Table 1 Demographic and clinical characteristics of the subjects under study.

From: Multiple respiratory assessment and thresholds for noninvasive ventilation in adult patients with spinal muscular atrophy

Characteristics

Subjects (n = 20)

Age, years

35.7 ± 14.8

Sex

13 F/7 M

BMI, kg/m2

20.9 ± 5.1

SMA type 2, n (%)

13 (65)

SMA type 3, n (%)

7 (35)

Walkers, n (%)

2 (10)

Sitters, n (%)

18 (90)

Scoliosis surgery, n (%)

13 (65)

HFMS

2 [0.0–4.5]

RULM

8 [0.0–20.7]

Respiratory function

Subjects (n = 20)

FVC, L

1.4 ± 1.2

FVC, % predicted

39.9 ± 27.5

MIP, cm H2O

33.8 ± 21.5

MIP, % predicted

41.8 ± 23.5

MEP, cm H2O

27.3 ± 19.9

MEP, % predicted

23.9 ± 18.7

SNIP, cm H2O

52.2 ± 19.1

SNIP, % predicted

53.9 ± 21.0

Baseline PaCO2, mmHg

37.0 ± 2.07

Mean PtcCO2 mmHg

42.9 ± 6.0

Peak PtcCO2, mmHg

48.5 ± 5.5

Time with SpO2 < 88, %

0.3 [0.0–1.8]

Mean SpO2, %

96.2 ± 1.5

  1. HFMS: Hammersmith Functional Motor Scale; RULM: revised; Upper Limb Module; FVC: forced vital capacity; MIP: maximum inspiratory pressure; MEP: maximum expiratory pressure; SNIP: sniff nasal inspiratory pressure; PaCO2: partial pressure of carbon dioxide; PtcCO2: transcutaneous carbon dioxide; SpO2: pulse oximeter oxygen saturation.