Dietary treatment for familial hypercholesterolaemia (Cochrane Review)

A substantive amendment to this systematic review was last made on 28 February 2001. Cochrane reviews are regularly checked and updated if necessary.

Background: Familial hypercholesterolaemia is an inherited disorder characterised by raised blood cholesterol, presence of xanthomatosis and premature heart disease. The aim of treatment is reduction of blood cholesterol concentrations to reduce the risk of heart disease. Current treatment is based on a cholesterol-lowering diet alone or in combination with drugs. Most drugs found to be effective in treating adults are not licensed for use in children, therefore diet is the main treatment of children with familial hypercholesterolaemia. Alternative dietary interventions have been suggested and consensus has yet to be reached on the appropriate diet for children and adults with familial hypercholesterolaemia.

Objectives: To examine the evidence that in children and adults with familial hypercholesterolaemia, a cholesterol-lowering diet is more effective at lowering cholesterol and reducing incidence of ischaemic heart disease than no intervention or other dietary interventions.

Search strategy: We searched the Cochrane Cystic Fibrosis and Genetic Disorders trials register which comprises references identified from comprehensive electronic database searches, handsearching relevant journals and abstract books of conference proceedings.Date of the most recent search of the Group's trials register: October 2002.

Selection criteria: Randomised controlled trials where a cholesterol-lowering diet in people with familial hypercholesterolaemia has been compared to other diets or no dietary intervention. Trials which include participants with familial hypercholesterolaemia alongside participants with non-familial hypercholesterolaemia were included if the group of familial participants was well-defined and the results for these participants were available.

Data collection and analysis: Two reviewers independently assessed the trial eligibility and methodological quality and one reviewer extracted the data, with independent verification of data extraction by a colleague.

Main results: Only short-term outcomes could be assessed in this review due to the length of the seven studies. Compliance to treatment, quality of life, mortality and evidence of ischaemic or atheromatous disease were not assessed. No differences were found between the cholesterol-lowering diet and other diets for the outcomes assessed.

Reviewers' conclusions: No conclusions can be made about the effectiveness of cholesterol-lowering diets, or other dietary interventions for familial hypercholesterolaemia, due to the lack of adequate data. A randomised controlled trial is needed to investigate dietary treatment for FH. It is possible that data from trials including participants with both familial and non-familial hypercholesterolaemia could alter the results of future updates of this review and until further evidence is available current dietary treatment of FH should continue to be observed and monitored with care.

Citation: Poustie VJ, Rutherford P. Dietary treatment for familial hypercholesterolaemia (Cochrane Review). In: The Cochrane Library, Issue 3, 2003. Oxford: Update Software.

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This is an abstract of a regularly updated, systematic review prepared and maintained by the Cochrane Collaboration. The full text of the review is available in The Cochrane Library (ISSN 1464-780X).

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