Miracle-dz/UniFAR-FTUs huggingface dataset:
Hepatolenticular Degeneration Combined with Primary Antiphospholipid Syndrome: A Case Report

biochemical parameters were within the normal reference ranges. Thrombocytes were 77 ! 10 9 /l; neither thrombocyte aggregation nor schistocytes were found on peripheral blood smear examination. The autoantibodies against ds-DNA, Sm, Ro/ SS-A, La/SS-B and RNP antigens (ELISA) and ANA titer (1: 40 HEP-2) were negative. The antiphospholipid antibodies – IgG isotype (48 GPL) and IgM isotype (56 GPL) anticardiolipin antibodies – were elevated (ELISA) as well as those of 2 -glycoprotein (IgG 11.5, IgM 2.0). The lupus anticoagulant (LAC) test was positive while the Wassermann and antithrombotic antibody tests were negative. A repeat antiphospholipid antibody test after 3 months was again positive. The samples tested for the WaalerRose rheumatoid factor were negative. The neuroborreliosis examination was negative both in serum and cerebrospinal fl uid. A bone marrow puncture was performed because of current thrombocytopenia: the cytologic examination evidenced bone marrow hyperplasia of hypercellular megakaryocytic type. Liver and spleen ultrasonography demonstrated hepatosplenomegaly (liver at the right medioclavicular line – 152 mm, homogeneous normoechogenic structure; cross-section size of spleen – 50 mm). Brain magnetic resonance imaging (MRI) at Dear Sir, Hepatolenticular degeneration (HLD), also known as Wilson’s disease, is an autosomal recessive defect in the metabolism of copper accompanied by a clinical constellation of symptoms of brain and liver damage [1, 2] . Clinical progression can vary from favorable to untreatable depending on an early and accurate diagnosis [3] . Primary antiphospholipid syndrome (PAS) is an autoimmune disorder in which the antiphospholipid antibody production causes recurrent arterial and venous thrombosis, thrombocytopenia and symptoms of connective tissue disease [4, 5] . We report and discuss the unusual combination of HLD and PAS.
Identification of Staphylococcus aureus exotoxins by combined sodium dodecyl sulfate gel electrophoresis and matrix‐assisted laser desorption/ ionization‐time of flight mass spectrometry

Staphylococcus aureus is an important human pathogen whose pathogenesis involves the synthesis of cell wall associated virulence factors and secreted toxins with damaging effects on the host cells. Most of these pathogenic factors are synthesized in a growth‐phase dependent manner as a response to environmental stress like heat, lack of nutrients or other deleterious conditions. Conventional identification of these pathogenic factors is based on Western blot analysis or enzyme‐linked immunosorbent assay (ELISA) and is limited by the commercial availability of antibodies against these toxins. We report here the use of matrix‐assisted laser desorption/ionization‐time of flight (MALDI‐TOF) mass spectrometry for monitoring the pathogenic factors of S. aureus. For the identification of pathogenic factors, a methicillin sensitive strain of S. aureus, ATCC‐29213, was grown at 37°C or 42°C in brain‐heart infusion broth and harvested during the early stationary phase of growth. Secreted proteins were separated by sodium dodecyl sulfate‐polyacrylamide gel electrophoresis, enzymatically digested with trypsin and analyzed by MALDI‐TOF mass spectrometry. When grown at 42°C, alpha‐ and beta‐hemolysins were found to accumulate in S. aureus supernatants while the concentration of protein A was slightly decreased. The identity of some of these toxins was confirmed by Western‐blot analysis. MALDI‐TOF mass spectrometry combined with sodium dodecyl sulfate gel electrophoresis represents a rapid and simple approach to characterize the virulence of S. aureus strains which seems to be particularly valuable for the identification of S. aureus exotoxins for which ELISA is not established.
Arginine Methylation Inhibits the Binding of Proline-rich Ligands to Src Homology 3, but Not WW, Domains*

