1. ICD-10-CM Codes
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2. E00-E89 Endocrine, nutritional and metabolic diseases
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3. E70-E88 Metabolic disorders
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4. E78- Disorders of lipoprotein metabolism and other lipidemias
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2020 ICD-10-CM Diagnosis Code E78.3

Hyperchylomicronemia

2016 2017 2018 2019 2020 Billable/Specific Code

• E78.3 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes.
• The 2020 edition of ICD-10-CM E78.3 became effective on October 1, 2019.
• This is the American ICD-10-CM version of E78.3 - other international versions of ICD-10 E78.3 may differ.

• Familial lipoprotein lipase deficiency

• A genetic disorder of lipoprotein metabolism caused by mutations in the lpl and apolipoprotein (apo) c-ii genes. It is characterized by increased levels of chylomicrons and triglycerides in the blood.
• An inherited condition due to a deficiency of either lipoprotein lipase or apolipoprotein c-ii (a lipase-activating protein). The lack of lipase activities results in inability to remove chylomicrons and triglycerides from the blood which has a creamy top layer after standing.
• Intestinal fat transport defect with hypobetalipoproteinemia and accumulation of apolipoprotein b-like protein in intestinal cells, deficient blood apolipoproteins, and avitaminosis e manifested by malnutrition, steatorrhea, and growth and mental retardation. Some clinical (but not biochemical) manifestations may disappear later in life.
• Rare familial condition characterized by massive chylomicronemia and decreased levels of other lipoproteins; due to deficiency of lipoprotein lipase, an alkaline triglyceride hydrolase which catalyzes an important step in the extrahepatic removal of triglyceride-rich lipoproteins from the blood.

• 642 Inborn and other disorders of metabolism

Convert E78.3 to ICD-9-CM

• 2016 (effective 10/1/2015): New code (first year of non-draft ICD-10-CM)
• 2017 (effective 10/1/2016): No change
• 2018 (effective 10/1/2017): No change
• 2019 (effective 10/1/2018): No change
• 2020 (effective 10/1/2019): No change

• Bürger-Grütz disease or syndrome E78.3
• Chylomicronemia E78.3 (fasting) (with hyperprebetalipoproteinemia)
• Disease, diseased - see also Syndrome
  • Bürger-Grütz E78.3 (essential familial hyperlipemia)
  • chylomicron retention E78.3
• Frederickson's hyperlipoproteinemia, type
  • I and V E78.3
• Hepatosplenomegaly R16.2
  ICD-10-CM Diagnosis Code R16.2

  Hepatomegaly with splenomegaly, not elsewhere classified

  2016 2017 2018 2019 2020 Billable/Specific Code

  Applicable To

  • Hepatosplenomegaly NOS
  • hyperlipemic E78.3 (Bürger-Grütz type)
• Hyperchylomicronemia (familial) (primary) E78.3
  • with hyperbetalipoproteinemia E78.3
• Hyperglyceridemia (endogenous) (essential) (familial) (hereditary) (pure) E78.1
  ICD-10-CM Diagnosis Code E78.1

  Pure hyperglyceridemia

  2016 2017 2018 2019 2020 Billable/Specific Code

  Applicable To

  • Elevated fasting triglycerides
  • Endogenous hyperglyceridemia
  • Fredrickson's hyperlipoproteinemia, type IV
  • Hyperlipidemia, group B
  • Hyperprebetalipoproteinemia
  • Very-low-density-lipoprotein-type [VLDL] hyperlipoproteinemia
  • mixed E78.3
• Hyperlipemia, hyperlipidemia E78.5
  ICD-10-CM Diagnosis Code E78.5

  Hyperlipidemia, unspecified

  2016 2017 2018 2019 2020 Billable/Specific Code
  • group
    • D E78.3
• Hyperlipoproteinemia E78.5
  ICD-10-CM Diagnosis Code E78.5

  Hyperlipidemia, unspecified

  2016 2017 2018 2019 2020 Billable/Specific Code
  • Fredrickson's type
    • I E78.3
    • V E78.3
• Lipemia - see also Hyperlipidemia
  • retina, retinalis E78.3
• Lipidosis E75.6
  ICD-10-CM Diagnosis Code E75.6

  Lipid storage disorder, unspecified

  2016 2017 2018 2019 2020 Billable/Specific Code
  • hepatosplenomegalic E78.3
• Prebetalipoproteinemia (acquired) (essential) (familial) (hereditary) (primary) (secondary) E78.1
  ICD-10-CM Diagnosis Code E78.1

  Pure hyperglyceridemia

  2016 2017 2018 2019 2020 Billable/Specific Code

  Applicable To

  • Elevated fasting triglycerides
  • Endogenous hyperglyceridemia
  • Fredrickson's hyperlipoproteinemia, type IV
  • Hyperlipidemia, group B
  • Hyperprebetalipoproteinemia
  • Very-low-density-lipoprotein-type [VLDL] hyperlipoproteinemia
  • with chylomicronemia E78.3
• Syndrome - see also Disease
  • Bürger-Grütz E78.3
• Xanthoma(s), xanthomatosis (primary) (familial) (hereditary) E75.5
  ICD-10-CM Diagnosis Code E75.5

  Other lipid storage disorders

  2016 2017 2018 2019 2020 Billable/Specific Code

  Applicable To

  • Cerebrotendinous cholesterosis [van Bogaert-Scherer-Epstein]
  • Wolman's disease
  • with
    • hyperlipoproteinemia
      • Type I E78.3
      • Type V E78.3

Reimbursement claims with a date of service on or after October 1, 2015 require the use of ICD-10-CM codes.