ДИФФЕРЕНЦИАЛЬНАЯ ДИАГНОСТИКА ГИПЕРТРОФИЧЕСКОЙ КАРДИОМИОПАТИИ


https://doi.org/10.21508/1027-4065-2017-62-3-20-31

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Аннотация

Гипертрофическая кардиомиопатия – наиболее частая форма кардиомиопатий, встречающаяся в детском возрасте, возникающая при мутации генов, кодирующих белки саркомерного и несаркомерного комплексов. В основу диагностики заболевания положены данные эхокардиографии, выявляющие структурные изменения в сердечной мышце по типу гипертрофии, при этом генез этих изменений остается невыясненным. Причины гипертрофической кардиомиопатии в детском возрасте разнообразны. Большое значение имеет ранняя диагностика метаболических форм гипертрофической кардиомиопатии, так, в ряде случаев возможен регресс гипертрофии на фоне ферментозамещающей или иной медикаментозной терапии. В статье представлены клинические (кардиальные и экстракардиальные симптомы) и лабораторные маркеры гипертрофической кардиомиопатии при мутациях генов белков саркомерного комплекса, врожденных нарушениях обмена веществ (гликогенозы, лизосомальная патология, нарушения обмена жирных кислот, митохондриальные заболевания), генетических синдромах (Нунан, LEOPARD, Костелло, сердечно-лице-кожный), нервно-мышечных заболеваниях. Приводятся критерии дифференциальной диагностики генетических форм гипертрофической кардиомиопатии и гипертрофии миокарда у спортсменов. 


Об авторе

И. В. Леонтьева
ОСП «Научно-исследовательский клинический институт педиатрии им. Ю.Е. Вельтищева» ФГБОУ ВО РНИМУ им. Н.И. Пирогова
Россия

Адрес для корреспонденции: Леонтьева Ирина Викторовна – доктор медицинских наук, профессор, гл. научный сотрудник отдела детской кардиологии и аритмологии 

125412 Москва, ул. Талдомская, д.2



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Дополнительные файлы

Для цитирования: Леонтьева И.В. ДИФФЕРЕНЦИАЛЬНАЯ ДИАГНОСТИКА ГИПЕРТРОФИЧЕСКОЙ КАРДИОМИОПАТИИ. Российский вестник перинатологии и педиатрии. 2017;62(3):20-31. https://doi.org/10.21508/1027-4065-2017-62-3-20-31

For citation: Leontyeva I.V. DIFFERENTIAL DIAGNOSIS OF HYPERTROPHIC CARDIOMYOPATHY. Rossiyskiy Vestnik Perinatologii i Pediatrii (Russian Bulletin of Perinatology and Pediatrics). 2017;62(3):20-31. (In Russ.) https://doi.org/10.21508/1027-4065-2017-62-3-20-31

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