Skip to main content

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%.
Back to article page

Orphanet Journal of Rare Diseases

ISSN: 1750-1172