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Project-based learning (PjBL) is becoming widespread in many schools. However, the evidence of its effectiveness in the classroom is still limited, especially in basic education. The aim of the present study was to perform a systematic review of the empirical evidence assessing the impact of PjBL on academic achievement of kindergarten and elementary students. We also examined the quality of studies, their compliance with basic prerequisites for a successful result, and their fidelity towards the key elements of PBL intervention. For this objective, we conducted a literature search in January 2020. The inclusion criteria for the review required that studies followed a pre-post design with control group and measured quantitatively the impact of PBL on content knowledge of students. The final sample included eleven articles comprising data from 722 students. The studies yielded inconclusive results, had important methodological flaws, and reported insufficient or no information about important aspects of the materials, procedure and key requirements from students and instructors to guarantee the success of PjBL. Educational implications of these results are discussed. [ABSTRACT FROM AUTHOR]

Teaching a diverse classroom is a challenging task. Educators are faced daily with the difficult task of making many decisions about how to educate each of their students. To do this, they mainly rely on their experience and that of their colleagues, their values, and thoughts. Although they are inherent and important in the profession of teaching, sometimes these resources may not suffice to make the best decisions, particularly when teachers are continuously bombarded with numerous fads and poorly grounded ideas about education. In this context, research-informed practice emerges as a promising approach. It involves integrating the professional expertise of teachers with the best evidence of researchers to make better decisions and improve education. However, for this approach to be successfully implemented, the gap between researchers and practitioners must first be bridged. The possible solutions to this challenge involve acting in three contexts: research production, research communication and research use. Specific measures in each of these contexts are described. [ABSTRACT FROM AUTHOR]

Over the last century, sporadic research has suggested that people whose hand, eye, foot, or ear dominances are not consistently right- or left-sided are at special risk of suffering academic difficulties. This phenomenon is known as crossed laterality. Although the bulk of this research dates from 1960’s and 1970’s, crossed laterality is becoming increasingly popular in the area of school education, driving the creation of several interventions aimed at restoring or consolidating lateral dominance. However, the available evidence is fragmentary. To determine the impact of crossed laterality on academic achievement and intelligence, we conducted a systematic review and meta-analysis of articles published since 1900. The inclusion criteria for the review required that studies used one or more lateral preference tasks for at least two specific parts of the body; they included a valid measure of crossed laterality; they measured the impact of crossed laterality on academic achievement or intelligence; and they included participants between 3 and 17 years old. The final sample included 26 articles that covered a total population of 3578 children aged 5 to 12. Taken collectively, the results of these studies do not support the claim that there is a reliable association between crossed laterality and either academic achievement or intelligence. Along with this, we detected important shortcomings in the literature, such as considerable heterogeneity among the variables used to measure laterality and among the tasks utilized to measure the outcomes. The educational implications of these results are discussed. [ABSTRACT FROM AUTHOR]

Fatty acid elongase ELOVL5 is part of a protein family of multipass transmembrane proteins that reside in the endoplasmic reticulum where they regulate long-chain fatty acid elongation. A missense variant (c.689G>T p.Gly230Val) in ELOVL5 causes Spinocerebellar Ataxia subtype 38 (SCA38), a neurodegenerative disorder characterized by autosomal dominant inheritance, cerebellar Purkinje cell demise and adult-onset ataxia. Having previously showed aberrant accumulation of p.G230V in the Golgi complex, here we further investigated the pathogenic mechanisms triggered by p.G230V, integrating functional studies with bioinformatic analyses of protein sequence and structure. Biochemical analysis showed that p.G230V enzymatic activity was normal. In contrast, SCA38-derived fibroblasts showed reduced expression of ELOVL5, Golgi complex enlargement and increased proteasomal degradation with respect to controls. By heterologous overexpression, p.G230V was significantly more active than wild-type ELOVL5 in triggering the unfolded protein response and in decreasing viability in mouse cortical neurons. By homology modelling, we generated native and p.G230V protein structures whose superposition revealed a shift in Loop 6 in p.G230V that altered a highly conserved intramolecular disulphide bond. The conformation of this bond, connecting Loop 2 and Loop 6, appears to be elongase-specific. Alteration of this intramolecular interaction was also observed when comparing wild-type ELOVL4 and the p.W246G variant which causes SCA34. We demonstrate by sequence and structure analyses that ELOVL5 p.G230V and ELOVL4 p.W246G are position-equivalent missense variants. We conclude that SCA38 is a conformational disease and propose combined loss of function by mislocalization and gain of toxic function by ER/Golgi stress as early events in SCA38 pathogenesis. [ABSTRACT FROM AUTHOR]

