Hyperlipoproteinemia Type I (Lipoprotein Lipase Deficiency, Familial)

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.
Also Known As:
Lipoprotein Lipase Deficiency, Familial; Apolipoprotein C II Deficiency; Familial Hyperchylomicronemia; Familial Lipoprotein Lipase Deficiency; Hyperchylomicronemia, Familial; Familial Hyperlipoproteinemia Type 1; Hyperlipoproteinemia Type Ia; Hyperlipoproteinemia Type Ib; Apolipoprotein C-II Deficiencies; Deficiencies, Apolipoprotein C-II; Deficiency, Apolipoprotein C-II; Familial Hyperchylomicronemias; Hyperchylomicronemias, Familial; Hyperlipoproteinemia Type Ias; Hyperlipoproteinemia Type Ibs; Hyperlipoproteinemia Type Is; Apolipoprotein C-II Deficiency
Networked: 66 relevant articles (0 outcomes, 6 trials/studies)

Disease Context: Research Results

Related Diseases

1. Hypertriglyceridemia
2. Pancreatitis
3. Hyperlipoproteinemia Type I (Lipoprotein Lipase Deficiency, Familial)
4. Hyperlipoproteinemias
5. Hyperlipoproteinemia Type II (Familial Hypercholesterolemia)


1. Stefanutti, Claudia: 1 article (05/2015)
2. Julius, Ulrich: 1 article (05/2015)
3. Okubo, Minoru: 1 article (01/2015)
4. Toromanovic, Alma: 1 article (01/2015)
5. Murase, Toshio: 1 article (01/2015)
6. Ebara, Tetsu: 1 article (01/2015)
7. Yamashita, Shizuya: 1 article (09/2013)
8. Brasen, Claus Lohman: 1 article (07/2013)
9. Overgaard, Martin: 1 article (07/2013)
10. Feddersen, Søren: 1 article (07/2013)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Hyperlipoproteinemia Type I:
1. Lipoprotein Lipase (Diacylglycerol Lipase)IBA
2. Lipoproteins (Lipoprotein)IBA
3. Lipase (Acid Lipase)FDA Link
4. Apolipoprotein C-II (ApoC2)IBA
5. Triglycerides (Triacylglycerol)IBA
6. Factor XII (Hageman Factor)IBA
7. ApolipoproteinsIBA
8. LipidsIBA
9. Insulin (Novolin)FDA Link
10. CholesterolIBA

Therapies and Procedures

1. Parenteral Nutrition