Miracle-dz/UniFAR-FTUs huggingface dataset:
[Failure to thrive and intestinal diseases in congenital disorders of glycosylation].

UNLABELLED Congenital disorders of glycosylation type I (GDG-I) is a class of genetic multisystem disorders characterised by defective glycosylation of glycoproteins. The characteristics and mechanisms of failure to thrive and intestinal diseases present in CDG-I are anectodal. PATIENTS AND METHODS The aim of this study was to analyse 7 CDG-I (4 CDG-Ia, 2 CDG-Ib and 1 CDG-Ix) with important digestive symptoms and failure to thrive in order to characterise the mechanisms implied. RESULTS Four children had no skin abnormality or dysmorphia (1 CDG-Ia, 2 CDG-Ib, 1 CDG-Ix). An encephalopathy with cerebellar hypoplasia was present only in the 4 CDG-Ia. Failure to thrive and diarrhea were present during the first month of life in 6 and appeared at 5 years in one CDG-Ia associated to mild or severe hepatopathy in all patients. One CDG-Ia, 1 CDG-Ib, 1 CDG-Ix had an exsudative enteropathy. A positive steatorrhea was present in 3 patients. Five patients had an abnormal small bowel biopsy. Abnormalities were variable: moderate inflammation of the chorion without villous atrophy in 2, intra-enterocyte fat accumulation without villous atrophy in 2, and partial villous atrophy with lymphangectasia in 1. In 2 CDG-Ia the intestinal biopsy was normal. Enteral nutrition in 4 and parenteral nutrition in 2 were effective in 4 patients and 1 patient with an exsudative enteropathy respond to a free fat diet (CDG-Ix). CONCLUSION The digestive symptoms with failure to thrive is a common feature of CDG-I and could be the first symptoms. The diagnostic should be suspected if no other cause is found. Mechanisms of the intestinal symptoms appear to be multiple such as inflammation, abnormal enterocyte lipid transport or intestinal permeability related to the abnormal glycosylation of intestinal mucosa glycoproteins.
How to Improve Attitudes Toward Disliked Groups

We propose a model of how messages about groups one personally dislikes affect individual attitudes. We build upon theories of message persuasion and out-group acceptance to account for evidence type (numerical vs. narrative), facilitating conditions (encouraging empathy vs. objectivity), and the underlying mechanisms (immersion). We test this model in a pretest-posttest experiment, in which a sample of Americans (N = 601) read counter-attitudinal commentaries below articles presenting either narrative or numerical evidence about illegal immigrants or same-sex couples. Narratives led to greater message acceptance and greater immersion, especially in the empathetic condition. In turn, numerical messages led to self-perceived attitude change in the objective condition. Persuasive effects of narratives in the empathetic, but not the objective, condition were mediated by immersion.
Legumes Symbioses: Absence of Nod Genes in Photosynthetic Bradyrhizobia

Leguminous plants (such as peas and soybeans) and rhizobial soil bacteria are symbiotic partners that communicate through molecular signaling pathways, resulting in the formation of nodules on legume roots and occasionally stems that house nitrogen-fixing bacteria. Nodule formation has been assumed to be exclusively initiated by the binding of bacterial, host-specific lipochito-oligosaccharidic Nod factors, encoded by the nodABC genes, to kinase-like receptors of the plant. Here we show by complete genome sequencing of two symbiotic, photosynthetic, Bradyrhizobium strains, BTAi1 and ORS278, that canonical nodABC genes and typical lipochito-oligosaccharidic Nod factors are not required for symbiosis in some legumes. Mutational analyses indicated that these unique rhizobia use an alternative pathway to initiate symbioses, where a purine derivative may play a key role in triggering nodule formation.
5-Alpha reductase inhibitors in the management of benign prostatic hyperplasia : A rationale

Benign Prostatic hyperplasia and its clinical manifestation as lower urinary tract symptoms is a major health concern for aging men. Prevalence increases with age, affecting about 60% of men aged over 50 years, and 80% by the age of 80 years. Nearly all men will develop microscopic BPH by the age of 90 [1]. Despite the high prevalence of BPH in aged men population, the disease pathogenesis is far from complete understanding. It is the non-malignant enlargement of the prostate gland caused by increase in the number of stromal and epithelial cells, resulting in obstruction of the proximal urethra, thus causes urinary flow disturbances. Although many men with mild to moderate symptoms do well without therapy, others have gradually increasing symptoms and require medical therapy or surgery. BPH is a rarely fatal but if left untreated, chronic obstructions can lead to the back up of urine into the ureters and compromises kidney function, thus serious life threatening complications such as acute urinary retention may arises [2].
Multiple roles of Ulk4 in neurogenesis and brain function

