Miracle-dz/UniFAR-FTUs huggingface dataset:
Renal biopsy findings in children and adolescents with Fabry disease and minimal albuminuria.

BACKGROUND Information concerning renal pathological characteristics in Fabry disease in childhood is limited. Our objective is to define renal morphological abnormalities in children and adolescents with Fabry disease and minimal proteinuria. STUDY DESIGN Case series. SETTING & PARTICIPANTS 9 symptomatic patients (7 males, 2 females; age range, 7 to 18 years); 2 patients had received enzyme replacement therapy for 2 years. OUTCOMES & MEASUREMENTS Renal morphological changes assessed by using light and electron microscopy. RESULTS All patients had acroparesthesia and typical eye changes, 7 showed hypohidrosis, 7 had gastrointestinal problems, and 2 had typical angiokeratomas. Mean albumin-creatinine ratio was 38 mg/g [corrected] (range, 5.3 to 104.3 mg/g). [corrected] Measured glomerular filtration rate was normal in all patients. Light microscopy showed changes in glomerular, tubulointerstitial, or vascular compartments alone or in combination in 7 patients. Electron microscopy showed lesions in all patients. LIMITATIONS Small sample size. CONCLUSIONS Glomerular and vascular changes are present before progression to overt proteinuria and decreased glomerular filtration rate. The combination of acroparesthesia and mild albuminuria, glomerular endothelial cell deposits, and arteriopathy may constitute a clinical and morphological combination heralding a potentially progressive renal disease.
Both oral and caudal parts of the spinal trigeminal nucleus project to the somatosensory thalamus in the rat

Recent evidence has been accumulated that not only spinal trigeminal nucleus caudalis (Sp5C) neurons but also spinal trigeminal nucleus oralis (Sp5O) neurons respond to noxious stimuli. It is unknown, however, whether Sp5O neurons project to supratrigeminal structures implicated in the sensory processing of orofacial nociceptive information. This study used retrograde tracing with Fluorogold in rats to investigate and compare the projections from the Sp5O and Sp5C to two major thalamic nuclei that relay ascending somatosensory information to the primary somatic sensory cortex: the ventroposteromedial thalamic nucleus (VPM) and the posterior thalamic nuclear group (Po). Results not only confirmed the existence of contralateral projections from the Sp5C to the VPM and Po, with retrogradely labelled neurons displaying a specific distribution in laminae I, III and V, they also showed consistent and similar numbers of retrogradely labelled cell bodies in the contralateral Sp5O. In addition, a topographic distribution of VPM projections from Sp5C and Sp5O was found: neurons in the dorsomedial parts of Sp5O and Sp5C projected to the medial VPM, neurons in the ventrolateral Sp5O and Sp5C projected to the lateral VPM, and neurons in intermediate parts of Sp5O and Sp5C projected to the intermediate VPM. All together, these data suggest that not only the Sp5C, but also the Sp5O relay somatosensory orofacial information from the brainstem to the thalamus. Furthermore, trigemino‐VPM pathways conserve the somatotopic distribution of primary afferents found in each subnucleus. These results thus improve our understanding of trigeminal somatosensory processing and help to direct future electrophysiological investigations.
Proprioceptive regulation of voluntary ankle movements, demonstrated using muscle vibration, is impaired by Parkinson’s disease

