The Mounier-Kuhn syndrome

Background. The Mounier-Kuhn syndrome (MKS) or tracheobronchomegaly (TBM) is a rare condition of unknown frequency, up to now about 100 cases have been reported. It presents by marked dilatation of the trachea and major bronchi, recurrent respiratory infections and consecutive bronchiectasis and sca...
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Autor*in:	Milić Rade [verfasserIn] Plavec Goran [verfasserIn] Stojisavljević Marko [verfasserIn] Tomić Ilija [verfasserIn] Šarac Sanja [verfasserIn]

Format:	E-Artikel
Sprache:	Englisch ; srp

Erschienen:	2010

Schlagwörter:	tracheobronchomegaly diagnosis, differential tomography

Übergeordnetes Werk:	In: Vojnosanitetski Pregled - Military Health Department, Ministry of Defance, Serbia, 2012, 67(2010), 10, Seite 867-870
Übergeordnetes Werk:	volume:67 ; year:2010 ; number:10 ; pages:867-870

Links:	Link aufrufen Link aufrufen Link aufrufen Journal toc

DOI / URN:	10.2298/VSP1010867M

Katalog-ID:	DOAJ074863312

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allfields	10.2298/VSP1010867M doi (DE-627)DOAJ074863312 (DE-599)DOAJccf9d0d306564ef3bc7b614cfdf65459 DE-627 ger DE-627 rakwb eng srp R5-920 Milić Rade verfasserin aut The Mounier-Kuhn syndrome 2010 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier Background. The Mounier-Kuhn syndrome (MKS) or tracheobronchomegaly (TBM) is a rare condition of unknown frequency, up to now about 100 cases have been reported. It presents by marked dilatation of the trachea and major bronchi, recurrent respiratory infections and consecutive bronchiectasis and scars in lung parenchyme. Sometimes enlargement of transversal colon may be present. Diagnosis is usually made radiologically. Cases report. We reviewed two patients 77 and 72 years old with typical clinical presentation and enlarged upper airways, in whom diagnosis of MKS was established by chest multislice computed tomography (MSCT). Transversal diameter of trachea was 30 mm in the first patient and 33 mm in the other one. Complications of syndrome (tracheal diverticulosis in the first patient, and pulmonary fibrosis, bulous emphysema and bronchiectasis in both patients) also were seen. Lung function tests showed mixed ventilation disorder, and disturbance of respiratory gases values in arterial blood samples. Conclusion. The Mounier-Kuhn syndrome is rare disorder, although diagnosis is often missed. Clinical presentation is similar to chronic obstructive pulmonary disease or bronchiectasis. Computed tomography is gold standard for diagnosis. Therapy is presumely supportive. tracheobronchomegaly diagnosis, differential tomography Medicine (General) Plavec Goran verfasserin aut Stojisavljević Marko verfasserin aut Tomić Ilija verfasserin aut Šarac Sanja verfasserin aut In Vojnosanitetski Pregled Military Health Department, Ministry of Defance, Serbia, 2012 67(2010), 10, Seite 867-870 (DE-627)474382613 (DE-600)2169819-3 24060720 nnns volume:67 year:2010 number:10 pages:867-870 https://doi.org/10.2298/VSP1010867M kostenfrei https://doaj.org/article/ccf9d0d306564ef3bc7b614cfdf65459 kostenfrei http://www.doiserbia.nb.rs/img/doi/0042-8450/2010/0042-84501010867M.pdf kostenfrei https://doaj.org/toc/0042-8450 Journal toc kostenfrei GBV_USEFLAG_A SYSFLAG_A GBV_DOAJ GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_702 GBV_ILN_2001 GBV_ILN_2003 GBV_ILN_2005 GBV_ILN_2006 GBV_ILN_2008 GBV_ILN_2009 GBV_ILN_2010 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2015 GBV_ILN_2020 GBV_ILN_2021 GBV_ILN_2025 GBV_ILN_2031 GBV_ILN_2044 GBV_ILN_2048 GBV_ILN_2050 GBV_ILN_2055 GBV_ILN_2056 GBV_ILN_2057 GBV_ILN_2061 GBV_ILN_2111 GBV_ILN_2153 GBV_ILN_2190 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 67 2010 10 867-870