The LEXIN database offers psycholinguistic indexes of the 13,184 different words (types) computed from 178,839 occurrences of these words (tokens) contained in a corpus of 134 beginning readers widely used in Spain. This database provides four statistical indicators: F (overall word frequency), D (index of dispersion across selected readers), U (estimated frequency per million words), and SFI (standard frequency index). It also gives information about the number of letters, syntactic category, and syllabic structure of the words included. To facilitate comparisons, LEXIN provides data from LEXESP's (Sebastiá-Gallés, Marti, Cuetos, & Carreiras, 2000), Alameda and Cuetos's (1995), and Martlnez and Garcia's (2004) Spanish adult psycholinguistic frequency databases. Access to the LEXIN database is facilitated by a computer program. The LEXIN program allows for the creation of word lists by letting the user specify searching criteria. LEXIN can be useful for researchers in cognitive psychology, particularly in the areas of psycholinguistics and education. [ABSTRACT FROM AUTHOR]

Since Gardner suggested that human beings hold multiple intelligences, numerous teachers have adapted and incorporated the multiple intelligence theory (MIT) into their daily routine in the classroom. However, to date, the efficacy of MIT–inspired methodologies remains unclear. The focus of the present study was to perform a systematic review and a meta–analysis to assess the impact of these interventions on academic achievement through reading, maths, or science tests. The inclusion criteria for the review required that studies should estimate quantitatively the impact of an MIT–based intervention on the academic performance and that they followed a pre–post design with a control group. The final sample included 39 articles comprising data from 3009 pre-school to high school students, with diverse levels of achievement, from 14 different countries. The results showed that the studies had important methodological flaws, like small sample sizes or lack of active control groups; they also reported insufficient information about key elements, such as the tools employed to measure the outcomes or the specific activities performed during training, and revealed signs of publication or reporting biases that impeded a valid evaluation of the efficacy of MIT applied in the classroom. The educational implications of these results are discussed. • A meta-analysis of the impact of MIT-inspired interventions on learning was performed. • The qualitative analysis showed that the primary studies have important methodological flaws. • The reported effect sizes were remarkably larger than the usual in education. • MIT-inspired interventions for enhancing academic achievement learning is not recommended. [ABSTRACT FROM AUTHOR]

Students who perform better academically are generally more accurate in assessing their performance. In other words, they are better calibrated, showing smaller discrepancy between their predicted and actual performance. Research into contextual variables related to performance has shown that students sitting position may be related to their performance in class. In the current study we attempted to bring several lines of research together and examine whether there are differences in academic performance, calibration, and confidence judgments among students who choose various sitting positions in the classroom. 130 college students (M = 21.52, SD = 1.59; 103 female) attending courses at the University of Jaen in Spain participated. Results revealed that students' calibration differed depending on their preferred sitting position. We found that students in middle rows were optimally calibrated, students in the front rows overestimated their performance, and those in the back rows tended to underestimate their performance. Furthermore, students in the front rows were the most confident in their estimates, whereas students in the middle and back rows were less confident, suggesting the manifestation of the Dunning-Kruger effect. Future directions for contextual variables contributing to students' optimal calibration are discussed. • Students who perform better academically are generally better calibrated. • Students' sitting position may be related to their performance in class. • Students' calibration differed depending on their preferred sitting position. • We show evidence of the Dunning-Kruger effect. [ABSTRACT FROM AUTHOR]

