The Ministry of Health of the Russian Federation jointly with professional association and experts in the field of pediatrics, infectious diseases and resuscitation has revised guidelines “Clinical Features and Management of the Disease Caused by New Coronaviral Infection (COVID-19) in Children” in order to provide the child population with effective medical care during the pandemic of the new coronaviral infection. The practical experience of specialists from various countries was considered during the development of this document. Special attention should be given to the evidence base of the presented data, as well as to the efficiency and safety issues of medications used in treatment of coronaviral infection and its complications. The authors highlight the problems of prevention, diagnostics and management of pathological conditions caused by COVID-19 in the article according to the presented guidelines. Patient’s management is presented depending on the age and severity of the disease itself. The therapy is considered with regard to etiological, pathogenetic and symptom focus.

Background. Non-compaction cardiomyopathy is a group of genetically heterogeneous, poorly studied myocardial diseases with a variety of clinical manifestations (from asymptomatic course to progressive systolic dysfunction with symptoms of chronic heart failure, arrhythmias, and thromboembolic complications). Considering the variety of genetic disorders associated with the development of noncompaction cardiomyopathy, genetic verification of the diagnosis is important for determining the prognosis and conducting genetic counselling of families with cases of the disease.Description of the Clinical Case. The article presents two clinical observations of a severe course of non-compaction cardiomyopathy with remodeling of the heart cavities according to the dilated phenotype. In order to clarify the disease etiology, a molecular genetic study was conducted using the method of direct automatic sequencing with the analysis of targeted regions of 404 genes which mutations are described in hereditary diseases of the heart and blood vessels. After verifying the mutation (in the ACTC1 and MYBPC3 genes), we performed a search for the detected nucleotide substitution in the venous blood samples of parents and in one case — in the fetal DNA sample. The mode of inheritance has been determined; the probability of recurrence of the disease in siblings in subsequent pregnancies has been estimated.Conclusion. The description of clinical cases shows the importance of genetic verification of the diagnosis in patients with non-compaction cardiomyopathy for determining the disease prognosis and developing an algorithm for monitoring relatives of a proband.

Space-occupying lesions in the heart refer to a rare congenital pathology with incidence of 0.32% in pediatric practice. In recent years, the detection  frequency  of this pathology  has increased  due to the achievements  of early diagnostics  (including prenatal diagnostics),  integration of high volume scanning and high-quality ultrasound images. The greatest number of tumors (72–77%) is detected in infants under 1 year of age, up to 26% of which — prenatally. Available international clinical data confirms the congenital nature of most heart tumors, approximately 50% are rhabdomyomas.  The presented clinical case demonstrates  the demand for a complex approach and the application of new 3D and 4D cardiac scanning in pediatric practice to assess the severity of cardiovascular damage and the prognosis of treatment in patients with rhabdomyomas of the heart.