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. 2018 Feb 12;6:13. doi: 10.3389/fped.2018.00013

Table 4.

Studies on infants using long-term NIV reporting hospitalization and mortality outcomes.

First author, year, country Study design Primary diagnosis Infants using NIV Age, mean ± SD or med (range) NIV type Hospitalization (per infant/year unless otherwise stated)
Mortality (% of total infants unless otherwise stated)
Supportive care NIV IMV Supportive care NIV IMV
Bach (46), 2007, USA R; Obs: cohort SMA1 n = 47 10.6 ± 5.7 months BPAP 1.58 0.37 100 17 19

Bach (44), 2002, USA R; Obs: case series SMA1 n = 33 11.2 ± 5.7 months BPAP 1.53 0.58* 100 6 6

Gregoretti (51), 2013, Italy R; Obs: case series SMA1 n = 31 12.6 ± 14.4 months BPAP 0.023 0.006 93 45 17

Ottonello (54), 2011, Italy R; Obs: cohort SMA1 n = 16 10.4 ± 6.2 months BPAP 0.15 13

Bach (43), 2000, USA R; Obs: case series SMA1 n = 8 3–28 months BPAP 13

Birnkrant (48), 1998, USA R; Obs: case series SMA1 n = 3 4–9 months BPAP 100

Chatwin (49), 2011, UK R; Obs: cohort SMA1 n = 13 11 months (4–24 months) BPAP 38

Vasconcelos (56), 2005, Portugal R; Obs: cohort SMA1 n = 7 13 months (3 months to 3 years) BPAP 71

Lemoine (53), 2012, USA R; Obs: cohort SMA1 n = 26 136 days (54–196) BPAP 46% 83% NIV group had a significantly longer survival than supportive care group (p = 0.047, reported narratively)

First author, year, country Study design Primary diagnosis Infants using NIV Age, mean ± SD or med (range) NIV type Length of hospital stay [mean ± SD or med (range)] Mortality (% of total infants)

No ventilation NIV IMV

Leboulanger (36), 2010, France P; Obs: case Series PRS n = 7 2 months (1–10 months) CPAP (n = 5)
BPAP (n = 2)

Amaddeo (31), 2016, France R; Obs: cohort PRS n = 9 0–2 months CPAP 1 month (20–40 days) 2 months (6 weeks to 4 months)

Kam (35), 2015, Canada R; Obs: cohort PRS n = 20 23 months (5 days to 8 years) CPAP 28 ± 24 days 66 ± 46 days 138 ± 76 days NR

First author, year, country Study design Primary diagnosis Infants using NIV Age, mean ± SD or med (range) NIV type Hospitalization (per infant/year or % of total) Mortality (% of total infants)

McNamara (27), 1995, Australia P, Obs: cohort OSA n = 5 8–12 weeks CPAP 0%

First author, year, country Study design Primary diagnosis Infants using NIV Age, mean ± SD or med (range) NIV type Length of hospital stay [mean ± SD or med (range)]
Mortality
No ventilation NIV IMV

Garcia Teresa (60), 2017, Spain P, Obs: cross-sectional CHS n/a 11.35 (5 months to 28.6 years) NIV 91 ± 51 days 319 ± 336 days** n = 2 infants

BPAP, bi-level positive airway pressure; IMV, invasive mechanical ventilation; NIV, non-invasive ventilation; Obs, observational study; OSA, obstructive sleep apnea; P, prospective; PRS, Pierre Robin sequence; R, retrospective; SMA1, spinal muscular atrophy type 1.

*p < 0.05.

**p < 0.01.

p < 0.001.