- Metabolite
- HMDB0000754 (3-Hydroxyisovaleric acid)
- Biospecimen
- Urine
- Status
- Detected and Quantified
- Data source
- Referenced
- Concentration
- <50 umol/mmol creatinine
- Age
- Children (1 - 13 years old)
- Sex
- Female
- Condition
- Normal
References
- Baumgartner ER, Suormala TM, Wick H, Probst A, Blauenstein U, Bachmann C, Vest M: Biotinidase deficiency: a cause of subacute necrotizing encephalomyelopathy (Leigh syndrome). Report of a case with lethal outcome. Pediatr Res. 1989 Sep;26(3):260-6. [PubMed:2587127 ]