Effect of different compatibilizers on injection-molded green composite pieces based on polylactide filled with almond shell flour

[EN] Green composites made of polylactide (PLA) filled with almond shell flour (ASF) at a constant weight content of 25¿wt.-% were manufactured by injection molding. In order to increase the interfacial adhesion between the biopolymer and the lignocellulosic fillers, three different compatibilizers were tested, namely multi-functional epoxy-based styrene-acrylic oligomer (ESAO), aromatic carbodiimide (AC), and maleinized linseed oil (MLO). The effect of each compatibilizer on the thermal, mechanical, and thermomechanical properties and water uptake of the injection-molded PLA/ASF pieces was analyzed. The obtained results indicated that all the here-studied compatibilizers had a positive influence on both the thermal stability and the mechanical and thermomechanical performance of the green composite pieces but low impact on their water uptake profile. In addition, the morphological analysis performed at the fracture surfaces of the green composite pieces revealed that the filler¿matrix gap was substantially reduced. Among the tested compatibilizers, ESAO and MLO yielded the highest performance in terms of mechanical strength and ductility, respectively. In the case of MLO, it also offers the advantage of being a plant-derived additive so that its application in green composites positively contributes to the development of sustainable polymer technologies.This research was supported by the Spanish Ministry of Economy and Competitiveness (MINECO) program number MAT2014-59242-C2-1-R and AGL2015-63855-C2-1-R and Generalitat Valenciana (GV) program number GV/2014/008. A. Carbonell-Verdu wants to thank Universitat Politecnica de Valencia (UPV) for his FPI grant. D. Garcia-Garcia wants to thank the Spanish Ministry of Education, Culture and Sports (MECD) for his FPU grant (FPU13/06011). L. Quiles-Carrillo also wants to thank GV for his FPI grant (ACT/2016/182) and the MECD for his FPU grant (FPU15/03812).Quiles-Carrillo, L.; Montanes, N.; Garcia-Garcia, D.; Carbonell-Verdu, A.; Balart, R.; Torres-Giner, S. (2018). Effect of different compatibilizers on injection-molded green composite pieces based on polylactide filled with almond shell flour. Composites Part B Engineering. 147:76-85. https://doi.org/10.1016/j.compositesb.2018.04.017S768514

X chromosome inactivation does not necessarily determine the severity of the phenotype in Rett syndrome patients

Rett syndrome (RTT) is a severe neurological disorder usually caused by mutations in the MECP2 gene. Since the MECP2 gene is located on the X chromosome, X chromosome inactivation (XCI) could play a role in the wide range of phenotypic variation of RTT patients; however, classical methylation-based protocols to evaluate XCI could not determine whether the preferentially inactivated X chromosome carried the mutant or the wild-type allele. Therefore, we developed an allele-specific methylation-based assay to evaluate methylation at the loci of several recurrent MECP2 mutations. We analyzed the XCI patterns in the blood of 174 RTT patients, but we did not find a clear correlation between XCI and the clinical presentation. We also compared XCI in blood and brain cortex samples of two patients and found differences between XCI patterns in these tissues. However, RTT mainly being a neurological disease complicates the establishment of a correlation between the XCI in blood and the clinical presentation of the patients. Furthermore, we analyzed MECP2 transcript levels and found differences from the expected levels according to XCI. Many factors other than XCI could affect the RTT phenotype, which in combination could influence the clinical presentation of RTT patients to a greater extent than slight variations in the XCI pattern

