Analysis of cognitive ability and adaptive behavior assessment tools used in an observational study of patients with mucopolysaccharidosis II

Table 2 Patient baseline characteristics

Characteristic	Study population (N = 38)
Male, n (%)	38 (100)
Age, years
Median (range)	4.90 (2.11–16.74)
Age subgroups, n (%)
< 7 years	27 (71.1)
≥ 7 years	11 (28.9)
Race, n (%)
Asian	2 (5.3)
African American	1 (2.6)
White	32 (84.2)
Other	3 (7.9)
Genotype category, n (%)
Frameshift	3 (7.9)
Large deletion	1 (2.6)
Missense/presumed missense	20 (52.6)
Nonsense	3 (7.9)
Premature truncated	2 (5.3)
Presumed splice site/splice site	9 (23.7)
Baseline DAS-II GCA score^a subgroups, n (%)
≤ 70	16 (42.1)
> 70	16 (42.1)
Missing	6 (15.8)
Baseline DAS-II GCA score^a, n = 32
Mean (SD)	73.1 (14.4)
Baseline VABS-II ABC score^b, n = 36
Mean (SD)	81.8 (11.8)

ABC Adaptive Behavior Composite; DAS-II Differential Ability Scales, second edition; GCA General Conceptual Ability; SD standard deviation; VABS-II Vineland Adaptive Behavior Scales, second edition
^aStandard scores of ≤ 69, 70–79, 80–89 and 90–109 indicate very low, low, below average and average function, respectively
^bStandard scores of ≤ 70, 71–85 and 86–114 indicate low, moderately low and adequate adaptive levels, respectively

ISSN: 1750-1172