Table 1. Patient demographics, treatment and tissue characteristics.
| Patient No. | MPS phenotype | Gender | Age @ enrolment | Age @ start of ERT | Surgery | Preop ODI4% | DS:CS Ratio |
|---|---|---|---|---|---|---|---|
| 1 | I HS | M | 6.2 | 4 | Revision adenoidectomy for OSA. Previous adenotonsillectomy, 2008 | 12.7 | 0.74 |
| 2 | I HS | F | 9.5 | 2 | Adenotonsillectomy for OSA | 15.3 | 0.7 |
| 3 | IIIA | M | 3.4 | - | Adenoidectomy for nasal obstruction and glue ear | 12.5 | - |
| 4 | IVA | F | 4.2 | 3 | Tonsillectomy for OSA. Previous adenoidectomy 2011 | 9.0 | - |
| 5 | IVA | M | 12.2 | 8 | Adenoidectomy for OSA. Previous tonsillectomy 2009 | 45.4 | - |
| 6 | IVA | M | 4.3 | 2 | Tonsillectomy for OSA | 12.1 | - |
| 7 | VI | F | 2.0 | 1 | Adenotonsillectomy for OSA | 15.1 | 2.5 (PRE ERT) |
| 8 | VI | M | 9.7 | 9 | Adenotonsillectomy for OSA. Late diagnosis. | - | 1.2 |
| 9 | Control | F | 4.3 | - | Adenoidectomy for nasal obstruction and glue ear | - | - |
| 10 | Control | F | 2.1 | - | Adenotonsillectomy for mild OSA | 5.1 | - |
| 11 | Control | M | 4.9 | - | Adenotonsillectomy for mild OSA | 1.3 | - |
| 12 | Control | M | 3.4 | - | Adenotonsillectomy for mild OSA | 12.0 | - |
ERT, Enzyme replacement therapy; Preop ODI4%. Preoperative sleep study oxygen desaturation index 4%; HS, Hurler-Scheie; OSA, Obstructive sleep apnoea.
ERT is not currently available for MPS III