TY - JOUR
T1 - Pravastatin in heterozygous familial hypercholesterolemia
T2 - Low-density lipoprotein (LDL) cholesterol-lowering effect and LDL receptor activity on skin fibroblasts
AU - Gaddi, A.
AU - Arca, M.
AU - Ciarrocchi, A.
AU - Fazio, S.
AU - D'Alò, G.
AU - Tiozzo, R.
AU - Descovich, G. C.
AU - Calandra, S.
N1 - Funding Information:
From the Cattedra di Medicina Interna e Centro Aterosclerosi. Universirti di Boiogna, Bologna; Istitufo di Terapia Medica Sistemati-ca, Universitci di Roma. Roma; Squibb SPA, Roma; and rhe Istituto Patologia Generale, Vniversitri di Modena. Modena, Ita[y. Supported in part by grants of the Emilia Romagna Region (Health Research Finalized Programmes) and by the Universities of Bologna and Modena. Address reprint requests to G. D’AU, MD, Squibb SPA. Via Paolo di Dono 73, Rome, Italy. Copyright 0 1991 by W.B. Saunders Company 0086-0495191 IlOIO-0014$03.00/O
PY - 1991/10
Y1 - 1991/10
N2 - The cholesterol-lowering effect of pravastatin, a new competitive inhibitor of 3-hydroxy-3-methyl-glutaryl coenzyme A (HMG-CoA) reductase, was studied in 10 patients with heterozygous familial hypercholesterolemia (FH). Residual low-density lipoprotein receptor (LDL-R) activity was also evaluated in cultured skin fibroblasts prior to treatment, and showed a wide range of reduction from 30% to 70% of the normal value. Treatment with pravastatin 40 mg once daily reduced total and LDL cholesterol (LDL-C) after 6 months by 19.7% and 25.4%, respectively (P < .001). Serum apolipoprotein (apo) B levels decreased significantly by 29.1% (P < .001). No significant changes were observed in mean serum total triglycerides or high-density lipoprotein cholesterol (HDL-C) levels. A positive correlation between residual LDL-R activity and maximum percent reduction of LDL-C levels was observed (r = .676, P < .05). No clinically important side effects were recorded and the treatment was well tolerated. Thus, pravastatin effectively reduces LDL in heterozygous FH, and this effect appears to be related to LDL-R status.
AB - The cholesterol-lowering effect of pravastatin, a new competitive inhibitor of 3-hydroxy-3-methyl-glutaryl coenzyme A (HMG-CoA) reductase, was studied in 10 patients with heterozygous familial hypercholesterolemia (FH). Residual low-density lipoprotein receptor (LDL-R) activity was also evaluated in cultured skin fibroblasts prior to treatment, and showed a wide range of reduction from 30% to 70% of the normal value. Treatment with pravastatin 40 mg once daily reduced total and LDL cholesterol (LDL-C) after 6 months by 19.7% and 25.4%, respectively (P < .001). Serum apolipoprotein (apo) B levels decreased significantly by 29.1% (P < .001). No significant changes were observed in mean serum total triglycerides or high-density lipoprotein cholesterol (HDL-C) levels. A positive correlation between residual LDL-R activity and maximum percent reduction of LDL-C levels was observed (r = .676, P < .05). No clinically important side effects were recorded and the treatment was well tolerated. Thus, pravastatin effectively reduces LDL in heterozygous FH, and this effect appears to be related to LDL-R status.
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U2 - 10.1016/0026-0495(91)90132-G
DO - 10.1016/0026-0495(91)90132-G
M3 - Article
C2 - 1658544
AN - SCOPUS:0026004496
SN - 0026-0495
VL - 40
SP - 1074
EP - 1078
JO - Metabolism
JF - Metabolism
IS - 10
ER -