Miracle-dz/UniFAR-FTUs huggingface dataset:
Clinical, electrophysiologic, and pathologic evidence for sensory abnormalities in ALS

Background: Amyotrophic lateral sclerosis (ALS) is a progressive degenerative disease of upper and lower motor neurons. Reports of the nature and frequency of sensory nerve involvement in ALS have varied. Methods: We reviewed the Emory University motor neuron disease registry between 1997 and 2004 to identify 103 patients with ALS without coexisting diseases that might cause sensory abnormalities and for whom electrodiagnostic studies were available for review. Neurophysiologic studies were interpreted based on age-adjusted normative data from our laboratory. Twelve control biopsies were evaluated alongside 22 samples from patients with ALS to ensure blinded evaluation of pathologic specimens. Results: Sensory symptoms or signs were present in 32% of patients, sural sensory nerve action potential amplitudes were abnormal in 27%, and pathologic abnormalities were present in 91% of patients. Large-caliber myelinated fibers were predominantly affected (reduced in 73%) and small-caliber myelinated fibers were affected less often (23%). Thinly myelinated fibers were present in 95% and regenerating clusters in 77% of the biopsies. Teased fiber analysis showed an increased frequency of axonal degeneration and regeneration as well as excessive myelin irregularity. Morphometry confirmed the loss of large-caliber fibers. Conclusions: These data indicate that one third of patients with amyotrophic lateral sclerosis report sensory symptoms and sural sensory response amplitudes are reduced in a similar proportion of subjects. Pathologic evidence of sensory nerve pathology was present in 91% of patients who underwent sural nerve biopsy. The electrophysiologic and pathologic findings indicate a pattern of axonal loss that predominantly affects large-caliber myelinated fibers.
Playing It Safe: Assessing Cumulative Impact and Social Vulnerability through an Environmental Justice Screening Method in the South Coast Air Basin, California

Regulatory agencies, including the U.S. Environmental Protection Agency (US EPA) and state authorities like the California Air Resources Board (CARB), have sought to address the concerns of environmental justice (EJ) advocates who argue that chemical-by-chemical and source-specific assessments of potential health risks of environmental hazards do not reflect the multiple environmental and social stressors faced by vulnerable communities. We propose an Environmental Justice Screening Method (EJSM) as a relatively simple, flexible and transparent way to examine the relative rank of cumulative impacts and social vulnerability within metropolitan regions and determine environmental justice areas based on more than simply the demographics of income and race. We specifically organize 23 indicator metrics into three categories: (1) hazard proximity and land use; (2) air pollution exposure and estimated health risk; and (3) social and health vulnerability. For hazard proximity, the EJSM uses GIS analysis to create a base map by intersecting land use data with census block polygons, and calculates hazard proximity measures based on locations within various buffer distances. These proximity metrics are then summarized to the census tract level where they are combined with tract centroid-based estimates of pollution exposure and health risk and socio-economic status (SES) measures. The result is a cumulative impacts (CI) score for ranking neighborhoods within regions that can inform diverse stakeholders seeking to identify local areas that might need targeted regulatory strategies to address environmental justice concerns.
When Is Handcrafting Not a Curse

Recently, with the proliferation of deep learning, there is a strong trend of abandoning handcrafted systems/features in machine learning and AI by replacing them with “end-to-end” systems “learned from scratch”. These learning paradigms have achieved tremendous success. Researchers show that learning based algorithms are general — they can be applied to new domains and achieve good performance. In contrast, handcrafted systems are becoming the machine learning new “taboo” that is repeatedly criticized in recent papers. Merely motivated by the idea of critical thinking, we ask this question: are handcrafted systems really always a curse? is there any hidden merit of it? In this short report, we discuss when handcrafted systems can in principle be used to solve tasks in new domains. We also discuss why sometimes handcrafted systems can be preferred. This work was supported by the Center for Brains, Minds and Machines (CBMM), funded by NSF STC award CCF 1231216. 1Identical to the 06/2017 Github version with only a few typo/words corrected. 2We have two other similar papers to this, all shared internally at our Github since 06/2017. One of them was publicly released in 09/2017 [1]. These three position papers directly motivated our work on “object-oriented deep learning” and a few other handcrafted/symbolic projects in 2017.
Woman and Management: A Conceptual Review, with a Focus on Muslim Women in Management Roles in Western and in Muslim-Majority Countries

