Lifelong learning and schools as community learning centres : key aspects of a national curriculum draft policy framework for Malta

The island of Malta has been engaged in policy document formulations for curriculum renewal in the country’s educational system (4-16 years of age) since 1988 when the first National Minimum Curriculum (henceforth NMC) was launched (Wain, 1991; Borg et al, 1995). In 1999 a revamped NMC (Ministry of Education, 1999) was developed following a long process of consultation involving various stages and stakeholders. It was a compromise document (Borg & Mayo, 2006) which emerged as a result of reactions to a more radical and coherent draft document produced in 1988. Both curricular documents were subject to debates and critiques (Wain, 1991; Darmanin, 1993; Borg et al, 1995; Giordmaina, 2000; Borg and Mayo, 2006). More recently a series of volumes providing guidelines, key principles and aims for a national curriculum framework (henceforth NCF) have been produced (MEEF, 2011a,b,c,d) and are currently the target of debate and the focus of reactions by various stakeholders in education including teachers who were asked to read the volumes and provide reactions in the form of answers to a set questionnaire. In this paper, I will focus on one aspect of the documents, the first of its three aims: ‘Learners who are capable of successfully developing their full potential as lifelong learners.’ It is that aspect of the framework documents that falls within the purview of the title for this special issue. The use of this notion attests to the influence of the EU’s policy communications on member states, Malta having joined the Union in 2004 (Mayo, 2007).peer-reviewe

Cardiac remodeling and renin-angiotensin system

Myocardial infarction (MI) leads to structural alterations involving the infarcted as well as the non-infarcted areas in a process known as ventricular remodeling. The early phase of remodeling, which starts at the beginning of the MI, is associated with geometric changes and dilation of the infarct zone, as a process collectively known as expansion of the scar. Subsequently, this dilation may progress to the whole ventricle and it is associated with myocytes hypertrophy and fibrosis in the remote zones. These structural changes start on during the initial phase of the healing process of the infarction, contribute to ventricular dysfunction, cause a progression to heart failure and increase mortality. The systemic and cardiac renin-angiotensin systems (RAS) are stimulated post-MI and actively participate in ventricular remodeling, favoring the healing process of the infarct area as well as myocytes hypertrophy, fibrosis, and most of the events associated to the remodeling of non-MI zones. In this review, we analyze different general aspects of postmyocardial infarct ventricular remodeling and the activation and participation of the renin- angiotensin system in its evolution.Sociedad Argentina de Fisiologí

