Annals of the Russian academy of medical sciencesAnnals of the Russian academy of medical sciencesВестник Российской академии медицинских наук0869-60472414-3545"Paediatrician" Publishers LLC6010.15690/vramn.v70i2.1317PEDIATRICS: CURRENT ISSUESАКТУАЛЬНЫЕ ВОПРОСЫ ПЕДИАТРИИMODERN VIEWS ON СHILDREN'S INTERSTITIAL LUNG DISEASEСОВРЕМЕННЫЕ ПРЕДСТАВЛЕНИЯ ОБ ИНТЕРСТИЦИАЛЬНЫХ ЗАБОЛЕВАНИЯХ ЛЕГКИХ У ДЕТЕЙBoitsovaE. V.БойцоваЕ. В.
Russian Federation

доктор медицинских наук, руководитель лаборатории детской пульмонологии НИИ пульмонологии Санкт-Петербургского государственного медицинского университета им. акад. И.П. Павлова.Адрес: 199111, Санкт-Петербург, ул. Рентгена, д. 12

evboitsova@mail.ruBelyashovaM. A.БеляшоваМ. А.
Russian Federation

клинический ординатор, лаборант кафедры педиатрии Российского университета дружбы народовАдрес:117198, Москва, ул. Миклухо-Маклая, д. 6, тел.: +7 (499) 236-11-52

belyashova@gmail.comOvsyannikovD. Yu.ОвсянниковД. Ю.
Russian Federation

доктор медицинских наук, заведующий кафедрой педиатрии Российского университета дружбы народов Адрес:117198, Москва, ул. Миклухо-Маклая, д. 6, тел.: +7 (499) 236-11-52

mdovsyannikov@yahoo.comI.P. Pavlov St. Petersburg State Medical University, Russian FederationНИИ пульмонологии Первого Санкт-Петербургского государственного медицинского университета им. акад. И.П. Павлова, Российская ФедерацияPeoples’ Friendship University of Russia, Moscow, Russian FederationРоссийский университет дружбы народов, Москва, Российская Федерация08042015702Vestnik Rossiiskoi akademii medetsinskikh nauk / Annals of the Russian academy of medical sciencesВестник Российской академии медицинских наук22723607082015Copyright ©; 2015, "Paediatrician" Publishers LLCCopyright ©; 2015, Издательство "Педиатръ"2015"Paediatrician" Publishers LLCИздательство "Педиатръ"https://vestnikramn.spr-journal.ru/jour/article/view/60

Interstitial lung diseases (ILD, diffuse lung diseases) are a heterogeneous group of diseases in which a pathological process primarily involved alveoli and perialveolar interstitium, resulting in impaired gas exchange, restrictive changes of lung ventilation function and diffuse interstitial changes detectable by X-ray. Сhildren’s interstitial lung diseases is an topical problem of pediatric pulmonology. The article presents current information about classification, epidemiology, clinical presentation, diagnostics, treatment and prognosis of these rare diseases. The article describes the differences in the structure, pathogenesis, detection of various histological changes in children’s ILD compared with adult patients with ILD. Authors cite an instance of registers pediatric patients with ILD. The clinical semiotics of ILD, the possible results of objective research, the frequency of symptoms, the features of medical history, the changes detected on chest X-rays, CT semiotics described in detail. Particular attention was paid to interstitial lung diseases, occurring mainly in newborns and children during the first two years of life, such as congenital deficiencies of surfactant proteins, neuroendocrine cell hyperplasia of infancy, pulmonary interstitial glycogenosis. The diagnostic program for children’s ILD, therapy options are presented in this article.

Интерстициальные заболевания легких (ИЗЛ, синоним: диффузные заболевания легких) являются гетерогенной группой болезней, при которых в патологический процесс в первую очередь вовлекаются альвеолы и периальвеолярный интерстиций, что приводит к нарушению газообмена, рестриктивным нарушениям вентиляционной функции легких и диффузным интерстициальным изменениям, выявляемым при рентгенологическом исследовании. ИЗЛ у детей являются актуальной проблемой детской пульмонологии. В статье представлены современные сведения о классификации, эпидемиологии, клинической картине, диагностике, терапии и прогнозе данных редких заболеваний. Перечислены различия в структуре, патогенезе, обнаружении различных гистологических изменений при ИЗЛ у детей, по сравнению со взрослыми. Приведены примеры регистров больных ИЗЛ детского возраста. Детально охарактеризованы клиническая семиотика ИЗЛ, возможные результаты объективного исследования, частота симптомов, особенности анамнестических данных, изменения, выявляемые на рентгенограммах органов грудной клетки, компьютерно-томографическая семиотика. Особое внимание уделено интерстициальным заболеваниям легких, встречающимся преимущественно у новорожденных детей и детей первых двух лет жизни, таким как наследственные дефициты белков сурфактанта, нейроэндокринная гиперплазия младенцев, легочный интерстициальный гликогеноз. Представлена диагностическая программа у детей с ИЗЛ, возможности терапии.

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