Research Article

Expression of uPAR in Urinary Podocytes of Patients with Fabry Disease

Table 2

General characteristics of untreated (no ERT) and ERT-treated Fabry patients.

VariablesNo ERT (: 23)ERT (: 21) value

Age (years)19 (11–75)35.8 (17–86)0.03
Gender (males)4 (14%)13 (59%)0.01
Hypertension1 (4.3%)5 (24%)0.0001
White matter ischaemia4 (17%)14 (68%)0.003
Myocardial hypertrophy1 (4%)5 (24%)0.001
Time on ERT (months)040 (34–50)<0.0001
eGFR (ml/min/1.73 m2)141 (60–165)131.5 (62–148)0.06
UPCR (g/g)0.06 (0.02–2.35)0.10 (0.02–5.68)0.04
Urinary uPAR+ podocytes/creatininuria (cells/g)40.18 (9.51–284.46)20.18 (0–191.26)0.009
MutationsD33G, L415P, R227X, A292T, N34D, C801, C326, C647A, C281, G640CD33G, L415P, R227X, A292T, N34D, D264Y, D155H, L180F

ERT, enzyme replacement therapy. Diagnosed by magnetic resonance imaging; asymptomatic cases, performed for screening purposes.