OBJECTIVE To test the hypothesis that the proprioceptive regulation of voluntary movement is disturbed by Parkinson’s disease, the effects of experimental stimulation of proprioceptors, using muscle vibration, on the trajectories of voluntary dorsiflexion movements of the ankle joint were compared between parkinsonian and control subjects. METHODS Twenty one patients with Parkinson’s disease, on routine medication (levodopa in all but one), and an equal number of age matched, neurologically intact controls, were trained initially to make reproducible ankle dorsiflexion movements (20° amplitude with a velocity of 9.7°/s) following a visual “go” cue while movement trajectories were recorded goniometrically. During 50% of the experimental trials, vibration (105 Hz; 0.7 mm peak to peak) was applied to the Achilles tendon during the ankle movement to stimulate antagonist muscle spindles; vibrated and non-vibrated trials were interspersed randomly. Subjects’ performance was assessed by measuring end point position—that is, the ankle angle attained 2 seconds after the visual “go” cue, from averaged (20 trials) trajectories. RESULTS Statistical analysis of the end point amplitudes of movement showed that, whereas the amplitudes of non-vibrated movements did not differ significantly between patients with Parkinson’s disease and controls, antagonist muscle vibration produced a highly significant reduction in the amplitudes of ankle dorsiflexion movements in both the patient and control groups. However, the extent of vibration induced undershooting produced in the patients with Parkinson’s disease was significantly less than that in the controls; the mean vibrated/non-vibrated ratios were 0.86 and 0.54 for, respectively, the patient and control groups. CONCLUSIONS The present finding of a reduction of vibration induced ankle movement errors in parkinsonian patients resembles qualitatively previous observations of wrist movements, and suggests that Parkinson’s disease may produce a general impairment of proprioceptive guidance.
Free living amoebae in water sources of critical units in a tertiary care hospital in India

Background: Isolation of free-living amoebae (FLA) is reported sparsely from water taps, ventilators, air conditioners, haemodialysis units and dental irrigation systems of hospitals worldwide. Their prevalence in hospital environment especially in wards having immunocompromised patients may pose a risk to this group of susceptible population as they may cause disease themselves or may carry pathogens inside them. No study from India has performed such surveillance. Objective: To evaluate extent of FLA contamination in water sources of bone marrow transplant (BMT) intensive care unit (ICU), transplant ICU, haemodialysis unit and high dependency unit in a tertiary care hospital in India. Materials and Methods: A total of hundred samples including fifty each of tap water samples and swabs from mouth of taps used for drinking, bathing and hand washing purposes in these units were collected according to standard procedure. Samples were inoculated onto non-nutrient agar plates at room temperature followed by morphological confirmation. Molecular identification including polymerase chain reaction (PCR) and sequencing was performed in culture positive samples. Results: Four tap water samples and ten swab samples showed growth of trophozoites and cyst formation. Morphologically, four amoebae resembled Acanthamoeba spp. which was further confirmed by PCR and sequencing showed them to be of T3 and T4 genotypes. Conclusion: The presence of these FLA in hospital water sources emphasises the urgent need of implementing effective preventive measures. Further studies are required to estimate the true prevalence of FLA in Indian hospitals by taking larger number of samples.
The Chevron Bunionectomy: A Trigonometric Analysis to Predict Correction

A trigonometric analysis was developed in an attempt to predict the postoperative hallux valgus angles and inter-metatarsal angles after Chevron bunionectomy. This analysis was used in 20 feet to predict postoperative angles, and these predicted values were compared with actual measured roentgenographic values. The postop erative intermetatarsal angles were evaluated to deter mine their correction to postoperative hallux valgus angles of 20° or less. Using the trigonometric analysis, the post operative intermetatarsal angles were highly predictable, but the hallux valgus angle could not be reliably predicted for individual patients. The intermetatarsal angle obtained at surgery, however, was indicative of the final hallux valgus angle: 75% of feet with intermetatarsal angles of more than 11° had postoperative hallux valgus angles of more than 20°. Thus, when an intermetatarsal angle of 11° or less can be obtained surgically, the hallux valgus angle can be corrected to 20° or less in most patients.
Genetic and Biochemical Analyses of thetec Operon Suggest a Route for Evolution of Chlorobenzene Degradation Genes

