Elsevier

Atherosclerosis

Volume 252, September 2016, Pages 21-27
Atherosclerosis

Dietary counseling is associated with an improved lipid profile in children with familial hypercholesterolemia

https://doi.org/10.1016/j.atherosclerosis.2016.07.913Get rights and content

Highlights

  • Guidelines recommend cholesterol-lowering medication and dietary recommendations from 8 to 10 years of age.

  • Little is known about the diet of children with FH and the effect of dietary counseling on plasma lipid Levels.

  • FH children had a higher intake of SFA than recommended.

  • SFA intake was positively correlated with plasma TC, LDL-C and apoB levels in FH children not using statins.

  • The plasma lipid profile was improved in FH children after dietary counseling where focus was on reducing intake of SFA and dietary cholesterol.

Abstract

Background and aims

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.

Methods

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.

Results

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.

Conclusions

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.

Introduction

Familial hypercholesterolemia (FH) is an autosomal dominantly inherited disease, mainly caused by a mutation in the gene encoding the low-density lipoprotein (LDL) receptor [1] resulting in a two-to threefold increase in circulating LDL cholesterol (LDL-C) levels [1], [2]. Treatment of FH is a lifelong process, as it is the total life-long exposure to LDL-C that may eventually lead to events of coronary heart disease [3]. Both medical and dietary treatments are recommended. The treatment target for primary prophylaxis in heterozygous FH is LDL-C below 3.5 mmol/L for children and below 2.5 mmol/l for adults [3]. However, in children cholesterol-lowering medication is not recommended until they reach the age of 8–10 years [4]. Total cholesterol (TC) and LDL-C levels can be modulated by dietary intake of fatty acids and cholesterol [5] and dietary recommendations are the first-line therapy before cholesterol-lowering medication may be initiated in children [6]. The dietary recommendations are according to the National Cholesterol Education Program (NCEP) and include the principles of a cholesterol-lowering diet, emphasizing a reduced intake of fat, especially saturated fat (SFA), and dietary cholesterol [7], [8].

Studies have shown that dietary adjustments can reduce plasma cholesterol levels by 10–30% in non-FH subjects [9], [10], [11]. However, a recent Cochrane review found that data were not adequate to conclude about the effectiveness of dietary modifications in FH subjects, except for that of plant sterols [12]. A review by Katan et al. concluded that the LDL-C can be lowered by 10% if consuming two grams of plant sterols per day, and that there was sufficient evidence to encourage the use of plant sterols in persons with elevated plasma cholesterol levels [13]. Little is known about the recommended cholesterol-lowering diet on plasma cholesterol in FH children. The aim of the study was therefore 1) to characterize the diet in children with FH with particular focus on dietary fat quality, 2) to investigate the relation between fat intake and plasma cholesterol levels, and 3) to investigate the effect of the dietary advices on plasma cholesterol levels in non-statin and non-plant sterol treated FH children.

Section snippets

Study sample

All children with FH aged 5–18 years that had an appointment at the outpatient Lipid Clinic, Oslo University Hospital, from September to December 2013 were invited to participate in the study. The children were invited to attend one study visit, subsequent to the already pre-scheduled appointment at the Lipid Clinic. Some of the children had never been to the Lipid Clinic before, other children had previously had an appointment at the Lipid Clinic (hereafter named follow-up patients).

Results

Characteristics of the study sample are shown in Table 1. The median age was 12 years (5–18 years, min-max). As expected, both TC and LDL-C were higher in the FH children compared to reference values. All other blood values were within normal range for age. There were few differences between girls and boys, except for plasma vitamin D levels which were significantly higher in girls (p = 0.004), and as expected, girls had a significantly higher plasma level of estradiol (p = 0.002) and a

Discussion

In the present study, intake of saturated fat was positively associated with plasma TC, LDL-C and apo B levels in FH children. A new and important finding was that the plasma lipid profile was improved in FH children after dietary counseling where focus was on reducing intake of SFA and dietary cholesterol despite the fact that FH children had higher intake of SFA than what is recommended.

Studies in children with FH have shown that initiation of statin therapy is safe when started from the age

Conflict of interest

During the past five years, Retterstøl has received honoraria for lectures or expert meetings from Merck, Pfizer, Mills DA, Melk.no, Apotek1, Pronova, Amgen, Genzyme, and Sanofi; none of which are related to the contents of this manuscript. During the past five years Holven has received research grants or honoraria from Tine, Mills, and Olympic Seafood, and Amgen; none of which are related to the contents of this manuscript. The rest of the authors declare no conflict of interest.

Financial support

This work was supported by grants from The Throne-Holst Foundation for Nutrition Research, Oslo, Norway, The National Advisory Unit on FH, Oslo University Hospital and the University of Oslo, Oslo, Norway.

References (51)

  • A. Wiegman et al.

    Familial hypercholesterolaemia in children and adolescents: gaining decades of life by optimizing detection and treatment

    Eur. heart J.

    (2015)
  • Fat and Fatty acids
  • Executive Summary of The Third Report of The National Cholesterol Education Program (NCEP) Expert Panel on Detection

    Evaluation, and treatment of high blood cholesterol in adults (Adult Treatment Panel III)

    Jama

    (2001)
  • D.J. Jenkins et al.

    The portfolio diet for cardiovascular risk reduction

    Curr. Atheroscler. Rep.

    (2007)
  • D.J. Jenkins et al.

    Effects of a dietary portfolio of cholesterol-lowering foods vs lovastatin on serum lipids and C-reactive protein

    Jama

    (2003)
  • N. Shafiq et al.

    Dietary treatment for familial hypercholesterolaemia

    Cochrane Database Syst. Rev.

    (2010)
  • M.A. Austin et al.

    Genetic causes of monogenic heterozygous familial hypercholesterolemia: a HuGE prevalence review

    Am. J. Epidemiol.

    (2004)
  • N. Vuorela et al.

    Prevalence of overweight and obesity in 5- and 12-year-old Finnish children in 1986 and 2006

    Acta Paediatr. (Oslo, Nor. 1992)

    (2009)
  • T.J. Cole et al.

    Establishing a standard definition for child overweight and obesity worldwide: international survey

    BMJ Clin. Res. ed)

    (2000)
  • P.B. Juliusson et al.

    Growth references for Norwegian children

    tidsskr. prakt. Med. ny raekke

    (2009)
  • L.F. Andersen et al.

    Validation of a pre-coded food diary used among 13-year-olds: comparison of energy intake with energy expenditure

    Public Health Nutr.

    (2005)
  • I.T. Lillegaard et al.

    Validation of a pre-coded food diary with energy expenditure, comparison of under-reporters v. acceptable reporters

    Br. J. Nutr.

    (2005)
  • I.T. Lillegaard et al.

    Relative validation of a pre-coded food diary among children, under-reporting varies with reporting day and time of the day

    Eur. J. Clin. Nutr.

    (2007)
  • I.T. Lillegaard et al.

    Can children and adolescents use photographs of food to estimate portion sizes?

    Eur. J. Clin. Nutr.

    (2005)
  • A. Svilaas et al.

    Reproducibility and validity of a short food questionnaire for the assessment of dietary habits. Nutrition, metabolism, and cardiovascular diseases

    NMCD

    (2002)
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