Miracle-dz/UniFAR-FTUs huggingface dataset:
Three-year follow-up of hydroxyurea treatment in severely ill children with sickle cell disease. The French Study Group on Sickle Cell Disease.

PURPOSE To observe the safety and efficacy of hydroxyurea (HU), a drug that stimulates fetal hemoglobin (Hb F) production, in previously severely ill children with sickle cell disease. PATIENTS AND METHODS HU was given in an uncontrolled study to 35 children with sickle cell disease, aged from 3 to 20 years, suffering from frequent painful crises. Mean duration of treatment was 32 months (range: 12-59 months). RESULTS HU induced an increase in Hb F levels in all children out one; this increase was maximal after 9 months of treatment, was largely sustained thereafter, and was related to HU dose and inversely to patients' age. We also noted an apparent reduction in crisis, which occurred principally after 3 months of therapy and did not seem strictly correlated with the rise in Hb F level. No serious hematopoietic complication was observed. Growth curves and sexual development were not modified. CONCLUSION Our data support the efficacy of HU in reducing painful events in children with sickle cell disease. Short- and middle-term tolerances are good. Thus, we think that HU can be given to children affected by frequent and severe painful crises. We recommend, however, very cautious use of this drug, because its long-term effects in children are still unknown.
Understanding User Requirements for Music Information Services

User studies in the music information retrieval and music digital library fields have been gradually increasing in recent years, but large-scale studies that can help detect common user behaviors are still lacking. We have conducted a large-scale user survey in which we asked numerous questions related to users’ music needs, uses, seeking, and management behaviors. In this paper, we present our preliminary findings, specifically focusing on the responses to questions of users’ favorite music related websites/applications and the reasons why they like them. We provide a list of popular music services, as well as an analysis of how these services are used, and what qualities are valued. Our findings suggest several trends in the types of music services people like: an increase in the popularity of music streaming and mobile music consumption, the emergence of new functionality, such as music identification and cloud music services, an appreciation of music videos, serendipitous discovery of music, and customizability, as well as users’ changing expectations of particular types of music information. Understanding what kinds of music information services people use, how they use them, and what they expect from them is critical in designing successful services. We have seen a gradual increase in different types of user and usability studies in recent years. However, many of these studies are based on a limited number of subjects, and tend to employ analysis of qualitative research methods, like in-depth interviews or focus groups. While these kinds of studies can help uncover rich data about music users, large-scale user studies are also necessary in order to test the generalizability of results and to complement the insights obtained from smaller qualitative studies. To fill this gap, we have conducted a large-scale user survey questioning people’s music needs, uses, and music seeking and management behaviors. This survey is an extension of previous research conducted in 2004 by Lee and Downie [7]. The information we acquired through this new study can help improve our general understanding of music users and their behaviors, as well as how they have changed as compared to the 2004 survey results. 2. LITERATURE REVIEW
The Repertoire of Chemical Defense Genes in the Coral Acropora digitifera Genome

Scleractinian corals are of fundamental ecological significance in tropical and sub-tropical shallow water. This ecological success is attributed to their ability of formation of obligate endosymbioses with dinoflagellates of the genus Symbiodinium. Nevertheless, approximately one-third of reef-building coral species are critically endangered and the remainder are under threat from the effects of climate change and local impacts. Molecular and cellular mechanisms involved in stress responses and the establishment and collapse of the symbiosis are therefore an urgent subject of research. Metazoans possess large numbers of genes that participate in response to environmental stressors, and chemical defense genes included P450 and other oxidases, various conjugating enzymes, ATP-dependent efflux transporters, oxidative detoxification proteins, as well as transcription factors that regulate these genes. Here we searched those genes in recently decoded the coral Acropora digitifera genome. We found that this genome contains a set of chemical defense genes in numbers comparable with other cnidarians and metazoans and that there are some lineagespecific gene family expansions in the coral genome. These provide information for future research into molecular mechanisms involved in coral stress responses.
Looking at People Using Partial Least Squares

