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Marfan syndrome is a rare connective tissue disorder secondary to mutations in the FBN1 gene, characterised by skeletal, ocular, and cardiovascular manifestations. We present an extensive cohort of paediatric patients with Marfan syndrome highlighting the vital importance of promptly referring paediatric first-degree relatives of Marfan syndrome paediatric patients to a tertiary hospital as our results confirm that they are at higher risk of aortic dilatation.
According to Plutarch (Sert. 7.1–2) and Sallust (Hist. 1.83–4), a certain P. Calpurnius Lanarius killed L. Livius Salinator, a member of Sertorius’ staff in charge of military operations in the Pyrenees, in the early stages of the Sertorian War (82–72 b.c.). Through the analysis of the verb δολοφονέω in Plutarch's and St. Jerome's use of Sallust's Histories, this article seeks to demonstrate that Lanarius was an exile of the Sullan regime who treacherously assassinated his superior Salinator. The article puts forward the suggestion—not hitherto considered—that Lanarius was one of those proscribed.
One of the markers of the Late Pleistocene is highly fluctuating climatic conditions, with the Last Glacial Maximum (LGM, 26.5–19 ka cal before present (BP)) known to be one of the coldest periods. This work explores how the environment of north-eastern Iberia changed in relation to global climatic changes experienced during the Late Pleistocene, specifically around the LGM. Small mammal assemblages from Cudó cave (Tarragona, Spain) were used considering their well-known reliability for palaeoenvironmental reconstructions. Based on the taxonomic identification and the taphonomic analysis, several methodologies covering both qualitative and quantitative approaches were used to obtain the palaeoenvironmental information corresponding to level 107 and level 105 of Cudó cave (31.2–24.4 and 15.5–10.2 ka cal BP, respectively). The taphonomic results obtained point out owls (category 3) as the main accumulator of the small mammals. The palaeoenvironmental reconstruction shows that both levels experienced colder (−7.2oC/–4.4 °C) and wetter (+848 mm/ + 586 mm) climatic conditions than nowadays. However, in level 107 the environment was dominated by mid-European species and rocky landscape, while in level 105 it was dominated by Mediterranean species and woodland habitat. These conditions are consistent with the trend in north-eastern Iberia following several climatic events before and after the LGM coinciding with the period of Cudó cave assemblages.
This systematic literature review aimed to provide an overview of the characteristics and methods used in studies applying the disability-adjusted life years (DALY) concept for infectious diseases within European Union (EU)/European Economic Area (EEA)/European Free Trade Association (EFTA) countries and the United Kingdom. Electronic databases and grey literature were searched for articles reporting the assessment of DALY and its components. We considered studies in which researchers performed DALY calculations using primary epidemiological data input sources. We screened 3053 studies of which 2948 were excluded and 105 studies met our inclusion criteria. Of these studies, 22 were multi-country and 83 were single-country studies, of which 46 were from the Netherlands. Food- and water-borne diseases were the most frequently studied infectious diseases. Between 2015 and 2022, the number of burden of infectious disease studies was 1.6 times higher compared to that published between 2000 and 2014. Almost all studies (97%) estimated DALYs based on the incidence- and pathogen-based approach and without social weighting functions; however, there was less methodological consensus with regards to the disability weights and life tables that were applied. The number of burden of infectious disease studies undertaken across Europe has increased over time. Development and use of guidelines will promote performing burden of infectious disease studies and facilitate comparability of the results.
Empirical and theoretical advances and application to society are moved at different speed. Application work is frequently developed later because it requires the integration of knowledge from different research areas. In the present paper, we integrate literature coming from diverse areas of research in order to design a deductive reasoning intervention, based on the involved executive functions. Executive functions include working memory (WM)’s online executive processes and other off-line functions such as task revising and planning. Deductive reasoning is a sequential thinking process driven by reasoners’ meta-deductive knowledge and goals that requires the construction and manipulation of representations. We present a new theoretical view about the relationship between executive function and higher-level thinking, a critical analysis of the possibilities and limitations of cognitive training, and a metacognitive training procedure on executive functions to improve deductive reasoning. This procedure integrates direct instruction on deduction and meta-deductive concepts (consistency, necessity) and strategies (search for counterexamples and exhaustivity), together with the simultaneous training of WM and executive functions involved: Focus and switch attention, update WM representations, inhibit and revise intuitive responses, and control the emotional stress yielded by tasks. Likewise, it includes direct training of some complex WM tasks that demands people to carry out similar cognitive assignment than deduction. Our training program would be included in the school curriculum and attempts not only to improve deductive reasoning in experimental tasks, but also to increase students’ ability to uncover fallacies in discourse, to automatize some basic logical skills, and to be able to use logical intuitions.
To date, a large number of functional magnetic resonance imaging (fMRI) studies have been conducted on psychosis. However, little is known about changes in brain functioning in psychotic patients using an emotional auditory paradigm at different stages of the disease. Such knowledge is important for advancing our understanding of the disorder and thus creating more targeted interventions. This study aimed to investigate whether individuals with first-episode psychosis (FEP) and chronic schizophrenia show abnormal brain responses to emotional auditory processing and to compare the responses between FEP and chronic schizophrenia.
Patients with FEP (n = 31) or chronic schizophrenia (n = 23) and healthy controls (HCs, n = 31) underwent an fMRI scan while presented with both emotional and nonemotional words.
