E71 Disord of branched-chain amino-acid metab & fatty-acid metab
ICD-10-CM category E71 sits in Chapter 4 (Endocrine, nutritional and metabolic diseases, E00-E89). It contains 38 billable codes for FY2027, effective October 1, 2026 to September 30, 2027. Always code to the most specific code available: a code with more characters that describes the diagnosis more precisely takes priority over a shorter one.
Subcategories
E71.1 Other disorders of branched-chain amino-acid metabolismE71.11 Branched-chain organic aciduriasE71.12 Disorders of propionate metabolismE71.3 Disorders of fatty-acid metabolismE71.31 Disorders of fatty-acid oxidationE71.4 Disorders of carnitine metabolismE71.44 Other secondary carnitine deficiencyE71.5 Peroxisomal disordersE71.51 Disorders of peroxisome biogenesisE71.52 X-linked adrenoleukodystrophyE71.54 Other peroxisomal disorders
Billable codes in E71
| 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 |
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