Beneath the floor: re-analysis of neurodevelopmental outcomes in untreated Hurler syndrome

Table 1 Summary data for the full sample and each group separately. Ages are in months unless otherwise indicated

	Age at test	Mental age	DQ	SS/MDI	Age at death (Years)
Group 1 (N = 23)
Mean	27.00	15.06	61.04	63.93	7.41
SD	16.60	5.76	21.89	17.55	2.81
Median	22.80	16.80	58.90	56.00	6.90 range: 3.3–11.47
Group 2 (N = 9)
Mean	21.41	16.75	83.33	82.00	3.38
SD	10.80	5.70	12.92	15.43	2.35
Median	18.50	14.60	85.00	82.00	1.85 range: 1.22–7.71
Total Sample (N = 32)
Mean	25.19	15.61	68.29	69.80	6.24
SD	15.05	5.73	21.97	18.76	3.23
Median	21.40	15.70	70.30	66.50	5.85 range: 1.22–11.47

Group 1 = Untreated
Group 2 = Eventually proceeded to BMT but died
Age at Test = age at time of neurocognitive testing
Mental Age = BSID Age Equivalent Score
DQ = Per Delaney et al. [15], Mental Age divided by Age at Test, times 100
SS/MDI = norm-based Bayley standard score, where mean = 100, SD = 15, and floor is 50

ISSN: 1750-1172