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Respiratory muscle decline in duchenne muscular dystrophy
Author(s) -
Khirani Sonia,
Ramirez Adriana,
Aubertin Guillaume,
Boulé Michèle,
Chemouny Chrystelle,
Forin Véronique,
Fauroux Brigitte
Publication year - 2014
Publication title -
pediatric pulmonology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.866
H-Index - 106
eISSN - 1099-0496
pISSN - 8755-6863
DOI - 10.1002/ppul.22847
Subject(s) - medicine , duchenne muscular dystrophy , respiratory system , muscular dystrophy , vital capacity , pulmonary function testing , neuromuscular disease , muscle weakness , physical therapy , pediatrics , cardiology , lung , lung function , disease , diffusing capacity
Summary Objectives Duchenne muscular dystrophy (DMD) causes progressive respiratory muscle weakness. The aim of the study was to analyze the trend of a large number of respiratory parameters to gain further information on the course of the disease. Study Design Retrospective study. Subject Selection 48 boys with DMD, age range between 6 and 19 year old, who were followed in our multidisciplinary neuromuscular clinic between 2001 and 2011. Methodology Lung function, blood gases, respiratory mechanics, and muscle strength were measured during routine follow‐up over a 10‐year period. Only data from patients with at least two measurements were retained. Results The data of 28 patients were considered for analysis. Four parameters showed an important decline with age. Gastric pressure during cough (Pgas cough) was below normal in all patients with a mean decline of 5.7 ± 3.8 cmH 2 O/year. Sniff nasal inspiratory pressure (SNIP) tended to increase first followed by a rapid decline (mean decrease 4.8 ± 4.9 cmH 2 O; 5.2 ± 4.4% predicted/year). Absolute forced vital capacity (FVC) values peaked around the age of 13–14 years and remained mainly over 1 L but predicted values showed a mean 4.1 ± 4.4% decline/year. Diaphragmatic tension‐time index (TTdi) increased above normal values after the age of 14 years with a mean increase of 0.04 ± 0.04 point/year. Conclusions This study confirms the previous findings that FVC and SNIP are among the most important parameters to monitor the evolution of DMD. Expiratory muscle strength, assessed by Pgas cough, and the endurance index, TTdi, which are reported for the first time in a large cohort, appeared to be informative too, even though measured through an invasive method. Pediatr Pulmonol. 2014; 49:473–481. © 2013 Wiley Periodicals, Inc.