ICD-10-CM category
E71: Disorders of branched-chain amino-acid metabolism and fatty-acid metabolism
Codes under E71 (Disorders of branched-chain amino-acid metabolism and fatty-acid metabolism). A ✓ marks a billable (fully specified) code; category codes without a ✓ are not billable on their own.
| Code | Description | Billable |
|---|---|---|
| E71.0 | Maple-syrup-urine disease | ✓ |
| E71.1 | Other disorders of branched-chain amino-acid metabolism | — |
| E71.2 | Disorder of branched-chain amino-acid metabolism, unspecified | ✓ |
| E71.3 | Disorders of fatty-acid metabolism | — |
| E71.4 | Disorders of carnitine metabolism | — |
| E71.5 | Peroxisomal disorders | — |
| E71.11 | Branched-chain organic acidurias | — |
| E71.12 | Disorders of propionate metabolism | — |
| E71.19 | Other disorders of branched-chain amino-acid metabolism | ✓ |
| E71.30 | Disorder of fatty-acid metabolism, unspecified | ✓ |
| E71.31 | 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 | ✓ |
| E71.44 | Other secondary carnitine deficiency | — |
| E71.50 | Peroxisomal disorder, unspecified | ✓ |
| E71.51 | Disorders of peroxisome biogenesis | — |
| E71.52 | X-linked adrenoleukodystrophy | — |
| E71.53 | Other group 2 peroxisomal disorders | ✓ |
| E71.54 | Other peroxisomal disorders | — |
| 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 | ✓ |
| E71.310 | 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 | ✓ |
| E71.440 | Ruvalcaba-Myhre-Smith syndrome | ✓ |
| E71.448 | Other secondary carnitine deficiency | ✓ |
| 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 | ✓ |
| E71.540 | Rhizomelic chondrodysplasia punctata | ✓ |
| E71.541 | Zellweger-like syndrome | ✓ |
| E71.542 | Other group 3 peroxisomal disorders | ✓ |
| E71.548 | Other peroxisomal disorders | ✓ |
More free billing tools: all ICD-10 categories · ICD-10 search · denial codes · Medicare fees
Informational only, not coding or medical advice. Verify against the official ICD-10-CM (FY2026) and your payer's guidelines.
Code the diagnosis right
Join providers across the country delivering modern virtual care with CareNiva. Free to start, ready in minutes.
100% Satisfaction Guaranteed — try it risk-free