Conditions / Genetic

familial lipoprotein lipase deficiency

info · Genetic · ICD-10: E78.3

A familial hyperlipemia characterized by a deficiency of the enzyme lipoprotein lipase and the subsequent build up of chylomicrons and increased plasma concentration of triglycerides.

Signs and symptoms

  • Eruptive xanthomas
  • Hypercholesterolemia
  • Lactescent serum
  • Acute pancreatitis
  • Lipemia retinalis
  • Vomiting
  • Episodic abdominal pain
  • Hyperlipidemia
  • Increased circulating chylomicron concentration
  • Nausea

Medications that may treat it

volanesorsen

Also known as: Fredrickson type I hyperlipoproteinemia; Fredrickson type I lipaemia; familial LPL deficiency; familial hyperlipoproteinemia type I; hypercholesterinaemic xanthomatosis