title: Lipid and lipoprotein profiles in children with familial hypercholesterolaemia: effects of therapy.
abstract: In 71 children with familial hypercholesterolaemia the effect of dietary and/or medical treatment was evaluated. Initial total cholesterol and low density lipoprotein (LDL)-cholesterol levels were significantly lower in children who were consecutively treated by diet (Step-One-Diet) than in those who received additional medication. By dietary treatment, the median total cholesterol level (236.5 mg/dl; range 210-510 mg/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 reduced by 29.7% and the median LDL-cholesterol level (263 mg/dl; 192-333 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 levels did not change during therapy. Initial apolipoprotein B levels were significantly higher in children who were treated by diet and medication and were reduced by 28.9% by combined therapy. In 28 patients (39.4%) an excess of lipoprotein (a) was detected. Regarding the apolipoprotein E phenotype, 32.2% of the patients carried the risk gene epsilon4 in a hetero- or homozygous form.
  
  
  CONCLUSIONS
  Early dietary and/or medical treatment in hypercholesterolaemic children significantly ameliorates the lipoprotein status. The pretherapy lipoprotein status seems to prognosticate the effectiveness of therapy.
authors.affiliation: Children's Hospital, Johann Wolfgang Goethe, University, Frankfurt/Main, Germany. jhennerm@charite.de
authors.forename: J B
authors.initials: JB
authors.lastname: Hennermann
authors.forename: J
authors.initials: J
authors.lastname: Herwig
authors.forename: W
authors.initials: W
authors.lastname: März
authors.forename: F
authors.initials: F
authors.lastname: Asskali
authors.forename: H J
authors.initials: HJ
authors.lastname: Böhles
mesh_terms: D000293:Adolescent; D000328:Adult; D001053:Apolipoproteins / Q000097:blood; D002648:Child; D002675:Child, Preschool; D008076:Cholesterol, HDL / Q000097:blood; D008078:Cholesterol, LDL / Q000097:blood; D005260:Female; D006801:Humans; D006938:Hyperlipoproteinemia Type II / Q000097:blood* / Q000178:diet therapy / Q000188:drug therapy* / Q000628:therapy*; D007223:Infant; D007231:Infant, Newborn; D008055:Lipids / Q000097:blood*; D008074:Lipoproteins / Q000097:blood*; D008297:Male; D012189:Retrospective Studies; D014280:Triglycerides / Q000097:blood
chemical_list: D001053:Apolipoproteins; D008076:Cholesterol, HDL; D008078:Cholesterol, LDL; D008055:Lipids; D008074:Lipoproteins; D014280:Triglycerides