Miracle-dz/UniFAR-FTUs huggingface dataset:
Classic and generalized variants of Pick's disease: A clinicopathological, ultrastructural, and immunocytochemical comparative study

Six sporadic cases of dementia with lobar atrophy and neuronal cytoplasmic inclusions (Pick's disease) could be separated into two groups on the basis of the involvement of subcortical structures, the distribution and the histochemical, immunochemical, and ultrastructural characteristics of the inclusions, and possibly the age at onset. The first group (classic) was characterized by predominantly cortical atrophy and the presence in the hippocampus and neocortex of argyrophilic cytoplasmic inclusion bodies that reacted with a monoclonal antibody against neurofilament proteins and antitubulin antisera. Ultrastructurally the bodies were composed of straight fibrils of variable diameter, averaging 15 nm, and long‐period constricted fibrils. The second group (generalized) showed subcortical as well as cortical atrophy. Cortical and subcortical cytoplasmic inclusions contained RNA and stained poorly with silver and antibodies against neurofilaments and microtubules. Ultrastructurally the straight fibrils composing the bodies were coated with granular material, presumed to be derived from ribosomes. The generalized cases occurred in younger patients than did the classic cases in this series.
Graft-versus-host disease following autologous transplantation.

269 K . T. , a 76-year-old woman, was diagnosed with stage IIA multiple myeloma in 2007 when she presented with severe back pain. K.T. underwent kyphoplasty, a minimally invasive surgery to relieve compression fractures, followed by eight cycles of lenalidomide with a good response. Peripheral blood stem cells were collected after mobilization with filgrastim and plerixafor. She was admitted to the hospital and conditioned for an autologous hematopoietic cell transplantation (HCT) with melphalan followed by reinfusion of peripheral blood stem cells, receiving a cell dose of 4.26 x 106 CD34 cells/kg. K.T. developed neutropenic fever on day 8 that responded to broad spectrum antimicrobials. She achieved a prompt hematologic recovery, reaching an absolute neutrophil count greater than 500 mm3 by day 11 after transplantation. On day 9, however, K.T. developed diarrhea, negative for Clostridium difficile, which was managed with antimotility agents. She was discharged 17 days following transplantation with blood counts within normal limits, except for mild anemia. On day 19, K.T. presented for follow-up with a pruritic, erythematous skin rash covering 45% of her body surface area, involving her upper extremities, anterior, and posterior torso. She also experienced gastrointestinal (GI) symptoms of nausea, abdominal cramping, weight loss, and anorexia. Tapered methylprednisolone dosing was prescribed for a suspected diagnosis of autologous graft-versus-host disease (GVHD). Skin rash resolved in response to corticosteroids and pruritis was managed with topical triamcinolone. GI symptoms initially improved, but recurred following completion of the methylprednisolone taper. On day 32, K.T. presented with progressive diarrhea and diffuse skin rash involving 100% of her body surface area. Medication review did not reveal a likely source for drug reaction. A skin biopsy was taken and showed foci of basal cell vacuolization with apoptosis consistent with acute GVHD. K.T. received a dose Graft-Versus-Host Disease Following Autologous Transplantation
EFFECT OF TARGET CONTENT AT HIGHER SPATIAL FREQUENCIES ON THE ACCURACY OF THE ACCOMMODATION RESPONSE

Abstract The accommodation responses to sinusoidal and square‐wave gratings at the same vergence (‐5.0 dioptres) are compared as a function of the modulation of the fundamental of both waveforms. At low spatial frequencies (< 2 c deg‐1), responses to square‐wave gratings are found to be more accurate than those to sine‐waves. At higher frequencies the responses become very similar. An explanation is offered in terms of the contribution of higher harmonics to the square‐wave response.
Semi-analytical minimum time solutions for a vehicle following clothoid-based trajectory subject to velocity constraints

