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Vol. 23. Issue 3.
Pages 136-138 (May - June 1987)
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Vol. 23. Issue 3.
Pages 136-138 (May - June 1987)
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Sindrome de disquinesia ciliar: a proposito de un caso
Ciliary dyskinesia syndrome: case report
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4762
F. Martin Santos, J. Ramos Gonzalez, V. Jimenez Montes, I. Zabalza Estevez, F. Vara Cuadrado
Hospital Santa Marina. Osakidetza. Servicio Vasco de Salud. Bilbao
J.J. Burgos Bretones*
* Departamento de Anatomía Patológica. Hospital de Cruces. Insalud. Baracaldo (Vizcaya)
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Se presenta el caso de una paciente de 27 años con situs inversus total y ausencia de bronquiectasias que, a lo largo de su vida, ha sufrido varios episodios infecciosos respiratorios, entre ellos dos neumonías demostradas radiológica y bacteriológicamente. El estudio ultraestructural de la mucosa tráqueobronquial con microscopía electrónica, permitió establecer el diagnóstico de síndrome de disquinesia ciliar, al estar presentes en los cilios de la mucosa las alteraciones estructurales que definen este síndrome. A propósito del mismo, los autores analizan los criterios básicos que le definen y, a tenor de los hallazgos habidos en el caso comunicado, se hace especial referencia al discutido tema de la naturaleza adquirida o congénita de las bronquiectasias que pueden estar presentes en el denominado síndrome de Kartagener.

We present a 27 years old female patient with total situs inversus without bronchiectasis who had previuosly developed several respiratory infections particularly two pneumonias confirmed by radiological and bacteriological examinations. The diagnosis of ciliary dyskinesia was established based on the findings of the ultrastructural examination by means of electron microscopy which showed the changes of mucose cilia that define this syndrome. Based on the findings of the case reported here, the definition criteria are reviewed and the controversial question of whether the bronchiectasis that may appear in the so call Kartagener syndrome are inborn or adquired is discussed.

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Bibliografía
[1.]
A.B. Novikoff, E. Holtzman.
Estructura y dinámica celular.
1.a ed, Nueva Editorial Interamericana, (1972), pp. 134143
[2.]
R. Elliason, B. Mossberg, P. Camner, B.A. Afzelius.
The immotile cilia syndrome. A congenital ciliary abnormality as an etiologic factor in chronic airway infections and male sterility.
N Engl J Med, 297 (1977), pp. 1-6
[3.]
C.M. Rossman, J.B. Forrest, R.M. Lee, M.T. Newhouse.
The dyskinetic cilia syndrome ciliary motility in the immotile cilia syndrome.
Chest, 78 (1980), pp. 580-582
[4.]
R.D. Miller, M.B. Divertie.
Kartagener's syndrome.
Chest, 62 (1972), pp. 130-135
[5.]
J.M. Sturgess, J. Chao, N. Aspin, P.J. Turner.
Cilia with defective radial spokes. A cause of human respiratory disease.
N Engl J Med, 300 (1979), pp. 53-56
[6.]
J.M. Sturgess, J. Chao, P.J. Turner.
Transposition of ciliary microtubules. Another cause of human impaired ciliary motility.
N Engl J Med, 303 (1980), pp. 318-322
[7.]
C.W. Corkey, H. Levison, J.A. Turner.
The immotile cilia syndrome. A longitudinal survey.
Am Rev Respir Dis, 124 (1981), pp. 544-548
Copyright © 1987. Sociedad Española de Neumología y Cirugía Torácica
Archivos de Bronconeumología
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