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Журнал «Здоровье ребенка» Том 17, №8, 2022

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Вплив дієти СHILD-1 на харчовий профіль і дієтичний комплаєнс в українських педіатричних пацієнтів із гетерозиготною сімейною гіперхолестеринемією

Вплив дієти СHILD-1 на харчовий профіль і дієтичний комплаєнс в українських педіатричних пацієнтів із гетерозиготною сімейною гіперхолестеринемією

Авторы: T. Marushko, T. Kurilina, Y.-E. Kulchytska
Shupyk National Healthcare University of Ukraine, Kyiv, Ukraine

Рубрики: Педиатрия/Неонатология

Разделы: Справочник специалиста

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Резюме

Актуальність. Гіперхолестеринемія є фактором ризику атеросклерозу та серцево-судинних захворювань, а також значним чинником смертності від основних несприятливих серцево-судинних подій. Лікувальна дієтотерапія та належний рівень фізичної активності є важливими складовими стратегії профілактики в пацієнтів із сімейною гіперхолестеринемією (СГ). Метою нашого дослідження було визначити вплив дієти CHILD-1 на нутритивний профіль пацієнтів із СГ порівняно зі здоровими однолітками та виявити можливі відхилення від необхідного добового споживання нутрієнтів. Матеріали та методи. Збор даних у 15 пацієнтів із СГ, включених у дослідження, було проведено за допомогою адаптованого опитувальника Food Frequency Questionnaire. Їхній нутритивний статус оцінювали за допомогою аналізаторa Anthro+ WHO. Дані про середньодобове споживання нутрієнтів FETA та можливі кореляції з іншими параметрами були проаналізовані з використанням програмного забезпечення SAS OnDemand for Academics. Результати. Отримані результати свідчать про відсутність повного дієтичного комплаєнсу в усіх вікових групах пацієнтів. Пацієнти з СГ віком 5–9 років споживали більше жирів. Спостерігався значний дефіцит вітаміну D. Діти з СГ віком 10–14 років споживали менше вуглеводів та жирів на добу, але мали дефіцит білка, кальцію, заліза, йоду, цинку та вітаміну D. Пацієнти з СГ віком 15–18 років мали дещо підвищене добове споживання холестерину та дефіцит вітаміну D. Ця вікова група була єдиною, яка перевищувала рекомендований дієтою рівень добового споживання холестерину. Кореляційний аналіз показав, що вік і стать не впливали на вживання нутрієнтів (p > 0.05). Статистично значуща гендерна різниця у вживанні певних нутрієнтів була зафіксована у віці 5–9 і 10–14 років тільки в групі пацієнтів із СГ, причому рівень споживання був вищим у хлопчиків. Висновки. Раціони харчування хворих на СГ та здорових дітей у всіх вікових групах були незбалансованими, містили мало білка та багато нерекомендованих інгредієнтів. Недотримання дієтотерапії у хворих на СГ пов’язане з відсутністю мотивації, недостатньою освітою дітей і батьків та нестачею уваги з боку лікарів. Лікування таких пацієнтів потребує мультидисциплінарної команди із застосуванням сімейно-орієнтованого підходу, спрямованого на розвиток у дитини навичок самоорганізації. Керівництво з ведення СГ повинно включати забезпечення лікувального харчування та поетапний моніторинг фізичного розвитку.

