[Choledochal cysts: surgical treatment in newborns and infants]. / Kisty kholedokha: osobennosti khirurgicheskogo lecheniya u detei pervykh trekh mesyatsev zhizni.

OBJECTIVE: To improve postoperative outcomes in newborns and infants with choledochal cysts and to determine the indications for surgery. MATERIAL AND METHODS: There were 13 children aged 0-3 months with choledochal cyst who underwent reconstructive surgery between 2019 and 2023. In all children, choledochal cyst was associated with cholestasis. Acholic stool was observed in almost half of the group (n=7). All children underwent cyst resection and Roux-en-Y hepaticoenterostomy. RESULTS: Symptoms of cholestasis regressed in all patients. Mean surgery time was 128±27 min. There were no complications. Enteral feeding was started after 1-2 postoperative days, abdominal drainage was removed after 6.2±1.6 days. Mean length of hospital-stay was 16±3.7 days. Adequate bile outflow is one of the main principles. For this purpose, anastomosis with intact tissues of hepatic duct should be as wide as possible. Roux-en-Y loop should be at least 40-60 cm to prevent postoperative cholangitis. CONCLUSION: Drug-resistant cholestasis syndrome and complicated choledochal cysts (cyst rupture, bile peritonitis) are indications for surgical treatment in newborns and infants. When forming Roux-en-Y hepaticoenterostomy, surgeon should totally excise abnormal tissues of the biliary tract to prevent delayed malignant transformation.

[The adaptation of methodology of expert selection of indicators for integral evaluation of effectiveness and efficiency of organization of medical care support at the level of subject of the Russian Federation at the "pediatrics" profile].

The adaptation of methodology of expert selection of indicators for integral evaluation of efficiency of organization of medical care on profile "pediatrics" at the level of the Subject of the Russian Federation. The study was organized as expert survey using structured interview technique. At the first stage, primary selection of indicators carried out. At the second stage, selection of experts in accordance with inclusion criteria and assessment of level of expert competence was organized. Then, criteria of expert evaluation of significance of selected indicators in accordance with international principles applied in of bench-marking systems construction were formulated. The Kendall's concordance coefficient (W) was calculated for quantitative assessment of degree of agreement between the experts answers. The non-parametric Friedman test was applied to evaluate significance of differences. The indicators with high degree of agreement between opinions of experts and availability of statistical significance according concordance coefficient are recommended as indicators for assessment of efficiency of organization of medical care of children at the level of the Subject of the Russian Federation. The following indicators satisfy these requirements: mortality of children aged 0-17 years (per 100 000 of population of corresponding age), mortality of children aged 0-1 year (per 1000 live births), mortality of children aged 0-4 years (per 1000 live births), support of population with beds for children, percentage of children aged 0-17 years under dispensary observation with diagnosis established for the first time. The study permitted to select indices (indicators) that can be applied both for integral rating of the Subjects of the Russian Federation and in formation of Federal projects or departmental target programs in pediatrics. The study results can also be applied in development of system of monitoring efficiency of implementation of these projects and programs.

[The medical social characteristic of disabled children residing in nursing homes of the Russian Federation].

The purpose of the study is to identify and systematize urgent problems and proposals for implementing innovations in medical and social care of disabled children in nursing homes. The analysis of official data and results of epidemiological study concerning disabled children aged 0-17 years resigning in nursing homes permitted to identify dynamics of changes in age structure of observed contingent, causes of disability and accessibility and quality of medical care and psychological pedagogical assistance. It was established that total number of disabled children living in nursing homes and their part among all disabled children is stable. The most common pathology and cause of disability are mental and behavioral disorders, diseases of nervous system, congenital anomalies, deformities and chromosomal abnormalities, diseases of eye and its adnexa. The insufficient accessibility and low quality of various types of medical care (preventive examination, specialized, hospital, rehabilitation and palliative care) is established. At organizing education of children, difficulties occurred due to lacking of special conditions and technical means, availability and qualification of specialists of psychological and pedagogical profile. In most cases, inmates reside in buildings with high technical wear and impossibility of complex implementation of state programs requirements. The article presents set of proposals, including consolidation of subordination of medical support to regional medical centers, introduction of modern education technologies for disabled children, improvement of competence of psychological and pedagogical personnel, external audit of technical conditions and facilities of nursing homes, widespread application of potential of non-governmental organizations.

Genetic Landscape of Nephropathic Cystinosis in Russian Children.

