意大利关于使用疾病改变疗法不断发展的 SMA 护理的调查:营养、吞咽、呼吸和康复护理共识研讨会

IF 2.7 4区 医学 Q2 CLINICAL NEUROLOGY
Stefania Corti , Valeria Sansone , Ilaria Bitetti , Noemi Brolatti , Giulio Gadaleta , Agata Katia Patanella , Giorgia Coratti , Eugenio Mercuri , on behalf of the SMAkers working group
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引用次数: 0

摘要

随着疾病改良疗法和新生儿筛查计划的出现,脊髓性肌萎缩症的治疗环境也在不断变化,这导致了新的表型和不断变化的患者需求。因此,有必要重新评估现有的护理建议,以应对脊髓性肌萎缩症治疗中的新挑战和新机遇。为了更好地评估自疾病改变疗法出现以来不同护理领域的发展情况,我们重点强调了现有做法仍然有效的领域、需要改变并有证据支持的领域以及需要进一步研究或经验的领域。该倡议汇集了意大利脊髓性肌肉萎缩症社区的专家,最初包括一项护理实践调查和为期两天的特定主题研讨会。调查和焦点小组的结果提供了专家们对关键领域护理方面变化的看法:营养和骨骼健康、吞咽、呼吸功能以及脊柱畸形的康复和管理。专家们提出了建议,确定了可立即行动的改变和需要进一步调查的领域。该框架是在新疗法时代更新护理建议的第一步。它对不断变化的护理环境提出了批判性的观点,平衡了既有实践与新出现的证据,并确定了未来研究的关键领域。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Italian survey on evolving SMA care with disease-modifying therapies: a consensus workshop on nutrition, swallowing, respiratory and rehabilitation care
The landscape of spinal muscular atrophy care is evolving with the emergence of disease-modifying therapies and newborn screening programs, leading to new phenotypes and changing patient needs. This necessitates a reevaluation of existing care recommendations to address new challenges and opportunities in spinal muscular atrophy management. To better evaluate how different areas of care have evolved since the advent of disease-modifying therapies, we focused on highlighting areas where existing practices remain valid, those in which changes are needed and supported by evidence, and those in which further research or experience is required. The initiative brought together experts from the Italian spinal muscular atrophy community, initially involving a survey of care practices and a two-day event with workshops on specific topics. The results of the survey and focus groups provide the experts' opinions of the changing aspects of care for key domains: nutrition and bone health, swallowing, respiratory function, and rehabilitation and management of spine deformities. Suggestions were provided, identifying immediate actionable changes and areas requiring further investigation. This framework represents an initial step towards updating care recommendations in the era of novel therapies. It offers a critical view of the evolving care landscape, balancing established practices with emerging evidence and identifying key areas for future research.
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来源期刊
Neuromuscular Disorders
Neuromuscular Disorders 医学-临床神经学
CiteScore
4.60
自引率
3.60%
发文量
543
审稿时长
53 days
期刊介绍: This international, multidisciplinary journal covers all aspects of neuromuscular disorders in childhood and adult life (including the muscular dystrophies, spinal muscular atrophies, hereditary neuropathies, congenital myopathies, myasthenias, myotonic syndromes, metabolic myopathies and inflammatory myopathies). The Editors welcome original articles from all areas of the field: • Clinical aspects, such as new clinical entities, case studies of interest, treatment, management and rehabilitation (including biomechanics, orthotic design and surgery). • Basic scientific studies of relevance to the clinical syndromes, including advances in the fields of molecular biology and genetics. • Studies of animal models relevant to the human diseases. The journal is aimed at a wide range of clinicians, pathologists, associated paramedical professionals and clinical and basic scientists with an interest in the study of neuromuscular disorders.
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