Miracle-dz/UniFAR-FTUs huggingface dataset:
PRIMARY HYPEROXALURIA TYPE 1: IMPROVED OUTCOME WITH TIMELY LIVER TRANSPLANTATION: A SINGLE-CENTER REPORT OF 36 CHILDREN

Background. The appropriate use of liver transplantation in children with type-1 primary hyperoxaluria (PH-1) is not well established. We reviewed our experience with 36 children with PH-1, including 12 who underwent liver transplantation. Patients and Methods. From 1989–1998, 36 children from 10 families in northern Israel were diagnosed with PH-1. Eight children presented with renal failure; seven of these eight had the severe infantile form of the disease. One child was treated with kidney transplantation alone. Combined liver-kidney transplantation has been performed in nine children and preemptive liver transplantation in three children. A review of the patients’ charts for the following parameters was performed: age, clinical signs, and renal sonographic findings at diagnosis, age at onset of dialysis, and current status. Type of transplant, pre- and posttransplant urine oxalate excretion, current renal function, survival, and complications were recorded in liver recipients. Results. Of the 23 nontransplanted children, 9 died of complications related to severe systemic oxalosis and 14 are alive (mean follow-up, 7.4 years), including 2 who are candidates for transplantation. The child who underwent only kidney transplantation died of unrelated causes. Of the 12 liver recipients, 2 died within the first 3 months posttransplant and another child underwent retransplantation due to hepatic arterial thrombosis. At intervals after transplant ranging from 6–54 months, 10 recipients are alive (7 of the 9 recipients of combined liver-kidney transplants and all 3 recipients of preemptive liver transplants). Mean GFR in the 10 survivors is 77 ml/min/m2. In 9 of these 10, daily urinary oxalate excretion normalized. Renal function has improved (mean GFR 86 vs. 58 ml/min/m2) but renal oxalate deposits remain in the three recipients of isolated liver grafts. Conclusions. Our decade-long experience with children with PH-1 supports strategies for early diagnosis and timely liver transplantation. Preemptive isolated liver transplantation should be considered in children who develop the disease during infancy or in those with slowly progressive disease when significant symptoms develop. Combined liver-kidney transplantation is suggested for children with end-stage renal disease.
Molecular Cloning and Characterization of Lustrin A, a Matrix Protein from Shell and Pearl Nacre of Haliotis rufescens *

A specialized extracellular matrix of proteins and polysaccharides controls the morphology and packing of calcium carbonate crystals and becomes occluded within the mineralized composite during formation of the molluscan shell and pearl. We have cloned and characterized the cDNA coding for Lustrin A, a newly described matrix protein from the nacreous layer of the shell and pearl produced by the abalone, Haliotis rufescens, a marine gastropod mollusc. The full-length cDNA is 4,439 base pairs (bp) long and contains an open reading frame coding for 1,428 amino acids. The deduced amino acid sequence reveals a highly modular structure with a high proportion of Ser (16%), Pro (14%), Gly (13%), and Cys (9%). The protein contains ten highly conserved cysteine-rich domains interspersed by eight proline-rich domains; a glycine- and serine-rich domain lies between the two cysteine-rich domains nearest the C terminus, and these are followed by a basic domain and a C-terminal domain that is highly similar to known protease inhibitors. The glycine- and serine-rich domain and at least one of the proline-rich domains show sequence similarity to proteins of two extracellular matrix superfamilies (one of which also is involved in the mineralized matrixes of bone, dentin, and avian eggshell). The arrangement of alternating cysteine-rich domains and proline-rich domains is strikingly similar to that found in frustulins, the proteins that are integral to the silicified cell wall of diatoms. Its modular structure suggests that Lustrin A is a multifunctional protein, whereas the occurrence of related sequences suggest it is a member of a multiprotein family.
Coping and Health Outcomes In Spouse Caregivers of Persons with Dementia

The effects of different coping patterns on the physical health, depression, and anxiety experienced by 60 spouse caregivers of persons with dementia were examined, using Lazarus and Folkman's cognitive model of stress and coping. In addition to coping, the model included the following predictors: severity of the patient's memory and behavior problems, caregiver's appraisal of the stressfulness of those problems, and caregiver's appraisal of their options for managing caregiving. The predictors did not explain a significant amount of the variance in caregivers' physical health, but they did explain 43% of the variance in both depression and anxiety. The only coping pattern that added to the explanations was Wishing-Emotive coping, which consisted of the coping subscales of escape-avoidance, confrontive coping, and accepting responsibility. Caregivers' appraisal of stress was a significant predictor of depression and anxiety, but neither the severity of the patients' problems nor caregivers' appraisal of options was a significant predictor of any of the health outcomes.
[Experimental models for assessment of pulmonary alterations in hepatopulmonary syndrome].

