| ICD-10 Code | Description |
| E71.0 | Maple-syrup-urine disease |
| E71.110 | Isovaleric acidemia |
| E71.111 | 3-methylglutaconic aciduria |
| E71.118 | Other branched-chain organic acidurias |
| E71.120 | Methylmalonic acidemia |
| E71.121 | Propionic acidemia |
| E71.128 | Other disorders of propionate metabolism |
| E71.19 | Other disorders of branched-chain amino-acid metabolism |
| E71.2 | Disorder of branched-chain amino-acid metabolism, unspecified |
| E71.30 | Disorder of fatty-acid metabolism, unspecified |
| E71.310 | Long chain/very long chain acyl CoA dehydrogenase deficiency |
| E71.311 | Medium chain acyl CoA dehydrogenase deficiency |
| E71.312 | Short chain acyl CoA dehydrogenase deficiency |
| E71.313 | Glutaric aciduria type II |
| E71.314 | Muscle carnitine palmitoyltransferase deficiency |
| E71.318 | Other disorders of fatty-acid oxidation |
| E71.32 | Disorders of ketone metabolism |
| E71.39 | Other disorders of fatty-acid metabolism |
| E71.40 | Disorder of carnitine metabolism, unspecified |
| E71.41 | Primary carnitine deficiency |
| E71.42 | Carnitine deficiency due to inborn errors of metabolism |
| E71.43 | Iatrogenic carnitine deficiency |
| E71.440 | Ruvalcaba-Myhre-Smith syndrome |
| E71.448 | Other secondary carnitine deficiency |
| E71.50 | Peroxisomal disorder, unspecified |
| E71.510 | Zellweger syndrome |
| E71.511 | Neonatal adrenoleukodystrophy |
| E71.518 | Other disorders of peroxisome biogenesis |
| E71.520 | Childhood cerebral X-linked adrenoleukodystrophy |
| E71.521 | Adolescent X-linked adrenoleukodystrophy |
| E71.522 | Adrenomyeloneuropathy |
| E71.528 | Other X-linked adrenoleukodystrophy |
| E71.529 | X-linked adrenoleukodystrophy, unspecified type |
| E71.53 | Other group 2 peroxisomal disorders |
| E71.540 | Rhizomelic chondrodysplasia punctata |
| E71.541 | Zellweger-like syndrome |
| E71.542 | Other group 3 peroxisomal disorders |
| E71.548 | Other peroxisomal disorders |