A possible strategy for finding the optimal path that connects two different configurations of a car-like vehicle can be addressed by solving two different sub-problems: 1. identifying a curve that connects the two points respecting the geometric constraints, 2. finding an optimal control strategy that drives the vehicle along the trajectory accounting for its dynamic constraints. In this paper, we focus on the second problem. Assuming that the vehicle moves along a specified clothoid, we find a semi-analytical solution for the optimal profile of the longitudinal acceleration (the control variable). Our technique explicitly considers non-linear dynamics, aerodynamic drag effect and bounds on the longitudinal and on the lateral acceleration.
Patent classifications as indicators of intellectual organization

Using the 138,751 patents filed in 2006 under the Patent Cooperation Treaty, co-classification analysis is pursued on the basis of three- and four-digit codes in the International Patent Classification (IPC, 8th edition). The co-classifications among the patents enable us to analyze and visualize the relations among technologies at different levels of aggregation. The hypothesis that classifications might be considered as the organizers of patents into classes, and that therefore co-classification patterns--more than co-citation patterns--might be useful for mapping, is not corroborated. The classifications hang weakly together, even at the four-digit level at the country level, more specificity can be made visible. However, countries are not the appropriate units of analysis because patent portfolios are largely similar in many advanced countries in terms of the classes attributed. Instead of classes, one may wish to explore the mapping of title words as a better approach to visualize the intellectual organization of patents.
Differences in the cell wall architecture of melanin lacking and melanin producing Cryptococcus neoformans clinical isolates from India: an electron microscopic study

Cryptococcus neoformans e um importante fungo oportunista patogenico que causa infeccao no sistema nervoso central, e que pode levar o paciente a morte. Um dos principais fatores de virulencia do C. neoformans e a producao de melanina na parede celular. Utilizando microscopia eletronica de transmissao, nos estudamos as paredes celulares de tres pares de isolados obtidos de pacientes com dupla infeccao pelo fungo, onde um isolado melanizado e um albino foram isolados do liquor de cada paciente. A microscopia eletronica de transmissao revelou que as cepas albinas nao apresentavam a camada de melanina enquanto que uma camada de melanina estava associada com a parede celular de cepas melanoticas, constituindo aproximadamente 75% da area da parede celular. O tamanho da parede celular das celulas produtoras de melanina foi aproximadamente o dobro do tamanho da parede celular dos isolados albinos (p < 0,003). Neste estudo, a microscopia eletronica de transmissao revelou que as diferencas na estrutura dos isolados albinos sem melanina e dos isolados produtores de melanina estava associada a parede celular e a camada de melanina.
Lack of efficacy of etanercept in Sjögren syndrome correlates with failed suppression of tumour necrosis factor α and systemic immune activation

Objective: To provide insight into the clinical failure of the tumour necrosis factor α (TNFα) inhibitor, etanercept, in primary Sjögren syndrome (pSS), an extensive analysis of the systemic immune profile of patients with pSS was carried out and the effect of etanercept treatment on these immune parameters monitored. Methods: Peripheral blood mononuclear cells of patients with pSS and healthy controls were compared by flow cytometry to determine differences in distribution of specific cell populations (T cells, B cells, monocytes), and to determine their expression of activation markers (CD25, HLA-DR), TNF receptors and chemokine receptors (CXCR1, 2) before and after treatment. Systemic cytokine levels were measured by multiplex ELISA assay in plasma and in lipopolysaccharide-stimulated whole blood from healthy controls and from patients with pSS before and after etanercept treatment. Baseline cytokine levels were correlated with clinical markers of disease. Results: Before treatment, salivary gland inflammatory focus scores did not correlate with circulating TNF levels. Furthermore, consistent with the lack of evidence of significant clinical benefit, enhanced markers of immune activation, frequency of cell subpopulations and aberrant cytokine profiles were not restored to normal levels by etanercept treatment. Remarkably, the levels of circulating TNFα were significantly increased after treatment. Conclusion: Etanercept is an ineffective therapeutic agent in pSS consistent with the absence of suppression of TNFα and other indicators of immune activation in this patient population. These data suggest that TNFα may not be a pivotal cytokine in the pathogenesis of pSS, impelling continued molecular characterisation of disease parameters to define appropriate intervention targets.
Clinical consequences of defects in peroxisomal β-oxidation

