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

A pilot study to determine the relationship of changes in the characteristics of β1- and β2-adrenoreceptors against the background of the use of β2-agonists with clinically significant parameters in patients with cardiovascular pathology

Background: Comorbid cardiovascular and bronchoobstructive diseases worsens disease course and prognosis and causes difficulties in therapy. Effectiveness 2-agonists (cornerstone of bronchoobstructive pathology treatment) depends on -adrenoreceptors state. Therefore, a detailed study of the functional state of beta-adrenoreceptors is necessary. Aim: to evaluate the relationship of changes in the characteristics of 1- and 2-adrenoreceptors, against the background of the use of 2-agonists in patients with cardiovascular and bronchoobstructive diseases with clinically significant parameters. Methods: In the pilot single-center prospective study during 2.5 years 45 patients (15 with cardiovascular and 30 with bronchoobstructive diseases) were included. Anamnestic data and complaints were collected and laboratory and instrumental studies were performed. Modified radioligand analysis on blood T-lymphocytes using radiolabeled Cyanopindolol and unlabeled selective ligands (ICI 118551, CGP 20712) before and after salbutamol and formoterol was performed and the binding activity of 1- and 2-adrenoreceptors was determined. Results: The correlation analysis between clinically significant parameters and changes in the specific binding index of 1- and 2-adrenoreceptors confirmed its clinical relevance. In test with beta-agonists in cardiovascular and bronchoobstructive diseases patients, an increase in the specific binding index of 1-adrenoreceptors is associated with clinical, laboratory and instrumental parameters of a favorable course of the disease, and an increase in the specific binding index of 2-adrenoreceptors is associated with negative. In the cardiovascular group without bronchoobstructive diseases with salbutamol an increase in the specific binding index of 1-adrenoreceptors was associated with parameters of unfavorable disease course, while an increase in the specific binding index of 2-adrenoreceptors did not have a clear associative relationship with clinical characteristics. Conclusions: The association of changes in the specific binding index of 1- and 2-adrenoreceptors in patients with cardiovascular and bronchoobstructive diseases with clinically significant parameters during acute tests with short- and long-acting 2-agonists was revealed, which in the future may provide an opportunity to identify patients with an unfavorable course of the disease

Correlation between Antioxidant Enzymes Activity and Intraerythrocyte Concentration of Fe, Mg, Zn, Cu in Pulmonary Arterial Hypertension and Cor Pulmonale in Children with Congenital Lung Disease and Cystic Fibrosis

Significant changes in the levels of the potential prooxidant Cu (increase) and the antioxidant Zn (decrease) in plasma were revealed in children having bronchopulmonary dysplasia (BPD) complicated by pulmonary arterial hypertension (PAH) and chronic cor pulmonale (CCP) when compared with the control. The Zn / Cu ratio in the blood plasma of patients with BPD, especially in CCP, was found to be lower than in the control group (p<0.001). This could indicate the activation of the prooxidant processes; simultaneously, the total antioxidant status (AOS) decreased. No significant increase in the intracellular free (“ionized” (i)) form of magnesium (iMg) was found; in fact, the concentration of iFe in all the patient groups was higher than in the control. An increase in the iCu and iZn levels (nonprotein-bound) was observed in the blood cells of the affected children. A significant increase in the glutathione peroxidase activity in the CCP patients may indicate an accumulation of organic peroxides, and partially compensate for the lesser activity of superoxide dismutase (SOD) and other antioxidants. The Zn / Cu and iZn/ iCu ratios were reduced in patients with CCP when compared with patients with PD without CCP