spelling	10.2298/VSP1010867M doi (DE-627)DOAJ074863312 (DE-599)DOAJccf9d0d306564ef3bc7b614cfdf65459 DE-627 ger DE-627 rakwb eng srp R5-920 Milić Rade verfasserin aut The Mounier-Kuhn syndrome 2010 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier Background. The Mounier-Kuhn syndrome (MKS) or tracheobronchomegaly (TBM) is a rare condition of unknown frequency, up to now about 100 cases have been reported. It presents by marked dilatation of the trachea and major bronchi, recurrent respiratory infections and consecutive bronchiectasis and scars in lung parenchyme. Sometimes enlargement of transversal colon may be present. Diagnosis is usually made radiologically. Cases report. We reviewed two patients 77 and 72 years old with typical clinical presentation and enlarged upper airways, in whom diagnosis of MKS was established by chest multislice computed tomography (MSCT). Transversal diameter of trachea was 30 mm in the first patient and 33 mm in the other one. Complications of syndrome (tracheal diverticulosis in the first patient, and pulmonary fibrosis, bulous emphysema and bronchiectasis in both patients) also were seen. Lung function tests showed mixed ventilation disorder, and disturbance of respiratory gases values in arterial blood samples. Conclusion. The Mounier-Kuhn syndrome is rare disorder, although diagnosis is often missed. Clinical presentation is similar to chronic obstructive pulmonary disease or bronchiectasis. Computed tomography is gold standard for diagnosis. Therapy is presumely supportive. tracheobronchomegaly diagnosis, differential tomography Medicine (General) Plavec Goran verfasserin aut Stojisavljević Marko verfasserin aut Tomić Ilija verfasserin aut Šarac Sanja verfasserin aut In Vojnosanitetski Pregled Military Health Department, Ministry of Defance, Serbia, 2012 67(2010), 10, Seite 867-870 (DE-627)474382613 (DE-600)2169819-3 24060720 nnns volume:67 year:2010 number:10 pages:867-870 https://doi.org/10.2298/VSP1010867M kostenfrei https://doaj.org/article/ccf9d0d306564ef3bc7b614cfdf65459 kostenfrei http://www.doiserbia.nb.rs/img/doi/0042-8450/2010/0042-84501010867M.pdf kostenfrei https://doaj.org/toc/0042-8450 Journal toc kostenfrei GBV_USEFLAG_A SYSFLAG_A GBV_DOAJ GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_702 GBV_ILN_2001 GBV_ILN_2003 GBV_ILN_2005 GBV_ILN_2006 GBV_ILN_2008 GBV_ILN_2009 GBV_ILN_2010 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2015 GBV_ILN_2020 GBV_ILN_2021 GBV_ILN_2025 GBV_ILN_2031 GBV_ILN_2044 GBV_ILN_2048 GBV_ILN_2050 GBV_ILN_2055 GBV_ILN_2056 GBV_ILN_2057 GBV_ILN_2061 GBV_ILN_2111 GBV_ILN_2153 GBV_ILN_2190 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 67 2010 10 867-870
allfields_unstemmed	10.2298/VSP1010867M doi (DE-627)DOAJ074863312 (DE-599)DOAJccf9d0d306564ef3bc7b614cfdf65459 DE-627 ger DE-627 rakwb eng srp R5-920 Milić Rade verfasserin aut The Mounier-Kuhn syndrome 2010 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier Background. The Mounier-Kuhn syndrome (MKS) or tracheobronchomegaly (TBM) is a rare condition of unknown frequency, up to now about 100 cases have been reported. It presents by marked dilatation of the trachea and major bronchi, recurrent respiratory infections and consecutive bronchiectasis and scars in lung parenchyme. Sometimes enlargement of transversal colon may be present. Diagnosis is usually made radiologically. Cases report. We reviewed two patients 77 and 72 years old with typical clinical presentation and enlarged upper airways, in whom diagnosis of MKS was established by chest multislice computed tomography (MSCT). Transversal diameter of trachea was 30 mm in the first patient and 33 mm in the other one. Complications of syndrome (tracheal diverticulosis in the first patient, and pulmonary fibrosis, bulous emphysema and bronchiectasis in both patients) also were seen. Lung function tests showed mixed ventilation disorder, and disturbance of respiratory gases values in arterial blood samples. Conclusion. The Mounier-Kuhn syndrome is rare disorder, although diagnosis is often missed. Clinical presentation is similar to chronic obstructive pulmonary disease or bronchiectasis. Computed tomography is gold standard for diagnosis. Therapy is presumely supportive. tracheobronchomegaly diagnosis, differential tomography Medicine (General) Plavec Goran verfasserin aut Stojisavljević Marko verfasserin aut Tomić Ilija verfasserin aut Šarac Sanja verfasserin aut In Vojnosanitetski Pregled Military Health