Table 1 Clinical characteristics of 34 Taiwanese MPS II patients who died between 1995 and 2012
No. | MPS type | Gender | Age at death (years) | Year at death | Age at onset of symptoms (years) | Age at diagnosis (years) | Gestational age (weeks) | Birth weight (grams) | Primary cause of death | Age at initial ERT (years) | Age at HSCT (years) |
|---|---|---|---|---|---|---|---|---|---|---|---|
1 | II (S) | M | 5.4 | 2003 | 0.1 | 0.3 | 37 | 2800 | Infection (sepsis) | – | 1.1 and 3.1, respectively |
2 | II (S) | M | 9.0 | 1997 | 4.5 | 7 | NA | NA | Unknown | – | – |
3 | II (S) | M | 9.5 | 2005 | 1.3 | 3.6 | 38 | 4700 | Respiratory failure | – | – |
4 | II (S) | M | 9.5 | 2008 | 0.1 | 1.2 | 37 | 2856 | Respiratory failure | – | – |
5 | II (S) | M | 9.8 | 2011 | 2.6 | 2.6 | 39 | 3450 | Respiratory failure | – | – |
6 | II (S) | M | 10.0 | 1995 | 0.2 | 9.3 | 41 | 3700 | Cardiac failure | – | 9.6 |
7 | II (S) | M | 11.3 | 2002 | 5.4 | 5.8 | 41 | 3200 | Respiratory failure | – | – |
8 | II (S) | M | 11.7 | 2007 | 2.5 | 3 | 40 | 3250 | Respiratory failure | – | – |
9 | II (S) | M | 11.9 | 1999 | 3 | 5.3 | 36 | 2800 | Unknown | – | – |
10 | II (S) | M | 11.9 | 2003 | 0.7 | 1.3 | 35 | 3150 | Respiratory failure | – | – |
11 | II (S) | M | 12.2 | 2002 | 0.2 | 5.1 | 39 | 3750 | Respiratory failure | – | – |
12 | II (S) | M | 12.4 | 2007 | 1 | 2.8 | 41 | 3900 | Respiratory failure | – | – |
13 | II (S) | M | 12.5 | 2009 | 1.3 | 1.3 | NA | NA | Respiratory failure | – | – |
14 | II (S) | M | 12.9 | 2002 | 5 | 6.7 | 38 | 4200 | Unknown | – | – |
15 | II (S) | M | 13.0 | 2003 | 1.7 | 5.0 | 40 | 3200 | Respiratory failure | – | – |
16 | II (S) | M | 13.1 | 1997 | 0.1 | 5 | 39 | 3870 | Cardiac failure | – | – |
17 | II (S) | M | 13.4 | 2001 | NA | NA | NA | NA | Respiratory failure | – | – |
18 | II (S) | M | 13.4 | 2004 | 3.6 | 5.5 | 40 | 3500 | Respiratory failure | – | – |
19 | II (S) | M | 13.9 | 2008 | 0.1 | 3.3 | 37 | 2400 | Unknown | – | – |
20 | II (S) | M | 14.3 | 2003 | 3 | 10.6 | 42 | 3695 | Respiratory failure | – | – |
21 | II (S) | M | 14.8 | 2010 | 1.2 | 1.5 | 40 | 3000 | Cardiac failure | – | – |
22 | II (S) | M | 15.1 | 2005 | 4.5 | 7.6 | 40 | 3000 | Respiratory failure | – | – |
23 | II (S) | M | 15.5 | 2009 | 3 | 3 | NA | NA | Unknown | – | – |
24 | II (S) | M | 15.6 | 2012 | 3 | 4 | 40 | 3550 | Unknown | – | – |
25 | II (S) | M | 16.1 | 2012 | 2.7 | 3.2 | NA | NA | Respiratory failure | – | – |
26 | II (S) | M | 16.1 | 2010 | 0.2 | 2 | 40 | 4150 | Respiratory failure | – | – |
27 | II (S) | M | 17.2 | 2002 | 3 | 12.2 | NA | NA | Post-traumatic organ failure | – | – |
28 | II (I) | M | 17.7 | 2009 | 4.2 | 4.3 | 40 | 3750 | Cardiac failure | 15.4 | – |
29 | II (S) | M | 17.8 | 2007 | NA | NA | NA | NA | Unknown | – | – |
30 | II (S) | M | 18.0 | 2008 | 3.3 | 3.3 | 41 | 4500 | Respiratory failure | – | – |
31 | II (S) | M | 18.9 | 2011 | 2.9 | 2.9 | NA | NA | Respiratory failure | – | – |
32 | II (S) | M | 19.4 | 2007 | 3.3 | 3.3 | 40 | 4160 | Respiratory failure | – | – |
33 | II (M) | M | 24.1 | 2009 | 10.1 | 10.1 | NA | NA | Cardiac failure | 23.9 | – |
34 | II (M) | M | 25.9 | 2006 | 2 | 10.8 | NA | NA | Cardiac failure | – | – |