E78.3 Hyperchylomicronemia

Billable ICD-10-CM code, FY2026.

Applicable to

• Chylomicron retention disease
• Fredrickson's hyperlipoproteinemia, type I or V
• Hyperlipidemia, group D
• Mixed hyperglyceridemia

Applies from E78 Disorders of lipoprotein metabolism and other lipidemias

Excludes1 (never code together)

• sphingolipidosis (E75.0-E75.3)

Risk adjustment (HCC)

• RxHCC V08: HCC 47 — Disorders of Lipoid Metabolism

MS-DRG index

Defines the logic of DRG(s) 642 in MDC 10.

Approximate ICD-9-CM (GEMs)

• 272.3 Hyperchylomicronemia

Code annotations containing back-references to E78.3

Back-references to E78

• Type 2 Excludes: E88.0 Disorders of plasma-protein metabolism, not elsewhere classified ("disorder of lipoprotein metabolism (E78.-)")
• Type 2 Excludes: G11 Hereditary ataxia ("metabolic disorders (E70-E88)")
• Code First: G63 Polyneuropathy in diseases classified elsewhere ("metabolic diseases (E70-E88)")
• Type 2 Excludes: G71 Primary disorders of muscles ("metabolic disorders (E70-E88)")
• Code First: H42 Glaucoma in diseases classified elsewhere ("specified metabolic disorder (E70-E88)")
• Type 2 Excludes: N25.0 Renal osteodystrophy ("metabolic disorders classifiable to E70-E88")
• Type 2 Excludes: N25.81 Secondary hyperparathyroidism of renal origin ("metabolic disorders classifiable to E70-E88")
• Type 1 Excludes: P59 Neonatal jaundice from other and unspecified causes ("jaundice due to inborn errors of metabolism (E70-E88)")

Diagnosis Index entries for E78.3

• Bürger-Grütz disease or syndrome
• Chylomicronemia (fasting) (with hyperprebetalipoproteinemia)
• Disease, diseased › Bürger-Grütz (essential familial hyperlipemia)
• Disease, diseased › chylomicron retention
• Frederickson's hyperlipoproteinemia, type › I and V
• Hepatosplenomegaly › hyperlipemic (Bürger-Grütz type)
• Hyperchylomicronemia (familial) (primary)
• Hyperchylomicronemia (familial) (primary) › with hyperbetalipoproteinemia
• Hyperglyceridemia (endogenous) (essential) (familial) (hereditary) (pure) › mixed
• Hyperlipemia, hyperlipidemia › group › D
• Hyperlipoproteinemia › Fredrickson's type › I
• Hyperlipoproteinemia › Fredrickson's type › V
• Lipemia › retina, retinalis
• Lipidosis › hepatosplenomegalic
• Prebetalipoproteinemia (acquired) (essential) (familial) (hereditary) (primary) (secondary) › with chylomicronemia
• Syndrome › Bürger-Grütz
• Xanthoma (s), xanthomatosis (primary) (familial) (hereditary) › with › hyperlipoproteinemia › Type I
• Xanthoma (s), xanthomatosis (primary) (familial) (hereditary) › with › hyperlipoproteinemia › Type V

Nearby codes

• E78.010 Homozygous familial hypercholesterolemia [HoFH]
• E78.011 Heterozygous familial hypercholesterolemia [HeFH]
• E78.019 Familial hypercholesterolemia, unspecified
• E78.1 Pure hyperglyceridemia
• E78.2 Mixed hyperlipidemia
• E78.3 Hyperchylomicronemia (this code)
• E78.4 Other hyperlipidemia
• E78.41 Elevated Lipoprotein(a)
• E78.49 Other hyperlipidemia
• E78.5 Hyperlipidemia, unspecified
• E78.6 Lipoprotein deficiency