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 | ✓ |
| E71.0 | Maple-syrup-urine disease | ✓ |
| E71.1 | Other disorders of branched-chain amino-acid metabolism | — |
| E71.1 | Other disorders of branched-chain amino-acid metabolism | — |
| E71.2 | Disorder of branched-chain amino-acid metabolism, unspecified | ✓ |
| E71.2 | Disorder of branched-chain amino-acid metabolism, unspecified | ✓ |
| E71.3 | Disorders of fatty-acid metabolism | — |
| E71.3 | Disorders of fatty-acid metabolism | — |
| E71.4 | Disorders of carnitine metabolism | — |
| E71.4 | Disorders of carnitine metabolism | — |
| E71.5 | Peroxisomal disorders | — |
| E71.5 | Peroxisomal disorders | — |
| E71.11 | Branched-chain organic acidurias | — |
| E71.11 | Branched-chain organic acidurias | — |
| E71.12 | Disorders of propionate metabolism | — |
| E71.12 | Disorders of propionate metabolism | — |
| E71.19 | Other disorders of branched-chain amino-acid metabolism | ✓ |
| E71.19 | Other disorders of branched-chain amino-acid metabolism | ✓ |
| E71.30 | Disorder of fatty-acid metabolism, unspecified | ✓ |
| E71.30 | Disorder of fatty-acid metabolism, unspecified | ✓ |
| E71.31 | Disorders of fatty-acid oxidation | — |
| E71.31 | Disorders of fatty-acid oxidation | — |
| E71.32 | Disorders of ketone metabolism | ✓ |
| E71.32 | Disorders of ketone metabolism | ✓ |
| E71.39 | Other disorders of fatty-acid metabolism | ✓ |
| E71.39 | Other disorders of fatty-acid metabolism | ✓ |
| E71.40 | Disorder of carnitine metabolism, unspecified | ✓ |
| E71.40 | Disorder of carnitine metabolism, unspecified | ✓ |
| E71.41 | Primary carnitine deficiency | ✓ |
| E71.41 | Primary carnitine deficiency | ✓ |
| E71.42 | Carnitine deficiency due to inborn errors of metabolism | ✓ |
| E71.42 | Carnitine deficiency due to inborn errors of metabolism | ✓ |
| E71.43 | Iatrogenic carnitine deficiency | ✓ |
| E71.43 | Iatrogenic carnitine deficiency | ✓ |
| E71.44 | Other secondary carnitine deficiency | — |
| E71.44 | Other secondary carnitine deficiency | — |
| E71.50 | Peroxisomal disorder, unspecified | ✓ |
| E71.50 | Peroxisomal disorder, unspecified | ✓ |
| E71.51 | Disorders of peroxisome biogenesis | — |
| E71.51 | Disorders of peroxisome biogenesis | — |
| E71.52 | X-linked adrenoleukodystrophy | — |
| E71.52 | X-linked adrenoleukodystrophy | — |
| E71.53 | Other group 2 peroxisomal disorders | ✓ |
| E71.53 | Other group 2 peroxisomal disorders | ✓ |
| E71.54 | Other peroxisomal disorders | — |
| E71.54 | Other peroxisomal disorders | — |
| E71.110 | Isovaleric acidemia | ✓ |
| E71.110 | Isovaleric acidemia | ✓ |
| E71.111 | 3-methylglutaconic aciduria | ✓ |
| E71.111 | 3-methylglutaconic aciduria | ✓ |
| E71.118 | Other branched-chain organic acidurias | ✓ |
| E71.118 | Other branched-chain organic acidurias | ✓ |
| E71.120 | Methylmalonic acidemia | ✓ |
| E71.120 | Methylmalonic acidemia | ✓ |
| E71.121 | Propionic acidemia | ✓ |
| E71.121 | Propionic acidemia | ✓ |
| E71.128 | Other disorders of propionate metabolism | ✓ |
| E71.128 | Other disorders of propionate metabolism | ✓ |
