Miracle-dz/UniFAR-FTUs huggingface dataset:
Digital necroses and vascular thrombosis in severe spinal muscular atrophy

Infantile spinal muscular atrophy (SMA) caused by homozygous SMN1 gene deletions/mutations is characterized by neuronal loss and axonopathy of motor neurons. We report two unrelated patients with severe SMA type I who had only one SMN2 copy and developed ulcerations and necroses of the fingers and toes. Sural nerve biopsy was normal in patient 1, whose affected skin displayed necroses and thrombotic occlusions of small vessels. Corresponding to a mouse model and other patients with similar findings, we believe that severe survival motor neuron (SMN) deficiency may present as vasculopathy. Muscle Nerve 42: 144–147, 2010
Has CEFTA Increased Members’ Mutual Trade? Evidence with an Enlarged Set of Plausibly Exogenous Instruments

The objective of the paper is to measure the effect of the Central European Free Trade Agreement (CEFTA) in its today’s composition on members’ mutual trade. Special consideration is given to CEFTA endogeneity stemming from members’ will to form a trade bloc as a vehicle to approximate EU sooner and prevent further misunderstandings in the Balkan. We use a Conditional Mixed Process estimator whereby CEFTA is instrumented by a set of variables measuring democracy and governments’ negotiation will. The recent Conley et al. (2012) method is used to deal with only plausibly exogenous instruments. The 1996-2015 period is covered. Results suggest that CEFTA played quantitatively large and statistically significant role for its members. When endogeneity is considered, the effect of CEFTA on its members’ mutual trade is found even higher by 50% to 61%. This may indirectly suggest that CEFTA trading power has been drawn not only from countries’ GDPs and proximity, but also from their governments’ will to establish level playing field for cooperation and approach the EU quicker. However, if instruments’ strict exogeneity cannot be secured by the means of argument or statistical tools, then results suggest that CEFTA effect’s amplification may be rather driven, at least partially, by the correlation between the instrument and trade shocks.
On the morphology of antennular sensory and attachment organs in cypris larvae of the deep‐sea vent/seep barnacles, Ashinkailepas and Neoverruca

Barnacle cypris larvae show high morphological variation in the organs used in search of and attaching to a substratum. This variation may represent adaptation to the habitat of the species. Here, we studied SEM level morphologies of cypris antennular sensory and attachment organs in a deep‐sea vent endemic species (Neoverruca sp.) and a vent/seep inhabiting species (Ashinkailepas seepiophila). We compare them with three species from other environments. The antennular morphologies of Neoverruca sp. and A. seepiophila were similar, which is consistent with recent molecular studies showing a close relationship of the two species. The setation pattern of the antennules was very conservative among species from various environments. In contrast, striking differences were observed in the structure of the attachment organ (the third antennular segment). Neoverruca sp. and A. seepiophila had no velum or a skirt surrounding the attachment disc on the third segment, while other cirripede cyprids almost always have either of these structures. In addition, both cyprids of A. seepiophila and Neoverruca sp. had the attachment disc angled toward the substratum, whereas it faces distally in cyprids from hard bottom inhabiting barnacles. We suggest that both velum/skirt and the angle of the attachment disc play an important role, when the antennules are contacting the substratum during surface exploration. Differences in attachment organ structures may be highly adaptive, enabling cirripede species to enter new habitats during evolution. J. Morphol. 277:594–602, 2016. © 2016 Wiley Periodicals, Inc.
The Geographic Spread of a Large Devaluation

Cravino and Levchenko (2017) establish that the 1994 Mexican peso devaluation raised the prices of consumption baskets of low-income households substantially more than the prices of the consumption baskets of high-income households. In this paper, we explore this result further by focusing on the regional variation in how much prices of consumption baskets changed following the devaluation. Our main finding is that the devaluation was anti-poor in all regions, but there is substantial regional dispersion in the relative inflation faced by the poor.
Factors Associated with Recurrence of Accessory Pathway Conduction After Radiofrequency Catheter Ablation