ABSTRACT Neurogenesis is essential for proper brain formation and function, and abnormal neural proliferation is an underlying neuropathology of many brain disorders. Recent advances on adult neurogenesis demonstrate that neural stem cells (NSCs) at the subventricular zone (SVZ) are largely derived during mid-embryonic neurogenesis from a subset of cells, which slow down in their pace of cell division,1 become quiescent cells and can be reactivated in need.2 The NSCs at birth constitute the stem cell pool for both postnatal oligodendrogenesis3 and adult neurogenesis.1,2 However, little is known about factors that control the size of NSC pool. The article published in Stem Cells on Jun 14, 2016 by Liu and colleagues described a member of the Unc-51-like serine/threonine kinase family, Ulk4, which plays a critical role in regulating the NSC pool size.4 Authors presented evidence of cell cycle-dependent Ulk4 expression in vitro and in vivo, and reduced NSC pool in targetedly disrupted Ulk4 newborn mice, with disturbed pathways of cell cycle regulation and WNT signaling (Fig. 1), suggesting that ULK4 may be associated with neurodevelopmental, neuropsychiatric as well as neurodegenerative diseases.
A high step-up switched-capacitor converter with zero current switching technique for using in solar system applications

Small size, low cost, high efficiency, and step-up voltage-ratio converters are the most significant features required for solar system applications. In this paper, a high step-up zero current switching switched-capacitor converter is introduced. Its high power density, low cost, and soft switching features meet all the solar system's requirements. To supply a single phase ac load, a half-bridge inverter is used in the second stage. Mathematical analyses of the proposed converter are given, in detail. To verify the given analyses, a 17 V to 68 V switched-capacitor converter has been simulated and implemented at 50 kHz switching frequency. The prototype converter has been tested under different conditions. The experimental and simulated results are in good agreement with the mathematical analyses.
Alternative Splicing Generates a Novel Truncated Cav1.2 Channel in Neonatal Rat Heart*

Background: L-type Cav1.2 Ca2+ channel undergoes extensive alternative splicing, generating functionally different channels. Results: Neonatal rat heart expresses a higher level of a truncated Cav1.2 Ca2+ channel from alternative splicing. Conclusion: The truncated channel can alter electrophysiological properties of a wild type channel. Significance: Although aberrantly spliced Cav1.2 channels may not conduct Ca2+ ions, they can affect functional channels. L-type Cav1.2 Ca2+ channel undergoes extensive alternative splicing, generating functionally different channels. Alternatively spliced Cav1.2 Ca2+ channels have been found to be expressed in a tissue-specific manner or under pathological conditions. To provide a more comprehensive understanding of alternative splicing in Cav1.2 channel, we systematically investigated the splicing patterns in the neonatal and adult rat hearts. The neonatal heart expresses a novel 104-bp exon 33L at the IVS3-4 linker that is generated by the use of an alternative acceptor site. Inclusion of exon 33L causes frameshift and C-terminal truncation. Whole-cell electrophysiological recordings of Cav1.233L channels expressed in HEK 293 cells did not detect any current. However, when co-expressed with wild type Cav1.2 channels, Cav1.233L channels reduced the current density and altered the electrophysiological properties of the wild type Cav1.2 channels. Interestingly, the truncated 3.5-domain Cav1.233L channels also yielded a dominant negative effect on Cav1.3 channels, but not on Cav3.2 channels, suggesting that Cavβ subunits is required for Cav1.233L regulation. A biochemical study provided evidence that Cav1.233L channels enhanced protein degradation of wild type channels via the ubiquitin-proteasome system. Although the physiological significance of the Cav1.233L channels in neonatal heart is still unknown, our report demonstrates the ability of this novel truncated channel to modulate the activity of the functional Cav1.2 channels. Moreover, the human Cav1.2 channel also contains exon 33L that is developmentally regulated in heart. Unexpectedly, human exon 33L has a one-nucleotide insertion that allowed in-frame translation of a full Cav1.2 channel. An electrophysiological study showed that human Cav1.233L channel is a functional channel but conducts Ca2+ ions at a much lower level.
Bloodstream infections caused by Klebsiella pneumoniae in onco‐hematological patients: clinical impact of carbapenem resistance in a multicentre prospective survey

