[Study of mucociliary transport and nasal ciliary ultrastructure in patients with Kartagener's syndrome].
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.


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.
Armengot Carceller
M
M
Servicio de Otorrinolaringología, Hospital General Universitario, Facultad de Medicina, Valencia, Spain. miar@eresmas.net
Carda Batalla
C
C
Escribano
A
A
Samper
G J
GJ
D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D002923:Cilia / Q000648:ultrastructure; D005260:Female; D006801:Humans; D007223:Infant; D007619:Kartagener Syndrome / Q000473:pathology* / Q000503:physiopathology*; D008297:Male; D008875:Middle Aged; D009079:Mucociliary Clearance; D009666:Nose / Q000648:ultrastructure*