Table 1

Patient demographics

Patient	Age	Diagnosis	SNIP	P _mo,W	P _I,max	P _E,max	FEV₁	FVC	FEV₁/FVC	S _p,O₂	P _ET,CO₂
no.	yrs		cmH₂O	cmH₂O	cmH₂O	cmH₂O	L	L	%	%	kPa
Paediatric
1	10	SMA	38.0	40.0	53.2	41.7	1.5	1.7	88.4	98	5.5
2	11	SMA	27.2	25.7	23.7	30.6	0.4	0.4	100.0	95	5.1
3	12	SMA	35.7	15.0			0.2	0.4	50.0	97	5.9
4	14	DMD	21.0	19.0	19.0	16.7	0.4	0.5	73.6	97	5.7
5	17	DMD	10.0	11.2	14.5	9.7	0.4	0.5	72.9	96	4.9
6	15	DMD	18.0	14.6	13.6	15.4	0.4	0.9	44.0	97	6.1
7	14	CMD	29.8	15.1	11.6	7.8	U	U		95	5.3
8	16	CMD	19.0	31.7	23.2	15.9	0.4	0.3	106.1	94	6.0
Mean±sd	14±2		24.8±9.5	21.5±10.0	22.7±14.3	19.7±12.2	0.5±0.4	0.7±0.5	76.41±23.63	96±1	5.6±0.4
Adult
9	18	SMA	51.1	18.8	34.6	21.1	1.1	1.2	97.4	97	5.0
10	18	DMD	6.8	6.8	7.5	8.1	0.3	0.3	93.5	94	htvf
11	44	SMA	28.1	55.0	30.8	61.9	2.7	3.3	81.9	97	6.0
12	22	SMA	30.6	37.8	63.0	31.5	1.6	2.0	76.7	97	5.6
13	20	SMA	13.3	11.5	12.6	11.9	U	U		94	6.1
14	28	SMA	30.1	16.9	33.3	17.8	0.9	0.9	98.8	97	5.7
15	35	SMA	63.5	20.9	46.8	23.5	1.3	1.4	97.1	98	5.0
16	22	SMA	14.2	11.0	15.5	14.8	0.3	0.4	62.8	98	4.8
17	25	DMD	6.4	4.6	6.9	5.4	0.3	0.5	58.3	96	7.7
18	30	DMD	5.0	4.4	8.9	4.6	U	U		98	7.1
19	22	CMD	14.6	20.0	18.3	23.2	0.3	0.4	94.4	94	5.0
20	56	Polio	36.6	65.0	36.3	52.7	0.6	0.9	70.3	94	5.2
21	53	Polio	28.0	53.0	17.5	47.8	0.9	1.0	86.4	96	5.6
22	45	Polio	45.0	111.5	40.5	94.8	0.7	0.9	81.3	96	5.2
Mean±sd	31±13		26.7±17.9	31.2±30.5	26.6±16.8	29.9±25.8	0.9±0.7	1.1±0.9	83.3±13.9	96±2	5.7±0.9
All patients
Mean±sd	25±13		26.0±14.8	27.7±24.6	25.3±15.4	26.5±21.9	0.8±0.6	0.9±0.7	80.7±17.3	96±1	5.6±0.7

SNIP: sniff nasal inspiratory pressure
P_mo,W: whistle mouth pressure
P_I,max: maximal static inspiratory mouth pressure
P_E,max: maximal static expiratory mouth pressure
FEV₁: forced expiratory volume in one second
FVC: forced vital capacity
S_p,O₂: arterial oxygen saturation measured by pulse oximetry
P_ET,CO₂: end-tidal carbon dioxide tension
SMA: spinal muscular atrophy
DMD: Duchenne's muscular dystrophy
CMD: congenital muscular dystrophy
polio: poliomyelitis
U: unrecordable