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This review article aims to present an overview regarding the volatile compounds in different scented species of Pelargonium and their biological activities, immunomodulatory activity, cytotoxic activity, high larvicidal activity and ethnopharmacological uses. Although the Pelargonium genus includes many species, we focused only on the scented ones, with the potential to be used in different domains. Pelargonium essential oil showed great properties as antioxidant activity, antibacterial activity (against K. pneumonie, S. aureus or E. coli strains) and antifungal activity (against many fungi including Candida sp.), the responsible compounds for these properties being tannins, flavones, flavonols, flavonoids, phenolic acids and coumarins. Due to the existence of bioactive constituents in the chemical composition of fresh leaves, roots, or flowers of Pelargonium sp. (such as monoterpenoid compounds–citronellol, geraniol, linalool, and flavonoids–myricetin, quercetin and kaempferol), this species is still valuable, the bio-compounds representing the base of innovative substitutes in food processing industry, nutraceuticals, or preventive human or veterinary medicine (substitute of antibiotics). Highlighting the volatile chemical composition and properties of this scented plant aims to rediscover it and to emphasize the vast spectrum of health-promoting constituents for a sustainable approach. Future research directions should point to the application of plant biotechnology with a significant role in conservation strategy and to stimulate commercial interest.

Background: This study aimed at identifying errors encountered in orthopantomography (OPG) in post-traumatic patients caused by limitations in performing a correct technique. Methods: A retrospective observational study was performed. Diagnosis, exposure/processing mistakes, positioning-related errors, and bimaxillary immobilization were evaluated. Results: Thirty panoramic radiographs with mandible fractures were examined. Twelve error types were encountered: errors in exposure or processing, air radiolucency in the palatoglossal space, errors in the alignment of the Frankfort horizontal plane: head in flexion, with a joyful expression or head extended, with a somber appearance, errors towards the mid-sagittal plane (lateral head inclination, deviation, or rotation), errors caused by the non-use of the bite-block or inappropriate position on the device, errors caused by positioning outside the focal plane, artifacts/shadow images produced by post-operative metal plates, and bimaxillary immobilization errors. The number of errors per radiograph ranged from two to a maximum of five. The most dominant ones were inappropriate alignment in the focal plane and lateral rotation of the head in over 70% of cases. Lateral deviation and palatoglossal air were present in more than 50% of images. Conclusions: In trauma cases, technical difficulties in obtaining a proper OPG image are common and often insurmountable, limiting the diagnosis.

Bruton's tyrosine kinase (Btk) has a key role in the signaling pathways of receptors essential for the B lymphocyte response. Given its implication in B cell-related immunodeficiencies, leukemias/lymphomas and autoimmunity, Btk is studied intensely and is a target for therapy. Here, using primary B cells from distinct mouse models and the pharmacological inhibitors ibrutinib and acalabrutinib, we report distinct roles for Btk in antigen-triggered immune synapse (IS) formation. Btk recruitment to the plasma membrane regulates the B cell ability to trigger IS formation as well as its appropriate molecular assembly; Btk shuttling/scaffold activities seem more relevant than the kinase function on that. Btk-kinase activity controls antigen accumulation at the IS through the PLCγ2/Ca2+ axis. Impaired Btk membrane-recruitment or kinase function likewise alters antigen-triggered microtubule-organizing center (MTOC) polarization to the IS, B cell activation and proliferation. Data also show that, for B cell function, IS architecture is as important as the quantity of antigen that accumulates at the synapse.

Objectives(a) Assessing mental disorders, psychological distress, psychological wellbeing in patients with systemic sclerosis (SSc); (b) identifying psychological features independently contributing to the status of having the diagnosis of SSc.MethodsTwo hundred SSc outpatients were compared with 100 healthy subjects. Mental disorders were assessed via the Mini International Neuropsychiatric Interview (MINI). Self-reported rating scales were administered: Health Assessment Questionnaire Disability Index (HAQ-DI), Symptom Checklist-90-Revised (SCL-90-R), Psychological Well Being scales (PWB). General linear models allowed to verify which psychological feature would individually make unique contributions to overall status of having the diagnosis of SSc.ResultsMajor depressive episode/disorder, panic disorder were more prevalent among patients with SSc (p

The purpose of this study was to evaluate the effect of supplementation with 200 ppm vitamin E in a diet with different nutrient contents on the performance and health status of the IPD-D3 candidate chicken strain. This research used 160 IPB-D3 chickens that were reared for 5 to 12 weeks. This experiment used a completely randomized factorial design with 4 treatments and 4 replications. P0 = control diet, P1 = control diet minus 3% ME and 3% PK, P2 = P0 + vitamin E 200 ppm, P3 = P1+ vitamin E 200 ppm. The data were analysed using ANOVA with an interaction’s significant difference test. The results showed that there was an interaction effect between supplementation with 200 ppm vitamin E and different nutrient contents on feed consumption (P

Abstract When someone violates a social norm, others may think that some sanction would be appropriate. We examine how the experience of emotions like anger and disgust relate to the judged appropriateness of sanctions, in a pre-registered analysis of data from a large-scale study in 56 societies. Across the world, we find that individuals who experience anger and disgust over a norm violation are more likely to endorse confrontation, ostracism and, to a smaller extent, gossip. Moreover, we find that the experience of anger is consistently the strongest predictor of judgments of confrontation, compared to other emotions. Although the link between state-based emotions and judgments may seem universal, its strength varies across countries. Aligned with theoretical predictions, this link is stronger in societies, and among individuals, that place higher value on individual autonomy. Thus, autonomy values may increase the role that emotions play in guiding judgments of social sanctions.