The aim of this study was to identify risk factors for mortality in patients suffering from hematological malignancies (HMs) with bloodstream infections (BSIs) caused by Klebsiella pneumoniae (KP). We conducted a prospective cohort study on KP BSI in 13 Italian hematological units participating in the HEMABIS registry–SEIFEM group. The outcome measured was death within 21 days of BSI onset. Survivor and non‐survivor subgroups were compared and Cox regression analysis was conducted to identify independent predictors of mortality. A total of 278 episodes of KP BSI were included in the study between January 2010 and June 2014. We found that 161 (57.9%) KP isolates were carbapenem resistant (CRKP). The overall 21‐day mortality rate was 36.3%. It was significantly higher for patients with CRKP BSI (84/161, 52.2%) than for those with BSI caused by carbapenem susceptible KP (CSKP) (17/117, 14.5%; P < 0.001). Septic shock (HR 3.86), acute respiratory failure (HR 2.32), inadequate initial antimicrobial therapy (HR 1.87) and carbapenem resistance by KP isolates (HR 1.85) were independently associated with mortality. A subanalysis was conducted in only 149 patients with CRKP BSI who had received ≥48 hr of adequate antibiotic therapy, and combination therapy was independently associated with survival (HR 0.32). Our study shows that in recent years carbapenem resistance has dramatically increased in HM patients with KP BSI in Italy and is associated with a worse outcome. The optimal management of such infections and the definition of new empirical/targeted antimicrobial strategies in HM patients can still be considered unmet clinical needs. Am. J. Hematol. 91:1076–1081, 2016. © 2016 Wiley Periodicals, Inc.
Chiasmatic course of temporal retinal axons in the developing ferret

Recent studies on the distribution of optic axons in the mature visual pathways, as well as on the genesis of their ganglion cells of origin, suggest that the time of axonal arrival at the optic chiasm determines the side of the brain to which a temporal retinal axon will project. The present study has examined this issue directly in fetal ferrets, by determining the projection of the temporal retina at different developmental stages. Fetuses of known gestational age were fixed with paraformaldehyde and subsequently implanted with crystals of the carbocyanine dye, DiI, into eithe the temporal retina, or into one optic tract. The lipophilic diffusion of the dye within the plasma membrane of the axons revealed the course of temporal retinal fibers through the fetal chiasm, as well as the distribution of ganglion cells across the two retinae projecting to one optic tract.
Sample selection bias in the emergency room: an examination of the role of alcohol in injury.

AIMS Injury location, injury cause and patient drinking patterns were used to predict blood alcohol content (BAC) and self-reported drinking before injury using emergency room (ER) data. DESIGN Models estimating both BAC and self-reported drinking among emergency room injury patients were used; the ER sample was also compared to an injured sample from the general population. SETTING Data were from three of six communities participating in the project "Preventing Alcohol Trauma: a community trial". PARTICIPANTS ER data were collected from nine hospitals on Friday and Saturday nights between 6 p.m. and 2 a.m. on alternate weekends from June 1992 to December 1995. Telephone survey data were collected between April 1992 and March 1996. MEASUREMENTS Drinking measures included drinking frequency, drinks per occasion, and variance. Other measures involved injury time, location, and type; drinking before and after injury; and age, race, gender, education, marital status and household income. Model estimation corrected both for selection bias and censoring of the dependent measure. FINDINGS The results indicate: (1) ER populations tended to be female, less well educated, non-white, poor and younger; (2) there were significant selection bias effects in the ER sample; (3) assaults were more likely to involve drinking than other injury types; (4) drinking patterns were significant non-linear predictors of alcohol involvement; and (5) self-reported drinking before injury was both a sensitive and specific indicator of measured BAC. CONCLUSIONS Assaults uniquely involve the use of alcohol and selection bias may threaten ER study validity.
Evolution on ecological time‐scales