Src homology 3 (SH3) and WW domains are known to associate with proline-rich motifs within their respective ligands. Here we demonstrate that the proposed adapter protein for Src kinases, Sam68, is a ligand whose proline-rich motifs interact with the SH3 domains of p59 fyn and phospholipase Cγ-1 as well as with the WW domains of FBP30 and FBP21. These proline-rich motifs, in turn, are flanked by RG repeats that represent targets for the type I protein arginine N-methyltransferase. The asymmetrical dimethylation of arginine residues within these RG repeats dramatically reduces the binding of the SH3 domains of p59 fyn and phospholipase Cγ-1, but has no effect on their binding to the WW domain of FBP30. These results suggest that protein arginine methylation can selectively modulate certain protein-protein interactions and that mechanisms exist for the irreversible regulation of SH3 domain-mediated interactions.
Opacity Factor Activity and Epithelial Cell Binding by the Serum Opacity Factor Protein of Streptococcus pyogenes Are Functionally Discrete*

Serum opacity factor (SOF) is a unique multifunctional virulence determinant expressed at the surface of Streptococcus pyogenes and has been shown to elicit protective immunity against GAS infection in a murine challenge model. SOF consists of two distinct domains with different binding capacities: an N-terminal domain that binds apolipoprotein AI and a C-terminal repeat domain that binds fibronectin and fibrinogen. The capacity of SOF to opacify serum by disrupting the structure of high density lipoproteins may preclude its use as a vaccine antigen in humans. This study generated mutant forms of recombinant SOF with reduced (100-fold) or abrogated opacity factor (OF) activity, for use as vaccine antigens. However, alterations introduced into the N-terminal SOF peptide (SOFΔFn) by mutagenesis to abrogate OF activity, abolish the capacity of SOF to protect against lethal systemic S. pyogenes challenge in a murine model. Mutant forms of purified SOFΔFn peptide were also used to assess the contribution of OF activity to the pathogenic processes of cell adhesion and cell invasion. Using latex beads coated with full-length SOF, SOFΔFn peptide, or a peptide encompassing the C-terminal repeats (FnBD), we demonstrate that adhesion to HEp-2 cells is mediated by both SOFΔFn and FnBD. The HEp-2 cell binding displayed by the N-terminal SOFΔFn peptide is independent of OF activity. We demonstrate that while the N terminus of SOF does not directly mediate intracellular uptake by epithelial cells, this domain enhances epithelial cell uptake mediated by full-length SOF, in comparison to the FnBD alone.
Violent behaviour in schizophrenia

Background A number of studies have reported increased violence in patients with schizophrenia. Aims To determine the prevalence of violence among those with schizophrenia in samples from 1949, 1969, 1989 and 2000 in Prague (Czech Republic) and to examine trends in this behaviour. Method Records from 404 patients meeting DSM–IV criteria for schizophrenia were screened for violence (defined as 3 points on the Modified Overt Aggression Scale) from the first observed psychotic symptoms until the time of latest available information. Results Logistic regression revealed a marginally significant increase in violence only inthe 2000 cohort. Overall, violence was associated with schizophrenia in 41.8% of men and 32.7% of women, with no association between substance misuse and violence. Conclusions The violence rate found in our sample is expected to remain stable over time under stable conditions. Substance misuse is not the leading cause of violence among those with schizophrenia.
Are Neutrophil CD64 Expression and Interleukin-6 Early Useful Markers for Diagnosis of Acute Appendicitis?

Abstract Background/Purpose The aim of the study is to evaluate the diagnostic value of interleukin-6 (IL-6) level and neutrophil cluster of differentiation 64 (CD64) expression in diagnosis of acute appendicitis (AA). Methods A prospective controlled trail was performed. Children who were hospitalized with a diagnosis of right lower quadrant pain were our cohort. Serum samples for white blood cell, C-reactive protein (CRP), leukocyte CD64 expression, and IL-6 were obtained from the patients just after their admission. Operation was performed if appendicitis seemed probable, others were observed actively. Patients who had noncomplicated appendicitis were Group 1, patients who had complicated appendicitis were Group 2, and patients who had discharged after observation without operation with a diagnosis of nonspecific abdominal pain and had negative appendectomy without another surgical disease were Group 3. Results In this study, 49 patients were enrolled. CRP and CD64 levels were found higher in Group 2. IL-6 levels were found to be lower in Group 3 than Groups 1 and 2. Conclusions There was a difference between Group 2 and the other groups about CD64 levels. The CRP level and expression of leukocyte CD64 level did not effectively predict the diagnosis of noncomplicated appendicitis, but it predicted well the patients with complicated appendicitis. However, IL-6 levels are statistically significantly different between Group 3 and Groups 1 and 2. According to this result, IL-6 levels predicted well the patient with appendicitis. Applying additional diagnostic methods such as IL-6 levels seems to be helpful in reducing the numbers of false-positive diagnosis of AA.
What is Right and What is Left in Semantic Processing: A Reply to Chiarello

