The System of Neutrophil Elastase and the Plasma Level of MMP-7 in Children with Pulmonary Arterial Hypertension and Chronic Cor Pulmonale

A significant increase in the activity of neutrophil elastase (NE) and anti-NE-protection in the plasma were detected in children having bronchopulmonary dysplasia (BPD) complicated by pulmonary arterial hypertension (PAH) and chronic cor pulmonale (CCP). The changes revealed were more pronounced in patients with CCP. The plasma concentration of the NE was slightly reduced, which was probably associated with the activation of anti-NE and an increase in the α1-antitrypsin level. A gradual increase was noted in the plasma level of the matrix metalloproteinase-7 (MMP-7) in patients with an increase in the severity of the condition. In patients with cystic fibrosis (with and without CCP), the pronounced increase in the MMP-7 level was observed. In patients with cystic fibrosis (CF), even without the additional complication with PAH and CCP, the MMP-7 level was significantly higher than in those with congenital broncho-pulmonary malformations (CBPM). The difference was increased in those patients with PAH and reached a maximum in those with CCP