Diagnosis and management of spinal muscular atrophy: Part 2: Pulmonary and acute care; medications, supplements and immunizations; other organ systems; and ethics
Diagnosis and management of spinal muscular atrophy: Part 2: Pulmonary and acute care; medications, supplements and immunizations; other organ systems; and ethics
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DOI：
10.1016/j.nmd.2017.11.004
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发表时间：
2018-03-01
影响因子：
2.8
通讯作者：
Sejersen, Thomas
中科院分区：
文献类型：
--
作者：
Finkel, Richard S.;Mercuri, Eugenio;Sejersen, Thomas
这是2007年发布的脊髓性肌萎缩症护理标准更新建议的两部分文件的第二部分。这部分包括对肺部管理和急性护理问题的最新建议，以及过去几年出现的主题，如严重形式的脊髓性肌萎缩症的其他器官参与和药物的作用。伦理问题和姑息治疗与支持性治疗的选择也得到了解决。鉴于最近的临床试验和商业化治疗可能改变这种疾病的生存和自然史的前景，这些建议变得越来越相关。(C)2017由Elsevier B.V.出版
This is the second half of a two-part document updating the standard of care recommendations for spinal muscular atrophy published in 2007. This part includes updated recommendations on pulmonary management and acute care issues, and topics that have emerged in the last few years such as other organ involvement in the severe forms of spinal muscular atrophy and the role of medications. Ethical issues and the choice of palliative versus supportive care are also addressed. These recommendations are becoming increasingly relevant given recent clinical trials and the prospect that commercially available therapies will likely change the survival and natural history of this disease. (C) 2017 Published by Elsevier B.V.