Table 1

Patient demographics on enzyme replacement therapy

Post-treatmentBaselineYear 1Year 2Year 5Year 7Year 10
Age (year)
 Non-CKD534.3±11.2*35.3±11.236.3±11.236.1±9.238.1±9.241.1±9.2
 CKD543.6±8.8*44.6±8.844.7±9.244.6±8.045.3±8.045.6±5.0
Hypertension (%)
 Non-CKD520 (3/15)20 (3/15)20 (3/15)16.7 (2/12)16.7 (2/12)25 (3/12)
 CKD533.3 (3/10)44.4 (4/9)55.5 (5/10)66.6 (4/6)66.6 (4/6)60 (3/5)
Heart rate (bpm)
 Non-CKD555.4±7.554.9±8.353.6±5.953.8±7.354.6±11.652.9±7.0
 CKD564.6±16.859.5±1261.3±10.461.4±10.456±7.756.6±7.0
GFR (mL/min/1.73 m2)
 Non-CKD586.9±16.683.9±19.188.5±21.786.5±21.784.0±26.482.4±25.4
  • Non-CKD5 cohort size: baseline (N=15), year 1 (N=15), year 2 (N=15), year 5 (N=12), year 7 (N=12) and year 10 (N=12).

  • CKD5 cohort size: baseline (N=10), year 1 (N=9), year 2 (N=10), year 5 (N=6), year 7 (N=6) and year 10 (N=5).

  • *Baseline age non-CKD5 versus CKD5 not statistically different (p=0.09).

  • CKD5, chronic kidney disease stage 5; GFR, glomerular filtration rate as determined by renal clearance of radionuclide 51Cr-EDTA corrected to body surface area.