Nutritional status of children with familial hypercholesterolemia

Aim. To study the features of the nutritional status, including physical growth, body composition, energy value of the diet and the amount of cholesterol consumed with food, in children with familial hypercholesterolemia (FH).

Patients and methods. The study included 39 children (19 girls, 20 boys) with familial hypercholesterolemia, both genetically confirmed (n = 11) and established on the basis of Simon Broome criteria (n = 28), aged 9.9 [6.0; 12.9] years. All children were assessed for physical growth based on the Z-score BMI, Z-score height, Z-score body weight/height calculated using the programs Anthro and Anthro plus; analysis of actual nutrition using a standard application program; study of body composition using the InBody 770 bioimpedance analyzer, the basal metabolic rate measured by indirect calorimetry.

Results. 55 % of children with FH had harmonious physical growth, 27 % had a body weight deficit, 18 % were overweight or obese. 53 % of patients consumed more than 200 mg of cholesterol per day, while half of them consumed more than 300 mg of cholesterol per day. The range of excess consumption of cholesterol in comparison to the recommended physiological needs for patints with FH ranged from 24 to 67 %. The fat mass according to the body composition in 47 % of patients with FH is within the normal range, in 39 % it is reduced by 18-74 %, in 14 % it is increased by 14-197 %. The median fat percentage was 17.8 [12.7; 22.4]%. According to indirect calorimetry, it was found that the level of resting energy needs corresponded to age needs in 21 % patients, in 8 % it was decreased of 2-26 %, in 2/3 of children its increase was recorded by 3-69 %. The rate of fat oxidation, on the contrary, was increased in 71 % of children by 3-86 % and reduced only in 6 %.

Conclusion. The physical growth of children with FH was mainly average, harmonious, while a third of the children had a mass deficit. The level of the main indicators of lipid metabolism did not depend on the physical growth of patients. This pathology in children with normal body weight or with a body weight deficit often remains undiagnosed.

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