Résumé :
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Vigorous coughing is a strong determinant factor of survival in patients with neuromuscular diseases (NMD). We measured the PCFR of 24 stable patients (DMD=9, SMA=5, others=10), with a median age of 14,3y (5,6-18,6y) and a forced vital capacity less than 50% predicted (11-50%), 8 of whom were on home mechanical ventilation (HMV). PCFR, FVC, peak expiratory flow rate (PEFR), maximal inspiratory (MIP) and expiratory pressures (MEP) were measured according to standard guidelines. The mean PCFR was 138±48 l/s and mean % predicted PCFR was 36±14 %. HMV patients had lower mean PCFR (108±39 l/s) and %PCFR (27±14%) as compared to non-HMV patients (153±46 l/s, 41±11%, p<0,05). % PCFR correlated with % FVC (r=0.55, p=0,0045) and % PEFR (r=0,73, p<0,0001). Absolute PCFR values correlated with MEP values (r=0,69, p=0,0004). One HMV patient with a PCFR of 12 % predicted died one month later from acute pneumonia. PCFR is easy to measure in school-aged children with severe lung impairment and may help to guide treatment strategies.
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