The Hospital for Sick Children, University of Toronto, Canada.
Neurology. 2012 Jun 5;78(23):e143-5. doi: 10.1212/WNL.0b013e318258f835.
Spinal muscular atrophy (SMA) is a group of progressive and fatal neurodegenerative disorders that are characterized by destruction of the anterior horn cells of the spinal cord. In this case report we outline the medical and ethical issues involved in a 7-year-old boy with SMA type 2 who experienced acute respiratory failure.
A review of the literature was conducted focusing particularly on the pathology, presentation, and outcomes of SMA and end-of-life decision-making in pediatrics.
In a world where 40%-60% of deaths in pediatric intensive care units are a result of withdrawal or limitation of life-sustaining treatment, end-of-life decision-making has become an integral and difficult part of pediatric practice.
Limitation or withdrawal of life-sustaining treatment in a cognitively normal child with SMA poses a significant medical and ethical dilemma. This difficult decision is influenced by confluence of parental, doctor, social, cultural, moral, religious, legal, and economic factors and more recently the media.
脊髓性肌萎缩症（SMA）是一组进行性和致命的神经退行性疾病，其特征是脊髓前角细胞的破坏。在本病例报告中，我们概述了一名 7 岁 SMA 2 型男孩急性呼吸衰竭所涉及的医学和伦理问题。
我们对文献进行了回顾，特别关注 SMA 的病理学、表现和结局，以及儿科临终决策。
在儿童重症监护病房中，40%-60%的死亡是由于停止或限制维持生命的治疗，因此临终决策已成为儿科实践的一个重要且困难的部分。
在认知正常的 SMA 儿童中限制或停止维持生命的治疗会带来重大的医学和伦理困境。这一艰难的决策受到父母、医生、社会、文化、道德、宗教、法律和经济因素的共同影响，最近还受到媒体的影响。