We welcome this opportunity to discuss further our study (Koivisto & Laine, 2000, this issue) and the theoretical conclusions we presented as a model on hemispheric mechanisms in semantic processing. The model put forth claims that when semantic priming occurs within the left visual field/right hemisphere (LVF/RH), it is attributable to postlexical semantic integration, and when priming can be attributed to automatic semantic activation, it only occurs within the right visual field/left hemisphere (RVF/LH). Chiarello (2000, this issue) argues in her commentary that (1) our data supporting this account are weak, (2) our model cannot accommodate the data, and (3) the support for our account from prior literature is not as strong as we claimed.
Left Ventricular Diastolic Dysfunction in Adolescents with Type 1 Diabetes Reflects the Long- but Not Short- Term Metabolic Control

ABSTRACT Objective: The aim of this study was to compare the LV morphology and function in adolescents with DM1 and their healthy peers. Subjects and Methods: In 59 DM1 patients (30 girls, 29 boys,aged 14-17 years), and in control group (15 girls, 15 boys) LV was assessed by M-mode, 2D and Doppler echocardiography. The metabolic control was assessed in the long- (mean HbA1c value for the entire treatment period-HbA1c1), medium- (mean HbA1c for the past two pre-study years-HbA1c2), and short-term (HbA1c on the day of the study- HbA1c3). Results: Diabetic patients presented an increase of IRT (0.062 vs. 0.056 s,p<0.05 in boys, and 0.062 vs. 0.056 s,p<0.05 in girls), A wave (56.1 vs. 53.95 cm/s in boys, and 60.3 vs. 58 cm/s in girls), and deceleration time values (0.16 vs. 0.15 s in boys, and 0.17 vs. 0.15 s in girls), decrease of E wave (97.35 vs. 104.9 cm/s in boys, and 99.67 vs. 101.8 cm/s in girls), as well as the E/A ratio (1.79 vs. 2.0 in boys, and 1.7 vs. 1.78 in girls). The systolic function was normal. In girls there was a correlation between IRT and HbA1c1 and HbA1c2 (r=0.42,r=0.46,p<0.05); between the A wave and HbA1c2 (r=0.46,p<0.05); between LV posterior wall systolic dimension and HbA1c1, HbA1c2 (r=0.45,r=0.41,p<0.05), and in boys between the A wave and HbA1c1 and HbA1c2 (r=0.48,p<0.01;r=0.37, p<0.05). Conclusions: Pubertal patients with DM1 demonstrate discrete impairment of the diastolic function beside normal systolic function of the LV. Further investigations are needed to assess the value of this observation. It seems that LV diastolic dysfunction in girls depends on the degree of insulin resistance and DM1 duration, and similarly as in boys, on the long-term metabolic control.
EEG-Based Brain–Computer Interfaces for Communication and Rehabilitation of People with Motor Impairment: A Novel Approach of the 21st Century

People with severe neurological impairments face many challenges in sensorimotor functions and communication with the environment; therefore they have increased demand for advanced, adaptive and personalized rehabilitation. During the last several decades, numerous studies have developed brain–computer interfaces (BCIs) with the goals ranging from providing means of communication to functional rehabilitation. Here we review the research on non-invasive, electroencephalography (EEG)-based BCI systems for communication and rehabilitation. We focus on the approaches intended to help severely paralyzed and locked-in patients regain communication using three different BCI modalities: slow cortical potentials, sensorimotor rhythms and P300 potentials, as operational mechanisms. We also review BCI systems for restoration of motor function in patients with spinal cord injury and chronic stroke. We discuss the advantages and limitations of these approaches and the challenges that need to be addressed in the future.
A Simple and Global Optimization Algorithm for Engineering Problems: Differential Evolution Algorithm

Differential Evolution (DE) algorithm is a new heuristic approach mainly having three advantages; finding the true global minimum regardless of the initial parameter values, fast convergence, and using few control parameters. DE algorithm is a population based algorithm like genetic algorithms using similar operators; crossover, mutation and selection. In this work, we have compared the performance of DE algorithm to that of some other well known versions of genetic algorithms: PGA, Grefensstette, Eshelman. In simulation studies, De Jong's test functions have been used. From the simulation results, it was observed that the convergence speed of DE is significantly better than genetic algorithms. Therefore, DE algorithm seems to be a promising approach for engineering optimization problems.
Spitzer Observations of V838 Monocerotis: Detection of a Rare Infrared Light Echo

