脊髓性肌萎缩症(spinal muscular atrophy，SMA)本文特指5q-SMA，属常染色体隐性遗传病，临床特征为脊髓前角α运动神经元退行性病变和神经肌肉接头发育异常导致的肌无力和肌萎缩。SMA常累及呼吸系统，引起呼吸系统病理生理改变，导致低通气、咳嗽减弱、痰液堵塞等一系列呼吸问题。呼吸衰竭是SMA患儿最常见的致死原因。近年来，随着国内外SMA多学科管理专家共识的相继发表和新药上市，SMA呼吸管理策略将会转变。本共识由中国医师协会儿科医师分会及中国医师协会儿科医师分会儿童呼吸学组组织我国相关领域的专家，总结国内外指南/共识和相关临床研究，结合中国人群实际临床情况，最终达成共识，以促进SMA患儿规范呼吸管理。“,”Spinal muscular atrophy (SMA, 5q-SMA) includes a group of autosomal recessive disorders cha-racterized by degeneration of alpha motor neurons in the spinal cord and abnormalities of neuromuscular junction which can develop myasthenia and amyotrophy.The respiratory system is also involved with pathophysiological changes, resulting in a series of respiratory complications such as hypoventilation, impaired cough, poor airway clearance.Respiratory failure is the leading cause of death among children with SMA.Recently, with the advent of published standard multidisciplinary care consensuses on SMA and approved new drugs for the treatment of SMA, pulmonary management strategy of SMA will be changed.This consensus is organized by the Pediatrician Branch of Chinese Medical Doctor Association and Subspecialty Group of Respiratory Diseases, Pediatrician Branch of Chinese Medical Doctor Association to summarize domestic and international guidelines/consensuses as well as related clinical studies by relevant experts, combined with the clinical characteristics of the Chinese population, and finally reached a consensus to promote the standardized pulmonary management of SMA patients.