Background. Hypercholesterolemia is a risk factor for atherosclerosis and cardiovascular disease; it is also a significant contributor to mortality from major adverse cardiovascular events. Medical nutrition therapy and proper physical activity level are all important parts of prevention strategy for patients with familial hypercholesterolemia (FH). The aim of our study was to determine the impact of the Cardiovascular Health Integrated Lifestyle Diet (CHILD-1) on the nutritional profile of patients with FH compared to the healthy peers and to identify possible deviations from the required daily nutrient intake. Materials and methods. Fifteen patients with FH included in the study were interviewed using an adapted Food Frequency Questionnaire. Their nutritional status was assessed with Anthro+ WHO software. The average daily intake of FETA nutrients and its possible correlation with other parameters were analyzed using SAS OnDemand for Academics software. Results. The results suggest a lack of complete dietary compliance in all age groups of patients. Patients with FH aged 5–9 years consumed more fat than recommended daily intake level. There was a significant deficit in vitamin D. Children with FH aged 10–14 years consumed less carbohydrates and fats per day, showed the deficiencies in protein, calcium, iron, iodine, zinc, vitamin D. Patients with FH aged 15–18 years had a slightly increased daily intake of cholesterol and vitamin D deficiency. This age group was the only to exceed the recommended daily cholesterol intake level. Age and gender had no effect on nutrient intake (p > 0.05). A statistically significant gender difference in intake of certain nutrients was recorded at ages 5–9 and 10–14 years only in the group of patients with FH, with higher levels in male patients. Conclusions. The diets of patients with FH and healthy children in all age groups were unbalanced, containing little protein and many non-recommended ingredients. Non-adherence to dietary treatment in patients with FH is associated with a lack of motivation, inadequate education of children and parents and a general lack of medical attention. Treatment of these patients requires a multidisciplinary team with a family-oriented approach that is focused on self-management skills development in a child. The FH management guidelines should include provision of medical nutrition therapy and step-by-step monitoring of growth and development.


Ключевые слова

діти; сімейна гіперхолестеринемія; харчовий профіль; дієтичний комплаєнс; CHILD-1

children; familial hypercholesterolemia; nutritional profile; dietary compliance; CHILD-1

doi: http://dx.doi.org/10.22141/2224-0551.17.8.2022.1543

Introduction

Heterozygous familial hypercholesterolemia (FH) is an autosomal dominant disease caused by genetic mutations of the low-density lipoprotein receptor (LDL) receptor, apolipoprotein B or proprotein convertase subtilisin/kexin type 9. The mutations disrupt normal plasma LDL-C clearance, resulting in lifelong increases in LDL-C levels and accelerating the development of atherosclerosis. Medical nutrition therapy is an important component of the familial hypercholesterolemia treatment, especially in pediatric patients who are not yet able to take statins and other lipid-lowering drugs due to age restrictions.
The international community has recognized that nutrition is the key to shaping a child’s health in adulthood [1]. Most initiatives focus on early childhood as a factor that can contribute to or destroy a child’s genetic potential. The newly released WHO/UNICEF Nurturing Care Framework outlines what is needed to help children thrive: good health, adequate nutrition, responsive caregiving, opportunities for early learning, security and safety [2]. The World Health Organization acclaims that people should receive essential nutrition and nutritional support to maintain their future health [2]. When children’s diets fail to provide the nutrients they need for healthy growth, they may require micronutrient supplements or treatment for malnutrition (including obesity).
At the same time, the pyramid of services for the pediatric population distinguishes three levels — universal, targeted and assigned. Children with familial hypercholesterolemia may benefit from a targeted approach to nutritional management that addresses individual risks and provides additional support for medical nutrition therapy, including the Cardiovascular Health Integrated Lifestyle Diet, or CHILD-1.
The aim of our study was to determine the impact of the CHILD-1 on the nutritional profile of pediatric patients with heterozygous familial hypercholesterolemia compared to their healthy peers using self-reported nutritional data and to identify possible deviations from the required daily nutrient intake.

