Hyperlipoproteinemia Type I

**Definition:** 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.

**Tree numbers:** - C16.320.565.398.465 - C18.452.584.500.500.644.237 - C18.452.584.563.465 - C18.452.648.398.465

**Synonyms:** - Burger-Grutz Syndrome - Chylomicronemia, Familial - Familial Chylomicronemia Syndrom - Familial Hyperchylomicronemia - Familial Hyperchylomicronemia Syndrome - Familial Hyperlipoproteinemia Type 1 - Familial Lipoprotein Lipase Deficiency - Familial LPL Deficiency - Hyperchylomicronemia, Familial - Hyperlipemia, Essential Familial - Hyperlipemia, Idiopathic, Burger-Grutz Type - Hyperlipoproteinemia Type Ia - Hyperlipoproteinemia, Type 1 - Hyperlipoproteinemia, Type I - Hyperlipoproteinemia, Type Ia - Lipase D Deficiency - LIPD Deficiency - Lipoprotein Lipase Deficiency - Lipoprotein Lipase Deficiency, Familial