International Center for the Assessment of Nutritional Status (ICANS), Department of Food Environmental and Nutritional Sciences (DeFENS), University of Milan, Milan, Italy.
SAPRE, Parents Early Habilitation Service, Fondazione IRCCS Ca' Granda, Policlinico Hospital, Milan, Italy.
Eur J Clin Nutr. 2019 Dec;73(12):1646-1648. doi: 10.1038/s41430-019-0392-2. Epub 2019 Jan 15.
Spinal muscular atrophy (SMA) is a neuromuscular disease associated with nutritional status derangement and altered body composition. New drugs are changing the natural history of the disease, so now more than ever is important to focus on the correct assessment of nutritional status in SMA. We implemented a standardization process for the anthropometric measurement as part of our ongoing longitudinal study of growth patterns in SMA patients. It features a procedural manual, included in this communication, observers training and reliability assessment, of which we publish the values obtained in our pilot study. The standardization process was able to produce inter-observer reliability values in agreement with the literature and a procedure manual is now available for multicentre studies of nutritional status and body composition in SMA and possibly other pediatric neuromuscular disorders.
脊髓性肌萎缩症(SMA)是一种与营养状态失调和身体成分改变有关的神经肌肉疾病。新的药物正在改变疾病的自然进程,因此现在比以往任何时候都更重要的是要关注 SMA 患者的营养状况的正确评估。我们实施了一项标准化的人体测量程序,作为我们正在进行的 SMA 患者生长模式的纵向研究的一部分。它包括一个程序手册,我们在本通讯中提供了该手册,以及观察者的培训和可靠性评估,我们公布了我们在试点研究中获得的数值。标准化程序能够产生与文献一致的观察者间可靠性值,现在已经有一个程序手册可供 SMA 及其他儿科神经肌肉疾病的营养状况和身体成分的多中心研究使用。