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

Table 2 Raw data for all patients. Ages are presented in months unless otherwise specified

ID	HCT?	Sex	Year seen	Visit #	Bayley edition	Age at test	Mental age	DQ	SS/MDI	Age at death (Years)
H201	No	female	1991	1	1	13.8	12.5	90.4	90	11.13
			1992	2	1	22.8	14.0	61.5	50
H203	No	male	1990	1	1	7.1	2.2	31.0	50	8.17
H205	No	male	1992	1	1	21.9	16.8	76.8	64	8.74
H207	No	male	1992	1	1	13.3	12.5	93.9	100	N.A.
H210	No	male	1990	1	1	42.7	19.0	44.5	67	10.48
H217	No	female	1995	1	2	40.4	20.6	50.9	50	N.A.
			1995	2	2	41.1	19.7	48.0	50
H218	No	male	1989	1	1	12.8	11.3	88.5	82	7.12
H220	No	male	1990	1	1	36.8	24.4	66.4	50	11.00
H223	No	male	1992	1	1	17.3	14.2	81.9	78	6.21
H225	No	female	1992	1	1	25.7	20.0	77.7	81	11.47
H226	No	male	1991	1	1	11.0	7.1	64.5	56	4.12
			1992	2	1	14.2	7.0	49.2	50
H228	No	female	1994	1	2	36.2	27.0	74.6	74	13.36
H232	No	female	1988	1	1	30.2	17.8	58.9	50	6.68
H238	No	male	1987	1	1	91.9	12.2	13.3	50	8.77
H239	No	female	1988	1	1	22.0	17.0	77.4	68	5.13
H240	No	male	1990	1	1	18.8	10.0	53.3	50	4.05
H247	No	female	1987	1	1	36.1	19.3	53.5	50	5.85
H249	No	female	1991	1	1	80.5	17.8	22.1	50	4.67
H291	No	female	1985	1	1	32.1	14.0	43.7	50	4.81
			1985	2	1	35.3	17.8	50.4	50
H328	No	female	1994	1	2	16.9	17.0	100.6	103	8.97
H341	No	male	1994	1	2	16.8	7.6	45.2	56	3.30
H357	No	male	1995	1	2	24.5	20.0	81.5	94	N.A.
H375	No	female	1995	1	2	16.1	7.8	48.4	63	8.14
H227	Yes	female	1994	1	2	15.8	14.6	92.4	94	7.71
			1994	2	2	20.9	17.8	85.0	85
H234	Yes	male	1988	1	1	26.8	19.3	71.9	66	6.38
H243	Yes	male	1989	1	1	14.4	12.0	83.3	82	1.22
H244	Yes	female	1993	1	1	20.4	17.6	86.3	81	1.64
H292	Yes	male	1986	1	1	11.0	11.8	107.3	106	3.70
H297	Yes	female	1989	1	1	15.2	14.2	93.2	98	1.83
H299	Yes	male	1993	1	1	13.5	12.4	91.9	97	1.72
H302	Yes	female	1988	1	1	25.6	17.6	68.7	57	4.38
			1988	2	1	32.7	20.6	63.1	63
H336	Yes	male	1992	1	1	12.9	12.2	94.4	94	1.85
			1992	2	1	18.5	14.6	78.8	76

Age at Test = age at time of neurocognitive testing
Mental Age = Bayley 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