Despite the manifestations of primary ciliary dyskinesia (PCD) in the neonatal period or in the first year of life, the diagnosis of this rare disease is usually established at the age of 4–7 years. The aim of the study was to search for reserves for early diagnosis of PCD. Materials and methods of research: 17 patients were observed with PCD, confirmed on the basis of the PICADAR scale, highspeed light video microscopy (all patients) and transmission electron microscopy (in 3 patients) of the mucous biopsy of the respiratory membrane using a genetic study (in one patient). Results: all patients were born full-term; neonatal respiratory distress syndrome (RDS) was observed in 71% of patients; the median duration of mechanical ventilation/oxygen therapy was 14 [7; 21] days; lateralization abnormalities were found in 35% of patients; all patients had persistent nasal congestion and/or rhinorrhea, 94% had chronic or recurrent sinusitis, otitis, recurrent pneumonia, chest x-ray and computed tomography (CT) scans in 71% had atelectasis with constant localization in the middle lobe, 12 of 15 patients, traced in the follow-up, – bronchiectasis (BE), also mainly in the middle lobe (in 9 patients). The average age at diagnosis was 5 [1.75; 7] years, patients with an established diagnosis over the age of 3 years were more often diagnosed with BE. The median of PICADAR scores was 7 [6; 8] points. Conclusions: to establish early diagnosis of PCD, it is important to consider neonatal RDS, lateralization of organs, difficulty in nasal breathing, starting from the first half of life.

The purpose of the study was to determine the predictors of severe bronchiectasis (BE) not associated with cystic fibrosis (CF) in children and to evaluate the effectiveness of a stepwise complex conservative therapy. Materials and methods of the study: study design - multicenter cohort prospective pilot study. 67 hospitalized pediatric patients aged from 11 months up to 17 years old (52% boys and 48% girls) with BE caused by previous pneumonia (22%), primary ciliary dyskinesia (22%), bronchial asthma (13%), Williams-Campbell syndrome (7%), bronchial foreign bodies (7%), gastroesophageal reflux disease (6%), bronchopulmonary dysplasia (6%), postinfectious bronchiolitis obliterans (5%), allergic bronchopulmonary aspergillosis (3%), chronic granulomatous disease (3%), AIDS (1%), protracted bacterial bronchitis (1%), and brain-lung-thyroid syndrome (1%) were observed. Predictors of severe BE were determined based on a comparison of groups of patients with mild (up to 4 exacerbations of BE per year) and moderate (from 4 to 6 exacerbations) course of BE (n=31) and patients with severe course of BE (>6 exacerbations per year, n=36). At the second stage of the study, in patients with changes in the severity of the course of BE, who were followed in dynamics (n=42), the frequency of exacerbations was assessed during the year after the appointment of a stepwise complex conservative therapy, which included, depending on the severity, daily drainage massage, exercise therapy, auxiliary devices for the respiratory tract clearance, long-term anti-inflammatory azithromycin, inhaled/intravenous antibiotics, and/or respiratory support. Results: the clinical picture of severe BE occurring with >6 exacerbations per year compared with mild/moderate course of the disease (≤6 exacerbations per year) is characterized by a statistically significantly more frequent registration of dyspnea (86% and 45%, p<0.001), exercise intolerance (69% and 39%, p=0.012), wet rales (89% and 61%, p=0.011), finger clubbing (17% and 0%, p=0.027). The prognostic model for determining the likelihood of severe BE not associated with CF in children includes the age of manifestation, a positive result of bacteriological examination of sputum/aspirates from the respiratory tract, localization of BE in the middle lobe of the right lung and/or lingual segments, and dyspnea. The appointment of gradual conservative therapy for BE, depending on the severity/frequency of exacerbations, makes it possible to statistically significantly reduce the severity of BE (p<0.001) and the median of exacerbations during the year from 9.00 [3.25-12.00] before treatment to 2.00 [1.00-3.00] after treatment (p<0.001). Conclusion: BEs are heterogeneous in severity, which determines the choice of therapy. Conservative therapy of BE not associated with CF in children has a stepwise principle depending on the severity of the course. Its appointment could therefore reduce the frequency of exacerbations.

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