Table 1.
Clinical characteristics of the study population, n = 20.
| Variable (n = 20, unless otherwise noted) | |
|---|---|
| Age at RAM cannula NIV initiation (months), median (IQR) | 5.8 (2.4-9.9) |
| Gender, n (%) | |
| Male | 6 (30) |
| Female | 14 (70) |
| Diagnoses, n (%) a | |
| Bronchopulmonary dysplasia | 4 (20) |
| Restrictive lung disease | 5 (25) |
| Genetic disorders | 13 (65) |
| Indications for NIV use, n (%) b | |
| Sleep-related hypoventilation | 3 (15) |
| Restrictive lung disease | 5 (25) |
| Obstructive sleep apnea | 9 (45) |
| Chronic respiratory failure with hypercapnia | 10 (50) |
| Polysomnography results, n = 13 | |
| Central sleep apnea, n (%) | 1 (7.7) |
| Obstructive sleep apnea, n (%) | 12 (92.3) |
| Sleep-related hypoxemia, n (%) | 6 (46.2) |
| Sleep-related hypoventilation, n (%) | 7 (53.8) |
| Total AHI (events per hour), median (IQR) | 16.5 (7.3-24.6) |
| Obstructive AHI (events per hour), median (IQR) | 10.2 (5.5-23.0) |
| Central AHI (events per hour), median (IQR) | 1.35 (0.5-3.5) |
Abbreviations: AHI, apnea-hypopnea index; IQR, interquartile range; NIV, noninvasive ventilation.
a
Genetic disorders included Trisomy 18 (n = 6), Trisomy 21 (n = 2), Prader-Willi syndrome (n = 1), partial trisomy 2q (n = 1), Crouzon syndrome (n = 1), and osteogenesis imperfecta (n = 2). Restrictive lung diseases included prune belly syndrome (n = 1), osteogenesis imperfecta (n = 2), arthrogryposis (n = 1), and thoracic insufficiency (n = 1). Two patients had Trisomy 21 and bronchopulmonary dysplasia. Two patients had osteogenesis imperfecta and restrictive lung disease.
b
Six patients had more than 1 indication for NIV use.