Table 6.
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% |