2026 ICD-10-CM Diagnosis Code E71.511Neonatal adrenoleukodystrophy

ICD-10-CM Codes›E00–E89›E70-E88›E71

ICD-10-CM E71.511
CMSSource: CMS FY 2026 ICD-10-CM dataset · Effective Oct 1, 2025 – Sep 30, 2026

E71.511 is a billable ICD-10-CM diagnosis code for neonatal adrenoleukodystrophy. It is valid on HIPAA claims for fiscal year 2026 (October 1, 2025 through September 30, 2026). Coders also document this condition as adrenoleukodystrophy. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Other specified and unspecified nutritional and metabolic disorders.

Code Identity

ICD-10-CM Code
E71.511
Billable Status
Yes — Valid for Submission
Code Describes
Neonatal adrenoleukodystrophy
Short Description
Neonatal adrenoleukodystrophy
Same as the full description in the CMS dataset.
Parent Code
E71.51
Disorders of peroxisome biogenesis

Code Classification

ChapterE00–E89Endocrine, nutritional and metabolic diseases
SectionE70-E88Metabolic disorders
CategoryE71Disorders of branched-chain amino-acid metabolism and fatty-acid metabolism
This CodeE71.511Neonatal adrenoleukodystrophy

Approximate SynonymsGuidance

Alternate terms and clinical phrases that map to this code.

  • Adrenoleukodystrophy
  • Neonatal adrenoleukodystrophy

Tabular List NotesGuidance

Coding notes and annotation back-references applicable to this code.

Type 1 Excludes

  • X-linked adrenoleukodystrophy E71.42

Index to Diseases and InjuriesGuidance

Alphabetical index entries that point to this code.

External Cause of Injuries IndexGuidance

References for this code in the External Cause of Injuries Index.

    • Adrenoleukodystrophy
      • neonatal
    • Disorder(of)
      • peroxisomal
        • biogenesis
          • neonatal adrenoleukodystrophy

Clinical ClassificationClinical

AHRQ’s CCSR groups this code into broader clinical categories.

CCSR END016
Other specified and unspecified nutritional and metabolic disorders
Default principal diagnosis: inpatient Yes · outpatient Yes

Clinical InformationClinical

  • Adrenoleukodystrophy

    an x-linked recessive disorder characterized by the accumulation of saturated very long chain fatty acids in the lysosomes of adrenal cortex and the white matter of central nervous system. this disease occurs almost exclusively in the males. clinical features include the childhood onset of ataxia; neurobehavioral manifestations; hyperpigmentation; adrenal insufficiency; seizures; muscle spasticity; and dementia. the slowly progressive adult form is called adrenomyeloneuropathy. the defective gene abcd1 is located at xq28, and encodes the adrenoleukodystrophy protein (atp-binding cassette transporters).

  • ATP Binding Cassette Transporter, Subfamily D, Member 1

    atp-binding cassette transporter that functions in the import of long chain (13-21 carbons) and very long chain fatty acids (> 22 carbons), or their acyl-coa-derivatives, into peroxisomes. mutations in the abcd1 gene are associated with the x-linked form of adrenoleukodystrophy.

  • 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.

  • Neonatal Adrenoleukodystrophy

    a rare metabolic disorder that affects neonates. it is characterized by damage of the white matter in the brain and degeneration of the adrenal glands. it manifests with hyperactivity, paralysis, muscular weakness, crossed eyes, hearing loss, seizures, and coma.

Patient EducationClinical

Leukodystrophies

Leukodystrophies are a group of rare genetic disorders that affect the central nervous system (CNS). The CNS is made up of your brain and spinal cord. Leukodystrophies damage the white matter of your CNS. The white matter includes:

The full article covers:

  • What are leukodystrophies?
  • What causes leukodystrophies?
  • What are the symptoms of leukodystrophies?
  • How are leukodystrophies diagnosed?
  • What are the treatments for leukodystrophies?

Read the full article at MedlinePlus

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

Convert E71.511 to ICD-9-CMHistory

The closest ICD-9-CM equivalents under the General Equivalence Mappings.

ICD-9-CM
277.86 Peroxisomal disorders
Approximate The match is approximate rather than exact.

Code HistoryHistory

FY 2016AddedAdded to the ICD-10-CM code setEffective October 1, 2015, the first year of ICD-10-CM.
FY 2017–2025No changes
FY 2026CurrentCurrent code set, no changesEffective October 1, 2025 through September 30, 2026.

Questions About E71.511Overview

Is E71.511 (Disorders of peroxisome biogenesis) a billable code?

Yes. This is a billable ICD-10-CM code, specific enough to report neonatal adrenoleukodystrophy on HIPAA-covered claims from October 1, 2025 through September 30, 2026.

What is the ICD-9 equivalent of E71.511?

Under the General Equivalence Mappings, neonatal adrenoleukodystrophy converts to ICD-9-CM 277.86 (peroxisomal disorders). The mapping is approximate, so confirm the match fits the documentation.

Footnotes

[1] Chronic - a chronic condition code indicates a condition lasting 12 months or longer and its effect on the patient based on one or both of the following criteria:

  • The condition results in the need for ongoing intervention with medical products,treatment, services, and special equipment
  • The condition places limitations on self-care, independent living, and social interactions.