Introduction. Allogeneic hematopoietic stem cell transplantation (allo-HSCT) is a cell therapy method that is widely used in hematological malignancies, solid tumors, hemoglobinopathies, primary immunodeficiencies, and other congenital diseases. One of the serious complications after allo-HSCT is graft-versus-host disease (GVHD). Acute GVHD affects the skin, liver, and gastrointestinal (GI) tract. In this connection, endoscopic diagnosis acquires special importance in the comprehensive diagnosis of gastrointestinal GVHD. The aim of the work was to determine the relationship between the severity of the clinical course of acute GVHD, visualization of villous atrophy during ileocolonoscopy and morphological changes in the mucous membrane of the terminal ileum in children after allo-HSCT.

Materials and methods. There was conducted a retrospective study including forty eight 9 months–8 years patients who underwent allo-HSCT between 2020 and 2023. Depending on the presence and severity of atrophy. All 48 children were divided into four groups: group 1 included 18 (37.5%) patients with signs of pronounced atrophy according to endoscopy; group 2 included 6 (12.5%) children with mild villous atrophy; Group 3 included 7 (14.6%) patients without atrophy, but with signs of ileitis; group 4 was formed by 17 (35.4%) children without signs of inflammation of the mucous membrane of the terminal ileum. All subjects underwent ileocolonoscopy using Pentax high-resolution endoscopes (Japan), with a biopsy of the mucous membrane of the colon and ileum. Statistical data processing was performed using IBM SPSS Statistics 23 software.

Results. When comparing endoscopy data with the clinical picture, there was noted a tendency to identify a more severe degree of acute GVHD in children from groups 1 and 2 with endoscopic signs of villous atrophy. Acute grade 3 GVHD was diagnosed only in children with endoscopic signs of villous atrophy — in 16.7%. In the 3^rd and 4^th groups of patients, there was a tendency towards more frequent detection of acute GVHD grade 0, compared to children with signs of atrophy (25% versus 8.3%); however, the differences were statistically insignificant (p > 0.05). According to histology, in the majority of patients in groups 1 and 2 (21 out of 24/87.5%), ileoscopy revealed “false” villous atrophy. In the 3^rd group, not a single child showed signs of atrophy confirmed histologically, which was consistent with expectations. In 13 out of 17 (76.5%) patients of the 4^th group, histological examination showed no pronounced signs of inflammation. In 3 (17.6%) children of the 4^th group there were no morphological changes in the ileal mucosa.

Conclusion. The clinical significance of atrophy and the prognostic value of this criterion remain controversial, which indicates to the need for continued research in this area.

Contribution:
Shubina T.S., Lokhmatov M.M., Balashov D.N. — concept and design of the study;
Shubina T.S., Zakharov I.V., Abramov D.S. — data collection and processing;
Shubina T.S. — statistical processing of the data;
Shubina T.S. — writing the text;
Lokhmatov M.M., Balashov D.N. — editing.
All co-authors — approval of the final version of the article, responsibility for the integrity of all parts of the article.

Acknowledgment. The study had no sponsorship.

Conflict of interest. The authors declare no conflict of interest.

Received: July 04, 2024
Accepted: August 07, 2024
Published: September 16, 2024

Introduction. Impaired fetal growth is a risk factor for the development of non-communicable diseases and pathological conditions in adolescents and adults. The incidence of newborn children with growth disorders and fetal malnutrition (GDFM) is a criterion for determining the health of future generations and clarifying the directions of prevention. Objective: to determine the incidence of GDFM in neonates in the city of Ekaterinburg and the Sverdlovsk region (SR) in comparison with national indicators in 2012–2022.

Materials and methods. A descriptive epidemiological study of the GDFM prevalence in newborns infants in the Sverdlovsk region (SR) and Ekaterinburg over 2012–2022 was conducted. To calculate the indices, we used Form No. 32 of the Federal Statistical Observation «Information on medical care for pregnant women, women in labour, and maternity hospitals». The incidence in newborns was calculated as the ratio of the number of sick newborns to the number of live births and expressed in promille (‰).

Results. The GDFM incidence in Ekaterinburg decreased by 1.83 times (p < 0.001), in SR — by 1.63 times (p = 0.01), in the Russian Federation — by 1.48 times (p = 0.171) in 2012–2022. In Ekaterinburg, the proportion of premature newborns with GDFM increased 2.87 times in 2019 (p = 0.02). This explains the concentration of high-risk pregnant and parturient women in institutions of the third group.