OBJECTIVE The aim of this study was to identify the best experimental model in which to observe the pulmonary alterations characterizing hepatopulmonary syndrome (HPS). METHODS Male Wistar rats, with mean weight of 250 g, were used in four experimental models: inhaled carbon tetrachloride; intraperitoneal carbon tetrachloride; partial portal vein ligation; and bile duct ligation (BDL). The animals in all groups were divided into control and experimental subgroups. The following variables were measured: transaminase levels; blood gases; lipoperoxidation, using thiobarbituric acid reactive substances (TBARS) and chemiluminescence; and levels of superoxide dismutase (SOD) anti-oxidant activity. Anatomopathological examination of the lung was also performed. RESULTS There were statistically significant differences between the BDL control and BDL experimental groups: aspartate aminotransferase (105.3 +/- 43 vs. 500.5 +/- 90.3 IU/L); alanine aminotransferase (78.75 +/- 37.7 vs. 162.75 +/- 35.4 IU/L); alkaline phosphatase (160 +/- 20.45 vs. 373.25 +/- 45.44 IU/L); arterial oxygen tension (85.25 +/- 8.1 vs. 49.9 +/- 22.5 mmHg); and oxygen saturation (95 +/- 0.7 vs. 73.3 +/- 12.07%). Lipoperoxidation and antioxidant activity also differed significantly between the two BDL groups (control vs. experimental): TBARS (0.87 +/- 0.3 vs. 2.01 +/- 0.9 nmol/mg protein); chemiluminescence (16008.41 +/- 1171.45 vs. 20250.36 +/- 827.82 cps/mg protein); and SOD (6.66 +/- 1.34 vs. 16.06 +/- 2.67 IU/mg protein). The anatomopathological examination confirmed pulmonary vasodilatation in the BDL model. In the other models, there were no alterations that were characteristic of HPS. CONCLUSIONS The data obtained suggest that the BDL model can be used in future studies involving hepatic alterations related to oxidative stress and HPS.
Combination of Alphavirus Replicon Particle–Based Vaccination with Immunomodulatory Antibodies: Therapeutic Activity in the B16 Melanoma Mouse Model and Immune Correlates

Avogadri and colleagues show that anti-CTLA-4 or anti-GITR immunomodulatory antibody improves the efficacy of a nonpathogenic viral vector–based vaccine (VRP-TRP-2) in the B16F10 melanoma mouse model. Superior antitumor protection conferred by anti-GITR was associated with enhanced humoral response and reduced CD4+PD-1+ T-cell intratumoral accumulation. Induction of potent immune responses to self-antigens remains a major challenge in tumor immunology. We have shown that a vaccine based on alphavirus replicon particles (VRP) activates strong cellular and humoral immunity to tyrosinase-related protein-2 (TRP2) melanoma antigen, providing prophylactic and therapeutic effects in stringent mouse models. Here, we report that the immunogenicity and efficacy of this vaccine is increased in combination with either antagonist anti-CTL antigen-4 (CTLA-4) or agonist anti-glucocorticoid-induced TNF family–related gene (GITR) immunomodulatory monoclonal antibodies (mAb). In the challenging therapeutic setting, VRP–TRP2 plus anti-GITR or anti–CTLA-4 mAb induced complete tumor regression in 90% and 50% of mice, respectively. These mAbs had similar adjuvant effects in priming an adaptive immune response against the vaccine-encoded antigen, augmenting, respectively, approximately 4- and 2-fold the TRP2-specific CD8+ T-cell response and circulating Abs, compared with the vaccine alone. Furthermore, while both mAbs increased the frequency of tumor-infiltrating CD8+ T cells, anti–CTLA-4 mAb also increased the quantity of intratumor CD4+Foxp3− T cells expressing the negative costimulatory molecule programmed death-1 (PD-1). Concurrent GITR expression on these cells suggests that they might be controlled by anti-GITR mAbs, thus potentially explaining their differential accumulation under the two treatment conditions. These findings indicate that combining immunomodulatory mAbs with alphavirus-based anticancer vaccines can provide therapeutic antitumor immune responses in a stringent mouse model, suggesting potential utility in clinical trials. They also indicate that tumor-infiltrating CD4+Foxp3−PD-1+ T cells may affect the outcome of immunomodulatory treatments. Cancer Immunol Res; 2(5); 448–58. ©2014 AACR.
Catecholaminergic systems in the zebrafish. II. Projection pathways and pattern of termination of the locus coeruleus

