Table 2 Comparison of biochemical data in blood and urine before and after treatment
C5-OH (μmol/L) | C3 (μmol/L) | C3/C2 | C5-OH/C3 | Pyruvate (mmol/mol Cr)) | 3-hydroxypropionate (mmol/mol Cr) | 3-hydroxyvaleric acid (mmol/mol Cr)) | 3-methylcrotonylglycine (mmol/mol Cr)) | Methylcitric acid (mmol/mol Cr)) | |
|---|---|---|---|---|---|---|---|---|---|
Before treatment (Median, range) | 7.01 (3.26–15.01) | 4.34 (0.61–14) | 0.15 (0.03–0.63) | 1.82 (0.12–11.59) | 42.05 (4.52–990) | 16.21 (4.07–470.3) | 212.8 (7.5–550.4) | 39.7 (8.54–177) | 3.94 (0.9–42.24) |
After treatment (Median, range) | 0.35 (0.08–1.87) | 1.64 (0.62–2.98) | 0.08 (0.02–0.11) | 0.27 (0.08–4.97) | 2.32 (0.0–11.7) | 0 (0.0–3.5) | 1.1 (0.0–43.32) | 1.01 (0.0–3.7) | 0.17 (0.0–1.6) |
P value | < 0.001 | 0.007 | 0.007 | 0.028 | 0.013 | 0.025 | < 0.001 | < 0.001 | 0.004 |
Reference range | 0.06–0.6 | 0.5–4.0 | 0.0–0.2 | 0.04–0.25 | 0.0–30.0 | 0.0–4.0 | 0.0–4.0 | 0.0–5.0 | 0.0–0.7 |