The disorders of peroxisomal β-oxidation, which have been well characterised at the molecular level, include defects of acyl-CoA oxidase, defects of the D-bifunctional protein (D-BP) (including specific defects of its enoyl-CoA hydratase and D-3-hydroxyacyl-CoA dehydrogenase components), defects of the very-long-chain fatty acid (VLCFA)-CoA importer [X-linked adrenoleukodystrophy (ALD)] and α-methylacyl-CoA racemase deficiency. A survey of the clinical consequences of these defects indicates that defects in the acyl-CoA oxidase and D-BP can produce neonatal hypotonia, seizures in early infancy, retinopathy and progressive neurological dysfunction with leukodystrophy on imaging. Defects in the VLCFA-CoA importer and in the racemase do not produce disease until a long time after the neonatal period. However, again the clinical picture is dominated by neurological disease: impaired cognitive function with leukodystrophy in childhood X-linked ALD and retinopathy and neuropathy in racemase deficiency. It is difficult to escape the conclusion that defective peroxisomal β-oxidation has effects (such as impaired neuronal migration in the developing brain), which are more serious than those produced by the accumulation of substrates (VLCFAs, pristanic acid) alone.
Mu opioid receptor gene polymorphisms and heroin dependence in Asian populations

The distribution of three polymorphisms of the mu opioid receptor gene (OPRM1) was investigated in four different Asian populations, and in heroin-dependent subjects deriving from three of these populations. For the A118G polymorphism, we found significant differences in allele and genotype frequencies between different ethnic groups and highly significant association with heroin dependence in Indians for both genotype distribution (p = 0.024) and allele frequency (p = 0.009). For the C17 T polymorphism, the minor allele was documented in Chinese and Malays for the first time. Molecular haplotyping revealed complete linkage dis-equilibrium between the A118G and C17 T polymorphisms. Linkage disequilibrium between the A118G and C1031G polymorphisms was found to be almost complete in all four ethnic groups.
Clinical and scientific perspectives on movement disorders: Stanley Fahn's contributions

Dr. Stanley Fahn, the H. Houston Merritt Professor of Neurology and Director Emeritus of the Center for Parkinson's Disease and Other Movement Disorders at Columbia University, one of the founders of the field of movement disorders, was the first president of the Movement Disorders Society (subsequently renamed as the International Parkinson and Movement Disorder Society). Together with his friend and colleague, Professor David Marsden, he also served as the first co‐editor of the journal Movement Disorders. By emphasizing phenomenology as the key element in differentiating various hypokinetic and hyperkinetic movement disorders, Dr. Fahn drew attention to the clinical history and the power of observation in the diagnosis of movement disorders. Dr. Fahn had major influence on the development of classifications and assessments of various movement disorders and in organizing various research groups such as the Parkinson Study Group. As the founder and president of the World Parkinson Coalition and an organizer of the initial three World Parkinson Congresses, he has demonstrated his long‐standing commitment to the cause of including patients as partners. The primary goal and objective of this invited review is to highlight some of Dr. Fahn's most impactful scientific and clinical contributions to the understanding and treatment of Parkinson's disease, dystonia, and other movement disorders. © 2015 International Parkinson and Movement Disorder Society
Correlates of Physical Activity in a Cypriot Sample of Sixth-Grade Children