Background Despite major progress in global vaccination coverage, immunization rates are falling, resulting in outbreaks of vaccine-preventable diseases. This study analyses content and source of the most popular tweets related to a recent case in Spain where an unvaccinated child contracted and later died from diphtheria. Understanding the characteristics of these tweets in the context of vaccination could inform efforts by health promotion professionals to increase their reach and impact. Methods We extracted tweets containing keywords related to the diphtheria case (from 1 May to 15 July 2015). We explored the prevalence of terms relating to policy and misinformation and manually coded the 194 most popular tweets (retweeted 100 or more times) with regard to source, topic, tone and sentiment. Results A total of 722 974 tweets were collected. Prevalence of terms relating to policy and misinformation increased at the onset of the case and after the death of the child. Popular tweets (194) were either pro-vaccination (58%) or neutral, with none classified as anti-vaccination. Popular topics included criticism towards anti-vaccination groups (35%) and effectiveness of immunization (22%). Popular tweets were informative (47%) or opinions (53%), which mainly expressed frustration (24%) or humour/sarcasm (23%). Popular Twitter accounts were newspaper and TV channels (15%), as well as individual journalists and authors of popular science (13.4%). Conclusions Healthcare organizations could collaborate with popular journalists or news outlets and employ authors of popular science to disseminate health information on social media, while addressing public concerns and misinformation in accessible ways. [ABSTRACT FROM AUTHOR]

Systemic AA-amyloidosis is a protein-misfolding disease characterized by fibril deposition of serum amyloid-A protein (SAA) in several organs in humans and many animal species. Fibril deposits originate from abnormally high serum levels of SAA during chronic inflammation. A high prevalence of AA-amyloidosis has been reported in captive cheetahs and a horizontal transmission has been proposed. In domestic cats, AA-amyloidosis has been mainly described in predisposed breeds but only rarely reported in domestic short-hair cats. Aims of the study were to determine AA-amyloidosis prevalence in dead shelter cats. Liver, kidney, spleen and bile were collected at death in cats from 3 shelters. AA-amyloidosis was scored. Shedding of amyloid fibrils was investigated with western blot in bile and scored. Descriptive statistics were calculated. In the three shelters investigated, prevalence of AA-amyloidosis was 57.1% (16/28 cats), 73.0% (19/26) and 52.0% (13/25), respectively. In 72.9% of cats (35 in total) three organs were affected concurrently. Histopathology and immunofluorescence of post-mortem extracted deposits identified SAA as the major protein source. The duration of stay in the shelters was positively associated with a histological score of AA-amyloidosis (B = 0.026, CI95% = 0.007–0.046; p = 0.010). AA-amyloidosis was very frequent in shelter cats. Presence of SAA fragments in bile secretions raises the possibility of fecal-oral transmission of the disease. In conclusion, AA-amyloidosis was very frequent in shelter cats and those staying longer had more deposits. The cat may represent a natural model of AA-amyloidosis. [ABSTRACT FROM AUTHOR]

We describe an autosomal dominant disorder associated with loss-of-function variants in the Cell cycle associated protein 1 (CAPRIN1 ; MIM*601178). CAPRIN1 encodes a ubiquitous protein that regulates the transport and translation of neuronal mRNAs critical for synaptic plasticity, as well as mRNAs encoding proteins important for cell proliferation and migration in multiple cell types. We identified 12 cases with loss-of-function CAPRIN1 variants, and a neurodevelopmental phenotype characterized by language impairment/speech delay (100%), intellectual disability (83%), attention deficit hyperactivity disorder (82%) and autism spectrum disorder (67%). Affected individuals also had respiratory problems (50%), limb/skeletal anomalies (50%), developmental delay (42%) feeding difficulties (33%), seizures (33%) and ophthalmologic problems (33%). In patient-derived lymphoblasts and fibroblasts, we showed a monoallelic expression of the wild-type allele, and a reduction of the transcript and protein compatible with a half dose. To further study pathogenic mechanisms, we generated s CAPRIN1 +/− human induced pluripotent stem cells via CRISPR–Cas9 mutagenesis and differentiated them into neuronal progenitor cells and cortical neurons. CAPRIN1 loss caused reduced neuronal processes, overall disruption of the neuronal organization and an increased neuronal degeneration. We also observed an alteration of mRNA translation in CAPRIN1 +/− neurons, compatible with its suggested function as translational inhibitor. CAPRIN1 +/− neurons also showed an impaired calcium signalling and increased oxidative stress, two mechanisms that may directly affect neuronal networks development, maintenance and function. According to what was previously observed in the mouse model, measurements of activity in CAPRIN1 +/− neurons via micro-electrode arrays indicated lower spike rates and bursts, with an overall reduced activity. In conclusion, we demonstrate that CAPRIN1 haploinsufficiency causes a novel autosomal dominant neurodevelopmental disorder and identify morphological and functional alterations associated with this disorder in human neuronal models. [ABSTRACT FROM AUTHOR]

