Table 1.

Progression rates, baseline proteinuria and GFR, and ERT dosing in Fabry nephropathy

Reference	Number	Male/female	ERT	Duration (months)	Baseline values			Proteinuria during ERT (mg/day)	Progression rate2
					Age (yr)	eGFR1	Proteinuria (mg/day)
(Warnock 2005)	1	1	Agalsidase-alfa; 0.2 mg/kg EOW	16	40.4	72	1510	350	−13.7 ± 1.50
(Schwarting et al 2006) #7,10,12,14	4	1/3	Agalsidase-alfa; 0.2 mg/kg EOW	12	57 ± 5	45 ± 5	203 ± 162	295 ± 208	−5.00 ± 4.08
(Schiffmann et al 2007) #3,8,9,7	4	4/0	Agalsidase-alfa; 0.2 mg/kg EOW	46	46 ± 7	90 ± 15	216 ± 79	287 ± 175	−6.63 ± 2.06
Weighted Mean ± SD				27.6	50.3 ± 5.3	68.0 ± 8.9	354 ± 107	298 ± 170	−6.69 ± 2.90
(Warnock 2005)	1	1	Agalsidase-beta; 1.0 mg/kg EOW	65	41.9	48.7	350	555 ± 237	−2.92 ± 0.44
(Breunig et al 2006)#9,10,11,12,20,21,22	7	4/3	Agalsidase-beta; 1.0 mg/kg EOW	22	40 ± 12	95 ± 20	152 ± 188	146 ± 171	−4.66 ± 6.86
(Schiffmann et al 2007) #3,8,9,7	4	4/0	Agalsidase-alfa; 0.2 mg/kg weekly	24	50 ± 7	62 ± 15	203 ± 60	260 ± 1725	4.20 ± 5.40
(Germain et al 2007)	42	40/2	Agalsidase-beta, 1.0 mg/kg EOW	52	29 ± 10	137 ± 50	277 ± 213	245 ± 247	−1.01 ± 0.97
(Tahir et al 2007) #3,4,5,8	4	2/2	Agalsidase-beta, 1.0 mg/kg EOW	30	32 ± 10	97 ± 17	449 ± 433	361 ± 245	1.18 ± 2.78
Weighted mean ± SD				45.2	32.2 ± 9.9	123 ± 41	270 ± 210	247 ± 232	−0.97 ±; 2.10

¹Estimated GFR calculated with the MRDR equation (mL/min/1.73 m²).

²Progression rate: annualized rate of change of the slope of the linear regression of the GFR (mL/min/1.73 m²/year).

Abbreviations: EOW, every other week; ERT, enzyme replacement therapy; GFR, glomerular filtration rate.