We consider the roles of Muslim women managers, executives and professionals in three ways: as women qua women; as women of the Muslim diaspora in Western countries; and as women in Muslim Majority Cultures (MMCs). In reviewing the literature on the “glass ceiling” which prevents women achieving parity with men in middle and senior management roles, we are impressed by the work of Helgeson and Johnson on the special qualities of women in management roles, and compare their social psychological model with accounts of “strong” Muslim women who, drawing on Islamic traditions have performed many leadership roles outside of the traditional family. Nevertheless, it is Muslim women’s strength as strong family managers, with men being supporters rather than leaders of such family roles, which we generalise in describing an ideal model of a Muslim women manager. We draw too on traditional Islamic models in describing the kind of capitalist enterprise which may thrive in MMCs, and in which women will play leading roles. Nevertheless, we observe that in many MMCs non-Muslim (but strong) traditions of patriarchy often prevail, and subvert women’s search for higher education and leadership roles. This review advocates reforms in MMCs which will allow women to enter the world of employment, and to rise to higher managerial positions. At the same time, we observe the strong prejudices against Muslim women entering managerial positions in Western cultures, based on multiple, intersecting prejudice and discrimination against women; against visible ethnic minorities; and against those who are visibly Muslim. Further qualitative work in this field is advocated.
What Is Known About Punctuated Equilibrium Theory: And What Does That Tell Us About the Construction, Validation and Replication of Knowledge in the Policy Sciences?

This article reviews how the Punctuated Equilibrium Theory (PET) has been applied in studies analyzing policy change. It builds on a systematic evidence synthesis of peerâ€ reviewed empirical literature that is based on the core readings by Frank R. Baumgartner and Bryan D. Jones. The article identifies trends in the geographical and policy focus of PET studies; maps, explores, and interrogates how core concepts of PET are applied in empirical analyses; and, finally, assesses the analytical and explanatory power of PET when applied to empirical phenomena. The article finds that PET studies have contributed a great deal to our knowledge on particular cases, but that more often than not PET is applied in a selective manner. The article deals with the implications of these findings for further theory development in policy analysis.e—´ae–­a¹³eiic †e®oaeœ‰a“aao›a†…a®¹i¼Ÿa®ƒa¯¹ae”? c­–c§‘a­¦a¸­a…³aoŽcŸ¥e¯†aež„a»oa€ eaŒe¯ a’Œa¤ aˆ¶cs„cœ‹ae³•ae˜¯a»€a¹ˆi¼Ÿ aeœ¬ae–‡ae£€eaŒao†e—´ae–­a¹³eiic †e®oi¼ˆPunctuated Equilibrium Theory, PETi¼‰a¦‚a½•e¢«ao”c”¨aoŽaˆ†aež ae”? c­–a ˜aŒ–cs„c ”c©¶a€‚aeœ¬ae–‡aŸoaoŽa¸€ei¹e’ˆa¯¹a ŒeiŒe¯„a®ia®že¯ ae–‡cŒ®cs„c³»c»Ÿae€§e¯ ae ®c»¼a ˆ, a Že€…aŸoaoŽa¼—a…°a…‹Â·e² a§†aS c‰¹co³i¼ˆFrank R. Baumgartneri¼‰a’Œa¸ƒeµ–ae ©Â·c ¼ae–¯i¼ˆBryan D. Jonesi¼‰ae a‡ocs„ae ¸a? ƒe§‚c‚¹a€‚ae–‡c« e¯†aˆ«ao†PET c ”c©¶a¸­aœ°c †c„¦c‚¹a’Œae”? c­–c„¦c‚¹cs„e¶‹aS? i¼›a Œae—¶c¼–aeŽ’a€ aeŽ¢c´¢a€ e´¨c–‘ao†PET ae ¸a? ƒae¦‚a? µa¦‚a½•ao”c”¨aoŽa®že¯ aˆ†aež i¼›aeœ€a Že¯„a¼°ao†PETcs„aˆ†aež eƒ½aS›a’Œe§£e‡Seƒ½aS›i¼ˆa½“ao”c”¨aoŽa®že¯ cŽ°e±iae—¶i¼‰a€‚aeœ¬ae–‡a ‘cŽ°, PETc ”c©¶aœ¨aeŸ ao›aeiˆa¾‹a¸­ae a¾›ao†c›¸a½“a¤scs„cŸ¥e¯†, a½†ae›´a¤scs„aeƒ…a†µaˆ™ae˜¯, PETae˜¯a¸€c§ e€‰ae‹©ae€§cs„ao”c”¨a€‚ae–‡c« ae a¾›ao†e? ™ao›c ”c©¶a ‘cŽ°cs„ae„ a¹‰, c”¨aoŽae”? c­–aˆ†aež cs„e? ›a¸€ae­¥c †e®oa ‘a±•a€‚Â? QuA© Se Sabe De La TeorA­a Del Equilibrio Puntuadoâ€”y QuA© Nos Dice Eso Acerca De La ConstrucciA³n, ValidaciA³n y ReplicaciA³n De Conocimiento En Las Ciencias PolA­ticas? Este artA­culo examina cA³mo la teorA­a del equilibrio puntuado (PET) ha sido aplicada en estudios que analizan los cambios polA­ticos. Le agrega a una sA­ntesis de evidencia sistemAitica de estudios empA­ricos revisados en pares que estAi basada en las lecturas centrales de Frank R. Baumgartner y Bryan D. Jones. El artA­culo identifica patrones en el enfoque geogrAifico y polA­tico de los estudios PET; esquematiza, explora e interroga cA³mo los conceptos centrales de la PET se aplican a los anAilisis empA­ricos; y finalmente, evalAoa el poder analA­tico y explicativo de la PET cuando se aplica a fenA³menos empA­ricos. El artA­culo encuentra que los estudios de PET han contribuido mucho a nuestro conocimiento de casos particulares, pero que a menudo la PET se aplica de una manera selectiva. El artA­culo tiene que ver con las implicaciones de estos hallazgos para avanzar el desarrollo de las teorA­as en el anAilisis polA­tico.
Healthy Food Availability Among Food Sources in Rural Maryland Counties

