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Tracheobronchomegaly (Mounier-Kuhn Syndrome) in a 43-Year-Old Male: A Case Report

Mounier-Kuhn syndrome (MKS) or congenital tracheobronchomegaly is a rare disorder characterized by marked dilatation of the trachea and main bronchi, bronchiectasis, and recurrent respiratory tract infections. The etiology of this disorder is uncertain and the clinical presentation is variable. The...

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Autores principales: Babirye, Deborah, Walubembe, Jonathan, Babirye, Juliet Allen, Baluku, Joseph Baruch, Byakika-Kibwika, Pauline, Nabawanuka, Eva
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Dove 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9651021/
https://www.ncbi.nlm.nih.gov/pubmed/36388238
http://dx.doi.org/10.2147/IMCRJ.S386083
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author Babirye, Deborah
Walubembe, Jonathan
Babirye, Juliet Allen
Baluku, Joseph Baruch
Byakika-Kibwika, Pauline
Nabawanuka, Eva
author_facet Babirye, Deborah
Walubembe, Jonathan
Babirye, Juliet Allen
Baluku, Joseph Baruch
Byakika-Kibwika, Pauline
Nabawanuka, Eva
author_sort Babirye, Deborah
collection PubMed
description Mounier-Kuhn syndrome (MKS) or congenital tracheobronchomegaly is a rare disorder characterized by marked dilatation of the trachea and main bronchi, bronchiectasis, and recurrent respiratory tract infections. The etiology of this disorder is uncertain and the clinical presentation is variable. The diagnosis is usually made based on the characteristic computed tomography (CT) scan findings. This report describes a case of a 43-year-old man presenting with persistent cough and recurrent lower respiratory tract infections since childhood associated with copious amounts of purulent sputum, difficulty in breathing, and weight loss. In addition, he reported palpitations, dyspnea, orthopnea, abdominal and lower limb swelling. The chest X-ray showed a dilated trachea (35mm) and bronchi (26mm (right) and 27mm (left)) with cystic bronchiectasis and reticulolinear opacities predominantly involving the middle and lower lung zones. Chest CT scan confirmed the diagnosis of MKS as evidenced by dilated trachea and bronchi complicated by diverticula formation. Electrocardiogram, echocardiography and abdominal ultrasound scan showed features of right-sided heart failure secondary to pulmonary hypertension. MKS, although rare, should be considered as a possible diagnosis in patients presenting with productive chronic cough, recurrent pneumonia, or incomplete response to appropriate antibiotic therapy for pneumonia.
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spelling pubmed-96510212022-11-15 Tracheobronchomegaly (Mounier-Kuhn Syndrome) in a 43-Year-Old Male: A Case Report Babirye, Deborah Walubembe, Jonathan Babirye, Juliet Allen Baluku, Joseph Baruch Byakika-Kibwika, Pauline Nabawanuka, Eva Int Med Case Rep J Case Report Mounier-Kuhn syndrome (MKS) or congenital tracheobronchomegaly is a rare disorder characterized by marked dilatation of the trachea and main bronchi, bronchiectasis, and recurrent respiratory tract infections. The etiology of this disorder is uncertain and the clinical presentation is variable. The diagnosis is usually made based on the characteristic computed tomography (CT) scan findings. This report describes a case of a 43-year-old man presenting with persistent cough and recurrent lower respiratory tract infections since childhood associated with copious amounts of purulent sputum, difficulty in breathing, and weight loss. In addition, he reported palpitations, dyspnea, orthopnea, abdominal and lower limb swelling. The chest X-ray showed a dilated trachea (35mm) and bronchi (26mm (right) and 27mm (left)) with cystic bronchiectasis and reticulolinear opacities predominantly involving the middle and lower lung zones. Chest CT scan confirmed the diagnosis of MKS as evidenced by dilated trachea and bronchi complicated by diverticula formation. Electrocardiogram, echocardiography and abdominal ultrasound scan showed features of right-sided heart failure secondary to pulmonary hypertension. MKS, although rare, should be considered as a possible diagnosis in patients presenting with productive chronic cough, recurrent pneumonia, or incomplete response to appropriate antibiotic therapy for pneumonia. Dove 2022-11-07 /pmc/articles/PMC9651021/ /pubmed/36388238 http://dx.doi.org/10.2147/IMCRJ.S386083 Text en © 2022 Babirye et al. https://creativecommons.org/licenses/by-nc/3.0/This work is published and licensed by Dove Medical Press Limited. The full terms of this license are available at https://www.dovepress.com/terms.php and incorporate the Creative Commons Attribution – Non Commercial (unported, v3.0) License (http://creativecommons.org/licenses/by-nc/3.0/ (https://creativecommons.org/licenses/by-nc/3.0/) ). By accessing the work you hereby accept the Terms. Non-commercial uses of the work are permitted without any further permission from Dove Medical Press Limited, provided the work is properly attributed. For permission for commercial use of this work, please see paragraphs 4.2 and 5 of our Terms (https://www.dovepress.com/terms.php).
spellingShingle Case Report
Babirye, Deborah
Walubembe, Jonathan
Babirye, Juliet Allen
Baluku, Joseph Baruch
Byakika-Kibwika, Pauline
Nabawanuka, Eva
Tracheobronchomegaly (Mounier-Kuhn Syndrome) in a 43-Year-Old Male: A Case Report
title Tracheobronchomegaly (Mounier-Kuhn Syndrome) in a 43-Year-Old Male: A Case Report
title_full Tracheobronchomegaly (Mounier-Kuhn Syndrome) in a 43-Year-Old Male: A Case Report
title_fullStr Tracheobronchomegaly (Mounier-Kuhn Syndrome) in a 43-Year-Old Male: A Case Report
title_full_unstemmed Tracheobronchomegaly (Mounier-Kuhn Syndrome) in a 43-Year-Old Male: A Case Report
title_short Tracheobronchomegaly (Mounier-Kuhn Syndrome) in a 43-Year-Old Male: A Case Report
title_sort tracheobronchomegaly (mounier-kuhn syndrome) in a 43-year-old male: a case report
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9651021/
https://www.ncbi.nlm.nih.gov/pubmed/36388238
http://dx.doi.org/10.2147/IMCRJ.S386083
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