Conclusion. The positive trend in the GDFM incidence in newborns in Ekaterinburg, the Sverdlovsk region, and the Russian Federation has been established.

Contribution:
Sevostyanova O.Yu. — concept and design of the study, writing the text;
Chumarnaya T.V. — statistical data processing, preparation of graphs;
Belomestnov S.R. — research concept, collection of material;
Ksenofontova O.L. — editing the text.
All co-authors — approval of the final version of the article, responsibility for the integrity of all parts of the article.

Acknowledgment. The research was supported by the RNF grant No. 24-25-20110.

Conflict of interest. The authors declare no conflict of interest.

Received: June 20, 2024
Accepted: August 07, 2024
Published: September 16, 2024

Introduction. Children suffering from congenital thrombophilia may suffer from manifestations of the increased bleeding in certain periods of childhood. With regard to the occurrence of acute cerebrovascular accident, infarctions, transient ischemic attacks, genetic polymorphisms of thrombophilia have been well studied, but they have not been fully analyzed in children with the aspects of hemorrhagic syndrome. The aim of the study: to assess the prognostic significance of permanent and temporary thrombogenic risk factors for the formation of a risk groups for the development of vascular complications in children with manifestations of hemorrhagic syndrome.

Materials and methods. The study included ninety 0 to 18 years children with clinical manifestations of hemorrhagic syndrome. The control group consisted of 115 children without clinical manifestations of bleeding. Determination of genetic polymorphisms of hemostasis system factors and folate cycle enzymes was carried out by the molecular genetic method, using the polymerase chain reaction. Statistical processing of the results was carried out using the statistical software package Statistica 10.0 (StatSoft Inc.) was used to organize, sort, and analyze data.

Results. Children with manifestation of hemorrhagic syndrome were established to have a statistically significant prevalence of carriage of the Hmzg genotype of the ITGA2 gene and the Hmzg gene PAI-1 (p < 0.05). The prognostic significance and informativeness of a number of constant and temporary signs were established, from which a prognostic table was compiled for the selection of children in the risk group for the development of vascular complications with the subsequent development of individual measures for primary thromboprophylaxis.

Conclusion. Thus, the developed prognostic table characterizes the maximum value of specificity, the maximum value of sensitivity and the prognostic randomness of a positive result. When working with the proposed table, no more than 5% of erroneous decisions can be made. Using this indicator, children with a risk of developing vascular diseases were identified and included in the regional register, with subsequent additional examination and specific thromboprophylaxis.

Contribution:
Strozenko L.A. — concept, research design;
Ponomarev V.S. — collection and processing of material, writing the text;
Lobanov Yu.F., Skudarnov E.V. — editing the text.
All co-authors — approval of the final version of the article, responsibility for the integrity of all parts of the article.

Acknowledgment. The study had no sponsorship.

Conflict of interest. The authors declare no conflict of interest.

Received: July 10, 2024
Accepted: August 07, 2024
Published: September 16, 2024

Introduction. In cases of osteoid osteoma (OO) of small sizes, visualization of tumor is difficult. In Russia, surgical removal under the control of an electron-optical converter (EOP) is often chosen for the treatment of OO, and only a few centers use CT control. The aim of this work is to identify the advantages and disadvantages of intraoperative imaging for surgical removal of OO in children using EOP and CT with navigation.

Materials and methods. Sixteen 5 to 18 years patients with a diagnosis of OO were treated. The patients were divided into 2 groups depending on the method of intraoperative imaging: group 1 — surgical removal of OO under the EOP control (n = 7), group 2 — removal under the control of CT with a navigation system (n = 6). Exclusion criterion: tumor location outside the upper third of the femur (n = 3). The following indicators were evaluated: radiation load over the entire treatment period, the volume of resection, and the presence of X-ray symptoms.

Results. In patients from group 2 with removal under the control of CT with navigation for the entire period of treatment, the radia­tion load was 5.24 ± 1.19 mSv, while in children from group 1 — 3.69 ± 1.28 mSv. In 4 patients, the diagnosis was not made. In 13 patients, CT revealed a focus of lytic destruction with a peripheral rim of sclerosis. The volume of postoperative defect of the bone defect in patients of group 1 was 5548 ± 1694.2 mm³, and in patients of group 2 — 2131.57 ± 290.301 mm³. It is obvious CT with a navigation system to be a highly sensitive method of detecting a tumor, allows minimizing the volume of bone tissue to be removed and instrumentally confirming the removal of OO.

