Vol 14, No 2 (2023)

BACKGROUND: To date, several options for helping pregnant women with rhesus immunization are known: (a) “active tactics” in carrying out methods of efferent therapy in the form of basic operations (plasmapheresis, hemosorption) in combination with adjuvant methods (immunoglobulin, blood photomodification with ultraviolet, laser beams, ozone therapy) to pregnant women; (b) “wait-and-see active tactics” with observation of the pregnant woman, followed by intrauterine intravascular transfusions of washed donor red blood cells; (c) “mixed active tactics” with a sequential combination, alternation of these methods. In Russia, only option 2 with fetal transfusions of washed donor red blood cells is accepted as the basis and paid for. The objective of the study is to conduct a comparative analysis of pregnancy outcomes in women with rhesus immunization using different management options.

MATERIALS AND METHODS: A total of 392 women were followed up at seven different institutions in Russia and at the Donetsk Center for Maternal and Child Health (DNR), of whom 345 pregnant women (Group 1) received efferent therapy, 33 women (Group 2) had fetuses intrauterine bypass surgery, and 14 pregnant women (Group 3) had mixed efferent therapy and fetal PEEP bypass surgery.

RESULTS: The analysis showed that the most favorable results for the main clinical indicators (premature, operative delivery, fetal hypoxia at birth, etc.) were in Group 1 and 3 women, in which the perinatal mortality was 14.5/1000 and 0/1000, respectively, which was significantly lower than in Group 2 (176.5/1000). It was also found that in Groups 2 and 3 women, the mean intervals between repeated transfusions of washed donor red blood cells were 8.8 ± 0.2 and 21.4 ± 3.8 days (p < 0.01), which may be explained by the detoxifying effect of efferent therapy methods, preservation of fetal red blood cells and transfused donor red blood cells to the fetus with prolonged gestation and obtaining healthier and more viable progeny.

CONCLUSIONS: 1. Severe Rh conflict is a manifestation of a syndrome of systemic effects of aggressive metabolites of specific and nonspecific nature. 2. The etiopathogenetic measure in the prevention and treatment of HDF/HDN in rhesus conflict is efferent therapy methods for the mother, and transfusion of washed donor rhesus-negative red blood cells to the fetus is effective, but a temporary, palliative measure, as is the case in multiple organ failure. 3. In the treatment protocols, efferent therapy methods must be present to prevent fetal red cell destruction and, equally importantly, to prevent destruction of Rh-negative donor red cells transfused to the fetus.

BACKGROUND: In children with dysphagia, an increase in body weight is observed after the placement of a feeding tube, however, subsequently, a regression of body weight is noted, symptoms of an erosive and ulcerative lesion of the gastrointestinal tract appear.

AIM: To identify the features of the gastric microbiome in children fed through a gastrostomy or a tube.

MATERIALS AND METHODS: A study of aspirates of gastric contents in 21 patients was carried out using metagenomic sequencing. The participants were divided into 2 groups: group 1 — 11 children fed through the gastrostomy for less than 1 year; group 2 — 10 children, fed through the gastrostomy for more than 1 year.

RESULTS: In group 1, from 8 to 19 phyla were identified, median 12.0. In the second group from 4 to 13, median 7.5, the differences are statistically significant (p < 0.05). The samples of both groups were dominated by the phyla Firmicutes, Proteobacteria, Bacteroidota, Actinobacteria, Fusobacteria. The number of representatives of the Bacteroidia and Fusobacteriia classes was significantly reduced in patients with long-term nutrition through the gastrostomy. At the same time, a small number of classes were observed in patients with a gastrostomy in the stomach for about 80 months, as well as in patients with identified gastric pathology. There were about 66 genera for each specimen. At the same time, in children fed through a gastrostomy for less than 1 year, the median is 69.5 OTU. In children fed through a gastrostomy for more than 1 year, even with its regular replacement, the median is significantly less — 41 OTU. A significant decrease in microbial biodiversity was revealed with an increase in the standing time of the gastrostomy, the median value of the Shannon index in group 1 was 1.95, in group 2 1.69 (p ≤ 0.05).

