Postprandial Fatty Acid Metabolism in Subjects With Lipoprotein Lipase Deficiency

Inclusion Criteria:

- 8 healthy LPL-deficient individuals (LPLD subjects) with history of fasting TG > 5 mmol/l and homozygote or compound heterozygote for a LPL-gene mutation;

- 8 control subjects (fasting glucose < 5.6, 2-hour post 75g OGTT glucose < 7.8 mmol/l and HbA1c < 5.8%; fasting TG < 1.5 mmol/l);

- age 18 to 75 yo;

- To be willing and able to adhere to the specifications of the protocol;

- To have signed an informed consent document indicating that they understood the purpose

Exclusion Criteria:

- age < 18 yo;

- overt cardiovascular disease as assessed by medical history, physical exam, and abnormal ECG

- Treatment with a fibrate, thiazolidinedione, beta-blocker or other drug known to affect lipid or carbohydrate metabolism (except statins, metformin, and other antihypertensive agents that can be safely interrupted);

- Treatment with anti-hypertensive medication (only for LPL-deficient individuals);

- presence of liver or renal disease; uncontrolled thyroid disorder;

- previous diagnosis of heparin-induced thrombocytopenia;

- Treatment with oral anticoagulation medication or platelet aggregation inhibiting drugs;

- A history of major hemorrhagic event;

- smoking (>1 cigarette/day) and/or consumption of >2 alcoholic beverages per day;;

- Female of child-bearing potential who is pregnant, breast feeding or intends to become pregnant or pre-menopausal female with a positive serum pregnancy test at the time of enrollment.