perinatologyРоссийский вестник перинатологии и педиатрииRossiyskiy Vestnik Perinatologii i Pediatrii (Russian Bulletin of Perinatology and Pediatrics)1027-40652500-2228Ltd. “The National Academy of Pediatric Science and Innovation”10.21508/1027-4065-2020-65-4-27-40perinatology-1198Research ArticleОБЗОРЫ ЛИТЕРАТУРЫLITERATURE REVIEWSСовременная стратегия диагностики и лечения семейной гетерозиготной гиперхолестеринемии у детейModern strategy of diagnosis and treatment of children with heterozygous familial hypercholesterolemiahttps://orcid.org/0000-0002-5273-6859ЛеонтьеваИ. В.LeontyevaI. V.

Леонтьева Ирина Викторовна — доктор медицинских наук, проф., главный научный сотрудник отдела детской кардиологии и аритмологии.

125412 Москва, ул. Талдомская, 2

ОСП Научно-исследовательский клинический институт педиатрии им. академика Ю.Е. Вельтищева ФГАОУ ВО РНИМУ им. Н.И. Пирогова Минздрава РоссииРоссияVeltischev Research and Clinical Institute for Pediatrics of the Pirogov Russian National Research Medical UniversityRussian Federation2020230920206542740Copyright © Ltd. “The National Academy of Pediatric Science and Innovation”, 20202020Ltd. “The National Academy of Pediatric Science and Innovation”Ltd. “The National Academy of Pediatric Science and Innovation”https://www.ped-perinatology.ru/jour/about/submissions#copyrightNoticehttps://www.ped-perinatology.ru/jour/article/view/1198

Семейная гетерозиготная гиперхолестеринемия — наследуемое по аутосомно-доминантному типу заболевание, характеризующееся высоким уровнем в крови холестерина липопротеинов низкой плотности, что приводит к началу атеросклеротического поражения сосудов уже в детском возрасте и развитию инфаркта миокарда на втором—третьем десятилетии жизни. Семейная гиперхолестеринемия остается малоизвестным заболеванием в клинической практике педиатра. Это ведет к запоздалой диагностике и позднему началу терапии. Заболевание длительно протекает бессимптомно, в связи с чем его выявление составляет менее 1% случаев. Освещены генетические аспекты заболевания. Изложены критерии диагностики семейной гетерозиготной гиперхолестеринемии у детей, представлены различные варианты скрининга. Рассмотрены возможности ранней диагностики сосудистого поражения атеросклеротического генеза. Освещена стратегия ведения пациентов с семейной гетерозиготной гиперхолестеринемией, обсуждаются возможности немедикаментозной и медикаментозной терапии. Изложены показания к назначению, эффективность и безопасность применения статинов как основного класса препаратов для лечения заболевания.

Familial heterozygous hypercholesterolemia Is an autosomal dominant disease characterized by high levels of low-density lipoprotein cholesterol, leading to an atherosclerotic vascular damage in children and myocardial infarction in 20 -30 years-old individuals. Familial hypercholesterolemia remains a little-known disease in the pediatric clinical practice, and it is characterized by late diagnosis and start of therapy. The disease is characterized by asymptomatic course for a long time, and therefore it is diagnosed in less than 1% of cases. The authors present genetic aspects of the disease. They describe the diagnostic criteria of familial heterozygous hypercholesterolemia in children and various screening options. They consider the possibilities of early diagnosis of vascular lesions of atherosclerotic origin. The authors describe the strategy of managing patients with familial heterozygous hypercholesterolemia and various options of non-drug and drug therapy. They provide indications, effectiveness and safety of statins as the main class of drugs for the treatment of the disease.

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