ICD-10-CM · Chapter 4 · E00-E89
E71 Disorders of branched-chain amino-acid metabolism and fatty-acid metabolism
Subcodes
Each subcode links to a detail page with full clinical annotations.
| Code | Description | Billable |
|---|---|---|
| E71..0 | Maple-syrup-urine disease | Yes |
| E71..1 | Other disorders of branched-chain amino-acid metabolism | No |
| E71..11 | Branched-chain organic acidurias | No |
| E71..110 | Isovaleric acidemia | Yes |
| E71..111 | 3-methylglutaconic aciduria | Yes |
| E71..118 | Other branched-chain organic acidurias | Yes |
| E71..12 | Disorders of propionate metabolism | No |
| E71..120 | Methylmalonic acidemia | Yes |
| E71..121 | Propionic acidemia | Yes |
| E71..128 | Other disorders of propionate metabolism | Yes |
| E71..19 | Other disorders of branched-chain amino-acid metabolism | Yes |
| E71..2 | Disorder of branched-chain amino-acid metabolism, unspecified | Yes |
| E71..3 | Disorders of fatty-acid metabolism | No |
| E71..30 | Disorder of fatty-acid metabolism, unspecified | Yes |
| E71..31 | Disorders of fatty-acid oxidation | No |
| E71..310 | Long chain/very long chain acyl CoA dehydrogenase deficiency | Yes |
| E71..311 | Medium chain acyl CoA dehydrogenase deficiency | Yes |
| E71..312 | Short chain acyl CoA dehydrogenase deficiency | Yes |
| E71..313 | Glutaric aciduria type II | Yes |
| E71..314 | Muscle carnitine palmitoyltransferase deficiency | Yes |
| E71..318 | Other disorders of fatty-acid oxidation | Yes |
| E71..32 | Disorders of ketone metabolism | Yes |
| E71..39 | Other disorders of fatty-acid metabolism | Yes |
| E71..4 | Disorders of carnitine metabolism | No |
| E71..40 | Disorder of carnitine metabolism, unspecified | Yes |
| E71..41 | Primary carnitine deficiency | Yes |
| E71..42 | Carnitine deficiency due to inborn errors of metabolism | Yes |
| E71..43 | Iatrogenic carnitine deficiency | Yes |
| E71..44 | Other secondary carnitine deficiency | No |
| E71..440 | Ruvalcaba-Myhre-Smith syndrome | Yes |
| E71..448 | Other secondary carnitine deficiency | Yes |
| E71..5 | Peroxisomal disorders | No |
| E71..50 | Peroxisomal disorder, unspecified | Yes |
| E71..51 | Disorders of peroxisome biogenesis | No |
| E71..510 | Zellweger syndrome | Yes |
| E71..511 | Neonatal adrenoleukodystrophy | Yes |
| E71..518 | Other disorders of peroxisome biogenesis | Yes |
| E71..52 | X-linked adrenoleukodystrophy | No |
| E71..520 | Childhood cerebral X-linked adrenoleukodystrophy | Yes |
| E71..521 | Adolescent X-linked adrenoleukodystrophy | Yes |
| E71..522 | Adrenomyeloneuropathy | Yes |
| E71..528 | Other X-linked adrenoleukodystrophy | Yes |
| E71..529 | X-linked adrenoleukodystrophy, unspecified type | Yes |
| E71..53 | Other group 2 peroxisomal disorders | Yes |
| E71..54 | Other peroxisomal disorders | No |
| E71..540 | Rhizomelic chondrodysplasia punctata | Yes |
| E71..541 | Zellweger-like syndrome | Yes |
| E71..542 | Other group 3 peroxisomal disorders | Yes |
| E71..548 | Other peroxisomal disorders | Yes |
Last reviewed: September 2026