The locus coeruleus is a widely projecting isthmal noradrenergic nucleus. In the zebrafish, it consists of between three and ten neurons, most of which have multiple, bilaterally projecting axons. Immunohodolgical studies show that the locus coeruleus provides most, if not all, of the noradrenergic innervation of the brain rostral to the isthmus. The pathways and targets in the zebrafish are similar to ascending coeruleal projections of other vertebrates. Axons ascend through two amin pathways: the longitudinal catecholamine bundle and the periventricular catecholamine pathway. The former is a dense meshwork of varicosity‐bearing axons which ascends along the lateral longitudinal fasciculus into the mesencephalon. In the posterior tuberal area, this bundle dives ventrally and assumes a lateral position. In the diencephalon, it takes up a position ventral to the medial forebrain bundle, and follows this bundle into the telencephalon, where it joins the medial olfactory tract to enter the olfactory bulb. The periventricular catecholamine pathway is a diffuse pathway consisting of thick, smooth axons. It is associated with the medial longitudianl fasciculus. Rostral to the nucleus of the medial longitudinal fasciculus, this pathway joins the longitudinal catecholamine bundle around the medial forebrain bundle. The Periventricular pathway gives rise to coarse terminal arbors with large but sparse varicosities, whereas the longitudinal catecholamine bundle gives rise to terminal plexuses with fine and dense fibers and varicosities. Among the more densely innervated regions are the raphé nucleus, the interpeduncular nucleus, the torus semicircularis, parts of the hypothalamus, and the suprachiasmatic and preoptic areas. The tours longitudinalis, optic tectum, cerebellum, habenular complex, the dorsomedial zone of area dorsalis telencephali, and the olfactory bulb are moderately innervated. The nucleus glomerulosus, the torus lateralis and lateral subnuclei of the nucleus diffusus, and the anterior tuberal nucleus are devoid of noradrenergic innervation. © 1994 Wiley‐Liss, Inc.
Emotion sensing from physiological signals using three defined areas in arousal-valence model

This paper aims to recognize the human emotional states into three defined areas in arousal-valence evaluation: Corresponding to calm, medium aroused, and excited, unpleasant, neutral valence and pleasant. And thanks to the relevance of the peripheral physiological signals in emotion recognition issue, we used in our contribution the multimodal dataset MAHNOB-HCI. In this database, there are emotional bodily responses of twenty four participants after watching twenty affective stimuli videos. In our work, we focused on the ElectroCardioGram (ECG), Galvanic Skin Response (GSR), Skin Temperature (Temp) and Respiration Volume (RESP). To accomplish our purpose, we pre-process the data, extract 169 features and finally, classify the emotional states by using the support vector machine (SVM). As a first step, we classify each signal to know the most relevant physiological signal for emotion assessing, then a level feature fusion is applied to compare our approach to related work. According to previous studies, our obtained results are promising and show that the respiration and electrocardiogram are the most relevant.
Prevalence of Barrett's esophagus in the general population: an endoscopic study.

BACKGROUND & AIMS Barrett's esophagus (BE) is associated with esophageal adenocarcinoma, the incidence of which has been increasing dramatically. The prevalence of BE in the general population is uncertain because upper endoscopy is required for diagnosis. This study aimed to determine the prevalence of BE and possible associated risk factors in an adult Swedish population. METHODS A random sample (n = 3000) of the adult population (n = 21,610) in 2 municipalities was surveyed using a validated gastrointestinal symptom questionnaire (response rate, 74%); a random subsample (n = 1000; mean age, 53.5 years; 51% female) underwent upper endoscopy. Endoscopic signs suggestive of columnar-lined esophagus (CLE) were defined as mucosal tongues or an upward shift of the squamocolumnar junction. BE was diagnosed when specialized intestinal metaplasia was detected histologically in suspected CLE. RESULTS BE was present in 16 subjects (1.6%; 95% confidence interval, 0.8-2.4): 5 with a long segment and 11 with a short segment. Overall, 40% reported reflux symptoms and 15.5% showed esophagitis; 103 (10%) had suspected CLE, and 12 (1.2%) had a visible segment > or = 2 cm. The prevalence of BE in those with reflux symptoms was 2.3% and in those without reflux symptoms was 1.2% (P = .18). In those with esophagitis, the prevalence was 2.6%; in those without, the prevalence was 1.4% (P = .32). Alcohol (P = .04) and smoking (P = .047) were independent risk factors for BE. CONCLUSIONS BE was found in 1.6% of the general Swedish population. Alcohol and smoking were significant risk factors.
Coronary artery remodelling is related to plaque composition

