ksmaКубанский научный медицинский вестникKuban Scientific Medical Bulletin1608-62282541-9544Kuban State Medical University10.25207/1608-6228-2018-25-6-192-200ksma-1497Research ArticleОБЗОРЫREVIEWЛЕГОЧНАЯ ГИПЕРТЕНЗИЯ В ПРАКТИКЕ ПЕДИАТРАPULMONARY HYPERTENSION IN PEDIATRIC PRACTICEТрильВ. Е.TrilV. E.

ул. Седина, д. 4, Краснодар, Россия, 350063.

4, Sedina str., Krasnodar, Russia, 350063.

v.tril@mail.ruБурлуцкаяА. В.BurlutskyA. V.

ул. Седина, д. 4, Краснодар, Россия, 350063.

4, Sedina str., Krasnodar, Russia, 350063.

Федеральное государственное бюджетное образовательное учреждение высшего образования «Кубанский государственный медицинский университет» Министерства здравоохранения Российской Федерации.РоссияFederal State Budget Educational Institution of Higher Education "Kuban State Medical University" of the Ministry of Healthcare of the Russian Federation.Russian Federation201821122018256192200Copyright © Триль В.Е., Бурлуцкая А.В., 20182018Триль В.Е., Бурлуцкая А.В.Tril V.E., Burlutsky A.V.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://ksma.elpub.ru/jour/article/view/1497Цель

Цель. Провести обзор российской и зарубежной литературы последних лет о современных методах диагностики итактике ведения детей с легочной гипертензией.

Материалы и методы

Материалы и методы. Проведен анализ российской и зарубежной литературы в базах РИНЦ, Scopus, Web ofScience, Pubmed.

Результаты

Результаты. Легочная гипертензия (ЛГ) редкая, тяжелая и прогрессирующая патология с неблагоприятным прогнозом. Впервые клинический случай ЛГ описан в начале XX века, и, только в последние десятилетия появились новые лекарственные препараты, которые способны улучшить выживаемость пациентов. Проблема ЛГ в клинической практике врача педиатра актуальна, данная патология часто является осложнением многих сердечно-сосудистых и бронхопульмональных заболеваний у детей. Больше половины врожденных пороков сердца у детей сопровождаются ЛГ, бронхолегочная дисплазия и интерстициальные поражения легких также являются ведущими причинами ЛГ в детской популяции. Эволюция медикаментозной терапии связана с новыми представлениями о патогенезе заболевания. Если ранее ЛГ связывали с нарушением сосудистого тонуса, то в последнее время, патогенез рассматривается как сосудистое ремоделирование, сопровождающееся патоморфологическим каскадом клеточной пролиферации, гипертрофией, апоптозом, продукцией и деградацией межклеточного матрикса. Однако, не смотря на очевидный прорыв в лечении ЛГ, заболевание остается неизлечимым и жизнеугрожающим.

 Заключение

 Заключение. Сегодня проблема совершенной диагностики и эффективного лечения ЛГ до конца не решена. Однако, достигнутый в последнее десятилетие прогресс в патогенетической терапии позволяет надеяться на появление новых препаратов, способных существенно улучшить прогноз и продлить жизнь больного.

Aim

Aim. The study was designed to review the Russian and foreign literature of recent years on modern methods of diagnosis and management of children with pulmonary hypertension.

Materials and methods

Materials and methods. The analysis of Russian and foreign literature was conducted in RSCI, Scopus, Web of Science, Pubmed databases.

Results

Results. Pulmonary hypertension (LH) is a rare, severe and progressive pathology with an unfavorable prognosis. For the first time, the clinical case of LH was described in the early XXth century, and only in recent decades, there were new drugs that could improve patients’ survival. The problem of LH is relevant in the clinical practice of a pediatrician. This pathology is often a complication of many cardiovascular and bronchopulmonary diseases in children. More than half of congenital heart defects in children are accompanied by LH. Bronchopulmonary dysplasia and interstitial lung lesions are also the leading causes of LH in the pediatric population. The evolution of drug therapy is associated with new ideas about the pathogenesis of the disease. If previously LH was associated with vascular tone disorders, recently, the pathogenesis is considered as a vascular remodeling, accompanied by a pathomorphological cascade of cell proliferation, hypertrophy, apoptosis, production and degradation of the intercellular matrix. However, despite the obvious breakthrough in the treatment of LH, the disease remains incurable and life-threatening.

Conclusion

Conclusion. The problem of perfect diagnosis and effective treatment of LH is not completely solved nowadays. However, the progress made in pathogenetic therapy in the last decade allows us to hope for the emergence of new drugs that can significantly improve the prognosis and prolong the life of the patient.

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