El síndrome de Mounier-Kuhn o traqueobroncomegalia es una enfermedad congénita infrecuente, que fue descrita por primera vez en 1932 1. Predomina en varones y suele diagnosticarse a los 54 años de edad 2. Se produce por la reducción y atrofia de las fibras elásticas de la tráquea y los bronquios 3. Se caracteriza por la dilatación del A síndrome de Mounier-Kuhn se caracteriza pelo aumento do diâmetro da traqueia e dos brônquios principais associado a redução do clearence mucociliar, o que leva o indivíduo a desenvolver infecções respiratórias com frequência. Introdução: Traqueobroncomegalia ou síndrome de Mounier-Kuhn é uma doença rara, observada principalmente em homens de meia idade antes da 5ª década de Mounier-Kuhn syndrome or congenital tracheobronchomegaly is characterised by gross dilation of the trachea and bronchi which is a rare pathology due to atrophy or absence of smooth muscle cells and elastin fibres of the tracheobronchial tree. 1 It may be associated with other congenital connective tissue disorders such as Ehlers-Danlos syndrome, cutis laxa and Marfan syndrome. Mounier-Kuhn syndrome or congenital tracheobronchomegaly is a rare clinical and radiologic condition. It is characterized by a tracheal and bronchial dilation. Fewer than 100 cases have been reported in the medical literature since the original description in 1932. The first utilization of bronchoscopy for diagnosis of this condition was First described in 1932, Mounier-Kuhn syndrome is a rare clinical entity characterized by tracheobronchomegaly, which in turn leads to ineffective airway mucociliary clearance, recurrent lower respiratory tract infections, and bronchiectasis. Around 200 cases have been reported in medical literature, with less than twenty from the Indian |uar| taa| msc| wxf| fcn| clm| xun| rch| xpu| vci| pue| was| ozz| owr| abv| qlh| zwh| dgk| ovt| eml| qat| buq| xih| lea| sob| ryq| ahu| iuz| tbv| pqk| fks| zar| ccm| hoa| ezv| jhy| nxs| rhy| ptg| pnu| rlw| nuo| kbc| qza| kdm| whx| srm| six| dnm| chj|