Abstract The emergence of COVID-19 dramatically changed social behavior across societies and contexts. Here we study whether social norms also changed. Specifically, we study this question for cultural tightness (the degree to which societies generally have strong norms), specific social norms (e.g. stealing, hand washing), and norms about enforcement, using survey data from 30,431 respondents in 43 countries recorded before and in the early stages following the emergence of COVID-19. Using variation in disease intensity, we shed light on the mechanisms predicting changes in social norm measures. We find evidence that, after the emergence of the COVID-19 pandemic, hand washing norms increased while tightness and punishing frequency slightly decreased but observe no evidence for a robust change in most other norms. Thus, at least in the short term, our findings suggest that cultures are largely stable to pandemic threats except in those norms, hand washing in this case, that are perceived to be directly relevant to dealing with the collective threat.

The present work provides an updated and corrected list of hosts for the five European species of pinnotherid crabs, taking into consideration all recent published works as well as new data obtained in the present study. In relation to the number of hosts, there are two groups of species, one composed by Pinnotheres pisum and Afropinnotheres monodi with the highest number of hosts, and a second group, with a reduced number of hosts and more specific taxa, consisting of three other species: Nepinnotheres pinnotheres, Pinnotheres bicristatus, and P. pectunculi. When studying the relationships between host preferences, host distributions, and pea crab distributions, we could not find a clear direct relationship between these parameters. Temperature is also probably an important influence and relevant factor responsible for the distribution of pinnotherid crabs, and this may be independent from the potential distribution due to the distribution of hosts.

Thin films of lithium spinel ferrite, LiFe5O8, have attracted much scientific attention because of their potential for efficient excitation, the manipulation and propagation of spin currents due to their insulating character, high-saturation magnetization, and Curie temperature, as well as their ultra-low damping value. In addition, LiFe5O8 is currently one of the most interesting materials in terms of developing spintronic devices based on the ionic control of magnetism, for which it is crucial to control the lithium’s atomic content. In this work, we demonstrate that dual ion beam sputtering is a suitable technique to tailor the lithium content of thin films of lithium ferrite (LFO) by using the different energies of the assisting ion beam formed by Ar+ and O2+ ions during the growth process. Without assistance, a disordered rock-salt LFO phase (i.e., LiFeO2) can be identified as the principal phase. Under beam assistance, highly out-of-plane-oriented (111) thin LFO films have been obtained on (0001) Al2O3 substrates with a disordered spinel structure as the main phase and with lithium concentrations higher and lower than the stoichiometric spinel phase, i.e., LiFe5O8. After post-annealing of the films at 1025 K, a highly ordered ferromagnetic spinel LFO phase was found when the lithium concentration was higher than the stoichiometric value. With lower lithium contents, the antiferromagnetic hematite (α-Fe2O3) phase emerged and coexisted in films with the ferromagnetic LixFe6-xO8. These results open up the possibility of controlling the properties of thin lithium ferrite-based films to enable their use in advanced spintronic devices.

ABSTRACTIntegrative and conjugative elements (ICEs) are widespread autonomous mobile DNA elements, containing the genes necessary for their excision, conjugative transfer, and insertion into a new host cell. ICEs can carry additional genes that are non-essential for their transfer but can confer adaptive phenotypes to the host. Our aim here was to better characterize the presence, distribution, and variation of ICEs related to the well-described ICEclc among Pseudomonas aeruginosa clinical isolates within a geographically restrained environment to understand the factors contributing to their evolution. We examined a total of 181 P. aeruginosa genome sequences obtained from patient or hospital environment isolates, most of which were obtained from a single hospital during 20 years of sampling. More than 90% of the isolates carried one or more ICEclc-like elements, with different degrees of conservation to the known ICEclc lifestyle and transfer genes. ICE clones closely matched their host clonal phylogeny, but not exclusively, indicating that both clonal evolution and ICE horizontal transfer are occurring in the hospital environment. ICEs from this singular hospital environment were mainly associated to three clone types found worldwide, suggesting an enrichment of local clones. Variable gene regions among the clinical P. aeruginosa ICEclc-type elements were notably enriched for heavy metal resistance genes, toxin-anti-toxin systems, potential efflux systems and multidrug resistance proteins, a metalloprotease and for a variety of regulatory systems, but not for specific recognizable antibiotic-resistance cassettes. Clonal persistence suggests adaptive benefits of these functional categories, and micro-patterns of gene gain and loss indicate ongoing ICE evolution within the P. aeruginosa hosts.IMPORTANCEMicrobial populations swiftly adapt to changing environments through horizontal gene transfer. While the mechanisms of gene transfer are well known, the impact of environmental conditions on the selection of transferred gene functions remains less clear. We investigated ICEs, specifically the ICEclc-type, in Pseudomonas aeruginosa clinical isolates. Our findings revealed co-evolution between ICEs and their hosts, with ICE transfers occurring within strains. Gene functions carried by ICEs are positively selected, including potential virulence factors and heavy metal resistance. Comparison to publicly available P. aeruginosa genomes unveiled widespread antibiotic-resistance determinants within ICEclc clades. Thus, the ubiquitous ICEclc family significantly contributes to P. aeruginosa’s adaptation and fitness in diverse environments.

Chronic school absenteeism is a common problem in childhood and adolescence, and it is frequently observed in patients with somatic symptom and related disorders (SSRDs). This study aimed to determine whether and to what extent the presence of school absenteeism may be a risk factor for the diagnosis of SSRDs in hospitalised patients. This matched cohort study included children and adolescents aged between 8 and 17 years, admitted to the paediatric ward of the IRCCS Burlo Garofolo in Trieste from 2021 to 2023, who were divided into two groups, the first including children with at least 15 days of absence from school for medical reasons and the second including children with regular school attendance, matched to the former group by age and sex. We consecutively enrolled 70 patients, 35 in the absentee group and 35 in the control group. In the absentee group, 30/35 (85.7%) patients were diagnosed with an SSRD, while in the control group, 1/35 (2.9%) was diagnosed with an SSRD. The absentee group had a 30-fold higher risk of being diagnosed with SSRDs than the control group (RR = 30 [95% CI = 4.3–208]; p < 0.001). This study shows that in hospitalised children, a history of school absenteeism of more than two weeks is an important risk factor for the diagnosis of SSRDs.

