medalphabetМедицинский алфавитMedical alphabet2078-56312949-2807ООО «Альфмед»10.33667/2078-5631-2024-13-74-80medalphabet-3727Research ArticleСтатьиЭффективность и безопасность применения ингибиторов PCSK9 у детей с семейной гиперхолестеринемиейEffectiveness and safety of PCSK9 inhibitors in children with family hypercholesterolemiahttps://orcid.org/0000-0002-1250-8798ХачатурянР. А.KhachaturyanR. A.

Хидирова Людмила Даудовна - д. м. н., проф. кафедры фармакологии, клинической фармакологии и доказательной медицины.

Новосибирск

Khidirova Lyudmila D. - DM Sci (habil.), professor of the Dept of Pharmacology, Clinical Pharmacology and Evidence-Based Medicine.

Novosibirsk

h_ludmila73@mail.ruhttps://orcid.org/0009-0002-1432-0550ХидироваЛ. Д.KhidirovaL. D.

Хачатурян Руслан Арсенович - студент 6-го курса педиатрического факультета.

Новосибирск

Khachaturyan Ruslan A. - 6th year student of the pediatric faculty.

Novosibirsk

dok955@mail.ruhttps://orcid.org/0009-0006-4496-0435КаравозоваА. Е.KaravozovaA. E.

Каравозова Анастасия Евгеньевна - студентка 6-го курса педиатрического факультета.

Новосибирск

Karavozova Anastasia E. - 6th year student of the pediatric faculty.

Novosibirsk

nastya_vae100@mail.ruФГБОУ ВО «Новосибирский государственный медицинский университет» Минздрава РоссииРоссияNovosibirsk State Medical UniversityRussian Federation202414052024013«Современная поликлиника» (1)7480Copyright © Хачатурян Р.А., Хидирова Л.Д., Каравозова А.Е., 20242024Хачатурян Р.А., Хидирова Л.Д., Каравозова А.Е.Khachaturyan R.A., Khidirova L.D., Karavozova A.E.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.med-alphabet.com/jour/article/view/3727

Семейная гиперхолестеринемия (СГХС) – моногенное заболевание с преимущественно аутосомно-доминантным типом наследования, сопровождающееся значительным повышением уровня холестерина липопротеидов низкой плотности в крови и, как следствие, преждевременным развитием и прогрессирующим течением атеросклероза, как правило, в молодом возрасте. Одной из частых причин заболевания является мутация в гене PCSK9. Пропротеин конвертаза субтилизин/кексин типа 9 (PCSK9) – основное звено в регуляции обмена липидов крови за счет непосредственного участия в деградации рецепторов ЛПНП. В настоящее время в качестве ингибиторов PCSK9 у детей старше 12 лет в РФ применяется только эволокумаб, который доказал безопасность и устойчивое снижение уровня холестерина ЛПНП у педиатрических пациентов с ГеСГ. Другой представитель данной фармакологической группы – алирокумаб, в настоящее время показан только лицам старше 18 лет в связи с отсутствием доказательной базы у детей.

Familial hypercholesterolemia (CGHS) is a monogenic disease with a predominantly autosomal dominant type of inheritance, accompanied by a significant increase in the level of low-density lipoprotein cholesterol in the blood, and as a result, premature development and progressive course of atherosclerosis, usually at a young age. One of the common causes of the disease is a mutation in the PCSK9 gene. Proprotein convertase subtilisin/kexin type 9 (PCSK9) is the main link in the regulation of blood lipid metabolism due to its direct participation in the degradation of LDL receptors. Currently, only evolocumab is used as PCSK9 inhibitors in children over 12 years of age in the Russian Federation, which has proven the safety and steady reduction of LDL cholesterol in pediatric patients with HES. Another representative of this pharmacological group is alirocumab, which is currently indicated only for people over the age of 18, due to the lack of evidence in children.

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The authors declare that there are no conflicts of interest present.