Daytime predictors of sleep hypoventilation in Duchenne muscular dystrophy.

摘要: Sleep hypoventilation is an inevitable consequence of Duchenne muscular dystrophy (DMD), usually preceding daytime respiratory failure. Appropriate scheduling polysomnography and the introduction noninvasive ventilation (NIV) during sleep are not defined. Our aim was to determine parameters lung function associated with in patients DMD. As our method we chose a prospective comparison wakeful (spirometry, volumes, maximal mouth pressures, arterial blood gases) outcomes polysomnography. All measurements were made subjects breathing air. Nineteen studied. The FEV(1) correlated Pa(CO(2)) (r = -0.70, p /= 2%); >/= 45 mm Hg equally sensitive (91%) but more specific (75%) indicator while base excess 4 mmol/L highly (100%) less (55%). After NIV (n 8), there significant reduction (54 +/- 7.4 49.1 Hg, particularly if mmol/L; (3) decrease after administered implicates pathogenesis failure; (4) impaired ventilatory drive possible mechanism for failure, as NIV-associated occurs despite further decline capacity, suggesting continuing deterioration muscle function.