This study examined the association between self-reported physical activity and a number of potential correlates in a sample of 256 Grade-6 children. Physical activity was assessed in both summer and winter, and children and parents completed questionnaires assessing potential correlates of physical activity. Analyses revealed that gender, time spent playing outside, self-efficacy in overcoming barriers, and number of items of exercise equipment at home were variables associated with physical activity in both seasons. School location was a variable associated with physical activity only in the summer, whereas private-lesson attendance, sports-club attendance, and best friend’s physical activity were variables associated with physical activity only in winter. Variance explained in physical activity were 42% and 51% in winter and summer, respectively.
The Crystal Structure of Iron-free Human Serum Transferrin Provides Insight into Inter-lobe Communication and Receptor Binding*

Serum transferrin reversibly binds iron in each of two lobes and delivers it to cells by a receptor-mediated, pH-dependent process. The binding and release of iron result in a large conformational change in which two subdomains in each lobe close or open with a rigid twisting motion around a hinge. We report the structure of human serum transferrin (hTF) lacking iron (apo-hTF), which was independently determined by two methods: 1) the crystal structure of recombinant non-glycosylated apo-hTF was solved at 2.7-Å resolution using a multiple wavelength anomalous dispersion phasing strategy, by substituting the nine methionines in hTF with selenomethionine and 2) the structure of glycosylated apo-hTF (isolated from serum) was determined to a resolution of 2.7Å by molecular replacement using the human apo-N-lobe and the rabbit holo-C1-subdomain as search models. These two crystal structures are essentially identical. They represent the first published model for full-length human transferrin and reveal that, in contrast to family members (human lactoferrin and hen ovotransferrin), both lobes are almost equally open: 59.4° and 49.5° rotations are required to open the N- and C-lobes, respectively (compared with closed pig TF). Availability of this structure is critical to a complete understanding of the metal binding properties of each lobe of hTF; the apo-hTF structure suggests that differences in the hinge regions of the N- and C-lobes may influence the rates of iron binding and release. In addition, we evaluate potential interactions between apo-hTF and the human transferrin receptor.
Reproducibility of responses to telephone interviews: demographic predictors of discordance in risk factor status.

The reproducibility of responses to the Behavioral Risk Factor Surveillance System questionnaire was examined across the demographic strata used by the Centers for Disease Control and Prevention and state health departments for reporting prevalence estimates (specifically age, sex, income, employment, and marital status), as well as race/ethnicity, which has been previously examined. The authors administered the questionnaire twice, 21-94 days apart, to randomly selected residents of Massachusetts (response rates: first administration, 68% of eligible households; second administration, 68% of persons who completed the first interview). Initial interviews were conducted in March and October 1992. Among 448 respondents to both interviews, group mean distributions of seven demographic characteristics and 19 risk factors were highly consistent across the two interviews. Discordance in individual risk factor status ranged from 1.2% to 21.8% (median, 7.8%) and was symmetric in direction, i.e., as many respondents were considered at increased risk on the basis of the first interview and at low risk on the basis of the second interview as the reverse. Kappas ranged from 0.30 to 0.90 (median, 0.75). Education, household income, and interval between administrations were not associated with prevalence of discordance for any risk factor. Sex, age, race/ethnicity, marital status, and employment status were each predictive of variation in discordance for one or more risk factors, but no consistent effect of any individual demographic characteristic across risk factors was observed. The questionnaire has relatively uniform and generally good reproducibility across all demographic strata used for monitoring the Health Objectives for Year 2000 and other chronic disease surveillance activities.
Vestibuloplasty: porcine collagen matrix versus free gingival graft: a clinical and histologic study.