Nephropathic cystinosis is a rare autosomal recessive disorder characterized by amino acid cystine accumulation and caused by biallelic mutations in the CTNS gene. The analysis methods are as follows: tandem mass spectrometry to determine the cystine concentration in polymorphonuclear blood leukocytes, Sanger sequencing for the entire coding sequence and flanking intron regions of the CTNS gene, multiplex PCR to detect a common mutation-a 57 kb deletion, and multiplex ligation-dependent probe amplification to analyze the number of exon copies in the CTNS gene. Haplotype analysis of chromosomes with major mutations was carried out using microsatellite markers D17S831, D17S1798, D17S829, D17S1828, and D17S1876. In this study, we provide clinical, biochemical, and molecular genetic characteristics of 40 Russian patients with mutations in the CTNS gene, among whom 30 patients were selected from a high-risk group of 85 people as a result of selective screening, which was carried out through cystine concentration measurement in polymorphonuclear blood leukocytes. The most common pathogenic variant, as in most described studies to date, was the 57 kb deletion, which represented 25% of all affected alleles. Previously non-described variants represented 22.5% of alleles. The founder effect in the Karachay and Chechen ethnic groups was shown for the following major variants: c.1015G > A and c.518A > G.

The using of the polymerase chain reaction for the detection of resistance genes in gram-negative bacteria in routine practice in a pediatric hospital.

Objective - assessment of RT-PCR for the detection of carbapenem-resistance genes in gram-negative bacteria. A total, 499 strains of gram-negative microorganisms isolated in two pediatric hospitals in 2019-2020 were studied. Species identification was performed using MALDI-ToF mass-spectrometry (Bruker Daltonics, Germany). Meropenem and imipenem minimal inhibitory concentration (MIC) was determined by E-test method (BioMerieux, France). The presence of acquired carbapenemase genes of IMP, NDM, VIM, KPC, OXA-48, OXA-23, OXA-40, OXA-58-groups was determined by RT-PCR. Klebsiella pneumoniae (34%), Escherichia coli (4%), Serratia marcescens (6%) and other members of Enterobacterales (6%), also gram-negative non-glucose-fermenting bacteria Acinetobacter baumannii (14%), Pseudomonas aeruginosa (36%) were found among selected strains. Carbapenemase production was found in 385 isolates (77%). The main mechanism determining carbapenem resistance in P. aeruginosa was the production of blaVIM (100%). A. baumanii strains harbored OXA-23 (55%) and OXA-40 (45%) carbapenemases. The major determinant of carbapenem resistance in K. pneumoniae isolates was OXA-48 carbapenemase, detected in 63% strains, 13% of the strains possessed blaNDM-group, 16% isolates had a combination of blaNDM-group and blaOXA-48-like. Carbapenemase of KPC-group was found in 8% K. pneumoniae strains. OXA-48 carbapenemase prevailed (95%) among S. marcescens strains. Most of E. coli isolates harbored metallo-beta-lactamase NDM (89%). Other members of Enterobacterales most often had OXA-48 carbapenemase (57%), 39% of the isolates carried blaNDM-group. In one strain, a combination of blaNDM-group and blaOXA-48-like was discovered. RT-PCR is a fast and reliable method for the detection of acquired carbapenemases and can be recommended for routine use in bacteriological laboratories.

IPSC-Derived Human Neurons with GCaMP6s Expression Allow In Vitro Study of Neurophysiological Responses to Neurochemicals.

The study of human neurons and their interaction with neurochemicals is difficult due to the inability to collect primary biomaterial. However, recent advances in the cultivation of human stem cells, methods for their neuronal differentiation and chimeric fluorescent calcium indicators have allowed the creation of model systems in vitro. In this paper we report on the development of a method to obtain human neurons with the GCaMP6s calcium indicator, based on a human iPSC line with the TetON-NGN2 transgene complex. The protocol we developed allows us quickly, conveniently and efficiently obtain significant amounts of human neurons suitable for the study of various neurochemicals and their effects on specific neurophysiological activity, which can be easily registered using fluorescence microscopy. In the neurons we obtained, glutamate (Glu) induces rises in [Ca2+]i which are caused by ionotropic receptors for Glu, predominantly of the NMDA-type. Taken together, these facts allow us to consider the model we have created to be a useful and successful development of this technology.

[PSYCHOLOGICAL CARE FOR SCHOOL-AGE PATIENTS WITH CORONAVIRUS INFECTION IN THE HOSPITAL].

For the first time in pediatric practice, the psychological state of 100 children (7-17 years old) with COVID-19 in the state of hospitalization was studied on the basis of the Scientific Medical Research Center of Children's Health of the Russian Ministry of Health Care and the directions of psychological and pedagogical assistance were determined. Analysis of medical and psychological-pedagogical documentation, conversation, observation, screening diagnostics were conducted. It was established that according to the totality of physical and psychological factors (the main psychological difficulty) patients can be grouped into the following groups: children in a severe physical state, with unstable manifestations of mental activity (n = 4; 4%); children in a predominantly moderately severe state with significant distress related to the disease (n = 48; 48%); children in a satisfactory physical state, with mild and mostly situational difficulties in adapting to the situation of hospitalization (n = 46; 46%); children in a satisfactory physical state, in a relatively stable positive psychological state (n = 2; 2%). Consideration of the severity of their physical and psychological condition and their basic psychological difficulties allows differentiated psychological aid to be rendered to children and teenagers in special conditions of the «red zone¼.