| E71.310 | Long chain/very long chain acyl CoA dehydrogenase deficiency | ✓ |
| E71.310 | Long chain/very long chain acyl CoA dehydrogenase deficiency | ✓ |
| E71.311 | Medium chain acyl CoA dehydrogenase deficiency | ✓ |
| E71.311 | Medium chain acyl CoA dehydrogenase deficiency | ✓ |
| E71.312 | Short chain acyl CoA dehydrogenase deficiency | ✓ |
| E71.312 | Short chain acyl CoA dehydrogenase deficiency | ✓ |
| E71.313 | Glutaric aciduria type II | ✓ |
| E71.313 | Glutaric aciduria type II | ✓ |
| E71.314 | Muscle carnitine palmitoyltransferase deficiency | ✓ |
| E71.314 | Muscle carnitine palmitoyltransferase deficiency | ✓ |
| E71.318 | Other disorders of fatty-acid oxidation | ✓ |
| E71.318 | Other disorders of fatty-acid oxidation | ✓ |
| E71.440 | Ruvalcaba-Myhre-Smith syndrome | ✓ |
| E71.440 | Ruvalcaba-Myhre-Smith syndrome | ✓ |
| E71.448 | Other secondary carnitine deficiency | ✓ |
| E71.448 | Other secondary carnitine deficiency | ✓ |
| E71.510 | Zellweger syndrome | ✓ |
| E71.510 | Zellweger syndrome | ✓ |
| E71.511 | Neonatal adrenoleukodystrophy | ✓ |
| E71.511 | Neonatal adrenoleukodystrophy | ✓ |
| E71.518 | Other disorders of peroxisome biogenesis | ✓ |
| E71.518 | Other disorders of peroxisome biogenesis | ✓ |
| E71.520 | Childhood cerebral X-linked adrenoleukodystrophy | ✓ |
| E71.520 | Childhood cerebral X-linked adrenoleukodystrophy | ✓ |
| E71.521 | Adolescent X-linked adrenoleukodystrophy | ✓ |
| E71.521 | Adolescent X-linked adrenoleukodystrophy | ✓ |
| E71.522 | Adrenomyeloneuropathy | ✓ |
| E71.522 | Adrenomyeloneuropathy | ✓ |
| E71.528 | Other X-linked adrenoleukodystrophy | ✓ |
| E71.528 | Other X-linked adrenoleukodystrophy | ✓ |
| E71.529 | X-linked adrenoleukodystrophy, unspecified type | ✓ |
| E71.529 | X-linked adrenoleukodystrophy, unspecified type | ✓ |
| E71.540 | Rhizomelic chondrodysplasia punctata | ✓ |
| E71.540 | Rhizomelic chondrodysplasia punctata | ✓ |
| E71.541 | Zellweger-like syndrome | ✓ |
| E71.541 | Zellweger-like syndrome | ✓ |
| E71.542 | Other group 3 peroxisomal disorders | ✓ |
| E71.542 | Other group 3 peroxisomal disorders | ✓ |
| E71.548 | Other peroxisomal disorders | ✓ |
| E71.548 | Other peroxisomal disorders | ✓ |
Frequently asked questions
What is ICD-10-CM code E71?
E71 is an ICD-10-CM category covering Disorders of branched-chain amino-acid metabolism and fatty-acid metabolism. It contains 98 more specific diagnosis codes, 76 of which are billable.
Is E71 a billable code?
No — E71 is a category header and isn't billable on its own. Code to the highest level of specificity using one of its subcodes (for example E71.0 — Maple-syrup-urine disease). 76 of the 98 codes under E71 are billable.
What codes fall under E71?
E71 includes E71.0, E71.0, E71.1, E71.1, E71.2, plus 93 more. See the full list with descriptions and billable status above.
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Informational only, not coding or medical advice. Verify against the official ICD-10-CM (FY2026) and your payer's guidelines.
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