Analysis of images involving humans is of significant interest in computer vision because problems such as detection, modeling, recognition, and tracking are fundamental to model interactions between people and understand highlevel activities. Visual information contained in images is generally represented using feature descriptors. Many general classes of descriptors have been proposed focusing on different characteristics of images. Therefore, if one considers only a single descriptor, one might ignore useful information for a given task, compromising performance. In this work we consider a rich set of image descriptors analyzed by a statistical technique known as Partial Least Squares (PLS). PLS is a class of methods for modeling relations between sets of observations by means of latent variables and it is used to project exemplars from a very high dimensional feature space onto a low dimensional subspace. We first propose a method based on PLS to detect humans. Then, a framework based on a one-against-all classification scheme using PLS regression is described for face recognition. Results obtained for human detection and face recognition outperform state-of-art methods. Keywords-human detection; face recognition; Partial Least Squares;
Watching Eyes and Living up to Expectations: Unkind, Not Kind, Eyes Increase First Mover Cooperation in a Sequential Prisoner's Dilemma

(1) Background: Why and when images of watching eyes encourage prosocial behavior is still subject to discussion, and two recent meta-analyses show no effect of watching eyes on generosity. This study aims to discern the effect of watching eyes of different valence on two separate aspects of prosocial behavior, and additionally investigates whether individuals’ social value orientation moderates the effect of eyes. (2) Methods: Individuals take on the role of either a first or second mover in an incentivized, anonymous sequential prisoner’s dilemma (n = 247), a two-person game which separates the need to form expectations about the other player (first mover cooperation, trust) from the motive of greed (second mover cooperation, reciprocity). During decision-making, a picture of either kind eyes, unkind eyes, or a control picture is presented above each decision matrix. (3) Results: The results indicate that unkind eyes, and not kind eyes, significantly boost first mover cooperation. In contrast, neither type of eye cues increase second mover cooperation. Social value orientation does not moderate these effects. (4) Conclusions: Thus, the data suggest that the valence of eye cues matters, and we propose that unkind eyes urge first movers to live up to the interaction partner’s expectations.
Blind Multicenter Study of the Efficacy and Safety of Injections of a Commercial Preparation of Botulinum Toxin Type A Reconstituted up to 15 Days Before Injection

BACKGROUND It is recommended that attention be given to the shelf life of botulinum toxin type A (BT‐A) after its reconstitution. OBJECTIVE To assess the efficacy and safety of 500 U of BT‐A after reconstitution up to 15 days after injection. MATERIAL AND METHODS BT‐A vials were diluted 15 days, 8 days, and 8 hours before injection. One hundred five volunteers were randomized to one of three treatment groups, according to dilution dates. They were evaluated at baseline and 28, 56, 84, and 112 days after treatment. At each visit, the investigator and the volunteer evaluated the motility of the treated area using a 4‐point qualitative scale. Five independent specialists, who scored the motility of the treated area on the same scale, blind analyzed photographs taken at each visit. The reconstituted vials of BT‐A were stored and analyzed before and after the study. RESULTS No significant difference was shown between the groups. No evidence of contamination was found in the BT‐A vials. CONCLUSION The results confirm the possibility of injecting 500 U of BT‐A up to 15 days after its reconstitution safely and without loss of efficacy.
Resistance of goat nematodes to multiple anthelmintics on a farm in France

ANTHELMINTIC resistance in nematodes of small ruminants has been reported on all continents ([Kaplan 2004][1]). In France, several cases of benzimidazole resistance have been reported in goats ([Chartier and Pors 1994][2], [Cabaret and others 1995][3], [Chartier and others 1998][4], 2001).
From Hans Selye's Discovery of Biological Stress to the Identification of Corticotropin‐ Releasing Factor Signaling Pathways

Selye pioneered the concept of biological stress in 1936, culminating in the identification of the corticotropin‐releasing factor (CRF) signaling pathways by Vale's group in the last two decades. The characterization of the 41 amino‐acid CRF and other peptide members of the mammalian CRF family, urocortin 1, urocortin 2, and urocortin 3, and the cloning of CRF1 and CRF2 receptors, which display distinct affinity for CRF ligands, combined with the development of selective CRF receptor antagonists enable us to unravel the importance of CRF1 receptor in the stress‐related endocrine (activation of pituitary‐adrenal axis), behavioral (anxiety/depression, altered feeding), autonomic (activation of sympathetic nervous system), and immune responses. The activation of CRF1 receptors is also one of the key mechanisms through which various stressors impact the gut to stimulate colonic propulsive motor function and to induce hypersensitivity to colorectal distension as shown by the efficacy of the CRF1 receptor antagonists in blunting these stress‐related components. The importance of CRF1 signaling pathway in the visceral response to stress in experimental animals provided new therapeutic approaches for treatment of functional bowel disorder such as irritable bowel syndrome, a multifactor functional disorder characterized by altered bowel habits and visceral pain, for which stress has been implicated in the pathophysiology and is associated with anxiety‐depression in a subset of patients.
Diagnostic outcome of self-reported hallucinations in a community sample of adolescents

