Miracle-dz/UniFAR-FTUs huggingface dataset:
Agalsidase alfa – a preparation for enzyme replacement therapy in Anderson–Fabry disease

Anderson–Fabry disease is an X-linked multisystemic disorder caused by a genetic deficiency of the lysosomal enzyme α-galactosidase A. The enzyme is responsible for degradation of glycolipids inside the lysosomes. Lack of catalytic activity leads to progressive depositions of undegraded glycolipids in a large number of organs. Crises of severe pain in the extremities (acroparesthesias), hypohidrosis, corneal opacities and dysfunction of several organs (kidney, brain, heart) are the leading symptoms in patients with Anderson–Fabry disease. Females may have the same symptoms as males but to a more variable degree. The variable manifestations seen in heterozygotes can be explained by the Lyon hypothesis. This hypothesis predicts that in X-linked diseases, the carriers are a mosaic of normal and mutant cells in varying proportions and hence have variable expression. As in Gaucher’s disease, enzyme replacement therapy recently became available for Anderson–Fabry disease. Two drugs have gained approval in the EU by the European Agency for the Evaluation of Medicinal Products. These are agalsidase beta (Fabrazyme®, Genzyme Corporation) and agalsidase alfa (Replagal®, Transkaryotic Therapies, Inc.). This review will describe clinical efficacy, safety and tolerability of agalsidase alfa.
Thermal transport measurements in multi-wall carbon nanotube strands using the 3/spl omega/ method

This work reports temperature-dependent thermal conductivity, specific heat and thermal diffusivity measurements of multi-wall carbon nanotube strands. Thermal property characterization has been carried out along the carbon nanotube alignment direction with an AC driven, self-heating 3/spl omega/ method over a temperature range from 90-310K. The specific heat and thermal conductivity increases linearly with temperature. The thermal diffusivity is 5/spl times/10/sup -6/ m/sup 2/s/sup -1/ at room temperature and is nearly constant across the temperature range, slightly increasing at lower temperatures. The observed trends in specific heat and thermal conductivity are similar with other published multi-wall carbon nanotube data. The measured thermal diffusivity agrees with data reported elsewhere for the same MWCNT material measured by a different technique.
A comparison of enalapril 20 mg once daily versus 10 mg twice daily in terms of blood pressure lowering and patient compliance.

OBJECTIVE To compare enalapril 20 mg once daily with 10 mg twice daily in terms of blood pressure reduction and patient compliance. DESIGN Cross-over study of patients randomly assigned to a sequence of enalapril 20 mg once daily or 10 mg twice daily in three 4-week periods following a 4-week placebo run-in. SETTING General practices in the greater Belfast and Lisburn area in Northern Ireland. PATIENTS Twenty-five hypertensive patients who had a mean diastolic blood pressure of between 90 and 110 mm Hg after receiving placebo for 4 weeks. MAIN OUTCOME MEASURES Reduction in blood pressure and estimation of patient compliance. RESULTS Patient compliance was superior on the once daily regimen. However, the twice daily regimen was associated with a greater blood pressure reduction which almost reached statistical significance at the 5% level. CONCLUSIONS Enalapril 20 mg should be prescribed as 10 mg twice daily and measures taken to improve patient compliance.
Introduction to the Special Issue on Usability and User Experience: Methodological Evolution

This special issue focuses on the evolution of development and assessment methodologies related to usability and user experience. The five articles have a diverse range of topics, including comparison of moderated and unmoderated think-aloud usability sessions, a new usability inspection method based on concept mapping, analysis of the fitness of scrum (agile) and kanban (lean) development methodologies to incorporate user experience methodologies, exploration of the relation between expectations and user experience, and a case study describing difficulties encountered when assessing usability “in the wild. ” These articles should prove to be of value to practitioners and researchers with an interest in the evolution of usability and user experience methodologies.
Effects of Practice in Repeated Administrations of the Wechsler Memory Scale-Revised in Normal Adults

