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In the article, the authors provided the overview of the results of international clinical studies and recent papers, as well as their own experience with the use of an enzyme mucolytic – dornase alpha in the treatment of cystic fibrosis and other chronic bronchitis affected by mucostasis. The dornase alfa has been shown to have two more non-mucolytic effects, the most important of them is anti-inflammatory one. It is the anti-inflammatory effect that plays an important role in patients with cystic fibrosis, when chronic bacterial inflammation and destruction of lung tissue take place in the pathogenesis of the disease. Dornase alpha counteracts neutrophil elastase, reducing its concentration in sputum. The authors emphasize on the fact that the same inflammatory reactions occur in covid infection and that it is dornase alfa that acts with an anti-inflammatory effect in ordinary patients, as well as in cystic fibrosis. The authors share their own experience and bring three clinical examples that demonstrate not only good tolerability and safety, but also the clinical efficacy of dornase alfa (Tigerase): in a young child when a diagnosis was established, in a teenager with a successful transition to a biosimilar after a long-term administration, and in a teenager with mucoviscidosis, who successfully managed a covid infection during therapy with this drug. The authors emphasize that dornaza alfa is now the main component of the background therapy of patients with mucoviscidosis, and its representative Tigerase may be successfully used in variously-aged children.
In the article, the authors provided the overview of the results of international clinical studies and recent papers, as well as their own experience with the use of an enzyme mucolytic – dornase alpha in the treatment of cystic fibrosis and other chronic bronchitis affected by mucostasis. The dornase alfa has been shown to have two more non-mucolytic effects, the most important of them is anti-inflammatory one. It is the anti-inflammatory effect that plays an important role in patients with cystic fibrosis, when chronic bacterial inflammation and destruction of lung tissue take place in the pathogenesis of the disease. Dornase alpha counteracts neutrophil elastase, reducing its concentration in sputum. The authors emphasize on the fact that the same inflammatory reactions occur in covid infection and that it is dornase alfa that acts with an anti-inflammatory effect in ordinary patients, as well as in cystic fibrosis. The authors share their own experience and bring three clinical examples that demonstrate not only good tolerability and safety, but also the clinical efficacy of dornase alfa (Tigerase): in a young child when a diagnosis was established, in a teenager with a successful transition to a biosimilar after a long-term administration, and in a teenager with mucoviscidosis, who successfully managed a covid infection during therapy with this drug. The authors emphasize that dornaza alfa is now the main component of the background therapy of patients with mucoviscidosis, and its representative Tigerase may be successfully used in variously-aged children.
The article describes the features of mucolytic therapy in patients with cystic fibrosis, as the leading pathogenetic mechanism of the disease lies in the compromised mucociliary transport due to the presence of viscous secretions. Particular attention is paid to the key mucolytic drug (endonuclear mucolytic) dornase alfa, which has three clinical effects: powerful mucolytic, anti-inflammatory and antibacterial. The extracellular DNA cleavage in secretions results in a decrease of bronchial mucus viscosity, and a decrease in elastase and IL-8 concentrations in sputum results in the reduction of lung tissue destruction in chronic bacterial inflammation. The authors pay attention to the adherence to the drug policy, possible dosing regimens and drug use variants to achieve a clinical effect. Also, the article extensively covers the results of international clinical studies on the use of dornase alfa in cystic fibrosis. The authors presented their own clinical observation – the experience of clinical and functional control over the efficacy of the drug when comparing two groups of children with cystic fibrosis: those who received (2018–2021) and those who did not receive dornase alfa (1995–1998 – before it was introduced into clinical practice). The remarkable thing is that the results of FEV1, MEF 50, MEF 75, PEF were significantly higher in the group of modern children with cystic fibrosis, who receive dornase alfa continuously as part of the backbone therapy, than in children of the same age 25 years ago. It has been substantiated that dornase alfa is one of the main components of the backbone therapy for patients with cystic fibrosis, which should be prescribed on an ongoing basis after the diagnosis has been established, under the functional control of its efficacy
Introduction. Cystic fibrosis is a hereditary disease characterized by the defeat of all exocrine glands, vital organs and systems. Currently, the life expectancy of patients with cystic fibrosis is increasing due to the development of new therapies.Goal. Analysis of the results of a comprehensive examination of patients with cystic fibrosis under the supervision of a pulmonologist of Republican Clinical Hospital (RCH) of the Ministry of Health of the Republic of Tatarstan.Materials and methods. The analysis of the database of patients with cystic fibrosis, older than 18 years, registered in the Regional Center of cystic fibrosis of the Republic of Tatarstan (RT).Results. As of April 2023, 47 patients over the age of 18 with a diagnosis of cystic fibrosis were registered in the RCH of the Ministry of Health of the Republic of Tatarstan. The diagnosis was made on the basis of a positive sweat sample and/or a genetic study. The age of patients is from 18 to 39 years (26.1 ± 1.1 years). Women – 49%, men – 51%. Median body weight – 51.5 kg, height – 165 cm, BMI – 18.05 kg/m2. 25 patients (53.2%) had a BMI of less than 18.5 kg/m2. Among patients in RT, the most common mutation is F508del. When assessing the microbiological profile of the respiratory tract, chronic infection was detected in 37 patients (78.7%). Ps. aeruginosa (66.0%) and S.aureus (35.6%) were the most frequently sown. The average FVC indicators were 72.6 ± 4.3% predicted values, the average FEV1 indicators were 61.6 ± 28.1% predicted values.Conclusion. On the territory of the Republic of Tatarstan in patients with cystic fibrosis, the F508del mutation was detected in 30 (66.7%) adult patients, 9 of whom receive targeted therapy elecsacaftor / tezacaftor / ivacaftor+ ivacaftor with a pronounced positive effect. The high incidence of P. aeruginosa infection (66.0%) and a decrease in respiratory function among adult patients indicate a more severe course of cystic fibrosis.
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