in Pseudomonas aeruginosa PAO1. This locus contained nine genes designated spuABCDEFGHI (spu for spermidine and putrescine utilization). The physiological functions of the spu genes in utilization of two polyamines (putrescine and spermidine) were analyzed by using Tn5 transposon-mediated spu knockout mutants. Growth and uptake experiments support that the spuDEFGH genes specify components of a major ABC-type transport system for spermidine uptake, and enzymatic measurements indicated that spuC encodes putrescine aminotransferase with pyruvate as the amino group receptor. Although spuA and spuB mutants showed an apparent defect in spermidine utilization, the biochemical functions of the gene products have yet to be elucidated. Assays of lacZ fusions demonstrated the presence of agmatine-, putrescine-, and spermidine-inducible promoters for the spuABCDEFGH operon and the divergently transcribed spuI gene of unknown function. Since the observed induction effect of agmatine was abolished in an aguA mutant where conversion of agmatine into putrescine was blocked, putrescine or spermidine, but not agmatine, serves as the inducer molecule of the spuA-spuI divergent promoters. S1 nuclease mappings confirmed further the induction effects of the polyamines on transcription of the divergent promoters and localized the transcription initiation sites. Gel retardation assays with extracts from the cells grown on putrescine or spermidine demonstrated the presence of a polyamine-responsive regulatory protein interacting with the divergent promoter region. Finally, the absence of the putrescine-inducible spuA expression and putrescine aminotransferase (spuC) formation in the cbrB mutant indicated that the spu operons are regulated by the global CbrAB two-component system perhaps via the putative polyamine-responsive transcriptional activator.
Gender and the Importance of Fringe Benefits: Exploring Their Link with Organizational Commitment and Job Satisfaction

The importance of fringe benefits for both employers and employees has been repeatedly pinpointed in the Human Resource Management academic and professional literature. How important are they, though, for either gender, in the midst of the current economic crisis in Europe? The present article draws on the findings of a recent large-scale survey in Greece, in order to establish and discuss the significance of fringe benefits to both employees and employers and explore their link with organizational commitment.
Entry of bluetongue vector Culicoides imicola into livestock premises in Spain

Culicoides imicola Kieffer is considered to be the main vector of bluetongue disease (BT) and African horse sickness (AHS) in the Mediterranean basin. It has been assumed that this midge species is exophilic and, consequently, that stabling of livestock should provide effective protection against these diseases. This study presents the results of sampling surveys for C. imicola carried out both inside and outside stables on three farms in mainland Spain. The number of C. imicola captured varied as a function of the populations sampled and trap location (inside vs. outside). The daily mean number captured inside during the sampling of each farm population was directly correlated with the daily mean number captured outside, but daily correlation of captures was not observed. By contrast with previous studies, the mean catch of C. imicola inside was consistently higher than that outside. No clear effect of stable characteristics on the degree of entry was detected. In addition, proportions of males and age‐graded female groups varied among populations and with trap location. Proportionately more males and fewer engorged females were captured outside than inside, although the proportions varied among stables. These results contrast with those of previous studies, and with the assumed pronounced exophilic behaviour of C. imicola, and raise important questions about the vector activity of this species in the study area and its implications for the epidemiology of BT and/or AHS.
Development and experimental evaluation of a steady‐state, multispecies biofilm model

A steady‐state model for quantifying the space competition in multispecies biofilms is developed. The model includes multiple active species, inert biomass, substrate utilization and diffusion within the biofilm, external mass transport, and detachment phenomena. It predicts the steady‐state values of biofilm thickness, species distribution, and substrate fluxes. An experimental evaluation is carried out in completely mixed biofilm reactors in which slow‐growing nitrifying bacteria compete with acetate‐utilizing heterotrophs. The experimental results show that the model successfully describes the space competition. In particular, increasing acetate concentrations causes NH4+‐N fluxes to decrease, because nitrifiers are forced deeper into the biofilm, where they experience greater mass‐transport resistance.
Triazolam (Halcion) versus flunitrazepam (Rohypnol) against midwinter insomnia in Northern Norway