Practice effects were examined over four administrations of the immediate (I) and delayed (II) portions of three subtests of the WMS-R: Logical Memory (LM), Verbal Paired Associates (VPA), and Visual Reproduction (VR). A repeated measures ANOVA revealed that large and significant (p < .001) increases occurred in the General Memory (GM) and Delayed Recall (DR) indices and in the LMI, LMII, and VPAI subtests (Effect Sizes [ESs] = 0.70-0.87). Small but significant (p < .001) increases occurred in VRI (ES = 0.24) and VRII (ES = 0.43). The greatest increase in scores occurred at the first retest session, whereas increases of smaller magnitudes occurred at Sessions 3 and 4. Ceiling effects occur in subtests (VPAII, VRI, and VRII) on which individuals score most of the total possible points at the first testing session; this makes interpretation of practice effects difficult. Test-retest reliability coefficients, mean change scores from Session 1 to Session 2, standard errors of prediction, and 95% confidence intervals are presented. These score changes need to be taken into consideration when interpreting performance at retest.
Effects of 18‐methoxycoronaridine on acute signs of morphine withdrawal in rats

IBOGAINE, an alkaloid found in the root bark of the African shrub Tabernanthe iboga, has been claimed to interrupt opioid dependence in humans; in animals, it has been shown to inhibit morphine self-administration and to attenuate signs of morphine withdrawal. However, ibogaine has some neurotoxicity, and because of this, efficacious and safer congeners of ibogaine have been sought. 18-Methoxycoronaridine (18-MC), a novel iboga alkaloid congener, has been shown, in animals, to mimic the effects of ibogaine on morphine self-administration without producing any ibogaine-like neurotoxiticity. In the present study, 18-MC was shown to attenuate five of seven signs of morphine withdrawal in rats. The data suggest that 18-MC will ameliorate symptoms of opioid dependence in humans.
Beamforming-Based Wavepacket Model for Noise Environment Predictions of Tactical Aircraft

Jet noise consists of extended, partially correlated sources such that a single-wavepacket source representation is inadequate. A multiple-wavepacket (MWP) model provides an analytical framework for jet-noise-like radiation to simulate jet noise field levels as well as the corresponding spatial coherence properties within the field. Here, a beamforming method with regularization is applied to noise measured by a linear array near a high-performance military aircraft. Beamforming results are decomposed into a reduced-order MWP model and the predicted radiation is validated in terms of level and coherence properties using benchmark measurements. Sound levels and coherence lengths generated by the beamforming results show good agreement with benchmark measurements over a range of frequencies that contribute significantly to the overall radiation. The MWP model is shown to predict full-scale specific features such as multilobe directivity patterns, and the addition of an uncorrelated distribution (UD) model adequately predicts the sideline radiation that is otherwise difficult to reproduce from wavepacket radiation. The MWP model predicted radiation characteristics are an improvement over single-wavepacket models, which do not incorporate spatiotemporal features of the radiation.
Changes in fat synthesis influenced by dietary macronutrient content

De novo lipogenesis is the biological process by which C2 precursors of acetyl-CoA are synthesized into fatty acids. In human subjects consuming diets higher in fat (>30 % energy), lipogenesis is down regulated and extremely low; typically < 10 % of the fatty acids secreted by the liver. This percentage will increase when dietary fat is reduced and replaced by carbohydrate, although the extent of carbohydrate-induced lipogenesis is dependent on the type of carbohydrate (monosaccharide v. polysaccharide) and the form in which the carbohydrate is fed (liquid meals, solid less-processed food). Clearly, massive overconsumption of carbohydrate can also increase lipogenesis. A second related phenomenon that occurs when dietary fat is reduced is hypertriacylglycerolaemia. This rise in blood triacylglycerol concentration could be due to increased de novo lipogenesis or to reduced clearance of lipid from the blood. The present paper will review the metabolic mechanisms leading to the elevations in blood triacylglycerol concentration that occur with dietary fat reduction. Studies considered will be those investigating fatty acid synthesis in subjects chronically fed low-fat high-carbohydrate diets and studies in which data were obtained in both the fasted and fed states. Also summarized will be data from subjects who had consumed diets of different carbohydrate types, as well as the most recent data from postprandial studies investigating factors that affect the magnitude of the rise in blood lipids following a meal. Given the changing availability of carbohydrate in the food supply, it will be important to understand how the balance of fat and carbohydrate in the diet influences lipogenesis, and the relative contribution of the process of de novo lipogenesis to the escalating incidence of obesity observed around the world.
Importance of transverse pedicle angle & chord length of lumbar pedicle in screw placement: a CT scan study on North West Indian population

