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71.

Background

Children with neuromuscular disorders with a progressive muscle weakness such as Duchenne Muscular Dystrophy and Spinal Muscular Atrophy frequently develop a progressive scoliosis. A severe scoliosis compromises respiratory function and makes sitting more difficult. Spinal surgery is considered the primary treatment option for correcting severe scoliosis in neuromuscular disorders. Surgery in this population requires a multidisciplinary approach, careful planning, dedicated surgical procedures, and specialized after care.

Methods

The guideline is based on scientific evidence and expert opinions. A multidisciplinary working group representing experts from all relevant specialties performed the research. A literature search was conducted to collect scientific evidence in answer to specific questions posed by the working group. Literature was classified according to the level of evidence.

Results

For most aspects of the treatment scientific evidence is scarce and only low level cohort studies were found. Nevertheless, a high degree of consensus was reached about the management of patients with scoliosis in neuromuscular disorders. This was translated into a set of recommendations, which are now officially accepted as a general guideline in the Netherlands.

Conclusion

In order to optimize the treatment for scoliosis in neuromuscular disorders a Dutch guideline has been composed. This evidence-based, multidisciplinary guideline addresses conservative treatment, the preoperative, perioperative, and postoperative care of scoliosis in neuromuscular disorders.  相似文献   
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ABSTRACT There are many challenges facing natural resources programs in North American higher education today. Pressures exerted by a new generation of students, changing workplace requirements (including undergraduate core-knowledge requirements), and an increasingly specialized professoriate are great but not insurmountable. We discuss each of these issues and pose potential solutions to address each including adopting new pedagogical techniques for content delivery (e.g., adapting courses to be inclusive of new technologies), revising curriculum to meet the needs of a new suite of learners (e.g., developing curricula that allow structured flexibility of choices, designing a core curriculum that is a mix of single-discipline courses and courses that integrate across disciplines), and new strategies for faculty engagement in discipline-specific survey courses. By remaining deliberate and effective in our pursuit of quality higher education we have the opportunity to ensure we are delivering the best possible education to the future professionals of our disciplines.  相似文献   
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Neural cells secrete diverse molecules, which accumulate in the extracellular space and form the extracellular matrix (ECM). Interactions between cells and the ECM are well recognized to play the crucial role in cell migration and guidance of growing axons, whereas formation of mature neural ECM in the form of perineuronal nets is believed to restrict certain forms of developmental plasticity. On the other hand, major components of perineuronal nets and other ECM molecules support induction of functional plasticity, the most studied form of which is long-term potentiation. Here, we review the underlying mechanisms by which ECM molecules, their receptors and remodeling proteases regulate the induction and maintenance of synaptic modifications. In particular, we highlight that activity-dependent secretion and activation of proteases leads to a local cleavage of the ECM and release of signaling proteolytic fragments. These molecules regulate transmitter receptor trafficking, actin cytoskeleton, growth of dendritic spines, and formation of dendritic filopodia.  相似文献   
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Summary For inhibition of DNA synthesis an antibiotic, edeine, acting specifically on DNA replication, was used. The inhibition of DNA synthesis in F cells caused only small decrease (three to four-fold) in recombination frequency. On the other hand a full inhibition of DNA synthesis in Hfr cells affected the recombination to the high extent, lowering its frequency 20–40 fold, at the same time lowering to the similar degree chromosome transfer (measured by zygotic induction frequency). However, the partial inhibition of DNA synthesis in Hfr cells, amounting to about 10 per cent of the control, permitted normal chromosome transfer and normal level of recombination. The results do not agree with Jacob and Brenner's model of chromosome transfer, yet they do not unequivocally confirm Bouck and Adelberg's model. It is possible that the limited DNA synthesis is necessary for other processes, and not for completing of the replication round. The results do not exclude also, that some residual DNA synthesis in female cells is of importance in mating.  相似文献   
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