In a double‐blind, cross‐over trial, triazolam (0.25 mg) was tested against flunitrazepam (1 mg) in the typical midwinter insomnia which is often seen among otherwise healthy people in Northern Norway. Each drug was given for five nights, in random order, with a five‐night placebo period between the active drugs (providing for a single blind comparison with placebo). A total of 22 outpatients started the trial; 19 completed. There were highly significant differences between each active drug and placebo on the subjectively scored variables sleep latency, duration of sleep, and total evaluation of sleep, and also significant differences for feeling in the morning, number of awakenings, and quality of sleep; all differences were in favour of the active drugs. Generally, the sleep variables were rated on the same level in the placebo period as they were for the last five nights prior to the trial.
Making Good Use of Partners: Differential Effects of Managerial Networking in the Social Care Domain

ABSTRACT Public managers engage in networking relationships with a wide variety of external actors and organizations from which they can draw different types of support and resources. Most empirical studies on managerial networking merely present different intensities of external networking in general, as if it were a unidimensional concept. In practice, however, public managers strategically differentiate between functional or task-related groups of external partners, based on the specific policy context. Moreover, such differentiation in networking behavior can be expected to systematically impact agency and public program performance outcomes. This article derives contextualized hypotheses on how managerial networking affects the performance of Dutch local governments in the social care domain. Multilevel structural equation analyses of 3,257 social care clients in 71 local governments provide evidence that bilateral client-interest networking is more beneficial to the Social Support Act's overall goal of improving the level of social participation of clients than professional networking. Moreover, the effect is indirect: managerial networking with client interest groups improves the physical self-reliance of clients, enabling them to engage in social activities.
Mutations in the Corneal Endothelial Dystrophy–Associated Gene SLC4A11 Render the Cells More Vulnerable to Oxidative Insults

Purpose: To investigate the effect of mutations in SLC4A11 on cellular localization of the protein, mitochondrial function, and apoptosis due to oxidative stress. Mutations in SLC4A11 have been associated with 2 different forms of corneal endothelial dystrophy that lead to degeneration of the corneal endothelium, causing opacity of the cornea and gradual vision loss. Methods: HEK 293 cells were transfected with wild-type SLC4A11 or mutants, Ser213Leu, Arg233Cys, Gly418Asp, and Thr584Lys, and exposed to oxidative stress. Cellular localization of the proteins was detected by confocal microscopy, whereas mitochondrial dysfunction, reactive oxygen species (ROS) generation, and apoptosis were analyzed by flow cytometry and a colorimetric assay. Expressions of antioxidant genes were quantitated by real-time polymerase chain reaction. Results: Although wild-type SLC4A11 was localized on the cell membrane, mutant proteins were found diffused in the cytoplasm. Mutations in SLC4A11 caused an increase in generation of ROS and mitochondrial dysfunction due to oxidative stress. NRF2, HO-1, and NQO expression decreased significantly, and a higher rate of apoptosis was detected in cells with mutant proteins under oxidative stress. Conclusions: Our data suggest that mutations in SLC4A11 cause retention of the protein in the cytoplasm and generate increased reactive oxygen species. We found that cells containing mutant SLC4A11 are more vulnerable to oxidative and mitochondrial damage, less able to overcome oxidative stress through the expression of sufficient levels of antioxidant genes, and are more prone to apoptotic death.
Hepatitis A: epidemiology in resource-poor countries

Purpose of review Transmission of hepatitis A virus (HAV) infection is primarily fecal–oral. Symptomatic hepatitis, severe disease, and death are more likely to occur when infection occurs at an older age. Improvements in socioeconomic and hygienic conditions have led to a change in its epidemiology worldwide. Recent findings In the last two decades, improved hygiene in several resource-poor countries has led to reduced transmission of HAV, an increase in average age at infection, and, consequently, a paradoxical increase in morbidity and mortality because of hepatitis A. In Argentina, introduction of one dose (instead of the conventional two doses, to reduce costs) of inactivated HAV vaccine at 12-month age in a universal childhood immunization program during such ‘epidemiologic transition’ has markedly reduced the incidence of symptomatic hepatitis A, and of fulminant hepatitis and liver transplantation caused by HAV infection. The monetary value of medical and nonmedical benefits of this strategy outweighed the expenditure on vaccination. These excellent results were possibly contingent upon a high vaccination coverage. Summary Resource-poor countries should closely monitor the epidemiology of HAV infection and periodically undertake cost-effectiveness analyses of HAV immunization strategies. This should allow timely identification of epidemiologic transition and introduction of preventive strategies before HAV infection becomes a public health problem.
WAGR Syndrome With Tetralogy of Fallot and Hydrocephalus

