Table 2.
All patients | N | % | Missing | Median | Range | |
---|---|---|---|---|---|---|
No of Studies | 150 | 2 per pt | 1.0-9.0 | |||
Duration since treatment start (years) | 3.3 | 0.1-15.7 | ||||
Duration of final assessment since treatment start (years) | 8 pts pre-treatment studies only | 4.4 | 0.1-15.7 | |||
Patients with sleep disordered breathing (present/absent) | 36/17 | 68/32 | 8 (SDB present in 6/8 with only pre-treatment studies) | |||
Progression of sleep disordered breathing (yes/no) | 15/22 | 41/59 | 24 | |||
ODI 4% Post treatment | 6.1 | 0.1-46.0 | ||||
Median oxygen saturation Post treatment | 95.4 | 83.9-98.1 | ||||
% Time < 90% Oxygen saturation Post treatment | 2.2 | 0.0-30.8 | ||||
HSCT treated Hurler patient | ||||||
No of Studies | 106 | |||||
Time post treatment start (years) | 3.1 | 0.1-14.9 | ||||
Sleep disordered breathing post HSCT present/absent | 24/11 | 69/31 | 6 | |||
Progression of SDB in patients with multiple studies yes/no | 6/19 | 24/76 | 16 | |||
No of pre-treatment studies | 19 | In 14 patients | ||||
Pre-treatment ODI 4% | 9.5 | 1.3-39.9 | ||||
Pre-treatment Median oxygen saturation | 96.2 | 81.6-98.5 | ||||
Pre-treatment % Time < 90% Oxygen saturation | 4.1 | 0-14.2 | ||||
No. of post treatment studies | 87 | In 35 patients | ||||
ODI 4% Post treatment | 5.8 | 0.2-19.8 | ||||
Median oxygen saturation Post treatment | 95.4 | 87.4-99.6 | ||||
% Time < 90% Oxygen saturation Post treatment | 1.75 | 0-30.8 | ||||
ERT treated Attenuated Patients | ||||||
No of Studies | 44 | |||||
Time post treatment start (years) | 3.5 | 0.7-8.0 | ||||
Sleep disordered breathing (SDB) present | 11/6 | 65/35 | ||||
Progression of SDB yes/no | 8/3 | 73/27 | 7 | |||
No of pre-treatment studies | 4 | In 3 patients | ||||
Pre-treatment ODI 4% | 5.7 | 0.2-14.8 | ||||
No of post treatment studies | 40 | In 17 patients | ||||
ODI 4% Post treatment | 6.9 | 0.1-46 | ||||
Median oxygen saturation Post treatment | 95.1 | 83.9-98.1 | ||||
% Time < 90% Oxygen saturation Post treatment | 3.2 | 0-17.25 |
N indicates number; SDB, Sleep disordered breathing.
ODI 4%, oxygen desaturation index 4%.