Catheter ablation of 215 accessory pathways (APs) using radiofrequency current (RF) was attempted in 204 consecutive patients. Two hundred twelve of the 215 (99%) APs were successfully ablated. After a minimum follow‐up period of 1 month (mean 8.5 ± 5.4 months), AP conduction had returned in 17 patients (8%). Recurrence of AP conduction was manifest by atrioventricular (AV) reentrant tachycardia in six patients, palpitations suggestive of AV reentrant tachycardia in five patients, ventricular preexcitation on electrocardiogram in five patients, and inducible AV reentrant tachycardia during a follow‐up electrophysiological study in one asymptomatic patient. AP conduction returned as early as 12 hours and as late as 4.7 months, but was evident within 2 months of ablution in 15 of 17 (88%) patients. AP conduction recurred in 12%‐14% of anteroseptal, right free‐wall, and posteroseptal APs, but only 5% of left free‐wall APs (P < 0.01). Retrograde only conducting APs (concealed APs) had recurrence of AP conduction more frequently (16%) than APs that exhibited antegrade conduction (5.5%; P < 0.01). Failure to record AP potentials from the ablation electrode, reflecting poor AP localization, was a strong predictor for recurrence of AP conduction. AP conduction returned in 19% of 48 APs when AP potentials were not recorded, compared to 5% of 164 APs where AP potentials were recorded from the ablation electrode (P < 0.01). The time to block of AP conduction from the onset of RF current application was longer in APs with recurrence of conduction (4.9 ± 6.1 sec vs 2.9 ± 3.4 sec; P < 0.02). Recurrence of AP conduction was more frequent when the stability of the ablation electrode was poor (12% of 41 APs vs 7% of 171 APs with stable electrode placement], and when the AP had multiple components (11% of 36 APs ablated at multiple sites vs 7% of 176 APs where AP was ablated at a single site), but these were not statistically significant. All 17 patients with recurrence of AP conduction underwent a second successful ablation. In conclusion, the overall incidence of recurrence of AP conduction is low, but is higher for right free‐wall and septal APs, concealed APs, and probably relates to poor AP localization.
Joint capsule treatment with enkephalin‐encoding HSV‐1 recombinant vector reduces inflammatory damage and behavioural sequelae in rat CFA monoarthritis

This study assessed enkephalin expression induced by intra‐articular application of recombinant, enkephalin‐encoding herpes virus (HSV‐1) and the impact of expression on nociceptive behaviours and synovial lining inflammation in arthritic rats. Replication‐conditional HSV‐1 recombinant vectors with cDNA encoding preproenkephalin (HSV‐ENK), or control transgene β‐galactosidase cDNA (HSV‐β‐gal; control) were injected into knee joints with complete Freund's adjuvant (CFA). Joint temperatures, circumferences and nociceptive behaviours were monitored on days 0, 7, 14 and 21 post CFA and vector treatments. Lumbar (L4–6) dorsal root ganglia (DRG) and spinal cords were immunostained for met‐enkephalin (met‐ENK), β‐gal, HSV‐1 proteins and Fos. Joint tissues were immunostained for met‐ENK, HSV‐1 proteins, and inflammatory mediators Regulated on Activation, Normal T‐cell Expressed and Secreted (RANTES) and cyclo‐oxygenase‐2, or stained with haematoxylin and eosin for histopathology. Compared to exuberant synovial hypertrophy and inflammatory cell infiltration seen in arthritic rats treated with CFA only or CFA and HSV‐β‐gal, the CFA‐ and HSV‐ENK‐treated arthritic rats had: (i) striking preservation of synovial membrane cytoarchitecture with minimal inflammatory cell infiltrates; (ii) significantly improved nociceptive behavioural responses to mechanical and thermal stimuli; (iii) normalized Fos staining in lumbar dorsal horn; and (iv) significantly increased met‐ENK staining in ipsilateral synovial tissue, lumbar DRG and spinal cord. The HSV‐1 and transgene product expression were confined to ipsilateral lumbar DRG (HSV‐1, met‐ENK, β‐gal). Only transgene product (met‐ENK and β‐gal) was seen in lumbar spinal cord sections. Targeted delivery of enkephalin‐encoding HSV‐1 vector generated safe, sustained opioid‐induced analgesia with protective anti‐inflammatory blunting in rat inflammatory arthritis.
Firm Heterogeneity and Aggregate Business Services Exports: Micro Evidence from Belgium, France, Germany and Spain

This paper uses detailed micro data on service exports at the firm-destination-service level to analyse the role of firm heterogeneity in shaping aggregate service exports in Belgium, France,Germany and Spain from 2003 to 2007. We decompose the level and the growth of aggregate service exports into different trade margins paying special attention to firm heterogeneity within countries. We find that the weak export growth of France is at least partly due to poor performance by small exporters. By contrast, small exporters are the most dynamic contributors to the aggregate exports of Belgium, Germany and Spain. Our results highlight the importance of firm heterogeneity in understanding aggregate export growth.
Effect of the preschool pertussis booster on national notifications of disease in Australia

