Miracle-dz/UniFAR-FTUs huggingface dataset:
Spinocerebellar ataxia 3 and machado‐joseph disease: Clinical, molecular, and neuropathological features

Patients with spinocerebellar ataxia 3 (SCA3) and Machado‐Joseph disease (MJD) carry an expanded CAG repeat in the MJDl gene. One hundred twenty families of different geographic origin with autosomal dominant cerebellar ataxia (ADCA) type I were tested. Thirty‐four families (126 patients) carried an expanded CAG repeat. The expanded and the normal allele did not overlap and the repeat was unstable during transmission, with variation in the size of the CAG length ranging from −8 to +5 and a mean expansion of 0.86 repeats without differences according to the parental sex. There was a combined effect of the number of CAG repeats of the expanded and normal allele on the age at onset, which accounted for 70% of its variability. The length of the CAG repeat influenced the frequency of clinical signs associated with cerebellar ataxia, such as abnormal tendon reflexes or decreased vibration sense, whereas the interindividual variation of supranuclear ophthalmoplegia, sphincter and swallowing difficulties, and amyotrophy was mostly determined by different disease durations. We compared the clinical profile of 91 SCA3/MJD patients with 51 SCAl and 32 SCA2 patients. There were striking differences between the SCA3/MJD and SCA2 but not with SCAl groups of patients. Despite their clinical similarities, distinct neuropathological features were observed in 2 SCA3/MJD and SCAl patients.
La mutualisation des activités logistiques : une application à la filière vitivinicole bourguignonne

RÉSUMÉ Cette contribution analyse les stratégies de mutualisation logistique dans la filière vitivinicole bourguignonne. Elle vise à mieux comprendre les conditions qui facilitent l’émergence et la réussite de ces projets collectifs. L’enquête menée auprès de 36 entreprises de la filière (domaines, négociants, coopératives), de cinq groupements de mutualisation des activités logistiques et de six prestataires de services logistiques, nous a permis de constater l’importance de la proximité organisée et de la confiance établie entre acteurs. Les partenaires doivent en effet partager une même vision des problèmes à résoudre et des objectifs à atteindre. La présence d’un « leader » dans le groupe est également un élément permettant de fédérer les énergies autour d’un projet de mutualisation.
Advantages in the use of carvedilol versus propranolol for the protection of cardiac mitochondrial function.

BACKGROUND Carvedilol is a neurohormonal antagonist of multiple action which is used in clinical practice for the treatment of congestive heart failure, mild to moderate hypertension and myocardial infarction. Previous results from our group have demonstrated that one of the main targets for the protective effect of carvedilol is the cardiac mitochondrial network. In-this work, we compare the effect of carvedilol with propranolol in different models of mitochondrial dysfunction and in the generation of transmembrane electric potential (EP). We further tested if carvedilol was able to inhibit the mitochondrial permeability transition (MPT) induced by doxorubicin and calcium-dependent cytochrome c release, a phenomenon frequently associated with apoptotic cell death. METHODS Cardiac mitochondria were isolated by differential centrifugation. Oxygen consumption and mitochondrial EP were determined using an oxygen electrode and a tetraphenylphosphonium-sensitive electrode, respectively. Changes in mitochondrial volume and the release of cytochrome c were measured with spectrophotometric techniques. RESULTS Propranolol, compared with carvedilol, had only a marginal effect, not only in protection against MPT induction, but also against oxygen consumption linked to the oxidation of external NADH, a process that is considered by several authors as key in the cardiotoxicity of doxorubicin. Regarding EP generation, propranolol had no effect, in contrast to carvedilol, which was confirmed to act as a protonophore. For the first time we also show that carvedilol inhibits the MPT induced by doxorubicin and calcium-dependent cytochrome c release. CONCLUSIONS With this work, we further support the notion that carvedilol is effective in several models of mitochondrial dysfunction, particularly those involving oxidative stress. The results demonstrate that for some pathological conditions, carvedilol and propranolol have different mechanisms of action at the sub-cellular level, as propranolol seems to lack effectiveness in the protection of cardiac mitochondria.
Early Percutaneous Endoscopic Gastrostomy Insertion Maintains Nutritional State in Patients With Aerodigestive Tract Cancer

