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Published on: September 15, 2018
Dietary treatment for familial hypercholesterolaemia
1Evidence Based Child Health Unit, Institute of Child Health, Royal Liverpool Children's Hospital NHS Trust, Alder Hey, Eaton Road, Liverpool, Merseyside, UK, L12 2AP. v.poustie@liv.ac.uk
Insights
Limited research exists on dietary interventions for familial hypercholesterolaemia (FH). Current evidence is insufficient to determine the effectiveness of cholesterol-lowering diets compared to other interventions for FH patients.
Area of Science:
- Cardiology
- Genetics
- Nutritional Science
Background:
- Familial hypercholesterolaemia (FH) is an inherited condition causing high blood cholesterol and premature heart disease.
- Dietary management is crucial, especially for children, as many drugs are not licensed for pediatric use.
- Current treatment guidelines for FH diets lack consensus.
Purpose of the Study:
- To evaluate the efficacy of cholesterol-lowering diets versus no intervention or other diets in managing FH.
- To assess the impact of dietary interventions on cholesterol levels and ischemic heart disease incidence in FH patients.
Main Methods:
- Searched specialized trial registers and relevant journals for randomized controlled trials (RCTs).
- Included published and unpublished RCTs comparing cholesterol-lowering diets with other diets or no intervention in FH patients.
- Data extraction and quality assessment were performed independently by two reviewers.
Main Results:
- Only five eligible studies were identified, limiting the assessment to short-term outcomes.
- Treatment compliance, quality of life, mortality, and ischemic disease evidence were not evaluated.
- No significant differences were observed between cholesterol-lowering diets and other diets for assessed short-term outcomes.
Conclusions:
- Insufficient data prevents conclusions on the effectiveness of cholesterol-lowering diets or other interventions for FH.
- A large RCT is needed to establish the effectiveness of various dietary strategies for FH.
- Current FH dietary management should be carefully monitored pending further evidence.
Background:
Familial hypercholesterolaemia is an inherited disorder characterised by a raised blood cholesterol, the presence of xanthomatosis and premature ischaemic heart disease. The aim of treatment is the reduction of blood LDL cholesterol concentrations in order to reduce the risk of ischaemic heart disease. Current treatment is based on a cholesterol lowering diet alone or in combination with drug therapy. Many of the drugs found to be effective in treating adults with this disease are not licensed for use in children, therefore diet is the main treatment of children with familial hypercholesterolaemia. In addition to the cholesterol-lowering diet, several other dietary interventions have been suggested and consensus has yet to be reached on the most appropriate dietary treatment 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 than other dietary interventions.
Search Strategy:
We searched the Cochrane Cystic Fibrosis and Genetic Disorders Trials Register, a specialist trials register which comprises references identified from comprehensive electronic database searches, handsearching relevant journals and handsearching abstract books of conference proceedings. Additional studies were identified from handsearching the Journal of Inherited Metabolic Disease (from inception, 1978 to 2000) and from the reference lists of identified studies.
Selection Criteria:
Randomised controlled trials (RCTs), 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. Trials which include patients with familial hypercholesterolaemia alongside patients with non-familial hypercholesterolaemia were only included if the group of familial patients was well defined and the results for these patients 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 five eligible studies. Compliance to treatment, quality of life, mortality and evidence of ischaemic or atheromatous disease were not assessed in the studies identified. No differences were found between the cholesterol-lowering diet and all other diets for all of the short term outcomes assessed.
Reviewer'S Conclusions:
No conclusions can be made about the effectiveness of the cholesterol-lowering diet, or any of the other dietary interventions suggested for familial hypercholesterolaemia, due to the lack of adequate data. A large, parallel, randomised controlled trial is needed to investigate the effectiveness of the cholesterol-lowering diet and other dietary interventions for FH. It is also possible that data from trials including subjects 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.
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