Materials and methods

A retrospective study was conducted of pediatric patients from all regions of Ukraine who were seen in the Department of Cardiology at Kyiv City Children’s Clinical Hospital No. 1.
Inclusion criteria for the study were: a confirmed diagnosis of familial hypercholesterolemia for at least 6 months, age between 5 and 18 years, adherence to prescribed lipid-lowering therapy and an appropriate diet (CHILD-1), signed informed consent by a child and parent(s) or legal guardian(s). Exclusion criteria were withdrawal of informed consent, age less than 5 years, interruption of lipid-lowering therapy > 1 month, presence of a confirmed disease or condition other than FH that causes lipid metabolism disorders (diabetes mellitus, hypothyroidism, nephrotic syndrome, chronic kidney disease, primary cholangitis, obstructive jaundice, obesity, Cushing’s syndrome, pheochromocytoma and etc.); intake of medications that cause lipid metabolism disorders (amiodarone, thiazide diuretics, beta-blockers, glucocorticoids, estrogens, androgens, immunosuppressants, anticancer agents, antipsychotics, HIV-1 protease inhibitors, anticonvulsants, retinoids, growth hormones and others).
One hundred and eighteen children were examined between January and December 2021. Fifteen of them met the inclusion criteria and agreed to participate in the study, with informed consent given by both the children and parent(s) or legal guardian(s). Three patients withdrew their informed consent.
Children with familial hypercholesterolemia (n = 15) were included in the study group (hereinafter referred to as patients). The Dutch Lipid Clinic Network Score was used to establish the diagnosis of familial hypercholesterolemia [3]. The surveyed children were mostly in the age range of 5–17 years (55 % boys and 45 % girls). The control group (n = 21) consisted of healthy peers (hereinafter referred to as controls). Subsequently, the children were stratified by age. The following age groups were identified according to the WHO guidelines: 5 to 9, 10 to 14, and 15 to 18 years. The groups were representative of age and sex.
The recommended nutrient intakes according to CHILD-1 were < 300 mg cholesterol per day, < 30 % fat, 50 % carbohydrate and 20 % protein of the age-appropriate daily caloric requirement [4]. Dietary recommendations for healthy peers in the control group were regulated by the current Order of the Ministry of Health of Ukraine “Norms of physiological needs of the Ukrainian population in basic nutrients and energy” [5].
Children with familial hypercholesterolemia included in the study were interviewed using an adapted Food Frequency Questionnaire (FFQ) EPIC Tool for Analysis (FETA, University of Cambridge) [6]. The interview was conducted with parents present as it was requested by all patients.
The FЕТА is designed to measure the participant’s usual food intake during the previous year. The main part of the questionnaire contains a list of the 130 most frequently and infrequently consumed food items. For each item on the list, participants are asked to indicate their usual frequency of consumption by selecting one of nine frequency categories. Categories range from “never” or “less than once a month” to “6+ times a day”. Portions are reported in units or usual portions (e.g., one apple, one slice of bread) or in household measures (e.g., glass, cup, spoon). Each item in the questionnaire was assigned an average portion size (this portion size is the same for all participants, regardless of their gender or age). The input data were processed in the FETA software.
The following data were obtained regarding the average daily intake of nutrients, such as: daily energy intake (kcal), cholesterol (g), total proteins (g), total fats (g), total carbohydrates (g), vitamins (A (mcg), B1 (mg), B2 (mg), B6 (mg), B12 (mcg), C (mg), D (mcg), E (mg)) and micronutrients (calcium (mg), copper (mg), iron (mg), iodine (mcg), magnesium (mg), phosphorus (mg), selenium (mcg), zinc (mg)). A deviation of 25 % or more from the recommended level was considered a deficiency or a surplus, respectively.
The auxological parameters (weight, height, body mass index (BMI)) were obtained by routine anthropometry. Nutritional status was assessed using the Anthro+ WHO analyser (weight-for-age, stature-for-age and BMI-for-age in ‰ and by z-score) and the corresponding WHO growth charts for each patient. The WHO classification criteria were used to determine the nutritional status [7]. The cut-off points of z > 2  identify children with high weight-for-stature (overweight) [8].
The study was conducted in accordance with the Declaration of Helsinki, the Council of Europe Convention on Human Rights and Biomedicine, and Ukrainian laws governing research on human subjects.
Statistical analysis
Average daily FETA nutrient intake data were described using mean (M) and standard deviation (SD). To evaluate possible correlations between variables of age, sex, patient/control group affiliation, weight, BMI, average daily energy intake (kcal), and nutrients (cholesterol (g), total proteins (g), total fats (g), total carbohydrates (g), vitamins and micronutrients), SAS OnDemand for Academics (SAS Institute Inc, North Carolina, USA) software was used. Data were assumed to be normally distributed (verified analytically by Shapiro-Wilk test and graphically by Q-Q plot). Statistical significance was set at p ≤ 0.05.

