Is E71 a billable code?
No. E71 is a header code that groups 38 more specific codes. Use one of the billable codes listed on this page instead.
ICD-10-CM FY2027 diagnosis code
E71: Disorders of branched-chain amino-acid metabolism and fatty-acid metabolism
Header code: not billableValid for dates of service October 1, 2026 to September 30, 2027
E71 groups related diagnoses under disorders of branched-chain amino-acid metabolism and fatty-acid metabolism. It cannot be used on a claim by itself. Choose the billable code below that matches the patient's record.
Billable codes under E71
- E71.0Maple-syrup-urine disease
- 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
- E71.19Other disorders of branched-chain amino-acid metabolism
- E71.2Disorder of branched-chain amino-acid metabolism, unspecified
- E71.30Disorder of fatty-acid metabolism, unspecified
- 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
- E71.32Disorders 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
- E71.440Ruvalcaba-Myhre-Smith syndrome
- E71.448Other secondary carnitine deficiency
- E71.50Peroxisomal disorder, unspecified
- 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
- E71.53Other group 2 peroxisomal disorders
- E71.540Rhizomelic chondrodysplasia punctata
- E71.541Zellweger-like syndrome
- E71.542Other group 3 peroxisomal disorders
- E71.548Other peroxisomal disorders
E71 questions
What does E71 mean?
E71 is the ICD-10-CM code for Disorders of branched-chain amino-acid metabolism and fatty-acid metabolism. It is in chapter 4, Endocrine, nutritional and metabolic diseases (E00-E89).
Code titles come from the official ICD-10-CM files published by the CDC National Center for Health Statistics. Always check payer rules and the clinical record before billing.