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.
Applicable To
  • Chylomicron retention disease
  • Fredrickson's hyperlipoproteinemia, type I or V
  • Hyperlipidemia, group D
  • Mixed hyperglyceridemia
The following code(s) above E78.3 contain annotation back-references
Annotation Back-References
In this context, annotation back-references refer to codes that contain:
  • Applicable To annotations, or
  • Code Also annotations, or
  • Code First annotations, or
  • Excludes1 annotations, or
  • Excludes2 annotations, or
  • Includes annotations, or
  • Note annotations, or
  • Use Additional annotations
that may be applicable to E78.3:
  • E00-E89
    2020 ICD-10-CM Range E00-E89

    Endocrine, nutritional and metabolic diseases

    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.
    Type 1 Excludes
    • transitory endocrine and metabolic disorders specific to newborn (P70-P74)
    Endocrine, nutritional and metabolic diseases
  • E70-E88
    2020 ICD-10-CM Range E70-E88

    Metabolic disorders

    Type 1 Excludes
    • androgen insensitivity syndrome (E34.5-)
    • congenital adrenal hyperplasia (E25.0)
    • Ehlers-Danlos syndrome (Q79.6-)
    • hemolytic anemias attributable to enzyme disorders (D55.-)
    • Marfan's syndrome (Q87.4)
    • 5-alpha-reductase deficiency (E29.1)
    Metabolic disorders
  • E78
    ICD-10-CM Diagnosis Code E78

    Disorders of lipoprotein metabolism and other lipidemias

      2016 2017 2018 2019 2020 Non-Billable/Non-Specific Code
    Type 1 Excludes
    Disorders of lipoprotein metabolism and other lipidemias
Approximate Synonyms
  • Familial lipoprotein lipase deficiency
Clinical Information
  • 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.
ICD-10-CM E78.3 is grouped within Diagnostic Related Group(s) (MS-DRG v37.0):
  • 642 Inborn and other disorders of metabolism

Convert E78.3 to ICD-9-CM

Code History
  • 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

Diagnosis Index entries containing back-references to E78.3:
  • 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
  • Hyperlipoproteinemia E78.5
    ICD-10-CM Diagnosis Code E78.5

    Hyperlipidemia, unspecified

      2016 2017 2018 2019 2020 Billable/Specific Code
  • 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

ICD-10-CM Codes Adjacent To E78.3
E77.0 Defects in post-translational modification of lysosomal enzymes
E77.1 Defects in glycoprotein degradation
E77.8 Other disorders of glycoprotein metabolism
E77.9 Disorder of glycoprotein metabolism, unspecified
E78 Disorders of lipoprotein metabolism and other lipidemias
E78.0 Pure hypercholesterolemia
E78.00 …… unspecified
E78.01 Familial hypercholesterolemia
E78.1 Pure hyperglyceridemia
E78.2 Mixed hyperlipidemia
E78.3 Hyperchylomicronemia
E78.4 Other hyperlipidemia
E78.41 Elevated Lipoprotein(a)
E78.49 Other hyperlipidemia
E78.5 Hyperlipidemia, unspecified
E78.6 Lipoprotein deficiency
E78.7 Disorders of bile acid and cholesterol metabolism
E78.70 Disorder of bile acid and cholesterol metabolism, unspecified
E78.71 Barth syndrome
E78.72 Smith-Lemli-Opitz syndrome
E78.79 Other disorders of bile acid and cholesterol metabolism

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