The case of a patient with Mounier-Kun syndrome
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.
Authors
- Sergey Nikolaevich Avdeev (ORCID: https://orcid.org/0000-0002-5999-2150)
- Zelimkhan G. M. Berikkhanov (ORCID: https://orcid.org/0000-0002-4335-3987)
- Viliya V. Gaynitdinova (ORCID: https://orcid.org/0000-0001-9928-926X)
- K. S. Ataman (ORCID: https://orcid.org/0000-0003-0249-4238)
- З. М. Мержоева (ORCID: https://orcid.org/0000-0002-3174-5000)
- Daria O. Zhukova (ORCID: https://orcid.org/0009-0009-1588-8798)
Institutions
- Sechenov University (RU)
- Federal Medical-Biological Agency (RU)
Publication Details
- Journal
- Journal of Respiratory Medicine
- Published
- 2025-06-06
- DOI
- https://doi.org/10.17116/respmed2025102183
- Primary Topic
- Tracheal and airway disorders
- Type
- article
- Field-Weighted Citation Impact
- 0.00