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Diseases

Genetic and Rare Diseases Information Center (GARD)

Mounier-Kuhn syndrome


Other Names for this Disease
  • Congenital tracheobronchomegaly
  • Idiopathic tracheobronchomegaly
  • Mounier Kuhn syndrome
  • Mounier-Kühn syndrome
  • Tracheobronchomegaly
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Overview

Mounier-Kuhn syndrome is a lung disorder that causes the respiratory tract to dilate or enlarge. People with this condition develop frequent respiratory tract infections and recurrent cough. The condition can be diagnosed by lung function testsbronchoscopy, and a chest CT scan.[1] The cause of Mounier-Kuhn syndrome is unknown, although cigarette smoke and air pollutants may act as irritating factors. Some cases are associated with connective tissue diseases such as Ehlers-Danlos syndrome, Marfan syndrome, and cutis laxa and may be inherited.[2] Treatment typically involves chest physical therapy and antibiotics to treat infections.[1][2]
Last updated: 8/5/2015

References

  1. Celik B, Bilgin S, Yuksel C. Mounier-Kuhn Syndrome: A Rare Cause of Bronchial Dilation. Texas Heart Institute Journal. 2011; 38(2):194-196. http://www.ncbi.nlm.nih.gov/pmc/articles/pmid/21494536/. Accessed 8/5/2015.
  2. Cordier JF, Cottin V, Lazor R, Prevot G. Mounier-Kühn syndrome. Orphanet. April 2014; http://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=en&Expert=3347. Accessed 8/5/2015.
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In Depth Information

  • Online Mendelian Inheritance in Man (OMIM) is a catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine. 
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs.  Access to this database is free of charge.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Mounier-Kuhn syndrome. Click on the link to view a sample search on this topic.
Other Names for this Disease
  • Congenital tracheobronchomegaly
  • Idiopathic tracheobronchomegaly
  • Mounier Kuhn syndrome
  • Mounier-Kühn syndrome
  • Tracheobronchomegaly
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.