E71 — Disorders of branched-chain amino-acid metabolism and fatty-acid metabolism
Is E71 billable?
No — E71 is a non-billable header code. E71 groups more specific codes and cannot be submitted on a claim. A valid ICD-10-CM code must be reported at the highest level of specificity available in its branch. Select one of the 38 billable codes beneath E71.
Billable codes under this header
- E71.0Maple-syrup-urine disease
- E71.2Disorder of branched-chain amino-acid metabolism, unspecified
- E71.19Other disorders of branched-chain amino-acid metabolism
- E71.30Disorder of fatty-acid metabolism, unspecified
- E71.32Disorders of ketone metabolism
- E71.39Other disorders of fatty-acid metabolism
- E71.40Disorder of carnitine metabolism, unspecified
- E71.41Primary carnitine deficiency
- E71.42Carnitine deficiency due to inborn errors of metabolism
- E71.43Iatrogenic carnitine deficiency
- E71.50Peroxisomal disorder, unspecified
- E71.53Other group 2 peroxisomal disorders
- E71.110Isovaleric acidemia
- E71.1113-methylglutaconic aciduria
- E71.118Other branched-chain organic acidurias
- E71.120Methylmalonic acidemia
- E71.121Propionic acidemia
- E71.128Other disorders of propionate metabolism
- E71.310Long chain/very long chain acyl CoA dehydrogenase deficiency
- E71.311Medium chain acyl CoA dehydrogenase deficiency
- E71.312Short chain acyl CoA dehydrogenase deficiency
- E71.313Glutaric aciduria type II
- E71.314Muscle carnitine palmitoyltransferase deficiency
- E71.318Other disorders of fatty-acid oxidation
- E71.440Ruvalcaba-Myhre-Smith syndrome
- E71.448Other secondary carnitine deficiency
- E71.510Zellweger syndrome
- E71.511Neonatal adrenoleukodystrophy
- E71.518Other disorders of peroxisome biogenesis
- E71.520Childhood cerebral X-linked adrenoleukodystrophy
- E71.521Adolescent X-linked adrenoleukodystrophy
- E71.522Adrenomyeloneuropathy
- E71.528Other X-linked adrenoleukodystrophy
- E71.529X-linked adrenoleukodystrophy, unspecified type
- E71.540Rhizomelic chondrodysplasia punctata
- E71.541Zellweger-like syndrome
- E71.542Other group 3 peroxisomal disorders
- E71.548Other peroxisomal disorders
E71 at a glance
| Code | E71 |
|---|---|
| Description | Disorders of branched-chain amino-acid metabolism and fatty-acid metabolism |
| Billable | No — header code |
| Code set | ICD-10-CM FY2027 |
| Valid for encounters | October 1, 2026 – September 30, 2027 |
Excludes1 — never code together — inherited from Chapter 4
The conditions below can never be reported together with E71 on the same claim. An Excludes1 note means the two conditions cannot occur in the same patient, so reporting both is a coding error.
- transitory endocrine and metabolic disorders specific to newborn (P70-P74)
Excludes1 — never code together — inherited from E70-E88
The conditions below can never be reported together with E71 on the same claim. An Excludes1 note means the two conditions cannot occur in the same patient, so reporting both is a coding error.
Excludes2 — not included here — inherited from E70-E88
The conditions below are not part of E71, but a patient may have both at the same time. When documentation supports it, E71 and the excluded code may both be reported.
- Ehlers-Danlos syndromes (Q79.6-)
What codes fall under E71? (6)
- E71.0Maple-syrup-urine disease
- E71.1Other disorders of branched-chain amino-acid metabolismnot billable
- E71.2Disorder of branched-chain amino-acid metabolism, unspecified
- E71.3Disorders of fatty-acid metabolismnot billable
- E71.4Disorders of carnitine metabolismnot billable
- E71.5Peroxisomal disordersnot billable
How long has E71 existed?
E71 has been in ICD-10-CM since at least FY2016, the earliest fiscal year on record here.
Derived from the official order files for FY2016–FY2026. FY2017 and FY2020 publish no order file, so those years are not covered.
Which codes are confused with E71?
ICD-10-CM declares these codes mutually exclusive with E71 — exactly one of each pair can be correct for a given encounter.