Nutrition and dietary interventions for treatment and management of familial hypercholesterolaemia in children and adolescents: A systematic review.

Aims: Familial hypercholesterolaemia (FH) is a common inherited metabolic disease caused by mutations of genes involved in low-density cholesterol (LDL-C) catabolism. The accumulation of LDL-C increases the risk of early atherosclerosis and premature coronary artery disease. The aim of this systematic review was to investigate the impact of dietary interventions to lower total cholesterol (TC) and LDL-C in FH children not on medication.

Data synthesis: We searched three databases to March 2024 to identify randomised-controlled trials (RCTs), crossover and non-randomised observational studies of dietary interventions conducted in children and adolescents, 2-<19 years, with FH and measuring TC and LDL-C pre- and post-intervention. Twenty-eight studies were found, testing interventions with modified percent of energy from fat, change in type of fat consumed, functional foods e.g., plant sterols, fibre and modification of specific nutrients. 8 studies using plant sterols (1.21 ± 0.13-2.76 ± 0.15g/day) demonstrated significant LDL-C reductions ranging from 10 to 18 % compared to control groups. Modifying fat content using rapeseed oil (59 % monounsaturated fat @15g/day) showed a 7 % reduction in LDL-C. 7 studies of functional foods showed reductions in LDL -C ranging from 4 % (calcium carbonate) to 27.5 % after consumption of pulverized blueberry tea with 32 mg/kg anthocyanins. 6 studies of dietary counselling interventions reported reductions in LDL-C from 3 to 22 %.

Conclusions: The addition of plant sterols had a significant cholesterol-lowering effect for children with FH from age 5. Additional studies are needed to investigate the efficacy of all other dietary interventions.

Registry number: PROSPERO (CRD42023392111).