Objective. We aimed to assess the diagnostic outcome of self-reported hallucinations in adolescents from the general population. Method. The sample consisted of 914 adolescents between ages 11–18 participating in an ongoing longitudinal study. The participation rate from the original sample was 70%. Responses on the Youth Self-Report questionnaire were used to ascertain hallucinations in adolescents. Eight years later, Axis 1 DSM-IV diagnoses were assessed using the 12-month version Composite International Diagnostic Interview in 783 (86%) of 914 study subjects. No subjects were diagnosed with schizophreniform disorders or schizophrenia. Results. Hallucinations were reported by 6% of adolescents and 3% of young adults. Self-reported hallucinations were associated with concurrent non-psychotic psychiatric problems in both age groups. Adolescents who reported auditory, but not visual, hallucinations, had higher rates of depressive disorders and substance use disorders, but not psychotic disorders, at follow-up, compared to controls. Conclusions. Self-reported auditory hallucinations in adolescents are markers of concurrent and future psychiatric impairment due to non-psychotic Axis 1 disorders and possibly Axis 2 disorders. It cannot be excluded that there was selective attrition of children and adolescents who developed Schizophrenic or other psychotic disorders later in life.
Comparison of real-time PCR and Kato smear microscopy for the detection of hookworm infections in three consecutive faecal samples from schoolchildren in Ghana.

BACKGROUND The classic technique used to detect hookworm infections in population-based surveys is microscopic examination of Kato thick smears of multiple faecal samples per person as variation in soil-transmitted helminth egg output is common. METHODS As an alternative to this time-consuming and logistically difficult procedure, a PCR-based method to detect hookworm infections was evaluated. Faecal samples collected from 65 Ghanaian school children during February-June 2006 were examined using both techniques. RESULTS Thirty-one children with a hookworm infection were detected by Kato examination of three faecal samples compared with 30 children detected by PCR of a single faecal sample and 39 detected by PCR of three faecal samples. CONCLUSION PCR provides a sensitive alternative to the conventional microscopic detection of hookworm infections.
Evidence-based provisional clinical classification criteria for autoinflammatory periodic fevers

The objective of this work was to develop and validate a set of clinical criteria for the classification of patients affected by periodic fevers. Patients with inherited periodic fevers (familial Mediterranean fever (FMF); mevalonate kinase deficiency (MKD); tumour necrosis factor receptor-associated periodic fever syndrome (TRAPS); cryopyrin-associated periodic syndromes (CAPS)) enrolled in the Eurofever Registry up until March 2013 were evaluated. Patients with periodic fever, aphthosis, pharyngitis and adenitis (PFAPA) syndrome were used as negative controls. For each genetic disease, patients were considered to be ‘gold standard’ on the basis of the presence of a confirmatory genetic analysis. Clinical criteria were formulated on the basis of univariate and multivariate analysis in an initial group of patients (training set) and validated in an independent set of patients (validation set). A total of 1215 consecutive patients with periodic fevers were identified, and 518 gold standard patients (291 FMF, 74 MKD, 86 TRAPS, 67 CAPS) and 199 patients with PFAPA as disease controls were evaluated. The univariate and multivariate analyses identified a number of clinical variables that correlated independently with each disease, and four provisional classification scores were created. Cut-off values of the classification scores were chosen using receiver operating characteristic curve analysis as those giving the highest sensitivity and specificity. The classification scores were then tested in an independent set of patients (validation set) with an area under the curve of 0.98 for FMF, 0.95 for TRAPS, 0.96 for MKD, and 0.99 for CAPS. In conclusion, evidence-based provisional clinical criteria with high sensitivity and specificity for the clinical classification of patients with inherited periodic fevers have been developed.
Electromyography in diagnosis of nerve root compression syndrome.