Short term treatment with low doses of glucocorticoid analogues has been shown to ameliorate neurological symptoms in Ataxia–Telangiectasia (A–T), a rare autosomal recessive multisystem disease that mainly affects the cerebellum, immune system, and lungs. Molecular mechanisms underlying this clinical observation are unclear. We aimed at evaluating the effect of dexamethasone on the induction of alternative ATM transcripts (ATMdexa1). We showed that dexamethasone cannot induce an alternative ATM transcript in control and A–T lymphoblasts and primary fibroblasts, or in an ATM-knockout HeLa cell line. We also demonstrated that some of the reported readouts associated with ATMdexa1 are due to cellular artifacts and the direct induction of γH2AX by dexamethasone via DNA-PK. Finally, we suggest caution in interpreting dexamethasone effects in vitro for the results to be translated into a rational use of the drug in A–T patients. [ABSTRACT FROM AUTHOR]

Allele-specific silencing by RNA interference (ASP-siRNA) holds promise as a therapeutic strategy for downregulating a single mutant allele with minimal suppression of the corresponding wild-type allele. This approach has been effectively used to target autosomal dominant mutations and single nucleotide polymorphisms linked with aberrantly expanded trinucleotide repeats. Here, we propose ASP-siRNA as a preferable choice to target duplicated disease genes, avoiding potentially harmful excessive downregulation. As a proof-of-concept, we studied autosomal dominant adult-onset demyelinating leukodystrophy (ADLD) due to lamin B1 (LMNB1) duplication, a hereditary, progressive and fatal disorder affecting myelin in the CNS. Using a reporter system, we screened the most efficient ASP-siRNAs preferentially targeting one of the alleles at rs1051644 (average minor allele frequency: 0.45) located in the 3' untranslated region of the gene. We identified four siRNAs with a high efficacy and allele-specificity, which were tested in ADLD patient-derived fibroblasts. Three of the small interfering RNAs were highly selective for the target allele and restored both LMNB1 mRNA and protein levels close to control levels. Furthermore, small interfering RNA treatment abrogates the ADLD-specific phenotypes in fibroblasts and in two disease-relevant cellular models: murine oligodendrocytes overexpressing human LMNB1, and neurons directly reprogrammed from patients' fibroblasts. In conclusion, we demonstrated that ASP-silencing by RNA interference is a suitable and promising therapeutic option for ADLD. Moreover, our results have a broad translational value extending to several pathological conditions linked to gene-gain in copy number variations. [ABSTRACT FROM AUTHOR]

Introduction: Spinocerebellar Ataxia 38 (SCA38) is caused by ELOVL5 gene mutation, with significant reduction of serum docosahexaenoic acid (DHA) levels. DHA supplementation has been proven effective at short-term follow-up. In the present paper, we evaluated long-term safety and efficacy of 600 mg/day oral DHA in SCA38 by a 2-year open label extension study.Methods: Nine SCA38 patients underwent standardised clinical assessment at 62 (T1), 82 (T2) and 104 (T3) weeks, and compared to pre-treatment scores (T0). Brain 18-Fluorodeoxyglucose Positron Emission Tomography and electroneurography were performed at T0 and T3.Results: We found a significant maintenance of clinical symptom improvement at each follow-up time-point (p < 0.001) as compared to T0, a sustained increase of cerebellar metabolism at T3 as compared to T0 (p = 0.013), and no worsening of neurophysiological parameters. No side effect was recorded.Conclusions: Long-term DHA supplementation is an eligible treatment for SCA38. [ABSTRACT FROM AUTHOR]

