Assessment of bone mineral density by dual energy x-ray absorptiometry in patients with mucopolysaccharidoses

Table 4 Baseline and follow-up data of the various parameters for 8 MPS patients underwent ERT

No.	MPS type	Gender	DXA	Age (yr)	ERT duration (yr)	Height z score	Weight z score	BMI z score	BMD (g/cm²)	BMD z score	HAZ adjusted BMD z score
The initiation of ERT at pre-pubertal age
1	MPS II (M*)	M	Baseline	5	2.5	−1.63	−0.69	−0.17	0.53	−0.81	−0.31
1	MPS II (M*)	M	Follow-up	7.5	2.5	−1.22	−0.77	−0.39	0.561	−1.48	−1.01
2	MPS VI	M	Baseline	6.6	3.8	−3.69	−1.69	0.17	0.496	−1.65	−0.26
2	MPS VI	M	Follow-up	10.4	3.8	−5.92	−1.82	0.11	0.51	−2.75	0.09
3	MPS VI	M	Baseline	8.7	5.1	−6.52	−1.69	1.29	0.442	−2.35	0.49
3	MPS VI	M	Follow-up	13.8	5.1	−7.83	−2.55	0.16	0.492	−3.20	1.25
4	MPS VI	F	Baseline	11.7	7.4	−5.88	−1.57	1.18	0.65	−2.05	1.07
4	MPS VI	F	Follow-up	19.1	7.4	−8.7	−2.23	2.06	0.671	−3.10	2.83
5	MPS II (M*)	M	Baseline	13.2	4.5	−2.77	−1.12	0.00	0.636	−2.07	−0.50
5	MPS II (M*)	M	Follow-up	17.7	4.5	−3.2	−0.68	0.51	0.867	−3.99	−1.76
The initiation of ERT at post-pubertal age
6	MPS II (M*)	M	Baseline	17.5	1.0	−4.47	−1.84	−0.62	0.774	−5.06	−2.02
6	MPS II (M*)	M	Follow-up	18.5	1.0	−3.97	−1.62	−0.49	0.796	−4.80	−1.99
7	MPS I (H/S**)	M	Baseline	18.5	5.3	−7.69	−2.13	0.53	0.648	−6.51	−1.18
7	MPS I (H/S**)	M	Follow-up	23.8	5.3	−7.23	−1.77	1	0.769	−5.11	NA
8	MPS VI	F	Baseline	22.8	4.5	−8.88	−3.25	−0.11	0.712	−2.82	NA
			Follow-up	27.3	4.5	−9.77	−3.14	0.81	0.789	−2.3	NA

MPS, mucopolysaccharidoses; M*, mild form; H/S**, Hurler/Scheie; DXA, dual energy x-ray absorptiometry; ERT, enzyme replacement therapy; z score, standard deviation score; BMI, body mass index; BMD, bone mineral density; HAZ, height-for-age z score; NA, not available.

ISSN: 1750-1172