Thanks to modern approaches to the treatment of children with oncohematological diseases, an increase in overall and relapse-free survival is noted. However, in the process of therapy of a malignant disease, a number of complications may arise, leading to the development of concomitant pathology with various functional disorders. The article presents a broad spectrum of morphologic and functional disorders in 52 patients completed an oncohematological disease treatment. It is justified the use of physical and psychological methods of rehabilitation for the most effective prophylaxis and treatment.

Modern medical scientific sources offer descriptions of cases of hereditary bone dysplasias occurring under the guise of rheumatoid arthritis (RA). These include the progressive pseudorheumatoid dysplasia (PPRD), an autosomal recessive disease caused by defects in the CCN6 gene, as well as autosomal dominant forms of pseudorheumatoid dysplasia associated with variants in the COL2A1 gene. Though there are no references to pseudorheumatoid dysplasia case observations in available domestic sources. This Article represents a discussion over the bibliographic sources on PPRD and a clinical case observation of a patient initially observed for RA but diagnosed subsequently with an autosomal dominant form of bone dysplasia caused by a de novo variant in the COL2A1 gene.

Purpose of the research was to study the structure of the cardiovascular system (CVS) lesions, risk factors and predictors for adverse outcomes in children with Kawasaki disease (KD). Materials and methods used: a single-center retrospective cohort study of 188 patients (126 boys/62 girls) aged 2 months to 11 years old with KD in 2014-2019. Depending on the outcome of the disease, the two groups of patients were identified: those with recovery (171/188, 91%), and with unfavorable outcomes (17/188, 9%) in the form of persistent coronary artery aneurysms (CAA) in 12 (6.4%) cases and death in 5 (2.7%) cases. Authors used the clinical-anamnestic method, clinical and biochemical blood tests and coagulogram. The diagnosis of CVS changes was based on the results of echocardiography, magnetic resonance angiography, coronary angiography and ultrasound of non-coronary vessels. The levels of N-terminal fragment of B-type brain natriuretic peptide (NT-proBNP) and atrial natriuretic peptide precursor (proANP) were determined. Results: CVS lesions in the acute period of KD were detected in 92 (48.9%) patients. Non-coronary changes in the cardiovascular system, represented by myocarditis (25%), pericarditis (9.6%), transient mitral valve insufficiency (26.1%) with valvulitis (4.3%) were determined in the acute and subacute periods of KD. Damage to the coronary arteries (CA) in the acute period, according to echocardiography, was detected in 61 (32.4%) children, of which: coronaritis - in 43 (22.8%), transient ectasia - in 9 (4.7% ), CAA of various sizes - in 49 (26.1%). Predictors of unfavorable KD outcomes are the presence of coronary (p=0.000) and non-coronary changes (p=0.001) in the cardiovascular system during the acute period of the disease, blood clots of any location (64.7% v. 3.5%, p=0.000). Based on the results of univariate logistic regression analysis, significant threshold levels of natriuretic peptides were established to determine the risk of developing adverse KD outcomes: an increase in NT-proBNP in the acute period of KD more than 984 pg/ml with 79% sensitivity, 84.8% specificity, 82.9% overall predictive value; an increase in proANP greater than 1.015 nmol/l with 87.5% a sensitivity, 75.8% specificity and 78.0% overall predictive value. Conclusion: risk factors and predictors for the unfavorable KD outcomes have been identified as follows: the appearance in the acute period of CAA of medium and especially giant sizes, pericarditis, myocarditis, damage to the valvular apparatus, thrombosis of the coronary artery and cardiac cavities, high levels of proANP, NT-proBNP, which can be used as a diagnostic tool in KD with poor outcomes.

Pediatric inflammatory multisystem syndrome (PIMS) associated with the new coronavirus infection is the most severe late complication of the COVID-19 infection in children. We present the Russia’s first clinical observation of a 9 years old girl with PIMS with successful use of extracorporeal membrane oxygenation (ECMO) in treatment. The disease was characterized by rapid progression of myocardial dysfunction, shock, multiple organ failure, secondary hemophagocytic syndrome, and resistance to therapy. Intensive therapy, including glucocorticosteroids, tocilizumab, intravenous immunoglobulin, antiplatelet agents and anticoagulants, respiratory and cardiotonic support, ECMO, renal replacement therapy and antimicrobials have allowed the child to stabilize and recover.

