1966
DOI: 10.1016/s0003-4975(10)66538-1
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Tracheobronchiomegaly

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Cited by 52 publications
(13 citation statements)
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“…Tracheobronchomegaly is characterized by severe dilatation of the trachea and main bronchi and is believed to be due to degeneration of elastic tissue in the trachea (Hasleton, 1996). A rare congenital form of tracheobronchomegaly (Mounier-Kuhn syndrome) has been reported in children with cutis laxa (Aaby and Blake, 1966;Wanderer et al, 1969), and in association with Marfan syndrome (Shivaram et al, 1990)-both of these conditions are genetic disorders of elastin synthesis. Finally, the effect of elastin degeneration with advancing age may be important.…”
Section: Discussion Elastic Fibers In the Trachea And Bronchimentioning
confidence: 99%
“…Tracheobronchomegaly is characterized by severe dilatation of the trachea and main bronchi and is believed to be due to degeneration of elastic tissue in the trachea (Hasleton, 1996). A rare congenital form of tracheobronchomegaly (Mounier-Kuhn syndrome) has been reported in children with cutis laxa (Aaby and Blake, 1966;Wanderer et al, 1969), and in association with Marfan syndrome (Shivaram et al, 1990)-both of these conditions are genetic disorders of elastin synthesis. Finally, the effect of elastin degeneration with advancing age may be important.…”
Section: Discussion Elastic Fibers In the Trachea And Bronchimentioning
confidence: 99%
“…It has been suggested that a congenital con nective tissue defect could be responsible [9]. This is corroborated by the occasional association of TBM with Ehler-Danlos syndrome [10] and cutis laxa [11]. Other authors consider TBM an acquired disease [12,13], Parris and Johnson [13] presented a case of an adult TBM secondary to intensive radiotherapy.…”
Section: Discussionmentioning
confidence: 98%
“…The aetiology of this rare syndrome is not clearly understood, although associations with cutis laxa in children [2], Ehlers-Danlos syndrome in adults [3], occurrence in two siblings [4] and absence of tracheobronchial muscle fibres and myenteric plexus [5] suggest that a developmental or congenital connective tissue defect is responsible for this disease. However, presentation of tracheobronchomegaly in the third or fourth decade of life and the absence of a connective tissue disorder in most cases favour an acquired cause.…”
Section: Discussionmentioning
confidence: 99%