Study of Mucociliary Transport and Nasal Ciliary Ultrastructure in Patients With Kartagener's Syndrome

Armengot Carceller, M.; Carda Batalla, C.; Escribano, A.; Samper, G.J.

doi:10.1016/S1579-2129(06)60388-1

Archivos de Bronconeumología

ISSN: 0300-2896

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Vol. 41. Issue 1.

Pages 11-15 (January 2005)

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Pages 11-15 (January 2005)

Original Articles

DOI: 10.1016/S1579-2129(06)60388-1

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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

Correspondence: 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, Spain

b Departamento de Patología, Facultad de Medicina, Valencia, Spain

c Servicio de Pediatría, Hospital Clínico Universitario, Facultad de Medicina, Valencia, Spain

d Servicio de Neumología, Hospital General Universitario, Facultad de Medicina, Valencia, Spain

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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 dyskinesia

Situs inversus

Mucociliary function

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

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