2026 ICD-10-CM Diagnosis Code E70.1Other hyperphenylalaninemias

ICD-10-CM Codes›E00–E89›E70-E88›E70

ICD-10-CM E70.1
CMSSource: CMS FY 2026 ICD-10-CM dataset · Effective Oct 1, 2025 – Sep 30, 2026

E70.1 is a billable ICD-10-CM diagnosis code for other hyperphenylalaninemias. 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
E70.1
Billable Status
Yes — Valid for Submission
Code Describes
Other hyperphenylalaninemias
Short Description
Other hyperphenylalaninemias
Same as the full description in the CMS dataset.
Parent Code
E70
Disorders of aromatic amino-acid metabolism

Code Classification

ChapterE00–E89Endocrine, nutritional and metabolic diseases
SectionE70-E88Metabolic disorders
CategoryE70Disorders of aromatic amino-acid metabolism
This CodeE70.1Other hyperphenylalaninemias

Approximate SynonymsGuidance

Alternate terms and clinical phrases that map to this code.

  • 6-Pyruvoyl-tetrahydrobiopterin synthase deficiency
  • Classical phenylketonuria
  • Dihydropteridine reductase deficiency
  • Disorder of tetrahydrobiopterin metabolism
  • GTP cyclohydrolase I deficiency
  • Hyperphenylalaninemia
  • Hyperphenylalaninemia due to DNAJC12 deficiency
  • Maternal phenylketonuria
  • Persistent hyperphenylalaninemia
  • Persistent hyperphenylalaninemia AND tyrosinemia
  • Phenylketonuria
  • Pterin-4-carbinolamine dehydratase deficiency
  • Sepiapterin reductase deficiency
  • Tetrahydrobiopterin synthesis defect
  • Transient hyperphenylalaninemia

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.

    • Deficiency, deficient
      • phenylalanine hydroxylase
    • Disorder(of)
      • metabolism NOS
        • amino-acid
          • aromatic
            • hyperphenylalaninemia
    • Hyperphenylalaninemia NEC
    • Phenylketonuria
    • Phenylketonuria
      • maternal

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 E70.1 to ICD-9-CMHistory

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

ICD-9-CM
270.1 Phenylketonuria - pku
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 E70.1Overview

Is E70.1 (Disorders of aromatic amino-acid metabolism) a billable code?

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

What is the ICD-9 equivalent of E70.1?

Under the General Equivalence Mappings, other hyperphenylalaninemias converts to ICD-9-CM 270.1 (phenylketonuria - pku). 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.