© Borgis - New Medicine 3/1999, s. 35-36
Hanna Mackiewicz, Anna Sinkiewicz, Stanisław Betlejewski
A case of kartagener´s syndrome in a child
Department of Otolaryngology, University of Medical Sciences, Bydgoszcz, Poland
Head of Department: Prof. Stanisław Betlejewski, M.D.
A case of a child with early diagnosed full symptomatic Kartagener´s syndrome is presented. The boy was hospitalized twice in the Otolaryngology Department of the University of Medical Sciences in Bydgoszcz because of disturbances in nose breathing, right otorrhea, and bilateral conductive hearing loss. The child underwent diagnostics and was eventually operated on. Nasopharyngoendoscopy, left paracentesis, and right antromastoidectomy with tympanoplasty were performed. No long-lasting improvement was obtained. The case is presented because of the rarity of Kartagener´s syndrome, and the treatment difficulties connected with a lack of ciliary transport.
Kartagener´s syndrome was described for the first time in 1933, with its characteristic diagnostic triad: chronic paranasal sinusitis, bronchiectasis and situs inversus of the viscera (2, 3, 4). The disease is genetically determined, inherited recessive autosomal, and included in Primary Ciliary Dyskinesis Syndrome - PCDS. The first papers concerning the structure and function of the cilia appeared in the fifties (Fawcett and Porter). In 1962 Satir described abnormal cilia for the first time, and in 1976 McDowell drew up a classification of cilia defects in which congenital anomalies were distinguished from acquired ones. Genetically - determined cilia defects usually concern several organs, and often coexist with hearing impairment and male infertility. Conversely acquired cilia anomalies usually concern some loci in the respiratory epithelium. In the seventies Pedersen and Rebe revealed that the cause of cilia immobilization in Kartagener´s syndrome is a lack of dynein brachia in its structure. Recently, disorders of neutrophil, monocyte and fibroblast chemotactic effects in Kartagener´s syndrome have also been described (3). These can be additional causes of frequent and serious infections. Up to now, 20 different congenital cilia anomalies have been defined. Kartagener´s syndrome can be diagnosed on the basis of a classic triad of symptoms. There are many diagnostic tests confirming mucociliary transport disorders e.g. the saccharose nose clearance test, and the mucociliary clearance test with the use of particles of radioaerosols inhaled by the patient. The evaluation of ciliary motion frequency is done by the use of the phase contrast or light microscope and material is sampled by means of a brush from the trachea or bronchi.
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1. Baum G.L. et al.: Mucociliary clearance from central airways in patients with excessive sputum production with and without primary ciliary dyskinesia. Chest, 1990, 98,3:608-12. 2. Gryczyńska D. i wsp.: Przyczynek do patogenezy zespołu Kartagenera. Otolaryng. Pol., 1979, 33, 4:449. 3. Pawlik J., Żebrak J.: Pierwotna dyskinezja rzęsek (w tym zespół Kartagenera). Klin. Pediatr., 1993, 4, 3:49-53. 4. Steiner I. i wsp.: Trzy przypadki zespołu Kartagenera u dzieci. Otolaryng. Pol., 1998, 52, 5:601-3.