We present Spitzer observations of the unusual variable V838 Monocerotis. Extended emission is detected around the object at 24, 70, and 160 μm. The extended infrared emission is strongly correlated spatially with the HST optical light echo images taken at a similar epoch. We attribute this diffuse nebulosity to an infrared light echo caused by reprocessed thermal emission from dust heated by the outward-propagating radiation from the 2002 eruption. The detection of an IR light echo provides us with an opportunity to estimate the mass in dust of the echo material and hence constrain its origin. We estimate the dust mass of the light echo to be on the order of a solar mass—thereby implying the total gas plus dust mass to be considerably more—too massive for the echo material to be the ejecta from previous outburst/mass-losing events. This suggests therefore that a significant fraction of the matter seen through the light echo is interstellar in origin. Unresolved emission at 24 and 70 μm is also seen at the position of the central star, possibly indicating the presence of hot dust freshly condensed in the outburst ejecta.
Stargardt disease: clinical features, molecular genetics, animal models and therapeutic options

Stargardt disease (STGD1; MIM 248200) is the most prevalent inherited macular dystrophy and is associated with disease-causing sequence variants in the gene ABCA4. Significant advances have been made over the last 10 years in our understanding of both the clinical and molecular features of STGD1, and also the underlying pathophysiology, which has culminated in ongoing and planned human clinical trials of novel therapies. The aims of this review are to describe the detailed phenotypic and genotypic characteristics of the disease, conventional and novel imaging findings, current knowledge of animal models and pathogenesis, and the multiple avenues of intervention being explored.
Asthmalike Symptoms Following Intratracheal Exposure of Guinea Pigs to Sulfur Mustard Aerosol: Therapeutic Efficacy of Exogenous Lung Surfactant Curosurf and Salbutamol

The purpose of the present study was to investigate: (1) the acute effects of sulfur mustard on airway, lung, and surface tension of bronchoalveolar lavage fluid (BALfluid) in guinea pigs following intratracheal (i.t.) exposure to 1LD50 of an aerosolized solution of sulfur mustard in saline, and (2) the therapeutic efficacy of i.t. administration of the natural surfactant Curosurf and the broncholytic Salbutamol. Intratracheally aerosolized sulfur mustard solution induced two clinically relevant symptoms, that is, asthmalike symptoms reflected by an early bronchoconstriction and “late asthmatic responses” (LAR), and ARDS-like symptoms, that is, pulmonary edema and damage to the lung surfactant. The respiratory minute volume (RMV) was enhanced. Histologically, inflammation and severe epithelial injury in the upper airways were observed, whereas the lungs were homogeneously affected. The surface tension of BAL fluid derived at 24 h after sulfur mustard exposure was much higher (20 ± 1 mN/m) than that of unexposed control animals (about 1.0 ± 0.5 mN/m), indicating that the lung surfactant had been altered, and justifying treatment with exogenous surfactant. Intratracheal nebulization of a Salbutamol solution (10 μ g/kg), or i.t. bolus administration of Curosurf (62.5 or 125 mg/kg), tended to reduce mortality, although Salbutamol appeared to be more effective than Curosurf in this respect. Although the present study does not give a definite answer to the question of whether the animal model used would be the most relevant for humans, a number of considerations in favor of i.t. aerosolization of sulfur mustard are discussed. Since it was noticed that sulfur mustard exposure induced damage to the lung surfactant, severe bronchoconstriction, and inflammation of the respiratory tract, the effectiveness of a combined treatment consisting of exogenous surfactant, anti-inflammatory drugs, and broncholytics is recommended to be further investigated.
Management of renal caliceal diverticular stones: A decade of experience

