439 research outputs found

    El símbolo perdido: estética y pensamiento en las adaptaciones cinematográficas de obras de Antonio Buero Vallejo

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    Cuatro adaptaciones cinematográficas se han hecho de obras de Antonio Buero Vallejo, cantidad modesta si se compara con la de otros dramaturgos de su talla. En el presente artículo se analizan por primera vez estos filmes en busca de las causas que expliquen el aparente desinterés del cine hacia dicho autor. La tesis que se plantea es la de que existen dos elementos estético-filosóficos en la dramaturgia de Buero, el simbolismo y la “tragedia esperanzada”, difíciles de llevar a la gran pantalla, por lo que los adaptadores han optado siempre por mitigarlos o incluso por anularlos

    Early disease progression of Hurler syndrome

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    Abstract Background Newborn screening for mucopolysaccharidosis type I (MPS I) shows promise to improve outcomes by facilitating early diagnosis and treatment. However, diagnostic tests for MPS I are of limited value in predicting whether a child will develop severe central nervous system disease associated with Hurler syndrome, or minimal or no central nervous system involvement associated with the attenuated phenotypes (Hurler–Scheie and Scheie syndromes). Given that the optimal treatment differs between Hurler syndrome and the attenuated MPS I phenotypes, the absence of a reliable prognostic biomarker complicates clinical decision making for infants diagnosed through newborn screening. Information about the natural history of Hurler syndrome may aid in the management of affected infants, contribute to treatment decisions, and facilitate evaluation of treatment effectiveness and prognosis. Thus, the aim of this study was to characterize the progression and timing of symptom onset in infants with Hurler syndrome. Results Clinical data from 55 patients evaluated at a single center were retrospectively reviewed. Information about each child’s medical history was obtained following a standardized protocol including a thorough parent interview and the review of previous medical records. All patients underwent systematic physical and neurodevelopmental evaluations by a multidisciplinary team. Nearly all patients (98%) showed signs of disease during the first 6 months of life. Common early disease manifestations included failed newborn hearing screen, respiratory symptoms, difficulty latching, and otitis media. Other symptoms such as kyphosis, corneal clouding, cardiac disease, joint restrictions, and enlarged head circumference typically appeared slightly later (median age, 8–10 months). During the first 12 months, gross motor development was the most severely affected area of functioning, and a significant number of patients also experienced language delays. Cognition was typically preserved during this period. Conclusions In this large cohort of patients with Hurler syndrome, the vast majority showed signs and symptoms of disease during the first months of life. More research is needed to determine the extent to which early clinical manifestations of MPS I can predict phenotype and treatment outcomes

    Study of the polycarbonate-urethane/metal contact in different positions during gait cycle

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    Nowadays, a growing number of young andmore active patients receive hip replacement.More strenuous activities in such patients involve higher friction and wear rates, with friction on the bearing surface being crucial to ensure arthroplasty survival in the long term. Over the last years, the polycarbonate-urethane has offered a feasible alternative to conventional bearings. A finite element model of a healthy hip joint was developed and adjusted to three gait phases (heel strike, mid-stance, and toe-off), serving as a benchmark for the assessment of the results of joint replacement model. Three equivalent models were made with the polycarbonate-urethane Tribofit system implanted, one for each of the three gait phases, after reproducing a virtual surgery over the respective healthy models. Standard body-weight loads were considered: 230% body-weight toe-off, 275% body-weight mid-stance, and 350% body-weight heel strike. Contact pressures were obtained for the different models. When comparing the results corresponding to the healthy model to polycarbonate-urethane joint, contact areas are similar and so contact pressures are within a narrower value range. In conclusion, polycarbonate-urethane characteristics are similar to those of the joint cartilage. So, it is a favorable alternative to traditional bearing surfaces in total hip arthroplasty, especially in young patients