The aim of this study was to measure the lumbar pedicle dimensions and its angulations for the development of techniques and devices for spinal instrumentation on computed tomography (CT) scan. Fifty CT scans of lumbar vertebral column of patients belonging to North West Indian population was randomly selected. Various morphometric parameters were recorded using software. The transverse pedicle angle increased from L1 to L5 in both males and females. In males it was maximum at L5 (25.7°) and minimum at L1 (7.5°). In females it was maximum at L5 (24.0°) and minimum at L1 (7.4°). The chord length in males was maximum at L5 (51.3 mm) and minimum at L1 (48.1 mm). In female chord length was maximum at L2 (51.1 mm) and minimum at L4 (47.6) on right side and on left side it was maximum at L3 (50.9 mm) and minimum at L1 (46.7 mm). A screw of 40 mm length appeared to be safe at all lumbar levels as all the vertebrae studied had a chord length well in excess of 40 mm. The length of the pedicular screw needs to be substantially smaller for Indian population than those mentioned in Western literature. At the lower lumber levels, greater lateral inclination of the pedicle should be kept in mind. Otherwise it may lead to the breach of the medial cortex of the pedicle with resultant risk to the neural tissues.
Från gammal man till den gamle mannen : Definitmarkering i fornsvenska nominalfraser med adjektivattribut

Modern Swedish uses both a free, pre-adjectival, definite article and a definite suffix on the noun to mark definiteness in noun phrases with adjectival attributes. The combination of two definite morphemes in a single noun phrase is commonly referred to as double definiteness. The definite inflexion of nouns, which applies to all the Scandinavian languages, probably arose around 1000, and the use of nouns in the definite form increases steadily over the following centuries, probably in a similar way all over Scandinavia. Interestingly, however, semantically definite noun phrases with adjectival attributes normally lack all definiteness marking in Old Swedish up to the beginning of the 14th century, which has not been brought to the attention in earlier research. During the latter half of the 14th century, nouns in the definite form could be preceded by adjectives, but only in the so-called weak form. A weakly inflected adjective could in turn be preceded by an article-like word, but a definite article in the strict sense is not developed until the 15th century. Norwegian has also developed double definiteness, but apparently via a different route. Already early Old Norwegian used a true definite article in pre-adjectival position, at first in combination with uninflected nouns, but later with nouns in the definite form.
The Effect of Thermal Quality on the Thermoregulatory Behavior of the Bearded Dragon Pogona vitticeps: Influences of Methodological Assessment

Metabolic functions are generally optimized within a narrow range of body temperatures (Tb's), conferring thermoregulation great importance to the survival and fitness of an animal. In lizards, Tb regulation is mainly behavioral, and the metabolic costs associated with behavioral thermoregulation are primarily locomotory. In reptiles, however, it has been proposed that they thermoregulate less precisely when the associated costs, metabolic or otherwise, are high. Such a strategy enhances fitness by allowing lizards to be more flexible to changing environmental conditions while maximizing the benefits of maintaining a high Tb and minimizing energy expenditure. We evaluated the behavioral thermoregulation of inland bearded dragons Pogona vitticeps under various thermal quality conditions requiring different locomotory investment for thermoregulation. The selected ambient temperature and preferred Tb ranges increased at lower environmental thermal qualities, indicating a decrease in thermoregulatory precision in environments where the costs associated with thermoregulation were high. The level of thermoregulation was also affected, exhibiting a decrease in preferred Tb of ∼2°C at the lowest‐thermal‐quality treatment. These data provide important implications for the procedural assessment of preferred Tb and a better understanding of thermal set points in reptiles in general. Our results emphasize that the precise maintenance and assessment of preferred Tb is contingent on the quality of the environment, laboratory or natural, that the animal inhabits.
Robust and comprehensive joint image-text representations

