ICD-10-CM 2027
ICD-10 code for Chylomicron retention disease
From the official ICD-10-CM alphabetic index entry “Disease, diseased › chylomicron retention”. Page updated September 29, 2026.
About coding Chylomicron retention disease
The ICD-10-CM code for Chylomicron retention disease is E78.3 (Hyperchylomicronemia).
Within E78 (disorders of lipoprotein metabolism and other lipidemias), E78.3 is specifically for Hyperchylomicronemia. Related codes cover pure hypercholesterolemia (E78.0), pure hyperglyceridemia (E78.1), mixed hyperlipidemia (E78.2), other hyperlipidemia (E78.4), hyperlipidemia, unspecified (E78.5), lipoprotein deficiency (E78.6), disorders of bile acid and cholesterol metabolism (E78.7), other disorders of lipoprotein metabolism (E78.8) and 1 more.
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.
Common questions about Chylomicron retention disease ICD-10 codes
What is the ICD-10 code for Chylomicron retention disease?
- E78.3 — Hyperchylomicronemia.
Is E78.3 billable?
- Yes. E78.3 is a billable/specific ICD-10-CM code valid for FY2027 (October 1, 2026 – September 30, 2027).
Can E78.3 be used as a principal diagnosis?
- Yes. The Medicare Code Editor doesn't restrict E78.3 as a principal diagnosis.
Is E78.3 a CC or MCC?
- No. E78.3 is neither a CC nor an MCC under MS-DRG v44.0.