ABSTRACT The TecA broad-spectrum chlorobenzene dioxygenase ofBurkholderia sp. strain PS12 catalyzes the first step in the mineralization of 1,2,4,5-tetrachlorobenzene. The catabolic genes were localized on a small plasmid that belongs to the IncPβ incompatibility group. PCR analysis of the genetic environment of thetec genes indicated high similarity to the transposon-organized catabolic tcb chlorobenzene degradation genes of Pseudomonas sp. strain P51. Sequence analysis of the regions flanking the tecA genes revealed an upstream open reading frame (ORF) with high similarity to thetodF 2-hydroxy-6-oxo-2,4-heptadienoate hydrolase gene ofPseudomonas putida F1 and a discontinuous downstream ORF showing high similarity to the todE catechol 2,3-dioxygenase gene of strain F1. Both homologues in strain P51 exist only as deletion remnants. We suggest that different genetic events thus led to inactivation of the perturbing meta-cleavage enzymes in strains P51 and PS12 during the evolution of efficient chlorobenzene degradation pathways. Biochemical characterization of TodF-like protein TlpF and a genetically refunctionalized TodE-like protein, TlpE, produced in Escherichia coli provided data consistent with the proposed relationships.
Soluble CD163 inhibits phorbol ester-induced lymphocyte proliferation.

CD163 is a member of the scavenger receptor cysteine-rich family which is expressed exclusively on human monocytes and macrophages. Upon an inflammatory stimulus the protein is shed rapidly from the membranes' surface. CD163 expression is significantly upregulated by glucocorticoids and IL-10. While the membrane-bound form of CD163 was recently identified as scavenger receptor for hemoglobin-haptoglobin complexes, there is no information about a possible role of the shed soluble CD163. It has been suggested earlier that CD163 plays a pivotal role in the downregulatory phase of inflammation. However, it has remained elusive so far as to how this protein might influence the inflammatory process. We have now identified a potential direct anti-inflammatory effect mediated by soluble CD163. The highly purified protein statistically significantly inhibits phorbol ester-induced human T-lymphocyte activation, thus attenuating the immune response to the inflammatory mediator.
Mutations in ribosomal protein L3 and 23S ribosomal RNA at the peptidyl transferase centre are associated with reduced susceptibility to tiamulin in Brachyspira spp. isolates

The pleuromutilin antibiotic tiamulin binds to the ribosomal peptidyl transferase centre. Three groups of Brachyspira spp. isolates with reduced tiamulin susceptibility were analysed to define resistance mechanisms to the drug. Mutations were identified in genes encoding ribosomal protein L3 and 23S rRNA at positions proximal to the peptidyl transferase centre. In two groups of laboratory‐selected mutants, mutations were found at nucleotide positions 2032, 2055, 2447, 2499, 2504 and 2572 of 23S rRNA (Escherichia coli numbering) and at amino acid positions 148 and 149 of ribosomal protein L3 (Brachyspira pilosicoli numbering). In a third group of clinical B. hyodysenteriae isolates, only a single mutation at amino acid 148 of ribosomal protein L3 was detected. Chemical footprinting experiments show a reduced binding of tiamulin to ribosomal subunits from mutants with decreased susceptibility to the drug. This reduction in drug binding is likely the resistance mechanism for these strains. Hence, the identified mutations located near the tiamulin binding site are predicted to be responsible for the resistance phenotype. The positions of the mutated residues relative to the bound drug advocate a model where the mutations affect tiamulin binding indirectly through perturbation of nucleotide U2504.
Time for Waste, Waste of Time? Assessing Heterogeneous Values of Saving Time from Recycling

EnglishAlthough the opportunity cost of time spent recycling has long been recognized as a key determinant of household recycling participation, very few empirical studies have attempted to provide estimates of it. In this paper, we propose a model of household recycling that, while including pecuniary and non pecuniary motives for decisions, such as social or moral norms, reveals heterogeneous values of saving time from recycling (VSTR). The predictions of our model are tested using data from a discrete choice experiment on waste management conducted in 2008 in Corsica and estimating a latent-class rank-ordered logit model. We find VSTR clearly heterogeneous across individuals, ranging from 8 % to 76 % of one’s income. francaisBien qu’il soit clairement etabli que le cout d’opportunite du temps consacre au recyclage constitue un determinant essentiel des decisions des menages en matiere de tri des dechets, tres peu de travaux empiriques ont tente d’en fournir une estimation. Dans ce travail, nous proposons un modele de prise de decision des menages en termes de tri des dechets incluant des motivations pecuniaires et non-pecuniaires, telles que celles liees a des normes sociales ou morales, qui permet de mettre en evidence l’heterogeneite des valeurs du gain de temps consacre au recyclage. Les resultats de notre modele sont testes en utilisant des donnees issues d’une etude basee sur la methode des choix experimentaux discrets conduite en 2008 en Corse et estimees en utilisant un modele logit de classement ordonne a classes latentes. Les resultats permettent d’etablir que les valeurs du gain de temps consacre au recyclage sont fortement heterogenes entre les individus puisqu’elles sont comprises entre 8 % et 76 % de leur revenu.
Photoelectric Hybrid Current Sensor Combination of LPCT and FFI