La presente these etudie la modelisation conjointe des contenus visuels et textuels extraits a partir des documents multimedias pour resoudre les problemes intermodaux. Ces tâches exigent la capacite de ``traduire'' l'information d'une modalite vers une autre. Un espace de representation commun, par exemple obtenu par l'Analyse Canonique des Correlation ou son extension kernelisee est une solution generalement adoptee. Sur cet espace, images et texte peuvent etre representes par des vecteurs de meme type sur lesquels la comparaison intermodale peut se faire directement.Neanmoins, un tel espace commun souffre de plusieurs deficiences qui peuvent diminuer la performance des ces tâches. Le premier defaut concerne des informations qui sont mal representees sur cet espace pourtant tres importantes dans le contexte de la recherche intermodale. Le deuxieme defaut porte sur la separation entre les modalites sur l'espace commun, ce qui conduit a une limite de qualite de traduction entre modalites. Pour faire face au premier defaut concernant les donnees mal representees, nous avons propose un modele qui identifie tout d'abord ces informations et puis les combine avec des donnees relativement bien representees sur l'espace commun. Les evaluations sur la tâche d'illustration de texte montrent que la prise en compte de ces information fortement ameliore les resultats de la recherche intermodale. La contribution majeure de la these se concentre sur la separation entre les modalites sur l'espace commun pour ameliorer la performance des tâches intermodales. Nous proposons deux methodes de representation pour les documents bi-modaux ou uni-modaux qui regroupent a la fois des informations visuelles et textuelles projetees sur l'espace commun. Pour les documents uni-modaux, nous suggerons un processus de completion base sur un ensemble de donnees auxiliaires pour trouver les informations correspondantes dans la modalite absente. Ces informations complementaires sont ensuite utilisees pour construire une representation bi-modale finale pour un document uni-modal. Nos approches permettent d'obtenir des resultats de l'etat de l'art pour la recherche intermodale ou la classification bi-modale et intermodale.
Multiple‐dose granulocyte‐macrophage–colony‐stimulating factor plus 23‐valent polysaccharide pneumococcal vaccine in patients with chronic lymphocytic leukemia

For the current study, the authors sought to determine whether administration of multiple‐dose granulocyte‐macrophage–colony‐stimulating factor (GM‐CSF) could improve response to standard 23‐valent polysaccharide pneumococcal vaccine (PPV) in patients with chronic lymphocytic leukemia (CLL).
Effect of day length, growth regulators and fertilization on growth and development of Michaelmas Daisy (Aster novi-belgii L.)

Effect of day length, growth regulators and fertilization on the height, plant diameter, number of buds and inflorescences was monitored in the species Aster novi-belgii L. ´Mary Ballard´ throughout three growing seasons in order to explore the possibility of its late-summer growing as a flowering pot plant. The total number of buds and inflorescences was by 20% larger under natural day length compared to plants grown under the shortened photoperiod. Bud opening dynamics was more intensive under the natural day length conditions. Comparison of the average number of open inflorescences per plant between plants grown under different photoperiod conditions revealed a significantly larger number of open inflorescences in plants grown under the short day conditions. Foliar application of daminozide was shown to be efficient in inhibiting the growth of asters up to 10%. Plant diameter was not much changed under the influence of daminozide, with the exception of second trial year, when it was reduced up to 25%. Daminozide application resulted in an increased number of buds in all trial variants. In all three years, significantly more flowers were determined in fertilized plants compared with non-fertilized, with the exception of plants grown under natural day length in first year of experiment.
Studies of the Commitment Step in the Germination of Spores of Bacillus Species

