目的:评价脊髓性肌萎缩症(spinal muscular atrophy，SMA)患儿的营养状况，为SMA的多学科管理提供理论依据。方法:采用横断面调查，选择就诊于浙江大学医学院附属儿童医院多学科诊治团队的SMA患儿，收集患儿病史、体格测量和实验室检查数据、膳食摄入情况、人体成分数据和骨密度值等资料并分析。结果:共纳入29例SMA患儿，消瘦、生长迟缓和肥胖的发生率分别为27.6%(8/29例)、10.3%(3/29例)和3.4%(1/29例)。13例使用双能X线吸收测定法检查，10例(76.9%)出现骨密度低下；与人群标准化值相比，SMA患儿脂肪百分数增加，而瘦体重指数减少(n P<0.05)；与6例消瘦患儿相比，7例无消瘦患儿脂肪百分数和脂肪指数更高(n P<0.05)。12例完成3 d膳食调查，仅2例(16.7%)能量摄入达到中国居民膳食营养素参考摄入量；钙、镁、铁、锌和维生素D摄入不足分别为83.3%、75.0%、41.7%、41.7%和83.3%。n 结论:SMA患儿存在营养不良，并伴有全身脂肪量增加、瘦体重减少、骨密度低下、能量及多种营养素摄入不足现象，开展全面的营养评价对SMA的多学科管理具有一定的临床意义。“,”Objective:To assess the nutritional status of children with spinal muscular atrophy (SMA) and to provide evidence for multidisciplinary management.Methods:A cross-sectional survey was conducted on children with SMA who were treated in the multidisciplinary team of the Children's Hospital, Zhejiang University School of Medicine. The medical history, physical measurement data, laboratory examination, dietary intake data were collected, body composition and bone mineral density were measured by dual energy X-ray absorptiometry (DXA).Results:A total of 29 SMA children were selected and the incidence of wasting, stunting and obesity were 27.6%(8/29), 10.3%(3/29) and 3.4%(1/29). 13 children completed DXA examination, with 10 patients (76.9%) of low bone mineral density. The percentage of fat mass (FM%) was higher and the lean body mass index was lower in children with SMA compared to population normalized values(n P<0.05). Six of the 13 children with SMA were wasting. The FM% and fat mass index of children with wasting were both higher than non-wasting children (n P<0.05). 12 children had completed a 3-day dietary survey and only 2 children (16.7%) had energy intake that met the dietary reference intakes for Chinese. Insufficient intake rates of calcium, magnesium, iron, zinc and vitamin D were 83.3%, 75.0%, 41.7%, 41.7% and 83.3% respectively.n Conclusions:Children with SMA are generally malnourished, with increased body fat, reduced lean body weight, low bone mineral density, insufficient intake of energy and multi-nutrients. Comprehensive nutritional assessment can guide the multidisciplinary management of SMA.