The article discusses results of a prospective observational study of long-term use of the biosimilar dornase alfa (Tigerase) (Generium, Russia) as part of complex therapy in patients with cystic fibrosis in real clinical practice.The aim was to analyze the outcomes of long-term use of the dornase alfa dornase alfa as part of complex therapy in patients with CF (protocol #DRN-CFR-N01).Methods. The study included patients (n = 165) aged 5 years and older from 11 centers for treatment of cystic fibrosis in the Russian Federation with a confirmed diagnosis of cystic fibrosis who were prescribed dornase alfa by their attending physician.Results. The analysis revealed that exacerbations of chronic pulmonary disease during the treatment with dornase alfa were observed in 29 (17.58%) patients included in the study. At the same time, there were no statistically significant changes in FEV1 and FVC (%) against baseline during the treatment in the study population. Adverse events related to the study drug were recorded in 9 (5.45%) patients.Conclusion. Biosimilar dornase alfa demonstrated a favorable efficacy and safety profile in routine clinical practice, which confirms the results of previously published studies.

Introduction. This article highlights the problem of thrombus formation in pediatric patients staying in ICU, namely, the problem of prevention and management of pulmonary embolism in children. Currently, there is a tendency to increasing pulmonary embolism occurrence in children, though there are no clear guidelines on the medicine dosage for the systemic thrombolytic therapy of pulmonary embolism in children and for the acute coronary syndrome in children with Kawasaki disease.Clinical observations. The authors present two clinical cases from their practice when a successful management was obtained in children with life threatening thrombotic complications due to the applied systemic thrombolytic therapy.Conclusion. The obtained success in the described clinical cases have resulted from a thorough analysis of ratios “risk-benefit” and “efficiency-safety”. But the authors conclude that further research work in this direction is still needed.

In order to improve the clinical use of antibiotics in surgical departments of Morozovskaya Children City Clinical Hospital of Moscow Healthcare Department authors prepared, discussed and agreed upon a protocol for perioperative and post-exposure antimicrobial prophylaxis. The choice of antibiotics for systemic use was made according to the Antimicrobial Stewardship Program (ASP) stratification both for patients of type I (community-acquired infection without risk for shedding of polyresistant infectious agents) and type II (community-acquired infections with risk factors for shedding of polyresistant infectious agents). Study determined indications for post-exposure antimicrobial prophylaxis within the approved protocol. Following that the Chief Physician issued an Order On Division of Responsibility of all Members of the Operating Team and On Approval of the Protocol for Conducting Perioperative and Post-Exposure Antimicrobial Prophylaxis. As a result of the implementation of the protocol, by the end of 2020 clinical use of antibiotics in surgical departments has improved.

Pulmonary complications in Stevens Johnson syndrome (SJS) or toxic epidermal ecrolysis (TEN) are rare and mostly manifest as broncho bron chiolitis obliterans with poor prognosis. Recently, there is no effective therapy for this condition in patients with SJS / TEN. We described a case of SJS with progressive hypercapnic respiratory failure in 4.5 year boy who died despite an intensive treatment. herefore, lung transplantation should be considered at the early stage of this disease.