Patients with advanced muscular dystrophy frequently develop ventilatory fa
ilure. Currently respiratory impairment usually is assessed by measuring vi
tal capacity and the mouth pressure generated during a maximal inspiratory
maneuver (P-1,max), neither of which directly measures ventilatory capacity
. We assessed inspiratory flow reserve in 26 boys [mean (SD) age 12.8 (3.8)
years] with Duchenne muscular dystrophy (DMD) without ventilatory failure
and in 28 normal boys [mean (SD) age 12.6 (1.9) years] by analyzing the rat
io between the largest inspiratory flow during tidal breathing (V ' (1),max
(t)) and during a forced Vital capacity maneuver (V ' (1),max((FVC))), (V '
(1),max(t)/V ' (1),max((FVC))). We have compared this ratio with the force
d vital capacity (Nc) and P-1,max measured at functional residual capacity.
Mean P-1,max was - 90(30)cmH(2)O, average 112% (range 57-179%) of predicted
values in control boys and - 31(11)cmH(2)O, average 40% predicted values i
n DMD boys (control vs DMD, P < 0.001). FVC was reduced in DMD boys [59(20)
% predicted values vs 86(10)% predicted values in controls, P < 0.01]. Abso
lute V ' (1),max((FVC)) was strongly related to FVC in both control and DMD
boys; V ' (1),max((FVC)) (expressed as FVC(.)s(-1)) was not related to P-1
,max in either group. The mean V-1,max(t)/V ' (1),max((FVC)) ratio was high
er in DMD 0.22 (0.08) than in controls 0.12 (0.03) (P < 0.001) indicating a
reduction in inspiratory flow reserve in DMD. Inspiratory flow reserve was
within the normal range in 8 of 19 DMD patients with P-1,max less than 50%
of predicted values.
We conclude that measurement of inspiratory flow reserve (V ' (1),max(t)/V
' (1),max((FVC)) ratio) provides a simple and direct assessment of dynamic
inspiratory muscle function which is not replicated by static measurement o
f P-1,max or vital capacity and might be useful in assessment of respirator
y impairment in boys with Duchenne muscular dystrophy. Follow-up studies ar
e required to establish whether measures of inspiratory flow reserve are of
clinical value in predicting subsequent ventilatory failure. (C) 2001 Wile
y-Liss, Inc.