Summary 1Ecologically significant evolutionary change, occurring over tens of generations or fewer, is now widely documented in nature. These findings counter the long-standing assumption that ecological and evolutionary processes occur on different time-scales, and thus that the study of ecological processes can safely assume evolutionary stasis. Recognition that substantial evolution occurs on ecological time-scales dissolves this dichotomy and provides new opportunities for integrative approaches to pressing questions in many fields of biology. 2The goals of this special feature are twofold: to consider the factors that influence evolution on ecological time-scales – phenotypic plasticity, maternal effects, sexual selection, and gene flow – and to assess the consequences of such evolution – for population persistence, speciation, community dynamics, and ecosystem function. 3The role of evolution in ecological processes is expected to be largest for traits that change most quickly and for traits that most strongly influence ecological interactions. Understanding this fine-scale interplay of ecological and evolutionary factors will require a new class of eco-evolutionary dynamic modelling. 4Contemporary evolution occurs in a wide diversity of ecological contexts, but appears to be especially common in response to anthropogenic changes in selection and population structure. Evolutionary biology may thus offer substantial insight to many conservation issues arising from global change. 5Recent studies suggest that fluctuating selection and associated periods of contemporary evolution are the norm rather than exception throughout the history of life on earth. The consequences of contemporary evolution for population dynamics and ecological interactions are likely ubiquitous in time and space.
Absence of radiographic asbestosis and the risk of lung cancer among asbestos-cement workers: Extended follow-up of a cohort.

BACKGROUND It has been a matter of controversy whether there is an increased risk of lung cancer among asbestos-exposed workers without radiographic asbestosis. A previous study of lung cancer risk among asbestos-cement workers has been updated with an additional 12 years of follow-up. METHODS Subjects had received radiographic examination at 20 and 25 years from first exposure to asbestos. Radiographs were interpreted by a single National Institute of Safety and Health (NIOSH)-certified B-reader using the 1971 International Labor Office (ILO) Classification of the pneumoconioses as reference standard. Asbestosis was defined as an ILO coding of 1/0 or higher. Standardized Mortality Ratios (SMRs) were calculated using the general population of Ontario as reference. RESULTS Among asbestos-cement workers without radiographic asbestosis at 20 years latency the lung cancer SMR was 3.84 (2.24-6.14). Among workers without asbestosis when examined at 25 years latency the SMR was 3.69 (1.59-7.26). CONCLUSIONS Workers from an Ontario asbestos-cement factory who did not have radiographic asbestosis at 20 or 25 years from first exposure to asbestos continued to have an increased risk of death from lung cancer during an additional 12 years of follow-up.
Inspection of defect on LCD panel using polynomial approximation

Nowadays, the CRT display devices are rapidly substituted for LCD display devices. In LCD panel production, the inspection process relies on human vision. So, the results of inspection is likely to be subjective. Therefore, we need the automated inspection system for LCD devices by obtaining objective decision level. In this paper, we propose an efficient automated inspection algorithm using polynomial approximation and optimal thresholding technique. The experimental result shows us that our proposed system can have enough performance for substitution of human inspectors.
SCREENING FOR ANTIBACTERIAL AND ANTIPROTOZOAL ACTIVITIES OF CRUDE EXTRACTS DERIVED FROM MEXICAN MEDICINAL PLANTS

Background: Crataegus mexicana, Hyptis albida, Larrea tridentata, Ocimum baislicum, Prunus serotina, and Smilax spp. are used in Mexican traditional medicine to treat respiratory and gastrointestinal diseases such as flu, cough, diarrhea, dysentery, and other parasitic or microbial infections. Therefore this study was aimed at the pharmacological prospection of these plants against eleven bacterial species and three amitochondrial protist pathogens. Material and methods: The fruits or aerial parts of C. mexicana, H. albida, L. tridentata, O. baislicum, P. serotina, and Smilax spp. were extracted with different solvents. The antibacterial properties of organic and aqueous extracts of these plants were determined by the microdilution method and the microplate alamar blue assay against Stenotrophomonas maltophilia, Escherichia coli, Acinetobacter baumannii, Pseudomonas aeruginosa, Klebsiella pneumoniae, Enterobacter cloacae, Staphylococcus aureus, methicillin-resistant S. aureus, Listeria monocytogenes, Enterococcus faecalis, and Mycobacterium tuberculosis, whereas anti-protozoal activities of extracts were evaluated by a vial micro-assay against strains of Entamoeba histolytica, Trichomonas vaginalis, and Giardia lamblia. Results: H. albida, Smilax spp, and C. mexicana showed good activity against the Gram-positive strains, S. aureus, methicillin-resistant S. aureus, and E. faecalis. Four extracts (C. mexicana, H. albida, O. basilicum, and L. tridentata) showed good activity against E. histolytica, T. vaginalis, and G. lamblia. Conclusion: The extracts of these six medicinal plants could be a source for new antibacterial and antiprotozoal drugs. For this reason they are currently under investigation to isolate and characterize their active compounds.
Ophthalmology and art: simulation of Monet's cataracts and Degas' retinal disease.

