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

{"title":"RNA analysis as a promising area of diagnostics in nephrology","authors":"S. L. Morozov, V. P. Pakhomova, V. Voinova, V. Dlin","doi":"10.21508/1027-4065-2023-68-4-24-xx","DOIUrl":"https://doi.org/10.21508/1027-4065-2023-68-4-24-xx","url":null,"abstract":"   Today, medicine is at a stage when traditional diagnostic tools no longer meet current needs, which has led to the rapid development of molecular diagnostic methods that not only complement traditional research methods but also provide insight from the point of view of molecular pathophysiology. mRNA analysis is a new field that could revolutionize the diagnosis and treatment of kidney disease. The study of mRNA can be used for diagnosing and classifying kidney diseases, predicting the progression of nephropathies, monitoring the effectiveness of treatment, and developing modern approaches to therapy. As a new field, mRNA analysis faces a number of challenges and limitations, but as technology advances, the technique becomes more accessible for clinical use.","PeriodicalId":21550,"journal":{"name":"Rossiyskiy Vestnik Perinatologii i Pediatrii (Russian Bulletin of Perinatology and Pediatrics)","volume":"176 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-09-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"82974736","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":"Cognitive development and social communication of premature low and extremely low weight at birth at age 24–36 month of life. Is it normal or pathology?","authors":"E. S. Keshishian, E. S. Sakharova","doi":"10.21508/1027-4065-2023-68-4-39-xx","DOIUrl":"https://doi.org/10.21508/1027-4065-2023-68-4-39-xx","url":null,"abstract":"","PeriodicalId":21550,"journal":{"name":"Rossiyskiy Vestnik Perinatologii i Pediatrii (Russian Bulletin of Perinatology and Pediatrics)","volume":"30 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-09-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"79435241","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":"The effect of the physical exercise on the redox status of chil- dren with repaired congenital heart diseases","authors":"A. Dubovaya, N. A. Usenko, E. V. Bordyugova","doi":"10.21508/1027-4065-2023-68-4-61-xx","DOIUrl":"https://doi.org/10.21508/1027-4065-2023-68-4-61-xx","url":null,"abstract":"   Purpose. Evaluation of the impact of the physical rehabilitation program with the inclusion of ubidecarenone on the redox status in children with repaired congenital heart diseases.   Material and methods. Markers of free radical oxidation (lactate, pyruvate, lactate-to-pyruvate ratio) and antioxidant system (catalase, reduced glutathione and glutathione peroxidase) were studied in 84 children, including 48 children with repaired congenital heart diseases and 36 healthy peers. The main group consisted of 28 patients who performed a rehabilitation program using physical activity in combination with taking ubidecarenone for a year, the comparison group consisted of 20 children who received only ubidecarenone.   Results. After the rehabilitation program was completed, both the main group and the comparison group had a significant (p < 0.05) decrease in lactate levels, the ratio of lactate to pyruvate, and an increase in catalase and reduced RBC glutathione. The group of children who performed physical exercises had significantly lower levels of markers of free radical oxidation and higher values of markers of the antioxidant system than the patients from the comparison group.   Conclusion. There are free radical oxidation and the antioxidant system impairments in children with repaired congenital heart diseases. The use of ubidecarenone reduces oxidative stress and increases antioxidant protection in children with repaired congenital heart diseases. The inclusion of physical activity in the rehabilitation program for children with repaired congenital heart diseases improves the redox status and increases the effectiveness of rehabilitation.","PeriodicalId":21550,"journal":{"name":"Rossiyskiy Vestnik Perinatologii i Pediatrii (Russian Bulletin of Perinatology and Pediatrics)","volume":"1 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-09-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"76057812","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":"Clinical adaptation and the state of innate and humoral im- munity of premature infants with extremely low body weight who received oropharyngeal administration of colostrum","authors":"G. Chistyakova, I. Remizova, A. A. Makhanek, D. Abakarova, P. A. Kadochnikova, S. V. Bychkova, A. Ustyuzhanin, K. P. Shakirova","doi":"10.21508/1027-4065-2023-68-4-32-xx","DOIUrl":"https://doi.org/10.21508/1027-4065-2023-68-4-32-xx","url":null,"abstract":"   Breast milk is the optimal food for premature infants. However, the serious condition of premature newborns requires a complex of primary resuscitation care in the maternity unit, which makes it impossible to carry out early attachment to the breast and start breastfeeding. Currently, the neonatal community is actively discussing the immune effects of an alternative method of oropharyngeal administration of colostrum on the child’s adaptation to extrauterine existence.   