Objective: To assess the potential relation between plaque composition and vascular remodelling by using spectral analysis of intravascular ultrasound (IVUS) radiofrequency data. Methods and results: 41 coronary vessels with non-significant (< 50% diameter stenosis by angiography), ⩽ 20 mm, non-ostial lesions located in non-culprit vessels underwent IVUS interrogation. IVUS radiofrequency data obtained with a 30 MHz catheter, were analysed with IVUS virtual histology software. A remodelling index (RI) was calculated and divided into three groups. Lesions with RI ⩾ 1.05 were considered to have positive remodelling and lesions with RI ⩽ 0.95 were considered to have negative remodelling. Lesions with RI ⩾ 1.05 had a significantly larger lipid core than lesions with RI 0.96–1.04 and RI ⩽ 0.95 (22.1 (6.3) v 15.1 (7.6) v 6.6 (6.9), p < 0.0001). A positive correlation between lipid core and RI (r = 0.83, p < 0.0001) and an inverse correlation between fibrous tissue and RI (r = −0.45, p = 0.003) were also significant. All of the positively remodelled lesions were thin cap fibroatheroma or fibroatheromatous lesions, whereas negatively remodelled lesions had a more stable phenotype, with 64% having pathological intimal thickening, 29% being fibrocalcific lesions, and only 7% fibroatheromatous lesions (p < 0.0001). Conclusions: In this study, in vivo plaque composition and morphology assessed by spectral analysis of IVUS radiofrequency data were related to coronary artery remodelling.
Idiopathic Thrombocytopenic Purpura Complicated by an Intracranial Hemorrhage Secondary to an Arteriovenous Malformation

Objective: To report a case of idiopathic thrombocytopenic purpura (ITP) complicated by an intracranial hemorrhage (ICH) in a child with a previously undiagnosed arteriovenous malformation. Case: We describe a child with known ITP who developed a severe headache, was evaluated in an emergency department of a community hospital, and was found by computer tomography (CT) scan to have an ICH. Despite treatment with platelets, corticosteroids, and intravenous immunoglobulin, she subsequently developed an acute change in mental status. A second CT scan showed that the hemorrhage had significantly increased in size despite treatment. The patient underwent an emergent splenectomy prior to a craniotomy to remove the hemorrhage. At the time of surgery, it was discovered that she had an arteriovenous malformation at the sight of the hemorrhage. Her recovery was unremarkable and she was discharged to home with no neurologic sequelae. Conclusions: ICH is a rare but life-threatening complication of ITP. Neurologic symptoms in a child with ITP should be quickly evaluated by CT scan. Most experts suggest careful observation for most cases of ITP. However, when neurologic symptoms occur, more aggressive treatment options must be used. Care of this child included an emergency splenectomy prior to her craniotomy. Pediatric emergency medicine practitioners must be aware of these neurologic symptoms and must not hesitate to involve pediatric surgeons and neurosurgeons in the care of the child. Prompt recognition and early intervention are the keys to improving outcomes when ICH complicates ITP.
SAMHD1 Degradation Enhances Active Suppression of Dendritic Cell Maturation by HIV-1

A hallmark of HIV-1 infection is the lack of sterilizing immunity. Dendritic cells (DCs) are crucial in the induction of immunity, and lack of DC activation might underlie the absence of an effective anti–HIV-1 response. We have investigated how HIV-1 infection affects maturation of DCs. Our data show that even though DCs are productively infected by HIV-1, infection does not induce DC maturation. HIV-1 infection actively suppresses DC maturation, as HIV-1 infection inhibited TLR-induced maturation of DCs and thereby decreased the immune stimulatory capacity of DCs. Interfering with SAMHD1 restriction further increased infection of DCs, but did not lead to DC maturation. Notably, higher infection observed with SAMHD1 depletion correlated with a stronger suppression of maturation. Furthermore, blocking reverse transcription rescued TLR-induced maturation. These data strongly indicate that HIV-1 replication does not trigger immune activation in DCs, but that HIV-1 escapes immune surveillance by actively suppressing DC maturation independent of SAMHD1. Elucidation of the mechanism of suppression can lead to promising targets for therapy or vaccine design.
Does fear reactivity during exposure predict panic symptom reduction?