Much has been written about the failing vision of Edgar Degas and Claude Monet, and theoretical arguments have been made about the extent to which it was or was not a factor in their late style. 1-7 Contemporaries of both Degas and Monet noted that their late works were strangely coarse or garish and seemed out of character or even unfitting relative to the finer works that these artists had produced over the years. 8,9 To better understand what Degas and Monet were facing in these late years, it would be helpful to know how they actually saw their world and saw their canvases. This article uses medical knowledge and computer simulation to demonstrate their perceptions and show the relevance of their different diseases and styles of painting.
Interleukin 6 production in the central nervous system during experimental autoimmune encephalomyelitis

Interleukin 6 (IL6) is one of the major inflammation‐associated cytokines. Elevated serum or tissue levels of IL 6 have been reported to occur in several human diseases, including infections of the central nervous system (CNS), but not in non‐infectious CNS inflammation, e.g. multiple sclerosis. While studying experimental autoimmune encephalomyelitis (EAE) as an animal model for autoimmune inflammation of the CNS, we found increased IL 6 levels in the CNS of mice suffering from a lethal form of the disease. IL 6 levels in the spleens and sera were not significantly increased. These findings are indicative of local production of IL6 in the CNS during EAE, and represent the first demonstration of IL6 production in non‐infectious CNS inflammatory disease.
Study of safe operating area and improvement for power management integrated circuit

LDMOS (Lateral Double-diffused Metal Oxide Semiconductor) is widely used to smart power management IC, which can be attributed to its high operation voltage and high current driving capability. Furthermore, LDMOS is compatible with conventional CMOS processes. It will be much easier for IC foundries to make it by existing process flows. Operating at both a high drain voltage and a high current, LDMOS is more sensitive to hot carrier degradation than the devices with low operation voltages [1]. Thus, the LDMOS HC-SOA (Hot Carrier Safe Operating Area) is a major reliability concern and requires more attentions. In this paper, the HC-SOA's of conventional core and IO MOS are also illustrated to show different failure mechanisms and we focus on the detailed HC-SOA test method in practice. Additionally, we study the SOA contours for different cores, IO, NPMOS and LDMOS. Finally, we discuss the HC-SOA extension methods for LDMOS.
Dealing With Debt

As known, to finish this book, you may not need to get it at once in a day. Doing the activities along the day may make you feel so bored. If you try to force reading, you may prefer to do other entertaining activities. But, one of concepts we want you to have this book is that it will not make you feel bored. Feeling bored when reading will be only unless you don't like the book. dealing with debt really offers what everybody wants.
Competing for Low-End Markets

Recent business research points to the fortune awaiting to be tapped in low-end markets. In this paper, we investigate how the size of the low-end market influences a firm's profits and the pioneering firm's quality choice. As low-valuation consumers increase in a market, on average, consumers' willingness to pay decreases. This may lead us to expect firms' profits to decrease as the size of the low-end market increases. Our analysis shows that, if the size of the low-end market is below a threshold, an increase in the size of the low-end market may actually dampen price competition and improve profits, as firms can then strategically choose their quality levels such that their products are more differentiated. Conventional wisdom also suggests that the pioneering firm will offer a higher-quality product and earn more profits compared with the later entrant. In contrast to this notion of quality advantage, our analysis identifies circumstances in which a pioneer can offer a lower-quality product and yet earn more profits. An experimental test lends support for some of our model's predictions. We further extend the model to consider markets with multiple firms, firms with multiple products, and consumers with limited purchasing power.