Abstract

A cholesterol-lowering diet and several other dietary interventions have been suggested as a management approach either independently or as an adjuvant to drug therapy in children and adults with familial hypercholesterolemia. However, a consensus has yet to be reached on the most appropriate dietary treatment. To examine whether a cholesterol-lowering diet is more effective in reducing ischaemic heart disease and lowering cholesterol than no dietary intervention in children and adults with familial hypercholesterolaemia. Further, to compare the efficacy of supplementing a cholesterol-lowering diet with either omega-3 fatty acids, soya proteins, plant sterols or plant stanols. We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Inborn Errors of Metabolism Trials Register.Most recent search of the Group's Inborn Errors of Metabolism Trials Register: 09 October 2009.We also searched PubMed till 01 June 2008. Randomised controlled trials, both published and unpublished, where a cholesterol-lowering diet in children and adults with familial hypercholesterolaemia has been compared to other forms of dietary treatment or to no dietary intervention were included. Two authors independently assessed the trial eligibility and methodological quality and one extracted the data, with independent verification of data extraction by a colleague. In the present update, four new trials have been added making eleven trials with a total of 331 participants eligible for inclusion. Only short-term outcomes could be assessed due to the short duration of follow up in the included studies. None of the primary outcomes, (incidence of ischaemic heart disease, number of deaths and age at death) were evaluated in any of the included studies. No significant difference was noted for the majority of secondary outcomes for any of the planned comparisons. However, a significant difference was found only for the following comparison and outcome: total cholesterol levels for the comparison between plant sterols and cholesterol-lowering diet, mean difference 0.70 (95% confidence interval 0.19 to 1.21). No conclusions can be made about the effectiveness of a cholesterol-lowering diet, or any of the other dietary interventions suggested for familial hypercholesterolaemia, due to the lack of adequate data. Large, parallel, randomised controlled trials are needed to investigate the effectiveness of a cholesterol-lowering diet and the addition of omega-3 fatty acids, plant sterols or stanols, soya protein to a cholesterol-lowering diet.

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