OBJECTIVE Fear reactivity during exposure is a commonly used indicator of learning and overall therapy outcome. The objective of this study was to assess the predictive value of fear reactivity during exposure using multimodal indicators and an advanced analytical design. We also investigated the degree to which treatment condition (cognitive training vs. respiratory skill training) moderated fear reactivity and therapeutic outcome. METHOD Thirty-four patients with panic disorder and agoraphobia completed a total of 123 in-vivo exposure sessions, comprising 3 weekly sessions and a 4th session 2 months following therapy completion. Sessions varied in length and phobic stimuli. Cardiorespiratory physiology (heart rate, carbon dioxide partial pressure [PCO2], respiration rate) and experiential symptoms (panic symptoms and anxiety) were assessed repeatedly throughout exposure sessions, in addition to weekly assessments of panic cognitions, avoidance, and functioning. RESULTS Panic symptomatology decreased substantially in both treatment conditions during therapy and follow-up. Significant cardiorespiratory and experiential reactivity was observed during all exposures, characterized by activation followed by reduction. Greater within-session activation of anxiety and panic symptoms was inversely related to improvement in panic symptoms severity, but neither physiological activation nor within- or between-session reduction of either physiological or experiential variables was predictive of outcome. No moderating effects of treatment condition were found. CONCLUSIONS Fear activation and reduction during exposure are weak predictors of corrective learning and fear extinction. Clinical implications for exposure therapy and directions for future research are discussed.
MPEG Surround – the ISO/MPEG Standard for Efficient and Compatible Multi-Channel Audio Coding

In 2004 the ISO/MPEG Audio standardization group started a new work item on efficient and backward-compatible coding of high-quality multichannel sound using parametric coding techniques. Finalized in the fall of 2006, the resulting MPEG Surround specification allows the transmission of surround sound at bit rates that have been commonly used for coding of mono or stereo sound. The results of the standardization process are summarized by describing the underlying ideas and providing an overview of the MPEG Surround technology. The performance of the scheme is characterized by the results of recent verification tests. These tests include several operation modes as they would be used in typical application scenarios to introduce multichannel audio into existing audio services.
Photosystem II Excitation Pressure and Development of Resistance to Photoinhibition (II. Adjustment of Photosynthetic Capacity in Winter Wheat and Winter Rye)

Winter wheat (Triticum aestivum L. cv Monopol), spring wheat (Triticum aestivum L. cv Katepwa), and winter rye (Secale cereale L. cv Musketeer) grown at 5[deg]C and moderate irradiance (250 [mu]mol m-2 s-1) (5/250) exhibit an increased tolerance to photoinhibition at low temperature in comparison to plants grown at 20[deg]C and 250 [mu]mol m-2 s-1 (20/250). However, 5/250 plants exhibited a higher photosystem II (PSII) excitation pressure (0.32–0.63) than 20/250 plants (0.18–0.21), measured as 1 - qP, the coefficient of photochemical quenching. Plants grown at 20[deg]C and a high irradiance (800 [mu]mol m-2 s-1) (20/800) also exhibited a high PSII excitation pressure (0.32–0.48). Similarly, plants grown at 20/800 exhibited a comparable tolerance to photoinhibition relative to plants grown at 5/250. In contrast to a recent report for Chlorella vulgaris (D.P. Maxwell, S. Falk, N.P.A. Huner [1995] Plant Physiol 107: 687–694), this tolerance to photoinhibition occurs in winter rye with minimal adjustment to polypeptides of the PSII light-harvesting complex, chlorophyll a/b ratios, or xanthophyll cycle carotenoids. However, Monopol winter wheat exhibited a 2.5-fold stimulation of sucrosephosphate synthase activity upon growth at 5/250, in comparison to Katepwa spring wheat. We demonstrate that low-temperature-induced tolerance to photoinhibition is not a low-temperature-growth effect per se but, instead, reflects increased photosynthetic capacity in response to elevated PSII excitation pressure, which may be modulated by either temperature or irradiance.