Aim To evaluate the epidemiological data, the clinical profile and explorative characteristics in children with cystic fibrosis (CF) from the Republic of Moldova.
Methods At present in Moldova according to official statistical data there are about 120 children with CF. There are 50 children with a confirmed diagnosis of cystic fibrosis in the Clinic of Pneumology. This study included 21 boys and 19 girls. Genetic diagnosis was made in children and their parents for 4–7 CFTR mutations.
Results In 26 children (52%) the ΔF508 mutation was revealed (21 children; homozygous), 1 child, N1303K; 1 case, R334W. The diagnostic of CF was established at the age of <1 year in 24 children, 1–5 years in 13 children, 5–10 years in 6 cases, 10–15 years in 6 children and 16–20 years in 1 child. Bacteriology of sputum was characterized by predominance of S. aureus in 66.15%, Ps. aeruginosae in 37.2%, H. influenzae in 35.6% and mixed infection with S. aureus + Ps. aeruginosae in 25.4%, S. aureus + H. influenzae in 11.9% and S. aureus + Ps. aeruginosae + H. influenzae in 3.4% cases. Spiral computed tomography has revealed saccate and cylindrical bronchiectasis (10 children), focal (4 children) and diffuse pulmonary fibrosis in 3 cases, and signs of bronchiolitis (3 cases) and chronic bronchitis in 18 children.
Conclusion There is a predominance of the ΔF508 CFTR mutation in children with CF in Moldova. Bronchopulmonary affects are characterized by chronic infection with Ps. aeruginosae, S. aureus, H. influenzae, ventilatory function disorders, bronchial remodeling and sectors of pulmonary fibrosis.