Objective: Spinocerebellar ataxia 38 (SCA38) is caused by mutations in the ELOVL5 gene, which encodes an elongase involved in the synthesis of polyunsaturated fatty acids, including docosahexaenoic acid (DHA). As a consequence, DHA is significantly reduced in the serum of SCA38 subjects. In the present study, we evaluated the safety of DHA supplementation, its efficacy for clinical symptoms, and changes of brain functional imaging in SCA38 patients.Methods: We enrolled 10 SCA38 patients, and carried out a double-blind randomized placebo-controlled study for 16 weeks, followed by an open-label study with overall 40-week DHA treatment. At baseline and at follow-up visit, patients underwent standardized clinical assessment, brain 18-fluorodeoxyglucose positron emission tomography, electroneurography, and ELOVL5 expression analysis.Results: After 16 weeks, we showed a significant pre-post clinical improvement in the DHA group versus placebo, using the Scale for the Assessment and Rating of Ataxia (SARA; mean difference [MD] = +2.70, 95% confidence interval [CI] = +0.13 to + 5.27, p = 0.042). At 40-week treatment, clinical improvement was found significant by both SARA (MD = +2.2, 95% CI = +0.93 to + 3.46, p = 0.008) and International Cooperative Ataxia Rating Scale (MD = +3.8, 95% CI = +1.39 to + 6.41, p = 0.02) scores; clinical data were corroborated by significant improvement of cerebellar hypometabolism (statistical parametric mapping analyses, false discovery rate corrected). We also showed a decreased expression of ELOVL5 in patients' blood at 40 weeks as compared to baseline. No side effect was recorded.Interpretation: DHA supplementation is a safe and effective treatment for SCA38, showing an improvement of clinical symptoms and cerebellar hypometabolism. Ann Neurol 2017;82:615-621. [ABSTRACT FROM AUTHOR]

Background context The most common adverse event after a lumbar puncture (LP) is a headache: In anaesthesiology, well studied is the protective effect of atraumatic spinal needles, and they are routinely used. However, this is less well known in diagnostic LP, and neurologists use atraumatic needles in less than 2% of times. Purpose The purpose of this study was to define the impact of needle type, atraumatic (Sprotte [S]) versus traumatic (Quincke [Q]) on postdural puncture headache (PDPH) incidence. Study design The study is based on a prospective, randomized, and simple-blinded clinical trial. Patient Sample Patients older than 14 years were scheduled for a diagnostic or therapeutic LP. Outcome Measures The outcome measure included the development of PDPH according to the International Headache Association criteria. Methods Patients fulfilling eligibility criteria were randomly allocated to one of two kinds of spinal needle: atraumatic or S-type or traumatic or Q-type. They were interviewed on days 2 and 7 about the development of PDPH. Results The incidence of PDPH was 22.43% with Q-type needle and 8.51% with S-type needle, p=.04. The duration of PDPH in patients in the S-type was 1 day or less, compared with a median of 4.14 days in the Q-type (p=.00). In the logistic regression model, the S-type needle together with the age of the patient were the only two statistically significant factors in the development of postlumbar puncture headache (PLPH), both of them being protective. Conclusions We found a lower incidence of PDPH with atraumatic needles, and it was statistically significant compared with the traumatic needles. Our study confirms the effectiveness of the atraumatic needles to prevent PDPH. [ABSTRACT FROM AUTHOR]