CONCLUSIONS: In patients with a long stay of the feeding tube in the stomach, the number of anti-inflammatory symbionts of the genus Prevotella, Parabacteroides is reduced. The contamination of the stomach with Helicobacter pylori was 50%, which further increased the predisposition of the gastric mucosa to inflammation.

BACKGROUND: Juvenile idiopathic arthritis is the most common rheumatic disease in children. A frequent extra-articular manifestation of juvenile idiopathic arthritis is uveitis, which is a serious clinical diagnostic problem in routine pediatric practice. Among the known risk factors for uveitis are the early age of the juvenile idiopathic arthritis onset, oligoarticular subtype, seropositivity by antinuclear factor.

AIM: to evaluate the influence of presence of uveitis on the course of juvenile idiopathic arthritis.

MATERIALS AND METHODS: A single-center retrospective study included 520 patients with uveitis. The analysis was carried out among patients who developed (n = 116) and did not develop (n = 404) uveitis. The minimum follow-up period was 2 years, for patients who did not develop uveitis.

RESULTS: Uveitis was diagnosed in 116 (22.3%) children with juvenile idiopathic arthritis. Most often, uveitis occurred in patients with oligoarthritis and psoriatic arthritis. When comparing the features of the articular status of patients with juvenile idiopathic arthritis who developed and did not develop uveitis, a lower frequency of involvement of the cervical spine, temporomandibular, shoulder, elbow, wrist, proximal and distal interphalangeal joints, hip, talus-heel joint, as well as a smaller number of active joints in children with uveitis was found. Patients with uveitis received methotrexate therapy more often, cumulative doses of corticosteroids were lower, the frequency of prescribing genetically engineered biological drugs was approximately the same in both groups. Remission of arthritis was achieved more often, but the proportion of children who developed an exacerbation was higher. When calculating the risk factors of uveitis by binary logistic regression, it was found that the main predictors of uveitis were oligoarthritis, the number of active joints <8, seropositivity by antinuclear factor and recurrent course of arthritis. The difference in the frequency of achieving remission may be due to more aggressive systemic therapy in the presence of uveitis.

CONCLUSIONS: Children with uveitis have a more severe course of juvenile idiopathic arthritis and may require more aggressive immuno-suppressive therapy. Further studies are required to determine the prognostic role of uveitis in the course and outcomes of juvenile idiopathic arthritis.

A systematic analysis of the data available in modern literature concerning the problem of connective tissue dysplasia in children and adolescents, and the results of studying the course of bronchial obstruction syndrome in patients with this pathology, was carried out. Mechanisms of connective tissue dysplasia is imperfect, elucidation of the genetic causes of hereditary connective tissue disorders has not yet led to cardinal improvements in diagnosis, prevention and treatment. Connective tissue dysplasia is characterized by the presence of changes in the bronchopulmonary system: tracheobronchial dyskinesia (expiratory collapse of the trachea and large bronchi), tracheobronchomalacia and tracheobronchomegaly, apical bullae, confirmed by radiographic and spontaneous pneumothorax (more common in young men), the presence of thoracodiaphragmatic syndrome, which includes an asthenic form of the chest, deformities chest and / or spine, change in standing height and excursions of the diaphragm. The etiological and pathogenetic factors in the development of bronchiectasis remain ambiguous. Patients with connective tissue dysplasia require special attention and deeper study. The presence of thoracodiaphragmatic and bronchopulmonary syndrome in children contributes to a longer course of broncho-obstructive syndrome, a brighter, vegetative color of seizures is described. It is noted that these patients need more aggressive and long-term therapy.