Results

Auxological parameters revealed the following characteristics in children with FH. The average weight-for-age and stature-for-age of children with familial hypercholesterolemia were within the normal range of the WHO growth charts. At the same time, BMI specified high body weight in 20 % and excessive body weight in 10 % of children with familial hypercholesterolemia of all ages. There were no obese children (weight-for-age z-score greater than +3). One child in the 5–9 years age group with familial hypercholesterolemia was found to have excessive body weight. On the other hand, one patient in the same group had a reduced BMI (z-score less than 1). In this age group, children mostly depend on parental dietary suggestions, and this variation may indicate low family compliance with dietary recommendations or inadequate parental education. In terms of BMI, children with familial hypercholesterolemia are somewhat stable at the age of 10–14 years, i.e., no children with excessive body weight or obesity were observed, although those with increased and decreased body weight were identified among the patients. Patients aged 15–18 years are distributed in the same way as the 10–14 years age group.
Beyond age stratification, a third of patients had a BMI z-score below 1 (i.e., underweight), 20 % were slightly overweight (z-score greater than +1) and a third had signs of being overweight (Fig. 1).
Analysis of anthropometric parameters by sex revealed that boys with familial hypercholesterolemia are heavier than girls, and 54.6 % of them have elevated BMI values, while the body weight and stature-for-age individually were within the normal reference levels, but differ by more than 2 corridors (i.e., development cannot be called harmonious). At the time of adolescence (15–18 years), 60 % boys had increased body weight or were overweight, while girls of this age did not have BMI that exceeds the maximum threshold.
There was no statistically significant difference in weight and BMI in all age groups and sexes between patients and controls (p > 0.05).
According to the FFQ, patients with familial hypercholesterolemia, regardless of sex and age, consumed chicken meat, wholemeal bread and crispbread, oatmeal, hard cheese and avocado more frequently (5–6 times per week). Sugar intake in children with FH was minimal (1–2 times a month). Healthy peers in the control group consumed white bread, chocolate biscuits, ketchup, sour cream, and sweet carbonated beverages more frequently. Foods and meals such as vegetable soup, chocolate, tea and various fruits were consumed on average equally often by both patients and controls (5–6 times a week).
The mean daily intake of nutrients over the last year (Table 1) in patients with FH aged 5–9 years was 1946.54 kcal (95% confidence interval (CI) 679.35–2701.56; SD 1104.12), 1375.88 kcal for those aged 10–14 years (95% CI 1009.83–1912.13; SD 474.62), 2254.87 kcal for those aged 15–18 years (95% CI 1590.78–2851.80; SD 536.02). On average per day, children in the control group aged 5–9 years consumed 1565.53 kcal (95% CI 1022.9–2428.2; SD 982.2), patients aged 10–14 years, 1763.37 kcal (95% CI 966.71–2356.96; SD 587.43), and those aged 15–18 years consumed 1727.21 kcal (95% CI 809.90–4129.52; SD 1379.92), respectively.
