Pediatr. praxi. 2010;11(5):295-297

Approach to patients with primary ciliary dyskinesia

MUDr.Petr Papoušek¹, MUDr.Vladimír Koblížek², MUDr.Tereza Dobešová², MUDr.Helena Hornychová, Ph.D.³, doc.MUDr.František Salajka, CSc.²

¹ Transfuzní oddělení, Fakultní nemocnice v Hradci Králové

² Plicní klinika, Fakultní nemocnice v Hradci Králové

³ Fingerlandův ústav patologie, Fakultní nemocnice v Hradci Králové

Primary ciliary dyskinesia is a rare genetic disease, which is underdiagnosed by us, because it mimics more frequent respiratory illnesses

with its symptoms and because special ciliary tests were not accessible by us. It is manifested mostly by chronic rhinosinusitis,

epitympanitis and neonatal respiratory difficulties. One half of patients has situs viscerum inversus. Cough is productive in PCD, but

breathlessness belongs more likely to the manifestation of COPD. A nasal saccharine test, high-speed digital videomicroscopy, electron

microscopy of a sample taken from a nose and the analysis of exhaled NO belong to special ciliary tests. This particular PCD diagnostics

has also already been available in the Czech Republic in the past years. Plain chest X-ray, X-ray of the paranasal sinuses, audiometry,

tympanometry and pulmonary function tests have its role in the algorithm. Mucociliary clearance is increased by physiotherapy. Acute

infectious exacerbation is treated with antibiotics, nebulization of salts, irrigation in sinusitis, topical application of nasal corticoids and

functional endonasal surgery eventually. By timely treatment we can prevent the impairment of pulmonary functions.

Keywords: primary ciliary dyskinesia, digital high-speed videomicroscopy, NO, rhinosinusitis, situs viscerum inversus

Published: October 15, 2010  Show citation