2026 ICD-10-CM Diagnosis Code E80.5Crigler-Najjar syndrome

ICD-10-CM Codes›E00–E89›E70-E88›E80

ICD-10-CM E80.5
CMSSource: CMS FY 2026 ICD-10-CM dataset · Effective Oct 1, 2025 – Sep 30, 2026

E80.5 is a billable ICD-10-CM diagnosis code for Crigler-Najjar syndrome. It is valid on HIPAA claims for fiscal year 2026 (October 1, 2025 through September 30, 2026) and groups to MS-DRG 441 through 443. 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
E80.5
Billable Status
Yes — Valid for Submission
Code Describes
Crigler-Najjar syndrome
Short Description
Crigler-Najjar syndrome
Same as the full description in the CMS dataset.
Parent Code
E80
Disorders of porphyrin and bilirubin metabolism

Code Classification

ChapterE00–E89Endocrine, nutritional and metabolic diseases
SectionE70-E88Metabolic disorders
CategoryE80Disorders of porphyrin and bilirubin metabolism
This CodeE80.5Crigler-Najjar syndrome

Approximate SynonymsGuidance

Alternate terms and clinical phrases that map to this code.

  • Crigler-Najjar syndrome
  • Crigler-Najjar syndrome, type I
  • Crigler-Najjar syndrome, type II
  • Inherited disorder of bilirubin metabolism
  • Neonatal jaundice due to Crigler-Najjar syndrome

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.

    • Crigler-Najjar disease or syndrome
    • Deficiency, deficient
      • glucuronyl transferase
    • Jaundice(yellow)
      • familial nonhemolytic (congenital) (Gilbert)
        • Crigler-Najjar
    • Jaundice(yellow)
      • newborn
        • due to or associated with
          • Crigler-Najjar syndrome

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

Liver Diseases

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Convert E80.5 to ICD-9-CMHistory

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

ICD-9-CM
277.4 Dis bilirubin excretion
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 E80.5Overview

Is E80.5 (Disorders of porphyrin and bilirubin metabolism) a billable code?

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

What MS-DRG does E80.5 group to?

When Crigler-Najjar syndrome is the principal diagnosis on an inpatient stay, it groups to MS-DRG 441, 442, 443, with relative weights from 0.6997 to 1.7947 depending on complications. Higher weights mean higher Medicare reimbursement.

What is the ICD-9 equivalent of E80.5?

Under the General Equivalence Mappings, Crigler-Najjar syndrome converts to ICD-9-CM 277.4 (dis bilirubin excretion). 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.