RT Journal Article
SR Electronic
T1 Which are the most informative parameters to follow the respiratory decline in Duchenne muscular dystrophy?
JF European Respiratory Journal
JO Eur Respir J
FD European Respiratory Society
SP 1392
VO 40
IS Suppl 56
A1 Sonia Khirani
A1 Adriana Ramirez
A1 Guillaume Aubertin
A1 Michèle Boulé
A1 Chrystelle Chemouny
A1 Veronique Forin
A1 Brigitte Fauroux
YR 2012
UL http://erj.ersjournals.com/content/40/Suppl_56/1392.abstract
AB RationaleDuchenne muscular dystrophy causes progressive respiratory muscle weakness. Few information is available on the natural evolution of respiratory parameters in boys with Duchenne muscular dystrophy.ObjectivesIn order to identify respiratory parameters associated with the earliest decline, lung function, blood gases, respiratory mechanics, and respiratory muscle strength were measured in 48 boys with Duchenne muscular dystrophy (mean age 12.7 ± 3.4, range 7.6 – 19.0 years), over a period of 10 years.Main ResultsOnly four parameters showed an important decline with age. Gastric pressure during cough was below normal in all patients with a mean 5.7 ± 3.8 cmH2O decline per year. Sniff nasal inspiratory pressure tended to increase before the age of 10 years followed by a rapid decline (mean decrease 4.8 ± 4.9 cmH2O or 5.2 ± 4.4 % predicted per year). Absolute forced vital capacity values peaked around 13-14 years of age and remained mainly over 1 liter but predicted values showed a mean 4.1 ± 4.4 % decline per year. Diaphragmatic tension time index increased above normal after the age of 14 years old with a mean increase of 0.04 ± 0.04 point per year.ConclusionRepeated gastric pressure during cough, sniff nasal inspiratory pressure, and forced vital capacity measurements provide simple tools to assess the progression of respiratory muscle weakness in young boys with Duchenne muscular dystrophy. Endurance indexes decline at a later age. These indexes may help to monitor treatment effects.