Using HC as a reference, patients with FEP showed decreased right temporal activation, while patients with chronic schizophrenia showed increased bilateral temporal activation. When comparing the patient groups, individuals with FEP showed lower frontal lobe activation.
To the best of our knowledge, this is the first study with an emotional auditory paradigm used in psychotic patients at different stages of the disease. Our results suggested that the temporal lobe might be a key issue in the physiopathology of psychosis, although abnormal activation could also be derived from a connectivity problem. There is lower activation in the early stage and evolution to greater activation when patients become chronic. This study highlights the relevance of using emotional paradigms to better understand brain activation at different stages of psychosis.
I argue for a novel interpretation of Leibniz’s conception of the kind of contingency that matters for freedom, which I label ‘agential contingency.’ In brief, an agent is free to the extent that she determines herself to do what she judges to be the best of several considered options that she could have brought about had she concluded that these options were best. I use this novel interpretation to make sense of Leibniz’s doctrine that the reasons that explain free actions are merely inclining and not necessitating.
This appendix collects a series of analytical methods that are needed in various parts of the book. All the tools are oriented toward the diagonalization of Hamiltonian, including some cases that allow a complete analytical diagonalization (e.g., coupled resonators) as well as perturbation theory methods for cases where the fully interacting model is too complex for an exact solution.
We introduce the notion of qubit as unit of quantum information, illustrating how this notion can be implemented in nonlinear superconducting circuits via the charge and current degrees of freedom. Within these two types of qubits, we discuss the charge qubit, the transmon, and the flux qubit, illustrating the nature of the states that implement the qubit subspace and how they can be controlled and measured. We discuss how qubits can interact with each other directly or through mediators, illustrating different limits of interaction, introducing the notion of dipolar electric and magnetic moments, and demonstrating the tunability of interactions by different means. The chapter closes with a brief study of qubit coherence along the history of this field, with an outlook to potential near-term improvements.
Almost all superconducting quantum technologies are built using a combination of qubits and microwave resonators. In this chapter, we develop the theory to study coherent qubit–photon interaction in such devices. We start with the equivalent of an atom in free space, studying a qubit in an open waveguide. We develop the spin-boson Hamiltonian, with specific methods to solve its dynamics in the limits of few excitations. Using these tools, we can study how an excited qubit can relax to the ground state, producing a photon, and how a propagating photon can interact with a qubit. We then move to closed environments where the photons are confined in cavities or resonators, developing the theory of cavity-QED. Using this theory, we study the Purcell enhancement of interactions, the Jaynes–Cummings model, Rabi oscillations, and vacuum Rabi splitting. We close the chapter illustrating some limits in which cavities can be used to control and measure qubits.
This chapter studies linear circuits built from capacitors, inductors, and waveguides. It shows how the excitations of these circuits are quantized and can be described as collections of quantum harmonic oscillators. It discusses the quantum states and quantum operations that are accessible by means of these circuits and external microwave drives. We show how to create coherent states, how microwave resonators decay and decohere, how to amplify and measure the quantum state of a resonator, and what states (e.g., Fock states, individual photons) require other, non-Gaussian means to be produced and detected.
Half of Guillain–Barré syndrome (GBS) present elevated cerebrospinal fluid (CSF) protein levels within 1 week since symptom onset and 80% within 2 weeks. Our objective was to determine the clinical and prognostic implication of albuminocytological dissociation in early GBS.
An ambispective cohort study was conducted. Good outcome was considered if the patient was able to walk unaided (Guillain-Barré disability score [GDS] ≤ 2 points) at 3-month follow-up. Patients were classified into two groups: with and without albuminocytological dissociation; we compared clinical and paraclinic characteristics between the groups. We analyzed clinical and electrophysiological factors related to presenting early dissociation through a multivariate model.
We included 240 patients who fulfilled Asbury criteria for GBS. On further selection, only 94 patients fulfilled inclusion. Mean age was 45.94 ± 17.1 years and 67% were male. Median time from symptom onset to admission was 5 days (IQR 3–6). Regarding albuminocytological dissociation and electrophysiological variants, we found a significant difference: acute inflammatory demyelinating polyneuropathy (AIDP) [60.6% vs 26.2%, p = 0.002], acute motor axonal neuropathy (AMAN) [21.2% vs 49.1%, p = 0.009] and acute motor sensory axonal neuropathy (AMSAN) [12.1% vs 1.6%, p = 0.05]. We did not observe significant differences in recovery of independent walking in short term between both groups. The presence of conduction block in any variant (OR 3.21, 95% CI 1.12–9.16, p = 0.02) and absence of sural registration (OR 5.69, 95% CI 1.48–21.83, p = 0.011) were independent factors related to early dissociation.
Early dissociation (<7 days) is not associated with any particular clinical feature or unfavorable outcome. It is more common to see in AIDP rather than axonal variants.
This appendix provide a self-contained presentation of the open systems and quantum optics methods used in other parts of the book (e.g., studying the relaxation of a microwave cavity or a qubit, the driving of a quantum amplifier, etc.). Half of the appendix is devoted to the derivation of master equations for small systems that are in contact with Markovian environments. The other half of the appendix is devoted to the development of an alternative input–output description of how those systems absorb information from the environment and reflect it back.