Rossiyskiy Vestnik Perinatologii i Pediatrii (Russian Bulletin of Perinatology and Pediatrics)最新文献

{"title":"Mitochondrial dynamics and the significance of its disturbances in the development of childhood diseases. Part II. Cardiological and endocrinological aspects","authors":"V. S. Sukhorukov, T. Baranich, A. V. Egorova, E. N. Fedorova, K. A. Skvortsova, D. A. Kharlamov, A. I. Krapivkin","doi":"10.21508/1027-4065-2024-69-2-12-18","DOIUrl":"https://doi.org/10.21508/1027-4065-2024-69-2-12-18","url":null,"abstract":"In recent years, the dynamics of mitochondrial transformations in cells have been of more concern to both representatives of basic science and researchers in the field of applied medicine. A growing number of observations demonstrate the important regulatory influence of mitochondrial dynamics on a variety of physiological and pathological processes in many, if not all, organ and tissue structures. The prospects for studying the features and regulators of these processes for understanding the pathogenesis of diseases, developing their new biomarkers, as well as treatment technologies seem increasingly significant. The purpose of this article is to review the facts obtained regarding mitochondrial dynamics, which, from the authors’ point of view, deserve the attention of pediatricians. The volume of relevant information turned out to be too extensive to fit into one article, which forced it to be divided into several successive publications. The second part provides information about the role of mitochondrial dynamics disturbances in the pathogenesis of cardiovascular and endocrine diseases in children.","PeriodicalId":21550,"journal":{"name":"Rossiyskiy Vestnik Perinatologii i Pediatrii (Russian Bulletin of Perinatology and Pediatrics)","volume":" 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-05-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140999054","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Epileptogenesis and neuroinflammation","authors":"P. L. Sokolov, A. I. Krapivkin","doi":"10.21508/1027-4065-2024-69-2-5-11","DOIUrl":"https://doi.org/10.21508/1027-4065-2024-69-2-5-11","url":null,"abstract":"Progress in the treatment of epilepsy in recent years has been determined mainly by the development and use of new anticonvulsants, as well as the search for new alternative methods for reducing epileptic seizures. The issue of pharmacoresistance remains relevant. This paper presents an analysis of information about neuroinflammation as a pathophysiological phenomenon, its possible role in epileptogenesis and the prospects for influencing the pathological process in epilepsy by influencing its elements. General data on epileptogenesis, the role of neuroinflammation in its formation and maintenance, the main cellular and humoral effectors of the neuroinflammatory process and the prospects for the development of new therapeutic approaches are presented.","PeriodicalId":21550,"journal":{"name":"Rossiyskiy Vestnik Perinatologii i Pediatrii (Russian Bulletin of Perinatology and Pediatrics)","volume":"105 4","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-05-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141002629","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Clinico-radiological characteristics of two patients with geroderma osteodysplasticum caused by new nucleotide variants in the GORAB gene","authors":"E. Merkuryeva, T. Markova, V. Kenis, А. Semyachkina, E. A. Nikolaeva, S. V. Bochenkov, A. Kuchina, O. Ryzhkova, N. A. Doroshchuk, E. Dadali","doi":"10.21508/1027-4065-69-1-86-93","DOIUrl":"https://doi.org/10.21508/1027-4065-69-1-86-93","url":null,"abstract":"   Geroderma osteodysplasticum (OMIM #231070) is a rare autosomal recessive disorder characterized by congenital wrinkled skin on the dorsal surface of the limbs and abdomen; marked osteoporosis in childhood. Pathogenic variants of the nucleotide sequence in the GORAB gene, which encodes the GORAB protein located in the Golgi apparatus and plays a crucial role in vesicle transport in the Golgi complex, lead to the disease. This is the first report of 2 patients with this disease in Russia. A detailed description of the clinical-radiological and genetic characteristics of these patients with new nucleotide variants in the GORAB gene in a compound heterozygous state is presented: c.170C>G (p.Ser57Ter) and c.790G>C (p.Ala264Pro); c.295C>T (p.Gln99Ter) and heterozygous deletion on chromosome 1 (chr1:g.170531967–170539494del), affecting exons 1 and 2 of the gene. The main clinical manifestations of the syndrome were congenital wrinkled skin, sagging cheeks, hypoplasia of the cheekbones, prognathism, muscle hypotonia and joint hypermobility. Radiological signs included congenital bilateral dislocation of the hip, compression fractures of the vertebral bodies, and disruption of the continuity