Mannitol therapy for children with cystic fibrosis: efficacy and safety

Abstract

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.