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Ultrastruct Pathol. 2005 Jan-Feb;29(1):3-8.

Ultrastructural patterns of primary ciliar dyskinesia syndrome.

Author information

1
Department of Pathology, Medical School, University of Valencia, Valencia, Spain.

Abstract

Clinical presentation, ciliary ultrastructure, and nasal mucociliary transport by a radioisotopic technique were analyzed in 14 Kartagener syndrome patients. In this study the most common pattern was the absence of outer and inner dynein arms in 57% of cases. Also reported are 14% patients with short inner dynein arms. A total of 29% of the patients showed normal dynein arms. Mucociliary stasis was observed in 13 cases. Primary ciliary dyskinesia syndrome and Kartagener syndrome are clinically homogeneous and morphologically heterogeneous. The authors conclude that a typical clinical presentation with an altered mucociliary transport obtained by radioisotopic technique is diagnostic although ciliary ultrastructure is normal.

PMID:
15931775
DOI:
10.1080/01913120490897538
[Indexed for MEDLINE]

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