When is ICD-10 code E71 used?

Doctors, hospitals, and insurers may use ICD-10 code E71 when recording disorders of branched-chain amino-acid metabolism and fatty-acid metabolism in a standardized format. This entry may also act as a broader category that points toward more specific child codes.

Does ICD-10 code E71 confirm a diagnosis?

No. A code helps standardize documentation and claims, but it should not be read as a substitute for a clinician's full assessment.

ICD10

ICD-10 Code E71: Disorders of branched-chain amino-acid metabolism and fatty-acid metabolism

Name: ICD-10 Code E71: Disorders of branched-chain amino-acid metabolism and fatty-acid metabolism
Creator: HealthAssure Clinical Team
Published: 2026-05-21T06:05:20.893Z

E71 is an ICD-10 diagnosis entry used to group records related to Disorders of branched-chain amino-acid metabolism and fatty-acid metabolism. You may see this entry in coding references, medical records, or claims workflows when a broader diagnosis category is being reviewed before a more specific code is chosen. ICD-10 entries help standardize how diagnoses are organized for coding, reporting, analytics, and documentation. This code sits within the broader ICD-10 area for Endocrine, nutritional and metabolic diseases (E00-E89).

Reviewed by HealthAssure Clinical TeamUpdated 21 May 2026

What is this code?

ICD-10 entries help standardize how diagnoses are organized for coding, reporting, analytics, and documentation. This code sits within the broader ICD-10 area for Endocrine, nutritional and metabolic diseases (E00-E89).

When is it used?

May be used when a clinician documents disorders of branched-chain amino-acid metabolism and fatty-acid metabolism in a patient's medical record.
May appear in hospital records, claims, referrals, and clinical documentation.
This code may act more like a grouping or parent code, so a more specific child code may be used in final documentation when available.

What it does not mean

A code alone does not explain severity, treatment plan, or outcome.
A medical code should not be treated as a substitute for a doctor's diagnosis or advice.
This entry may represent a broader category rather than the most specific billable code.

Code hierarchy

chapter

4Endocrine, nutritional and metabolic diseases (E00-E89)

block

E70-E88Metabolic disorders

currentE70-E88

Where you may see this code

You may see this entry in coding references, medical records, or claims workflows when a broader diagnosis category is being reviewed before a more specific code is chosen.

Related specialists

EndocrinologistGeneral Physician

Sibling codes

E70Disorders of aromatic amino-acid metabolism E72Other disorders of amino-acid metabolism E73Lactose intolerance E74Other disorders of carbohydrate metabolism E75Disorders of sphingolipid metabolism and other lipid storage disorders E76Disorders of glycosaminoglycan metabolism E77Disorders of glycoprotein metabolism E78Disorders of lipoprotein metabolism and other lipidemias E79Disorders of purine and pyrimidine metabolism E80Disorders of porphyrin and bilirubin metabolism E83Disorders of mineral metabolism E84Cystic fibrosis E85Amyloidosis E86Volume depletion E87Other disorders of fluid, electrolyte and acid-base balance E88Other and unspecified metabolic disorders

Coding guidelines

Compatibility

Legacy code aligns to an official FY 2026 category. Frontend should resolve to the official category page and surface the billable child codes.

Legacy codes

E71

Replacement codes

E71.0 — Maple-syrup-urine diseaseE71.2 — Disorder of branched-chain amino-acid metabolism, unspecified

Common synonyms

Disorders of branched-chain amino-acid metabolism and fatty-acid metabolism

Frequently asked questions

About this content

This page is prepared by HealthAssure's clinical team using official coding standards from ICD-10. AI tools assist with drafting explanations, which are then reviewed and verified by healthcare professionals for accuracy. This content is for informational purposes and does not replace professional medical advice. Meet our team.