“A proibição da eutanásia” e o juramento médico de Hippocratic stemma

It has been debated whether the Hippocratic Oath’s commitment referring to not administering poisonous/ deadly drugs prohibits: euthanasia, assisted suicide or murder. The first goal was to analyze if the prohibition of administering poisonous/deadly drugs was kept and how it changed in medical oaths of Hippocratic stemma of different time periods and religious orientations. The second aim was discern what is forbidden: euthanasia, assisted suicide or murder. Seventeen medical oaths: 4 Medieval, 2 Modern and 11 Contemporary oaths were studied and divided into those expressing the commitment like the original, those that may include it depending on the interpretation and those that do not mention it. Medieval and Modern oaths express it similarly to the Hippocratic Oath, possibly due to religious and Hippocratic/Galenic influences. What they forbid cannot be inferred. Contemporary oaths maintaining the commitment tend to include phrases regarding active euthanasia and assisted suicide. Other contemporary oaths may generalize it. It would be advisable that medical oaths would contain clear and specific premises regarding this commitment depending on the country, school and the student body’s idiosyncrasies.Ha sido debatido qué es lo que prohíbe el compromiso del Juramento Hipocrático de no administrar drogas venenosas/mortales: la eutanasia, el suicidio asistido o el asesinato. El primer objetivo fue analizar si la prohibición de administrar drogas venenosas/mortales se mantuvo y cómo cambió en juramentos médicos de stemma hipocrática en diferentes tiempos y con distinta orientación religiosa. El segundo objetivo fue discernir qué se prohíbe: si la eutanasia, el suicidio asistido o el asesinato. Se analizaron 17 juramentos médicos: 4 medievales, 2 modernos y 11 contemporáneos. Se dividieron en aquellos que expresan el compromiso como el original, aquellos que podrían incluirlo o no dependiendo de la interpretación y aquellos que no mencionan nada al respecto. Los juramentos medievales y modernos expresan el compromiso de manera similar al Juramento Hipocrático, posiblemente por influencias religiosas e hipocrático/galénicas. Qué es lo que prohíben no puede ser inferido. Los juramentos contemporáneos que mantienen el compromiso suelen incluir frases en relación a la eutanasia activa y al suicidio asistido. Otros juramentos contemporáneos lo generalizarían. Sería recomendable que los juramentos incorporaran compromisos claros dependiendo de la idiosincrasia de los países, instituciones y cuerpo estudiantil.Tem sido debatido se o compromisso do juramento de Hipócrates, referindo-se a não administrção de drogas venenosas /mortais, proíbe: a eutanásia, o suicídio assistido ou o assassinato. O primeiro objetivo foi analisar se a proibição de administrar drogas venenosas/mortais foi mantida e como isso mudou em juramentos médicos de Hippocratic stemma em diferentes períodos de tempo e orientações religiosas. O segundo objetivo foi discernir o que é proibido: eutanásia, suicídio assistido ou assassinato. Dezessete juramentos médicos: 4 medievais, 2 modernos e 11 juramentos contemporâneos foram estudados e divididos naqueles que expressavam o compromisso semelhante ao original, aqueles que podem incluir, consoante a interpretação e aqueles que não o mencionam. Os juramentos medievais e modernos expressam da mesma forma que o juramento de Hipócrates, possivelmente devido a influência religiosa e de Hipócrates/galênica. O que eles proíbem não podem ser inferido. Os juramentos contemporâneos, mantendo o compromisso tendem a incluir frases sobre eutanásia ativa e suicídio assistido. Outros juramentos contemporâneos podem generalizá-lo. Seria aconselhável que os juramentos médicos conteria premissas claras e específicas sobre este compromisso dependendo do país, a escola e as idiossincrasias do corpo estudantil

MRI and clinical syndrome in dura materrelated Creutzfeldt-Jakob disease

Objective : Iatrogenic Creutzfeldt-Jakob disease (iCJD) is mainly associated with dura mater (DM) grafts and administration of human growth hormones (hGH). Data on disease course in DM-CJD are limited. We describe the clinical and diagnostic findings in this patient group with special emphasis on MRI signal alterations. Methods : Ten DM-CJD patients were studied for their clinical symptoms and diagnostic findings. The MRIs were evaluated for signal increase of the cortical and subcortical structures. Results : DM-CJD patients had a median incubation time of 18 years and median disease duration of 7 months. The majority of patients were MM homozygous at codon 129 of the prion protein gene (PRNP) and presented with gait ataxia and psychiatric symptoms. No correlation between the graft site and the initial disease course was found. The MRI showed cortical and basal ganglia signal increase each in eight out of ten patients and thalamic hyperintensity in five out of ten cases. Of interest, patients with thalamic signal increase were homozygous for methionine. Conclusion : The MRI findings in DM-CJD largely resemble those seen in sporadic CJD, as the cortex and basal ganglia are mainly affecte

Does ALS-FUS without FUS mutation represent ALS-FET? Report of three cases

Altres ajuts: This study was partially funded by Fundacio Marató de TV3 (grant no. 20143810 to RSV, no. 20141610 to EG and no. 201437.10 to RRG) and Fondo Europeo de Desarrollo Regional (FEDER) (PI16/01673 to JG and PI15/01618 to RRG). We are indebted to the Neurological Tissue Bank of the Biobanc-Hospital Clinic-IDIBAPS, Barcelona, Spain, for data and sample procurement. We thank Sara Charif, Veronica Santiago, Carmen Schweiger, Leire Etxarri and Abel Muñoz for technical assistance

Determinants of diagnostic investigation sensitivities across the clinical spectrum of sporadic Creutzfeldt-Jakob disease

