Absorption and metabolism of cholesterol in familial hypercholesterolemia

1989 ◽  
Vol 76 (3) ◽  
pp. 297-301 ◽  
Author(s):  
Helena Gylling ◽  
Tatu A. Miettinen
Keyword(s):  
Cholesterol Synthesis ◽  
Familial Hypercholesterolaemia ◽  
Cholesterol Metabolism ◽  
Low Density Lipoprotein ◽  
Dietary Cholesterol ◽  
Density Lipoprotein ◽  
Cholesterol Absorption ◽  
Lipoprotein Cholesterol ◽  
Low Density ◽  
Cholesterol Intake

1. The present study investigated the role of intestinal cholesterol absorption in the regulation of cholesterol metabolism and serum lipoprotein levels in 22 patients with heterozygous familial hypercholesterolaemia on low to normal cholesterol intake. 2. The results showed that the higher the dietary cholesterol absorption, the lower was the overall synthesis of cholesterol. Efficient cholesterol absorption actually reduced the elimination of cholesterol as faecal neutral sterols but not consistently as bile acids. 3. In multifactorial analysis, body mass index and dietary plant sterols were negatively associated with cholesterol absorption, but were unrelated to cholesterol synthesis. 4. Fractional cholesterol absorption was related only to the serum very-low-density triacylglycerol level. It was not associated with the total or low-density lipoprotein cholesterol levels. On the other hand, cholesterol synthesis was significantly associated with the serum concentrations of very-low-density lipoprotein and intermediate-density lipoprotein cholesterol and triacylglycerols, and with those of low-density lipoprotein triacylglycerols. 5. In conclusion, dietary cholesterol absorption is an essential regulator of cholesterol homoeostasis in familial hypercholesterolaemia, even in patients on low cholesterol intake.

Treatment of familial hypercholesterolaemia in children and adolescents in the last three decades

2013 ◽  
Vol 24 (3) ◽  
pp. 437-441 ◽  
Author(s):  
Avishay Elis ◽  
Rong Zhou ◽  
Evan A. Stein
Keyword(s):  
Children And Adolescents ◽  
Familial Hypercholesterolaemia ◽  
Low Density Lipoprotein ◽  
Density Lipoprotein ◽  
Lipoprotein Cholesterol ◽  
Low Density ◽  
Low Density Lipoprotein Cholesterol ◽  
Cholesterol Levels ◽  
Heterozygous Familial Hypercholesterolaemia

AbstractBackground:This study evaluated the effectiveness of long-term intensive lipid-lowering therapy in children and adolescents with familial hypercholesterolaemia.Methods:The charts of 89 children and adolescents with heterozygous familial hypercholesterolaemia among ∼1000 patients treated from 1974 to 2008 were reviewed. Familial hypercholesterolaemia was defined as low-density lipoprotein cholesterol level >90th percentile in individuals with a history of familial hypercholesterolaemia.Results:Of the 89 patients, 51% were male; the mean age at diagnosis was 8 ± 4 years, and the mean follow-up was 13 ± 8 years. Baseline and most recent low-density lipoprotein cholesterol levels (mg/dl) under treatment were 250 ± 50 and 142 ± 49, respectively, reduced 43% from baseline (p < 0.0001). At the most recent visit, 39 patients received statin monotherapy, mainly atorvastatin or rosuvastatin, and 50 (56%) patients received combination therapy, mainly vytorin or rosuvastain/ezetimibe, 15 patients were >30 years of age, and none developed symptomatic cardiovascular disease or needed revascularisation.Conclusions:Long-term statin-based therapy can reduce low-density lipoprotein cholesterol levels in most children and adolescents with heterozygous familial hypercholesterolaemia and decrease cardiovascular risk significantly.

Serum Levels, Absorption Efficiency, Faecal Elimination and Synthesis of Cholesterol during Increasing Doses of Dietary Sitostanol Esters in Hypercholesterolaemic Subjects

1994 ◽  
Vol 87 (1) ◽  
pp. 61-67 ◽  
Author(s):  
H. T. Vanhanen ◽  
J. Kajander ◽  
H. Lehtovirta ◽  
T. A. Miettinen
Keyword(s):  
Dietary Fat ◽  
Rapeseed Oil ◽  
Low Density Lipoprotein ◽  
Density Lipoprotein ◽  
Serum Levels ◽  
Cholesterol Absorption ◽  
Absorption Efficiency ◽  
Lipoprotein Cholesterol ◽  
Low Density ◽  
Low Density Lipoprotein Cholesterol

