Skip to main content

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

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

Statement Percentage consensus
CPAP therapy is recommended for patients with MPS IVA who display the presence of obstructive sleep apnoea (OSA) that persists after tonsillectomy and/or adenoidectomy
Evidence Grade: D (limited published evidence)
97%
NIPPV therapy is recommended for patients with MPS IVA who display nocturnal hypoventilation and are unresponsive to CPAP, or display daytime hypoventilation with increased PaCO2 and/or serum HCO3 levels
Evidence Grade: D (level 5 expert clinical opinion)
91%
Oxygen supplementation during sleep is recommended for patients with MPS IVA who exhibit sleep apnoea with nocturnal hypoxemia, and who do not tolerate CPAP or NIPPV masks
Evidence Grade: D (level 5 expert clinical opinion)
77%
Patients with MPS IVA should be monitored for development of hypercapnia after starting oxygen therapy using measurement of PaCO2 and/or serum HCO3
Evidence Grade: D (level 5 expert clinical opinion)
97%