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Vol. 41. Issue 1.
Pages 11-15 (February 2005)
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Vol. 41. Issue 1.
Pages 11-15 (February 2005)
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DOI: 10.1157/13070275
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Estudio del transporte mucociliar y de la ultraestructura ciliar nasales en pacientes con síndrome de Kartagener
Study of Mucociliary Transport and Nasal Ciliary Ultrastructure in Patients With Kartagener's Syndrome
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M. Armengot Carcellera,
Corresponding author
miar@eresmas.net

Correspondencia: Dr. M. Armengot Carceller. Mediterrani, 33. 46134 Foios. Valencia. España.
, C. Carda Batallab, A. Escribanoc, G.J. Samperd
a Servicio de Otorrinolaringología. Hospital General Universitario. Facultad de Medicina. Valencia. España
b Departamento de Patología. Facultad de Medicina. Valencia. España
c Servicio de Pediatría. Hospital Clínico Universitario. Facultad de Medicina. Valencia. España
d Servicio de Neumología. Hospital General Universitario. Facultad de Medicina. Valencia. España
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Abstract
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Objetivo

El síndrome de Kartagener (SK) es una variante clínica de la discinesia ciliar primaria que asocia a las infecciones crónicas de las vías respiratorias un situs inversus. La ausencia de brazos de dineína ha sido el defecto ciliar asociado a este síndrome. El objeto de este trabajo es el estudio del transporte mucociliar y de la ultraestructura ciliar en 14 pacientes con SK.

Pacientes y métodos

Hemos estudiado el transporte mucociliar nasal, mediante una técnica radioisotópica, y la ultraestructura ciliar en 14 pacientes con SK.

Resultados

En 13 pacientes había estasis mucociliar y en uno, un transporte muy enlentecido (1,3 mm/min). Mostraban cilios con brazos de dineína normales 4 pacientes (29%); brazos internos de dineína cortos, 2 pacientes (14%), y ausencia completa de brazos internos y externos de dineína, 8 casos (57,1%).

Conclusiones

Concluimos que la presentación clínica típica junto con un transporte mucociliar alterado, objetivado con una técnica isotópica, es diagnóstica del SK, aunque la ultraestructura ciliar sea normal. El SK es clínicamente homogéneo y morfológicamente heterogéneo.

Palabras clave:
Discinesia ciliar primaria
Situs inversus
Función mucociliar
Objective

Kartagener's syndrome (KS) is a clinical variant of primary ciliary dyskinesia involving situs inversus associated with chronic airway infections. The ciliary defect associated with this syndrome is the absence of dynein arms. The aim of this study was to evaluate mucociliary transport and ciliary ultrastructure in 14 patients with KS.

Patients and methods

We studied nasal mucociliary transport using a radioisotopic technique and ciliary ultrastructure in 14 patients with KS.

Results

Thirteen patients had mucociliary stasis and 1 had severely slowed transport (1.3 mm/min). Four patients (29%) had cilia with normal dynein arms, 2 patients (14%) had short inner dynein arms, and 8 patients (57.1%) had total absence of inner and outer dynein arms.

Conclusions

We conclude that the typical clinical presentation, together with altered mucociliary transport as identified by an isotopic technique, is diagnostic of KS, even when the ciliary ultrastructure is normal. KS is clinically homogenous and morphologically heterogenous.

