Normal cholesterol levels with lovastatin (mevinolin) therapy in a child with homozygous familial hypercholesterolemia following liver transplantation
C. East, S. M. Grundy and D. W. Bilheimer
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.