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May 18, 1984

Dual Defect in Metabolism of Very-Low-Density Lipoprotein Triglycerides: Patients With Type 5 Hyperlipoproteinemia

Author Affiliations

From the Veterans Administration Medical Center, and the Department of Medicine, University of California, San Diego. Dr Grundy is now with the University of Texas Health Sciences Center, Dallas, and Dr Kesaniemi with the University of Helsinki, Finland.

JAMA. 1984;251(19):2542-2547. doi:10.1001/jama.1984.03340430040026

Transport of very-low-density lipoprotein (VLDL) triglycerides (TGs) were studied in eight patients with type 5 hyperlipoproteinemia (HLP). Results were compared to those from patients with type 4 HLP. Type 4 patients had either overproduction of VLDL TGs (nine patients) or defective clearance of VLDL TGs (eight patients). Patients with type 5 HLP were found to have increased production rates of VLDL TG, but also reduced fractional catabolic rates of VLDL TGs. Synthesis rates of VLDL TGs in type 5 were similar to those in type 4 patients with overproduction of VLDL TGs. The mean fractional catabolic rate in patients with type 5 HLP also were similar to those of type 4 patients with defective clearance. This study demonstrates that in most patients with type 5 HLP there is a dual defect in TG metabolism—overproduction of VLDL TGs and a clearance defect for TG-rich lipoproteins.

(JAMA 1984;251:2542-2547)