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. 2017 Jul 28;10:351. doi: 10.1186/s13104-017-2681-y

Table 2.

Changes in mobility, pulmonary function, body mass index and bone mineral density after enzyme replacement therapy at the time of follow-up DXA

	Duration of ERT (months)	6MWT (m)		Walton score		FVC (L) (% predicted)		BMI (kg/m²)		BMD (g/cm²)			∆BMD (%)
	Duration of ERT (months)	Pre-ERT	Post-ERT	Pre-ERT	Post-ERT	Pre-ERT	Post-ERT	Pre-ERT	Post-ERT		Pre-ERT	Post-ERT	∆BMD (%)
Patient 1	62	NA	NA	7	7	0.94 (20%)	1.02 (22%)	14.2	17.0	33% radius^a	0.765	0.825	+7.8
										Z-score	−1.9	−1.3	+7.8
										Hip total	0.675	0.749	+11
										Z-score	−2.5	−1.9	+11
Patient 2	61	473	630	2	2	1.63 (38%)	2.32 (54%)	16.2	19.6	Spine L1–L4	1.209	1.328	+9.8
										Z-score	0.9	2.0	+9.8
										Hip total	0.928	0.939	+1.2
										Z-score	−0.5	−0.4	+1.2
Patient 3	61	429	425	2	2	0.74 (28%)	0.70 (27%)	14.3	13.6	Spine L1–L4	0.959	1.027	+7.1
										Z-score	−1.3	−0.7	+7.1
										Hip total	0.576	0.642	+11.5
										Z-score	−3.1	−2.4	+11.5
Patient 4	33	364	428	2.5	2	0.97 (36%)	0.99 (38%)	16.2	20.6	Spine L1–L4	0.940	1.341	+42.7
										Z-score	−1.5	1.9	+42.7
										Hip total	0.41	0.773	+84.4
										Z-score	−4.3	−1.5	+84.4

Patients 1 and 2 were brothers, patients 3 and 4 were sisters

FVC forced vital capacity, BMI body mass index, NA not applicable

^aPatient 1 had spinal surgery for scoliosis, BMD was measured from forearm instead of lumbar vertebra