Department, Ministry of Defance, Serbia, 2012 67(2010), 10, Seite 867-870 (DE-627)474382613 (DE-600)2169819-3 24060720 nnns volume:67 year:2010 number:10 pages:867-870 https://doi.org/10.2298/VSP1010867M kostenfrei https://doaj.org/article/ccf9d0d306564ef3bc7b614cfdf65459 kostenfrei http://www.doiserbia.nb.rs/img/doi/0042-8450/2010/0042-84501010867M.pdf kostenfrei https://doaj.org/toc/0042-8450 Journal toc kostenfrei GBV_USEFLAG_A SYSFLAG_A GBV_DOAJ GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_702 GBV_ILN_2001 GBV_ILN_2003 GBV_ILN_2005 GBV_ILN_2006 GBV_ILN_2008 GBV_ILN_2009 GBV_ILN_2010 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2015 GBV_ILN_2020 GBV_ILN_2021 GBV_ILN_2025 GBV_ILN_2031 GBV_ILN_2044 GBV_ILN_2048 GBV_ILN_2050 GBV_ILN_2055 GBV_ILN_2056 GBV_ILN_2057 GBV_ILN_2061 GBV_ILN_2111 GBV_ILN_2153 GBV_ILN_2190 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 67 2010 10 867-870
allfieldsGer	10.2298/VSP1010867M doi (DE-627)DOAJ074863312 (DE-599)DOAJccf9d0d306564ef3bc7b614cfdf65459 DE-627 ger DE-627 rakwb eng srp R5-920 Milić Rade verfasserin aut The Mounier-Kuhn syndrome 2010 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier Background. The Mounier-Kuhn syndrome (MKS) or tracheobronchomegaly (TBM) is a rare condition of unknown frequency, up to now about 100 cases have been reported. It presents by marked dilatation of the trachea and major bronchi, recurrent respiratory infections and consecutive bronchiectasis and scars in lung parenchyme. Sometimes enlargement of transversal colon may be present. Diagnosis is usually made radiologically. Cases report. We reviewed two patients 77 and 72 years old with typical clinical presentation and enlarged upper airways, in whom diagnosis of MKS was established by chest multislice computed tomography (MSCT). Transversal diameter of trachea was 30 mm in the first patient and 33 mm in the other one. Complications of syndrome (tracheal diverticulosis in the first patient, and pulmonary fibrosis, bulous emphysema and bronchiectasis in both patients) also were seen. Lung function tests showed mixed ventilation disorder, and disturbance of respiratory gases values in arterial blood samples. Conclusion. The Mounier-Kuhn syndrome is rare disorder, although diagnosis is often missed. Clinical presentation is similar to chronic obstructive pulmonary disease or bronchiectasis. Computed tomography is gold standard for diagnosis. Therapy is presumely supportive. tracheobronchomegaly diagnosis, differential tomography Medicine (General) Plavec Goran verfasserin aut Stojisavljević Marko verfasserin aut Tomić Ilija verfasserin aut Šarac Sanja verfasserin aut In Vojnosanitetski Pregled Military Health Department, Ministry of Defance, Serbia, 2012 67(2010), 10, Seite 867-870 (DE-627)474382613 (DE-600)2169819-3 24060720 nnns volume:67 year:2010 number:10 pages:867-870 https://doi.org/10.2298/VSP1010867M kostenfrei https://doaj.org/article/ccf9d0d306564ef3bc7b614cfdf65459 kostenfrei http://www.doiserbia.nb.rs/img/doi/0042-8450/2010/0042-84501010867M.pdf kostenfrei https://doaj.org/toc/0042-8450 Journal toc kostenfrei GBV_USEFLAG_A SYSFLAG_A GBV_DOAJ GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_702 GBV_ILN_2001 GBV_ILN_2003 GBV_ILN_2005 GBV_ILN_2006 GBV_ILN_2008 GBV_ILN_2009 GBV_ILN_2010 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2015 GBV_ILN_2020 GBV_ILN_2021 GBV_ILN_2025 GBV_ILN_2031 GBV_ILN_2044 GBV_ILN_2048 GBV_ILN_2050 GBV_ILN_2055 GBV_ILN_2056 GBV_ILN_2057 GBV_ILN_2061 GBV_ILN_2111 GBV_ILN_2153 GBV_ILN_2190 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 67 2010 10 867-870
