Review of clinical guidelines “Cystic fibrosis”, 2020

Abstract

The aim of the experts who provide health care for patients with cystic fibrosis (CF) has been to develop clinical guidelines on cystic fibrosis for children and adults.Methods. The 2020 Clinical Guidelines were based on the European consensus documents on cystic fibrosis and the national consensus “Cystic fibrosis: definition, diagnostic criteria, therapy” (2016, 2019). When creating clinical guidelines, the experts were guided by Article 2 of the Federal Law No.323-FL “On the basics of health care in the Russian Federation” as of November 21st, 2011, and the regulatory legal documents governing the development and approval of clinical guidelines (https://rosmedex.ru/kr/).Results. The clinical guidelines were approved by the Federal State Budgetary Institution “Centre for Expertise and Quality Control of Medical Care” of the Russian Ministry of Health in August 2020. Standards of medical care for children and adults with cystic fibrosis were prepared based on these guidelines. The diagnostic algorithms have been expanded with a three-stage DNA diagnostic strategy, functional assays of CFTR channel, laboratory and instrumental diagnostic tools for assessing disorders of respiratory and other systems, diagnosis of the microbial inflammatory process, microbiological monitoring, and therapeutic approaches. The antibiotic therapy with dosages and courses is described in detail. The updated document should describe a unified tactic for the management of Russian CF patients.Conclusion. Unified clinical guidelines for children and adults should develop unified tactics for the diagnosis, therapy, and follow-up of CF patients in the Russian Federation and ensure continuity between health care services for children and adults.