Stefania Corti , Valeria Sansone , Ilaria Bitetti , Noemi Brolatti , Giulio Gadaleta , Agata Katia Patanella , Giorgia Coratti , Eugenio Mercuri , on behalf of the SMAkers working group
{"title":"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","authors":"Stefania Corti , Valeria Sansone , Ilaria Bitetti , Noemi Brolatti , Giulio Gadaleta , Agata Katia Patanella , Giorgia Coratti , Eugenio Mercuri , on behalf of the SMAkers working group","doi":"10.1016/j.nmd.2025.105278","DOIUrl":null,"url":null,"abstract":"<div><div>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.</div></div>","PeriodicalId":19135,"journal":{"name":"Neuromuscular Disorders","volume":"48 ","pages":"Article 105278"},"PeriodicalIF":2.7000,"publicationDate":"2025-01-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Italian survey on evolving SMA care with disease-modifying therapies: a consensus workshop on nutrition, swallowing, respiratory and rehabilitation care\",\"authors\":\"Stefania Corti , Valeria Sansone , Ilaria Bitetti , Noemi Brolatti , Giulio Gadaleta , Agata Katia Patanella , Giorgia Coratti , Eugenio Mercuri , on behalf of the SMAkers working group\",\"doi\":\"10.1016/j.nmd.2025.105278\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<div><div>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.</div></div>\",\"PeriodicalId\":19135,\"journal\":{\"name\":\"Neuromuscular Disorders\",\"volume\":\"48 \",\"pages\":\"Article 105278\"},\"PeriodicalIF\":2.7000,\"publicationDate\":\"2025-01-27\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Neuromuscular Disorders\",\"FirstCategoryId\":\"3\",\"ListUrlMain\":\"https://www.sciencedirect.com/science/article/pii/S0960896625000057\",\"RegionNum\":4,\"RegionCategory\":\"医学\",\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q2\",\"JCRName\":\"CLINICAL NEUROLOGY\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Neuromuscular Disorders","FirstCategoryId":"3","ListUrlMain":"https://www.sciencedirect.com/science/article/pii/S0960896625000057","RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q2","JCRName":"CLINICAL NEUROLOGY","Score":null,"Total":0}
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.
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.