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Table 1 Respiratory disorders in MPS patients: a review of the literature

From: ENT and mucopolysaccharidoses

 

No. of patients

MPS type (n)

Respiratory disorders

Kiely, 2017 [3]

55

MPS I

RI (54.5%)

Snoring (85.5%)

OSA (32.7%)

Laryngomalacia (9.1%)

Cohen, 2017 [34]

43

MPS III

RI (34.88%)

OSA (30.23%)

T&A (34.88%)

Chiong, 2017 [35]

23

MPS II

UAO (39%)

RI (78.3%)

OSA (39%)

T&A (39%)

Lin, 2014 [36]

35

MPS I (1)

MPS II (12)

MPS III (4)

MPS IV (16)

MPS VI (6)

T&A (96%)

OSA (71%)

Laryngomalacia (38%)

Muhlebach, 2013 [37]

31

MPS I (9)

MPS II (19)

MPS III (1)

MPS IV (1)

MPS VI (1)

T&A (72%)

Bronchomalacia (46%)

Laryngomalacia (31%)

Wold, 2010 [9]

9

MPS I (5)

MPS II (3)

MPS VI (1)

UAO (44.4%)

Yeung, 2009 [29]

27

MPS IH (8)

MPS IH/S (3)

MPS II (5)

MPS III B (1)

MPS VI (1)

UAO (70%)

RI (47%)

Bredenkamp, 1992 [38]

45

MPS IH (13)

MPS II (7)

MPS III (12)

MPS IV (6)

MPS VI (5)

MPS VII (2)

UAO (38%)

Semenza, 1988 [17]

21

MPS IH (4)

MPS IH/S (3)

MPS II (3)

MPS IV (6)

MPS VI (3)

MPS VII (4)

UAO (50–90%)

T&A (67%)

Supraglottic narrowing (92%)

OSA (89%)

  1. MPS mucopolysaccharidosis, OSA obstructive sleep apnoea, RI respiratory infection, T&A adenoid and/or tonsillar hypertrophy, UAO upper airway obstruction,