Assessment of diaphragmatic thickness by ultrasonography in Duchenne muscular dystrophy (DMD) patients

Laviola, Marianna; Priori, Rita; D’Angelo, Maria Grazia; Aliverti, Andrea

Abstract

Introduction
In Duchenne muscular dystrophy (DMD) the assessment of diaphragmatic function is crucial because respiratory muscle weakness can cause respiratory failure. We aimed to noninvasively assess diaphragmatic function in DMD by measuring diaphragmatic thickness by ultrasonography, under the hypothesis that the progressive decrease of lung function is related to alterations of diaphragmatic thickness.

Methods
Forty-four DMD patients and thirteen healthy controls were enrolled and subdivided into three age groups. Diaphragmatic thickness was measured during quiet breathing, inspiratory capacity, maximal inspiratory pressure and expiratory pressure maneuvers.

Results
In DMD, absolute values of diaphragmatic thickness were significantly lower than in controls in the majority of the manoeuvers and diaphragmatic thickness significantly decreased with age at end-expiration, remaining constant at end-inspiration and during maximal inspiratory pressure maneuvers. Comparing to controls, absolute values of diaphragmatic thickness and diaphragmatic thickness variations were significantly lower (p less than 0.001), with the exception of quiet breathing and maximal expiratory pressure maneuvers in the youngest DMD. During maximal inspiratory pressure maneuver, variation of diaphragmatic thickness was
not significantly different in the all groups, nevertheless maximal inspiratory pressure decreases with age.

Conclusions
The diaphragm is prone to pseudo-hypertrophy in the youngest DMD, and to progressive atrophy in middle-age and oldest DMD. Diaphragm impairment could be expressed as a dissociation between muscle drive and muscle developed force. Ultrasonography could be
used as a noninvasive method to assess progressive diaphragmatic weakness.

Citation

Laviola, M., Priori, R., D’Angelo, M. G., & Aliverti, A. (2018). Assessment of diaphragmatic thickness by ultrasonography in Duchenne muscular dystrophy (DMD) patients. PLoS ONE, 13(7), 1-13. https://doi.org/10.1371/journal.pone.0200582

• Journal Article Type: Article
• Acceptance Date: Jul 1, 2018
• Online Publication Date: Jul 26, 2018
• Publication Date: Jul 26, 2018
• Deposit Date: Apr 30, 2019
• Publicly Available Date: May 10, 2019
• Journal: PLoS ONE
• Electronic ISSN: 1932-6203
• Publisher: Public Library of Science
• Peer Reviewed: Peer Reviewed
• Volume: 13
• Issue: 7
• Article Number: e0200582
• Pages: 1-13
• DOI: https://doi.org/10.1371/journal.pone.0200582
• Public URL: https://nottingham-repository.worktribe.com/output/1859447
• Publisher URL: https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0200582