ICD-10-CM Code E71.548

Other peroxisomal disorders

Version 2021 Billable Code

Valid for Submission

E71.548 is a billable code used to specify a medical diagnosis of other peroxisomal disorders. The code is valid for the fiscal year 2021 for the submission of HIPAA-covered transactions. The ICD-10-CM code E71.548 might also be used to specify conditions or terms like bifunctional peroxisomal enzyme deficiency, dihydroxycholestanoic acidemia and trihydroxycholestanoic acidemia, glutaryl-coa oxidase deficiency, isolated alkyldihydroxyacetone phosphate synthase deficiency, isolated dihydroxyacetone phosphate acyltransferase deficiency, peroxisomal thiolase deficiency, etc

Short Description:Other peroxisomal disorders
Long Description:Other peroxisomal disorders

Index to Diseases and Injuries

The Index to Diseases and Injuries is an alphabetical listing of medical terms, with each term mapped to one or more ICD-10 code(s). The following references for the code E71.548 are found in the index:


The following clinical terms are approximate synonyms or lay terms that might be used to identify the correct diagnosis code:

  • Bifunctional peroxisomal enzyme deficiency
  • Dihydroxycholestanoic acidemia and trihydroxycholestanoic acidemia
  • Glutaryl-CoA oxidase deficiency
  • Isolated alkyldihydroxyacetone phosphate synthase deficiency
  • Isolated dihydroxyacetone phosphate acyltransferase deficiency
  • Peroxisomal thiolase deficiency

Clinical Information

  • PEROXISOMAL DISORDERS-. a heterogeneous group of inherited metabolic disorders marked by absent or dysfunctional peroxisomes. peroxisomal enzymatic abnormalities may be single or multiple. biosynthetic peroxisomal pathways are compromised including the ability to synthesize ether lipids and to oxidize long chain fatty acid precursors. diseases in this category include zellweger syndrome; infantile refsum disease; rhizomelic chondrodysplasia chondrodysplasia punctata rhizomelic; hyperpipecolic acidemia; neonatal adrenoleukodystrophy; and adrenoleukodystrophy x linked. neurologic dysfunction is a prominent feature of most peroxisomal disorders.

Convert E71.548 to ICD-9

  • 277.86 - Peroxisomal disorders (Approximate Flag)

Code Classification

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

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

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.

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