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Vol. 30. Issue 10.
Pages 508-510 (December 1994)
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Vol. 30. Issue 10.
Pages 508-510 (December 1994)
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Traqueobroncomegalia asociada a enfermedad por depósito de cadenas ligeras
Tracheobronchiomegaly associated with light chain deposition disease
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O. Miró1, J. Fernández-Solá, E. Gómez-Angelats, M.V. Andreu
Servicios de Medicina Interna General, Hospital Clínic i Provincial. Barcelona
M. Solé*
* Servicios de Anatomía Patológica. Hospital Clínic i Provincial. Barcelona
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La traqueobroncomegalia o síndrome de Mounier-Kühn se caracteriza por la marcada dilatación de la tráquea y de los bronquios principales, y ha sido referida asociada a diferentes enfermedades, especialmente del tejido conectivo. La enfermedad por depósito de cadenas ligeras tiene una patogenia y unas manifestaciones clínicas similares a la amiloidosis de cadenas ligeras, en la cual el depósito de estas cadenas se realiza en forma de material amorfo y no con las características tintoriales del amiloide. Se presenta un caso de asociación de ambas entidades, hecho que no había sido referido previamente, y se realiza una revisión de las principales características de estas dos enfermedades.

Palabras clave:
Traqueobroncomegalia
Enfermedad por depósito de cadenas ligeras
Biopsia traqueal

Tracheobronchiomegaly, also known as Mounier-Kühn's Syndrome, is characterized by market dilation of the trachea and main bronchi and has been reported in association with several conditions, particularly connective tissue disease. The pathogenesis and clinical signs of light chain deposition disease are similar to those of light chain amyloidosis, in which these chains are deposited as amorphous material lacking the tinctorial features of the amyloid. We present a case involving both entities, an association that has not been previously reported, and we review the main characteristics of both diseases.

Key words:
Tracheobronchiomegalia
Light chain deposition disease
Tracheal biopsy
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Copyright © 1994. Sociedad Española de Neumología y Cirugía Torácica
Archivos de Bronconeumología
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