ABSTRACT Most studies examining the association of the food environment and health outcomes have focused on urban settings. However, rural adults and children have high rates of obesity, which may be related to their food environments. This study examines the food environment in 7 rural counties in Maryland. A cross-sectional study was conducted at 244 retail food stores from 2012 to 2013. Data on store-level characteristics were collected and a healthy food availability index (HFAI) ranging from 0 to 31 points was calculated for each store. Convenience stores (18.9%) and other nontraditional stores (16.8%) were the most common store types, and box stores were the least (4.9%). Supermarkets had the highest mean HFAI (24.8), whereas gas stations (8.7) had the lowest; convenience stores were also low (11.1). In rural Maryland, the most common food source types have low healthy food availability, and nontraditional food sources are an important part of the food environment.
Why does emissions trading under the EU Emissions Trading System (ETS) not affect firms’ competitiveness? Empirical findings from the literature

ABSTRACT Environmental policies may have important consequences for firms’ competitiveness or profitability. For the European Union Emissions Trading System (EU ETS) the empirical literature documents that significant emissions reductions have resulted from it. Surprisingly, however, the literature shows that there have been hardly any concurrent negative effects on firms’ competitiveness during the first two phases of the scheme (2005–2012). We show that the main explanations for the absence of negative impacts on competitiveness are a large over-allocation of emissions allowances leading to a price drop and the ability of firms to pass costs onto consumers in some sectors. Cost pass-through combined with free allocation, in turn, partly generated windfall profits. In addition, the relatively low importance of energy costs indicated by their average share in the budgets of most manufacturing industries may have limited the impact of the EU ETS. Finally, small but significant stimulating effects on innovation have been found so far. Several factors suggest that over-allocation is likely to remain substantial in the upcoming periods of the scheme. Therefore, we expect to see no negative competitiveness effects from the EU ETS in Phases III and IV (2013–2030). Key policy insights Empirical literature on the EU ETS shows that there have been hardly any effects on firms’ competitiveness or profitability. One main explanation is a large over-allocation of emissions allowances leading to a price drop. This reduced incentives for innovation. Moreover, firms were able to pass costs on to consumers in some sectors which partly generated windfall profits. Innovation effects have so far been small but positive. We expect to see no negative competitiveness effects on regulated firms in the near future suggesting that no further reliefs for regulated firms are required.
The up to 21-year clinical outcome and survival of feldspathic porcelain veneers: accounting for clustering.

