The atypical hemolytic uremic syndrome (aHUS) is an ultra-rare disease with a genetically determined dysregulation of the alternative complement pathway, which leads to the development of thrombotic microangiopathy. The application of the targeted therapy in Russia has changed the natural course of the disease since 2012: from death of a patient to achieving a long-term remission of the aHUS. The authors report the clinical case of the aHUS associated with a heterozygous mutation in CFH gene c.2850G>T (p.Gln950His) in a 2 years and 2 months old pediatric patient. The difficulty in diagnosing of the aHUS was the lack of specific laboratory criteria confirming the diagnosis as well as the presence of a diarrheal prodrome at the onset. The diagnosis has been made on the basis of the development of microangiopathic hemolytic anemia, thrombocytopenia, acute kidney injury and the exclusion of other forms of thrombotic microangiopathy. The treatment of the patient in accordance with the clinical guidelines for the management of patients with this nosology proved to be effective and led to a stable remission of the aHUS, which also confirmed the correctness of the diagnosis. The described clinical case observation is of interest to pediatrician physicians and nephrologists. The important factors in the management of such patients are the timely diagnosis of the aHUS along with the initiation of complement-inhibiting Eculizumab therapy.

Pneumonia is one of the most urgent and serious pathologies in pediatrics, the pathogenesis of which is determined by the pathogen-induced acute inflammatory response in the bronchial mucosa and lung tissue. In this regard, along with etiotropic medications, anti-inflammatory agents play an important role in the treatment of this group of patients. Objective. To evaluate the experience of using the anti-inflammatory drug ammonium glycyrrhizinate in the complex treatment of children with community-acquired pneumonia. Patients and methods. Fifty children aged 3 to 7 years diagnosed with community-acquired pneumonia participated in the observational program. Patients were divided into two groups: the control group (n = 30), which represented patients who received standard therapy, and the study group (n = 30), which represented patients who received ammonium glycyrrhizinate (AG) for 10 days in addition to standard therapy. Daily physical examination, scoring of symptoms (cough, sputum production) and adverse events were performed in the groups. Results. Patients in the study group, in comparison with the control group, showed a significantly more pronounced positive dynamics against the background of AG therapy, which was expressed in a considerable decrease in the intensity of daytime/nighttime cough already from the 3rd day of treatment. It allowed 50–60% of children to achieve complete relief of cough symptoms by day 5–6, and the remaining proportion of patients had its minimal manifestations (1 or less scores), which did not affect daytime activity or sleep (p < 0.05). The use of AG in patients in the study group reduced the presence of daytime/nighttime cough symptoms by 25% compared with the control group (p < 0.05). On the 4th day of observation, there were statistically significant differences in the incidence of fever; it was absent in a significant proportion (97%) of patients in the study group (p < 0.05). By the 7th day of treatment against the background of AG therapy, pulmonary function was restored in all patients in the study group, which was characterized by the absence of sputum production. Conclusion. Inclusion of ammonium glycyrrhizinate in the treatment of children with community-acquired pneumonia provides a rapid regression of the severity of the main respiratory symptoms of pneumonia (daytime/nighttime cough, sputum production) and a significant reduction in the duration of their presence and relief, which considerably accelerates the rate of recovery and improvement in the overall health of children, as well as the prognosis of the disease in general. Key words: pneumonia, children, rehabilitation, ammonium glycyrrhizinate