Patients with upper aerodigestive tract (UAT) cancers often suffer from malnutrition and compromised functional ability. We compared clinical outcome with percutaneous endoscopic gastrostomy (PEG) tube feeding begun at two different time points. The records of 151 patients with UAT carcinomas were reviewed retrospectively. We included patients undergoing radical radiochemotherapy and PEG tube feeding. Subjects were evaluated before PEG insertion and at the end of the treatment. Patients (n = 15, 100%) were divided into two groups according to the presence (group A) or absence (group B) of mucositis. Group A (51.7% of patients) received early PEG: before or within 2 wk of radiotherapy. Group B (48.3%) received delayed PEG: between 2 wk and 3 mo after the start of radiotherapy. Mean weight loss was 1.03 kg in group A vs. 4.0 kg in group B, P = 0.004. Treatment interruptions were significantly (P = 0.01) more common in group B. Early PEG placement at the beginning of radiochemotherapy in patients with UAT tumors maintains the patient's nutritional state and reduces treatment interruptions.
Silver-coated endotracheal tubes and incidence of ventilator-associated pneumonia: the NASCENT randomized trial.

CONTEXT Ventilator-associated pneumonia (VAP) causes substantial morbidity. A silver-coated endotracheal tube has been designed to reduce VAP incidence by preventing bacterial colonization and biofilm formation. OBJECTIVE To determine whether a silver-coated endotracheal tube would reduce the incidence of microbiologically confirmed VAP. DESIGN, SETTING, AND PARTICIPANTS Prospective, randomized, single-blind, controlled study conducted in 54 centers in North America. A total of 9417 adult patients (> or = 18 years) were screened between 2002 and 2006. A total of 2003 patients expected to require mechanical ventilation for 24 hours or longer were randomized. INTERVENTION Patients were assigned to undergo intubation with 1 of 2 high-volume, low-pressure endotracheal tubes, similar except for a silver coating on the experimental tube. MAIN OUTCOME MEASURES Primary outcome was VAP incidence based on quantitative bronchoalveolar lavage fluid culture with 10(4) colony-forming units/mL or greater in patients intubated for 24 hours or longer. Other outcomes were VAP incidence in all intubated patients, time to VAP onset, length of intubation and duration of intensive care unit and hospital stay, mortality, and adverse events. RESULTS Among patients intubated for 24 hours or longer, rates of microbiologically confirmed VAP were 4.8% (37/766 patients; 95% confidence interval [CI], 3.4%-6.6%) in the group receiving the silver-coated tube and 7.5% (56/743; 95% CI, 5.7%-9.7%) (P = .03) in the group receiving the uncoated tube (all intubated patients, 3.8% [37/968; 95% CI, 2.7%-5.2%] and 5.8% [56/964; 95% CI, 4.4%-7.5%] [P = .04]), with a relative risk reduction of 35.9% (95% CI, 3.6%-69.0%; all intubated patients, 34.2% [95% CI, 1.2%-67.9%]). The silver-coated endotracheal tube was associated with delayed occurrence of VAP (P = .005). No statistically significant between-group differences were observed in durations of intubation, intensive care unit stay, and hospital stay; mortality; and frequency and severity of adverse events. CONCLUSION Patients receiving a silver-coated endotracheal tube had a statistically significant reduction in the incidence of VAP and delayed time to VAP occurrence compared with those receiving a similar, uncoated tube. TRIAL REGISTRATION clinicaltrials.gov Identifier: NCT00148642.
Effect of thiazolidinediones on albuminuria and proteinuria in diabetes: a meta-analysis.

