Table 1 Characteristic urinary organic acids profile with evident increasing in 3-hydroxy-isovaleric, 3-methylglutaric, glutaric, 3-methyl-glutaconic, 3-hydroxy-3-methyl-glutaric acids and 3-methyl-crotonyl-glicine
From: A neonatal case of 3-hydroxy-3-methylglutaric-coenzyme A lyase deficiency
Organic acid | Concentration (μMol/mMol Creatinine) | Upper normal range (μMol/mMol Creatinine) |
|---|---|---|
3-hydroxy-3-methyl-glutaric | 1028.4 | 50 |
3-methyl-crotonyl-glycine | 27.6 | 2 |
3-hydroxy-isovaleric | 1154.8 | 46 |
3-methyl-glutaric | 348 | 7 |
glutaric | 1274.9 | 5 |
3-methyl-glutaconic | 4166.4 | 6 |