Fauroux Brigitte, Quijano-Roy Susana, Desguerre Isabelle, Khirani Sonia
Pediatric Noninvasive Ventilation and Sleep Unit, Necker University Hospital, AP-HP, Paris; Research Unit Inserm U955 Equipe 13, Créteil.
Pediatric Department Centre de Référence Maladies Neuromusculaires (GNMH), Raymond Poincaré Hospital, AP-HP, Garches, Université Versailles UVSQ Inserm, UMRS_974, Paris.
Chest. 2015 Feb;147(2):552-559. doi: 10.1378/chest.14-0819.
Routine lung function and respiratory muscle testing are recommended in children with neuromuscular disease (NMD), but these tests are based on noninvasive volitional maneuvers, such as the measurement of lung volumes and maximal static pressures, that young children may not always be able to perform. The realization of simple natural maneuvers such as a sniff or a cough, and the measurement of esophageal and gastric pressures during spontaneous breathing can add valuable information about the strength and endurance of the respiratory muscles in young children. Monitoring respiratory muscles in children with NMD may improve understanding of the natural history of NMD and the evaluation of disease severity. It may assist and guide clinical management and it may help the identification and selection of optimal end points, as well as the most informative parameters and patients for clinical trials.
对于患有神经肌肉疾病（NMD）的儿童，建议进行常规肺功能和呼吸肌测试，但这些测试基于非侵入性的自主动作，如肺容量测量和最大静态压力测量，而幼儿可能并不总能完成这些动作。实现诸如吸气或咳嗽等简单自然动作，以及在自主呼吸过程中测量食管和胃内压力，可以为幼儿呼吸肌的力量和耐力提供有价值的信息。监测患有NMD的儿童的呼吸肌可能有助于更好地了解NMD的自然病史和疾病严重程度评估。它可以辅助和指导临床管理，有助于识别和选择最佳终点，以及用于临床试验的最具信息量的参数和患者。
