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Войти в исследования Archivos de bronconeumologia · 2005
[Study of mucociliary transport and nasal ciliary ultrastructure in patients with Kartagener's syndrome].
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Armengot Carceller M, Carda Batalla C, Escribano A, Samper G J
Аннотация
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.
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.