M. O. Revnova’s research while affiliated with Saint Petersburg State Pediatric Medical Academy and other places

Complete blood count is one of the most accessible and common laboratory tests available today. In present-day healthy newborns, complete blood count levels differ from the normative values used in the 1970s, which may require critical analysis, clarification, further research, and revision of the concept of norm. Objectives. To identify changes in the hematologic parameters of healthy newborns born in 2022 as compared to those reported in the 1930s and 1967–1970s. Methods. A retrospective cohort study of neonatal records was conducted at the Neonatal Physiology Unit of the Perinatal Center of the Saint Petersburg State Pediatric Medical University (Ministry of Health of the Russian Federation). Eleven hematologic parameters were analyzed in 378 healthy full-term newborns (born in 2022) in their first three days of life with subsequent comparison of the obtained results with the data of 20th-century domestic publications, as well as with Russian and foreign scientific articles published in the last 15 years. The blood of all children was tested once using a Micros ES 60 automated hematology analyzer (HORIBA ABX S.A.S., France). Mathematical and statistical data processing was performed using Microsoft Office Excel 2021 (Microsoft, U.S.). Results. A comparison of red blood cell and hemoglobin levels reported in newborns in their second and third days of life in 1970 and 2022 revealed statistically significant differences, with lower levels observed at present. Mean platelet counts are also significantly lower than those reported in the 1930 and 1970 studies but are almost the same as those reported by other authors in 2012–2019. The mean white blood cell count is slightly higher as compared to the 20th-century normative values. The dynamics of changes in the mean white blood cell count are similar in all studies presented in the article, revealing a marked decrease by the third day of life as compared to the first and second days; however, 20th century data reveals even lower levels on the third day of life. Noteworthy is the decrease in the relative numbers of lymphocytes and band neutrophils with an increase in segmented neutrophils in this study as compared to the 1930, 1970, and 2012 studies. Conclusion. The current mean values and reference intervals of hematologic parameters in healthy newborn babies differ from those presented in scientific studies and medical articles of the 20th century. This fact requires repeat analysis and establishment of new norms due to the increased sensitivity of analysis, the emergence of new procedures, and higher accuracy of equipment, as well as, potentially, due to changes in the maturity criteria and physical development indicators for newborns in the 21st century.

In 2024, the medical community will celebrate the 130th anniversary of the birth of the outstanding pediatrician, scientist, creator of scientific and practical schools of pediatrics — Alexander F. Tur. Alexander F. Tur was born on September 3 (15), 1894. In 1911 he was enrolled in the Imperial Military Medical Academy. From April 1915 he worked as a doctor in the active army; in August of the same year, A.F. Tur was captured and worked as a doctor in the infirmary for 3 years. In 1918, A.F. Tur resumed his studies at the Academy. From 1921 to 1930 — he worked part-time in the clinic of childhood diseases of the Military Medical Academy, under the guidance of Professor M.S. Maslov, in 1925 he was enrolled in the staff of the Leningrad Scientific and Practical Institute of Maternity and Infancy Protection named after Clara Zetkin. In 1939, the Department of Hospital Pediatrics was organized at the institute, headed by Alexander F. Tur and worked there until the end of his life. During the war years, A.F. Tur supervised the organization of clinic bomb shelters, advised children, conducted rounds, lectured students, and gave lectures to residents of the besieged city. A.F. Tur worked on a wide range of scientific problems in pediatrics, devoting his work to the most pressing issues of medicine: physiological characteristics and education of healthy children, physiology and pathology of newborns and premature babies, hematology of a healthy and sick child, dietetics of a healthy and sick child, rickets in children and its prevention, organization of children’s healthcare. A.F. Tur laid the foundations of preventive medicine at the outpatient stage, which are still followed today. The legacy of scientist is a fundamental pediatric school, a large number of scientific discoveries and works, thousands of trained do.