Multisystem inflammatory syndrome in children (MIS-C) associated with COVID-19 is a rare life-threatening immunopathological complication of COVID-19 that develops 1-6 weeks after the acute coronavirus infection. MIS-C is characterized by fever and multiorgan inflammation.We present a clinical case of a 10-year-old boy with skin lesions at the onset of MIS-C (erythematous malar rash, lacelike rash on the trunk and extremities and petechiae) with macrophage activation syndrome development and the early stage of primary Epstein-Barr virus infection (EBV infection) which required the exclusion of X-linked lymphoproliferative disease.This clinical case demonstrates the complexity of diagnosis in MIS-C with skin manifestations at the onset of the disease, especially with concurrent activation of other infections, particularly EBV infection.

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.

Coronavirus disease 2019 (COVID-19) in children in most cases is asymptomatic or mild, and its most severe late complication is multisystem inflammatory syndrome in children (MIS-C). The aim of the study is to find clinical, laboratory and instrumental characteristics, description of therapeutic tactics, including determination of the profile of patients requiring Tocilizumab prescription and the outcomes of MIS-C associated with COVID-19. Materials and methods of research: 245 children aged 3 months – 17 years old were included in the pilot prospective multicenter open-label comparative study with MIS-C associated with COVID-19, verified based on CDC criteria (2020). Results: the median age of patients was 8 [5; 10] years, boys predominated among the patients (57.1%); MIS-C manifested itself as a combination of the symptom complex of Kawasaki disease (KD, 53.1% of patients), more often of atypical form, cardiovascular (66.1%), gastrointestinal (61.2%), neurological (27.3%) symptoms and signs of detection of the urinary (29.4%) and respiratory (19.6%) systems; macrophage activation syndrome (MAS) was diagnosed in 19.5% of patients. Therapy included glucocorticosteroids (97.6%), antibiotics (95.5%), anticoagulants (93.9%), intravenous immunoglobulin (34.7%), vasoactive/vasopressor support (31.8%), Tocilizumab (15.1%), mechanical ventilation (2.4%), extracorporeal membrane oxygenation (0.4%). Patients receiving Tocilizumab, statistically significantly more often compared with patients without this therapy, were in the intensive care unit (ICU, 86.5% versus 40.9%, p<0.001), more often required vasopressor therapy (70.3% versus 25%, p<0.001), had statistically significantly higher markers of laboratory inflammatory activity. Treatment in 47.8% of cases was carried out in an ICU; one child has died. In 4.1%, according to echocardiography, coronaritis, ectasia of the coronary arteries without the formation of persistent aneurysms were detected. Conclusion: MIS-C associated with COVID-19 has clinical signs of KD, often of the incomplete form, accompanied by arterial hypotension/shock, MAS, which requires intensive therapy, and the prescription of Tocilizumab.

The severe course of chronic pancreatitis (CP) in children is rare. More often it is caused by hereditary factors and abnormalities of the bile and pancreatic ducts. Cases of the development of pancreatitis against the background of systemic inflammatory diseases are described. Therapy of a systemic disease can aggravate the course of pancreatitis, and the general serious condition of the patient does not allow for the necessary surgical treatment. The article describes the clinical case report of a complicated course of CP against the background of juvenile idiopathic arthritis in a 17-year-old girl. The effectiveness of intraluminal endoscopic methods of treatment (wirsungotomy and stenting of the main pancreatic duct) for relieving exacerbation and complications of CP has been demonstrated.

For the first time in the domestic literature, the article presents a clinical observation of multisystem inflammatory syndrome in children (MIS-C) associated with COVID-19 in the 6-year-old patient with manifestation of type 1 diabetes mellitus (T1DM) in the form of diabetic ketoacidosis. Anamnestic, clinical and laboratory data are presented on the basis of which two life-threatening diseases was diagnosed, as well as tactics of therapy, which made it possible to achieve a positive result. This clinical observation is compared with observations of foreign colleagues. Possible pathogenetic mechanisms of MIS-C and T1DM comorbidity are discussed.

Multisystem Inflammatory Syndrome in Children (MIS-C) associated with new coronavirus infection (COVID-19), with signs of Kawasaki disease (KD) and toxic shock syndrome, well-defined diagnostic criteria, is the most severe manifestation of COVID-19 in pediatric patients. MIS-C is analogous to the cytokine storm in children with COVID-19. The article presents a clinical observation of a child with MIS-C with a lethal outcome. Clinical and anamnestic data, the results of laboratory and instrumental research allowed to diagnose MIS-C in a 2-year-old girl with full KD form. Autopsy results, detailed microscopic examination, which revealed systemic vasculitis of small and mediumsized vessels, inflammatory infiltrates in different organs, are presented, clinical and morphological comparisons are made.