1. Serum cholesterol reduction and changes in cholesterol metabolism were studied during rapeseed oil feeding without and with increasing amounts of sitostanol trans-esterified with rapeseed oil fatty acids and dissolved in rapeseed oil mayonnaise. Fifteen mildly hypercholesterolaemic subjects replaced 50 g of their usual dietary fat by 50 g of rapeseed oil fat mayonnaise for 6 weeks followed by randomization so that eight subjects continued on rapeseed oil mayonnaise alone (control group) for 15 weeks and seven on rapeseed oil mayonnaise with a small dose of sitostanol ester (800 mg/day of sitostanol) for 9 weeks followed by 6 weeks with higher dose of sitostanol ester (2000 mg/day of sitostanol). 2. During the rapeseed oil period the reduction in serum low-density lipoprotein cholesterol was 14% from the home diet. The control-adjusted reduction by the low sitostanol ester dose was 7.4% (not significant) and by the higher dose it was 15.7%. 3. The low dose of sitostanol ester had no consistent effect on cholesterol precursors or cholestanol in serum, reduced serum levels of campesterol and sitosterol by 28.2% and 23.6%, respectively, and reduced cholesterol absorption efficiency significantly from 28.7% to 23.4%. In accordance, faecal excretion of neutral and particularly endogenous neutral sterols increased (16.7% and 19.7%, respectively), but faecal cholesterol elimination and cholesterol synthesis were only insignificantly increased. 4. During the high dose of sitostanol ester the high-density lipoprotein- to low-density lipoprotein-cholesterol ratio increased. Serum levels of cholesterol precursor sterols, indicators of cholesterol synthesis, increased up to 12%, whereas those of cholestanol were slightly decreased and those of campesterol and sitosterol values were further reduced by 30% and 25.6%, respectively. 5. Associations of serum plant sterols and cholesterol precursors with cholesterol absorption efficiency and synthesis and the sitostanol-ester-induced changes in serum campesterol and lathosterol proportions with those in serum low-density lipoprotein-cholesterol suggest that reduced cholesterol absorption efficiency was the main reason for cholesterol reduction and that there was a compensatory increase in cholesterol synthesis. 6. The findings indicate that sitostanol ester dissolved in dietary fat is apparently unabsorbable and interferes with sterol absorption so that the serum levels of cholesterol and low-density lipoprotein-cholesterol are reduced to the extent that sitostanol ester-fat mixture in reasonable daily amounts can be recommended to replace dietary fat for lowering of serum cholesterol.

scholarly journals Differential Expression of Intestinal Genes in Opossums with High and Low Responses to Dietary Cholesterol

2010 ◽  
Vol 2010 ◽  
pp. 1-8 ◽  
Author(s):  
Jeannie Chan ◽  
Rampratap S. Kushwaha ◽  
Jane F. VandeBerg ◽  
Jelica Gluhak-Heinrich ◽  
John L. VandeBerg
Keyword(s):  
Low Density Lipoprotein ◽  
Dietary Cholesterol ◽  
Density Lipoprotein ◽  
Cholesterol Absorption ◽  
Low Density ◽  
Mrna Levels ◽  
High Cholesterol ◽  
Phospholipase Activity ◽  
Low Responders ◽  
High Responders

High and low responding opossums (Monodelphis domestica) differ in their plasma very low density lipoprotein and low density lipoprotein (VLDL+LDL) cholesterol concentrations when they consume a high cholesterol diet, which is due in part to absorption of a higher percentage of dietary cholesterol in high responders. We compared the expression of a set of genes that influence cholesterol absorption in high and low responders fed a basal or a high cholesterol and low fat (HCLF) diet. Up-regulation of theABCG5,ABCG8, andIBABPgenes by the HCLF diet in high and low responders may reduce cholesterol absorption to maintain cholesterol homeostasis. Differences in expression of the phospholipase genes (PLA2andPLB) and phospholipase activity were associated with differences in cholesterol absorption when opossums were fed cholesterol-enriched diets. HigherPLA2andPLBmRNA levels and higher phospholipase activity may increase cholesterol absorption in high responders by enhancing the release of cholesterol from bile salt micelles for uptake by intestinal cells.

Sign in / Sign up

Export Citation Format

Share Document