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. 2016 Jun 27;11:85. doi: 10.1186/s13023-016-0471-6

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 %)

MPS mucopolysaccharidosis, SDS standard deviation score, ERT enzyme replacement therapy, HSCT hematopoietic stem cell transplantation