2027 ICD-10-CM Diagnosis Code E71Disorders of branched-chain amino-acid metabolism and fatty-acid metabolism

ICD-10-CM Codes›E00–E89›E70-E88›E71

ICD-10-CM E71
CMSSource: CMS FY 2027 ICD-10-CM dataset · Effective Oct 1, 2026 – Sep 30, 2027

E71 is a non-billable ICD-10-CM category code for disorders of branched-chain amino-acid metabolism and fatty-acid metabolism, so it cannot be submitted on claims. Use a more specific code from this category instead, such as E71.0, E71.110, E71.111, and E71.118.

Code Identity

ICD-10-CM Code
E71
Billable Status
No — Non-Billable Category
Code Describes
Disorders of branched-chain amino-acid metabolism and fatty-acid metabolism
Short Description
Disord of branched-chain amino-acid metab & fatty-acid metab
Chapter
E70-E88
Metabolic disorders

Code Classification

ChapterE00–E89Endocrine, nutritional and metabolic diseases
SectionE70-E88Metabolic disorders
CategoryE71Disorders of branched-chain amino-acid metabolism and fatty-acid metabolism
This CodeE71Disorders of branched-chain amino-acid metabolism and fatty-acid metabolism

Specific Coding for Disord of branched-chain amino-acid metab & fatty-acid metabOverview

Non-specific codes like E71 require more characters. Use one of these billable codes instead:

  • Use E71.0 for Maple-syrup-urine disease

  • E71.1 for Other disorders of branched-chain amino-acid metabolism

  • E71.11 for Branched-chain organic acidurias

  • E71.12 for Disorders of propionate metabolism

  • Use E71.19 for Other disorders of branched-chain amino-acid metabolism

  • Use E71.2 for Disorder of branched-chain amino-acid metabolism, unspecified

  • E71.3 for Disorders of fatty-acid metabolism

  • Use E71.30 for Disorder of fatty-acid metabolism, unspecified

  • E71.31 for Disorders of fatty-acid oxidation

  • Use E71.32 for Disorders of ketone metabolism

  • Use E71.39 for Other disorders of fatty-acid metabolism

  • E71.4 for Disorders of carnitine metabolism

  • Use E71.40 for Disorder of carnitine metabolism, unspecified

  • Use E71.41 for Primary carnitine deficiency

  • Use E71.42 for Carnitine deficiency due to inborn errors of metabolism

  • Use E71.43 for Iatrogenic carnitine deficiency

  • E71.44 for Other secondary carnitine deficiency

  • E71.5 for Peroxisomal disorders

  • Use E71.50 for Peroxisomal disorder, unspecified

  • E71.51 for Disorders of peroxisome biogenesis

  • E71.52 for X-linked adrenoleukodystrophy

  • Use E71.53 for Other group 2 peroxisomal disorders

  • E71.54 for Other peroxisomal disorders

Instructional NotesGuidance

Instructions from the official ICD-10-CM Tabular List that apply to E71: its own notes plus those printed at block E70-E88 and Chapter 4. A note printed at a category, block or chapter applies to every code under it.

Excludes1

Not coded here: the excluded code is never reported together with this one, unless the two conditions are unrelated.

From Block E70-E88 Metabolic disorders applies to 286 codes
  • androgen insensitivity syndrome (E34.5-)
  • congenital adrenal hyperplasia (E25.0)
  • hemolytic anemias attributable to enzyme disorders (D55.-)
  • Marfan syndrome (Q87.4-)
  • 5-alpha-reductase deficiency (E29.1)
From Chapter 4 (E00-E89) Endocrine, nutritional and metabolic diseases applies to 973 codes
  • transitory endocrine and metabolic disorders specific to newborn (P70-P74)

Excludes2

Not included here: the excluded condition is not part of this code, but a patient may have both, so both codes may be reported.

From Block E70-E88 Metabolic disorders applies to 286 codes
  • Ehlers-Danlos syndromes (Q79.6-)

Notes

General instructions on how these codes are used.

From Chapter 4 (E00-E89) Endocrine, nutritional and metabolic diseases applies to 973 codes
  • All neoplasms, whether functionally active or not, are classified in Chapter 2. Appropriate codes in this chapter (i.e. E05.8, E07.0, E16-E31, E34.-) may be used as additional codes to indicate either functional activity by neoplasms and ectopic endocrine tissue or hyperfunction and hypofunction of endocrine glands associated with neoplasms and other conditions classified elsewhere.

Source: CMS ICD-10-CM Tabular List. How to read instructional notes.

Referenced in Other NotesGuidance

Instructional notes printed at other codes that name E71, its category, or a range that includes it.

Excludes1 3

These codes carry an Excludes1 note naming E71: they are not reported together with it, unless the two conditions are unrelated.

  • R79 Other abnormal findings of blood chemistry
    specific findings indicating disorder of amino-acid metabolism (E70-E72) names E70-E72, which includes this code
  • R79.83 Abnormal findings of blood amino-acid level
    disorders of amino-acid metabolism (E70-E72) names E70-E72, which includes this code
  • Block R80-R82 Abnormal findings on examination of urine, without diagnosis
    specific findings indicating disorder of amino-acid metabolism (E70-E72) names E70-E72, which includes this code

Patient EducationClinical

Metabolic Disorders

Metabolism is the process your body uses to get or make energy from the food you eat. Food is made up of proteins, carbohydrates, and fats. Chemicals in your digestive system break the food parts down into sugars and acids, your body's fuel.

Read the full article at MedlinePlus

Courtesy of MedlinePlus, a service of the U.S. National Library of Medicine.

Code HistoryHistory

FY 2016AddedAdded to the ICD-10-CM code setEffective October 1, 2015, the first year of ICD-10-CM.
FY 2017–2026No changes
FY 2027CurrentCurrent code set, no changesEffective October 1, 2026 through September 30, 2027.

Questions About E71Overview

Is E71 a billable code?

No. This is a category header that groups the codes for disorders of branched-chain amino-acid metabolism and fatty-acid metabolism, and headers cannot be submitted on claims. Claims for disorders of branched-chain amino-acid metabolism and fatty-acid metabolism need a more specific code from this category, such as E71.0, E71.110, and E71.111.

Can E71 be reported with a code from E34.5?

Not as a rule. The Excludes1 note at block E70-E88, which applies to E71, lists "androgen insensitivity syndrome (E34.5-)". An Excludes1 note means the excluded code is never reported together with this one; the only exception is when the two conditions are unrelated to each other (Official Guidelines, Section I.A.12.a).