2027 ICD-10-CM Diagnosis Code E80.5Crigler-Najjar syndrome
ICD-10-CM Codes›E00–E89›E70-E88›E80
- Billable — Valid for Submission
- Chronic Condition
E80.5 is a billable ICD-10-CM diagnosis code for Crigler-Najjar syndrome. It is valid on HIPAA claims for fiscal year 2027 (October 1, 2026 through September 30, 2027) 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
Code Classification
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
Instructional NotesGuidance
Instructions from the official ICD-10-CM Tabular List that apply to E80.5: its own notes plus those printed at E80, block E70-E88, and Chapter 4. A note printed at a category, block or chapter applies to every code under it.
Includes
Further defines, or gives examples of, the content of the category.
- defects of catalase and peroxidase
Excludes1
Not coded here: the excluded code is never reported together with this one, unless the two conditions are unrelated.
Excludes2
Not included here: the excluded condition is not part of this code, but a patient may have both, so both codes may be reported.
- Ehlers-Danlos syndromes (Q79.6-)
Notes
General instructions on how these codes are used.
- All neoplasms, whether functionally active or not, are classified in Chapter 2. Appropriate codes in this chapter (i.e. E05.8, E07.0, E16-E31, E34.-) may be used as additional codes to indicate either functional activity by neoplasms and ectopic endocrine tissue or hyperfunction and hypofunction of endocrine glands associated with neoplasms and other conditions classified elsewhere.
Source: CMS ICD-10-CM Tabular List. How to read instructional notes.
Referenced in Other NotesGuidance
Instructional notes printed at other codes that name E80.5, its category, or a range that includes it.
Excludes1 2
These codes carry an Excludes1 note naming E80.5: they are not reported together with it, unless the two conditions are unrelated.
- Block P50-P61 Hemorrhagic and hematological disorders of newbornCrigler-Najjar syndrome (E80.5)
- P57.8 Other specified kernicterusCrigler-Najjar syndrome (E80.5)
Index to Diseases and InjuriesGuidance
Alphabetical index entries that point to this code.
Clinical ClassificationClinical
AHRQ’s CCSR groups this code into broader clinical categories.
Clinical InformationClinical
Crigler-Najjar Syndrome
a familial form of congenital hyperbilirubinemia transmitted as an autosomal recessive trait. it is characterized by icterus and brain damage caused by a glucuronyl transferase deficiency in the liver and faulty bilirubin conjugation.
Patient EducationClinical
Liver Diseases
Your liver is the largest organ inside your body. It helps your body digest food, store energy, and remove poisons.
Read the full article at MedlinePlus
Courtesy of MedlinePlus, a service of the U.S. National Library of Medicine.
Convert E80.5 to ICD-9-CMHistory
The closest ICD-9-CM equivalents under the General Equivalence Mappings.
Code HistoryHistory
Questions About E80.5Overview
What is the ICD-10 code for Crigler-Najjar syndrome?
The ICD-10-CM code for Crigler-Najjar syndrome is E80.5 (sometimes written as E805). It is billable on HIPAA-covered claims from October 1, 2026 through September 30, 2027.
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, 2026 through September 30, 2027.
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 442, 441, 443, with relative weights from 0.6890 to 1.8226 depending on complications. Higher weights mean higher Medicare reimbursement.
Can E80.5 be reported with a code from E34.5?
Not as a rule. The Excludes1 note at block E70-E88, which applies to E80.5, lists "androgen insensitivity syndrome (E34.5-)". An Excludes1 note means the excluded code is never reported together with this one; the only exception is when the two conditions are unrelated to each other (Official Guidelines, Section I.A.12.a).
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.