Information about the natural nevogenesis of pigmented skin lesions, evolutionary and morphological features in pediatric patients is still rare in the medical literature despite the progress in dermatooncology. Clinical examination or analysis with the “naked eye” of skin neoplasms carries a certain diagnostic information content. However, this method is much less informative compared to the use of an epiluminescent microscope. Dermoscopy of skin neoplasms is a non-invasive highly informative diagnostic method. Nowadays, the technique is widely used in the clinical practice of pediatric oncodermatologists. This painless diagnosis is ideal for a primary analysis of the morphological picture melanocytic or other skin neoplasms, malignancy risk assessment and determining the tactics of managing pediatric patients. The necessity for differential diagnosis the spectrum of skin neoplasms dictates a trend towards improving research methods and an age-adapted approach. The method of skin microscopy can be attributed to the methods of choice among those currently available. The history of method, diagnostic informativeness and detailed morphological characteristics of melanocytic skin neoplasms in children and adolescents are presented.

The new coronavirus infection does not lose its relevance, which is due to the ubiquity of the virus, the variety of strains, clinical manifestations and their consequences of COVID-19. In children, one of the severe complications of new coronavirus infection is the multisystem inflammatory syndrome associated with SARS-CoV-2 (MIS-C), which is characterized by a hyperinflammatory response with damage to two or more organs and systems. MIS-C requires differential diagnosis with other diseases with similar symptoms: bacterial infections, sepsis, toxic shock syndrome, Kawasaki syndrome, hemophagocytic syndrome. As an example, the article considers a clinical case of MIS-C in a 13-year-old girl 4 weeks after undergoing new coronavirus infection (confirmed). At the onset of the disease, the leading manifestations were systemic inflammatory response syndrome (fever, increased markers of inflammation) with damage to the cardiovascular system, skin and mucous membranes, lymphadenitis, thrombocytopenia. According to the severity of the condition, the child required observation and treatment in the intensive care unit. The absence of the expected effect of the recommended MIS-C therapy (intravenous immunoglobulin and pulse therapy with methylprednisolone) on the first day did not allow to exclude bacterial infection complicated by sepsis in the above clinical situation, therefore, it was suggested that the combined course of MIS-C and bacterial infection of unspecified etiology, which probably led to a more severe course of the disease. In the outcome of the MIS-C the child had the formation of a long-term depressive state (diagnosed by a psychiatrist), for which the patient received drug therapy and psychological assistance.

The described case showed difficulties in the formulation of the final diagnosis due to the presence in the patient of several symptoms and syndromes characteristic of various diseases, the absence of an identified etiological agent and the absence of the expected positive effect with standard therapy of the disease. In addition, a pathology was revealed in the catamnesis, which required dynamic observation of the child by several specialized specialists.

Hypoglycemia is the most common metabolic disorder occurring in early childhood, which can be the first, and sometimes the only symptom of a whole range of diseases. Etiology determines the characteristic clinical features of the course of hypoglycemic syndrome. Diagnosis is complicated by the fact that the clinical manifestations of hypoglycemia are variable and little specific. The main cause of persistent hypoglycemia in children of the first years of life is congenital hyperinsulinism.

Congenital hyperinsulinism is a hereditary disease characterized by inadequate hypersecretion of insulin by beta cells of the pancreas. Manifesting as a rule in the neonatal period, congenital hyperinsulinism is a great threat, both in terms of survival of patients with late diagnosis, and in terms of the risks of severe neurological complications with inadequate therapy of emerging hypoglycemia.

The article describes a family case of congenital organic hyperinsulinism associated with a rare heterozygous mutation in the ABCC8 gene, the early diagnosis of which allowed avoiding severe complications of the disease and timely prescribing adequate treatment. The clinical variability of the course of the disease in related patients was shown, which required a personalized approach to diagnosis and treatment.

Thus, early detection of hypoglycemia, clarification of its etiology, including with the help of molecular genetic analysis, timely drug therapy and monitoring of the state of carbohydrate metabolism, are important aspects in the treatment and supervision of patients with congenital hyperinsulinism and the prevention of severe neurological complications in them.