Key words:
Primary ciliary dysk inesia
Situs inversus
Mucociliary function
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Bibliografía
[1.]
M. Kartagener.
Zur pathologie der bronchiectasien bei situs viscerum inversus.
Beitrage Zur Klinic Der Tubekulose Und Spezifischen, 83 (1933), pp. 489-501
[2.]
R. Oeri.
Bronchiectasis in situs inversus.
Francfurter Zeitschrift fur Pathologie, 3 (1901), pp. 393-398
[3.]
A. Siewert.
Uber einen fall von bronchiectasie bei einen patienten mit situs inversus viscerum.
Berliner Klinische Wochenschrift, 41 (1904), pp. 139-141
[4.]
M. Armengot, C. Carda, J. Basterra.
Disquinesias mucociliares.
Tratado de otorrinolaringología y cirugía de cabeza y cuello, pp. 638-646
[5.]
B. Afzelius.
Situs inversus and ciliary abnormalities. What is the connection?.
Int J Dev Biol, 39 (1995), pp. 839-844
Medline
[6.]
P. Noone, M. Leigh, A. Sannuti, S. Minnix, J. Carson, M. Hazucha, et al.
Primary ciliary dyskinesia: diagnostic and phenotypic features.
Am J Respir Crit Care Med, 169 (2004), pp. 457-459
[7.]
N. Munro, D. Currie, K. Lindsay, T. Ryder, A. Rutman, A. Dewar, et al.
Fertility in men with primary ciliary dyskinesia presenting with respiratory infections.
Thorax, 49 (1994), pp. 684-687
Medline
[8.]
S. Halbert, D. Patton, P. Zarutskie, M. Soules.
Function and structure of cilia of the Fallopian tube of an infertile woman with Kartagener's syndrome.
Human Reprod, 12 (1997), pp. 55-58
[9.]
M. Greenstone, A. Rutman, A. Dewar, I. Mackay, P. Cole.
Primary ciliary dyskinesia: cytological and clinical features.
Q J Med, 67 (1988), pp. 405-430
Medline
[10.]
M. Armengot, C. Carda, J. Basterra.
Axonema ciliar incompleto: ¿otra causa de discinesia ciliar primaria?.
Acta Otorrinolaringol Esp, 49 (1998), pp. 57-59
Medline
[11.]
B. Afzelius.
A human syndrome caused by immotile cilia.
Science, 193 (1976), pp. 317-319
Medline
[12.]
M. Jorissen, B. Bertrand.
Ciliary dyskinesia in the nose and paranasal sinuses.
Acta Otorhinolaringol Belg, 51 (1997), pp. 353-366
[13.]
O. Bounty, D. Machillot, G. Bellon, B. Massonnet, C. Dumontel, G. Dutailly, et al.
Heterogeneite ultrastructurale dans le syndrome de dyskinesie ciliaire primitive.
Ann Pediatr (Paris), 37 (1990), pp. 432-436
[14.]
F. Herzon, S. Murphy.
Normal ciliary ultrastructure in children with Kartagener's syndrome.
Ann Otol, 89 (1983), pp. 81-83
[15.]
M. Greenstone, A. Dewar, P. Cole.
Ciliary dyskinesia with normal ultrastructure.
Thorax, 38 (1983), pp. 875-876
[16.]
M. Armengot, G. Juan, R. Barona, L. Garin, J. Basterra.
Immotile cilia syndrome: nasal mucociliary function and nasal ciliary abnormalities.
Rhinology, 32 (1994), pp. 109-111
Medline
[17.]
M. Armengot, G. Ruiz, C. Romero de Ávila, C. Carda, J. Basterra.
Utilidad diagnóstica del estudio del transporte mucociliar nasal con isótopos radiactivos en pacientes con infecciones respiratorias recidivantes.
Rev Esp Med Nucl, 17 (1998), pp. 21-26
Medline
[18.]
M. Armengot, J. Basterra, L. Garin.
Valores normales de aclaramiento mucociliar nasal. Comparación de diferentes técnicas y sustancias.
Acta Otorrinolaring Esp, 41 (1990), pp. 333-336
[19.]
M. Lurie, G. Rennert, S. Goldberg, J. Rivlin, E. Greenberg, I. Katz.
Ciliary ultrastructure in primary ciliary dyskinesia and other chronic conditions: the relevance of microtubular abnormalities.
Ultrastruct Pathology, 16 (1992), pp. 547-553
[20.]
M. Nielsen, M. Pedersen, B. Christensen, N. Mygind.
Blind quantitative electron microscopy of cilia from patients with primary ciliary dyskinesis and from normal subjects.
Eur J Respir Dis, 64 (1983), pp. 19-30
[21.]
C. Rossman.
Primary ciliary dyskinesia: evaluation and management.
Pediatr Pulmonol, 5 (1998), pp. 36-50
Medline
[22.]
M. Benninger.
Adult chronic rhinosinusitis: definitions, diagnosis, epidemiology and pathophysiology.
Otolaryngol Head Neck Surg, 129 (2003), pp. S1-S32
Medline
[23.]
W. Stannard, A. Rutman, C. Wallis, C. O’Callaghan.
Central microtubular agenesis causing primary ciliary dyskinesia.
Am J Respir Crit Care Med, 169 (2004), pp. 634-637
http://dx.doi.org/10.1164/rccm.200306-782OC | Medline
[24.]
B. Carlen, S. Lindberg, U. Stenram.
Absence of nexin links as a possible cause of primary ciliary dyskinesia.
Ultrastruct Pathol, 27 (2003), pp. 123-126
Medline
[25.]
M. Chilvers, A. Rutman, C. O’Callaghan.
Ciliary beat pattern is associated with specific ultrastructural defects in primary ciliary dyskinesia.
J Allergy Clin Immunol, 112 (2003), pp. 518-524
Medline
[26.]
M. Brauer, L. Viettro.
Aportes de la microscopía electrónica de transmisión al diagnóstico de la disquinesia ciliar.
Rev Med Uruguay, 19 (2003), pp. 140-148
[27.]
M. Sleigh.
Primary ciliary dyskinesia.
Lancet, 2 (1981), pp. 476
Medline
[28.]
J. Carson, A. Collier, S. Shih-Chin.
Acquired ciliary defects in nasal epithelium of children with acute viral upper respiratory infections.
N Engl J Med, 312 (1985), pp. 463-468
http://dx.doi.org/10.1056/NEJM198502213120802 | Medline
[29.]
B. Afzelius, P. Camner, B. Mossberg.
Acquired ciliary defects compared to those seen in the immotile cilia syndrome.
Eur J Respir Dis, 64 (1983), pp. 5-10
[30.]
S. Pizzi, S. Cazzato, F. Bernardi, W. Mantovani, G. Cenacchi.
Clinicopathological evaluation of ciliary dyskinesia: diagnostic role of electron microscopy.
Ultrastruct Pathol, 27 (2003), pp. 243-252
Medline
[31.]
M. Armengot, J. Basterra, J. Castillo, V. Marco.
Diagnóstico precoz del síndrome de inmotilidad ciliar mediante la técnica de la seroalbúmina marcada con Tc99m.
Acta Otorrinolaring Esp, 40 (1989), pp. 137-140
[32.]
M. Armengot, J. Basterra.
Nasal mucociliary function in the normal newborn.
Int J Pediatr Otorhinolaryngol, 22 (1991), pp. 109-113
Medline
Copyright © 2005. Sociedad Española de Neumología y Cirugía Torácica

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