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Recent Advances in Chest Medicine |

The Value of Respiratory Muscle Testing in Children With Neuromuscular DiseaseRespiratory Muscle Tests in Neuromuscular Disease

Brigitte Fauroux, MD, PhD; Susana Quijano-Roy, MD, PhD; Isabelle Desguerre, MD, PhD; Sonia Khirani, PhD
Author and Funding Information

From the Pediatric Noninvasive Ventilation and Sleep Unit (Drs Fauroux and Khirani), Necker University Hospital, AP-HP, Paris; Paris Descartes University (Drs Fauroux and Desguerre), Paris; Research Unit Inserm U955 (Dr Fauroux), Equipe 13, Créteil; Pediatric Department (Dr Quijano-Roy), Centre de Référence Maladies Neuromusculaires (GNMH), Raymond Poincaré Hospital, AP-HP, Garches, Université Versailles UVSQ Inserm, UMRS_974, Paris; Pediatric Neurology Department (Dr Desguerre), Centre de Référence Maladies Neuromusculaires (GNMH), Necker University Hospital, AP-HP, Paris; and ASV Santé (Dr Khirani), Gennevilliers, France.

CORRESPONDENCE TO: Brigitte Fauroux, MD, PhD, Pediatric Noninvasive Ventilation and Sleep Unit, Necker University Hospital, 149 rue de Sèvres, 75015 Paris, France; e-mail: brigitte.fauroux@nck.aphp.fr


Reproduction of this article is prohibited without written permission from the American College of Chest Physicians. See online for more details.


Chest. 2015;147(2):552-559. doi:10.1378/chest.14-0819
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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.


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