Cargando…
Mounier-Kuhn Syndrome in an Elderly Female with Pulmonary Fibrosis
Mounier-Kuhn syndrome (MKS), or tracheobronchomegaly, is a rare clinical and radiologic condition characterized by pronounced tracheobronchial dilation and recurrent lower respiratory tract infections. Tracheobronchomegaly presents when the defect extends to the central bronchi. MKS can be diagnosed...
Autores principales: | , , , , |
---|---|
Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Hindawi Publishing Corporation
2016
|
Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5002455/ https://www.ncbi.nlm.nih.gov/pubmed/27597867 http://dx.doi.org/10.1155/2016/8708251 |
_version_ | 1782450569844097024 |
---|---|
author | Boglou, Panagiotis Papanas, Nikolaos Oikonomou, Anastasia Bakali, Stamatia Steiropoulos, Paschalis |
author_facet | Boglou, Panagiotis Papanas, Nikolaos Oikonomou, Anastasia Bakali, Stamatia Steiropoulos, Paschalis |
author_sort | Boglou, Panagiotis |
collection | PubMed |
description | Mounier-Kuhn syndrome (MKS), or tracheobronchomegaly, is a rare clinical and radiologic condition characterized by pronounced tracheobronchial dilation and recurrent lower respiratory tract infections. Tracheobronchomegaly presents when the defect extends to the central bronchi. MKS can be diagnosed in adult women when the transverse and sagittal diameters of the trachea, right mainstem bronchus, and left mainstem bronchus exceed 21, 23, 19.8, and 17.4 mm, respectively. Its diagnosis is based on chest radiograph and chest computed tomography (CT). Patients, usually middle-aged men, may be asymptomatic or present with clinical manifestations ranging from minimal symptoms with preserved lung function to severe respiratory failure. Pulmonary function tests (PFTs) typically reveal a restrictive pattern. This report presents an elderly woman with previously diagnosed pulmonary fibrosis with symptoms of increased sputum production and haemoptysis. High-resolution chest CT showed tracheal and main stem bronchi dilatation along with bronchial diverticulosis. PFTs indicated a restrictive pattern characteristic of the underlying pulmonary fibrosis. The patient is the oldest, referred to the female gender, at presentation of MKS hitherto reported. This case highlights the need to include MKS in the differential diagnosis of recurrent lower respiratory tract infections, even in older subjects. |
format | Online Article Text |
id | pubmed-5002455 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2016 |
publisher | Hindawi Publishing Corporation |
record_format | MEDLINE/PubMed |
spelling | pubmed-50024552016-09-05 Mounier-Kuhn Syndrome in an Elderly Female with Pulmonary Fibrosis Boglou, Panagiotis Papanas, Nikolaos Oikonomou, Anastasia Bakali, Stamatia Steiropoulos, Paschalis Case Rep Med Case Report Mounier-Kuhn syndrome (MKS), or tracheobronchomegaly, is a rare clinical and radiologic condition characterized by pronounced tracheobronchial dilation and recurrent lower respiratory tract infections. Tracheobronchomegaly presents when the defect extends to the central bronchi. MKS can be diagnosed in adult women when the transverse and sagittal diameters of the trachea, right mainstem bronchus, and left mainstem bronchus exceed 21, 23, 19.8, and 17.4 mm, respectively. Its diagnosis is based on chest radiograph and chest computed tomography (CT). Patients, usually middle-aged men, may be asymptomatic or present with clinical manifestations ranging from minimal symptoms with preserved lung function to severe respiratory failure. Pulmonary function tests (PFTs) typically reveal a restrictive pattern. This report presents an elderly woman with previously diagnosed pulmonary fibrosis with symptoms of increased sputum production and haemoptysis. High-resolution chest CT showed tracheal and main stem bronchi dilatation along with bronchial diverticulosis. PFTs indicated a restrictive pattern characteristic of the underlying pulmonary fibrosis. The patient is the oldest, referred to the female gender, at presentation of MKS hitherto reported. This case highlights the need to include MKS in the differential diagnosis of recurrent lower respiratory tract infections, even in older subjects. Hindawi Publishing Corporation 2016 2016-08-15 /pmc/articles/PMC5002455/ /pubmed/27597867 http://dx.doi.org/10.1155/2016/8708251 Text en Copyright © 2016 Panagiotis Boglou et al. https://creativecommons.org/licenses/by/4.0/ This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. |
spellingShingle | Case Report Boglou, Panagiotis Papanas, Nikolaos Oikonomou, Anastasia Bakali, Stamatia Steiropoulos, Paschalis Mounier-Kuhn Syndrome in an Elderly Female with Pulmonary Fibrosis |
title | Mounier-Kuhn Syndrome in an Elderly Female with Pulmonary Fibrosis |
title_full | Mounier-Kuhn Syndrome in an Elderly Female with Pulmonary Fibrosis |
title_fullStr | Mounier-Kuhn Syndrome in an Elderly Female with Pulmonary Fibrosis |
title_full_unstemmed | Mounier-Kuhn Syndrome in an Elderly Female with Pulmonary Fibrosis |
title_short | Mounier-Kuhn Syndrome in an Elderly Female with Pulmonary Fibrosis |
title_sort | mounier-kuhn syndrome in an elderly female with pulmonary fibrosis |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5002455/ https://www.ncbi.nlm.nih.gov/pubmed/27597867 http://dx.doi.org/10.1155/2016/8708251 |
work_keys_str_mv | AT bogloupanagiotis mounierkuhnsyndromeinanelderlyfemalewithpulmonaryfibrosis AT papanasnikolaos mounierkuhnsyndromeinanelderlyfemalewithpulmonaryfibrosis AT oikonomouanastasia mounierkuhnsyndromeinanelderlyfemalewithpulmonaryfibrosis AT bakalistamatia mounierkuhnsyndromeinanelderlyfemalewithpulmonaryfibrosis AT steiropoulospaschalis mounierkuhnsyndromeinanelderlyfemalewithpulmonaryfibrosis |