Attenuated variants of Lesch-Nyhan disease

Lesch–Nyhan disease is a neurogenetic disorder caused by deficiency of the enzyme hypoxanthine–guanine phosphoribosyltransferase. The classic form of the disease is described by a characteristic syndrome that includes overproduction of uric acid, severe generalized dystonia, cognitive disability and self-injurious behaviour. In addition to the classic disease, variant forms of the disease occur wherein some clinical features are absent or unusually mild. The current studies provide the results of a prospective and multi-centre international study focusing on neurological manifestations of the largest cohort of Lesch–Nyhan disease variants evaluated to date, with 46 patients from 3 to 65 years of age coming from 34 families. All had evidence for overproduction of uric acid. Motor abnormalities were evident in 42 (91%), ranging from subtle clumsiness to severely disabling generalized dystonia. Cognitive function was affected in 31 (67%) but it was never severe. Though none exhibited self-injurious behaviours, many exhibited behaviours that were maladaptive. Only three patients had no evidence of neurological dysfunction. Our results were compared with a comprehensive review of 78 prior reports describing a total of 127 Lesch–Nyhan disease variants. Together these results define the spectrum of clinical features associated with hypoxanthine–guanine phosphoribosyltransferase deficiency. At one end of the spectrum are patients with classic Lesch–Nyhan disease and the full clinical phenotype. At the other end of the spectrum are patients with overproduction of uric acid but no apparent neurological or behavioural deficits. Inbetween are patients with varying degrees of motor, cognitive, or behavioural abnormalities. Recognition of this spectrum is valuable for understanding the pathogenesis and diagnosis of all forms of hypoxanthine–guanine phosphoribosyltransferase deficiency

A922 Sequential measurement of 1 hour creatinine clearance (1-CRCL) in critically ill patients at risk of acute kidney injury (AKI)

Meeting abstrac

Stable or improved neurological manifestations during miglustat therapy in patients from the international disease registry for Niemann-Pick disease type C: an observational cohort study

Background: Niemann-Pick disease type C (NP-C) is a rare neurovisceral disease characterised by progressive neurological degeneration, where the rate of neurological disease progression varies depending on age at neurological onset. We report longitudinal data on functional disease progression and safety observations in patients in the international NPC Registry who received continuous treatment with miglustat. Methods: The NPC Registry is a prospective observational cohort of NP-C patients. Enrolled patients who received ≥1 year of continuous miglustat therapy (for ≥90 % of the observation period, with no single treatment interruption >28 days) were included in this analysis. Disability was measured using a scale rating the four domains, ambulation, manipulation, language and swallowing from 0 (normal) to 1 (worst). Neurological disease progression was analysed in all patients based on: 1) annual progression rates between enrolment and last follow up, and; 2) categorical analysis with patients categorised as 'improved/stable' if ≥3/4 domain scores were lower/unchanged, and as 'progressed' if <3 scores were lower/unchanged between enrolment and last follow-up visit. Results: In total, 283 patients were enrolled from 28 centers in 13 European countries, Canada and Australia between September 2009 and October 2013; 92 patients received continuous miglustat therapy. The mean (SD) miglustat exposure during the observation period (enrolment to last follow-up) was 2.0 (0.7) years. Among 84 evaluable patients, 9 (11 %) had early-infantile (<2 years), 27 (32 %) had late-infantile (2 to <6 years), 30 (36 %) had juvenile (6 to <15 years) and 18 (21 %) had adolescent/adult (≥15 years) onset of neurological manifestations. The mean (95%CI) composite disability score among all patients was 0.37 (0.32,0.42) at enrolment and 0.44 (0.38,0.50) at last follow-up visit, and the mean annual progression rate was 0.038 (0.018,0.059). Progression of composite disability scores appeared highest among patients with neurological onset during infancy or childhood and lowest in those with adolescent/adult-onset. Overall, 59/86 evaluable patients (69 %) were categorized as improved/stable and the proportion of improved/stable patients increased with age at neurological onset. Safety findings were consistent with previous data. Conclusions: Disability status was improved/stable in the majority of patients who received continuous miglustat therapy for an average period of 2 years

Relationship between functional status prior to onset of critical illness and mortality: a prospective multicentre cohort study.

This prospective study aimed to assess the association between prior functional status and hospital mortality for patients admitted to four intensive care units in Spain between 2006 and 2012. Prior functional status was classified into three groups, using a modification of the Glasgow Outcome Scale (GOS), including group 1 with no limitations on activities of daily living; group 2 with some limitations but self-sufficient; and group 3 who were dependent on others for their activities of daily living. Of the 1,757 patients considered (mean Simplified Acute Physiology Score [SAPS] predicted mortality 14.8% and hospital mortality 13.7%), group 1 had the lowest observed hospital mortality (8.3%) compared to the SAPS 3 predicted mortality (11.6%). The observed mortality for group 2 (20.6%) and group 3 (27.4%) were both higher than predicted (19.2% and 21.2% respectively; odds ratio [OR] 1.97, 95% confidence interval [CI] 1.38-2.82 for group 2 and OR 2.90, 95% CI 1.78-4.72 for group 3 compared to group 1). Combining prior functional status and Sequential Organ Failure Assessment (SOFA) score with SAPS 3 further improved the ability of the SAPS 3 scores in predicting hospital mortality (area under the receiver operating characteristic curve 0.85 [95% CI 0.82-0.88] versus 0.84 [95% CI 0.81-0.87] respectively). In summary, patients with limited functional status prior to ICU admission had a higher risk of observed hospital mortality than predicted. Assessing prior functional status using a relatively simple questionnaire, such as a modified GOS, has the potential to improve the accuracy of existing prognostic models