ELECTROMYOGRAPHY, long used in physiologic research, has recently emerged as a clinical diagnostic method with a high degree of accuracy and widespread clinical application. With the recent advances in electronics it is now possible to produce commercially a sensitive instrument which utilizes a needle electrode placed directly into a denervated muscle and which records characteristic action potentials by means of a cathode ray oscilloscope. Such an instrument1has been used in this study to determine the exact location of lesions causing compression of spinal nerve roots. This localization depends on the accurate detection of the action potentials of denervation fibrillation, whereas heretofore electromyographic localization of a lesion of a single nerve root has depended on the detection of fasciculation by the use of percutaneous electrodes, a much less accurate method (page 96). Visible fibrillation of a denervated muscle was first described by Schiff in 1851, when he sectioned the
PML expression in soft tissue sarcoma: Prognostic and predictive value in alkylating agents/antracycline‐based first line therapy

Soft tissue sarcomas are aggressive tumors representing <1% of all adult neoplasms. Aim of our study was to evaluate promyelocytic leukemia gene expression value as prognostic factor and as a factor predicting response to alkylating agents/antracycline‐based first line therapy. One hundred eleven patients affected by locally advanced and metastatic soft tissue sarcoma were selected. PML expression was evaluated by immunohistochemical analysis in pathological samples and in the corresponding normal tissue from each case. PML immunohistochemical results were correlated with prognosis and with radiological response to alkylating agents/antracycline‐based first line therapy. PML expression was significantly reduced in synovial sarcomas (P < 0.0001), in myofibroblastic sarcomas (P < 0.0001), angiosarcomas (P < 0.0001), in leiomyosarcomas (P = 0.003), in mixoid liposarcomas (P < 0.0001), and in dedifferentiated liposarcomas (P < 0.0001). No significant difference was found for pleomorphic sarcoma [31.8 (95% CI: 16.7–41.0); P = 0.21]. and pleomorphic liposarcomas (P = 0.51). Loss of PML expression was found to be statistically correlated with TTP (P < 0.0001), median duration of response (P = 0.007), and OS (P = 0.02). No correlation was observed between PML expression and treatment efficacy. PML IHC expression is down‐regulated in synovial sarcomas, myofibroblastic sarcomas, angiosarcomas, liposarcoma, and leiomyosarcomas and its expression correlated with prognosis. J. Cell. Physiol. 227: 1657–1662, 2012. © 2011 Wiley Periodicals, Inc.
The Dopamine Receptor D5 May Influence Age of Onset

The objective was to investigate contribution of the dopamine receptor 5 (DRD5) gene variants in the symptoms of attention-deficit/hyperactivity disorder (ADHD) probands since brain regions identified to be affected in these group of patients have higher expression of the DRD5 receptor. Out of 22 exonic variants, 19 were monomorphic in the Indo-Caucasoid individuals. rs6283 “C” and rs113828117 “A” exhibited significant higher occurrence in families with ADHD probands. Several haplotypes showed biased occurrence in the probands. Early and late onset groups exhibited significantly different genotypic frequencies. A new G>A substitution was observed in the control samples only. The late onset group exhibited higher scores for hyperactivity as compared to the early onset group. The authors infer that the age of onset of ADHD may at least partially be affected by DRD5 variants warranting further investigation on the role of DRD5 in the disease etiology.
Regulation of Experimental Autoimmune Encephalomyelitis by Interleukin‐12

We have evaluated the effects of rmIL-12 on the course of adoptively transferred EAE. When mice were injected with LNC that had been stimulated in vitro with PLP in the presence of rmIL-12, the subsequent course of disease was more severe and prolonged than controls. In vitro stimulation with PLP in the presence of IL-12 was associated with an increase in IFN-gamma and decrease in IL-4-producing cells, indicating a preferential expansion of Th1 effector cells. At peak disease, no notable differences in either the cellular composition or cytokine expression within CNS lesions was seen between groups. However, the frequency of macrophages that stained positively for inducible nitric oxide synthase (iNOS) was increased in animals challenged with rmIL-12 treated LNC. These data suggest that in addition to promoting the preferential expansion of IFN-gamma-producing cells by rmIL-12 treatment in vitro, in vivo effects leading to macrophage activation and iNOS expression may contribute to the severe, protracted course of CNS inflammation in this model. In contrast, treatment of mice with an antibody to murine IL-12 following cell transfer completely prevented paralysis with only 40% of the mice developing mild disease. These data suggest that endogenous IL-12 plays a pivotal role in the pathogenesis of this model of autoimmune disease.
Determination of sialic acid and biochemical parameters level in β-thalassemic patients

This research was concerned with the biochemical changes that might occur in patients with β-thalassemia major, multiple determinations were obtained 20 subjects: - 13 children with β-thalassemia major and 7 normal children (as normal healthy control). The parameters deduced from this study includes:- serum total sialic acid (TSA), erythrocytes sedimentation rate (ESR) and seromucoid total protein (TP). From the data obtained for these parameters investigate the decrease in serumTSA and TP of thalassemic patients compared to normal individuals, while the ESR levels increase for patients as compared to other individuals.