title: Dietary counseling is associated with an improved lipid profile in children with familial hypercholesterolemia.
abstract: Familial hypercholesterolemia (FH) is a genetic disorder characterized by elevated levels of total cholesterol (TC) and low-density lipoprotein cholesterol (LDL-C). Guidelines recommend cholesterol-lowering medication from 8 to 10 years of age and dietary recommendations. Little is known about the diet of FH children and the effect of dietary counseling. The aim of the study was to describe the diet of FH children with respect to fat quality, and to investigate if dietary counseling improved lipid profile.
  
  
  
  Fifty-four FH children (5-18 years) were included in the study and dietary intake was recorded with a pre-coded food diary for four days. Information about plasma lipid levels was obtained.
  
  
  
  Median intake of total fat, monounsaturated fat, polyunsaturated fat (PUFA) and saturated fat (SFA) was 30.8, 10.4, 5.9 and 12.0 E %, respectively. Among non-statin treated FH children, SFA intake was significantly correlated with TC, LDL-C and apolipoprotein (apo) B (rsp = 0.55; p = 0.004, rsp = 0.46; p = 0.02, and rsp = 0.45; p = 0.02, respectively), and PUFA/SFA ratio significantly inversely correlated with TC (rsp = -0.42; p = 0.03). Compared to the first visit, non-statin and non-plant sterol treated FH children (n = 10) had significantly reduced levels of TC (p < 0.01), LDL-C (p = 0.01), high-density lipoprotein cholesterol (p = 0.02), apo B (p = 0.05) and apo A-1 (p = 0.02) levels at a later visit.
  
  
  
  FH children had a higher intake of SFA than recommended and the SFA intake was positively correlated with plasma TC, LDL-C and apo B levels in FH children not using statins. Importantly, the plasma lipid profile was improved in FH children after dietary counseling where focus was on reducing intake of SFA and dietary cholesterol.
authors: Department of Nutrition, Institute of Basic Medical Sciences, University of Oslo, Oslo, Norway; The Lipid Clinic, Oslo University Hospital, Oslo, Norway.
authors: Kristin
authors: K
authors: Torvik
authors: Department of Nutrition, Institute of Basic Medical Sciences, University of Oslo, Oslo, Norway.
authors: Ingunn
authors: I
authors: Narverud
authors: Department of Nutrition, Institute of Basic Medical Sciences, University of Oslo, Oslo, Norway.
authors: Inger
authors: I
authors: Ottestad
authors: The Lipid Clinic, Oslo University Hospital, Oslo, Norway.
authors: Arne
authors: A
authors: Svilaas
authors: Oslo Centre for Biostatistics and Epidemiology, Department of Biostatistics, Institute of Basic Medical Sciences, University of Oslo, Oslo, Norway.
authors: Jon Michael
authors: JM
authors: Gran
authors: Department of Nutrition, Institute of Basic Medical Sciences, University of Oslo, Oslo, Norway; The Lipid Clinic, Oslo University Hospital, Oslo, Norway.
authors: Kjetil
authors: K
authors: Retterstøl
authors: The Lipid Clinic, Oslo University Hospital, Oslo, Norway.
authors: Asta
authors: A
authors: Ellingvåg
authors: The Lipid Clinic, Oslo University Hospital, Oslo, Norway.
authors: Ellen
authors: E
authors: Strøm
authors: Department of Nutrition, Institute of Basic Medical Sciences, University of Oslo, Oslo, Norway.
authors: Leiv
authors: L
authors: Ose
authors: Department of Nutrition, Institute of Basic Medical Sciences, University of Oslo, Oslo, Norway; Oslo Centre for Biostatistics and Epidemiology, Department of Biostatistics, Institute of Basic Medical Sciences, University of Oslo, Oslo, Norway.
authors: Marit B
authors: MB
authors: Veierød
authors: Department of Nutrition, Institute of Basic Medical Sciences, University of Oslo, Oslo, Norway; Norwegian National Advisory Unit on Familial Hypercholesterolemia, Oslo University Hospital, Rikshospitalet, P.O Box 4950, Nydalen, Norway. Electronic address: kirsten.holven@medisin.uio.no.
authors: Kirsten B
authors: KB
authors: Holven
mesh_terms: D000293:Adolescent; D001055:Apolipoproteins B / Q000097:blood; D002648:Child; D002675:Child, Preschool; D002784:Cholesterol / Q000097:blood; D002791:Cholesterol, Dietary; D008076:Cholesterol, HDL / Q000097:blood; D008078:Cholesterol, LDL / Q000097:blood; D003376:Counseling; D004032:Diet; D015930:Diet Records; D004041:Dietary Fats / Q000008:administration & dosage; D005227:Fatty Acids / Q000009:adverse effects; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D019161:Hydroxymethylglutaryl-CoA Reductase Inhibitors / Q000627:therapeutic use; D006938:Hyperlipoproteinemia Type II / Q000097:blood* / Q000178:diet therapy*; D008055:Lipids / Q000097:blood; D008297:Male; D014280:Triglycerides / Q000097:blood
chemical_list: D001055:Apolipoproteins B; D002791:Cholesterol, Dietary; D008076:Cholesterol, HDL; D008078:Cholesterol, LDL; D004041:Dietary Fats; D005227:Fatty Acids; D019161:Hydroxymethylglutaryl-CoA Reductase Inhibitors; D008055:Lipids; D014280:Triglycerides; D002784:Cholesterol
keywords: Children; Diet; Dietary fat; Familial hypercholesterolemia; Plasma cholesterol levels