    New technology in Museums: AR and VR video games are coming

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    Museums have gone through a modernization process which has seen the adoption of new technologies in what they offer visitors. Within the framework of the new critical museology, these organizations have been transformed into places of encounter and experience, the key tools in this change being socialization and play. Gamification are now intrinsic to collections and are a way of inviting visitors to share new museum experiences through the latest technology such as AR (Augmented Reality) and VR (Virtual Reality). In this way, the museum becomes a playground and a space for creativity (Borja-Villel et al., 2014). In this research, we focus on what we consider to be an important link between the three central aspects of museum change: sociability, gamificaction and virtualization; and the growing interest in museums for videogames. Our aim is to reach a better understanding of the AR and VR video games developed for museums and how these technologies can not only motivate visitors’ interest but also improve their learning skills. Our analysis focuses of literature published between 2015 to 2018 and follows the analytic structure established by Connolly et al. (2012) with additional features related to learning experience, platforms, and the use of technologies (VR and AR). The general aim is to map the interest of the research community in the field of museum-developed video games, more specifically those that use augmented and virtual reality

    Risk factors for symptomatic retears after arthroscopic repair of full-thickness rotator cuff tears

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    Background: Factors affecting a rotator cuff symptomatic retear after arthroscopic repair have yet to be clearly identified, since they usually influence the surgical decisions. Methods: Consecutive patients with full-thickness tear of the supraspinatus who underwent arthroscopic repair were retrospectively analyzed. Cases of symptomatic retear, defined as Sugaya type IV and V on magnetic resonance imaging, associated with intensive pain and/or functional impairment were identified at follow-up. The patients with no symptomatic retear were selected as the control group. Information from potential risk factors of symptomatic retear, including depression and subacromial corticosteroid injections, was extracted from the medical records. The statistical analysis included multivariant logistic regression. Results: The symptomatic retear rate was 9.5% in 158 patients. Patients in the symptomatic retear group were more likely to be smoking, to have massive tears, a short acromiohumeral distance, and moderate to severe fatty infiltration. They also had had more frequently subacromial corticosteroid injections and depression. However, following the multiple logistic regression analysis, only massive tears and moderate to severe fatty infiltration remained significantly associated. Similarly, in relation to the study hypothesis, both corticosteroid injections (odds ratio [OR] 6.66, 95% confidence interval [CI] 1.49, 29.81; P =.013) and depression (OR 8.26, IC 1.04, 65.62; P =.046) were significantly associated with symptomatic retear risk. Conclusions: This study found support for the hypothesis that both depression and corticosteroid infiltration before surgery are independent risk factors for symptomatic retear after arthroscopic repair of rotator cuff

    Cementless hydroxyapatite coated hip prostheses

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    More than twenty years ago, hydroxyapatite (HA), calcium phosphate ceramics, was introduced as a coating for cementless hip prostheses. The choice of this ceramic is due to its composition being similar to organic apatite bone crystals. This ceramic is biocompatible, bioactive, and osteoconductive. These qualities facilitate the primary stability and osseointegration of implants Our surgical experience includes the implantation of more than 4,000 cementless hydroxyapatite coated hip prostheses since 1990 The models implanted are coated with HA in the acetabulum and in the metaphyseal area of the stem.The results corresponding to survival and stability of implants were very satisfactory in the long-term. From our experience, HA-coated hip implants are a reliable alternative which can achieve long termsurvival, provided that certain requirements aremet: good design selection, sound choice of bearing surfaces based on patient life expectancy,meticulous surgical technique, and indications based on adequate bone quality

    treatment of brain disease in the mucopolysaccharidoses

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    Abstract The mucopolysaccharidosis (MPS) disorders are a group of lysosomal storage diseases caused by lysosomal enzyme deficits that lead to glycosaminoglycan accumulation, affecting various tissues throughout the body based on the specific enzyme deficiency. These disorders are characterized by their progressive nature and a variety of somatic manifestations and neurological symptoms. There are established treatments for some MPS disorders, but these mostly alleviate somatic and non-neurological symptoms and do not cure the disease. Patients with MPS I, II, III, and VII can present with neurological manifestations such as neurocognitive decline and behavioral problems. Treatment of these neurological manifestations remains challenging due to the blood-brain barrier (BBB) that limits delivery of therapeutic agents to the central nervous system (CNS). New therapies that circumvent this barrier and target brain disease in MPS are currently under development. They primarily focus on facilitating penetration of drugs through the BBB, delivery of recombinant enzyme to the brain by gene therapy, or direct CNS administration. This review summarizes existing and potential future treatment approaches that target brain disease in MPS. The information in this review is based on current literature and presentations and discussions during a closed meeting by an international group of experts with extensive experience in managing and treating MPS

    Bradyrhizobium japonicum senses iron through the status of haem to regulate iron homeostasis and metabolism