Objective: The objective of this study is to evaluate our methods for management of renal caliceal diverticular stones (CDS). Materials and Methods: We conducted a retrospective study from January 2005 to July 2015 and included patients who were treated for renal CDS. Patients were evaluated for treatment modality, puncture site (in case percutaneous nephrolithotomy [PCNL] attempted), operative time, stone clearance rate, and complications. During PCNL, if the infundibulum was found to connect the diverticulum to the calyx, then a double J stent was placed. No attempt was made to dilate the diverticular neck or to create a neoinfundibulum. Results: Twenty-four patients were treated for CDS during the study period. Two patients underwent shockwave lithotripsy, and 22 were managed by PCNL. Mean stone size was 16.37 mm (range: 6–35 mm) and mean diverticulum size was 20.62 mm (range: 12–37 mm). No fulguration was done in initial 17 patients, while fulguration by Holmium Laser was performed in the last five cases treated with PCNL. Mean operative time was 70.31 min (range: 47–90 min). Mean follow-up was 34 months, diverticulum resolved in 14 patients and reduced in size in 7 patients. Conclusion: Caliceal diverticular calculi can be treated most efficiently by PCNL. Stone-guided puncture and no attempt to dilate or create neoinfundibulum reduces operative time and morbidity while yielding high stone-free rate.
Gene Expression and Antiviral Activity of Alpha/Beta Interferons and Interleukin-29 in Virus-Infected Human Myeloid Dendritic Cells

ABSTRACT Dendritic cells (DCs) respond to microbial infections by undergoing phenotypic maturation and by producing multiple cytokines. In the present study, we analyzed the ability of influenza A and Sendai viruses to induce DC maturation and activate tumor necrosis factor alpha (TNF-α), alpha/beta interferon (IFN-α/β), and IFN-like interleukin-28A/B (IFN-λ2/3) and IL-29 (IFN-λ1) gene expression in human monocyte-derived myeloid DCs (mDC). The ability of influenza A virus to induce mDC maturation or enhance the expression of TNF-α, IFN-α/β, interleukin-28 (IL-28), and IL-29 genes was limited, whereas Sendai virus efficiently induced mDC maturation and enhanced cytokine gene expression. Influenza A virus-induced expression of TNF-α, IFN-α, IFN-β, IL-28, and IL-29 genes was, however, dramatically enhanced when cells were pretreated with IFN-α. IFN-α priming led to increased expression of Toll-like receptor 3 (TLR3), TLR7, TLR8, MyD88, TRIF, and IFN regulatory factor 7 (IRF7) genes and enhanced influenza-induced phosphorylation and DNA binding of IRF3. Influenza A virus also enhanced the binding of NF-κB to the respective NF-κB elements of the promoters of IFN-β and IL-29 genes. In mDC IL-29 induced MxA protein expression and possessed antiviral activity against influenza A virus, although this activity was lower than that of IFN-α or IFN-β. Our results show that in human mDCs viruses can readily induce the expression of IL-28 and IL-29 genes whose gene products are likely to contribute to the host antiviral response.
Chemotaxis of Pseudomonas putida toward chlorinated benzoates

The chlorinated aromatic acids 3-chlorobenzoate and 4-chlorobenzoate are chemoattractants for Pseudomonas putida PRS2000. These compounds are detected by a chromosomally encoded chemotactic response to benzoate which is inducible by beta-ketoadipate, an intermediate of benzoate catabolism. Plasmid pAC27, encoding enzymes for 3-chlorobenzoate degradation, does not appear to carry genes for chemotaxis toward chlorinated compounds.
Three-dimensional analysis of the root morphology of mandibular first molars with distolingual roots.

AIM To determine the prevalence of distolingual roots in mandibular first molar teeth in Taiwanese Han Chinese, and its impact on root morphology. METHODOLOGY The presence of distolingual roots in 375 subjects (521 molars) were assessed from 624 patients attending the dental clinics of medical centres around Taiwan island from August 2004 to April 2007 using computed tomography. The following observations were made: (i) numbers of roots and canals, (ii) mesial and distal root types and (iii) levels of furca in the molars presence or absence of distolingual root. RESULTS The mean age of the subject was 45; 43% were women. Among all the examined molars, 56%, 27% and 18% were two-, three- and four-rooted, respectively. Two per cent, 72% and 26% of molars had two, three and four canals, respectively. All of the four-rooted molars had four canals, but all of the molars with four canals varied in the number of roots. All molars with distolingual roots had two mesial canals. Bilateral consistency in terms of distolingual root, root canal number, root number and root type was observed in subjects with bilateral molars. In molars with distolingual roots, a higher prevalence of two mesial roots and a shorter mesial root trunk were observed than in teeth without distolingual roots. CONCLUSIONS A distolingual root was found in 22% of molars and in 24% of the subjects examined. Most subjects with a distolingual root had them bilaterally. The presence of a distolingual root was associated with variation in the root morphology, including the furcation level, the root type and the number of roots and canals.