A new kind of photoelectric hybrid current sensor is designed. The low-power current transformer (LPCT) is used as the primary sensor for current conversion. The optical fiber Fizeau interferometer acts as the secondary sensor for noncontact microvibration detection. An electro-dynamic structure works as a bridge connecting the two sensors. Using two different ways as frequency response analysis and linear fitting method, similar sensitivity results are obtained. It is considered to be 3.5152 nm/A when the current frequency is 50 Hz. When LPCT works at the rated primary current of 450 A, the maximum measurement ratio error and phase displacement are <;0.25% and 0.066 rad, respectively.
Active polysomes in the axoplasm of the squid giant axon

Axons and axon terminals are widely believed to lack the capacity to synthesize proteins, relying instead on the delivery of proteins made in the perikaryon. In agreement with this view, axoplasmic proteins synthesized by the isolated giant axon of the squid are believed to derive entirely from periaxonal glial cells. However, squid axoplusm is known to contain the requisite components of an extra‐mitocliondrial protein synthetic system, including protdn factors, tRNAs, rRNAs, and a heterogeneous family of mRNAs. Hence, the giant axon could, in principle, maintain an endogenous protein synthetic capacity. Here, we report that the squid giant axon also contains active polysomes and niRNA, which hybridizes to a riboprobe encoding murine neurofilament protein. Taken together, these findings provide direct evidence that proteins (including the putative neuron‐specific neurofilament protein) are also synthesized de novo in the axonal compartment.
Study on farmers land consolidation adaptation intention

Purpose The purpose of this paper is to examine the effects of media and social network in the development of farmers land consolidation (LC) awareness, perception and adaptation intention decisions. Design/methodology/approach A face-to-face interview was carried out with randomly selected farmers from three districts (Neijiang, Ya’an and Dujiangyan) of Sichuan province, China. A structural equation modeling (SEM) technique was used to test the conceptual framework of this study. The SEM was set to confirmatory factor analysis of the measurement and structural models for maximum likelihood estimate by means of IBM-AMOS to capture the models goodness-of-fit. Findings The result indicates that social network contributes a significant role in the development of farmers LC awareness, perception and adaptation intention decisions than media. The total (direct and indirect) effect of social network and media toward the farmers LC adaptation intention is 56.7 and 14 percent, respectively. When farmers are aware and perceived of LC and rehabilitation program, their adaptation intention improves significantly. Therefore, social network is a useful tool in improving the awareness, perception and adaptation intention decisions of Sichuan farmers compared to media. Originality/value This study tests the applicability of SEM techniques to understand the farmers LC adaptation intentions; and synthesizes the impact of social network and media in the development of the farmers LC awareness, perception as well as adaptation intentions. This study serves as an outline for assessing the adaptation intention of farmers toward sustainable land management.
CD200-Fc, a novel antiarthritic biologic agent that targets proinflammatory cytokine expression in the joints of mice with collagen-induced arthritis.

