Abstract：
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.
journal_name
Arch Bronconeumoljournal_title
Archivos de bronconeumologiaauthors
Armengot Carceller M,Carda Batalla C,Escribano A,Samper GJdoi
10.1016/s1579-2129(06)60388-1keywords:
subject
Has Abstractpub_date
2005-01-01 00:00:00pages
11-5issue
1eissn
0300-2896issn
1579-2129pii
13070275journal_volume
41pub_type
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journal_title：Archivos de bronconeumologia
pub_type： 杂志文章
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journal_title：Archivos de bronconeumologia
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journal_title：Archivos de bronconeumologia
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journal_title：Archivos de bronconeumologia
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journal_title：Archivos de bronconeumologia
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journal_title：Archivos de bronconeumologia
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journal_title：Archivos de bronconeumologia
pub_type： 杂志文章,评审
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更新日期：1994-10-01 00:00:00
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pub_type： 杂志文章,多中心研究
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pub_type： 杂志文章,评审
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pub_type： 杂志文章,多中心研究
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pub_type： 杂志文章,评审
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pub_type： 杂志文章
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pub_type： 杂志文章,评审
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pub_type： 杂志文章,评审
doi：10.1016/j.arbres.2010.10.001
更新日期：2010-12-01 00:00:00
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journal_title：Archivos de bronconeumologia
pub_type： 杂志文章
doi：
更新日期：2008-04-01 00:00:00
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