The most severe manifestation of the new coronavirus infection COVID-19 in children is the multisystem inflammatory syndrome in children (MIS-C). A systematic review of foreign publications as of July 25, 2020 contains an analysis of the disease course in 662 children with this syndrome and is used for comparison with the data obtained. Objective of the research: to characterize clinical manifestation, results of laboratory and instrumental studies, therapy, outcomes and consequences of the COVID-19- associated MIS-C, based on the observation of patients hospitalized to Morozov Children's City Clinical Hospital and Children’s clinical hospital of infectious diseases № 6 from May 1 to September 15, 2020. Materials and methods: the pilot study included 32 children aged 9 months – 15 years with COVID-19-associated MIS-C, verified based on WHO criteria (2020), including symptoms of Kawasaki disease (KD), arterial hypotension/shock, laboratory and instrumental signs of heart damage, signs of coagulopathy, gastrointestinal symptoms, increased inflammation markers, COVID-19 markers. Results: the median age of patients was 6 years, boys predominated among the patients (66%), all patients had antibodies to SARS-CoV-2 (31 children of the IgG class); MIS-C manifested itself as a combination of KD symptom complex (75% of patients) with arterial hypotension/shock (28%), neurological (50%), respiratory (41%), gastrointestinal (59%) symptoms; macrophage activation syndrome (MAS) was verified in 16% of patients. Therapy included intravenous immunoglobulin (75%), systemic glucocorticosteroids (88%), anticoagulants (91%), vasoactive/vasopressor support (31%). In 38% of cases treatment was performed in intensive care unit; one child died. According to echocardiography, 16% of patients had coronariitis, ectasia, and coronary arteries aneurysms. Conclusion: COVID-19-associated MIS-C is characterized by a severe course, cross-features with KD, shock syndrome with KD, MAS which requires intensive therapy and can cause acquired pathology of the cardiovascular system in children.

Objective: to evaluate the efficiency and safety of therapy with subcutaneous (SC) methotrexate (MTX) in patients with juvenile idiopathic arthritis (JIA) without systemic manifestations.Patients and methods. The paper presents the results of a prospective study of the efficacy and safety of MTX in 247patients aged 1 to 17 years with articular variants of JIA, as well as the frequency of indications for prescribing biological agents for this category of patients. JIA occurred without systemic manifestations in all the patients, including 106 with oligoarticular articular JIA, 94 with rheumatoid factor (RF)-negative polyarticular JIA, 15 with RF-positive polyarticular JIA, 20 with enthesitis-related JIA, and 12 with psoriatic arthritis. JIA was diagnosed according to the ILAR criteria. After verification of the diagnosis, all the patients were prescribed SC injections of MTX at a dose of 15 mg/m2/week.Results and discussion. After 3 months of MTX therapy, 50 and 70% improvements according to the ACR pediatric criteria were registered in 65 and 53% of the patients, respectively. After 6, 9, and 12 months of therapy, the stage of inactive disease or remission was observed in 44.5, 85, and 100% of the patients, respectively.Conclusion. Parenteral MTX administration contributes to the achievement of disease remission and the restoration of joint functions in patients with JIA without extra-articular manifestations. Along with its high therapeutic efficacy, MTX had a good tolerability and a favorable safety profile.

Timely diagnosis of juvenile idiopathic arthritis in children and early use of genetic engineering biological therapy are the leading vector of modern rheumatology. This clinical observation presents the working experience of City Children's Rheumatology Center that allowed to significantly reduce percent age of disability of children with JIA in Moscow.

The problem of obesity is currently being widely discussed by various specialists. The literature dealing with this problem places a considerable emphasis on investigations of lipid metabolism disorders that occupy a prominent place in the genesis of cardiovascular diseases in the populations of most countries. The special role of long chain polyunsaturated fatty acids that are major functional components of brain membrane structure phospholipids, retinal photoreceptors is emphasized. Pediatricians pay great attention to the nutrition of infants, by adjusting adapted milk-based formulas for their adequate growth and development. At the same time, no consideration is given to the possibility of correcting obesity-associated problems with appropriate adapted milk-based formulas in older infants. However, dietary recommendations for older infants with obesity and overweight should be developed not only in the context of optimization of a qualitative and quantitative diet that ensures the needs of the growing organism at the present moment, but should take into account the prevention of cardiovascular diseases and other complications of obesity in the future.