Conclusion. Intraoperative CT with navigation has a number of advantages over EOP control due to the less volume of resection of healthy bone tissue, it becomes possible to obtain intraoperative confirmation of OO removal, without need for immobilization during the postoperative period.

Contribution:
Kavkovskaya Ya.I., Zherdev K.V. — the concept and design of the study;
Kavkovskaya Ya.I., Gladkov A.D. — preparation of the material and writing of the text;
Zherdev K.V. — editing.
All co-authors — approval of the final version of the article, responsibility for the integrity of all parts of the article.

Acknowledgment. The study had no sponsorship.

Conflict of interest. The authors declare no conflict of interest.

Received: July 03, 2024
Accepted: August 07, 2024
Published: September 16, 2024

Introduction. The issues of safe formation of primary intestinal anastomosis (PIA) in conditions of peritonitis and impaired intestinal hemoperfusion in children remains relevant. The “classic ban” on PIA in conditions of inflammation or ischemia is often passed down from generation to generation of surgeons without clear reasoning, scientific proofs, and understanding of the pathogenesis of complications. However, in a number of clinical situations, a pediatric or newborn surgeon is forced to use PIA approach when understanding and predicting the risks of complications of this surgical technique become important. This position requires clarification and scientific justification in modern clinical guidelines. Objective. To determine potential risk factors for complications of intestinal anastomosis in conditions of peritonitis and impaired intestinal hemoperfusion in children.

Materials and methods. The first stage was a comparative analysis of intestinal anastomosis outcomes in newborns and older children according to the data of the chief pediatric surgeons of 75 constituent entities of the Russian Federation, including the DPR and LPR (2021–2022). Also, the outcomes of intestinal anastomosis in groups of patients with “presence” and “absence” of peritonitis and impaired intestinal hemoperfusion were assessed.

The second stage was a multivariate analysis of clinical and laboratory signs in 11 children from different clinics of the Russian Federation. The PIA management in the conditions of peritonitis and impaired intestinal hemoperfusion in these patients was complicated by anastomotic failure or stenosis.

Results. PIA in conditions of peritonitis and impaired intestinal hemoperfusion was performed in 1207 children (26.48% of all intestinal anastomosis). Death was recorded in 21 (1.74%) patients, failure — also in 21 (1.74%), stenosis requiring reconstruction — in 42 (4.1%). The factor “presence of peritonitis and impaired intestinal hemoperfusion” in all children was determined to significantly increase only the incidence of intestinal anastomotic stenosis, requiring subsequent reconstruction (χ² test = 12.102; p < 0.01), and in newborn patients all differences in outcomes were statistically confirmed: mortality — χ² test = 23.235; p < 0.01, failure — χ² test = 29.790; p < 0.01, stenosis — χ² test = 42.344; p < 0, 01.

In newborns the risk of death, anastomotic failure, and stenosis in conditions of peritonitis or impaired intestinal hemoperfusion has also been scientifically proven to be significantly higher (p < 0.01) than in the group of older children. Multivariate analysis of data of patients treated by PIA in conditions of peritonitis or impaired intestinal hemoperfusion showed hypotension, hypovolemia, anemia, hypoalbuminemia, respiratory failure, and comorbidity to be recorded in the majority of patients. These patients required volume fluid therapy, transfusion therapy, and cardiotonic/vasopressor therapy.

Contribution:
Morozov D.A. — concept and design of the study, editing the text;
Novozhilov V.A., Barova N.K., Rozhdenkin E.A., Sharkov S.M., Hayrapetyan M.I., Kozlov M.Yu., Mordvin P.A., Stepanova N.M., Rekhviashvili M.G. — collection and processing of material, editing of the text;
Morozov K.D. — collection and statistical processing of the material, writing the text.
All co-authors — approval of the final version of the article, responsibility for the integrity of all parts of the article.

Acknowledgment. The study had no sponsorship.

Conflict of interest. The authors declare no conflict of interest.

Received: July 05, 2024
Accepted: August 07, 2024
Published: September 16, 2024

Psychological and pedagogical support is a significant area of ​​non-drug assistance in pediatric practice. The purpose of the work: to determine the effectiveness of the activities of specialists of the psychological and pedagogical profile in pediatrics for its optimization.

