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...

Descripción completa

Detalles Bibliográficos
Autores principales: Boglou, Panagiotis, Papanas, Nikolaos, Oikonomou, Anastasia, Bakali, Stamatia, Steiropoulos, Paschalis
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