Authors represent a clinical case of short bowel syndrome, pathological condition caused by a decrease in the absorptive surface of the small intestine, which arose in a child as a result of surgical interventions for small intestinal atresia type IV (multiple atresias). The use of the Teduglutide drug experience is described in a child with severe manifestations of short bowel syndrome.

Authors represent the Russia’s first clinical case description of a pediatric patient with ulcerative colitis whose diagnosis was combined with Lambert-Eaton myasthenic syndrome (LEMS), a rare autoimmune disorder of neuromuscular junction transmission. The observed case draws specialists’ attention to the complexity of diagnosis and the importance of a multidisciplinary approach in the diagnosis and management of such patients. Another purpose was to increase physicians’ awareness of LEMS thereby reducing the period needed to establishing of the diagnosis, which in its turn improves the prognosis of the course of the disease for patients.

BACKGROUND: The problem of the complicated course of COVID-19 and deaths in children with severe comorbid pathology is relevant. Severe forms of the disease are often the cause of critical conditions and deaths, including decompensated respiratory failure, acute respiratory distress syndrome, septic shock, cardiac, hepatic, renal insufficiency, coagulation disorders, damage to the central nervous system. AIM: The aim is to analyze the features of the course of COVID-19 in children who require mandatory hospitalization in specialized hospitals for background pathology, to identify predictors of the development of severe forms of COVID-19 in order to choose the optimal ways of treating this pathology. MATERIALS AND METHODS: Under the supervision of the St. Petersburg State Pediatric Medical University in the department for children with new coronavirus infection COVID-19 with intensive care and intensive care wards in the period 2020–2022 with a confirmed diagnosis of “new coronavirus infection” there were 791 patients aged 0 to 17 years. The diagnosis was made on the basis of clinical and epidemiological data, the results of a comprehensive laboratory and instrumental study. The severity of the disease was determined in accordance with the methodological recommendations of the Ministry of Health of the Russian Federation. RESULTS: The severe form of COVID-19 was diagnosed in 34 patients out of 791 (4.3%). Adverse outcomes were 1.4%. In 27 out of 34 children (79.4%), new coronavirus infection occurred against the background of severe comorbid pathology. Pneumonia was diagnosed in 91.2% of cases. 88.2% of patients needed oxygen therapy. CONCLUSIONS: Severe forms of COVID-19 in children are characterized by high fever with severe intoxication, respiratory tract damage with a significant volume of lung damage (CT-3 and CT-4) with the development of multiple organ failure. Predictors that require mandatory hospitalization in specialized hospitals for background pathology in severe forms of COVID-19 are: primary immunodeficiency conditions, oncohematological pathology with postcytostatic aplasia of hematopoiesis, severe organic lesions of the central nervous system, epilepsy, protein-energy deficiency of 3–4 degrees, bronchopulmonary dysplasia, atypical emolytic uremic syndrome with acute kidney damage. Comprehensive treatment of such patients should be carried out by a multidisciplinary team in a specialized department with extensive diagnostic and therapeutic capabilities.

Background: Patients with immune-mediated diseases, such as juvenile idiopathic arthritis (JIA) and inflammatory bowel disease (IBD) are at increased risk of developing infections, due to disease-related immune dysfunction and applying of immunosuppressive drugs. Aim: To evaluate vaccine coverage in patients with IBD and JIA, and compare it with healthy children. Methods: In the cross-sectional study we included the data from a questionnaire survey of 190 Legal representatives of children with JIA (n = 81), IBD (n = 51), and healthy children (HC, n = 58). An electronic online questionnaire was created for the survey. Results: There were female predominance in JIA patients and younger onset age. Parents of JIA had higher education levels. Employment level and family status were similar in the three studied groups. Patients with JIA and IBD had lower vaccine coverage, without parental rejection of vaccinations in IBD, compare to JIA and healthy controls. The main reason for incomplete vaccination was medical conditions in IBD and JIA. IBD patients had a lower rate of normal vaccine-associated reactions compared to JIA and HC. The encouraging role of physicians for vaccinations was the lowest in JIA patients. IBD patients had more possibilities to check antibodies before immune-suppressive therapy and had more supplementary vaccinations compared to JIA and HC. Conclusion: JIA and IBD patients had lower vaccine coverage compared to HC. Physicians' encouragement of vaccination and the impossibility of discus about future vaccinations and their outcomes seemed the main factors for patients with immune-mediated diseases, influencing vaccine coverage. Further investigations are required to understand the reasons for incomplete vaccinations and improve vaccine coverage in both groups, especially in rheumatic disease patients. The approaches that stimulate vaccination in healthy children are not always optimal in children with immune-mediated diseases. It is necessary to provide personalized vaccine-encouraging strategies for parents of chronically ill children with the following validation of these technics.

