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ICD-10-CM Codes
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E00-E89
Endocrine, nutritional and metabolic diseases
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E70-E88
Metabolic disorders
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E71-
Disorders of branched-chain amino-acid metabolism and fatty-acid metabolism
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2021 ICD-10-CM Diagnosis Code E71.5
2021 ICD-10-CM Diagnosis Code E71.5
Peroxisomal disorders
2016 2017 2018 2019 2020 2021 Non-Billable/Non-Specific Code
- E71.5 should not be used for reimbursement purposes as there are multiple codes below it that contain a greater level of detail.
- The 2021 edition of ICD-10-CM E71.5 became effective on October 1, 2020.
- This is the American ICD-10-CM version of E71.5 - other international versions of ICD-10 E71.5 may differ.
Type 1 ExcludesType 1 Excludes Help
A type 1 excludes note is a pure excludes. It means "not coded here". A type 1 excludes note indicates that the code excluded should never be used at the same time as E71.5. A type 1 excludes note is for used for when two conditions cannot occur together, such as a congenital form versus an acquired form of the same condition.
The following code(s) above
E71.5 contain annotation back-references
Annotation Back-References
In this context, annotation back-references refer to codes that contain:
- Applicable To annotations, or
- Code Also annotations, or
- Code First annotations, or
- Excludes1 annotations, or
- Excludes2 annotations, or
- Includes annotations, or
- Note annotations, or
- Use Additional annotations
that may be applicable to
E71.5:
Clinical Information
- A group of congenital disorders of lipid metabolism, caused by loss of the normal peroxisomes. Signs and symptoms include developmental delays, mental retardation, facial abnormalities, hepatomegaly, and hypotonia.
- 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.
Code History
- 2016 (effective 10/1/2015): New code (first year of non-draft ICD-10-CM)
- 2017 (effective 10/1/2016): No change
- 2018 (effective 10/1/2017): No change
- 2019 (effective 10/1/2018): No change
- 2020 (effective 10/1/2019): No change
- 2021 (effective 10/1/2020): No change
Code annotations containing back-references to E71.5:
- Aciduria
- glutaric (type I) E72.3
ICD-10-CM Diagnosis Code E72.3
Disorders of lysine and hydroxylysine metabolism
2016 2017 2018 2019 2020 2021 Billable/Specific Code
Applicable To- Glutaric aciduria NOS
- Glutaric aciduria (type I)
- Hydroxylysinemia
- Hyperlysinemia
ICD-10-CM Codes Adjacent To E71.5
E71.32 Disorders of ketone metabolism
E71.39 Other disorders of fatty-acid metabolism
E71.4 Disorders of carnitine metabolism
E71.40 Disorder of carnitine metabolism, unspecified
E71.41 Primary carnitine deficiency
E71.42 Carnitine deficiency due to inborn errors of metabolism
E71.43 Iatrogenic carnitine deficiency
E71.44 Other secondary carnitine deficiency
E71.440 Ruvalcaba-Myhre-Smith syndrome
E71.448 Other secondary carnitine deficiency
E71.5
Peroxisomal disorders
E71.50 Peroxisomal disorder, unspecified
E71.51 Disorders of peroxisome biogenesis
E71.511 Neonatal adrenoleukodystrophy
E71.518 Other disorders of peroxisome biogenesis
E71.52 X-linked adrenoleukodystrophy
E71.520 Childhood cerebral X-linked adrenoleukodystrophy
E71.521 Adolescent X-linked adrenoleukodystrophy
E71.528 Other X-linked adrenoleukodystrophy
Reimbursement claims with a date of service on or after October 1, 2015 require the use of ICD-10-CM codes.