E78.01

Familial hypercholesterolemia

Risk-adjusting (HCC)Header / non-billable

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HCC risk adjustment mapping

RxHCC V08

RXHCC47

Disorders of Lipoid Metabolism

Coding notes

Excludes 1 never coded together · Excludes 2 may co-exist

Inherited from E78Disorders of lipoprotein metabolism and other lipidemias

Excludes 1

sphingolipidosis (E75.0-E75.3)

Inherited from E70-E88Metabolic disorders

Excludes 1

androgen insensitivity syndrome (E34.5-) congenital adrenal hyperplasia (E25.0) hemolytic anemias attributable to enzyme disorders (D55.-) Marfan syndrome (Q87.4-) 5-alpha-reductase deficiency (E29.1)

Excludes 2

Ehlers-Danlos syndromes (Q79.6-)

Inherited from chapter 4Endocrine, nutritional and metabolic diseases

Excludes 1

transitory endocrine and metabolic disorders specific to newborn (P70-P74)

Note

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.

Official Guidelines for E78.01

FY 2026 ›

I.C.4Chapter-specific guidanceChapter 4: Endocrine, Nutritional, and Metabolic Diseases (E00-E89)

Subsections