Nutritional status, swallowing disorders and respiratory prognosis in
adult Duchenne muscular dystrophy patients
Malnutrition and swallowing disorders are common in Duchenne muscular
dystrophy (DMD) patients. We assessed, in adult DMD with HMV (home
mechanical ventilation) and cough assist device, its prevalence and the
relationships with respiratory muscle strength and long-term respiratory
prognosis. We reviewed the patients (n=117, age 18 to 39 years (median
24)), followed in a reference center, from 2006 to 2015, to obtain
clinical baseline, nutritional status, vital capacity (VC), maximal
inspiratory pressure (MIP), maximal expiratory pressure (MEP). Median
body mass index was low (15.6). Prevalence of malnutrition, swallowing
disorders and gastrostomy were respectively 62, 34 and 11%. BMI and
serum albumin level were significantly associated with MIP, MEP and VC.
The 1/5-years cumulative incidences of respiratory events (pulmonary
sepsis and acute respiratory distress) were respectively 20.7/44.5%.
Using univariate analysis, predictive factors for respiratory events
were swallowing disorders (p=0.001), transthyretinemia (p=0.034), MIP
(p=0.039) and MEP (p=0.03) but not BMI or albuminemia. Using
multivariate analysis, only swallowing disorders remained significantly
associated with respiratory events (OR=4.2, IC95% 1.31-12.2, p=0.01).
If prevalence of malnutrition and swallowing disorders are high in adult
DMD on HMV, and respiratory muscles strength associated with serum
albumin level and BMI, respiratory prognosis is mainly related to