Table 2.

CRIM status, antibody titer, and clinical outcome

	Age			Antibody titer				Clinical outcome at 4 years of ERTb
Pt	At start of ERT (months)	Current (years)	CRIM status	Peak titer through 4 years of ERT	Titer at 4 years of ERTb	Last titer (years after start of ERT)		Alive	Invasive ventilationc		Motor status	LVMI in SD	Dose of ERT
1	0.1	5.6	+	1:6,250	1:1,250	1:6,250	(5.0)	Y	Y	2.7 (1:250)	Sittinge	−0.6	40f
2	0.5	8.0	+	1:1,250	1:250	1:250	(7.1)	Y	N		Walking	−0.7	20
3	1.2	8.5	+	1:6,250	1:1,250	1:6,250	(8.0)	Y	N		Walking	−1.5	40f
4	1.9	4.4 a	−	1:6,250	1:50	1:50	(3.4)	Y	Y	2.0 (1:6,250)	Sittinge	+0.9	20
5	2.2	5.2	+	1:31,250	1:31,250	1:31,250	(4.0)	Y	N		Sitting	+1.1	40f
6	2.4	4.1	+	1:31,250	1:31,250	1:31,250	(3.0)	Y	N		Walking	+3.7	40
7	3.0	4.3 a	−	1:156,250	1:156,250	1:156,250	(3.8)	Y	Y	2.2 (1:31,250)	Sitting	+4.4	40f
8	3.6	0.6 a	−	1:31,250	1:31,250	1:31,250	(0.3)	N	N		Minimal motor gain	+28.6	20
9	3.8	14.1	+	1:6,250	1:250	1:6,250	(12.8)	Y	N		Walking	+0.4	40f
10	7.2	14.3	+	1:31,250	1:6,250	1:156,250	(12.9)	Y	Y	0.6d	Tetraplegic	+4.9	40f
11	8.3	14.3	+	1:156,250	1:156,250	1:156,250	(11.5)	Y	Y	0.9 (1:250)	Tetraplegic	+4.5	40f

Pt Patient number, ERT enzyme-replacement therapy, CRIM cross reactive immunological material, LVMI left ventricular mass index, SD standard deviation

^aAge at death

^bLast available data are presented if patient did not yet receive 4 years of ERT

^cAge in years (antibody titer around that time)

^dPatient was ventilator dependent before start of ERT

^ePatients lost ability to walk

^fDose was augmented to 40 mg/kg/week during treatment