- Metabolite
- HMDB0000019 (alpha-Ketoisovaleric acid)
- Biospecimen
- Blood
- Status
- Detected and Quantified
- Data source
- Referenced
- Concentration
- 237 uM
- Age
- Children (1-13 years old)
- Sex
- Female
- Condition
- Maple syrup urine disease
Comments
n=1. While patient was in coma.
References
- Wendel, U., Becker, K., Przyrembel, H. et al. (1980). Peritoneal dialysis in maple-syrup-urine disease: Studies on branched-chain amino and keto acids. Eur J Pediatr (1980) 134: 57. https://doi.org/10.1007/BF00442404. Eur J Pediatr.