BACKGROUND Because of the major clinical and economic burden of diabetic nephropathy, new therapeutic tools to delay its progression are needed. Recent studies suggest that thiazolidinediones have renal benefits. We aimed to evaluate the effect of thiazolidinediones on urinary albumin and protein excretion in patients with diabetes mellitus. STUDY DESIGN Systematic review and meta-analysis by searching MEDLINE/PubMed, EMBASE, and Cochrane CENTRAL databases (1991 to September 2009). SETTING & POPULATION Patients with diabetes mellitus. SELECTION CRITERIA FOR STUDIES Randomized controlled trials. INTERVENTION Thiazolidinediones (rosiglitazone and pioglitazone) compared with placebo or other antidiabetic agents. OUTCOMES Weighted (WMDs) and standardized mean differences (SMDs) for changes in urine albumin or protein excretion between the thiazolidinedione and control groups. RESULTS Of 171 originally identified articles, 15 studies (5 with rosiglitazone and 10 with pioglitazone) involving 2,860 patients were included in the analysis. In participants with baseline normo- or microalbuminuria, the WMD of proportional changes between the thiazolidinedione and control groups in urinary albumin excretion measured using time-specified collections was -64.8% (95% CI, -75.6 to -53.9) and the WMD of changes in albumin-creatinine ratio was -24.8% (95% CI, -39.6 to -10.0). Overall, in participants with normo- and microalbuminuria, thiazolidinedione treatment was associated with a significant decrease in urinary albumin excretion (SMD, -0.6 units of standard deviation [SD]; 95% CI, -0.8 to -0.4). Similarly, thiazolidinediones were associated with a significant decrease in urinary protein excretion in patients with proteinuria (SMD, -1.1 units of SD; 95% CI, -1.8 to -0.4). LIMITATIONS Significant heterogeneity across included studies in several subgroup analyses; patient-level data not available. CONCLUSIONS Treatment with thiazolidinediones significantly decreases urinary albumin and protein excretion in patients with diabetes. This finding calls for clinical trials with hard renal outcomes to elucidate the potential benefits of thiazolidinediones on diabetic nephropathy.
Factors Associated with HIV Discussion and Condom Use with Sexual Partners in an Underserved Community in South Africa

We examined factors associated with discussing HIV and condom use with a sexual partner. Two cross-sectional surveys were conducted in 2004 prior to the implementation of an HIV awareness campaign in a South African community and in 2008 after a three-year education program. Overall, the proportion of individuals who had discussed HIV with a sexual partner increased from 76% in 2004 to 89% in 2008 (p <.001). Among respondents who had sex six months before completing the surveys, condom use significantly increased from 64% in 2004 to 79% in 2008 (p <.05). Respondents who discussed HIV with a sexual partner were more likely to use condoms than respondents who had not discussed HIV with a sexual partner (OR=2.08, 95% CI=1.16, 3.72). These findings indicate the importance of interventions aimed at promoting HIV awareness and discussion of HIV in communities with individuals at risk of acquiring HIV.
Frequency Response Analysis of Current Controllers for Selective Harmonic Compensation in Active Power Filters

This paper compares four current control structures for selective harmonic compensation in active power filters. All controllers under scrutiny perform the harmonic compensation by using arrays of resonant controllers, one for the fundamental and one for each harmonic of interest, in order to achieve zero phase shift and unity gain in the closed-loop transfer function for selected harmonics. The complete current controller is the superposition of all individual harmonic controllers and may be implemented in various reference frames. The analysis is focused on the comparison of harmonic and total closed-loop transfer functions for each controller. Analytical similarities and differences between schemes in terms of frequency response characteristics are emphasized. It is concluded that three of them have identical harmonic behavior despite the fact that their implementation is significantly different. It emerges that the fourth one has superior behavior and robustness and can stably work at higher frequencies than the others. Theoretical findings and analysis are supported by comparative experimental results on a 7-kVA laboratory setup. The highest harmonic frequency that can be stably compensated with each control method has been determined, indicating significant differences in the control performance.
Evaluation of the membrane attack complex of complement for the detection of a recent myocardial infarction in man

