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Mounier-Kühn syndrome

Disease definition

Mounier-Kühn syndrome, also known as idiopathic tracheobronchomegaly, is a congenital disorder characterized by marked dilatation of the trachea and proximal bronchi that leads to impaired airway secretion clearance and recurrent lower respiratory tract infections.

ORPHA:3347

  • Synonym(s):
    • Congenital tracheobronchomegaly
    • Idiopathic tracheobronchomegaly
    • Tracheobronchomegaly
  • Prevalence: Unknown
  • Inheritance: Not applicable 
  • Age of onset: All ages
  • ICD-10: J98.0
  • OMIM: 275300
  • UMLS: C0040587  C2713583
  • MeSH: D014137
  • GARD: 3793
  • MedDRA: 10044316

Detailed information

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