ICD-10-CM Code E88.4

Mitochondrial metabolism disorders

Version 2021 Non-Billable Code

Not Valid for Submission

E88.4 is a "header" nonspecific and non-billable code code, consider using a code with a higher level of specificity for a diagnosis of mitochondrial metabolism disorders. The code is NOT valid for the year 2021 for the submission of HIPAA-covered transactions.

ICD-10:E88.4
Short Description:Mitochondrial metabolism disorders
Long Description:Mitochondrial metabolism disorders

Consider the following ICD-10 codes with a higher level of specificity:

  • E88.40 - Mitochondrial metabolism disorder, unspecified
  • E88.41 - MELAS syndrome
  • E88.42 - MERRF syndrome
  • E88.49 - Other mitochondrial metabolism disorders

Tabular List of Diseases and Injuries

The Tabular List of Diseases and Injuries is a list of ICD-10 codes, organized "head to toe" into chapters and sections with guidance for inclusions, exclusions, descriptions and more. The following references are applicable to the code E88.4:

Type 1 Excludes

Type 1 Excludes
A type 1 excludes note is a pure excludes note. It means "NOT CODED HERE!" An Excludes1 note indicates that the code excluded should never be used at the same time as the code above the Excludes1 note. An Excludes1 is used when two conditions cannot occur together, such as a congenital form versus an acquired form of the same condition.
  • disorders of pyruvate metabolism E74.4
  • Kearns-Sayre syndrome H49.81
  • Leber's disease H47.22
  • Leigh's encephalopathy G31.82
  • Mitochondrial myopathy, NEC G71.3
  • Reye's syndrome G93.7

Code Classification

  • Endocrine, nutritional and metabolic diseases (E00–E90)
    • Metabolic disorders (E70-E88)
      • Other and unspecified metabolic disorders (E88)

Code History

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

Information for Patients


Mitochondrial Diseases

Metabolism is the process your body uses to make energy from the food you eat. Food is made up of proteins, carbohydrates, and fats. Chemicals in your digestive system (enzymes) 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. If you have a metabolic disorder, something goes wrong with this process.

Mitochondrial diseases are a group of metabolic disorders. Mitochondria are small structures that produce energy in almost all of your cells. They make it by combining oxygen with the fuel molecules (sugars and fats) that come from your food. When the mitochondria are defective, the cells do not have enough energy. The unused oxygen and fuel molecules build up in the cells and cause damage.

The symptoms of mitochondrial disease can vary. It depends on how many mitochondria are defective, and where they are in the body. Sometimes only one organ, tissue, or cell type is affected. But often the problem affects many of them. Muscle and nerve cells have especially high energy needs, so muscular and neurological problems are common. The diseases range from mild to severe. Some types can be fatal.

Genetic mutations cause these diseases. They usually happen before age 20, and some are more common in infants. There are no cures for these diseases, but treatments may help with symptoms and slow down the disease. They may include physical therapy, vitamins and supplements, special diets, and medicines.


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