ABSTRACT Spores of Bacillus species are said to be committed when they continue through nutrient germination even when germinants are removed or their binding to spores' nutrient germinant receptors (GRs) is both reversed and inhibited. Measurement of commitment and the subsequent release of dipicolinic acid (DPA) during nutrient germination of spores of Bacillus cereus and Bacillus subtilis showed that heat activation, increased nutrient germinant concentrations, and higher average levels of GRs/spore significantly decreased the times needed for commitment, as well as lag times between commitment and DPA release. These lag times were also decreased dramatically by the action of one of the spores' two redundant cortex lytic enzymes (CLEs), CwlJ, but not by the other CLE, SleB, and CwlJ action did not affect the timing of commitment. The timing of commitment and the lag time between commitment and DPA release were also dependent on the specific GR activated to cause spore germination. For spore populations, the lag times between commitment and DPA release were increased significantly in spores that germinated late compared to those that germinated early, and individual spores that germinated late may have had lower appropriate GR levels/spore than spores that germinated early. These findings together provide new insight into the commitment step in spore germination and suggest several factors that may contribute to the large heterogeneity among the timings of various events in the germination of individual spores in spore populations.
Preliminary data on two voice therapy interventions in the treatment of presbyphonia

Presbyphonia is common among elderly individuals, yet few studies have evaluated behavioral treatment approaches for presbyphonia. The primary aim of this study was to assess the short‐term efficacy of two types of voice therapy—vocal function exercises (VFE) and phonation resistance training exercise (PhoRTE) therapy—in the treatment of presbyphonia. The secondary aim was to determine if differences in adherence and treatment satisfaction existed between the two therapy approaches.
Parieto-occipital syndrome following carbon monoxide poisoning.

1. A complex syndrome which includes spatial disorientation, constructional apraxia, Gerstmann's syndrome and dressing disability has been described, following carbon monoxide poisoning. 2. It is considered that a bilateral parieto-occipital lesion underlies the main symptoms. 3. There was no lucid interval following the stage of acute intoxication. 4. The case has been observed for five years. Mental deterioration came on early, and has persisted. The total improvement has been slight. 5. Brief reference has been made to the relevant literature.
Effect of superstrate thickness on the performance of broadband circularly polarised stacked patch antenna

Electrically thin superstrates have certain effects on the performance of singly fed CP stacked patch antennas. The thicknesses in the range of 0.0127 to 0.026 /spl lambda//sub o/ can be regarded as the good choices for an X-band CP-EMCP element. The effect of superstrate thickness on the P-plane mutual coupling is more obvious than the C-plane. In the frequency domain, the concave mutual coupling curve of the CP-EMCP elements explains the unexpected behaviour of the nonmonotonic decay in the spatial domain. Moreover, when designing an array using CP elements, an additional bandwidth in which mutual coupling stays at a specific level should also be considered.
Effects of GH replacement on endothelial function and large‐artery stiffness in GH‐deficient adults: a randomized, double‐blind, placebo‐controlled study

objectives Hypopituitary adults with growth hormone deficiency (GHD) have an increased cardiovascular mortality, although the mechanisms remain unclear. Endothelial dysfunction, characterized by reduced nitric oxide (NO) bioavailability, is a key early event in atherogenesis and is associated with increased vascular smooth muscle tone and arterial stiffening.
Distinguishing Positive Selection From Neutral Evolution: Boosting the Performance of Summary Statistics

Summary statistics are widely used in population genetics, but they suffer from the drawback that no simple sufficient summary statistic exists, which captures all information required to distinguish different evolutionary hypotheses. Here, we apply boosting, a recent statistical method that combines simple classification rules to maximize their joint predictive performance. We show that our implementation of boosting has a high power to detect selective sweeps. Demographic events, such as bottlenecks, do not result in a large excess of false positives. A comparison to other neutrality tests shows that our boosting implementation performs well compared to other neutrality tests. Furthermore, we evaluated the relative contribution of different summary statistics to the identification of selection and found that for recent sweeps integrated haplotype homozygosity is very informative whereas older sweeps are better detected by Tajima's π. Overall, Watterson's θ was found to contribute the most information for distinguishing between bottlenecks and selection.