For children with FH aged 5–9 years, the daily caloric intake exceeded the CHILD-1 requirements by 11.23 %, while the control group had a reduced intake by 10.54 % despite the Ministry of Health of Ukraine requirements. At the age of 10–14 years, children with FH had a reduced intake by 41.45 %, the control group of the same age also had a reduced intake by 24.96 %. The 15–18-year patients had a reduced caloric intake by 11.77 %, while the control group of the same age had it reduced by 32.27 %.
A statistically significant gender difference in caloric intake was also observed in the group of FH patients aged 15–18 years. Boys consumed more calories (M = 2658.4, SD = 273.5) than girls (M = 1871.6, SD = 262.9) (t = –3.23, p = 0.04, 95% CI [–1561.0; –12.68]).
Analysis of protein intake in children with FH revealed that on average, patients aged 5–9 years consumed 88.05 grams per day (95% CI 32.08–135.72; SD 52.31), children aged 10–14 years, 55.37 grams (95% CI 41.50–81.86; SD 22.94), those aged 15–18 years consumed 120.40 grams (95% CI 80.57–139.56; SD 27.18). The controls aged 5–9 years consumed 69.18 grams of protein (95% CI 53.14–86.02; SD 14.98), those aged 10–14 years, 75.24 grams (95% CI 28.99–120.65; SD 37.42) and those aged 15–18 years consumed 75.16 grams (95% CI 30.52–189.86; SD 68.81).
Thus, it was found that children with FH aged 5–9 years exceeded the required protein level by 0.63 %, while the control group had the reduced protein intake by 72.33 %. Children with FH aged 10–14 years had a reduced protein intake by 52.88 %, and the control group of the same age had it reduced by 76.99 %. The patients aged 15–18 years had a reduced protein intake by 5.57 %, while the control group had it reduced by 14.59 %.
The average daily intake of fats in patients was 68.15 grams (95% CI 23.47–90.52; SD 38.69) in the 5–9 years group, 44.24 grams (95% CI 29.71–67.60; SD 20.42) in the 10–14 years group and 86.16 grams (95% CI 66.42–104.68; SD 15.93) in the 15–18 years group. Children in the control group aged 5–9 years consumed 53.76 grams of fat (95% CI 42.12–105.86; SD 24.53), those aged 10–14 years, 65.99 grams (95% CI 23.25–96.84; SD 32.07) and those aged 15–18 years consumed 69.76 grams (95% CI 25.04–188.83; SD 69.83).
The findings have revealed that children with FH aged 5–9 years exceeded the required fat level by 16.84 %, while the control group had the reduced fat intake by 12.34 %. Children with FH aged 10–14 years had a reduced protein intake by 43.52 %, and the control group of the same age had it reduced by 17.51 %. The patients aged 15–18 years exceeded requirements of fat intake by 1.36 %, while the control group had it reduced by 14.59 %.
Carbohydrate intake analysis showed that patients with FH aged 5–9 years consumed 262.11 grams (95% CI 90.64–401.29; SD 157.82), children aged 10–14 years, 202.29 grams (95% CI 139.64–261.16; SD 60.84), those aged 15–18 years consumed 266.50 grams (95% CI 179.14–376.07; SD 87.42). The average carbohydrate intake for the control group was 215.39 grams (95% CI 188.73–376.34; SD 67.29) for those aged 5–9 years, 232.02 grams (95% CI 171.46–301.41; SD 54.12) for those aged 10–14 and for those aged 15–18 years, 213.98 grams (95% CI 123.98–446.75; SD 132.27).
Children with FH aged 5–9 years exceeded the required carbohydrate level by 19.82 %, while the control group by 240.11 %. Children with FH aged 10–14 years had a reduced carbohydrate intake by 61.15 %, and the control group of the same age by 40.51 %. The patients aged 15–18 years had a reduced carbohydrate intake by 16.37 %, while the control group had it reduced by 39.64 %.