[The effect of medical social and social economic factors on development of disability in children].

The trends in economic situation requires periodic repetition of studies concerning qualitative evaluation of impact of medical, social and economic factors on population health at the regional level. The purpose of the study is to establish the relationship between the disability of children and the main medical, social and socioeconomic indices in the Russian Federation. The materials and methods. The official data on prevalence of disability in children of 0-17 years and the level of basic medical, social and socioeconomic indices were analyzed in the context of 83 subjects of the Russian Federation in 2017-2019. The demographic and epidemiological data and data on medical care support of children population were used as medical and social indices. The socioeconomic indices (unemployment rate, cash income, housing conditions and consumption of basic food products were used as the socioeconomic indices. The Spearman rank correlation method was used to determine the particular relationships. The results. It was established that the significant inter-regional variability of disability of children both in the Federal Okrugs and the subjects of The Russian Federation persists. The relationship of various degrees of intensity between the prevalence of disability of children in the subjects of the Russian Federation and many medical, social and socioeconomic factors such as demographic indices (fertility, mortality); level of primary and total morbidity of children of all age groups; medical care support (medical personnel, pediatric beds); unemployment level; average per capita cash income; percentage of population with cash incomes below cost of living; total area of residential premises that is on average per inhabitant; share of young families registered as needing housing from total number of families with children aged under 18 years; consumption of cheaper food. The conclusion. The obtained results are to be taken into account in the development of programs of preventing disability of children avoiding to place full responsibility on the health care system only.

Neuroprotective Potential of Peptides HFRWPGP (ACTH6-9PGP), KKRRPGP, and PyrRP in Cultured Cortical Neurons at Glutamate Excitotoxicity.

Glutamate (Glu) excitotoxicity, which accompanies brain ischemia or traumatic brain injury, is the leading mechanism of neuronal death. In the present work, we studied the effects of the peptides HFRWPGP (ACTH6-9PGP), KKRRPG, and PyrRP on the survival of cultured cortical neurons on the background of excitotoxic effect of Glu (100 µM). Biochemical (MTT/WST) and morphometric analyzes showed that, depending on the dose, ACTH6-9PGP and KKRRPGP protect neurons from the cells death, while PyrRP, conversely, enhances it. The neuroprotective effect of ACTH6-9PGP is accompanied by a slowdown in the development of delayed calcium dysregulation and synchronous mitochondrial depolarization. Among the studied peptides, only ACTH6-9PGP significantly increased the number of neurons that restored Ca2+ homeostasis after Glu was abolished. The influence of KKRRPGP was less pronounced, whereas PyrRP, on the contrary, reduced the number of neurons with low [Ca2+]i. Thus, this study revealed the high therapeutic significance of ACTH6-9PGP and allowed assessing the prospects for its possible clinical use.

[Studies on the function of external respiration in adults and children with anomalous position of the heart chordae]. / Dannye issledovaniia funktsii vneshnego dykhaniia u vzroslykh i detei s anomal'no raspolozhennymi khordami serdtsa.

AIM: To compare respiratory function in adults and children whose heart chordae were in anomalous position (AC) with reference to severity of heart connective tissue dysplasia syndrome. MATERIALS AND METHODS: The study included ninety one 6-14-year-olds and 125 adults with AC. The patients were divided into groups by the degree of atrioventricular valvular prolapse (AVVP). External respiration function was examined, and the loop flow-volume was registered by the pulmonological computer system. Inhalation bronchospasmolytic test with berotec was performed. RESULTS: Tracheobronchial dyskinesia related in degree to AVVP severity was found in 76 and 35% of the adults and children with AC and AVVP, respectively. The berotec test allowed more precise evaluation of tracheobronchial dyskinesia. CONCLUSION: Functional insufficiency of the connective tissue in patients with cardiac connective tissue dysplasia syndrome progresses with age.

[Effect of diucifon on the natural cytotoxic activity of splenic cells in old rats]. / Vliianie diutsifona na estestvennuiu tsitotoksicheskuiu aktivnost' kletok selezenki starykh myshei.

The results of comparative investigation of diucifon effect on the level of natural killer cell activity in young and old mice are presented. An increase in the natural cytotoxic activity, as indicated by 51Cr-release micro-test, was considerably higher in old than in young mice during 1-3 days after diucifon administration. However, after diucifon stimulation the level of natural killer cell activity in old mice was significantly lower than in intact young mice.