3-hydroxyisobutyric aciduria https://rarediseases.info.nih.gov/diseases/5662/3-hydroxyisobutyric-aciduria https://www.malacards.org/card/3_hydroxyisobutyric_aciduria UMLS:C0342737 MEDGEN:90996 MESH:C535312 MONDO:0009371 3-hydroxyisobutyric aciduria GARD:0005662 SCTID:237957007 disorder of valine metabolism OMIM:236795 Orphanet:939 icd11.foundation:1293648631 ICD9:791.9 3 hydroxyisobutyric aciduria is characterized by ketoacidotic episodes, cerebral anomalies and facial dysmorphism. It is an organic aciduria that involves valine metabolism. Thirteen cases have been described in the literature so far. Transmission is thought to be autosomal recessive. classic organic aciduria inborn disorder of branched-chain amino acid metabolism valine metabolism disease