Lovastatin therapy in receptor-negative homozygous familial hypercholesterolemia: Lack of effect on low-density lipoprotein concentrations or turnover
- 1 August 1988
- journal article
- research article
- Published by Elsevier BV in The Journal of Pediatrics
- Vol. 113 (2), 387-392
- https://doi.org/10.1016/s0022-3476(88)80289-0
Abstract
To determine whether at least part of the fall in low density lipoprotein (LDL) levels during lovastatin therapy might be the result of a reduced secretion of lipoproteins by the liver, three children 6 to 9 years of age with receptor-negative homozygous familial hypercholesterolemia underwent treatment with lovastatin. These patients have no capacity to synthesize LDL receptors. During lovastatin therapy, at dose of 12 mg/kg/day, there was no decrease in LDL-cholesterol levels, nor was the turnover rate of LDL affected by the drug. The only significant change was a 74% drop in very low-density lipoprotein during treatment. We conclude that lovastatin is not effective in treatment of receptor-negative homozygous familial hypercholesterolemia. The most likely mechanism of action for this drug is to increase LDL receptor activity.This publication has 19 references indexed in Scilit:
- The Effect of Mevinolin on Steroidogenesis in Patients with Defects in the Low Density Lipoprotein Receptor PathwayJournal of Clinical Endocrinology & Metabolism, 1987
- Development of an integrated model for analysis of the kinetics of apolipoprotein B in plasma very low density lipoproteins, intermediate density lipoproteins, and low density lipoproteins.JCI Insight, 1985
- Defective Lipoprotein Receptors and AtherosclerosisNew England Journal of Medicine, 1983
- Mevinolin and colestipol stimulate receptor-mediated clearance of low density lipoprotein from plasma in familial hypercholesterolemia heterozygotes.Proceedings of the National Academy of Sciences of the United States of America, 1983
- Regulatory role for hepatic low density lipoprotein receptors in vivo in the dog.Proceedings of the National Academy of Sciences of the United States of America, 1981
- Therapeutic effects of ML-236B in primary hypercholesterolemiaAtherosclerosis, 1980
- Metabolic Studies in Familial HypercholesterolemiaJCI Insight, 1979
- Simultaneous measurement of apolipoprotein B turnover in very-low- and low-density lipoproteins in familial hypercholesterolaemiaAtherosclerosis, 1977
- Competitive inhibition of 3‐hydroxy‐3‐methylglutaryl coenzyme a reductase by ML‐236A and ML‐236B fungal metabolites, having hypocholesterolemic activityFEBS Letters, 1976
- Reduction in cholesterol and low density lipoprotein synthesis after portacaval shunt surgery in a patient with homozygous familial hypercholesterolemia.JCI Insight, 1975