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Merzhoeva Z.M.

I.M. Sechenov First Moscow State Medical University of the Ministry of Health of the Russian Federation (Sechenov University);
University Clinical Hospital No. 4 of the Sechenov University

Gainitdinova V.V.

I.M. Sechenov First Moscow State Medical University (Sechenov University)

Berikkhanov Z.G.

I.M. Sechenov First Moscow State Medical University of the Ministry of Health of the Russian Federation (Sechenov University);
University Clinical Hospital No. 4 of the Sechenov University;
National Medical Research Center for Pulmonology of the Sechenov University

Ataman K.S.

I.M. Sechenov First Moscow State Medical University of the Ministry of Health of the Russian Federation (Sechenov University);
University Clinical Hospital No. 4 of the Sechenov University;
National Medical Research Center for Pulmonology of the Sechenov University

Zhukova D.O.

I.M. Sechenov First Moscow State Medical University of the Ministry of Health of the Russian Federation (Sechenov University)

Avdeev S.N.

I.M. Sechenov First Moscow State Medical University of the Ministry of Health of the Russian Federation (Sechenov University);
National Medical Research Center for Pulmonology of the Sechenov University;
Pulmonology Scientific Research Institute under Federal Medical and Biological Agency of Russian Federation

The case of a patient with Mounier-Kun syndrome

Authors:

Merzhoeva Z.M., Gainitdinova V.V., Berikkhanov Z.G., Ataman K.S., Zhukova D.O., Avdeev S.N.

More about the authors

Journal: Journal of Respiratory Medicine. 2025;1(2): 83‑87

DOI: 10.17116/respmed2025102183

Read: 656 times

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Table of contents

THE CASE OF A PATIENT WITH MOUNIER-KUN SYNDROME
THE CASE OF A PATIENT WITH MOUNIER-KUN SYNDROME

To cite this article:

Merzhoeva ZM, Gainitdinova VV, Berikkhanov ZG, Ataman KS, Zhukova DO, Avdeev SN. The case of a patient with Mounier-Kun syndrome. Journal of Respiratory Medicine. 2025;1(2):83‑87. (In Russ.)
https://doi.org/10.17116/respmed2025102183

Подписка 2026 Респираторная медицина (Journal of Respiratory Medicine)
 
МСФ на ЯМ
Использование инфографики авторами научных работ
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Abstract:

Mounier-Kuhn syndrome (MKS) is a rare disease characterized by significant enlargement of tracheobronchial tree, recurrent cough and lower respiratory tract infections. Condition is typically diagnosed through imaging techniques such as computed tomography (CT) scans of the chest and bronchoscopy as well. There are asymptomatic or symptomatic cases in the disease while symptoms can be varied from minimal with preserved lung function to severe, including respiratory failure with recurrent pneumonia and bronchiectasis. Therapy is mainly symptomatic and aimed to manage symptoms. Surgery rarely has a place in the treatment of MKS. Although in the world literature reports of surgical interventions in some patients with MKS surgery have been presented, this type of treatment has not been widely adopted. Due to the lack of specific features in the clinical presentation of the condition, it should be taken into consideration in patients with recurrent episodes of pneumonia and frequent exacerbations of chronic bronchitis. This particular case is presented the patient with non-specific symptoms in the form of a non-productive cough and recurrent episodes of suffocation attacks. To evaluate the patient’s condition, CT of the chest and bronchoscopy are performed. These tests revealed tracheobronchomegaly and tracheal diverticula, which are both associated with the condition.

Keywords:

Munier-Kuhn syndrome
tracheobronchomegaly
tracheal diverticula
bronchiectasis

Authors:

Merzhoeva Z.M.

I.M. Sechenov First Moscow State Medical University of the Ministry of Health of the Russian Federation (Sechenov University);
University Clinical Hospital No. 4 of the Sechenov University

Gainitdinova V.V.

I.M. Sechenov First Moscow State Medical University (Sechenov University)

Berikkhanov Z.G.

I.M. Sechenov First Moscow State Medical University of the Ministry of Health of the Russian Federation (Sechenov University);
University Clinical Hospital No. 4 of the Sechenov University;
National Medical Research Center for Pulmonology of the Sechenov University

Ataman K.S.

I.M. Sechenov First Moscow State Medical University of the Ministry of Health of the Russian Federation (Sechenov University);
University Clinical Hospital No. 4 of the Sechenov University;
National Medical Research Center for Pulmonology of the Sechenov University

Zhukova D.O.

I.M. Sechenov First Moscow State Medical University of the Ministry of Health of the Russian Federation (Sechenov University)

Avdeev S.N.

I.M. Sechenov First Moscow State Medical University of the Ministry of Health of the Russian Federation (Sechenov University);
National Medical Research Center for Pulmonology of the Sechenov University;
Pulmonology Scientific Research Institute under Federal Medical and Biological Agency of Russian Federation

Received:

04.02.2025

Accepted:

24.03.2025

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References:

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