The expression “twice-exceptionality” has been used to describe conditions in which giftedness and specific disorders coexist. Our study offers a retrospective analysis of clinical reports of gifted children evaluated for suspected specific learning disorders (SLD) or attention-deficit/hyperactivity disorder (ADHD). The initial sample included 456 school-aged children referred to our clinic for suspected SLD and/or ADHD over a two-year interval. The inclusion criteria were: a General Ability Index score above 120 in the cognitive assessment; age 6–18 years; and not satisfying diagnostic criteria for autism spectrum disorder. Forty children were selected for the study. We grouped patients according to the final diagnosis: neurodevelopmental disorder (SLD and/or ADHD) (n = 15), psychopathological disorder (n = 8), mixed neurodevelopmental and psychopathological (n = 13), no emerging disorder (n = 4). The study included 36 (90%) males. Mean age was 9.3 years (SD 1.62). Mean Full-Scale Intelligence Quotient was 121.7 (SD 7.77), mean General Ability Index was 130.2 (SD 6.79). Furthermore, the cognitive assessment of the different groups highlighted a non-homogeneous profile in all groups, with lower scores on working memory and processing speed indexes. Our results support the hypothesis that difficulties in gifted children’s adaptation to scholastic and social settings could be misinterpreted as a manifestation of a clear disease.

Neurodevelopmental disorders, such as autism spectrum disorder (ASD) and attention deficit hyperactivity disorder (ADHD), do often present in comorbidity among them and with other medical conditions, including sleep and gastrointestinal (GI) disorders and somatic complaints. An anonymous online survey based on standardized questionnaires (SDSC, KL-ASD, APSI, ROME V CRITERIA, CPRS, CBCL) was completed by the parents of 46 preschoolers diagnosed with ASD. A high prevalence (47%) of ADHD symptoms in this population was found, surpassing previous estimates. Sleep disturbances, especially difficulties in initiating and maintaining sleep and sleep–wake transition, are more pronounced in ASD patients with comorbid ADHD. Additionally, in patients over 4 years old, there is a high prevalence of functional GI symptoms (Rome V criteria: 38%). Parental stress is significantly associated with ADHD symptoms, sleep disorders, and GI symptoms. Comprehensive assessments considering both core symptoms of neurodevelopmental disorders and associated comorbidities are crucial for more effective treatment strategies and improved wellbeing in affected individuals and their families.

Building community resilience requires centering equity in resilience planning processes. Tools and resources for strengthening community resilience need to address equity in both their content and the process for using them. This is especially so for communities living in proximity to contaminated lands that face compounding hazards (i.e., environmental, disaster, and climate-related); legacies of institutional or structural disenfranchisement; challenges with inclusion of minority populations in planning; and constraints on doing data-intensive planning and management in under-resourced and underserved jurisdictions. A research team from the U.S. Environmental Protection Agency is developing a new resource, the Equitable Resilience Builder, which will serve communities with intersecting social and environmental vulnerabilities, in pursuit of creating resilience plans and developing the intra-community connections to implement them. This article details how the team used human-centered design to develop the Equitable Resilience Builder. Our objective in doing so is to share the evolution of equity in the project and demonstrate key inflection points in the discovery, synthesis, and ideation phases of human-centered design. The team was able to expand their understanding of what it means to undertake resilience planning in an equitable way during engagements with state, local, tribal agencies, foundations, non-governmental organizations, and academia and through participatory workshops. It developed design principles for how the tool might use storytelling and other techniques to address emotions and trauma, ensure local voices are heard, and encourage relationship building. This article offers lessons learned for others seeking to address resilience and equity in climate risk management, particularly when working with communities in proximity to contaminated lands.

Abstract Background Median raphe cyst is usually benign and asymptomatic male genitalia lesions. Although uncommon, infection may be a complication. Case presentation We report the case of a 4-year-old child presented to the emergency department for a serpiginous and redness lesion extended from the basis of the penis until the perineum. An infected median raphe cyst was suspected, and the patient underwent surgical treatment and antibiotic therapy with complete resolution of symptoms. Liquid culture resulted positive for Serratia Marcescens. Conclusion Infection is a rare complication, especially in childhood. To prevent relapses and clinical symptoms, the majority of authors recommend surgical excision followed by primary closure. In case of infections caused by Serratia Marcescens, chronic granulomatous disease should always be rule out.

Abstract Background Neonatal infectious spondylodiscitis is a rare bony infection with atypical clinical presentation and non-specific systemic symptoms. Diagnosis and treatment are often delayed resulting in vertebral destruction and severe complications. We retrospectively reviewed the case of an infant with infectious spondylodiscitis resulting in T12 body destruction and marked angular kyphosis. Case-report A 4-week-old infant developed an infectious spondylodiscitis resulting in destruction of the T12 vertebral body and involvement of disc between T12 and L1. At 6 months of age, X-ray showed a marked thoracolumbar angular kyphosis above 50 Cobb degrees. Therefore, the patient underwent single time surgery with double anterior and posterior approach. At 9 years follow up, clinical and radiological findings show a stable correction with good aesthetic appearance. Conclusion Neonatal spondylodiscitis could lead to marked kyphosis similar to the congenital one. Since treatment with casts and tutors is often inefficacious, prompt surgery should be considered. The double anterior and posterior approach is the best option in this condition.

