Table 2.

Respiratory dysfunction

	Total			BMD			IMD
	n	%		n	%		n	%
FVC (%)	60	75.9	48.5 +/–20.9	31	88.6	59.2 +/–14.8	29	65.9	36.4 +/–17.3
			(50.8, 5.9–80.4)			(59.9, 14.0–80.4)			(37.0. 5.9–63.0)
no FVC data	19	24.1		4	11.3		15	34.1
ambulation
ambulant	17	21.5		17	48.6		0	0.0
non-ambulant	62	78.5		18	51.4		44	100.0
age at loss of ambulation (y.o.)	63	79.7	18.9 +/–11.2	18	51.4	29.4 +/–5.6	44	100.0	14.0 +/–1.0
			(14, 13–65)			(20, 17–65)			(14, 13–16)
Steroid
never	58	73.4		28	80.0		30	68.2
current	11	13.9		4	11.4		7	15.9
former	10	12.7		3	8.6		7	15.9
cardiac function
dysfunction	53	67.1		19	54.3		34	77.3
normal	26	32.9		16	45.7		10	22.7
genotyping
in-frame	28	35.4		19	54.3		9	20.5
out-of-frame	35	44.3		6	17.1		29	65.9
Small mutation	16	20.3		10	28.6		6	13.6
IHC
faint and patchy	11	13.9		5	14.3		5	11.4
negative	10	12.7		4	11.4		7	15.9
no data	58	73.4		26	74.3		32	72.7