2026 ICD-10-CM Diagnosis Code E71.19Other disorders of branched-chain amino-acid metabolism

ICD-10-CM Codes›E00–E89›E70-E88›E71

ICD-10-CM E71.19
CMSSource: CMS FY 2026 ICD-10-CM dataset · Effective Oct 1, 2025 – Sep 30, 2026

E71.19 is a billable ICD-10-CM diagnosis code for other disorders of branched-chain amino-acid metabolism. It is valid on HIPAA claims for fiscal year 2026 (October 1, 2025 through September 30, 2026). 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
E71.19
Billable Status
Yes — Valid for Submission
Code Describes
Other disorders of branched-chain amino-acid metabolism
Short Description
Other disorders of branched-chain amino-acid metabolism
Same as the full description in the CMS dataset.
Parent Code
E71.1
Other disorders of branched-chain amino-acid metabolism

Code Classification

ChapterE00–E89Endocrine, nutritional and metabolic diseases
SectionE70-E88Metabolic disorders
CategoryE71Disorders of branched-chain amino-acid metabolism and fatty-acid metabolism
This CodeE71.19Other disorders of branched-chain amino-acid metabolism

Approximate SynonymsGuidance

Alternate terms and clinical phrases that map to this code.

  • 2-methylbutyryl-coenzyme A dehydrogenase deficiency disease
  • 3-Hydroxyisobutyric aciduria
  • Autism epilepsy syndrome due to branched chain ketoacid dehydrogenase kinase deficiency
  • Autistic disorder of childhood onset
  • Clinical manifestation of enzyme deficiency
  • Combined malonic and methylmalonic aciduria
  • Deficiency of acetyl-CoA acetyltransferase
  • Deficiency of isoleucine-tRNA ligase
  • Deficiency of leucine aminotransferase
  • Deficiency of leucine-tRNA ligase
  • Deficiency of valine-tRNA ligase
  • Developmental delay due to methylmalonate semialdehyde dehydrogenase deficiency
  • Disorder of isoleucine metabolism
  • Disorder of valine metabolism
  • Hydroxymethylglutaric aciduria
  • Hyperleucine-isoleucinemia
  • Hyperleucinemia
  • Hypervalinemia
  • Inborn error of amino acid metabolism
  • Intermittent branched-chain ketonuria
  • Isobutyryl-CoA dehydrogenase deficiency disease
  • Isobutyrylglycinuria
  • Isoleucinosis
  • Leucine-induced hypoglycemia
  • Methylcrotonyl-CoA carboxylase deficiency
  • Mitochondrial 2-methylacetoacetyl-CoA thiolase deficiency - potassium stimulated
  • Valinosis

Tabular List NotesGuidance

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

Inclusion Terms

  • Hyperleucine-isoleucinemia
  • Hypervalinemia

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.

    • Disorder(of)
      • branched-chain amino-acid metabolism
        • specified NEC
    • Disorder(of)
      • hyperleucine-isoleucinemia
    • Disorder(of)
      • hypervalinemia
    • Disorder(of)
      • metabolism NOS
        • amino-acid
          • branched chain
            • hyperleucine-isoleucinemia
    • Disorder(of)
      • metabolism NOS
        • amino-acid
          • branched chain
            • hypervalinemia
    • Disorder(of)
      • metabolism NOS
        • amino-acid
          • branched chain
            • other specified
    • Disorder(of)
      • metabolism NOS
        • isoleucine
    • Disorder(of)
      • metabolism NOS
        • leucine
    • Disorder(of)
      • metabolism NOS
        • valine
    • Hyperleucine-isoleucinemia
    • Hypervalinemia
    • Hypoglycemia(spontaneous)
      • leucine-induced
    • Isoleucinosis

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

Patient EducationClinical

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.

Read the full article at MedlinePlus

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

Convert E71.19 to ICD-9-CMHistory

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

ICD-9-CM
270.3 Bran-chain amin-acid dis
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 E71.19Overview

Is E71.19 (Other disorders of branched-chain amino-acid metabolism) a billable code?

Yes. This is a billable ICD-10-CM code, specific enough to report other disorders of branched-chain amino-acid metabolism on HIPAA-covered claims from October 1, 2025 through September 30, 2026.

What is the ICD-9 equivalent of E71.19?

Under the General Equivalence Mappings, other disorders of branched-chain amino-acid metabolism converts to ICD-9-CM 270.3 (bran-chain amin-acid dis). 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.