PURPOSE This study aimed to investigate the clinical outcome and estimated cumulative survival rate of feldspathic porcelain veneers in situ for up to 21 years while also accounting for clustered outcomes. MATERIALS AND METHODS Porcelain veneers(n = 499) placed in patients (n = 155) by a single prosthodontist between 1990 and 2010 were sequentially included, with 239 veneers (88 patients) placed before 2001 and 260 veneers (67 patients) placed thereafter. Nonvital teeth, molar teeth, or teeth with an unfavorable periodontal prognosis were excluded. Preparations had chamfer margins, incisal reduction, palatal overlap, and at least 80% enamel. Feldspathic veneers from refractory dies were etched (hydrofluoric acid), silanated, and bonded. Many patients received more than 1 veneer (mean: 5.8 ± 4.3). Clustered outcomes were accounted for by randomly selecting (random table) 1 veneer per patient for analysis. Clinical outcome (success, survival, unknown, dead, repair, failure) and Kaplan-Meier estimated cumulative survival were reported. Differences in survival were analyzed using the log-rank test. RESULTS For the random sample of veneers (n = 155), the estimated cumulative survival rates were 96% ± 2% (10 years) and 96% ± 2% (20 years). For the entire sample, the survival rates were 96% ± 1% (10 years) and 91% ± 2% (20 years). Survival did not statistically differ between these groups (P = .65). Seventeen veneers in 8 patients failed, 75 veneers in 30 patients were classified as unknown, and 407 veneers in 130 patients survived. Multiple veneers in the same mouth experienced the same outcome, clustering the results. CONCLUSIONS Multiple dental prostheses in the same mouth are exposed to the same local and systemic factors, resulting in clustered outcomes. Clustered outcomes should be accounted for during analysis. When bonded to prepared enamel substrate, feldspathic porcelain veneers have excellent long-term survival with a low failure rate. The 21-year estimated cumulative survival for feldspathic porcelain veneers bonded to prepared enamel was 96% ± 2%.
Polymer Resins with Amino Acid Containing Pendants for Sorption of Bilirubin. II. Polyamide Resins with Various Basic Amino Acids

Short peptides, three to eight amino acids in length, containing various combinations of alanine, arginine, lysine, histidine and tyrosine have been synthesized onto water-swellable polyamide resin by the solid phase peptide synthesis method. The amount of bilirubin adsorbed from aqueous buffer solution (pH = 7.8) by the resins increases with increasing basicity of the amino acids in the pendant. As the number of basic amino acids on the pendant is increased fron one to five a 4.7 fold enhancement in the adsorption capacity is seen for arginine while a 9.3 fold enhancement is obtained for lysine. A corresponding increase in length for the non-basic histidine results in a 6 fold enhancement. With alanine the adsorption capacity is uneffected by an increase in pendant length.
Ecotourism and rewilding: the case of Swedish Lapland

ABSTRACT This paper critically engages with the role of ecotourism in the emergent concept of rewilding, by examining the case of Rewilding Lapland, an initiative launched by the Rewilding Europe Foundation in 2015 (Rewilding Lapland was renamed Rewilding Sweden in early 2018). The paper investigates how ecotourism is discussed and approached in the Rewilding Lapland focal area, investigating the conflicts between local views and institutional approaches. There are tensions between local views and institutional approaches to ecotourism. The paper discusses these tensions in the context of the neoliberalisation of nature, and the friction between exclusive ecotourism and the Swedish Right of Public Access. The paper concludes that for respondents, the benefits of rewilding are influenced by the relationship fostered between themselves and the environment. For them, ecotourism offers a medium to engage with the environment, with the medium of engagement greatly affecting the relations that are fostered. The paper presents tensions as arising through the (deepening) neoliberalisation of nature the Rewilding Lapland ecotourism agenda pursues. To be successful, rewilding initiatives like Rewilding Lapland that promote ecotourism ventures must be aware of the types of human–nature relations these ventures foster, and how these may not always be valued by a local community.
Treatment of Actinic Cheilitis Using Photodynamic Therapy with Methylaminolevulinate: Report of Three Cases