of the growth plate of the distal femur and proximal tibia, referred to as “insertion.” The rarity of the syndrome, as well as the similarity of clinical manifestations with a heterogeneous group of hereditary diseases accompanied by decreased skin elasticity, leads to a late correct diagnosis. This necessitates the description of the clinical-genetic characteristics of the disease, the study of the dynamics of the formation of its phenotypic manifestations, and the methods of molecular-genetic diagnostics.","PeriodicalId":21550,"journal":{"name":"Rossiyskiy Vestnik Perinatologii i Pediatrii (Russian Bulletin of Perinatology and Pediatrics)","volume":"15 7","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-03-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140257867","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Erythema nodosum in children","authors":"K. Khaertynov, V. Anokhin, K. A. Makarova, S. V. Khaliullina, A. Bulatova, S. Y. Yumasheva","doi":"10.21508/1027-4065-2024-69-1-74-77","DOIUrl":"https://doi.org/10.21508/1027-4065-2024-69-1-74-77","url":null,"abstract":"   Erythema nodosum, which is often associated with infectious diseases, is one of the rare skin manifestations in the practice of pediatricians and pediatric infectious disease specialists.   Purpose. The purpose was to study the clinical and laboratory manifestations of erythema nodosum in children.   Material and methods. The study included 17 children with erythema nodosum aged 2 to 17 years who were hospitalized at the City Children’s Hospital No. 1 of Kazan.   Results. In 6 (35 %) children, elevated values of antistreptolysin-O were detected, in 5 (29 %) — high titers of antibodies to Salmonella, in 2 (12 %) cases — IgM to Chlamidia pneumonia, in 2 (12 %) other children — IgM to capsid antigen of EBV, in one case (6 %) — IgM to Mycoplasma pneumonia. Increased erythrocyte sedimentation rate and C-reactive protein level was observed in 15 (88 %) and 12 (70 %) children, respectively. In 12 (70 %) of 17 children, increased level of soluble fibrin-monomer complex was detected in the blood.   Conclusion. Erythema nodosum in children in most cases is associated with streptococcal infection and salmonellosis. The immune-inflammatory process in erythema nodosum is accompanied with an increase in the level of soluble fibrin-monomer complex in the blood.","PeriodicalId":21550,"journal":{"name":"Rossiyskiy Vestnik Perinatologii i Pediatrii (Russian Bulletin of Perinatology and Pediatrics)","volume":"39 2","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-03-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140257367","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Management of a child with portopulmonal hypertension due to cavernous transformation of the portal vein and sple- norenal anastomosis","authors":"E. Emelyanchik, N. G. Volf, T. V. Cherkashina, E. A. Anikina, I. Naumenko, L. N. Antsiferova, E. N. Kuzminykh","doi":"10.21508/1027-4065-2024-69-1-102-107","DOIUrl":"https://doi.org/10.21508/1027-4065-2024-69-1-102-107","url":null,"abstract":"   A clinical case of the development of portopulmonary hypertension in a child after splenorenal anastomosis is presented. The surgical intervention was performed to correct the congenital malformation — cavernous transformation of the portal vein, which led to portal hypertension and varicose veins of the esophagus and stomach. In the long-term postoperative period, pulmonary arterial hypertension, hypersplenism with bilinear cytopenia, and moderate liver failure developed. Combined therapy provided a temporary improvement of the patient status, therefore, during subsequent surgery, the size of the anastomosis was changed. This led to a sustained decrease in pulmonary artery blood pressure and clinical improvement in the boy’s condition.","PeriodicalId":21550,"journal":{"name":"Rossiyskiy Vestnik Perinatologii i Pediatrii (Russian Bulletin of Perinatology and Pediatrics)","volume":"89 S77","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-03-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140257153","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Mitochondrial dynamics and the significance of its disturbances in the development of childhood diseases. Part I. Physiological and neurological aspects","authors":"V. S. Sukhorukov, T. Baranich, A. V. Egorova, E. N. Fedorova, K. A. Skvortsova, D. A. Kharlamov, A. Krapivkin","doi":"10.21508/1027-4065-2024-69-1-25-33","DOIUrl":"https://doi.org/10.21508/1027-4065-2024-69-1-25-33","url":null,"abstract":"   In recent years, the dynamics of mitochondrial transformations in cells have been of more concern to both representatives of basic science and researchers in the field of applied medicine. A growing number of observations demonstrate the important regulatory influence of mitochondrial dynamics on a variety of physiological and pathological processes in many, if not all, organ and tissue structures. The prospects for studying the features and regulators of these processes for understanding the pathogenesis of diseases, developing their new biomarkers, as well as treatment technologies seem increasingly significant.   