pulmoПульмонологияPULMONOLOGIYA0869-01892541-9617Scientific and Practical Journal “PULMONOLOGIYA” LLC10.18093/0869-0189-2024-4313pulmo-4313Research ArticleЗАМЕТКИ ИЗ ПРАКТИКИPRACTICAL NOTESДиффузная идиопатическая гиперплазия нейроэндокринных клеток легких у взрослыхDiffuse idiopathic pulmonary neuroendocrine cell hyperplasia in adultshttps://orcid.org/0000-0001-8198-9313БаймакановаГ. Е.BaimakanovaG. E.

Баймаканова Гульсара Есенгельдиевна – д. м. н., заведующая отделением пульмонологии.

111123, Москва, шоссе Энтузиастов, 86

Тел.: (495) 304-30-39

Gulsara E. Baimakanova - Doctor of Medicine, Head of the Department of Pulmonology, State Budgetary Institution of Healthcare of the city of Moscow “Moscow Clinical Scientific and Practical Center named after A.S.Loginov of the Department of Healthcare of the City of Moscow”.

Shosse Entuziastov 86, Moscow, 111123

Tel.: (495) 304-30-39

gulsara.bai@mail.ruhttps://orcid.org/0000-0002-6547-8553КириченкоН. Д.KirichenkoN. D.

Кириченко Наталья Дмитриевна – врач-пульмонолог отделения пульмонологии.

111123, Москва, шоссе Энтузиастов, 86

Тел.: (495) 304-30-39

Natalia D. Kirichenko - Pulmonologist, Pulmonology Department, State Budgetary Institution of Healthcare of the city of Moscow “Moscow Clinical Scientific and Practical Center named after A.S. Loginov of the Department of Healthcare of the City of Moscow”.

Shosse Entuziastov 86, Moscow, 111123

Tel.: (495) 304-30-39

n.kirichenko@mknc.ruhttps://orcid.org/0000-0003-0889-2720КарнауховН. С.KarnaukhovN. S.

Карнаухов Николай Сергеевич – к. м. н., заведующий патологоанатомическим отделением.

111123, Москва, шоссе Энтузиастов, 86

Тел.: (495) 304-30-39

Nikolai S. Karnaukhov - Candidate of Medicine, Head of the Pathology Department, State Budgetary Institution of Healthcare of the city of Moscow “Moscow Clinical Scientific and Practical Center named after A.S. Loginov of the Department of Healthcare of the City of Moscow”.

Shosse Entuziastov 86, Moscow, 111123

Tel.: (495) 304-30-39

n.karnaukhov@mknc.ruhttps://orcid.org/0009-0008-3295-8236КолягоЕ. М.KolyagoE. M.

Коляго Елена Максимовна – врач-онколог отделения химиотерапии.

111123, Москва, шоссе Энтузиастов, 86

Тел.: (495) 304-30-39

Elena M. Kolyago - Oncologist, Chemotherapy Department, State Budgetary Institution of Healthcare of the city of Moscow “Moscow Clinical Scientific and Practical Center named after A.S. Loginov of the Department of Healthcare of the City of Moscow”.

Shosse Entuziastov 86, Moscow, 111123

Tel.: (495) 304-30-39

elenakolyago@yandex.ruhttps://orcid.org/0000-0002-4695-7866БродскаяО. Н.BrodskayaO. N.

Бродская Ольга Наумовна – к. м. н., доцент кафедры госпитальной терапии педиатрического факультета.

117997, Москва, ул. Островитянова, 1

Тел.: (499) 780-08-50

Olga N. Brodskaya - Candidate of Medicine, Associate Professor, Department of Hospital Internal Medicine, Pediatric Faculty, Federal State Autonomous Educational Institution of Higher Education “N.I. Pirogov Russian National Research Medical University” of the Ministry of Health of the Russian Federation.

Ostrovityanova ul. 1, Moscow, 117997

Tel.: (499) 780-08-50

brodskaya@inbox.ruБутюгинаИ. Н.ButyuginaI. N.

Бутюгина Ирина Николаевна – врач-пульмонолог пульмонологического отделения для больных муковисцидозом ГБУЗ г. Москвы «ГКБ им. Д.Д. Плетнева» ДЗМ; научный сотрудник лаборатории муковисцидоза ФГБУ «НИИ пульмонологии ФМБА России».

105077, Москва, ул. 11-я Парковая, 32; 115682, Москва, Ореховый бульвар, 28

Тел.: (495) 965-23-24

Irina N. Butyugina - Pulmonologist, Pulmonology Department for patients with cystic fibrosis, State Budgetary Healthcare Institution “City Clinical Hospital named after D.D. Pletnev of the Department of Health of Moscow City”; Researcher, Cystic Fibrosis Laboratory, Federal State Budgetary Institution “Pulmonology Scientific Research Institute” under Federal Medical and Biological Agency of Russian Federation.

Odinnadtsataya Parkovaya ul. 32, Moscow, 105077; Orekhovyy bul’var 28, Moscow, 115682

Tel.: (495) 965-23-24

maxibut2@mail.ruhttps://orcid.org/0000-0003-2083-0864ГрипасД. Ю.GripasD. Yu.

Грипас Дарья Юрьевна – студентка VI курса.

