Table 1

Demographic characteristics

Characteristicsn*NH (n=7)n*Without NH (n=39)p Value
Age, mean (SD)712.1 (3.2)3910.8 (3.4)0.325
Male, n (%)74 (57.1)3924 (61.5)1.000
Height, mean (SD)7149.3 (16.8)38145.9 (20.4)0.682
Weight, mean (SD)742.4 (16.8)3845.3 (23.0)0.755
BMI, median (IQR)716.4 (14.3–24.4)3819.8 (14.9–22.3)0.635
Wheelchair enabled, n (%)73 (42.9)384 (10.3)0.124
Diagnosis738
 Charcot-Marie-Tooth1
 Spinal muscular atrophy type II23
 Limb girdle dystrophy2
 Congenital myotonia14
 Myasthenic syndrome2
 Congenital muscular dystrophy24
 Metabolic myopathy15
 Nemaline myopathy12
 Clinical myopathy without confirmed genetic aetiology15
FVC, mean (SD)758.4 (39.3)3877.5 (24.1)0.048
FEV1, mean (SD)755.0 (26.7)3878.3 (21.5)0.022
MIP, mean (SD)765.0 (39.1)3474.5 (37.3)0.556
MEP, mean (SD)751.0 (19.4)3473.6 (39.5)0.107
Baseline CO2 awake, median (IQR)741 (38–43)2940 (37–44)0.888
Apnoea-hypopnoea index
 Central45.9 (0.9–12.0)61.5 (0.2–3.1)0.352
 Obstructive34.2 (4.1–10.9)151.0 (0.6–5.6)0.130
 Total78.2 (4.2–13.2)292.0 (0.8–4.9)0.018
Lowest oxygen saturation789 (80–94)2798 (94–99)0.015
Baseline oxygen saturation asleep797 (96–97)3897 (97–98)0.187
Baseline et/tc pCO2 asleep749 (49–53)3046 (43–49)0.015
Maximum tc/et pCO2756 (46–58)2950 (44–53)0.111
  • * Indicates the number of participants for whom the data were available. The ranges in the last 7 rows refer to medians and interquartile ranges.

  • BMI, body mass index; et, end-tidal measurement of carbon dioxide; FEV1, forced expiratory volume in 1 s; FVC, forced vital capacity; MEP, maximal expiratory pressure; MIP, maximal inspiratory pressure; NH, nocturnal hypoventilation; tc, transcutaneous measurement of carbon dioxide. The units are millimeters of mercury.