To validate the provisional findings of a number of smaller studies and explore additional determinants of characteristic diagnostic investigation results across the entire clinical spectrum of sporadic Creutzfeldt-Jakob disease (CJD), an international collaborative study was undertaken comprising 2451 pathologically confirmed (definite) patients. We assessed the influence of age at disease onset, illness duration, prion protein gene (PRNP) codon 129 polymorphism (either methionine or valine) and molecular sub-type on the diagnostic sensitivity of EEG, cerebral MRI and the CSF 14-3-3 immunoassay. For EEG and CSF 14-3-3 protein detection, we also assessed the influence of the time point in a patient's illness at which the investigation was performed on the likelihood of a typical or positive result. Analysis included a large subset of patients (n = 743) in whom molecular sub-typing had been performed using a combination of the PRNP codon 129 polymorphism and the form of protease resistant prion protein [type 1 or 2 according to Parchi et al. (Parchi P, Giese A, Capellari S, Brown P, Schulz-Schaeffer W, Windl O, Zerr I, Budka H, Kopp N, Piccardo P, Poser S, Rojiani A, Streichemberger N, Julien J, Vital C, Ghetti B, Gambetti P, Kretzschmar H. Classification of sporadic Creutzfeldt-Jakob disease based on molecular and phenotypic analysis of 300 subjects. Ann Neurol 1999; 46: 224-233.)] present in the brain. Findings for the whole group paralleled the subset with molecular sub-typing data available, showing that age at disease onset and disease duration were independent determinants of typical changes on EEG, while illness duration significantly influenced positive CSF 14-3-3 protein detection; changes on brain MRI were not influenced by either of these clinical parameters, but overall, imaging data were less complete and consequently conclusions are more tentative. In addition to age at disease onset and illness duration, molecular sub-type was re-affirmed as an important independent determinant of investigation results. In multivariate analyses that included molecular sub-type, time point of the investigation during a patient's illness was found not to influence the occurrence of a typical or positive EEG or CSF 14-3-3 protein result. A typical EEG was most often seen in MM1 patients and was significantly less likely in the MV1, MV2 and VV2 sub-types, whereas VV2 patients had an increased likelihood of a typical brain MRI. Overall, the CSF 14-3-3 immunoassay was the most frequently positive investigation (88.1%) but performed significantly less well in the very uncommon MV2 and MM2 sub-types. Our findings confirm a number of determinants of principal investigation results in sporadic CJD and underscore the importance of recognizing these pre-test limitations before accepting the diagnosis excluded or confirmed. Combinations of investigations offer the best chance of detection, especially for the less common molecular sub-types such as MV2 and MM

Homozygous R136S mutation in PRNP gene causes inherited early onset prion disease

Altres ajuts: Fundació la Marató de TV3/201821-31Background: More than 40 pathogenic heterozygous PRNP mutations causing inherited prion diseases have been identified to date. Recessive inherited prion disease has not been described to date. Methods: We describe the clinical and neuropathological data of inherited early-onset prion disease caused by the rare PRNP homozygous mutation R136S. In vitro PrP propagation studies were performed using recombinant-adapted protein misfolding cyclic amplification technique. Brain material from two R136S homozygous patients was intracranially inoculated in TgMet129 and TgVal129 transgenic mice to assess the transmissibility of this rare inherited form of prion disease. Results: The index case presented symptoms of early-onset dementia beginning at the age of 49 and died at the age of 53. Neuropathological evaluation of the proband revealed abundant multicentric PrP plaques and Western blotting revealed a ~ 8 kDa protease-resistant, unglycosylated PrP fragment, consistent with a Gerstmann-Sträussler-Scheinker phenotype. Her youngest sibling suffered from progressive cognitive decline, motor impairment, and myoclonus with onset in her late 30s and died at the age of 48. Genetic analysis revealed the presence of the R136S mutation in homozygosis in the two affected subjects linked to homozygous methionine at codon 129. One sibling carrying the heterozygous R136S mutation, linked to homozygous methionine at codon 129, is still asymptomatic at the age of 74. The inoculation of human brain homogenates from our index case and an independent case from a Portuguese family with the same mutation in transgenic mice expressing human PrP and in vitro propagation of PrP studies failed to show disease transmissibility. Conclusion: In conclusion, biallelic R136S substitution is a rare variant that produces inherited early-onset human prion disease with a Gerstmann-Sträussler-Scheinker neuropathological and molecular signature. Even if the R136S variant is predicted to be "probably damaging", heterozygous carriers are protected, at least from an early onset providing evidence for a potentially recessive pattern of inheritance in human prion diseases