The purpose of this article is to review the facts obtained regarding mitochondrial dynamics, which, from the authors’ point of view, deserve the attention of pediatricians.   The volume of relevant information turned out to be too extensive to fit into one article, which forced it to be divided into several successive publications. The first part provides information about the main processes included in the concept of “mitochondrial dynamics,” the importance of maintaining the balance of the latter for ontogenesis and tissue homeostasis, as well as data on its disturbances in diseases of the nervous system in children.","PeriodicalId":21550,"journal":{"name":"Rossiyskiy Vestnik Perinatologii i Pediatrii (Russian Bulletin of Perinatology and Pediatrics)","volume":"11 7","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-03-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140260737","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Risk factors for the development of arterial hypertension in children and young people in present-day developments","authors":"G. A. Ignatenko, A. Dubovaya, Yu. V. Naumenko, A. P. Dudchak, V. V. Sosna","doi":"10.21508/1027-4065-2024-69-1-16-24","DOIUrl":"https://doi.org/10.21508/1027-4065-2024-69-1-16-24","url":null,"abstract":"   In modern society, there is an intensive increase in the incidence of hypertension among young people, which is mainly due to lifestyle features and associated risk factors.   Purpose. To analyze the results of Russian and foreign scientific research concerning the study of risk factors in the development of hypertension in children and young people.   Material and methods. A qualitative analysis of the current data available in the literature on the risk factors of hypertension and its prevention has been carried out.   Results. It has been established that the occurrence of arterial hypertension in childhood is influenced not only by hereditary factors, but also by a number of environmental risk factors.   Conclusion. Further research is needed to study new and established risk factors that need to be considered when conducting primary prevention of the disease in children and young people from the high-risk group.","PeriodicalId":21550,"journal":{"name":"Rossiyskiy Vestnik Perinatologii i Pediatrii (Russian Bulletin of Perinatology and Pediatrics)","volume":"24 4","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-03-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140259794","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Retracted: Drug Utilization Pattern of Antibiotics in Neonatal intensive care unit in a Tertiary Care Hospital, India","authors":"V. G Doddawad","doi":"10.21508/1027-4065-68-6-127-131","DOIUrl":"https://doi.org/10.21508/1027-4065-68-6-127-131","url":null,"abstract":"Article retractedMillions of babies are admitted to neonatal intensive care units worldwide, with a significant proportion facing various indications. Given that sepsis is a leading cause of mortality and morbidity among infants globally, including in India, the importance of efficient antibiotic use cannot be overstated. As the global problem of drug resistance intensifies, prudent antibiotic usage becomes crucial in mitigating treatment failures and ensuring the survival of newborns. Aim: to evaluate the pattern of antimicrobial drug utilization in neonatal intensive care unit (NICU) to assess the criteria for drug selection in tertiary care hospital.Materials and Methods. It was an observational prospective study conducted for six months in which 200 neonates admitted to neonatal intensive care unit at tertiary care Hospital, were included. Demographic details, duration of hospital stay, reason for admission (disease pattern), various drugs prescribed were noted. Results. Out of 200 neonates the number of male babies admitted to neonatal intensive care unit were 115 (57.5%) and female babies were 85 (42.5%). Many of the neonates were from age group of 1 to 5 (58.5%) days. Most of the neonates were from weight group of 2.5 to 3 (26.5%) kg. Majority of neonates hospital admission duration was 3 to 6 days is 98 (49%) neonates. Most common disorder requiring neonatal intensive care unit admission was Preterm with Respiratory Distress Syndrome (29%) followed by Neonatal Sepsis (19.5%) and Preterm (11%). Other distress reported include birth asphyxia (16 neonates or 8%), Neonatal convulsions (12 neonates or 6%), Late preterm (13 neonates or 6.5%), Hypernatremia (9 neonates or 4.5%), Meconium Aspiration Syndrome (3 neonates or 1.5%), Neonatal Hypoglycemia (5 neonates or 2.5%) and others (23 neonates or 11.5%). Among 423 antibiotics most prescribed antibiotic was Inj. Piperacillin + Tazobactam (35.4%) followed by Gentamycin (30.2%). Out of 423 drugs 152 are fixed dose combination and single drugs are 271 in this study in which piperacillin + tazobactam of fixed dose combination and gentamicin dose combination and single drugs are 271 in this study in which piperacillin + tazobactam of fixed dose combination and gentamicin of single drug had prescribed in more amount.Conclusion. The findings of this study will contribute to the existing body of knowledge regarding the appropriate use of antibiotics in the NICU, thereby providing insights for healthcare professionals to make informed decisions regarding antibiotic therapy. Empirical antibiotics were administered to every newborn based on their individual conditions. The antibiotic utilization pattern within this study group was determined to be rational.","PeriodicalId":21550,"journal":{"name":"Rossiyskiy Vestnik Perinatologii i Pediatrii (Russian Bulletin of Perinatology and Pediatrics)","volume":"16 8","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-01-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139531114","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Algorithm for early diagnosis, prevention and management of children with sick sinus syndrome and limitary bradycardia","authors":"E. B. Polyakova","doi":"10.21508/1027-4065-2023-68-6-132-136","DOIUrl":"https://doi.org/10.21508/1027-4065-2023-68-6-132-136","url":null,"abstract":"The article presents algorithm for early diagnosis and prevention of sick sinus syndrome in children considering the degree and stability of bradycardia that demonstrated management of patient during the progression of sick sinus syndrome.","PeriodicalId":21550,"journal":{"name":"Rossiyskiy Vestnik Perinatologii i Pediatrii (Russian Bulletin of Perinatology and Pediatrics)","volume":"26 4","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-01-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139530990","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Pathophysiological mechanisms and clinical consequences of exposure of the fetus and newborn to new psychoactive substances — “bath salts”","authors":"E. Loshkova, I. V. Doroshenko, T. S. Lyulka, Y. S. Rafikov, V. Zhelev, S. P. Ermolenko, E. V. Mikhalev, I. R. Grishkevich, N. E. Melnikov, A. L. Solnyshko, A. A. Bogunetsky, E. I. Kondratieva, A. I. Khavkin, N. D. Odinaeva, E. I. Makarevich","doi":"10.21508/1027-4065-68-6-85-93","DOIUrl":"https://doi.org/10.21508/1027-4065-68-6-85-93","url":null,"abstract":"The use of new psychoactive substances (NPS) is increasing every year among various social groups around the world. The use of narcotic drugs during pregnancy, according to various authors, remains at a high level from 2.8% to 7% of pregnant women. The use of new psychoactive substances during pregnancy is characterized by multi-organ damage to the fetus, like other groups of narcotic drugs, however, no organ and tissue specificity for new psychoactive substances has been described. Experimental studies have demonstrated negative pathophysiological mechanisms triggered by synthetic cathinones in the form of increased proapoptotic activity, the formation of autophagolysosomes and reactive oxygen species, and the pro-inflammatory orientation of the immune system. A clinical observation describes a predominant lesion of the nervous system (malformations of the brain, damage to the respiratory center) and the musculoskeletal system (pronounced myotonic syndrome, congenital pathological fractures of the femur), which leads to multiorgan dysfunction, an uncontrolled inflammatory response, and, as a result, to the development of severe disability in such children and growth of the cohort of palliative pediatric patients. In the presented literature review, the authors focus on the pathophysiological mechanisms of action of NPS for a better, deeper and more holistic understanding of the pathological process occurring in the body, in order to form and improve the clinical thinking of medical specialists and cite their own clinical observation as an illustration of the consequences of using new psychoactive substances during pregnancy. The authors believe that this review of the literature with a description of a clinical case is valuable in terms of practical applicability, both for clinicians in various fields and for researchers.","PeriodicalId":21550,"journal":{"name":"Rossiyskiy Vestnik Perinatologii i Pediatrii (Russian Bulletin of Perinatology and Pediatrics)","volume":"32 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-01-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139530700","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}