Materials and methods. An analysis of the federal legislative and regulatory framework regulating the activities of psychological and pedagogical specialists in pediatrics was carried out; medical and demographic indicators of the health status of the child population and the activities of medical institutions of the Russian Federation (RF) were analyzed using approved statistical forms; the results of the analysis of data from an online survey of heads of psychological and pedagogical services from ninety one medical institutions in 42 regions of the RF and 8 federal districts are summarized.

Results. According to the analysis of the legislative and regulatory framework, psychological and pedagogical support is one of the areas of non-drug assistance in pediatric practice. Taking into account the results of the analysis of health indicators in children and adolescents, the priority areas of activity of psychological and pedagogical specialists are: prevention of psychophysical overload in children (including children with health problems), assistance in creating psychologically safe conditions in the family and school; formation of sustainable motivation in the family to adhere to the principles of a healthy lifestyle and an attitude towards high medical activity; increasing the responsibility of parents for the life and health of children, educating adults in matters of upbringing and care, and observing the principles of life safety; assistance to parents and teachers in creating special conditions to meet the special educational and psychological needs for children with disabilities and disabled children, their most complete social adaptation.A study of the management of the activities of psychological and pedagogical assistance specialists revealed the content of the activities of psychological and pedagogical specialists to do not fully correspond to the needs for children and pediatric practice, despite a fairly good supply of psychological and pedagogical personnel in pediatrics.

Conclusion. Improving the management of the activities of psychological and pedagogical specialists consists of introducing a unified standard of care taking into account the health status in children and regulating the activities of a medical psychologist, neuropsychologist, speech pathologist, and medical speech therapist in pediatrics.

Contribution of authors:
Lazurenko S.B., Aprosimova S.I. — concept and design of the study;
Sviridova T.V., Gerasimova A.M. — collection and processing of material;
Konova S.R., Sviridova T.V. — statistical processing, writing the text;
Lazurenko S.B., Konova S.R. — editing the text.
All co-authors — approval of the final version of the article, responsibility for the integrity of all parts of the article.

Acknowledgment. The study had no sponsor support. Performed within the framework of the State Assignment № 075-00693-23-00 dated 27.12.2022 FGBU “Russian Academy of Education”.

Conflict of interest. The authors declare that there is no conflict of interest.

Received: June 29, 2024
Accepted: August 07, 2024
Published: September 16, 2024

Introduction. In the modern society, more and more attention is being paid to the quality of medical care and the protection of patients’ rights. The most important area of work of medical and nursing staff is the development of children’s healthcare and the provision of qualified personnel to medical institutions, and, in this regard, legal training and the ability to apply their knowledge in practice is a necessary component of successful occupational activity. Objective of the work: to determine the level of training and degree of interest in obtaining additional legal knowledge among nursing specialists in various regions of Russia.

Materials and methods. The results of the survey of nurses during the annual scientific and practical conferences “Filatov readings. Current issues of children’s health” over 2021–2023.

Results. At the first stage of the study, all respondents rated their level of general legal literacy as average (from 3 to 4 points), while self–assessment of preparedness in the field of medical law was significantly lower — from 2 to 3 points, i.e. insufficient. Also, all the respondents showed an extremely high interest in obtaining additional knowledge in the field of medical law. At the second stage of the study, respondents were shown to consider knowledge of patients’ rights to be the most important area of legal knowledge for their professional activities. Also in demand topics are: the main types of offenses in the field of healthcare, types of responsibility of medical workers, circumstances excluding the guilt of medical workers, as well as their rights and obligations.

Conclusion. Increasing the level of legal awareness of nursing staff will minimize the risk of violation of citizens’ rights when providing them with medical care, provided that medical workers faithfully perform their occupational duties, and also allow protecting themselves from unfounded accusations of patients, which is a guarantee in terms of protecting professional activities.

Acknowledgment. The study had no sponsorship.

Conflict of interest. The authors declare no conflict of interest.

Received: July 12, 2024
Accepted: August 07, 2024
Published: September,16 2024

Introduction. All current methods of hemostasis used in ovarian surgery inevitably lead to damage to healthy follicles. Significant loss of ovarian reserve in childhood can lead to the development of premature ovarian insufficiency in the future. Therefore, the choice of a method of hemostasis that is most gentle on ovarian tissue becomes particularly important in pediatric gynecology.

Materials and methods. Paper search and analysis was completed using the PubMed, Embase, Cochrane Library and Google Scholar databases. This literature review includes systematic reviews, cohort studies, case series, and randomized controlled trials (RCTs) from 1989 to 2024.