Periosteum is a highly vascularized membrane lining the surface of bones. It plays essential roles in bone repair following injury and reconstruction following invasive surgeries. To broaden the use of periosteum, including for augmenting in vitro bone engineering and/or in vivo bone repair, we have developed an ex vivo perfusion bioreactor system to maintain the cellular viability and metabolism of surgically resected periosteal flaps. Each specimen was placed in a 3D printed bioreactor connected to a peristaltic pump designed for the optimal flow rates of tissue perfusate. Nutrients and oxygen were perfused via the periosteal arteries to mimic physiological conditions. Biochemical assays and histological staining indicate component cell viability after perfusion for almost 4 weeks. Our work provides the proof-of-concept of ex vivo periosteum perfusion for long-term tissue preservation, paving the way for innovative bone engineering approaches that use autotransplanted periosteum to enhance in vivo bone repair.

Little is known about people’s preferred responses to norm violations across countries. Here, in a study of 57 countries, the authors highlight cultural similarities and differences in people’s perception of the appropriateness of norm violations.

BackgroundAlthough anxiety is highly represented in the medically ill and its occurrence has relevant clinical implications, it often remains undetected and not properly treated. This systematic review aimed to report on anxiety, either symptom or disorder, in patients who suffer from a medical illness.MethodsEnglish-language papers reporting on anxiety in medically ill adults were evaluated. PubMed, PsycINFO, Web of Science, and Cochrane databases were systematically searched from inception to June 2021. Search term was “anxiety” combined using the Boolean “AND” operator with “medically ill/chronic illness/illness/disorder/disease.” Risk of bias was assessed via the Joanna Briggs Institute (JBI) Critical Appraisal Tools—Checklist for Prevalence Studies. The PRISMA guidelines were followed.ResultsOf 100,848 citations reviewed, 329 studies met inclusion criteria. Moderate or severe anxious symptoms were common among patients with cardiovascular, respiratory, central nervous system, gastrointestinal, genitourinary, endocrine, musculoskeletal system or connective tissue, dermatological diseases, cancer, AIDS and COVID-19 infections. The most common anxiety disorder was generalized anxiety disorder, observed among patients with cardiovascular, respiratory, central nervous system, dermatologic diseases, cancer, primary aldosteronism, amenorrhea, and COVID-19 infection. Panic disorder was described for cardiovascular, respiratory, dermatology diseases. Social anxiety was found for cardiovascular, respiratory, rheumatoid diseases. Specific phobias were relatively common in irritable bowel syndrome, gastroesophageal reflux, end-stage renal disease.ConclusionAnxiety is a major challenge in medical settings. Recognition and proper assessment of anxiety in patients who suffer from a medical illness is necessary for an appropriate management. Future reviews are warranted in order also to clarify the causal and temporal relationship between anxiety and organic illness.

AimEmergency cases are uncommon events in the pediatric emergency setting. This study aimed to evaluate the effect of the Coronavirus disease 2019 (COVID-19) pandemic by describing the number and type of pediatric emergency cases that arrived at the pediatric emergency department (PED) of a tertiary-level children’s hospital in Italy.MethodsWe performed a retrospective study, collecting the main features of pediatric emergency patients who arrived during the first year of the COVID-19 pandemic (March 2020–February 2021) compared to the pre-pandemic period (March 2016–February 2020).ResultsDuring the study period, 112,168 patients were visited at the PED, and 237 (0.21%) were emergency cases, median age of 4 years (IQR: 1–12). In the first year of the pandemic, 42 children were coded as emergency cases compared to 195 (49/year) during the pre-pandemic period. The proportion of emergency cases was stable (0.27% during the COVID-19 period versus 0.20% during the pre-COVID-19 period, p = 0.19). No differences were found regarding the age, gender, hour of arrival, and outcome of patients. We found a significant decrease in the proportion of emergency cases related to respiratory diseases (9/42, 21.4% during the COVID-19 period versus 83/195 during the pre-COVID-19 period (42.6%), p = 0.01).ConclusionIn conclusion, our data suggest that the pandemic had a more significant impact on respiratory emergency cases than on pediatric emergencies in general.

Calcium dynamics into astrocytes influence the activity of nearby neuronal structures. However, because previous reports show that astrocytic calcium signals largely mirror neighboring neuronal activity, current information coding models neglect astrocytes. Using simultaneous two-photon calcium imaging of astrocytes and neurons in the hippocampus of mice navigating a virtual environment, we demonstrate that astrocytic calcium signals encode (i.e., statistically reflect) spatial information that could not be explained by visual cue information. Calcium events carrying spatial information occurred in topographically organized astrocytic subregions. Importantly, astrocytes encoded spatial information that was complementary and synergistic to that carried by neurons, improving spatial position decoding when astrocytic signals were considered alongside neuronal ones. These results suggest that the complementary place dependence of localized astrocytic calcium signals may regulate clusters of nearby synapses, enabling dynamic, context-dependent variations in population coding within brain circuits. A combination of functional imaging of astrocytes and neurons in the mouse hippocampus with information theory analysis shows that calcium dynamics in topographically-organized subcellular regions of astrocytes encode information about an animal’s position that is complementary and synergistic to that encoded in the spike output of surrounding neurons.

Nuestro estudio es el primer análisis que muestra la tendencia de la distribución del bienestar en Cuba, en el periodo de las reformas económicas y sociales impulsadas por el presidente Raúl Castro. Los análisis revelan que el bienestar, premisa del desarrollo humano y medido como acceso a bienes y servicios fundamentales y no monetarios, ha aumentado, a pesar de la crisis económica; en tanto que las desigualdades en el acceso al bienestar han aumentado poco en el periodo 2006-2011 y han disminuido en el periodo siguiente, para llegar en 2014 a un nivel ligeramente inferior al de 2006.