OBJECTIVE The CD200 receptor (CD200R) is an inhibitory receptor expressed by myeloid cells that is postulated to play an important role in regulation of the immune system. The purpose of this study was to evaluate the efficacy of a soluble ligand of CD200R in established collagen-induced arthritis (CIA) in mice and to analyze changes in cytokine expression following therapy in order to understand its primary mechanism of action. METHODS Arthritis was induced in DBA/1 mice, and CD200-Fc fusion protein, an isotype control monoclonal antibody, or TNFR-Fc fusion protein was administered over a period of 10 days (total of 4 doses). Cytokine expression in the joint was assessed by flow cytometry, enzyme-linked immunosorbent assay, and quantitative real-time polymerase chain reaction. RESULTS CD200-Fc significantly reduced the severity of established arthritis at the clinical and histologic levels. The therapeutic effect of CD200-Fc at 1 mg/kg was comparable with that of TNFR-Fc at 4 mg/kg. CD200R was found to be expressed in arthritic synovia and in lymph nodes, yet no changes in T cell cytokine levels (interferon-gamma, interleukin-5 [IL-5], IL-10, IL-17) were detected after CD200-Fc therapy. There was no evidence of an expansion of forkhead box P3-positive regulatory T cells or a change in serum anticollagen IgG1 and IgG2a levels. However, administration of CD200-Fc markedly decreased the expression of messenger RNA for tumor necrosis factor alpha, IL-1beta, IL-10, and matrix metalloproteinase 13 in the joint to the same extent as administration of TNFR-Fc. CONCLUSION CD200-Fc is an effective therapeutic agent in established CIA that targets proinflammatory cytokine expression in the joint without any obvious systemic immunosuppressive effects. Our findings indicate that CD200-Fc has considerable potential as a novel therapeutic agent in rheumatoid arthritis in humans.
Psychometric Analysis of the Selective Reminding Procedure in a Sample from the General Elderly Population

The selective reminding procedure (SRP) has been proposed for the assessment of distinct aspects of episodic memory, i.e. storage to and retrieval from short-term and long-term memory, item learning and list learning, and as dementia screening tool. In the present study SRP results were analysed in 256 probands from the general elderly population. SRP scores were highly intercorrelated, and principal component analysis yielded only one single factor. The SRP scores were moderately and not differentially correlated with immediate and delayed free recall and recognition and with verbal fluency. All SRP scores discriminated nondemented probands with episodic long-term memory impairment from those without. The MMSE performed significantly better than any SRP score in detecting dementia. The theory-based assumption that the SRP allows assessment of different, independent aspects of memory could not be validated. It is suggested that the SRP is a mixed measure of semantic memory, episodic long-term and short-term memory, and working memory, and that the different SRP scores do not allow to assess different memory functions. Thus, the SRP may neither be recommended for assessment of different subfunctions of memory nor for dementia screening.
Reconfiguration of balanced and unbalanced distribution systems for cost minimization

Distribution system reconfiguration is very important operational aspect of distribution systems. When distribution system is fed from different substations/feeders, and various types of generating stations owned by various agencies, energy purchase cost may vary from owner to owner. In such cases formulating reconfiguration problem with loss minimization may not be acceptable. It is important to design the appropriate optimization techniques to find optimal solutions. Mostly, the used approaches are metaheuristics in nature. Few heuristic methods are presented but limited to balanced systems only. Hence, the motivation in this paper is to develop a simple heuristic method for distribution system reconfiguration, while minimizing the real power purchase cost. The proposed method is suitable for both balanced and unbalanced distribution systems. The algorithm is tested on two balanced and unbalanced distribution systems and results are verified with the ones obtained with more rigorous improved harmony search (IHS) technique.
Carrier Mobility in Undoped Triple-Gate FinFET Structures and Limitations of Its Description in Terms of Top and Sidewall Channel Mobilities

This paper presents an extensive experimental study of the effective mobility in the long-channel undoped triple-gate FinFETs. The mobility behavior in FinFETs is studied as compared with that in quasi-planar very wide fin FETs made on the same wafers and as a function of the fin width. Devices with two types of the gate dielectrics are investigated. New insights about the carrier transport in triple-gate FinFETs are obtained using a careful analysis of the results for various fin widths. In particular, it is shown that the model treating the triple-gate FinFET in terms of the (100) top and (110) lateral channels is not perfectly accurate for describing the transport properties in real FinFETs with relatively narrow fins; at low and moderate inversion charge densities, this is due to different inversion carrier distributions (and, thus, nonidentical scattering rates for various fin widths), and at high charge densities, it is presumably due to fin rounding which results in ambiguous crystallographic orientation, different from the postulated (100) and (110).