Background Patients with actinic cheilitis should be treated to prevent transformation into invasive squamous cell carcinoma. Treatment options comprise, for instance, destructive approaches, such as vermilionectomy and carbon dioxide laser ablation. Photodynamic therapy (PDT) has demonstrated high efficacy in patients with epithelial skin cancers, but there is only one report on PDT with free d-aminolevulinic acid (ALA) and noncoherent light in the treatment of actinic cheilitis. Objective We report the treatment of three patients with actinic cheilitis with PDT using the new photosensitizing agent methylaminolevulinate (methyl-5-amino-4-oxopenthanoate [MAOP]) combined with red light. Methods MAOP was topically applied on the lower lip 3 hours before treatment with red light. Two consecutive treatments, 1 week apart, were administered. Clinical assessment was performed up to 13 months after the initial treatment. Results A good clinical response with an excellent cosmetic outcome was observed in all three patients. Moderate to severe pain was associated with the application of the red light, and mild inflammation with edema of the lower lip occurred. Conclusion This is the first use of PDT with MAOP and 634 mm wavelength red light in patients with actinic cheilitis. The clinical results demonstrate that PDT might be an alternative for patients who refuse surgical procedures. However, further studies are needed to compare the efficacy and cosmetic outcome with conventional treatment modalities.
Linker histone subtypes and their allelic variants

Members of histone H1 family bind to nucleosomal and linker DNA to assist in stabilization of higher‐order chromatin structures. Moreover, histone H1 is involved in regulation of a variety of cellular processes by interactions with cytosolic and nuclear proteins. Histone H1, composed of a series of subtypes encoded by distinct genes, is usually differentially expressed in specialized cells and frequently non‐randomly distributed in different chromatin regions. Moreover, a role of specific histone H1 subtype might be also modulated by post‐translational modifications and/or presence of polymorphic isoforms. While the significance of covalently modified histone H1 subtypes has been partially recognized, much less is known about the importance of histone H1 polymorphic variants identified in various plant and animal species, and human cells as well. Recent progress in elucidating amino acid composition‐dependent functioning and interactions of the histone H1 with a variety of molecular partners indicates a potential role of histone H1 polymorphic variation in adopting specific protein conformations essential for chromatin function. The histone H1 allelic variants might affect chromatin in order to modulate gene expression underlying some physiological traits and, therefore could modify the course of diverse histone H1‐dependent biological processes. This review focuses on the histone H1 allelic variability, and biochemical and genetic aspects of linker histone allelic isoforms to emphasize their likely biological relevance.
The Adaptor 3BP2 Is Required for Early and Late Events in FcεRI Signaling in Human Mast Cells

Adaptor molecules are essential in organizing signaling molecules and in coordinating and compartmentalizing their activity. SH3-binding protein 2 (3BP2) is a cytoplasmic adaptor protein mainly expressed by hematopoietic cells that has been shown to act as a positive regulator in T, B, and NK cell signal transduction. 3BP2 is an important regulator of cytotoxic granule release in NK cells. Mast cells (MCs) similarly degranulate following Ag-dependent aggregation of the FcεRI on the cell surface. Activation of these cells induces the release of preformed inflammatory mediators and the de novo synthesis and secretion of cytokines and chemokines. Thus, MCs participate in both innate and acquired responses. We observed that 3BP2 is expressed in human MCs (huMCs) from diverse origins. Moreover, 3BP2 coimmunoprecipitates with essential MC signaling mediators such as Lyn, Syk, and phospholipase C γ; thus, a role for this adaptor in MC function was postulated. In the present work, we used the short hairpin RNA lentiviral targeting approach to silence 3BP2 expression in huMCs. Our findings point to a requirement for 3BP2 in optimal immediate and late MCs responses such as degranulation and IL-8 or GM-CSF secretion. 3BP2 was determined to be necessary for optimal phosphorylation of Syk, linker for activation of T cells, and phospholipase C γ1, critical signals for calcium release from intracellular stores. Taken together, our results show that by participating in FcεRI- mediated signal transduction 3BP2 is an important regulator of huMC activation. Thus, 3BP2 could be a potential therapeutic target for IgE-dependent MC-mediated inflammatory disease.
FISCAL RESPONSIBILITY WITHIN AN UNSTABLE ECONOMIC AND POLITICAL ENVIRONMENT

It is hypothesised that the economic and political environments from Romania are characterized by instability. Also, election cycles represent a major source of volatility for economic policies, particularly for fiscal policy. However, it is very difficult to observe accurately which fiscal proposals/changes have electoral motivations and which are backed by ideologies or technical considerations. The paper presents a theoretical analysis of fiscal responsibility and a conceptual analysis of economic and political stability. The paper is completed by a series of analyses and observations based on the situation of Romania, related to stability and responsibility.