The article describes the mechanisms of mucostasis and lung damage in cystic fibrosis. Types of mucolytic therapy and importance of inhalation therapy are considered. The authors presented their own classification of mucoactive agents. The principle of action, advantages and efficacy of mucoactive drugs based on the mannitol molecule are discussed based on the results of multicenter randomized clinical trials, including those conducted in children with cystic fibrosis. The authors present their own experience in the use of mannitol at the Morozovsky Municipal Children Clinical Hospital and 42 BIDA tests for its tolerability, of which 38 tests were passed satisfactorily. We present various clinical cases demonstrating the failure of a tolerance test due to increased signs of respiratory failure (dyspnea), an example of a repeat test 8 months later with satisfactory results, the development of hemoptysis in a patient seen in the pulmonology department of the National Medical Research Center for Children’s Health. The case was described in an adolescent, and after mannitol inhalation was added to the existing basic therapy, there was a clear positive trend in flowmetry: FEV1-74% (a significant increase of 14%). Thus, the authors noted that mannitol is effective in children with cystic fibrosis over 6 years of age; its use is characterized by good compliance and general adherence to therapy due to convenience of use: no need for additional technical means to perform inhalation in the form of solutions outside the home and saving overall free time. An important observation noted by the authors is that the BIDA test should be performed in complete clinical and laboratory remission to obtain objective results.
The aim of the program was to study the tolerability of Tigerase® in patients with cystic fibrosis (CF) of all ages in rutine clinical practice. Study design: retrospective open uncontrolled comparative multicenter solid. Materials and methods of research: retrospective data of clinical observations were collected from medical records of patients with CF on the use of Tigerase®. Results: therapy with Tigerase® was well tolerated by 668 (93,4%) of 715 patients included in the study. In 47 (6,6%) patients, 127 adverse reactions (ADRs) associated with the use of Tigerase® were recorded. ADRs from the respiratory system were the most common. Of these, 24 (3,4%) were coughing and 10 (1,4%) had increased viscosity of bronchial secretion. Among all patients included in the study, the proportion of patients in whom ADRs were registered based on clinical manifestations (3,9%) did not differ statistically significantly from the proportion of patients in whom ADRs were recorded based on complaints only (2,8%) (p=0,30). The distribution of ADRs by the source of registration and place of residence of patients did not depend on their gender and age. Registration of ADRs in different regions of the country differed statistically significantly both in frequency and in the source of detection (p<0,001). ADRs were not recorded in several regions, and the largest number of ADRs were registered in patients living in Moscow, and most of them were based only on patient complaints. 22 patients (47% of the number of patients with ADR) had medical commissions for ADR, and only in 8 (17% of the number of patients with ADR) of them had expertise of specialists with experience in the treatment of patients with CF. In 29 patients (62% of the number of patients with ADR), the development of ADR did not require cessation of the Tigerase® therapy. Conclusion: in the majority of CF patients (93,4%) tolerated the Tigerase® therapy well.
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