Portuguese Version

Year:  1998  Vol. 64   Ed. 5 - ()

Artigos Originais

Pages: 444 to 449

Primary Ciliary Dyskinesia in Patients with Repeated Upper Airvay Infections.

Author(s): Aracy P. S. Balbaní* ,
Silvio A. M. Marone** ,
Ossamu Butugan***;
Paulo H. N. Saldiva****.

Keywords: primary ciliary dyskinesia; Kartagener's syndrome; rhinitis; sinusitis; secretory otitis media

Abstract:
The aim of this study is to evaluate eight cases of recurrent infections of the upper airways compatible with the diagnosis of primary ciliary dyskinesia. lmmunodeficiencies and cystic fibrosis were excluded. There were five men and three women whose ages ranged between 3 and 30 years-old (mean 15.3 years), and 50% of the patients have presented family history of relation by blood. Purulent nasal discharge was seen in 100% of the patients, secretory otitis media was diagnosed in 50% and chronic otitis media in 37.5% of cases. Paranasal sinus disease was diagnosed in all the patients after plain X-ray evaluation. Phase contrast optic microscopy revealed absence of ciliary beat in three cases, and electron transmission microscopy diagnosed ultrastructural ciliary deffects in other three patients (lack of dynein arms and displaeement of central microtubuli). The autoors conclude that assessment of ciliary beat frequency and ciliary ultrastructure are of paramount importance for the diagnosis of the disease.

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