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Health care is uncertain, dynamic, and fast growing. With digital technologies set to revolutionise the industry, hospital capacity optimisation and planning have never been more relevant. The purposes of this article are threefold. The first is to identify the current state of the art, to summarise/analyse the key achievements, and to identify gaps in the body of research. The second is to synthesise and evaluate that literature to create a holistic framework for understanding hospital capacity planning and optimisation, in terms of physical elements, process, and governance. Third, avenues for future research are sought to inform researchers and practitioners where they should best concentrate their efforts. In conclusion, we find that prior research has typically focussed on individual parts, but the hospital is one body that is made up of many interdependent parts. It is also evident that past attempts considering entire hospitals fail to incorporate all the detail that is necessary to provide solutions that can be implemented in the real world, across strategic, tactical and operational planning horizons. A holistic approach is needed that includes ancillary services, equipment medicines, utilities, instrument trays, supply chain and inventory considerations.
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OBJECTIVES: We developed clinical guidelines for the management of bone health in Rett syndrome through evidence review and the consensus of an expert panel of clinicians. METHODS: An initial guidelines draft was created which included statements based upon literature review and 11 open-ended questions where literature was lacking. The international expert panel reviewed the draft online using a 2-stage Delphi process to reach consensus agreement. Items describe the clinical assessment of bone health, bone mineral density assessment and technique, and pharmacological and non-pharmacological interventions. RESULTS: Agreement was reached on 39 statements which were formulated from 41 statements and 11 questions. When assessing bone health in Rett syndrome a comprehensive assessment of fracture history, mutation type, prescribed medication, pubertal development, mobility level, dietary intake and biochemical bone markers is recommended. A baseline densitometry assessment should be performed with accommodations made for size, with the frequency of surveillance determined according to individual risk. Lateral spine x-rays are also suggested. Increasing physical activity and initiating calcium and vitamin D supplementation when low are the first approaches to optimizing bone health in Rett syndrome. If individuals with Rett syndrome meet the ISCD criterion for osteoporosis in children, the use of bisphosphonates is recommended. CONCLUSION: A clinically significant history of fracture in combination with low bone densitometry findings is necessary for a diagnosis of osteoporosis. These evidence and consensus-based guidelines have the potential to improve bone health in those with Rett syndrome, reduce the frequency of fractures, and stimulate further research that aims to ameliorate the impacts of this serious comorbidity.
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Osteoporosis/diagnóstico , Osteoporosis/terapia , Guías de Práctica Clínica como Asunto , Síndrome de Rett/complicaciones , Absorciometría de Fotón , Densidad Ósea , Conservadores de la Densidad Ósea/uso terapéutico , Consenso , Difosfonatos/uso terapéutico , Manejo de la Enfermedad , Testimonio de Experto , Humanos , Osteoporosis/etiologíaRESUMEN
Anti-GQ1b ganglioside antibodies are the serological hallmark of the Miller Fisher syndrome (MFS) variant of the paralytic neuropathy, Guillain-Barré syndrome, and are believed to be the principal pathogenic mediators of the disease. In support of this, we previously showed in an in vitro mouse model of MFS that anti-GQ1b antibodies were able to bind and disrupt presynaptic motor nerve terminals at the neuromuscular junction (NMJ) as one of their target sites, thereby causing muscle paralysis. This injury only occurred through activation of complement, culminating in the formation and deposition of membrane attack complex (MAC, C5b-9) in nerve membranes. Since this step is crucial to the neuropathic process and an important convergence point for antibody and complement mediated membrane injury in general, it forms an attractive pharmacotherapeutic target. Here, we assessed the efficacy of the humanized monoclonal antibody eculizumab, which blocks the formation of human C5a and C5b-9, in preventing the immune-mediated motor neuropathy exemplified in this model. Eculizumab completely prevented electrophysiological and structural lesions at anti-GQ1b antibody pre-incubated NMJs in vitro when using normal human serum (NHS) as a complement source. In a novel in vivo mouse model of MFS generated through intraperitoneal injection of anti-GQ1b antibody and NHS, mice developed respiratory paralysis due to transmission block at diaphragm NMJs, resulting from anti-GQ1b antibody binding and complement activation. Intravenous injection of eculizumab effectively prevented respiratory paralysis and associated functional and morphological hallmarks of terminal motor neuropathy. We show that eculizumab protects against complement-mediated damage in murine MFS, providing the rationale for undertaking clinical trials in this disease and other antibody-mediated neuropathies in which complement activation is believed to be involved.
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Anticuerpos Monoclonales/uso terapéutico , Síndrome de Miller Fisher/prevención & control , Enfermedad Autoinmune Experimental del Sistema Nervioso/prevención & control , Animales , Anticuerpos Monoclonales Humanizados , Activación de Complemento/inmunología , Modelos Animales de Enfermedad , Evaluación Preclínica de Medicamentos/métodos , Gangliósidos/inmunología , Masculino , Ratones , Ratones Endogámicos BALB C , Síndrome de Miller Fisher/inmunología , Síndrome de Miller Fisher/fisiopatología , Contracción Muscular , Enfermedad Autoinmune Experimental del Sistema Nervioso/inmunología , Enfermedad Autoinmune Experimental del Sistema Nervioso/fisiopatología , Unión Neuromuscular/inmunología , Unión Neuromuscular/fisiopatología , Parálisis Respiratoria/inmunología , Parálisis Respiratoria/fisiopatología , Parálisis Respiratoria/prevención & control , Sinapsis/ultraestructura , Técnicas de Cultivo de TejidosRESUMEN
Migraine headaches are frequent in adolescents. Although many adolescents are adequately treated palliatively with analgesics, an important subgroup requires prophylactic treatment. Medical treatments for adolescents with frequent severe headaches is often problematic. Prophylactic pharmacological treatments are often shunned by adolescents and their parents because of concern over drug usage. Moreover, propranolol, the most widely used prophylactic drug with adults, is frequently not effective. Psychological interventions are effective but are costly and often not available. A randomized controlled trial was undertaken to evaluate the efficacy and efficiency of a predominantly self-administered treatment that could be delivered in a very cost-efficient format. Eighty seven adolescents (63 females and 24 males) ranging in age from 11 to 18 years were randomly assigned to receive a self-administered treatment, the same treatment delivered by a therapist or a control treatment. Self-administered and clinic treatment were equally effective and superior to the control treatment. However, the self-administered treatment was substantially more efficient. Both active treatments were durable at 1-year follow-up.
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Terapia Cognitivo-Conductual , Trastornos Migrañosos/terapia , Adaptación Psicológica , Adolescente , Niño , Depresión/psicología , Femenino , Humanos , Masculino , Trastornos Migrañosos/psicología , Terapia por RelajaciónRESUMEN
The present study compared the efficacy of two active treatments, relaxation training and cognitive coping, with a non-specific placebo control in the treatment of 42 children and adolescents with migraine. The first treatment is a simplified version of progressive deep muscle relaxation; the second, a form of cognitive restructuring involving the alteration of dysfunctional thought processes. The results demonstrated that each active treatment was superior to the non-specific intervention in reducing overall headache activity and frequency but not duration or intensity. There were no differences between the experimental groups, and both continued to improve through a 16-week follow-up period, but the control group did not. Initial level of headache severity was an important factor in treatment outcome, with children with severe headaches responding better than those with milder headaches. Possible reasons for the differential treatment effects are discussed, and the implications for future research are considered.