BACKGROUND A free gingival graft (FGG) is currently the gold standard for augmenting small areas of keratinized mucosa. The porcine collagen matrix (CM) represents an alternative to autologous tissue harvesting. This study aims to compare the CM versus FGGs for augmenting keratinized peri-implant mucosa based on clinical and histologic evaluations. METHODS The study included 14 patients who underwent a vestibuloplasty with either a FGG from the palate (n = 7) or the CM (n = 7). An implant-fixed vestibular retention splint was inserted for 30 days. Follow-up examinations were performed at 4, 10, 30, and 90 days after surgery. Width of keratinized mucosa was measured in the region of each implant (days 10, 30, and 90). After 90 days, a biopsy was harvested for histologic and immunohistologic analyses. To characterize newly formed soft tissue, the authors stained for tissue-and differentiation-specific markers, cytokeratin (CK) 5/6, 13, and 14, to detect presence or absence of keratinization. RESULTS The groups showed similar healing, with increased peri-implant keratinized mucosa. The CM group had overall significantly shorter operation times than the FGG group. Both groups showed similar overall shrinkage (32.98% CM versus 28.35% FGG). All biopsies showed a multilayered, keratinized, squamous epithelium. CKs 5/6 and 14 were detected in the basal and suprabasal layers, and spots of CK 13 were detected in the suprabasal layer. CONCLUSIONS During the whole observation period, both groups showed comparable clinical and histologic outcomes. Within the limitations of the present study, CM seems to be a promising alternative for the regeneration of keratinized mucosa without tissue harvesting. Comparative long-term studies are needed to investigate changes over time.
The Prevalence of Dental, Facial and Head Injuries Sustained by Schoolboy Rugby Players. A Pilot Study

Aim The aim of the study was to determine the prevalence of dental, orofacial and head injuries and of mouthguard use among schoolboy rugby players. Materials and methods All members of the first and second XV rugby squads at three secondary schools (two in England and one in Australia) were included in the study. All participants answered a questionnaire that sought information regarding dental, orofacial and head injuries. Statistical differences between groups were determined using chi-square tests for categorical variables and Kruskal-Wallis tests for discrete (count) variables. Results One hundred and seventy-eight completed questionnaires were returned (100% response rate). One hundred and twenty-five (70%) players reported having sustained at least one injury (range 1–4). Facial injuries were common. Dental injuries were the most prevalent injury: 46 (26%) were reported. Fractured teeth were reported by 20 (11%) players, and avulsed teeth by 7 (4%). There was evidence of a difference between schools in the prevalence of injured players (P=0.014), but among those reporting injuries, there was no difference between schools in the number of injuries (P=0.95). All players said that they used a mouthguard regularly. Conclusions Dental, orofacial and head injuries were commonly reported. Dental injuries were the most prevalent type of injury. All players used mouthguards regularly.
Stress, Distress, and Bodytalk: Co-constructing Formulations with Patients Who Present with Somatic Symptoms.

In the context of stress-internal or external events that threaten the individual's physical or psychological well-being-the human body signals distress along with disruptions in physiological regulation. When stress-related disruptions are extreme or are not limited in time, they may result in a broad range of somatic, behavioral, and cognitive symptoms. This article aims to (1) provide clinicians with a theoretical framework for understanding the body systems that mediate stress-induced somatic symptoms, and (2) illustrate how this framework can be applied clinically. The article begins with a brief overview of the key body systems involved in homeostasis, paying special attention to how those systems take on self-protective functions in the face of stress. Against that background, the focus then turns to a discussion of commonly occurring somatic symptoms and their probable neurophysiological underpinnings. Short vignettes illustrate typical presentations and how potential etiologies can be discussed with patients, be used to co-construct formulations, and be integrated into jointly determined treatment plans.
Social Comparison Affects Reward-Related Brain Activity in the Human Ventral Striatum

Whether social comparison affects individual well-being is of central importance for understanding behavior in any social environment. Traditional economic theories focus on the role of absolute rewards, whereas behavioral evidence suggests that social comparisons influence well-being and decisions. We investigated the impact of social comparisons on reward-related brain activity using functional magnetic resonance imaging (fMRI). While being scanned in two adjacent MRI scanners, pairs of subjects had to simultaneously perform a simple estimation task that entailed monetary rewards for correct answers. We show that a variation in the comparison subject's payment affects blood oxygenation level–dependent responses in the ventral striatum. Our results provide neurophysiological evidence for the importance of social comparison on reward processing in the human brain.