Table 3 Biochemical and visual outcomes at most recent clinic visit using current management strategies
From: Clinical, biochemical and molecular analysis in a cohort of individuals with gyrate atrophy
Patient no | Compliance with current treatment (Y/N/P)§ | Protein restriction (daily intake [g/kg] excluding EAA) | Daily Lysine Supplementation (g) | Current additional amino acid supplement regime with total daily dosage (where available) | Plasma ornithine at diagnosis (NR: 40–150 μmol/L) | Average plasma ornithine in last 5 years (μmol/L) | Average plasma lysine in last 5 years (NR: 100–160 μmol/L) | Average visual acuity at last ophthalmic examination (logMAR) |
|---|---|---|---|---|---|---|---|---|
1 | P | No restriction | 5 | None | 1120 | 1067 | 87 | 0.7 |
2 | N | No restriction | 0 | None | 1018 | 1342 | 73 | 0.61 |
3 | P | 0.5–0.6 | 0 | None | 1007 | 863 | 72 | 0.23 |
4 | Y | 1 | 4 | EAA (3–4 sachets) | 458 | 868 | 84 | 0.38 |
5 | P | No restriction | 4 | EAA (3–4 sachets) | 695 | 564 | 87 | 0.36 |
7 | Y | 0.5–0.8 | 0 | EAA (4 sachets) UCD Amino 5 (1 sachet) Dialamine (100 g) | 576 | 528 | 150 | 0.1 |
8 | Y | 0.2–0.3 | 0 | EAA (4 sachets) | 754 | 597 | 130 | 0.34 |
9 | Y | 1 | 4 | None | 775 | 640 | 106 | 0.57 |
10 | Y | 0.7–1 | 10 | None | 826 | 572 | 76 | 0.92 |
11 | P | No restriction | EAA Pyridoxine (300 mg) | 800 | 718 | 204 | 0.55 | |
12 | P | No restriction | 10 | EAA (4 sachets) | 734 | 641 | 103 | 0.35 |
13 | Y | 0.15 | 0 | Dialamine (22 scoops) Phlexyvits Zinc biotin | 458 | 392 | 169 | 0.23 |
14 | Y | 0.4 | 0 | EAA (3 sachets) Docomega | 1232 | 879 | 195 | 0.0 |
15 | N | No restriction | 4 | None | 742 | 716 | 108 | 0.31 |
17 | Y | 0.5–0.6 | 0 | Dialamine (100 g) Fruityvit | + | 458 | 247 | 0.36 |