Aim is a comparative analysis of clinical, laboratory and instrumental characterization of patients with and without Kartagener’s syndrome.

Material and Method The data of 157 patients with suspected PCD were studied and divided into two groups: Group I 60 (38.2%) patients with Kartagener’s syndrome, Group II 97 (62.8%) patients without Kartagener’s syndrome. Anamnestic method, instrumental methods, microbiological examination of the respiratory tract, transmission electron microscopy of the ciliated epithelium were used in the examination, DNA diagnostics.

Results Groups of patients did not differ by age (14.3 ± 10.3 and 14.9 ± 11.4, respectively). The patients‘ history was dominated by recurrent bronchitis in 53 (88.3%) with Kartagener’s syndrome and 91 (93.8%) without the syndrome, pneumonias (46 (76.7%) and 75 (77.2%)), sinusitis (45 (75%) and 77 (79.4%)), and otitis media in 30 (50.0%) and 55 (56.7%), respectively. Hearing loss was significantly more common in 26.8% of patients without Kartagener syndrome and 13.3% with Kartagener syndrome. Intensive care unit in the neonatal period was hospitalized 46.7% of group I patients more frequently compared to 28.9% of group II patients (p=0.024), heart defect were more frequent in 28.3% and 13.4%, respectively (p=0.021). According to PICADAR scale, more than 5 points were noted in 95% of group I patients and 37.1% of group II patients (p=0.001). When evaluating chest CT, 48.5% of patients with Kartagener’s syndrome had fibroatelectasis in the left lung compared to 28.9% of patients without Kartagener’s syndrome (p=0.04). In microbiologic cultures, Pseudomonas aeruginosa was prevalent in two groups (32.5% with Kartagener’s syndrome, and in 30.6% without the syndrome). In 60% of patients with Kartagener syndrome, the most frequent genetic variant was DNAH5, which is characterized by defects in the outer and inner dynein arms of the ciliated epithelium.

Conclusions In the study found that the problems of timely diagnosis, effective management and adequate treatment remain relevant for Russian patients.