Table 5.
Scoring of podocytes, GL3 inclusions, vacuolization, and composite scores
| Patient No. | Age (yr)/Sex | Time (yr) | Score Osmicated Toluidine Semithin Sections | Score PAS Sections | Composite Score | |||
| No Scorable Glomeruli | Inclusions (0–4) | No Scorable Glomeruli | Vacuolization (0–3) | No Scorable Glomeruli | Inclusions and Vacuolization (0–7) | |||
| 1 | 7/M | 0 | 3 | 4.0 | 31 | 3.0 | 34 | 7.0 |
| 5 | 8 | 0.13 | 12 | 0.25 | 20 | 0.4 | ||
| 2 | 11/M | 0 | 23 | 4.0 | 29 | 2.9 | 52 | 6.9 |
| 3 | 4 | 4.0 | 30 | 3.0 | 34 | 7.0 | ||
| 5 | 4 | 4.0 | 15 | 2.8 | 19 | 6.8 | ||
| 3 | 11/F | 0 | 3 | 4.0 | 32 | 3.0 | 35 | 7.0 |
| 5 | 15 | 1.53 | 12 | 1.33 | 27 | 2.9 | ||
| 4 | 12/M | 0 | 14 | 4.0 | 13 | 3.0 | 27 | 7.0 |
| 5 | 5 | 4.0 | 11 | 2.64 | 16 | 6.6 | ||
| 5 | 16/M | −2 | ||||||
| 0a | 3 | 4.0 | 10 | 2.9 | 13 | 6.9 | ||
| 1 | 4 | 2.25 | 10 | 1.3 | 14 | 3.6 | ||
| 5 | 6 | 0.67 | 16 | 1.1 | 22 | 1.8 | ||
| 6 | 16/M | 0 | 7 | 4.0 | 16 | 2.88 | 23 | 6.9 |
| 3 | 18 | 4.0 | 18 | 3.0 | 36 | 7.0 | ||
| 5 | 6 | 4.0 | 15 | 3.0 | 21 | 7.0 | ||
| 7 | 17/M | −2 | ||||||
| 0a | 3 | 4.0 | 9 | 3.0 | 12 | 7.0 | ||
| 1 | 21 | 4.0 | 9 | 3.0 | 30 | 7.0 | ||
| 5 | 4 | 4.0 | 14 | 3.0 | 18 | 7.0 | ||
| 8 | 17/M | 0 | 14 | 4.0 | 15 | 3.0 | 29 | 7.0 |
| 1 | 11 | 4.0 | 35 | 3.0 | 46 | 7.0 | ||
| 9 | 18/M | 0 | 6 | 4.0 | 19 | 3.0 | 25 | 7.0 |
| 1 | 2 | 4.0 | 15 | 3.0 | 17 | 7.0 | ||
| 5 | 6 | 2.33 | 12 | 2.58 | 18 | 4.9 | ||
| 10 | 23/M | 0 | 4 | 4.0 | 16 | 3.0 | 20 | 7.0 |
| 1 | 23 | 4.0 | 13 | 3.0 | 36 | 7.0 | ||
| 5 | 1 | 4.0 | 16 | 3.0 | 17 | 7.0 | ||
| 11 | 30/M | 0 | 5 | 4.0 | 20 | 2.85 | 25 | 6.9 |
| 1 | 3 | 4.0 | 24 | 3.0 | 27 | 7.0 | ||
| 5 | 2 | 4.0 | 14 | 3.0 | 16 | 7.0 | ||
| 12 | 33/M | −3 | ||||||
| 0b | 26 | 4.0 | 16 | 3.0 | 42 | 7.0 | ||
| 5 | 30 | 4.0 | 12 | 3.0 | 42 | 7.0 | ||
Time, time of biopsy: 0 is baseline, numbers represent years after baseline; M, male; F, female.
a
Two years of ERT at baseline.
b
Three years of ERT at baseline.