E78.3

Applicable To

• Chylomicron retention disease

• Fredrickson’s hyperlipoproteinemia, type I or V

• Hyperlipidemia, group D

• Mixed hyperglyceridemia

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.

Details

• E78.3 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes.

• The 2023 edition of ICD-10-CM E78.3 became effective on October 1, 2022.

• This is the American ICD-10-CM version of E78.3 – other international versions of ICD-10 E78.3 may differ.