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    The Irr protein from the bacterium Bradyrhizobium japonicum is expressed under iron limitation to mediate iron control of haem biosynthesis. The regulatory input to Irr is the status of haem and its precursors iron and protoporphyrin at the site of haem synthesis. Here, we show that Irr controls the expression of iron transport genes and many other iron-regulated genes not directly involved in haem synthesis. Irr is both a positive and negative effector of gene expression, and in at least some cases the control is direct. Loss of normal iron responsiveness of those genes in an irr mutant, as well as a lower total cellular iron content, suggests that Irr is required for the correct perception of the cellular iron status. Degradation of Irr in iron replete cells requires haem. Accordingly, control of Irr-regulated genes by iron was aberrant in a haem-defective strain, and iron replete mutant cells behave as if they are iron-limited. In addition, the haem mutant had an abnormally high cellular iron content. The findings indicate that B. japonicum senses iron via the status of haem biosynthesis in an Irr-dependent manner to regulate iron homeostasis and metabolism

    Gender differences in the incidence of and risk factors for hip fracture: A 16-year longitudinal study in a southern European population

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    Objectives To analyze independently in men and women the incidence rate of and risk factors for hip fracture in a southern European population. Illiteracy, dementia, clinically significant depression and disability were factors to receive special emphasis. Study design A community sample of 4803 individuals aged over 55 years was assessed in a two-phase case-finding study in Zaragoza, Spain, and was followed up for 16 years. Medical history and psychiatric history were collected with standardized instruments, including the History and Aetiology Schedule, the Geriatric Mental State (GMS) scale, and a Risk Factors Questionnaire. Operational criteria were used to define covariates, including diagnostic criteria for both dementia and depression. The statistical analysis included calculations of incidence rate, IR; women/men incidence rate ratio (IRR); and Hazard Ratios (HR) in multivariate Cox proportional hazards regression models. Main outcome measures Cases of hip fracture (International Classification of Diseases, WHO) identified in the treating hospitals, validated by blinded researchers. Results Hip fractures were more frequent among women than men (IRR = 3.1). Illiteracy (HR = 1.55) and depression (HR = 1.44) increased the risk in women, and smoking (HR = 2.13) and disability in basic activities of daily living (HR = 3.14) increased the risk in men. Dementia was associated with an increased risk in an univariate analysis, but the association disappeared (power = 85% in men, 95% in women) when disability was included in the multivariate models. Conclusions The IR of hip fractures was three times higher among women. Illiteracy and clinically significant depression among women and active smoking and disability (HR = 3.14) among men independently increased the risk, but dementia did not

    Improved Brain Pathology and Progressive Peripheral Neuropathy in a 15 Year Old Survivor of Infantile Krabbe Disease Treated With Umbilical Cord Transplantation

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    ObjectiveKrabbe disease is a fatal leukodystrophy caused by deficiency in galactocerebrosidase enzyme activity. The only currently available therapy is hematopoietic stem cell transplantation with bone marrow or umbilical cord blood (UCBT), which leads to increased lifespan and functional abilities when performed in the preclinical stage. While stabilization of white matter disease has been seen on serial MRI studies, neuropathological changes following transplantation have not been documented so far.Materials and MethodsWe report the first postmortem examination of a 15-year-old female patient with infantile Krabbe disease after UCBT in infancy.ResultsIn contrast to an untreated Krabbe disease brain, which showed severe myelin and oligodendrocyte loss with occasional globoid cells, the transplanted brain displayed markedly improved myelin preservation, but not reaching normal myelination levels. Consistent with the transplanted patient’s clinical presentation of pronounced deficits in gross motor skills, corticospinal tracts were most severely affected. No globoid cells or evidence of active demyelination were observed in the central nervous system, indicative of at least partially successful functional restoration. This was corroborated by the identification of male donor-derived cells in the brain by in situ hybridization. Unlike the observed disease stabilization in the central nervous system, the patient experienced progressive peripheral neuropathy. While diminished macrophage infiltration was seen postmortem, peripheral nerves exhibited edema, myelin and axon loss and persistent Schwann cell ultrastructural inclusions.ConclusionUmbilical cord blood transplantation was able to alter the natural disease progression in the central but less so in the peripheral nervous system, possibly due to limited cross-correction of Schwann cells
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