It is worth noting that there is a statistically significant difference in nutrient intake between the sexes in the patient group, which was not observed in the control group. Thus, in the 10–14 years age group, boys with FH consumed on average more fat (M = 67.01, SD = 0.83) than girls (M = 32.57, SD = 4.03) (t = –11.83, p < 0.01, 95% CI [–46.97; –21.91]). Boys with FH also consumed more protein (M = 81.21, SD = 0.91) than girls (M = 42.13, SD = 0.90) (t = –43.15, p < 0.01, 95% CI [–42.97; –35.18]).
Regarding daily cholesterol intake (Fig. 2), FH patients aged 5–9 years consumed 238.83 mg (95% CI 91.50–398.03; SD 153.60), children aged 10–14 years, 145.21 mg (95% CI 77.08–232.97; SD 79.77), and those aged 15–18 years consumed 389.89 mg (95% CI 336.77–446.35; SD 45.44). In the control group, the daily cholesterol intake was 266.71 mg for those aged 5–9 years, 236.62 mg for those aged 10–14 years (95% CI 91.93–394.86; SD 135.17), and 239.54 mg for those aged 15–18 years (95% CI 56.76–764.14; SD 299.36).
FH patients aged 5–9 years had a required cholesterol level by 20.39 %, while the control group had it reduced by 11.10 %. Children with FH aged 10–14 years had a reduced cholesterol intake by 51.60 %, and the control group of the same age had it reduced by 21.13 %. The patients aged 15–18 years exceeded the requirements of cholesterol intake by 29.96 %, while the control group had it reduced by 20.15 %. The CHILD-1 recommended cholesterol level of less than 300 mg/day was exceeded by FH patients only in the 15–18 years age group. No statistically significant differences in cholesterol intake were observed between patients and controls.
An analysis of daily average micronutrient and vitamin intake over the last year revealed the following in children with FH and controls (Fig. 3, 4, Table 2).
Female FH patients aged 10–14 years consumed less calcium with food (M = 439.9, SD = 85.42) than males (M = 867.3, SD = 89.21) (t = –4.85, p = 0.03, 95% CI [–803.3; –51.64]). Boys consumed more iodine (M = 128.4, SD = 4.84) than girls (M = 55.43, SD = 14.49) (t = –6.75, p = 0.02, 95% CI [–119.5; –26.42]). Male patients consumed more phosphorus (M = 1359.9, SD = 104.3) than females (M = 750.5, SD = 5.79) (t = –8.25, p = 0.01, 95% CI [–927.3; –291.5]).
Vitamin B2 intake was statistically significantly different in the 10–14-year-old patient group. Boys with FH consumed more vitamin B2 (M = 1.60, SD = 0.03) than girls (M = 0.85, SD = 0.13) (t = –7.74, p = 0.01, 95% CI [–1.17; –0.33]).
Male patients in the 15–18 years age group consumed more iodine (M = 154.1, SD = 8.15) than females (M = 129.3, SD = 3.77) (t = –4.84, p = 0.01, 95% CI [–41.17; –8.49]). Thus, boys with FH consumed more phosphorus (M = 2086.4, SD = 9.41) than girls (M = 1476.2, SD = 214.4) (t = –3.82, p = 0.03, 95% CI [–1119.1; –101.3]).
Vitamin B2 intake was also statistically significantly different in patients aged 15–18 years. Boys with FH consumed more vitamin B2 (M = 2.05, SD = 0.13) than girls (M = 1.67, SD = 0.11) (t = –3.45, p = 0.04, 95% CI [–0.73; –0.02]).
A partial Pearson correlation was performed to test whether there was an association between the variables age, sex, patient/control group affiliation, weight, BMI, average daily energy intake (kcal) and nutrients (cholesterol (g), protein (g), fat (g), carbohydrate (g), and other variables) in the patient and control groups. Partial correlation results showed that when controlling for the variables age, sex and weight, there was no such relationship in the patient group and in the control group (p > 0.05). Thus, age and sex had no effect on nutrient intake.

