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Table 6 Guidance statements for CPAP, NIPPV, oxygen supplementation and hypercapnia monitoring

From: Recommendations for the management of MPS VI: systematic evidence- and consensus-based guidance

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%