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. 2019 May 29;14:118. doi: 10.1186/s13023-019-1080-y

Table 6.

Guidance statements for CPAP, NIPPV, oxygen supplementation and hypercapnia monitoring

Statement Percentage consensus

CPAP therapy is recommended for patients with MPS VI who display the presence of obstructive sleep apnoea (OSA) which persists after tonsillectomy and/or adenoidectomy

Evidence Grade: B (extrapolations from level 1 studies)

100%

NIPPV therapy is recommended for patients with MPS VI who display nocturnal hypoventilation and are unresponsive to CPAP, or display daytime hypoventilation with increased PaCO2 and/or serum HCO3 levels

Evidence Grade: B (extrapolations from level 1 studies)

94%

Oxygen supplementation is recommended for patients with MPS VI who display sleep apnoea with nocturnal hypoxemia and who do not tolerate CPAP or NIPPV masks

Evidence Grade: B (extrapolations from level 1/3/4 studies)

83%

Patients with MPS VI should be monitored for development of hypercapnia after starting oxygen therapy with measurement of PaCO2 and/or serum HCO3

Evidence Grade: D (level 5, expert clinical opinion)

97%