ICD-9-CM 748.3 DiseasesDB 31501 | OMIM 275300 MeSH D014137 | |
Mounier-Kuhn syndrome (also called tracheobronchomegaly) is a very rare congenital disorder of the lung primarily characterized by an abnormal widening of the upper airways. The abnormally widened trachea and mainstem bronchi are associated with recurrent lower respiratory tract infection and copious purulent sputum production, eventually leading to bronchiectasis and other respiratory complications.
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Diagnosis
Woodring et al. (1991) suggested the following diagnostic criteria for tracheomegaly in adults based on chest radiography:
History
The term "Mounier-Kuhn syndrome" derives from the characterization of the condition by Mounier-Kuhn in 1932, while the name "tracheobronchomegaly" was introduced by Katz et al. in 1962.
References
Tracheobronchomegaly Wikipedia(Text) CC BY-SA