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141.

Background  

Tools for predicting post-ICU patients’ outcomes are scarce. A single-center study showed that the Sabadell score classified patients into four groups with clear-cut differences in ward mortality.  相似文献   
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Introduction: Congenital central hypoventilation syndrome (CCHS) is a rare genetic disorder due to paired-like homeobox gene (PHOX2B) mutations. CCHS patients suffer from dysregulation of the autonomic nervous system characterized by the absence of or extremely reduced response to hypercapnia and hypoxia, with neuropsychological deficits. The aim of this exploratory study is to describe the longitudinal neuropsychological profile and its correlations with magnetic resonance imaging (MRI) of a child with CCHS with a PHOX2B mutation. Method: A comprehensive neuropsychological evaluation was conducted serially at age 7 years 4 months and 10 years 3 months, including assessment of intellectual functioning (IQ), motor functioning, perception, attention, executive functions, language, memory, social cognition, academic skills, and psychopathology. Reliable change index (RCI) scores were used to assess changes between assessments. We collected spin lattice relaxation time (T1)-weighted, fluid-attenuated inversion recovery (FLAIR), and spin spin lattice relaxation time (T2)-weighted images from the child at age 10 years 3 months using a 1.5-tesla MRI scanner. Results: IQ, processing speed index (PSI), social cognition (theory of mind and facial emotion recognition), selective attention, naming, academic skills (reading/comprehension), and manual speed with right hand declined in the second evaluation relative to the initial evaluation, while visuoconstructional praxis, receptive vocabulary, working memory, and arithmetic skill improved. The patient showed a remarkable global deterioration in executive functions (planning, task flexibility, behavioral regulation, and metacognition) as revealed by parental report and clinical evaluation. MRI revealed gliosis from the head to tail of the hippocampus and thinning of parahippocampal gyri. Conclusions: In a clinical case of CCHS, serial evaluation revealed deterioration of executive functions and social cognition over a 3-year interval. These changes corresponded to hippocampal damage as revealed in MRI, which may have affected social cognition through its role in the default mode network. Serial neuropsychological assessment is clinically useful in managing the needs of these patients.  相似文献   
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The objective was to evaluate the effects of a stress management cognitive behavioural therapy followed during pregnancy on subsequent childhood on hair cortisol at birth and on neurodevelopment and Hair Cortisol Concentrations (HCC) at 6 months of age. The study sample included 48 pregnant women, divided into two groups: 24 women in the Therapy Group (TG) and 24 women who received standard pregnancy care (control group (CG); CG). To test the therapy efficacy, an evaluation of the HCC and psychological stress, psychopathological symptomatology and resilience was conducted before and after the treatment. The level of cortisol in their hair was obtained during pregnancy and that of their babies at birth. Six months after birth, a cortisol sample was taken from the hair and the babies' neurodevelopment was evaluated based on a Bayley-III test. The TG presented reductions in psychological stress and psychopathological symptomatology after treatment. On the other hand, the CG increased their cortisol concentrations between the pre and post intervention, remaining stable in the TG. Moreover, results showed that TG babies had lower cortisol concentrations at birth and obtained significantly higher cognitive and motor development scores at 6 months. These findings support that providing psychological care to pregnant women may not only have a benefit on these women's mental state, but may also benefit the neurodevelopment of their offspring.  相似文献   
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The administration of the selective β3 adrenergic receptor (β3AR) agonist BRL-37344 protects from myocardial ischemia/reperfusion injury (IRI), although the lack of clinical approval limits its translatability. We tested the cardioprotective effect of mirabegron, the first-in-class β3AR agonist approved for human use. A dose-response study was conducted in 6 pigs to select the highest intravenous dose of mirabegron without significant detrimental hemodynamic effect. Subsequently, closed chest anterior myocardial infarction (45 min ischemia followed by reperfusion) was performed in 26 pigs which randomly received either mirabegron (10 μg/kg) or placebo 5 min before reperfusion. Day-7 cardiac magnetic resonance (CMR) showed no differences in infarct size (35.0?±?2.0% of left ventricle (LV) vs. 35.9?±?2.4% in mirabegron and placebo respectively, p?=?0.782) or LV ejection fraction (36.3?±?1.1 vs. 34.6?±?1.9%, p?=?0.430). Consistent results were obtained on day-45 CMR. In conclusion, the intravenous administration of the clinically available selective β3AR agonist mirabegron does not reduce infarct size in a swine model of IRI.  相似文献   
149.
Sickle cell anaemia is still responsible for severe crippling and death in young patients living in developing countries. Apart from prophylaxis and treatment of infections, no active treatment can be safely proposed in such areas of the world. Therefore a bone marrow transplantation was performed in 12 patients staying in Belgium and planning to return to Africa. Twelve patients, aged between 11 months and 23 years (median 4 years), underwent a HLA identical bone marrow transplantation. The conditioning regimen included oral busulphan for four consecutive days (4 mg/kg) followed by four days of intravenous cyclophosphamide (50 mg/kg). In 10 patients the engraftment was rapid and sustained. A further patient suffered transient red cell hypoplasia and another underwent a second bone marrow transplantation from the same donor at day 62 because of graft rejection. All patients are alive and well with a follow up ranging from 9-51 months (median 27 months). In all cases a complete cessation of vaso-occlusive episodes and haemolysis was observed as was a change in the haemoglobin pattern in accordance with the donor''s electrophoretic pattern.  相似文献   
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With increasing availability of large datasets derived from administrative and other sources, there is an increasing demand for the successful linking of these to provide rich sources of data for further analysis. Variation in the quality of identifiers used to carry out linkage means that existing approaches are often based upon ‘probabilistic’ models, which are based on a number of assumptions, and can make heavy computational demands. In this paper, we suggest a new approach to classifying record pairs in linkage, based upon weights (scores) derived using a scaling algorithm. The proposed method does not rely on training data, is computationally fast, requires only moderate amounts of storage and has intuitive appeal. Copyright © 2017 John Wiley & Sons, Ltd.  相似文献   
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