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Normal cholesterol levels with lovastatin (Mevinolin) therapy in a child with homozygous familial hypercholesterolemia following liver transplantation

Journal Article · · JAMA, J. Am. Med. Assoc.; (United States)
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Patients with homozygous familial hypercholesterolemia produce no normal low-density lipoprotein (LDL) receptors, and as a result, LDL accumulates in plasma, causing severe premature atherosclerosis. Two years ago, liver transplantation was performed in a child with homozygous familial hypercholesterolemia, restoring LDL receptor activity to about 60% of normal and reducing the LDL cholesterol level by 81%. However, the patient's lipoprotein levels remained significantly elevated for her age and sex. Treatment with lovastatin (mevinolin) one year after transplantation produced a marked improvement in the patient's lipoprotein profile. The total and LDL cholesterol levels fell 40% and 49%, respectively, to values within the normal range. The level of very low-density lipoprotein cholesterol fell 41%, and the level of total triglycerides declined 28%. While lovastatin therapy decreased the production rate of LDL by 35%, it did not affect the LDL fractional clearance rate. Thus, the combination of liver transplantation and lovastatin restored total and LDL cholesterol levels to normal in this patient with homozygous familial hypercholesterolemia.

Research Organization:
Univ. of Texas Health Science Center, Dallas
OSTI ID:
6906732
Journal Information:
JAMA, J. Am. Med. Assoc.; (United States), Journal Name: JAMA, J. Am. Med. Assoc.; (United States) Vol. 256:20; ISSN JAMAA
Country of Publication:
United States
Language:
English

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Related Subjects

550601* -- Medicine-- Unsealed Radionuclides in Diagnostics
551001 -- Physiological Systems-- Tracer Techniques
59 BASIC BIOLOGICAL SCIENCES
62 RADIOLOGY AND NUCLEAR MEDICINE
AGE GROUPS
ARTERIOSCLEROSIS
BETA DECAY RADIOISOTOPES
BLOOD CHEMISTRY
BODY
CARDIOVASCULAR DISEASES
CHEMOTHERAPY
CHILDREN
CHOLESTEROL
DAYS LIVING RADIOISOTOPES
DIGESTIVE SYSTEM
DISEASES
ELECTRON CAPTURE RADIOISOTOPES
EVALUATION
GLANDS
HEREDITARY DISEASES
HYDROXY COMPOUNDS
INTERMEDIATE MASS NUCLEI
IODINE 125
IODINE ISOTOPES
ISOTOPES
KIDNEYS
LIPIDS
LIPOPROTEINS
LIVER
METABOLIC DISEASES
NUCLEI
ODD-EVEN NUCLEI
ORGANIC COMPOUNDS
ORGANS
PROTEINS
RADIOISOTOPES
RADIONUCLIDE KINETICS
STEROIDS
STEROLS
THERAPY
TRANSPLANTS
VASCULAR DISEASES