Discussion

A condition such as familial hypercholesterolemia requires mandatory medical nutrition therapy in addition to targeted lipid-lowering treatment for effective secondary prevention of cardiovascular diseases.
Our research findings suggest that not only FH patients but also their healthy peers in the control group need medical intervention to change eating habits. From the perspective of Williams (2022) [9], a family-oriented approach is needed for pediatric patients with dyslipidemia, among others. Modi [10] mentioned family coping style, which the child perceives during the process of growing up, as one of the modifiable factors in the structure of children’s self-management. Therefore, to implement a successful medical nutrition therapy, a physician will have to change the eating habits of the whole family, which, given our specific circumstances, will not have one FH patient, but his affected sibling and/or parent. In our view, every emphasis should be placed on developing the self-management skills of the pediatric patient from a very early age, with comprehensive information and psychological support from health care providers and the family.
In addition to the family coping style, many other external factors influence a child’s adherence. A concept for decision making by parents (or carers) as formulated by Hughes (2020) [11], according to which a balance between the needs and preferences of the child can be achieved by taking into account influences such as the socioeconomic status of the family, the availability and knowledge of provision in the market, the ability to judge its quality (including through proxies and after directed learning), etc.
Based on our extensive empirical evidence, the multidisciplinary team approach to FH treatment is made easier when a dietitian is involved.
The available literature does not provide conclusive data on the macro-, micronutrient, and vitamin deficiencies and surpluses in children with familial hypercholesterolemia.
Despite counselling support from a dietitian, our patients failed to achieve complete dietary compliance, with controls in the 5–9 and 10–14 years age groups having a more irrational diet, as in the Molven (2013) study [12]. It implies that the eating habits of healthy children may also be compromised for various reasons, and that the youngest age groups need if not advice from a dietitian, then at least the attention of a general practitioner or pediatrician to their diet. In preschoolers, a healthy eating lifestyle is essential in preparation for the transition to an adult diet. Often trying new foods and freely choosing favorite foods in small portions leads to irrational component composition. The nutritional habits of school-age children are strongly influenced by environmental factors, so it is important to shape food behaviour in advance because of the risks involved and to counterbalance negative peer influences by increasing dietary compliance.
A lack of motivation is cited as one of the reasons for low compliance in 86 % of pediatric and adolescent patients with FH in a study by Langslet (2021) [13]. According to Faehn [14], it was the genetic confirmation of familial hypercholesterolemia that motivated patients to take better care of their diet. At the same time, it should be noted that dietary disturbances in healthy controls are a global problem and require increased medical interventions to promote healthy lifestyles, in particular a rational diet and adequate levels of physical activity.
An unbalanced diet, foods high in trans fats and large quantities of high-processed food can lead to premature puberty, a process we have seen for decades according to Soliman [15].
Energy-dense, nutrient-poor food consumption is not only a problem in children, but also in adults [16]. All age groups of controls consumed sugar, sweetened carbonated drinks, white bread, chocolate biscuits, sour cream and tea equally often (5–6 times a week). The consumption of energy-dense, nutrient-poor foods accounts for a significant proportion of the modern children diet, which may be a risk factor implementing a genetic predisposition to the development of various chronic pathologies in adults. Overall, the results of the nutrition profile analysis of healthy peers suggest that there is a lack of nutrition education culture in families.
An analysis of the Health Behaviour in School-aged Children study, the Ukrainian Dietitian Association study, IPSOS (2019) and STEPS in Ukraine (2019–2020) suggests a number of conclusions:
— child nutrition standards in Ukraine have not been revised for more than 15 years;
— the nutritional profile structure of children of all ages does not correspond to current trends in healthy eating;
— the changes that have taken place in legislation regarding the physiological needs of the population for basic nutrients and energy concern only the categories of school children provided with free meals;
— the link between nutrition and the occurrence of non-communicable diseases, such as obesity, diabetes, cardiovascular disease and certain cancers, has been proven.

Conclusion

The diets of patients with familial hypercholesterolemia and healthy children in all age groups were unbalanced, containing little protein and many non-recommended ingredients. Healthy children in the control group were equally at increased risk of being overweight or obese.
Patients with familial hypercholesterolemia often fail to comply with dietary treatment recommendations. Non-adherence to recommended dietary treatment in those with familial hypercholesterolemia is associated with a lack of motivation, inadequate education of children and/or parents and a general lack of medical attention. Treatment of these patients requires a multidisciplinary team that includes a psychologist and dietitian. Although a family-oriented approach is necessary, as it involves parents and siblings with FH, it must also be balanced with the development of self-management skills in pediatric patients and encouraged in every possible way by the health care provider.
The guideline for the management of familial hypercholesterolemia should include provision of medical nutrition therapy, careful step-by-step monitoring of growth and development, which we consider to be the two essential components necessary for the successful treatment of these patients.
 
Received 01.10.2022
Revised 09.10.2022
Accepted 18.10.2022

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