allfieldsSound	10.2298/VSP1010867M doi (DE-627)DOAJ074863312 (DE-599)DOAJccf9d0d306564ef3bc7b614cfdf65459 DE-627 ger DE-627 rakwb eng srp R5-920 Milić Rade verfasserin aut The Mounier-Kuhn syndrome 2010 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier Background. The Mounier-Kuhn syndrome (MKS) or tracheobronchomegaly (TBM) is a rare condition of unknown frequency, up to now about 100 cases have been reported. It presents by marked dilatation of the trachea and major bronchi, recurrent respiratory infections and consecutive bronchiectasis and scars in lung parenchyme. Sometimes enlargement of transversal colon may be present. Diagnosis is usually made radiologically. Cases report. We reviewed two patients 77 and 72 years old with typical clinical presentation and enlarged upper airways, in whom diagnosis of MKS was established by chest multislice computed tomography (MSCT). Transversal diameter of trachea was 30 mm in the first patient and 33 mm in the other one. Complications of syndrome (tracheal diverticulosis in the first patient, and pulmonary fibrosis, bulous emphysema and bronchiectasis in both patients) also were seen. Lung function tests showed mixed ventilation disorder, and disturbance of respiratory gases values in arterial blood samples. Conclusion. The Mounier-Kuhn syndrome is rare disorder, although diagnosis is often missed. Clinical presentation is similar to chronic obstructive pulmonary disease or bronchiectasis. Computed tomography is gold standard for diagnosis. Therapy is presumely supportive. tracheobronchomegaly diagnosis, differential tomography Medicine (General) Plavec Goran verfasserin aut Stojisavljević Marko verfasserin aut Tomić Ilija verfasserin aut Šarac Sanja verfasserin aut In Vojnosanitetski Pregled Military Health Department, Ministry of Defance, Serbia, 2012 67(2010), 10, Seite 867-870 (DE-627)474382613 (DE-600)2169819-3 24060720 nnns volume:67 year:2010 number:10 pages:867-870 https://doi.org/10.2298/VSP1010867M kostenfrei https://doaj.org/article/ccf9d0d306564ef3bc7b614cfdf65459 kostenfrei http://www.doiserbia.nb.rs/img/doi/0042-8450/2010/0042-84501010867M.pdf kostenfrei https://doaj.org/toc/0042-8450 Journal toc kostenfrei GBV_USEFLAG_A SYSFLAG_A GBV_DOAJ GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_702 GBV_ILN_2001 GBV_ILN_2003 GBV_ILN_2005 GBV_ILN_2006 GBV_ILN_2008 GBV_ILN_2009 GBV_ILN_2010 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2015 GBV_ILN_2020 GBV_ILN_2021 GBV_ILN_2025 GBV_ILN_2031 GBV_ILN_2044 GBV_ILN_2048 GBV_ILN_2050 GBV_ILN_2055 GBV_ILN_2056 GBV_ILN_2057 GBV_ILN_2061 GBV_ILN_2111 GBV_ILN_2153 GBV_ILN_2190 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 67 2010 10 867-870
language	English
source	In Vojnosanitetski Pregled 67(2010), 10, Seite 867-870 volume:67 year:2010 number:10 pages:867-870
sourceStr	In Vojnosanitetski Pregled 67(2010), 10, Seite 867-870 volume:67 year:2010 number:10 pages:867-870
format_phy_str_mv	Article
institution	findex.gbv.de
topic_facet	tracheobronchomegaly diagnosis, differential tomography Medicine (General)
isfreeaccess_bool	true
container_title	Vojnosanitetski Pregled
authorswithroles_txt_mv	Milić Rade @@aut@@ Plavec Goran @@aut@@ Stojisavljević Marko @@aut@@ Tomić Ilija @@aut@@ Šarac Sanja @@aut@@
publishDateDaySort_date	2010-01-01T00:00:00Z
hierarchy_top_id	474382613
id	DOAJ074863312
language_de	englisch
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Lung function tests showed mixed ventilation disorder, and disturbance of respiratory gases values in arterial blood samples. Conclusion. The Mounier-Kuhn syndrome is rare disorder, although diagnosis is often missed. Clinical presentation is similar to chronic obstructive pulmonary disease or bronchiectasis. Computed tomography is gold standard for diagnosis. 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callnumber-first	R - Medicine
author	Milić Rade
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topic_title	R5-920 The Mounier-Kuhn syndrome tracheobronchomegaly diagnosis, differential tomography
topic	misc R5-920 misc tracheobronchomegaly misc diagnosis, differential misc tomography misc Medicine (General)
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title	The Mounier-Kuhn syndrome
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title_full	The Mounier-Kuhn syndrome
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author_browse	Milić Rade Plavec Goran Stojisavljević Marko Tomić Ilija Šarac Sanja
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title_sort	mounier-kuhn syndrome
callnumber	R5-920
title_auth	The Mounier-Kuhn syndrome
