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Table 2 Sleep oximetry characteristics

From: Sleep disordered breathing in mucopolysaccharidosis I: a multivariate analysis of patient, therapeutic and metabolic correlators modifying long term clinical outcome

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
  1. N indicates number; SDB, Sleep disordered breathing.
  2. ODI 4%, oxygen desaturation index 4%.