Results. All modern methods of hemostasis lead to a decrease in the ovarian reserve. The rate and potential of ovarian tissue repair depends on the method as well as the technique of hemostasis. According to the presented data, there is no unambiguous opinion between coagulation and ligature hemostasis methods when working with ovarian tissue. Coagulation hemostasis is technically easier and less time-consuming than suturing, especially for an inexperienced surgeon. Complications associated with technical errors during hemostasis can lead to a significant deterioration of the ovarian reserve in the long-term perspective. Thus, the appropriate method of hemostasis in each clinical situation is determined according to technical capabilities, skills, and preferences of the surgeon and the operation plan.

Conclusion. Careful preservation of ovarian reserve in pediatric practice is of paramount importance for the future reproductive health of female patients. When choosing a method of hemostasis, it is important to take into account the technical capabilities and skills of the surgeon. Further study is needed to assess the impact of modern hemostasis methods on ovarian reserve.

Contribution:
Sibirskaya E.V. — study concept and design, editing the text;
Sharkov S.M. — editing the text;
Nikiforova P.O. — analysis of the results, editing the article;
Gusarova O.I. — writing the text, review of publications on the topic of the article, analysis of the data obtained.
All co-authors — approval of the final version of the article, responsibility for the integrity of all parts of the article.

Acknowledgment. The study had no sponsorship.

Conflict of interest. The authors declare no conflict of interest.

Received: June 25, 2024
Accepted: August 07, 2024
Published: September 16, 2024

Ulcerative colitis is a chronic relapsing inflammatory disease of the colon with continuous spread of lesions from the rectum in the proximal direction. The etiology of the disease is unknown, but it is assumed to be developed as a result of a combination of several factors. The clinical picture is often similar to the picture of intestinal infection, which complicates the timely diagnosis of the disease, especially in children. Refractory to standard therapy limits therapeutic options and increases the frequency of surgical interventions. However, the emergence of new drugs, such as upadacitinib, opens up new opportunities for treating such patients and studying the effectiveness of therapy. We present a case of successful treatment of ulcerative colitis with upadacitinib in a child with the onset of the disease at the age of 6 months. The purpose of the description is to show the possibilities of using the janus kinase blocker Upadacitinib in severe ulcerative colitis in a child with a very early onset of the disease and without effect from previous therapy. The importance of early diagnosis and therapy is emphasized.

Contribution:
Usoltseva O.V., Potapov A.S., Anushenko A.O., Vinokurova A.V., Krasnovidova A.E., Zenkova K.I., Movsisyan G.B. — concept and design of the study;
Usoltseva O.V. — collection and processing of the material;
Usoltseva O.V., Vinokurova A.V. — statistical processing of the material;
Usoltseva O.V. — writing the text;
Potapov A.S. — editing the text.
All co-authors — approval of the final version of the article, responsibility for the integrity of all parts of the article.

Acknowledgment. The study had no sponsorship.

Conflict of interest. The authors declare no conflict of interest.

Received: July 12, 2024
Accepted: August 07, 2024
Published: September 16, 2024

Introduction. Generalized infantile arterial calcification (IAC) is a rare autosomal recessive disease. The diagnosis of IAC is associated with great difficulties due to the variability of clinical manifestations. The high mortality rate in patients at the onset of the disease in early childhood is characterized due to limited possibilities of drug treatment. Aim: to present two clinical reports concerning patients with a genetically verified diagnosis of IAC with long-term catamnestic follow-up (from 2016 to 2023).

Results. When coronary vessels are affected with IAC, pronounced ischemic changes are detected on the ECG with confirmed normal anatomy of the coronary arteries, which should alert clinicians and contribute to the inclusion of this form of pathology in the spectrum of differential diagnoses.

Conclusion. For the diagnosis of genetically determined IAC, it is extremely important to conduct molecular genetic studies using high-throughput sequencing methods.

Contribution:
Sdvigova N.A., Basargina E.N., Savostyanov K.V. — concept and design of the study;
Sdvigova N.A., Zharova O.P., Silnova I.V. — collection, processing of the material;
Sdvigova N.A., Basargina E.N., Gandaeva L.A. — writing the text;
Basargina E.N., Savostyanov K.V., Barskiy V.I. — editing the text.
All co-authors — approval of the final version of the article, responsibility for the integrity of all parts of the article.

Acknowledgment. The study had no sponsorship.

Conflict of interest. The authors declare no conflict of interest.

Received: June 28, 2024
Accepted: August 07, 2024
Published: September 16, 2024