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Primary tracheobronchial amyloidosis associated with tracheobronchomegaly evaluated by novel four‐dimensional functional CT

Amyloid is a heterogeneous family of extracellular proteinaceous deposits characterized by apple‐green birefringence on polarized light microscopy. There are rare case reports of these extracellular deposits accumulating in the upper and central airways. Progressive infiltration may impair glottic a...

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Autores principales: Uddin, A.K.M. Nizam, Mansfield, Darren R., Farmer, Michael W., Lau, Kenneth K.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: John Wiley and Sons Inc. 2015
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4694593/
https://www.ncbi.nlm.nih.gov/pubmed/26740884
http://dx.doi.org/10.1002/rcr2.134
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author Uddin, A.K.M. Nizam
Mansfield, Darren R.
Farmer, Michael W.
Lau, Kenneth K.
author_facet Uddin, A.K.M. Nizam
Mansfield, Darren R.
Farmer, Michael W.
Lau, Kenneth K.
author_sort Uddin, A.K.M. Nizam
collection PubMed
description Amyloid is a heterogeneous family of extracellular proteinaceous deposits characterized by apple‐green birefringence on polarized light microscopy. There are rare case reports of these extracellular deposits accumulating in the upper and central airways. Progressive infiltration may impair glottic and airway function with some cases requiring intervention to improve flow. Bronchoscopy and lung function testing provide dynamic information to monitor for disease progression; however, the recent development of 320 multislice computed tomography (320 CT) enables dynamic, four‐dimensional (4‐D) evaluation of laryngeal and tracheal structure and function and presents as a noninvasive, low‐radiation dose surveillance tool. We reviewed a 43‐year‐old man with primary amyloidosis of the larynx and central airways who presented with an 18‐year history of progressive dysphonia without breathlessness and preserved lung function. 4‐D CT demonstrated marked thickening of supraglottic folds and trachea with marked tracheal dilatation. Despite gross structural abnormalities, dynamic function assessed throughout inspiration and expiration was normal, demonstrating neither rigidity nor dynamic collapse. This combination of structural and functional assessment of the proximal airway by 4‐D CT is a novel application to surveillance for laryngeal and tracheal amyloid.
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spelling pubmed-46945932016-01-06 Primary tracheobronchial amyloidosis associated with tracheobronchomegaly evaluated by novel four‐dimensional functional CT Uddin, A.K.M. Nizam Mansfield, Darren R. Farmer, Michael W. Lau, Kenneth K. Respirol Case Rep Case Reports Amyloid is a heterogeneous family of extracellular proteinaceous deposits characterized by apple‐green birefringence on polarized light microscopy. There are rare case reports of these extracellular deposits accumulating in the upper and central airways. Progressive infiltration may impair glottic and airway function with some cases requiring intervention to improve flow. Bronchoscopy and lung function testing provide dynamic information to monitor for disease progression; however, the recent development of 320 multislice computed tomography (320 CT) enables dynamic, four‐dimensional (4‐D) evaluation of laryngeal and tracheal structure and function and presents as a noninvasive, low‐radiation dose surveillance tool. We reviewed a 43‐year‐old man with primary amyloidosis of the larynx and central airways who presented with an 18‐year history of progressive dysphonia without breathlessness and preserved lung function. 4‐D CT demonstrated marked thickening of supraglottic folds and trachea with marked tracheal dilatation. Despite gross structural abnormalities, dynamic function assessed throughout inspiration and expiration was normal, demonstrating neither rigidity nor dynamic collapse. This combination of structural and functional assessment of the proximal airway by 4‐D CT is a novel application to surveillance for laryngeal and tracheal amyloid. John Wiley and Sons Inc. 2015-11-06 /pmc/articles/PMC4694593/ /pubmed/26740884 http://dx.doi.org/10.1002/rcr2.134 Text en © 2015 The Authors. Respirology Case Reports published by John Wiley & Sons Ltd on behalf of The Asian Pacific Society of Respirology. This is an open access article under the terms of the Creative Commons Attribution‐NonCommercial (http://creativecommons.org/licenses/by-nc/4.0/) License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited and is not used for commercial purposes.
spellingShingle Case Reports
Uddin, A.K.M. Nizam
Mansfield, Darren R.
Farmer, Michael W.
Lau, Kenneth K.
Primary tracheobronchial amyloidosis associated with tracheobronchomegaly evaluated by novel four‐dimensional functional CT
title Primary tracheobronchial amyloidosis associated with tracheobronchomegaly evaluated by novel four‐dimensional functional CT
title_full Primary tracheobronchial amyloidosis associated with tracheobronchomegaly evaluated by novel four‐dimensional functional CT
title_fullStr Primary tracheobronchial amyloidosis associated with tracheobronchomegaly evaluated by novel four‐dimensional functional CT
title_full_unstemmed Primary tracheobronchial amyloidosis associated with tracheobronchomegaly evaluated by novel four‐dimensional functional CT
title_short Primary tracheobronchial amyloidosis associated with tracheobronchomegaly evaluated by novel four‐dimensional functional CT
title_sort primary tracheobronchial amyloidosis associated with tracheobronchomegaly evaluated by novel four‐dimensional functional ct
topic Case Reports
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4694593/
https://www.ncbi.nlm.nih.gov/pubmed/26740884
http://dx.doi.org/10.1002/rcr2.134
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