LIPID AND LIPOPROTEIN PROFILES IN CHILDREN WITH FAMILIAL HYPERCHOLESTEROLEMIA - EFFECTS OF THERAPY

In 71 children with familial hypercholesterolaemia the effect of dieta ry and/or medical treatment was evaluated. Initial total cholesterol a nd low density lipoprotein (LDL)-cholesterol levels were significantly lower in children who were consecutively treated by diet (Step-One-Di et) than in those who received additional medication. By dietary treat ment, the median total cholesterol level (236.5 mg/dl; range 210-510 m g/dl) was reduced by 7.4% and the median LDL-cholesterol level (162 mg /dl; range 126-423 mg/dl) by 9.9%. By dietary and medical therapy, the median total cholesterol level (330 mg/dl; range 270-424 mg/dl) was r educed by 29.7% and the median LDL-cholesterol level (263 mg/dl; 192-3 33 mg/dl) by 25.9%. High density lipoprotein (HDL)-cholesterol and HDL 3 remained unchanged. HDL 2 showed a significant decrease of 15.6% up to 27 mg/dl (13-42 mg/dl) on medical treatment. Apolipoprotein A I le vels did not change during therapy. Initial apolipoprotein B levels we re significantly higher in children who were treated by diet and medic ation and were reduced by 28.9% by combined therapy. In 28 patients (3 9.4%) an excess of lipoprotein (a) was detected. Regarding the apolipo protein E phenotype, 32.2% of the patients carried the risk gene epsil on 4 in a hetero- or homozygous form. Conclusion Early dietary and/or medical treatment in hypercholesterolaemic children significantly amel iorates the lipoprotein status. The pretherapy lipoprotein status seem s to prognosticate the effectiveness of therapy.