2022 ICD-10-CM Code E71

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

Version 2021

Not Valid for Submission

ICD-10:E71
Short Description:Disord of branched-chain amino-acid metab & fatty-acid metab
Long Description:Disorders of branched-chain amino-acid metabolism and fatty-acid metabolism

Code Classification

  • Endocrine, nutritional and metabolic diseases (E00–E90)
    • Metabolic disorders (E70-E88)
      • Disord of branched-chain amino-acid metab & fatty-acid metab (E71)

E71 is a non-specific and non-billable diagnosis code code, consider using a code with a higher level of specificity for a diagnosis of disorders of branched-chain amino-acid metabolism and fatty-acid metabolism. The code is not specific and is NOT valid for the year 2022 for the submission of HIPAA-covered transactions. Category or Header define the heading of a category of codes that may be further subdivided by the use of 4th, 5th, 6th or 7th characters.

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

Non-specific codes like E71 require more digits to indicate the appropriate level of specificity. Consider using any of the following ICD-10 codes with a higher level of specificity when coding for disord of branched-chain amino-acid metab & fatty-acid metab:

  • BILLABLE CODE - Use E71.0 for Maple-syrup-urine disease
  • NON-BILLABLE CODE - E71.1 for Other disorders of branched-chain amino-acid metabolism
  • NON-BILLABLE CODE - E71.11 for Branched-chain organic acidurias
  • NON-BILLABLE CODE - E71.12 for Disorders of propionate metabolism
  • BILLABLE CODE - Use E71.19 for Other disorders of branched-chain amino-acid metabolism
  • BILLABLE CODE - Use E71.2 for Disorder of branched-chain amino-acid metabolism, unspecified
  • NON-BILLABLE CODE - E71.3 for Disorders of fatty-acid metabolism
  • BILLABLE CODE - Use E71.30 for Disorder of fatty-acid metabolism, unspecified
  • NON-BILLABLE CODE - E71.31 for Disorders of fatty-acid oxidation
  • BILLABLE CODE - Use E71.32 for Disorders of ketone metabolism
  • BILLABLE CODE - Use E71.39 for Other disorders of fatty-acid metabolism
  • NON-BILLABLE CODE - E71.4 for Disorders of carnitine metabolism
  • BILLABLE CODE - Use E71.40 for Disorder of carnitine metabolism, unspecified
  • BILLABLE CODE - Use E71.41 for Primary carnitine deficiency
  • BILLABLE CODE - Use E71.42 for Carnitine deficiency due to inborn errors of metabolism
  • BILLABLE CODE - Use E71.43 for Iatrogenic carnitine deficiency
  • NON-BILLABLE CODE - E71.44 for Other secondary carnitine deficiency
  • NON-BILLABLE CODE - E71.5 for Peroxisomal disorders
  • BILLABLE CODE - Use E71.50 for Peroxisomal disorder, unspecified
  • NON-BILLABLE CODE - E71.51 for Disorders of peroxisome biogenesis
  • NON-BILLABLE CODE - E71.52 for X-linked adrenoleukodystrophy
  • BILLABLE CODE - Use E71.53 for Other group 2 peroxisomal disorders
  • NON-BILLABLE CODE - E71.54 for Other peroxisomal disorders

Information for Patients


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. Your body can use this fuel right away, or it can store the energy in your body tissues, such as your liver, muscles, and body fat.

A metabolic disorder occurs when abnormal chemical reactions in your body disrupt this process. When this happens, you might have too much of some substances or too little of other ones that you need to stay healthy. There are different groups of disorders. Some affect the breakdown of amino acids, carbohydrates, or lipids. Another group, mitochondrial diseases, affects the parts of the cells that produce the energy.

You can develop a metabolic disorder when some organs, such as your liver or pancreas, become diseased or do not function normally. Diabetes is an example.


[Learn More in MedlinePlus]

Code History

  • FY 2021 - No Change, effective from 10/1/2020 through 9/30/2021
  • FY 2020 - No Change, effective from 10/1/2019 through 9/30/2020
  • FY 2019 - No Change, effective from 10/1/2018 through 9/30/2019
  • FY 2018 - No Change, effective from 10/1/2017 through 9/30/2018
  • FY 2017 - No Change, effective from 10/1/2016 through 9/30/2017
  • FY 2016 - New Code, effective from 10/1/2015 through 9/30/2016 (First year ICD-10-CM implemented into the HIPAA code set)