117997, Москва, ул. Островитянова, 1

Тел.: (985) 452-37-52

Daria Yu. Gripas - VI year student, Federal State Autonomous Educational Institution of Higher Education “N.I. Pirogov Russian National Research Medical University” of the Ministry of Health of the Russian Federation.

Ostrovityanova ul. 1, Moscow, 117997

Tel.: (985) 452-37-52

dagripas@mail.ruhttps://orcid.org/0000-0003-3704-3437УколоваС. К.UkolovaS. K.

Уколова Софья Кирилловна – студентка VI курса.

117997, Москва, ул. Островитянова, 1

Тел.: (910) 415-88-81

Sofia K. Ukolova - VI year student, Federal State Autonomous Educational Institution of Higher Education “N.I. Pirogov Russian National Research Medical University” of the Ministry of Health of the Russian Federation.

Ostrovityanova ul. 1, Moscow, 117997

Tel.: (910) 415-88-81

sofia.ukolova888@gmail.comГосударственное бюджетное учреждение здравоохранения города Москвы «Московский клинический научно-практический центр имени А.С. Логинова Департамента здравоохранения города Москвы»РоссияState Budgetary Institution of Healthcare of the city of Moscow “Moscow Clinical Scientific and Practical Center named after A.S.Loginov of the Department of Healthcare of the City of Moscow”Russian FederationФедеральное государственное автономное образовательное учреждение высшего образования «Российский национальный исследовательский медицинский университет имени Н.И. Пирогова» Министерства здравоохранения Российской ФедерацииРоссияFederal State Autonomous Educational Institution of Higher Education “N.I. Pirogov Russian National Research Medical University” of the Ministry of Health of the Russian FederationRussian FederationГосударственное бюджетное учреждение здравоохранения города Москвы «Городская клиническая больница имени Д.Д. Плетнева Департамента здравоохранения города Москвы»; Федеральное государственное бюджетное учреждение «Научно-исследовательский институт пульмонологии Федерального медико-биологического агентства России»РоссияState Budgetary Healthcare Institution “City Clinical Hospital named after D.D. Pletnev of the Department of Health of Moscow City”; Federal State Budgetary Institution “Pulmonology Scientific Research Institute” under Federal Medical and Biological Agency of Russian FederationRussian Federation202426022024344559568Copyright © Баймаканова Г.Е., Кириченко Н.Д., Карнаухов Н.С., Коляго Е.М., Бродская О.Н., Бутюгина И.Н., Грипас Д.Ю., Уколова С.К., 20242024Баймаканова Г.Е., Кириченко Н.Д., Карнаухов Н.С., Коляго Е.М., Бродская О.Н., Бутюгина И.Н., Грипас Д.Ю., Уколова С.К.Baimakanova G.E., Kirichenko N.D., Karnaukhov N.S., Kolyago E.M., Brodskaya O.N., Butyugina I.N., Gripas D.Y., Ukolova S.K.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://journal.pulmonology.ru/pulm/article/view/4313

Диффузная идиопатическая гиперплазия нейроэндокринных клеток легких (ДИГНКЛ) – редкое малоизученное заболевание, чаще всего поражающее некурящих женщин в возрасте около 60 лет, которое должно учитываться при проведении дифференциальной диагностики резистентной к терапии бронхообструкции в сочетании с очаговыми изменениями и мозаичной воздушностью по данным компьютерной томографии (КТ) органов грудной клетки (ОГК). Целью работы явилось обсуждение аспектов клинической и морфологической диагностики, возможностей терапии, оценки прогноза и необходимости динамического наблюдения на примере 55-летней пациентки с ДИГНКЛ. Также представлен краткий обзор научной литературы, посвященной ДИГНКЛ. Наиболее частыми клиническими проявлениями являются кашель, одышка, стридор, бронхообструктивный синдром. Диагноз возможно подтвердить только при помощи гистологического и иммуногистохимического исследований. ДИГНКЛ следует рассматривать как часть дифференциальной диагностики у пациентов с длительным кашлем, одышкой неясного генеза и изменениями на КТ ОГК в виде легочных очагов и мозаичной воздушности. Заключение. На примере клинического наблюдения продемонстрированы трудность и важность своевременной диагностики редкого заболевания.

Diffuse Idiopathic Pulmonary NeuroEndocrine Cell Hyperplasia (DIPNECH) is an understudied rare disease that should be taken into account in the differential diagnosis of therapy-resistant bronchial obstruction combined with focal pulmonary lesions and mosaic attenuation on chest CT. The aim of this article is to discuss the aspects of clinical and morphological diagnosis, therapeutic options, prognosis assessment, and the need for follow-up using a clinical case as an example. The article describes a clinical case of DIPNECH in a 55-year-old woman, and also provides a brief review of the scientific literature on DIPNECH. DIPNECH is a rare disease that most often affects non-smoking women aged around 60 years. The most frequent clinical manifestations are cough, dyspnea, stridor, and broncho-obstructive syndrome. Only histological and immunohistochemical assays can confirm the diagnosis. DIPNECH should be considered as part of the differential diagnosis in patients with prolonged cough, dyspnea of unclear genesis and changes on chest CT in the form of pulmonary focal lesions and mosaic attenuation. Conclusion. The presented clinical case shows the challenges and importance of timely diagnosis.

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