Among the diseases of the gastrointestinal tract, inflammatory bowel diseases occupy a special place and are the subject of a thorough, comprehensive study, attracting the attention of scientists and doctors around the world. The relevance of numerous studies is due to the steady increase in incidence, the presence of severe disabling forms, a variety of extraintestinal and systemic manifestations. Social and economic importance is determined by the manifestation of inflammatory bowel diseases mainly among young people of working age. At the same time, the mechanisms that trigger the inflammatory process have not been studied enough. The selection of risk factors also seems to be very arbitrary, which significantly reduces the possibility of preventing these conditions, predicting relapse and, as a result, the quality of life of patients. This literature review is an attempt to collect together the available data on the role of acetylcholine, serotonin, substance P and the vasoactive intestinal peptide in the development of the inflammatory response and modulation of the immune response in Crohn's disease and ulcerative colitis. The study of the content of neurotransmitters in the blood of patients and colon biopsy specimens can be important in developing criteria for laboratory diagnostics, assessing the degree of activity of the pathological process, making a forecast about the effectiveness of the therapy and the outcome of the disease. Key words: acetylcholine, Crohn's disease, inflammatory bowel disease, neurotransmitters, serotonin, substance P, ulcerative colitis, vasointestinal peptide

A clinical case of mitochondrial neurogastrointestinal encephalopathy (MNGIE), a rare autosomal recessive multisystem disease caused by TYMP gene mutations and thymidine phosphorylase (TP) deficiency, is presented. Authors draw the specialists’ attention to the complexity of making a diagnosis and the importance of an interdisciplinary approach in the diagnosis and management of such patients with the purpose of increasing the pediatric practitioners’ MNGIE awareness thereby reducing the time to diagnosis, which in its turn improves the prognosis for the course of the disease for the patients.

Diabetes mellitus continues to be the most common endocrine-metabolic pathology. In childhood, type 1 diabetes mellitus prevails - an autoimmune, genetically determined disease, as a result of which this type of diabetes is often combined with other autoimmune diseases. Autoimmune gastritis, the marker of which is antibodies to the parietal cells of the stomach, is quite rare in the children’s population, but with type 1 diabetes, its frequency increases significantly. The article presents the results of recent studies of the relationship between type 1 diabetes mellitus and autoimmune gastritis in children. The results of a study of the prevalence of antibodies to parietal cells of the stomach in type 1 diabetes mellitus in children, factors affecting this indicator are presented. The pathogenetic aspects of the relationship of these diseases are analyzed.

The authors represent the clinical case observation of the Schimmelpenning-Feuerstein-Mims syndrome, a congenital dermatological-neurological disease, which includes an organoid epidermal nevus with a wide range of multiple organ dysfunction (neurological, skeletal, cardiovascular, ophthalmological and urological), complicated by the development of rhabdomyosarcoma in a patient. The predominant neurological manifestations of the syndrome are as follows: epilepsy, intellectual and focal disorders. The authors pay specialists’ attention to the complexity of establishing diagnosis and the importance of an interdisciplinary approach in the diagnosis and management in such kind of patients.