ICD-10-CM 2027
ICD-10 code for Hyperbetalipoproteinemia
From the official ICD-10-CM alphabetic index entry “Hyperbetalipoproteinemia (familial)”. Page updated September 29, 2026.
About coding Hyperbetalipoproteinemia
The ICD-10-CM code for Hyperbetalipoproteinemia is E78.00 (Pure hypercholesterolemia, unspecified). The official index lists 1 more specific code, so check the documentation for details such as type, cause, site or severity before settling on E78.00.
Within E78.0, choose E78.00 (unspecified) only when documentation doesn't support a more specific option: familial hypercholesterolemia (E78.01).
It is not a CC or MCC, so as a secondary diagnosis it doesn't change the MS-DRG severity level.
In MS-DRG v44.0, it is part of the grouping logic for DRG 642 (Inborn and Other Disorders of Metabolism, relative weight 1.3219), in MDC 10 (Endocrine, Nutritional and Metabolic Diseases and Disorders), as a principal or secondary diagnosis. The DRG a claim lands in depends on the principal diagnosis, procedures and any CC/MCC secondary diagnoses.
More specific Hyperbetalipoproteinemia codes (official index)
- with prebetalipoproteinemia E78.2 — Mixed hyperlipidemia
Common questions about Hyperbetalipoproteinemia ICD-10 codes
What is the ICD-10 code for Hyperbetalipoproteinemia?
- E78.00 — Pure hypercholesterolemia, unspecified. More specific codes apply when the documentation supports them: E78.2.
Is E78.00 billable?
- Yes. E78.00 is a billable/specific ICD-10-CM code valid for FY2027 (October 1, 2026 – September 30, 2027).
Can E78.00 be used as a principal diagnosis?
- Yes. The Medicare Code Editor doesn't restrict E78.00 as a principal diagnosis.
Is E78.00 a CC or MCC?
- No. E78.00 is neither a CC nor an MCC under MS-DRG v44.0.