The diagnosis of an acute myocardial infarction (MI) can be cumbersome for pathologists. Even with a positive nitroblue tetrazolium (NBT) reaction, haematoxylin and eosin (H&E) evaluation of the myocardial tissue can remain inconclusive. Early signs presumed diagnostic for myocardial infarction, such as hypereosinophilia, waviness, and contraction band necrosis, have to be considered non‐specific and are probably reversible signs of ischaemia. Several studies implicate the complement system, and especially complement factor C9, as part of the membrane attack factor (MAC), in cardiomyocyte damage during MI. In a post‐mortem study on well‐documented cardiological autopsies, we evaluated the use of complement factor C9 immunostaining as a marker for the detection of very recent MI. Forty‐three tissue samples from 40 patients were obtained from the left ventricular free wall only, a region that can be specifically attributed to one corresponding coronary artery. As some patients presented with MIs of various stages in that perfusion area, in total 57 observations were possible. C9 immunostaining specifically detected irreversibly damaged (=infarcted) cardiomyocytes, as is implied by the lytic activity of C9/MAC binding to cell membranes. Most interesting was the group of clinically suspected, NBT‐positive MIs resulting from very recent myocardial ischaemia. In this population, where H&E evaluation by (cardio‐) experienced pathologists was not conclusive, C9 immunostaining clearly pointed towards myocardial infarction in 47% of the cases. In conclusion, C9 immunostaining, routinely practicable in the pathology laboratory, has an additional value in discriminating between reversible ischaemia and infarcted cardiomyocytes in very early MIs. Copyright © 2000 John Wiley & Sons, Ltd.
Heparin‐induced thrombocytopenia thrombosis after cardiac surgery. A case report

We report a rare case of cerebral infarct resulting in brain death due to heparin‐induced thrombocytopenia thrombosis (HITT), manifested in the immediate postoperative period following aortic valve replacement in a 46‐year‐old woman whose only prior exposure to heparin was during catheterization four months prior to surgery. The diagnosis of HITT was suspected after a significant decrease of the platelet count and it was confirmed by a heparin‐induced platelet activation assay showing platelet aggregation in the presence of heparin.
Vector Autoregressive Modelling of the Interaction among Macroeconomic Stability Indicators in Nigeria (1981-2016)

The dynamic behaviour of macroeconomic stability indicators particularly their; evolution, interaction and interdependence, obviously cause shocks among themselves. This study is a multivariate time-series modelling and investigation of the interaction and pattern of causality among exchange rates, inflation rate, interest rates, and implicit price deflator in Nigeria using unrestricted Variance Autoregression (VAR). Quarterly data on the variables spanning the period from 1981 to 2016 were sourced from CBN Statistical bulletin and used for the study. The study used both descriptive and analytical design. The result of the inverse root of AR characteristic polynomial indicated that the VAR model was stable. The Trace Statistics and Max Eigen result showed no co-integrating relationship. The Schwarz Information Criterion showed a lag length of 2. The VAR estimates indicated that the exchange rate was significantly affected by its first lag and second lag, while inflation rates was significantly affected by its first lag. The Wald statistics showed that both lags of each variable were jointly significant in affecting itself. The impulse response showed that all variables were instantaneously affected by own shocks, however, it ruled out the Original Research Article Tuaneh; AJEBA, 9(4): 1-17, 2018; Article no.AJEBA. 46515 2 response in exchange rate to contemporaneous shocks in inflation rate, interest rate and implicit price deflator. The variance decomposition further showed that at least 80% of the impulse response were from own shocks. It was consequently recommended that government should regulates interest rates and exchange rates.
From commercial to cultural: The role of the festival “Firatast una fira boníssima” in the promotion and appreciation of Catalan cuisine

Abstract The article presents a study on the profile of the visiting public to the 23a Firatast Una fira boníssima and perceptions regarding its organization, and the gastronomic and cultural elements presented during the event. The methodology employed is both qualitative and quantitative, using direct observation, survey questionnaire scripts with 119 participants, and in-depth interviews with the festival’s managers. The highlight of this research is to have identified that, although originally created for commercial purposes, the festival has gradually turned into a cultural event and is now perceived by its public as a tool for enhancing and promoting the techniques and traditions of Catalan cuisine.
Do Independent Directors Matter

