BLOOD MATRIX METALLOPROTEINASES LEVELS IN CYSTIC FIBROSIS CHILDREN (TEN YEARS OBSERVATION)

Abstract

Introduction. Destructive fibrotic changes in lung tissue play a key role in the pathogenesis of cystic fibrosis (CF) in children. The development of pulmonary fibrosis may be caused by a violation of the pattern of matrix metalloproteinases (MMPs) and elevated production of profibrogenic growth factors (TGF-β1). Aim of the study. To compare the peculiarities of MMP patterns and transforming growth factor TGF-β1 with the data of the visualisation of airways features in cystic fibrosis (CF) children. Patients and Methods. The study included 80 inpatients aged of from 3 months to 18 years suffered from СF with the involvement of the lungs and digestive system observed for ten years. All patients were administered antibiotics (cefoperazone/sulbactam, ceftazidime, tienam, meropenem, amikacin) and inhalation (colisthmethate sodium, tobramycin) intravenously for a long time period. The reference group consisted of 16 children without pulmonary pathology. Blood serum concentrations of transforming growth factor-β1 (TGF-β1), matrix metalloproteinases (MMP-2, MMP-8, MMP-9) and tissue inhibitor of matrix metalloproteinases (TIMP-1) were determined by ELISA method. The morphological features of airways were evaluated by means of computer tomography (CT) with (GE Discovery CT750 HD). Results. In CF children patients blood serum MMP9 levels were significantly higher whereas TIMP-1 and MMP-2 appeared to be less than in children with intact airways. TGF-β1 levels in CF children were 9.8 times more than in cases from the reference group. CT data showed the pronounced changes in the airways structure as multiple bronchoectasias and pneumofibrosis. Conclusion. The revealed morphologic signs of the deterioration in airways’ structure in СF children patients can be related to the elevation of the rate of the fibrosis development due to the violation in the MMP and profibrogenic factors patterns and transforming growth factor TGF-β1.