SUN-190 - Cognitive Function in Children with Heterozygous Familial Hypercholesterolemia

Introduction: Heterozygous familial hypercholesterolemia (HeFH) is the most common autosomal dominant genetic disorder and is a major cause of premature cardiovascular disease. However, there is limited information on cognitive functioning in this population. The objective of this study was to evaluate cognitive function, specifically the domains of processing speed and working memory, in children with familial hypercholesterolemia.

Methods: Cross-sectional study evaluating children aged 8 to 16 years with a confirmed diagnosis of HeFH. Before staring therapy with statins. The WISC-IV scale was used to assess working memory and processing speed domains. The lipid profile, the distribution of domains by normative categories of IQ, and the relationship between LDL-C levels and cognitive performance were analyzed using Pearson's correlation.

Results: 22 children with age of 10.7 ± 2.1 years , 12 (54.5%) boys with LDL-C of 202 ± 50.2 mg/dL were included. According to the normative IQ categories by domain, 11 (50%) were in the average range for working memory and 12 (54.5%) were in the average range for processing speed. However, 8 (36.2%) were in the low to below average range for working memory and 7 (31.7%) were in the low to below average range for processing speed. Although descriptively higher LDL-C levels were observed in the group with low/below-average cognitive performance, these differences did not reach statistical significance in any of the domains evaluated. The possible relationship between LDL-C levels and cognitive domains was explored using Pearson correlations. A weak and non-significant negative correlation was obtained with the working memory coefficient and for the processing speed coefficient (r(20) = –0.176, p = 0.433), (r(20) = 0.015, p = 0.947) respectively .

Conclusion: In this pediatric cohort with heterozygous familial hypercholesterolemia, baseline cognitive function was predominantly within normal ranges, although a significant proportion had low to below-average performance. A study with a larger number of patients is needed to clarify these associations and have more tools for treating dyslipidemia in children.

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