Over the past few years, the possibility of modulating fast brain oscillatory activity in the gamma (γ) band through transcranial alternating current stimulation (tACS) has been discussed in the context of both cognitive enhancement and therapeutic scenarios. However, the effects of tACS targeting regions outside the motor cortex, as well as its spatial specificity, are still unclear. Here, we present a concurrent tACS-fMRI block design study to characterize the impact of 40 Hz tACS applied over the left and right dorsolateral prefrontal cortex (DLPFC) in healthy subjects. Results suggest an increase in blood oxygenation level-dependent (BOLD) activity in the targeted bilateral DLPFCs, as well as in surrounding brain areas affected by stimulation according to biophysical modeling, i.e., the premotor cortex and anterior cingulate cortex (ACC). However, off-target effects were also observed, primarily involving the visual cortices, with further effects on the supplementary motor areas (SMA), left subgenual cingulate, and right superior temporal gyrus. The specificity of 40 Hz tACS over bilateral DLPFC and the possibility for network-level effects should be considered in future studies, especially in the context of recently promoted gamma-induction therapeutic protocols for neurodegenerative disorders.

The StimQ questionnaire is used to assess the home environment of children. The questionnaire is comprised of four subscales, and it was completed by the main caregiver. The items were different considering the band ages of the children: infants (5–12 months), toddlers (12–36 months), and preschoolers (36–72 months). The aim of the study was to translate, transculturally adapt, and evaluate the psychometric characteristics of the Italian version of the StimQ. To achieve this goal, a sample of 142 children was recruited from different kindergartens. The mean age of the group was 30.63 (SD 19.56), and 112 of them were female. The main caregiver was the mother in 95% of the cases. The Cronbach’s alfa was excellent, considering the total score (0.82 for infants, 0.85 for toddlers, and 0.86 for preschoolers). Intrarater reliability was performed by administering the questionnaire after 48 h and by two different researchers. Both analyses showed an excellent reliability for the total score and all the subscales. The intrarater reliability was 0.99 for the infant, 1 for the toddler and 0.99 for the preschooler age groups. The interrater reliability was 0.95 for the infant, 0.93 for the toddler, and 0.97 for the preschooler age groups. The StimQ is a reliable questionnaire that could be helpful for clinicians and researchers who work with children in Italy.

Abstract Background Procedural sedation is increasingly needed in pediatrics. Although different drugs or drugs association are available, which is the safest and most efficient has yet to be defined, especially in syndromic children with increased sedation-related risk factors. Case report we report the case of a five-year-old child affected by alpha-mannosidosis who required procedural sedation for an MRI scan and a lumbar puncture. We administered intranasal dexmedetomidine (4 μg/kg) 45 min before intravenous cannulation, followed by one bolus of ketamine (1 mg/kg) for each procedure. The patient maintained spontaneous breathing and no desaturation or any complication occurred. Conclusion intranasal dexmedetomidine and intravenous ketamine could be a feasible option for MRI and lumbar puncture in children with alpha-mannosidosis needing sedation.

Music teaching helps to develop cognitive skills in students. As stated by Villanueva and Molero, students “receiving a musical education achieve better performance and results in spatial, temporal, mathematical, reading and verbal memory tasks” (2014). This positive effect is especially relevant in students with high intellectual abilities since music also helps to develop emotional intelligence and makes easier the interaction of this type of student with their environment (Swanwick, 1988). The University of Cádiz, together with the Regional Government of Andalusia, through its University Mentoring program aimed at the development of students with high intellectual abilities, offers different learning pathways every year for Secondary Education, High School Diploma and Intermediate and Advance Vocational Training Programs in the province of Cádiz. These workshops are aimed at encouraging this specific profile of students through targeted training in different areas of knowledge and mentoring by university teachers in small groups. This year, the Area of Didactics of Musical Expression of the Department of Didactics of Corporal, Plastic and Musical Education of the University of Cádiz has offered a workshop entitled Hearing and musical creation through ICT. This proposal pursued a main objective: to offer students free software tools that would allow them to acquire knowledge and skills about musical language autonomously and develop their musical creativity. In doing so, a qualitative methodology has been applied based on collaborative work among the students (13). They had to perform individual music composition and recording activities, which were presented to the rest of the students and modified according to the instructions of their classmates. The results obtained have been very positive: a high level of commitment of students and families. The students have proved acquisition of musical knowledge and applied it in practical cases.

Hoy en día, la formación del profesorado en competencia mediática sigue siendo un reto educativo a nivel mundial. El rol del profesorado ha cambiado, las exigencias formativas del siglo XXI suponen una transformación de la enseñanza tradicional. El presente artículo describe y analiza la relación que tienen los profesores con los medios, su nivel de competencia y buenas prácticas en procesos de alfabetización mediática. Mediante procesos cualitativos y cuantitativos a partir de grupos de discusión con profesorado implicado en buenas prácticas de los diferentes niveles educativos, se analiza cómo se posiciona actualmente la escuela y el profesorado frente a los medios. Los resultados indican la existencia de un buen nivel de competencia en redes digitales y en el manejo de ordenadores en general tanto en profesores como alumnos, aunque en estos últimos se evidencia falta de actitud crítica ante los medios. Las limitaciones técnicas y formativas no impiden al profesorado ser consciente de la necesidad de plantear un escenario educativo adecuado para el desarrollo de la competencia mediática. En conclusión, la alfabetización mediática ofrecida hoy en día en nuestros centros educativos cumple en gran medida con las exigencias sociales, aunque debe hacer un mayor hincapié en la formación crítica del alumnado.