The organization of medical support during the evacuation of the population, including children and adolescents during the Great Patriotic War, especially in 1941–1942, is unique. Attention is drawn to the scale — the timing of the organization of evacuation, the number of evacuated people, methods — by water, aviation, land transport. Special attention in conditions of shortage deserves catering — all evacuees received a hot lunch and rations for the road in the form 1 kg of bread. Gradually, the prevention of infectious diseases was established, vaccination against dysentery was actively used, teams of epidemiologists and disinfection and washing teams were formed, medical support was provided. In total, from the beginning of the blockade to the end of 1942, about 1 million 100 thousand people were evacuated, of which 871 thousand Leningraders. And during the war, about 1 million 738 thousand people were taken out of Leningrad in an organized manner, of which 1 million 360 thousand Leningraders. Priority in mass evacuation was given to children and adolescents — the future of the country. All the sick and wounded, whose treatment required more than 45 days, were subject to evacuation. Thanks to the precise organization of care for evacuees on the way and during the period of overload from one mode of transport to another, difficulties were transferred to the wounded and sick relatively easily. Thanks to the planned work during the evacuation, mass outbreaks of infectious diseases were prevented by medical workers, mortality was reduced as much as possible and the possibility of restoring normal nutrition and life in the evacuation areas was ensured.

Palliative care is an actively developing area. A separate area of palliative care is palliative care for children and their families, that is, support for children and adolescents and incurable, progressive diseases up to the age of 18. The relevance and demand for palliative and hospice care is confirmed by the growing number of patients in the North-Western Federal District who need them. In 2018, the Leningrad Region opened the State Autonomous Health Institution of the Leningrad Region “Children’s Hospice” (hereinafter – the Children’s Hospice). The mission of the Children’s Hospice is to provide assistance where recovery is impossible, which is realized in an individual approach to each patient, taking into account his resources and environment, and not a diagnosis. The main goal is to improve the quality of life of children with incurable diseases and their families through providing them with multidisciplinary assistance. Children’s Hospice implements unique support programs for children with life-threatening or life-limiting diseases. The directions of development of the Children’s Hospice are outlined, related to the presence of myths and stereotypes in society, the remoteness of the districts of the Leningrad Region, the difficulties of interdepartmental interaction with regional healthcare institutions, the peculiarities of interaction of patients with specialists of various healthcare institutions and the occurrence of iatrogenic injuries, issues related to the anesthesia of patients. Promising growth points have been outlined that will optimize palliative care for children. In particular, conducting educational work, opening outpatient branches in certain districts of the Leningrad Region which will allow observing the time interval of the assistance provided.

The article presents the history of World Kidney Day 2006—2023. The World Kidney Day, which started in 2006, has become an annual successful initiative of the International Society of Nephrologists, the International Federation of Kidney Foundations to inform the world community and politicians, healthcare about the importance of kidneys for general health, to raise awareness about the significance and consequences of congenital, hereditary and acquired kidney diseases, chronic kidney disease, to adopt a strategy, aimed at reducing the frequency, severity and impact of kidney diseases on health and life. The global nature of the problem of kidney disease in children and adults is evidenced by the fact that in 2011 the Political Declaration on Non-infectious diseases, approved at the summit of the World Healthcare Organization, included kidney disease under paragraph 19. Chronic kidney disease is the 6^th cause of death in the world. The annual campaign of World Kidney Day 2006–2023 poses medical and socio-economic problems of global importance and reminds that kidney diseases affecting children and adults are common, dangerous and curable. World Kidney Day – 2023, under the motto: Kidney Health for all — preparedness for the unexpected, support for the vulnerable discusses the impact of disasters, in particular the COVID-19 pandemic, on the provision of specialized nephrological care to the population and its resources around the world.