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Congenital tracheobronchomegaly

MedGen UID:
11871
Concept ID:
C0040587
Disease or Syndrome
Synonyms: Mounier-Kuhn syndrome; Tracheobronchomegaly
SNOMED CT: Mounier-Kuhn syndrome (57451009); Tracheobronchomegaly (57451009)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Monarch Initiative: MONDO:0010148
OMIM®: 275300
Orphanet: ORPHA3347

Definition

Tracheobronchiomegaly is characterized by striking dilatation of the intrathoracic trachea and of the major bronchi (summary by Johnston and Green, 1965). [from OMIM]

Clinical features

From HPO
Bronchiectasis
MedGen UID:
14234
Concept ID:
C0006267
Disease or Syndrome
Persistent abnormal dilatation of the bronchi owing to localized and irreversible destruction and widening of the large airways.
Diverticulosis of trachea
MedGen UID:
573615
Concept ID:
C0340213
Disease or Syndrome
Tracheal diverticula are blind-ended outpouchings arising from the trachea. They are usually an incidental finding. Occasionally they can mimic pneumomediastinum. Tracheal diverticula are either congenital or acquired, in the latter case the most common causes are prolonged increased intraluminal pressure, e.g. chronic cough, COPD, tracheomalacia, and iatrogenic (e.g. post-surgical).
Recurrent bronchopulmonary infections
MedGen UID:
488990
Concept ID:
C2169795
Finding
An increased susceptibility to bronchopulmonary infections as manifested by a history of recurrent bronchopulmonary infections.

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVCongenital tracheobronchomegaly
Follow this link to review classifications for Congenital tracheobronchomegaly in Orphanet.

Recent clinical studies

Clinical prediction guides

Simon M, Vremaroiu P, Andrei F
J Bronchology Interv Pulmonol 2014 Apr;21(2):145-9. doi: 10.1097/LBR.0000000000000068. PMID: 24739689

Recent systematic reviews

Krustins E, Kravale Z, Buls A
Respir Med 2013 Dec;107(12):1822-8. Epub 2013 Sep 6 doi: 10.1016/j.rmed.2013.08.042. PMID: 24070565

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