Table 2 Clinical characteristics of the 34 Taiwanese patients with MPS II who died between 1995 and 2012
Clinical characteristics | Number (%) |
|---|---|
Age at onset of symptoms (years) (n = 32) | 2.5 ± 2.1 |
Age at diagnosis (years) (n = 32) | 4.8 ± 3.1 |
Gestational age (weeks) (n = 24) | 39.2 ± 1.8 |
Birth weight (gram) (n = 24) | 3522 ± 581 |
Birth weight (SDS) (n = 24) | 0.56 ± 1.30 |
Primary cause of death, n (%) | |
Respiratory failure | 19 (56 %) |
Cardiac failure | 6 (18 %) |
Post-traumatic organ failure | 1 (3 %) |
Infection (sepsis) | 1 (3 %) |
Unknown | 7 (21 %) |
Age at death (years) | 14.2 ± 4.2 |
Age at death, n (%) | |
<5 years | 0 |
≧5 to <10 years | 5 (15 %) |
≧10 to <15 years | 16 (47 %) |
≧15 to <20 years | 11 (32 %) |
≧20 years | 2 (6 %) |
Year of death, n (%) | |
1995–2000 | 4 (12 %) |
2001–2004 | 10 (29 %) |
2005–2008 | 10 (29 %) |
2009–2012 | 10 (29 %) |
Phenotype, n (%) | |
Severe | 31 (91 %) |
Intermediate | 1 (3 %) |
Mild | 2 (6 %) |
ERT, n (%) | |
Yes | 2 (6 %) |
No | 32 (94 %) |
HSCT, n (%) | |
Yes | 2 (6 %) |
No | 32 (94 %) |