Background. Australia introduced a fifth dose of pertussis vaccine at 4 to 5 years of age in 1994, the first country to do so for some 40 years. We report trends in national pertussis notifications from 1993 to 2001. Methods. Notified pertussis cases were analyzed by age and year of disease onset. Results. Before the fifth dose was introduced, notification rates were higher among 5- to 9-year-olds than 10- to 14-year-olds (76 per 100 000 population vs. 65 per 100 000). As more 5- to 9-year-olds became eligible for the fifth dose, their notification rates as a group and by year of age progressively fell to below those of 10- to 14-year-olds, consistent with a vaccine effect. Comparison of notification rates for the epidemic years of 1997 and 2001 shows that 5- to 10-year-olds (eligible for fifth dose) had lower notification rates in 2001 (61 per 100 000) than 5- to 10-year-olds in 1997 (196 per 100 000). This contrasts with children who were not eligible for the fifth dose (12- to 14-year-olds), who had higher notification rates in 2001 (223 per 100 000) than 12- to 14-year-olds in 1997 (160 per 100 000). Conclusions. The pattern of age-specific notification rates provides strong evidence that the fifth dose reduced the incidence of pertussis in older children. It will be important to track the impact of the fifth dose on adolescent pertussis notifications to assess the duration of vaccine-acquired immunity.
Ruminant Rhombencephalitis-Associated Listeria monocytogenes Alleles Linked to a Multilocus Variable-Number Tandem-Repeat Analysis Complex

ABSTRACT Listeria monocytogenes is among the most important food-borne pathogens and is well adapted to persist in the environment. To gain insight into the genetic relatedness and potential virulence of L. monocytogenes strains causing central nervous system (CNS) infections, we used multilocus variable-number tandem-repeat analysis (MLVA) to subtype 183 L. monocytogenes isolates, most from ruminant rhombencephalitis and some from human patients, food, and the environment. Allelic-profile-based comparisons grouped L. monocytogenes strains mainly into three clonal complexes and linked single-locus variants (SLVs). Clonal complex A essentially consisted of isolates from human and ruminant brain samples. All but one rhombencephalitis isolate from cattle were located in clonal complex A. In contrast, food and environmental isolates mainly clustered into clonal complex C, and none was classified as clonal complex A. Isolates of the two main clonal complexes (A and C) obtained by MLVA were analyzed by PCR for the presence of 11 virulence-associated genes (prfA, actA, inlA, inlB, inlC, inlD, inlE, inlF, inlG, inlJ, and inlC2H). Virulence gene analysis revealed significant differences in the actA, inlF, inlG, and inlJ allelic profiles between clinical isolates (complex A) and nonclinical isolates (complex C). The association of particular alleles of actA, inlF, and newly described alleles of inlJ with isolates from CNS infections (particularly rhombencephalitis) suggests that these virulence genes participate in neurovirulence of L. monocytogenes. The overall absence of inlG in clinical complex A and its presence in complex C isolates suggests that the InlG protein is more relevant for the survival of L. monocytogenes in the environment.
Trade Unions and Political Parties in Italy (1946–2014): Ideological Positions and Critical Junctures

Abstract The paper investigates party-union relationships in Italy (1946–2014) by hand-coding parties’ parliamentary speeches and trade unions’ congress motions. In line with the cartel party thesis, a time series analysis shows that the ideological closeness between the left-wing Italian General Confederation of Labour and left-wing parties deteriorated when the Italian Socialist Party (1980) and the heirs of the Italian Communist Party (1998) converged toward the centre of the ideological spectrum. Conversely, the closeness between the Catholic-inspired Italian Confederation of Workers’ Unions and the heirs of Christian Democracy increased after 1994, when the former party’s leftist factions became the major part of the Italian Popular Party.
Becoming a Young Breadwinner? The Education, Employment and Training Trajectories of Young Fathers

The entry into fatherhood is a major life course transition involving the acquisition of new adult roles and responsibilities. This transition is rarely planned for young fathers, and may involve a range of challenges, not least their capacity to provide materially and financially for their child. Drawing on a Qualitative Longitudinal study of young fathers in the UK, this article charts their very different pathways through education, training and employment, showing how these are shaped by a constellation of life circumstances. The implications for policy are considered in the light of a shifting landscape of welfare reform and ‘austerity’ measures.
Gadoxetic acid‐enhanced fat suppressed three‐dimensional T1‐weighted MRI using a multiecho dixon technique at 3 tesla: Emphasis on image quality and hepatocellular carcinoma detection

