Table 1. Metabolic analysis of organic acids (excretion in urine) and acylcarnitine (plasma concentration) in patient as compared with controls.
| Acylcarnitines | μmol/l | Ref range | Conclusion |
|---|---|---|---|
| C4/(Iso)Butyrylcarnitine | 3.09 | 0.04–0.82 | Increased |
| C5/Isovalerylcarnitine + 2-methylbutyrylcarnitine | 2.74 | 0.06–0.50 | Increased |
| C6/Hexanoylcarnitine | 0.84 | 0.02–0.16 | Increased |
| C8/Octanoylcarnitine | 2.99 | 0.02–0.77 | Increased |
| C10/Decanoylcarnitine | 2.46 | 0.03–0.87 | Increased |
| C10:1/Decenoylcarnitine | 0.99 | 0.03–0.46 | Increased |
| C5-DC/Glutarylcarnitine | 0.30 | 0–0.10 | Increased |
| C12/Dodecanoylcarnitine | 0.25 | 0.01–0.25 | Increased—within ref range |
| C14/Tetradecanoylcarnitine | 0.06 | 0–0.11 | Within ref range |
| C14:1/Tetradecenoylcarnitine | 0.19 | 0–0.23 | Within ref range |
| C16/Hexadecanoylcarnitine | 0.12 | 0.03–0.22 | Within ref range |
| C16:1/Hexadecenoylcarnitine | 0.04 | 0–0.09 | Within ref range |
| C18/Octadecanoylcarnitine/Steroylcarnitine | 0.11 | 0.01–0.13 | Within ref range |
| C18:1/Oleylcarnitine | 0.12 | 0.02–0.38 | Within ref range |
| C18:2/Linoleylcarnitine | 0.07 | 0.02–0.23 | Within ref range |
| Organic acids | μmol/mmol creatinine | Ref range | Conclusion |
| Isovalerylglycine | 61.4 | 0–1.2 | Increased |
| 3-hydroxy-isovaleric acid | 7.6 | 6.0–49.0 | Within ref range |
| 2-methylbutyrylglycine | 2.2 | 0–0.4 | Increased |
| Isobutyrylglycine | 3.5 | 0–1.1 | increased |
| Glutaric acid | 1.3 | 1.0–4.0 | Within ref range |
| 2-hydroxy-glutaric acid | 15.5 | 4.0–16.0 | Within ref range |
| Ethylmalonic acid | 68.7 | 2.0–10.0 | Increased |
| Hexanoylglycine | 1.3 | 0–0.3 | Increased |
| Suberylglycine | 0.2 | 0–0.1 | Increased |
| Adipic acid | 2.6 | 1.0–7.0 | Within ref range |
| Suberic acid | 0.9 | 1.0–6.0 | Within ref range |
| Sebacic acid | 0.6 | 0 | Increased |
| 3-hydroxy-sebacic acid | 0.5 | 0–4.0 | Within ref range |
Metabolic investigations were performed before riboflavin supplementation.