Nutritional Treatment in a Cohort of Pediatric Patients with Familial Hypercholesterolaemia: Effect on Lipid Profile

Capra, Maria Elena; Pederiva, Cristina; Viggiano, Claudia; Fabrizi, Enrico; Banderali, Giuseppe; Biasucci, Giacomo

doi:10.3390/nu14142817

Cited by 4 publications

(2 citation statements)

References 29 publications

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“…It is important to keep in mind that dietary recommendations for children with FH do not involve a strict and mandatory diet; they are based on nutritional recommendations that all children and adolescents should follow to lead a healthy lifestyle. The goal of the dietary intervention is to promote healthy lipid-controlled diets, including for members of the entire family, and thus to increase the chances of continued adherence to the diet into adulthood [ 24 , 25 ].…”

Section: Discussionmentioning

confidence: 99%

Diagnosis of Familial Hypercholesterolemia in Children and Young Adults

Timoshchenko,

Ivanoshchuk,

Semaev

et al. 2023

IJMS

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The early detection and treatment of familial hypercholesterolemia (FH) in childhood and adolescence are critical for increasing life expectancy. The purpose of our study was to investigate blood lipid parameters, features of physical signs of cholesterol accumulation, and a personal and family history of premature cardiovascular diseases in children and young adults when FH is diagnosed. The analysis included patients under 18 years of age (n = 17) and young adults (18–44 years of age; n = 43) who received a diagnosis of FH according to clinical criteria. Targeted high-throughput sequencing was performed using a custom panel of 43 genes. A family history of cardiovascular diseases was more often noted in the group under 18 years of age than in young adults (p < 0.001). Among young adults, there was a high prevalence of typical signs of the disease such as tendon xanthomas and the early development of arterial atherosclerosis (p < 0.001). By molecular genetic testing, “pathogenic” and “probably pathogenic” variants were identified in the genes of 73.3% of patients under 18 years of age and 51.4% of patients 18–44 years of age. Thus, blood lipid screening tests combined with an accurate assessment of the family history is a highly relevant and inexpensive option for diagnosing FH in childhood. Molecular genetic testing allows us to make an accurate diagnosis and to improve adherence to treatment.

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Section: Discussionmentioning

confidence: 99%

Diagnosis of Familial Hypercholesterolemia in Children and Young Adults

Timoshchenko,

Ivanoshchuk,

Semaev

et al. 2023

IJMS

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“…We are currently working on this aspect so as to elaborate nutritional indications that are more specific and targeted for paediatric patients with FH. According to data available so far, nutritional lipid-lowering treatment is safe [10][11][12][13], and if well established, it remains a lifestyle model for the patient to follow for their lifetime.…”

Section: Nutritional Interventionmentioning

confidence: 99%

Clinical Approach in the Management of Paediatric Patients with Familial Hypercholesterolemia: A National Survey Conducted by the LIPIGEN Paediatric Group

et al. 2023

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Detection and treatment of patients with familial hypercholesterolemia (FH) starting from childhood is fundamental to reduce morbidity and mortality. The activity of National realities such as the LIPIGEN (LIpid transPort disorders Italian GEnetic Network) Paediatric Group, founded in 2018, is a milestone in this context. The aim of this exploratory survey, conducted in October 2021 among Italian lipid clinics included in the LIPIGEN Paediatric Group, was to investigate the current clinical approach in the management and treatment of paediatric patients with suspected FH. A digital questionnaire composed of 20 questions investigating nutritional treatment and nutraceutical and pharmacological therapy for children and adolescents with FH was proposed to the principal investigators of 30 LIPIGEN centres. Twenty-four centres responded to the section referring to children aged < 10 years and 30 to that referring to adolescents. Overall, 66.7% of children and 73.3% of adolescents were given lipid-lowering nutritional treatment as the first intervention level for at least 3–4 months (29.2% and 23.3%) or 6–12 months (58.3% and 53.3%). Nutraceuticals were considered in 41.7% (regarding children) and 50.0% (regarding adolescents) of the centres as a supplementary approach to diet. Lipid-lowering drug therapy initiation was mainly recommended (91.7% and 80.0%). In 83.3% of children and 96.7% of adolescents, statins were the most frequently prescribed drug. We highlighted several differences in the treatment of paediatric patients with suspected FH among Italian centres; however, the overall approach is in line with the European Atherosclerosis Society (EAS) recommendations for FH children and adolescents. We consider this survey as a starting point to reinforce collaboration between LIPIGEN centres and to elaborate in the near future a consensus document on the management of paediatric patients with suspected FH so as to improve and uniform detection, management, and treatment of these patients in our country.

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Dietary intervention for children and adolescents with familial hypercholesterolaemia

et al. 2023

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Familial hypercholesterolaemia (FH) is a frequent genetic disorder characterised by high plasma levels of total and LDL-cholesterol and premature atherosclerosis. If left untreated, affected subjects have a high risk of cardiovascular disease, as they are exposed to very high levels of LDL-cholesterol from birth. Healthy dietary habits and lifestyle are the first treatment option and, if started from childhood, represent a milestone in the prevention of atherosclerotic disease, both as a starting point and in combination with drug therapy. In this work, based on the main consensus documents available so far, we have evaluated the most up-to-date indications of the dietetic-nutritional intervention for the treatment of FH, delving into the peculiar aspects of the diet of the child/adolescent affected by FH. After an analysis of the macro- and micronutrients and the most common dietary patterns currently recommended, we highlighted some practical aspects, some frequent errors and some risks we could fall into when dealing with paediatric nutritional treatment. In conclusion, the dietary intervention for the child/adolescent with FH is a complex task, that should be individualised and tailored taking into account, first of all, the nutritional adequacy for growth and development, but also the multiple aspects linked to the child/adolescent's age, tastes and preferences, the family they belong to, the socio-economic context and the Country they live in.

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Nutritional Treatment in a Cohort of Pediatric Patients with Familial Hypercholesterolaemia: Effect on Lipid Profile

Cited by 4 publications

References 29 publications

Diagnosis of Familial Hypercholesterolemia in Children and Young Adults

Diagnosis of Familial Hypercholesterolemia in Children and Young Adults

Clinical Approach in the Management of Paediatric Patients with Familial Hypercholesterolemia: A National Survey Conducted by the LIPIGEN Paediatric Group

Dietary intervention for children and adolescents with familial hypercholesterolaemia

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