To compare the image quality between T1 high‐resolution isotropic volume examination using the multi‐echo Dixon technique (mDixon‐eTHRIVE) and that using spectrally adiabatic inversion recovery (SPAIR‐eTHRIVE) in gadoxetic acid‐enhanced liver MRI, and to evaluate the detectability of hepatocellular carcinoma (HCC) on mDixon‐eTHRIVE.
Glomerular mRNAs in human type 1 diabetes: biochemical evidence for microalbuminuria as a manifestation of diabetic nephropathy.

BACKGROUND In patients with type 1 diabetes, some consider microalbuminuria to be a predictor of diabetic nephropathy while others believe it is an early feature of diabetic nephropathy. METHODS Levels of mRNAs that are of pathogenetic relevance in diabetic nephropathy were compared in glomeruli isolated from microalbuminuric and overtly proteinuric subjects and in control normoalbuminuric diabetic subjects and living renal transplant donors. RESULTS In subjects with microalbuminuria and overt proteinuria, glomerular mRNAs were virtually identical and approximately twofold higher for connective tissue growth factor (CTGF; P < 0.01) and collagen alpha2(IV) (P < 0.03) compared to living renal donors and normoalbuminuric patients. Glomerular glyceraldehyde-3-phosphate dehydrogenase (GAPDH) levels were not significantly different among the groups (P = 0.4). Weak but statistically significant correlations were noted between CTGF mRNA and albuminuria (assessed by rank), fractional mesangial surface area, and a composite renal biopsy index. Glomerular CTGF mRNA correlated inversely with creatinine clearance. Glomerular collagen alpha2(IV) mRNA levels correlated with albuminuria (by rank) and less strongly with fractional mesangial area. CONCLUSION To our knowledge, these data provide the first biochemical evidence demonstrating that the glomeruli of microalbuminuric patients and those with overt proteinuria do not differ significantly. The data support the concept that microalbuminuria is not "predictive" of diabetic nephropathy, but rather is an earlier point in the spectrum of diabetic nephropathy.
N-acetylcysteine inhibits kinase phosphorylation during 3T3-L1 adipocyte differentiation

ABSTRACT Objectives: Reports investigating the effects of antioxidants on obesity have provided contradictory results. We have previously demonstrated that treatment with the antioxidant N-acetylcysteine (NAC) inhibits cellular triglyceride (Tg) accumulation as well as total cellular monoamine oxidase A (MAOA) expression in 3T3-L1 mature adipocytes (Calzadilla et al., Redox Rep. 2013;210–218). Here we analyzed the role of NAC on adipogenic differentiation pathway. Methods: Assays were conducted using 3T3-L1 preadipocytes (undifferentiated cells: CC), which are capable of differentiating into mature adipocytes (differentiated cells: DC). We studied the effects of different doses of NAC (0.01 or 1 mM) on DC, to evaluate cellular expression of phospho-JNK½ (pJNK½), phospho-ERK½ (pERK½) and, mitochondrial expression of citrate synthase, fumarate hydratase and MAOA. Results: Following the differentiation of preadipocytes, an increase in the expression levels of pJNK½ and pERK½ was observed, together with mitotic clonal expansion (MCE). We found that both doses of NAC decreased the expression of pJNK½ and pERK½. Consistent with these results, NAC significantly inhibited MCE and modified the expression of different mitochondrial proteins. Discussion: Our results suggested that NAC could inhibit Tg and mitochondrial protein expression by preventing both MCE and kinase phosphorylation.
Comparison of hepatectomy with or without hepatic inflow occlusion in patients with hepatocellular carcinoma: a single-center experience.

