This condition is inherited in an autosomal recessive manner
Tracheobronchomegaly is a very rare congenital disorder of the lung primarily characterized by an abnormal widening of the upper airways.[1] 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.[2]
Contents
1Cause
2Diagnosis
3Treatment
4History
5References
6External links
Cause
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Diagnosis
Woodring et al. (1991) suggested the following diagnostic criteria for tracheomegaly in adults based on chest radiography:[3]
Adult Males: Tracheal transverse diameter > 25 mm and sagittal diameter > 27 mm.
Adult Females: Tracheal transverse diameter > 21 mm and sagittal diameter > 23 mm.
Treatment
This section is empty. You can help by adding to it. (August 2017)
History
The term "Mounier-Kuhn syndrome" derives from the characterization of the condition by Prof. Pierre-Louis Mounier-Kuhn in 1932, while the name "tracheobronchomegaly" was introduced by Katz et al. in 1962.[4][5][6]
References
^Schwartz, W; Rossoff L (Nov 1994). "Tracheobronchomegaly" (PDF). Chest. American College of Chest Physicians. 106 (5): 1589–1590. doi:10.1378/chest.106.5.1589. PMID 7956426. Retrieved 2008-02-27.
^Menon B, Aggarwal B, Iqbal A (2008). "Mounier-Kuhn syndrome: report of 8 cases of tracheobronchomegaly with associated complications". South Med J. 101 (1): 83–87. doi:10.1097/SMJ.0b013e31815d4259. PMID 18176298.
^Smith DL, Withers N, Holloway B, Collins JV (August 1994). "Tracheobronchomegaly: an unusual presentation of a rare condition". Thorax. 49 (8): 840–1. doi:10.1136/thx.49.8.840. PMC 475137. PMID 8091335.
^KATZ I, LEVINE M, HERMAN P (December 1962). "Tracheobronchiomegaly. The Mounier-Kuhn syndrome". Am J Roentgenol Radium Ther Nucl Med. 88: 1084–94. PMID 13958486.
^Mounier-Kuhn P. Dilatation de la trachée: constatations radiographiques etbronchoscopiques. Lyon Med. 1932;150:106-9.
External links
Classification
D
ICD-9-CM: 748.3
OMIM: 275300
MeSH: D014137
DiseasesDB: 31501
External resources
Orphanet: 3347
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Congenital malformations and deformations of respiratory system (Q30–Q34, 748)
Upper RT
Nose
Choanal atresia
Arrhinia
Larynx
Laryngeal cyst
Laryngocele
Laryngomalacia
Lower RT
Trachea and bronchus
Tracheomalacia
Tracheal stenosis
Bronchomalacia
Tracheobronchomegaly
Lung
Bronchiectasis
Pulmonary hypoplasia
Pulmonary sequestration
Congenital cystic adenomatoid malformation
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UpToDate Contents
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…also present as tracheobronchomegaly (Mounier-Kuhn syndrome) when the defect extends into the central bronchi . The peripheral airways maintain a normal diameter . Tracheobronchomegaly can be diagnosed …
…weakness of the trachea and/or mainstem bronchi and are discussed separately. Tracheobronchomegaly (eg, Mounier-Kuhn syndrome) can be diagnosed when the diameter of the trachea (measured 2 cm above the main …
…right upper lobe. Dilation of the trachea can be either diffuse or focal. Tracheobronchomegaly (ie, Mounier-Kuhn syndrome) and traction tracheomegaly can cause diffuse tracheal dilation. This pattern …
…bleeding persists, surgical resection may be necessary . For patients with tracheobronchomegaly (eg, Mounier-Kuhn syndrome), tracheal stabilizing procedures, such as proximal stents or tracheobronchoplasty …
…the few cases reported in the literature, the prognosis is variable. Congenital tracheobronchomegaly (Mounier-Kuhn syndrome) is a congenital disorder that is characterized by markedly dilated trachea and…
The Medical clinics of North America. 2019 May;103(3)503-515.
There are hundreds of rare orphan lung diseases. We have highlighted five of them, one from each of the five major categories of pulmonary disorders: pleuroparenchymal fibroelastosis (a rare diffuse parenchymal lung disease), pulmonary alveolar proteinosis (a rare autoimmune and diffuse parenchymal
Mounier-Kuhn syndrome, or tracheobronchomegaly, is a rare clinical and radiologic condition characterized by marked tracheobronchial dilation and recurrent lower respiratory tract infections. Diagnosis is typically ...
Mounier-Kuhn syndrome is a somewhat controversial entity and used synonymously with tracheobronchomegaly by most authors 7-9. Epidemiology Mounier-Kuhn syndrome is most frequently seen in middle age men before the age of ...
A collection of disease information resources and questions answered by our Genetic and Rare Diseases Information Specialists for Mounier-Kuhn syndrome ... If you need medical advice, you can look for doctors or ...