In three siblings with idiopathic hyperlipemia, administration of heparin failed to release lipoprotein lipase into the circulation, suggesting a genetic deficiency of the enzyme causes their defective triglyceride removal.
Observational (n=10)
No
Does impaired lipoprotein lipase activity cause defective removal of exogenous fat in patients with idiopathic hyperlipemia?
This early metabolic study provides evidence that a familial form of idiopathic hyperlipemia is caused by a defect in the release or function of lipoprotein lipase, leading to impaired chylomicron clearance.
The clinical syndrome of idiopathic hyperlipemia is, characterized by lactescent blood plasma in the postabsorptive state. The milky appearance of the plasma is caused by its abnormally high content of triglyceride-rich lipoproteins of high molecular weight. Omission of fat from the diet of such patients decreases the concentration of triglycerides, suggesting that defective removal of exogenous fat from the circulation may be an important meta
Havel et al. (Thu,) conducted a observational in Idiopathic hyperlipemia (n=10). Heparin administration and dietary fat restriction vs. Healthy volunteers and other hyperlipemic subjects was evaluated on Lipoprotein lipase activity and triglyceride removal from plasma. In three siblings with idiopathic hyperlipemia, administration of heparin failed to release lipoprotein lipase into the circulation, suggesting a genetic deficiency of the enzyme causes their defective triglyceride removal.