BACKGROUND The negative effects of hepatic inflow occlusion (HIO) on postoperative liver function of patients with hepatocellular carcinoma (HCC) after liver resection have been reported. Nevertheless, whether or not HIO could influence the long-term outcomes remains unclear. METHODS A total of 396 patients were included in this study and divided into without occlusion (WO) group (N.=112) and HIO group (N.=284). Aiming to minimize influence of selection bias and confounding variables, we used propensity score matching (PSM) of a 0.2 caliper to balance baseline variables. Overall survival (OS) and disease-free survival (DFS) were compared by the Kaplan-Meier method. Independent prognostic factors for OS and DFS were identified by Cox proportional hazards regression model. RESULTS PSM were used to generate 101 pairs of patients. After PSM, OS was not significantly different between WO and HIO group (1-year: 86.1% vs. 83.2%; 3-year: 61.4% vs. 61.4%; 5-year: 45.5% vs. 39.6%; P = 0.626). Similar results of DFS were obtained between WO and HIO group (1-year: 54.5% vs. 53.5%; 3-year: 30.5% vs. 28.7%; 5-year: 14.2% vs. 14.9%; P=0.873). WO and HIO groups did not differ in 30-day, 90-day mortality and rate of postoperative complications (all P>0.05). CONCLUSIONS Our data indicates that HIO might not negatively affect the OS and DFS of HCC patients undergoing liver resection and is likely to be a safe and viable option for intraoperative blood loss control.
Transcriptional coactivator HCF-1 couples the histone chaperone Asf1b to HSV-1 DNA replication components

The cellular transcriptional coactivator HCF-1 interacts with numerous transcription factors as well as other coactivators and is a component of multiple chromatin modulation complexes. The protein is essential for the expression of the immediate early genes of both herpes simplex virus (HSV) and varicella zoster virus and functions, in part, by coupling chromatin modification components including the Set1 or MLL1 histone methyltransferases and the histone demethylase LSD1 to promote the installation of positive chromatin marks and the activation of viral immediately early gene transcription. Although studies have investigated the role of HCF-1 in both cellular and viral transcription, little is known about other processes that the protein may be involved in. Here we demonstrate that HCF-1 localizes to sites of HSV replication late in infection. HCF-1 interacts directly and simultaneously with both HSV DNA replication proteins and the cellular histone chaperone Asf1b, a protein that regulates the progression of cellular DNA replication forks via chromatin reorganization. Asf1b localizes with HCF-1 in viral replication foci and depletion of Asf1b results in significantly reduced viral DNA accumulation. The results support a model in which the transcriptional coactivator HCF-1 is a component of the HSV DNA replication assembly and promotes viral DNA replication by coupling Asf1b to DNA replication components. This coupling provides a novel function for HCF-1 and insights into the mechanisms of modulating chromatin during DNA replication.
Environmentally friendly tourists and spending on nature-based activities

ABSTRACT The purpose of this study is to examine whether environmentally friendly tourists spend more (or less) on nature-based activities. Using survey data collected from local and international tourists in Kuching (the capital city of Sarawak state in Malaysia), we find evidence that eco-friendly tourists spend less on nature-based activities.
Multimeasure Pain Assessment in an Ethnically Diverse Group of Patients with Cancer

The purposes of this study were (a) to describe the relationship between pain perception and ethnic identity and socioeconomic status, (b) to evaluate the intercorrelations between pain measures in different ethnic groups, and (c) to determine whether ethnicity or socioeconomic status influences patient’s pain control beliefs and satisfaction with the pain management provided. The sample consisted of 51 English-, Korean-, or Spanish-speaking participants experiencing cancer pain who were 18 years and older and were having a Karnofsky score of no less than 30. The Visual Analogue Scale (VAS), Memorial Pain Scale (MPS), and Face Scale (FS) were used to measure pain perception. In all pain analyses, Hispanics, African Americans, and Anglos did not differ significantly. The data suggest that the pain scales used in this study are appropriate for use in a multicultural population.
OUTLAW HEAVEN: WHY STATES BECOME TAX HAVENS

OF DISSERTATION OUTLAW HEAVEN: WHY STATES BECOME TAX HAVENS It is the argument of this dissertation that states become tax havens as a conscious economic development strategy. These states – more properly referred to as “jurisdictions” because some lack the sovereignty of the traditional Westphalian state – do not have the natural resources or the population to pursue more traditional economic development strategies, but they do have the ability to write and implement laws that create a virtual resource: banking secrecy. These jurisdictions are to carry out this strategy because they tend to be well-governed, stable, and relatively wealthy, making them attractive partners for the international banking, law, and accounting firms that drive offshore finance, and then for their customers – both individual and corporate – as well. The qualities tax havens possess also enable them to calculate that the benefits they reap from pursuing this strategy outweigh any penalties assessed by anti-tax haven international collective action activities, such as the naming and shaming campaigns of 2000.