Spinocerebellar ataxias (SCAs) are a heterogeneous group of autosomal-dominant neurodegenerative disorders involving the cerebellum and 23 different genes. We mapped SCA38 to a 56 Mb region on chromosome 6p in a SCA-affected Italian family by whole-genome linkage analysis. Targeted resequencing identified a single missense mutation (c.689G>T [p.Gly230Val]) in ELOVL5. Mutation screening of 456 independent SCA-affected individuals identified the same mutation in two further unrelated Italian families. Haplotyping showed that at least two of the three families shared a common ancestor. One further missense variant (c.214C>G [p.Leu72Val]) was found in a French family. Both missense changes affect conserved amino acids, are predicted to be damaging by multiple bioinformatics tools, and were not identified in ethnically matched controls or within variant databases. ELOVL5 encodes an elongase involved in the synthesis of polyunsaturated fatty acids of the ω3 and ω6 series. Arachidonic acid and docosahexaenoic acid, two final products of the enzyme, were reduced in the serum of affected individuals. Immunohistochemistry on control mice and human brain demonstrated high levels in Purkinje cells. In transfection experiments, subcellular localization of altered ELOVL5 showed a perinuclear distribution with a signal increase in the Golgi compartment, whereas the wild-type showed a widespread signal in the endoplasmic reticulum. SCA38 and SCA34 are examples of SCAs due to mutations in elongase-encoding genes, emphasizing the importance of fatty-acid metabolism in neurological diseases. [ABSTRACT FROM AUTHOR]

The aim of the study was to investigate the effects of a Reciprocal Teaching-based program (Palincsar & Brown, 1984) on the reading comprehension, reading habits, and fluency of 239 purposively selected lower-middle class students from 3rd to 6th grade (8 to 13 years of age, 49% females) from 5 public schools of Vizcaya and Álava, Spain. A pre and posttest quasi-experimental design with control group was used, randomly assigning whole or split classrooms to each group, for the convenience of each school. The experimental group (n = 135) engaged in 12 50-minutes weekly training sessions, while the control group (n = 104) was exposed to traditional language teaching during the same period. Results show that the intervention had positive effects on reading comprehension, measured by summary and recall tasks, but no effects on fluency and reading habits. The implications for research and practice are discussed, such as the program's flexibility and its implementation by university students. [ABSTRACT FROM AUTHOR]

Simple Summary: PIVKA-II is an aberrant form of vitamin K that has been demonstrated to be increased in human coagulation disorders and in some neoplastic diseases. In veterinary medicine, PIVKA-II levels have been demonstrated to be useful for distinguishing anticoagulant poisoning from other coagulopathies. In forensic pathology, there is the need to distinguish malicious poisoning from other causes of death and, in some cases, identifying poisoned dogs from dogs that died as a result of other coagulative disorders can be challenging. This study evaluated the usefulness of the immunohistochemical expression of PIVKA-II in hepatic and renal tissues in order to identify patients with coagulative disorders due to clinical condition or the ingestion of anticoagulants substances. PIVKA-II is an aberrant form of vitamin K that has been demonstrated to be increased in human coagulation disorders and in some neoplastic diseases. In veterinary medicine, PIVKA-II levels have been demonstrated to be useful for distinguishing anticoagulant poisoning from other coagulopathies. In forensic pathology, there is the need to distinguish malicious poisoning from other causes of death and, in some cases, identifying poisoned dogs from dogs that died as a result of other coagulative disorders can be challenging. In this study, dogs that suddenly died underwent necropsy, histological examination, and toxicological analysis to establish cause of death. PIVKA-II immunohistochemical expression was evaluated on hepatic and renal tissues, and on neoplastic lesions when present. A total of 61 dogs were analyzed and anticoagulant substances were identified in 16 of the 61. Immunolabelling for PIVKA-II was observed in 27 of 61 cases in the liver and in 24 of 61 cases in the kidneys. Among the poisoned dogs, the PIVKA-II expression was present in the liver in 15 of 16 cases and in the kidneys in 16 of 16. Neoplastic lesions represented mainly by haemangiosarcomas were negative. This study highlights how the immunohistochemical expression of PIVKA-II in hepatic and renal tissues can be useful to identify patients with coagulative disorders due to clinical condition or the ingestion of anticoagulants substances. [ABSTRACT FROM AUTHOR]