Table 1.

Clinical characteristics of Fabry registry patients grouped by renal disease progression status

MenWomen
Faster Progression (eGFR Slope ≤ −1)Slower Progression (eGFR Slope > −1)Faster Progression (eGFR Slope ≤ −1)Slower Progression (eGFR Slope > −1)
Patients with eGFR data, n8635133208
    age at baseline, mean (SD), years36 (14)30 (10)b41 (12)39 (14)
    eGFRa at baseline, mean (SD)89 (29)94 (22)96 (23)95 (21)
Follow up time, mean (SD), years5.1 (4.71)4.2 (4.11)4.3 (3.95)4.3 (3.6)
    median (25th, 75th)3.2 (2.0, 6.7)2.4 (1.4, 5.3)2.8 (2.0, 5.4)3.4 (2.1, 5.1)
Patients with baseline UP/Cr data, n371167111
    UP/Cr, baseline mean (SD)1.4 (1.2)0.4 (0.85)b1.3 (1.5)0.5 (0.7)b
    median (25th, 75th)1.1 (0.7, 1.7)0.1 (0.1, 0.2)0.6 (0.2, 2.0)0.1 (0.1, 0.8)
Patients with averaged UP/Cr data, n8635133208
    UP/Cr, averaged mean (SD)1.5 (1.2)0.2 (0.3)b1.4 (1.5)0.5 (0.7)b
    median (25th, 75th)1.1 (0.7, 1.9)0.1 (0.1, 0.2)0.5 (0.2, 2.1)0.2 (0.1, 1.8)
BP, n7632124198
    systolic BP, averaged mean (SD), mmHg127 (12)127 (11)124 (14)122 (15)
    median (25th, 75th)127 (118, 135)128 (121, 132)123 (114, 135)121 (111, 131)
    diastolic BP, averaged mean (SD), mmHg78 (8)73 (8)b77 (9)75 (9)
    median (25th, 75th)78 (72, 84)73 (70, 76)77 (69, 84)75 (69, 82)
Reported history of ACEi/ARB use, n (%)20 (23)6 (17)29 (22)35 (17)
  • All data are from adult Fabry Registry patients (≥18 years) with two or more eGFR assessments (calculated by the chronic kidney disease epidemiology collaboration equation) over a period of ≥12 months during the natural history period (i.e., before any treatment with enzyme replacement therapy) and before any chronic dialysis or renal transplant events. “Averaged” data reflect the average of all values reported within 6 months of the date of the first eGFR assessment to the most recent assessment.

  • a eGFR data are expressed as ml/min per 1.73 m2.

  • b P < 0.05 for patients with faster versus slower progression within each gender (see Materials and Methods section for description of statistical analyses used for specific clinical parameters).