Wilms tumor, aniridia, genitourinary abnormalities, and mental retardation (WAGR) syndrome occurs sporadically due to deletion of chromosome 11p13. A variety of other abnormalities involving different systems have been reported in patients with WAGR syndrome. We report on a patient with WAGR syndrome with accompanying tetralogy of Fallot and hydrocephalus.
Effect of an unstirred layer on the membrane permeability of anandamide Published, JLR Papers in Press, December 19, 2005.

To study the effect of an unstirred layer (UL), we have investigated the exchange efflux kinetics of anandamide at 0°C, pH 7.3, from albumin-free as well as from albumin-filled human red blood cell ghosts to media of various BSA concentrations ([BSA]o). The rate constant (km) of unidirectional flux from the outer membrane leaflet to BSA in the medium increased with the square root of [BSA]o in accordance with the existence of a UL, which is a water layer adjacent to the membrane that is not subject to the same gross mixing that takes place in the rest of the medium. From km, it is possible to calculate the rate constant of anandamide dissociation from BSA (k1) if we know the membrane binding of anandamide, the equilibrium dissociation constant of BSA-anandamide complexes, and the diffusion constant of anandamide. We estimated k1 to be 3.33 ± 0.27 s−1. The net flux of [3H]anandamide is balanced by an equal and opposite movement of nonradioactive anandamide in exchange efflux experiments. This means that our results are also valid for uptake. We show that for anandamide with rapid membrane translocation, UL causes a significant resistance to cellular uptake. Depicting the rate of anandamide uptake as a function of equilibrium water phase concentrations results in a parabolic uptake dependence. Such apparent “saturation kinetics” is often interpreted as indicating the involvement of transport proteins. The validity of such an interpretation is discussed.
Pure-tone threshold estimation from extrapolated distortion product otoacoustic emission I/O-functions in normal and cochlear hearing loss ears.

A new method for direct pure-tone threshold estimation from input/output functions of distortion product otoacoustic emissions (DPOAEs) in humans is presented. Previous methods use statistical models relating DPOAE level to hearing threshold including additional parameters e.g., age or slope of DPOAE I/O-function. Here we derive a DPOAE threshold from extrapolated DPOAE I/O-functions directly. Cubic 2 f1-f2 distortion products and pure-tone threshold at f2 were measured at 51 frequencies between f2=500 Hz and 8 kHz at up to ten primary tone levels between L2=65 and 20 dB SPL in 30 normally hearing and 119 sensorineural hearing loss ears. Using an optimized primary tone level setting (L1 = 0.4L2 + 39 dB) that accounts for the nonlinear interaction of the two primaries at the DPOAE generation site at f2, the pressure of the 2 f1-f2 distortion product pDP is a linear function of the primary tone level L2. Linear regression yields correlation coefficients higher than 0.8 in the majority of the DPOAE I/O-functions. The linear behavior is sufficiently fulfilled for all frequencies in normal and impaired hearing. This suggests that the observed linear functional dependency is quite general. Extrapolating towards pDP=0 yields the DPOAE threshold for L2. There is a significant correlation between DPOAE threshold and pure-tone threshold (r=0.65, p<0.001). Thus, the DPOAEs that reflect the functioning of an essential element of peripheral sound processing enable a reliable estimation of cochlear hearing threshold up to hearing losses of 50 dBHL without any statistical data.
A dynamic weighting ensemble approach for wind energy production prediction

In this work, we propose a method to predict wind energy production. The method is based on an ensemble of Artificial Neural Networks (ANNs), which receive in input weather forecast variables and predict the wind plant energy production. We investigate different strategies for aggregating the outcomes of the individual models of the ensemble and compare them with a real dataset. A dynamic weighting ensemble which combines the individual models outcomes proportionally to their local performances in the neighborhood of the test pattern under analysis is found to provide the most accurate predictions.