Abstract
Inflammation of the respiratory tract in patients with cystic fibrosis (CF) occurs at the beginning of the disease, manifests itself even in the absence of infection. Therefore, the use of antibiotics is not sufficient to stop the inflammation. The accumulation of sputum with high content of DNA contributes to the development of a large amount of transforming growth factor (TGF-β). TGF-β is the mediator of fibrosis, the rate of disease progression and pulmonary complications.
The aim is to study the role of Dornase alpha in the prevention of pulmonary fibrosis in children with CF.
Methods: 47 children with CF were examined, among them patients without respiratory symptoms and with chronic bronchitis. TGF-β was determined in the endothelial tissue of the bronchi and in the blood serum.
Results: TGF-β in endothelial bronchial tissue was identified in the quarter of children. The intensity of expression of TGF-β was more pronounced in children without chronic bronchitis. This is a prognostic criterion for the development of “inadequate” pneumofibrosis. In children with positive TGF-β expression in the bronchial endothelium the level of this marker in the blood was significantly higher. It was found that the treatment with Dornase alpha for 4 weeks significantly lowered TGF-β levels in children by 12 % during the exacerbation of the disease.
Conclusion: The inclusion of Dornase alpha in the treatment of patients with CF should begin even in the absence of respiratory symptoms. Determination of serum TGF-β levels in children with CF can help predict the severity of the course and select an optimal therapeutic strategy that will slow down the progression of the disease.
Footnotes
Cite this article as: European Respiratory Journal 2019; 54: Suppl. 63, PA966.
This is an ERS International Congress abstract. No full-text version is available. Further material to accompany this abstract may be available at www.ers-education.org (ERS member access only).
- Copyright ©the authors 2019