We present the case of a 18-month-old girl with renal and cardiac manifestations of atypical haemolytic uraemic syndrome (aHUS), and a novel complement factor H mutation. Transient haematological remission was achieved with intensive plasmapheresis, but cardiac function deteriorated and renal function was not restored. Initiation of eculizumab after 6 months of dialysis significantly improved organ function. At 43 months after presentation, haematological values had normalised and cardiac function had improved. Dialysis was discontinued after 10 months (the longest reported time in a patient with aHUS) and the estimated glomerular filtration rate had recovered to 70 mL/min/1.73 m(2). In conclusion, treatment of aHUS with eculizumab, even after long-term dialysis, can significantly improve renal function. Discontinuation of dialysis and resolution of cardiac function has implications on the potential recovery and treatment choice of such patients. Earlier initiation of eculizumab, however, might have prevented the irreversible renal sclerosis and cardiac dysfunction.

The problem of community-acquired pneumonia in children remains relevant at the present time. Complicated forms, which include pleural empyema, abscess, necrotizing pneumonia, bronchopleural fistulas and acute respiratory distress syndrome do not decrease, despite modern antibacterial therapy and the availability of vaccination against pneumococcus. The main pathogens associated with lung destruction in children are S. pneumoniae and S. aureus, often MRSA. The role of other pathogens in necrotizing pneumonia is much less frequently reported: Streptococcus pyogenes, Haemophilus influenzae, Pseudomonas aeruginosa, Fusobacterium nucleatum, Legionella pneumophila, Klebsiella pneumoniae, anaerobes. However not only pathogenic factors of the pathogen are important for necrotizing pneumonia development. Often, a viral prodrome, often associated with the influenza A (H1N1) virus, precedes complicated pneumonia. During the epidemic of COVID-19, endothelial damage with a high degree of probability was a predisposing factor for the development of a secondary bacterial infection with lung tissue necrosis. Significant destruction and liquefaction of the lung tissue may develop despite adequate antibiotic therapy. Great importance in the development of necrosis is attached to the activation of hemostasis and thrombus formation in the vessels of the lungs. Timely diagnosis often is difficult due to the predominance of general symptoms over local ones, especially in young children. Chest x-ray is the standard for diagnosing. However, the diagnostic capabilities of this method in necrotizing pneumonia are limited. To assess a number of parameters of the state of the pleural cavity and lung tissue, ultrasound is preferred. It is necessary to analyze the current features of the course of necrotizing pneumonia in children and develop clinical guidelines for the management of patients in the acute period and rehabilitation.

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Community-acquired pneumonia is a potentially serious infection in children with high morbidity rate, risk of severe course and unfavorable outcomes. Specialists have noted the increased incidence of the destructive forms in the recent years.Aim. To present a clinical case of destructive pneumonia in a 1 year 2 month old child, hospitalized in the State Budgetary Healthcare Institution “Children’s City Clinical Hospital of St. Vladimir Moscow Healthcare Department”, and analyze it in terms of current understanding on the disease pathogenesis.Conclusion. During COVID-19 (COronaVIrus Disease 2019) pandemic, pulmonologists and pediatric surgeons encountered an unconventional course of destructive pneumonia. A large number of studies of pathophysiological processes in acute viral interstitial pneumonias have recently allowed to expand our understanding of the role of coagulation system. At the same time, new questions arose concerning the clinical course and development of the pathological infectious process.