abstract	Background. The Mounier-Kuhn syndrome (MKS) or tracheobronchomegaly (TBM) is a rare condition of unknown frequency, up to now about 100 cases have been reported. It presents by marked dilatation of the trachea and major bronchi, recurrent respiratory infections and consecutive bronchiectasis and scars in lung parenchyme. Sometimes enlargement of transversal colon may be present. Diagnosis is usually made radiologically. Cases report. We reviewed two patients 77 and 72 years old with typical clinical presentation and enlarged upper airways, in whom diagnosis of MKS was established by chest multislice computed tomography (MSCT). Transversal diameter of trachea was 30 mm in the first patient and 33 mm in the other one. Complications of syndrome (tracheal diverticulosis in the first patient, and pulmonary fibrosis, bulous emphysema and bronchiectasis in both patients) also were seen. Lung function tests showed mixed ventilation disorder, and disturbance of respiratory gases values in arterial blood samples. Conclusion. The Mounier-Kuhn syndrome is rare disorder, although diagnosis is often missed. Clinical presentation is similar to chronic obstructive pulmonary disease or bronchiectasis. Computed tomography is gold standard for diagnosis. Therapy is presumely supportive.
abstractGer	Background. The Mounier-Kuhn syndrome (MKS) or tracheobronchomegaly (TBM) is a rare condition of unknown frequency, up to now about 100 cases have been reported. It presents by marked dilatation of the trachea and major bronchi, recurrent respiratory infections and consecutive bronchiectasis and scars in lung parenchyme. Sometimes enlargement of transversal colon may be present. Diagnosis is usually made radiologically. Cases report. We reviewed two patients 77 and 72 years old with typical clinical presentation and enlarged upper airways, in whom diagnosis of MKS was established by chest multislice computed tomography (MSCT). Transversal diameter of trachea was 30 mm in the first patient and 33 mm in the other one. Complications of syndrome (tracheal diverticulosis in the first patient, and pulmonary fibrosis, bulous emphysema and bronchiectasis in both patients) also were seen. Lung function tests showed mixed ventilation disorder, and disturbance of respiratory gases values in arterial blood samples. Conclusion. The Mounier-Kuhn syndrome is rare disorder, although diagnosis is often missed. Clinical presentation is similar to chronic obstructive pulmonary disease or bronchiectasis. Computed tomography is gold standard for diagnosis. Therapy is presumely supportive.
abstract_unstemmed	Background. The Mounier-Kuhn syndrome (MKS) or tracheobronchomegaly (TBM) is a rare condition of unknown frequency, up to now about 100 cases have been reported. It presents by marked dilatation of the trachea and major bronchi, recurrent respiratory infections and consecutive bronchiectasis and scars in lung parenchyme. Sometimes enlargement of transversal colon may be present. Diagnosis is usually made radiologically. Cases report. We reviewed two patients 77 and 72 years old with typical clinical presentation and enlarged upper airways, in whom diagnosis of MKS was established by chest multislice computed tomography (MSCT). Transversal diameter of trachea was 30 mm in the first patient and 33 mm in the other one. Complications of syndrome (tracheal diverticulosis in the first patient, and pulmonary fibrosis, bulous emphysema and bronchiectasis in both patients) also were seen. Lung function tests showed mixed ventilation disorder, and disturbance of respiratory gases values in arterial blood samples. Conclusion. The Mounier-Kuhn syndrome is rare disorder, although diagnosis is often missed. Clinical presentation is similar to chronic obstructive pulmonary disease or bronchiectasis. Computed tomography is gold standard for diagnosis. Therapy is presumely supportive.
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title_short	The Mounier-Kuhn syndrome
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up_date	2024-07-04T00:53:34.368Z
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