Systematic study of constitutive cyclooxygenase-2 expression: Role of NF-κB and NFAT transcriptional pathways.

Cyclooxygenase-2 (COX-2) is an inducible enzyme that drives inflammation and is the therapeutic target for widely used nonsteroidal antiinflammatory drugs (NSAIDs). However, COX-2 is also constitutively expressed, in the absence of overt inflammation, with a specific tissue distribution that includes the kidney, gastrointestinal tract, brain, and thymus. Constitutive COX-2 expression is therapeutically important because NSAIDs cause cardiovascular and renal side effects in otherwise healthy individuals. These side effects are now of major concern globally. However, the pathways driving constitutive COX-2 expression remain poorly understood. Here we show that in the kidney and other sites, constitutive COX-2 expression is a sterile response, independent of commensal microorganisms and not associated with activity of the inflammatory transcription factor NF-κB. Instead, COX-2 expression in the kidney but not other regions colocalized with nuclear factor of activated T cells (NFAT) transcription factor activity and was sensitive to inhibition of calcineurin-dependent NFAT activation. However, calcineurin/NFAT regulation did not contribute to constitutive expression elsewhere or to inflammatory COX-2 induction at any site. These data address the mechanisms driving constitutive COX-2 and suggest that by targeting transcription it may be possible to develop antiinflammatory therapies that spare the constitutive expression necessary for normal homeostatic functions, including those important to the cardiovascular-renal system

Road slope revegetation in semiarid mediterranean environments. Part I: Seed dispersal and spontaneous colonization

9 páginas, 2 figuras, 2 tablas.The importance of neighboring vegetation as a seed reservoir for spontaneous colonization of adjacent road slopes was analyzed in a semiarid region of east Spain. Two independent methodological approaches were used to examine the relative contribution of seed from neighboring vegetation and the efficiency of different seed dispersal strategies in plant colonization. We first used a randomization test to compare floristic similarity between road slopes, neighboring flora, and local flora (the regional species pool found in the same climate and soil conditions as the road slopes). Second, we compared seed dispersal mechanisms of road slope vegetation with those of the surrounding area using frequency analysis. Species composition of road slopes was more similar to that of the flora of adjacent surrounding areas than expected by chance. Anemochorous (wind-dispersed) plants were over-represented in road slopes 8 years after road slopes were built. We concluded that seed dispersal from neighboring vegetation is an important factor in the vegetative colonization of road slopes. However, this initial species pool was also strongly shaped by the harsh environmental conditions of roadcuts and southern aspect. These results have important implications in road slope restoration because they suggest that naturally vegetated areas should be maintained adjacent to road slopes to enhance seed immigration from species adapted to local site conditions, which will accelerate the successional process. The application of this single reclamation strategy and mixed strategies that combine the use of natural colonization and soil amendment for road slope restoration in Mediterranean environmental conditions is discussed.This research was supported by the Ministerio de Ciencia y Tecnología (projects FEDER and TALMED). We are grateful to Hans Jacquemyn (KUL, University of Leuven, Belgium) for providing the simulation procedure and to Olga Cueto (CEA-Commissariat à l’Énergie Atomique, Grenoble, France) for modifying the procedure to match our experimental design. We would also like to acknowledge the field assistance of José Antonio Bellido, Daniel Montesinos, Manuel Monsalve, and David Correa (CIDE–Centro de Investigaciones sobre Desertificatión, Valencia, Spain). We are also grateful to Cynthia Jones and Jenny Brown for language editing this article.Peer reviewe