Table 2.
Demographic, renal functional and glomerular structural parameters of Fabry patients and controls.
| Fabry Males (n=8) | Fabry Females (n=6) | Fabry Total (n=14) | Controls (M/F=6/3) (n=9) | p-value | |
|---|---|---|---|---|---|
| Age (years) | 14[4–19] | 12[8–14 | 12[4–19] | 32[16–52] | 0.0005‡, 0.0008*, 0.0009** |
| UPCR (mg/g) | 55±90 | 18±25 | 40±71 | NA | NS† |
| Serum Cr (mg/dl) | 0.70±0.15 (n=6) | 0.63±0.12 (n=5) | 0.65±0.13 (n=11) | NA | NS† |
| GFR (ml/min/1.73m2) | 120±37 (n=5) | 101±8 (n=5) | 111±27 (n=10) | NA | NS† |
| Vv(Inc/PC) | 0.37±0.11 | 0.26±0.06 | 0.32±0.11 | -- | 0.06† |
| Vv(Inc/Endo) | 0.19±0.12 | 0.03±0.03 | 0.12±0.12 | -- | 0.008† |
| Vv(Inc/Mes) | 0.08±0.06 | 0.03±0.03 | 0.06±0.05 | -- | 0.06† |
| FPW (nm) | 558±133 | 455±67 | 514±118 | 430±61 | 0.06‡, 0.06†, 0.01** |
| % endothelial fenestration | 48±14 | 49±9 | 48±12 | 61±9 | 0.01‡, 0.06*, 0.03** |
All values are mean±SD except for age and urine protein creatinine ratio which are median [range]. To convert UPCR from mg/g to mg/mmol; multiply by 0.887. which are median [range].
Female Fabry vs. control;
male Fabry vs. control;
male vs. female Fabry;
combined male and female Fabry vs. control.
Abbreviations: UPCR= urinary protein creatinine ratio; S/Cr= serum creatinine; GFR=glomerular filtration rate; Vv(Inc/PC)=fractional volume of podocyte GL-3 inclusions per cytoplasm; Vv(Inc/Endo)= fractional volume of endothelial cell GL-3 inclusions per cytoplasm; Vv(Inc/Mes)=mesangial cell GL-3 inclusions per cytoplasm; FPW=foot process width