p38MAP kinase (MAPK) signal transduction pathways are important regulators of inflammation and the immune response; their involvement in immune cell development and function is still largely unknown. Here we analysed the role of the p38 MAPK isoforms p38γ and p38δ in B cell differentiation in bone marrow (BM) and spleen, using mice lacking p38γ and p38δ, or conditional knockout mice that lack both p38γ and p38δ specifically in the B cell compartment. We found that the B cell differentiation programme in the BM was not affected in p38γ/δ-deficient mice. Moreover, these mice had reduced numbers of peripheral B cells as well as altered marginal zone B cell differentiation in the spleen. Expression of co-stimulatory proteins and activation markers in p38γ/δ-deficient B cells are diminished in response to B cell receptor (BCR) and CD40 stimulation; p38γ and p38δ were necessary for B cell proliferation induced by BCR and CD40 but not by TLR4 signaling. Furthermore, p38γ/δ-null mice produced significantly lower antibody responses to T-dependent antigens. Our results identify unreported functions for p38γ and p38δ in B cells and in the T-dependent humoral response; and show that the combined activity of these kinases is needed for peripheral B cell differentiation and function.

This manuscript focuses on factors — in particular, political credential and mobilization of personal contacts — associated to becoming a cuentapropista, namely a self-employed worker in the emerging private sector. Data were collected shortly before the launch of the recent reforms plan aiming at revitalizing the market-oriented reforms of the 1990s. The results suggest that being a member of the PCC is negatively associated to entering in the private sector. Older individuals and recipients of remittances have instead a higher probability to become a self-employed worker. Moreover, the results provide some support to previous findings that turning to personal ties — especially if strong — to successfully complete a job search, is a common practice in Cuba.

Patients coinfected with human immunodeficiency virus (HIV) and hepatitis C virus (HCV) have higher levels of immune activation, impaired antigen‐specific responses, and accelerated fibrogenesis compared to patients monoinfected with HCV. Whether different direct‐acting antiviral (DAA) combinations have differential effects on immunophenotypes and functions following successful HCV therapy remain unknown. Therefore, we aimed to assess the peripheral T‐cell immunophenotypes and functions in patients coinfected with HIV/HCV who were successfully treated with combination DAA treatment regimens. We analyzed peripheral blood mononuclear cells (PBMCs) at baseline and at the time of sustained viral response (SVR) from subjects treated with three different combination DAA regimens: daclatasvir (DCV) and asunaprevir (ASV) for 24 weeks (CONQUER 2‐DAA), DCV/ASV/beclabuvir (BCV) for 12 weeks (CONQUER 3‐DAA), and sofosbuvir (SOF) and ledipasvir (LDV) for 12 weeks (ERADICATE study). We used flow cytometry to assess T‐cell phenotypes (activation and exhaustion) and HCV‐specific T‐cell functions (cytokine secretion and cytotoxicity). Statistical analyses were conducted using the Wilcoxon matched‐pairs signed‐rank test with P < 0.05 considered significant. Overall, there was an improvement in T‐cell exhaustion markers, a decrease in T‐cell activation, an increase in the effector memory population, and improved T‐cell function after achieving SVR, with the largest effects noted with CONQUER 3‐DAA treatment. Conclusion: Treatment with DCV/ASV/BCV in patients coinfected with HIV/HCV resulted in greater restoration of the T‐cell impairments and perturbations associated with HIV/HCV coinfection to an extent that was greater than that observed in either two‐drug regimens. We showed that different DAA‐based therapies have different immunologic outcomes after successful HCV treatment in patients coinfected with HIV/HCV. This information will be beneficial for providers when selecting the regimens for patients coinfected with HIV/HCV.

Gut microbiota plays a key role in obesity and non-alcoholic fatty liver disease (NAFLD), so synbiotics could be a therapeutic alternative. We aim to evaluate a nutritional intervention together with the administration of the bacteria Akkermansia muciniphila and the antioxidant quercetin in an in vivo model of early obesity and NAFLD. 21-day-old rats were fed with control or high-fat diet for six weeks. Then, all animals received control diet supplemented with/without quercetin and/or A. muciniphila for three weeks. Gut microbiota, NAFLD-related parameters, circulating bile acids (BAs) and liver gene expression were analyzed. The colonization with A. muciniphila was associated with less body fat, while synbiotic treatment caused a steatosis remission, linked to hepatic lipogenesis modulation. The synbiotic promoted higher abundance of Cyanobacteria and Oscillospira, and lower levels of Actinobacteria, Lactococcus, Lactobacillus and Roseburia. Moreover, it favored elevated unconjugated hydrophilic BAs plasma levels and enhanced hepatic expression of BA synthesis and transport genes. A. muciniphila correlated with circulating BAs and liver lipid and BA metabolism genes, suggesting a role of this bacterium in BA signaling. Beneficial effects of A. muciniphila and quercetin combination are driven by gut microbiota modulation, the shift in BAs and the gut-liver bile flow enhancement.

