OBJECTIVE

Familial hypercholesterolemia (FH) is a predominantly inherited disorder, which contributes to a defect of the LDL-cholesterol receptor. For adults with familial hypercholesterolemia (FH), it is known that a supplementary diet of monounsaturated fatty acids reduces elevated levels of total cholesterol and LDL-cholesterol and may further increase HDL-cholesterol. In particular the reduced intake of dietary fat reduces total serum cholesterol and LDL-cholesterol in the range of 10% to 15% and inhibits LDL-oxidation. Once the diagnosis of familial hypercholesterolemia is made in early childhood a supplementary diet with rapeseed oil should be started as early as possible to prevent development of atherosclerosis and subsequent complications. So far there are no reports of a lipid lowering diet enriched with rapeseed oil in children and adolescents.

METHODS

Seventeen children and young adolescents (male = 6, female = 11, ages 4 to 19 years) diagnosed with FH were enrolled in this study. They received dietary training and a classical low fat/low cholesterol diet enriched with rapeseed oil over five months. In the first two months they received orally mean 15 g/day (8-23 g/day), for the remaining three months mean 22 g/day (15-30 g/day) rapeseed oil. The calculation of the three-days dietary protocols showed the following characteristics: 29.5% calories from fat, 14.3% calories from protein and 54.6% calories from carbohydrates. The subjects had six sessions of dietary counseling, and serum lipids levels and lipoprotein(a) were estimated; each month's diet adherence was controlled by a dietitian and discussed with the patients and their families during this five-month study.

RESULTS

During five months of rapeseed oil diet serum triglycerides decreased by 29% (119.2+/-62.8 mg/dL vs. 84.9 mean +/- 39.7 mg/dL), VLDL-cholesterol by 27% (23+/-12 mg/dL vs. 17+/-8 mg/dL), total cholesterol by 10% (233+/-35 mg/dL vs. 213+/-36 mg/dL), LDL-cholesterol by 7% (151+/-31 mg/dL vs. 142+/-31 mg/dL). HDL-cholesterol (59+/-15 mg/dL vs. 57+/-11 mg/dL) and Lp(a) (29.8+/-36.3 mg/dL vs. 32.6+/-40.7 mg/dL) were not changed significantly. The diet was well accepted; in most families a sustained change was reported.

CONCLUSIONS

Our results indicate that in children and adolescents with FH a lipid-lowering diet with rapeseed oil has a similar effect on total serum cholesterol and LDL-cholesterol compared to classical cholesterol reduction diets (step I). However, an additional pronounced effect on lowering of triglycerides and VLDL-cholesterol can be observed.