The generally weak correlation between board independence and firm performance is a major empirical puzzle. One possible explanation is that director independence alone is not enough. To explore this possibility, we examine the full employment histories of independent directors at S&P 1500 companies between 2000 and 2008. We define an independent director with industry experience (IDIEs) as an independent director who has worked in the same 2-digit SIC industry as the company where he/she serves as an independent director. We show that the proportion of IDIEs on a board is positively and significantly correlated with firm performance, but the proportion of independent directors without industry experience (IDNIEs) is not. These effects hold for firms with positive R&D expenditure, but not for firms with zero R&D expenditure. These effects also hold after we control for endogeneity by using Heckman 2-step or 2SLS approach. As an instrumental variable, we use the local pool of potential independent directors with industry expertise. We then explore the channels through which IDIEs influence firm performance. We find that the proportion of IDIEs is negatively and significantly correlated with earnings restatements, but the proportion of IDNIEs is not. These effects hold for firms with less entrenched CEOs, but not for firms with more entrenched CEOs. We find that the proportion of IDIEs is associated with more cash holdings, but the proportion of IDNIEs is not. These effects hold for firms with positive R&D expenditures, but not for firms with zero R&D expenditures. We find firms with IDIEs have significantly higher CEO pay-performance sensitivity, significantly higher CEO turnover-performance sensitivity, and significantly more patents with more citations. We find significant and positive stock market reactions to IDIE appointments, but not to IDNIE appointments. We also find that CEO power is negatively and significantly correlated with the presence of IDIEs.
Recent Advances in Ultra Wideband Radar and Ranging Systems

This invited paper describes advances in short pulse electromagnetics as applied to ultra wideband (UWB) radar and precision ranging. UWB sensors designed for perimeter intrusion detection, obstacle and collision avoidance and industrial safety applications was described. The design of a part 15 compliant, UWB radar development kit is also discussed.
Hip Dislocation in Spastic Cerebral Palsy: Long‐Term Consequences

We evaluated 38 noninstitutionalized patients with spastic quadriplegic cerebral palsy with 51 dislocated hips. Nine hips had been reduced. The mean follow-up was 18 years, with an average age of 26 years. At follow-up, four were ambulatory with aids. Patients who could walk had normal intelligence and a level pelvis. In patients with 18 unreduced unilateral hip dislocations, pelvic obliquity and scoliosis were present in 12. In seven patients with reduced unilateral hip dislocations, similar findings were present in only two patients. Half of the dislocated hips were painful. Based on these findings, we recommend reduction of unilateral dislocations. Bilateral dislocations may benefit from reduction if treatment is undertaken before significant adaptive deformity of the femoral head occurs.
Mechanisms of appearance of the Pasteur effect in Saccharomyces cerevisiae: inactivation of sugar transport systems

Saccharomyces cerevisiae does not show a noticeable Pasteur effect (activation of sugar catabolism by anaerobiosis) when growing with an excess of sugar and nitrogen source, but it does do so after exhaustion of the nitrogen source in the medium (resting state). We have found that this different behavior of growing and resting S. cerevisiae seems due to differences in the contribution of respiration to catabolism under both states. Growing S. cerevisiae respired only 3 to 20% of the catabolized sugar, depending on the sugar present; the remainder was fermented. In contrast, resting S. cerevisiae respired as much as 25 to 100% of the catabolized sugar. These results suggest that a shift to anaerobiosis would have much greater energetic consequences in resting than in growing S. cerevisiae. In resting S. cerevisiae anaerobiosis would strongly decrease the formation of ATP; as a consequence, various regulatory mechanisms would switch on, producing the observed increase of the rate of glycolysis. The greater significance that respiration reached in resting cells was not due to an increase of the respiratory capacity itself, but to a loss of fermentation which turned respiration into the main catabolic pathway. The main mechanism involved in the loss of fermentation observed during nitrogen starvation was a progressive inactivation of the sugar transport systems that reduced the rate of fermentation to less than 10% of the value observed in growing cells. Inactivation of the sugar transports seems a consequence of the turnover of the sugar carriers whose apparent half-lives were 2 to 7 h.
Opsoclonus-Myoclonus Syndrome during Rituximab Treatment for Autoimmune Autonomic Ganglionopathy

Adult-onset opsoclonus-myoclonus syndrome (OMS) is an autoimmune disorder with paraneoplastic, parainfectious, or idiopathic etiologies.1 Nonparaneoplastic cases are generally immunotherapy responsive.1 Although previously described accompanying encephalopathy in NMDA receptor (NMDA-R) encephalitis,2 OMS occurring as an isolated CNS finding in an NMDA-R antibody–positive patient appears to be unusual, particularly among patients with preexisting α3AChR antibody–associated dysautonomia. We describe a patient in whom an autoimmune CNS disorder isolated to OMS arose in the context of rituximab treatment administered for refractory autoimmune dysautonomia.