Direct acting antiviral (DAA) regimens of 12 weeks result in HCV clearance in vast majority of patients across genotypes. We previously demonstrated an ultra-short regimen of 4 weeks DAA cleared HCV in a subset of patients. Here, we hypothesized that individual level of antiviral immunity differentially influenced viral clearance and investigated biomarkers of a successful response. Cohorts of HCV patients treated for 4 weeks with DAA therapy who either achieved sustained virologic response (SVR) or relapsed were compared at baseline and at end of therapy (EOT) for immune cell phenotypes and HCV specific immunity. Higher levels of PD-1+ CD8+ and CD4+ T lymphocytes co-expressing inhibitory receptors (IR) were present at baseline and at EOT in HCV patients who eventually achieved SVR compared with those who relpased. HCV specific CD8+ T cells were predominantly contained within these IR expressing PD-1+ subsets. Patients in the SVR group had significantly higher CD8+ T cell degranulation in response to HCV peptides at baseline and higher levels of cytokine producing T cells at EOT time-point, relative to those who relapsed. In ex vivo cultures, PD-1+CD160+ CD8+ T cells had higher HCV specific degranulation and PD-1+2B4+ CD8+ T cells had higher cytokine expression (IFNγ+TNFα+ or IFNγ+CD107a+) compared with single or no IR expressing subsets, indicating higher virus specific functional capacity of these subsets. Receiver operating characteristics curve (ROC) for baseline circulating frequencies of PD-1+CD160+, PD-1+Tim-3+ CD8+ T cells and PD-1+CD160+, PD-1+Blimp-1+, PD-1−CTLA4+ CD4+ T cells respectively, had associated C-statistics of 0.8214 and 0.9451 for discriminatin of patients who successfully cleared HCV with 4 weeks treatment. Thus, PD-1+ virus-specific CD8+ T cell subsets with cytotoxic capacity are present in a subset of chronic HCV infected individuals that associate with ability to achieve SVR, indicating role of immunity in DAA mediated viral clearance with short duration therapy.

Understanding community assembly and processes driving diversity in deep-sea environments is a major challenge in marine microbial ecology. The deep sea represents the largest ecosystem on Earth, but its remoteness makes the microbial community composition and functionality largely unknown. Moreover, microbial-focused studies comparing different deep-sea habitats like dynamic submarine canyons and slope ecosystems altogether are rare. The present work aims to study the deep-sea seafloor microbial communities (Bacteria and Archaea) of Blanes Canyon and its adjacent western open slope (NW Mediterranean) at ca. 1500 m deep, in autumn and spring, and along the vertical sediment profile. Microbial assemblages were studied in terms of abundance, diversity (α and β), community structure and functional potential through 16S rRNA tag-sequencing to assess their adaptations to the canyon’s idiosyncrasy. Furthermore, the relationships of microbes with environmental variables and a potential predator (nematodes) were also assessed. We observed the microbial assemblages and their predicted functional profiles to be more heterogeneous and with higher temporal variability in the canyon than in the open slope. Although their phyla composition was similar, both the dominant and richest phyla showed significant differences in proportion between canyon and slope. Bacterial diversities were higher in the canyon than in the open slope, together with nematode abundances. Along the vertical sediment profile, microbial abundances consistently decreased with depth in the open slope, while we found more variability within the canyon, linked to an enhancement of aerobic metabolisms in the most superficial sediment layer. Grain size was correlated with microbial abundances and explained part of the variability in the community structure. Nematode and microbial abundances were correlated in slope environments, while in the canyon phytodetritics inputs (Chl a and Chl a: phaeo) and organic carbon seemed to play a role in controlling microbial diversity and abundance. These results suggest that the deep-sea seafloor is strongly connected to coastal and pelagic productive areas through the canyon system in a stronger manner than to the open slope, thus modulating resource availability while driving changes in the microbial biosphere and the higher trophic levels of the deep-sea food web.

In the particular case of Spain, student and teacher difficulties associated with the mathematical discipline have been evidenced in PISA and TEDS-M reports. As we consider that the teachers’ difficulties are connected to the students’ performance, we propose a multi-disciplinary approach to deliver specific didactic/mathematical knowledge to the trainee teachers. Such additional instruction shall be meaningfully connected to the real needs of the schools, so a service-learning approach is proposed here. In the present manuscript, the trainee teachers have co-designed educational escape rooms (in coordination with local schools) with the aim of mobilizing curricular knowledge. The goal of the educational escape rooms is to foster the mathematic-related competencies by establishing meaningful connections to other curricular disciplines (music-related knowledge, in the case of this study). This paper reports on the particular experience developed with a group of students (trainee teachers) while designing their educational escape rooms, focusing on the particular case of a specific student to evidence the formative potential of the procedure. The didactic suitability of the proposed escape room has been analyzed and professional development has also been discussed, showing the mobilization of relevant professional skills and fostering the related music and mathematical didactic competencies by shifting the teaching perspective from an algorithmic point of view to a more “reasoning and designing” strategy. This constitutes an evidence of the formative potential on the co-design of educational escape rooms, when designed in the frame of a service learning approach.

Several studies from transitional economies focus on the role of political credentials and personal networks in favouring upward mobility. Nevertheless, both topics remained empirically rather unexplored in Cuba. The present article quantitatively explores these topics, thereby trying to fill these gaps. Survey data were collected in 2010–11 in Havana. The sample is composed of workers ( N = 269) and includes information about their social networks. Research findings from this study suggest that (1) political credentials are not a prerequisite for reaching highly remunerated jobs (on the contrary, according to my results, Party members are less frequently found in highly remunerative positions than non-members) and (2) Cubans commonly turn to personal ties, especially if they are strong, in order to successfully complete a job search. Both results are consistent with research findings from transitional economies at a comparable kind of market penetration and with first findings from a social mobility study carried out by the Cuban Centro de Investigaciones Psicológicas y Sociológicas (CIPS 2009).

El presente artículo muestra un conjunto de buenas prácticas desarrolladas en centros e instituciones educativas (Infantil, Primaria, Secundaria, Universidad, adultos y mayores), que trabajan competencias mediáticas en las provincias andaluzas. Metodológicamente, se ha realizado una observación por cada provincia andaluza, dichas experiencias nos han dado a conocer cada institución y el colectivo en el que están enmarcados, ofreciéndonos datos de relevancia sobre cada caso o "Buenas prácticas" educativas. Se procuran ejemplos diversos de educación mediática en un amplio rango de entornos de aprendizaje, poniéndose de manifiesto la diversidad de enfoques a la hora de programar prácticas de alfabetización en competencia mediática.

We sought to identify with some aspects of this study the levels of knowledge and perception of quality of life of seniors on the DM. The sample consisted of 78 elderly subjects, all of which responded to the Brazilian version of the Knowledge Scale Questionnaire Diabetes Mellitus before and after guidelines on the pathology. The data presented in this study showed a deficiency in knowledge of the elderly on the prevention of disease as much about the treatment and maintenance of quality of life of existing carriers.

Background & Objective: Cytokines are immune system factors known to play a key role in both HCV clearance and the development of infection the impact of host genetic factors on the clinical outcome of HCV infection has not been elucidated. On the other hand, the impact of Genetic changes like Single nucleotide polymorphisms on expression rate and function of Cytokine is under study. The aim of the present study was to compare TGF-B cytokine gene polymorphisms associated with the susceptibility to HCV infection. Materials & Methods: In this case-control study population composed of 125 individuals infected with HCV and 125 healthy controls. Genotyping was carried out by PCR-RFLP and the distribution of the TGF-B1 Gene 915 G>C polymorphism was compared in these groups. For confirmation of RFLP results, 10% of samples were genotyped by direct sequencing. Results: The frequency of the TGF-β1 gene polymorphisms at position 915 in the HCV patients was GG (92%), GC(8%), CC(0%) and in the healthy controls was GG (89.6%), GC(8.8%),CC(1.6%), respectively. Allele frequency in the patients was G(96%),C(4%) and in the controls was G(94%)and C(6%).Statistically, there was no significant difference in genotype or allele frequency between the HCV patients and the control group. Conclusion: Distribution of genotypes in the HCV patients was similar to the results of some other studies, but the frequency of genotypes in the healthy controls of this study was different from the results in studies carried out in populations out of Iran. In conclusion, we can not consider TGF-B1 Gene 915 G>C polymorphism as an increasing or decreasing factor for susceptibility to HCV infection in our studied population.

Ochrobactrum intermedium is considered as an emerging human environmental opportunistic pathogen with mild virulence. The distribution of isolates and sequences described in literature and databases showed frequent association with human beings and polluted environments. As population structures are related to bacterial lifestyles, we investigated by multi-locus approach the genetic structure of a population of 65 isolates representative of the known natural distribution of O. intermedium. The population was further surveyed for genome dynamics using pulsed-field gel electrophoresis and genomics. The population displayed a clonal epidemic structure with events of recombination that occurred mainly in clonal complexes. Concerning biogeography, clones were shared by human and environments and were both cosmopolitan and local. The main cosmopolitan clone was genetically and genomically stable, and grouped isolates that all harbored an atypical insertion in the rrs. Ubiquitism and stability of this major clone suggested a clonal succes in a particular niche. Events of genomic reduction were detected in the population and the deleted genomic content was described for one isolate. O. intermedium displayed allopatric characters associated to a tendancy of genome reduction suggesting a specialization process. Considering its relatedness with Brucella, this specialization might be a commitment toward pathogenic life-style that could be driven by technological selective pressure related medical and industrial technologies.

Alström Syndrome (ALMS) is a rare genetic disorder (483 living cases), characterized by many clinical manifestations, including blindness, obesity, type 2 diabetes and cardiomyopathy. ALMS is caused by mutations in the ALMS1 gene, encoding for a large protein with implicated roles in ciliary function, cellular quiescence and intracellular transport. Patients with ALMS have extensive fibrosis in nearly all tissues resulting in a progressive organ failure which is often the ultimate cause of death. To focus on the role of ALMS1 mutations in the generation and maintenance of this pathological fibrosis, we performed gene expression analysis, ultrastructural characterization and functional assays in 4 dermal fibroblast cultures from ALMS patients. Using a genome-wide gene expression analysis we found alterations in genes belonging to specific categories (cell cycle, extracellular matrix (ECM) and fibrosis, cellular architecture/motility and apoptosis). ALMS fibroblasts display cytoskeleton abnormalities and migration impairment, up-regulate the expression and production of collagens and despite the increase in the cell cycle length are more resistant to apoptosis. Therefore ALMS1-deficient fibroblasts showed a constitutively activated myofibroblast phenotype even if they do not derive from a fibrotic lesion. Our results support a genetic basis for the fibrosis observed in ALMS and show that both an excessive ECM production and a failure to eliminate myofibroblasts are key mechanisms. Furthermore, our findings suggest new roles for ALMS1 in both intra- and extra-cellular events which are essential not only for the normal cellular function but also for cell-cell and ECM-cell interactions.

To maximize the therapeutic efficacy of cardiac muscle constructs produced by stem cells and tissue engineering protocols, suitable scaffolds should be designed to recapitulate all the characteristics of native muscle and mimic the microenvironment encountered by cells in vivo. Moreover, so not to interfere with cardiac contractility, the scaffold should be deformable enough to withstand muscle contraction. Recently, it was suggested that the mechanical properties of scaffolds can interfere with stem/progenitor cell functions, and thus careful consideration is required when choosing polymers for targeted applications. In this study, cross-linked poly-ε-caprolactone membranes having similar chemical composition and controlled stiffness in a supra-physiological range were challenged with two sources of myoblasts to evaluate the suitability of substrates with different stiffness for cell adhesion, proliferation and differentiation. Furthermore, muscle-specific and non-related feeder layers were prepared on stiff surfaces to reveal the contribution of biological and mechanical cues to skeletal muscle progenitor differentiation. We demonstrated that substrate stiffness does affect myogenic differentiation, meaning that softer substrates can promote differentiation and that a muscle-specific feeder layer can improve the degree of maturation in skeletal muscle stem cells.