Purpose. To assess the clinical outcomes, the state of the monocytic immunity, the production of sIgA in coprofiltrates in premature infants with extremely low body weight who received colostrum in the first hours of life.   Material and methods. 39 premature infants with extremely low body weight who received oropharyngeal administration of colostrum were examined. The expression of CD14+CD282+, CD14+CD284+, CD14+HLA-DR, CD14+CD64+, CD14+CD11b+, CD14+CD11c+ monocytes and the phagocytic ability of mononuclear cells in blood serum were determined by laser flow cytometry. The secretory IgA concentration was assessed in coprofiltrates.   Results. In premature infants who did not receive oropharyngeal colostrum, there was an increase in leukocyte phagocytosis, the level of monocyte expression, which was due to an increased infectious morbidity. Mucosal immunity of children who received colostrum was characterized by an increased concentration of secretory IgA.   Conclusion. It was found that children who received oropharyngeal administration of colostrum have a faster correction of transient hypoglycemia after birth, a shorter duration of parenteral nutrition, greater body weight at the time of discharge, and a decrease in the incidence of infectious pathology.","PeriodicalId":21550,"journal":{"name":"Rossiyskiy Vestnik Perinatologii i Pediatrii (Russian Bulletin of Perinatology and Pediatrics)","volume":"17 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-09-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"89635175","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":"A child with newly diagnosed kidney disease and his family: determination of early factors of disease progression and features of nephroprotection","authors":"G. Makovetskaya, G. Poretskova, L. Mazur, O. A. Sedashkina, V. N. Barinov, S. N. Reshetova","doi":"10.21508/1027-4065-2023-68-3-99-106","DOIUrl":"https://doi.org/10.21508/1027-4065-2023-68-3-99-106","url":null,"abstract":"Chronic kidney disease and a decrease in its progression is a global health problem. However, the risk factors associated with the primary nosology of glomerular diseases of children have not been sufficiently studied.Purpose. To determine early risk factors for the progression of chronic kidney disease for more effective prevention of chronization of the process, based on the analysis of clinical and anamnestic data, the features of the manifestation of the primary developed glomerular pathology, and taking into account nosological forms.Material and methods. The experience of own clinical observations, retro- and prospective, related to the formation of chronic kidney disease of children with glomerular pathology is summarized. The study was conducted on the basis of the children’s uronephrological center of the Samara Regional Clinical Hospital named after V.D. Seredavin.Results. The universal risk factors are systematized: transferred critical conditions in the perinatal period with the development of acute kidney injury; prenatally established CAKUT syndrome; the presence of a genetic predisposition to kidney diseases, a complex of allergic diseases in the family; the carriage of persistent infections, hemolytic strains of streptococcus, social determinant, environmental conditions, etc. Specific early risk factors for disease progression, features of the onset of kidney pathology and biomarkers of progression that determine early kidney damage have been identified. The features of nephroprotection are formulated: interdisciplinarity with the inclusion of organizational, social components; operational communication with the outpatient link; the use of social networks for educational processes — the patient’s family, training of first-contact doctors.","PeriodicalId":21550,"journal":{"name":"Rossiyskiy Vestnik Perinatologii i Pediatrii (Russian Bulletin of Perinatology and Pediatrics)","volume":"50 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-07-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"91350742","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":"Hemophilia in children - modern paradigms for treatment and prevention","authors":"K. I. Grigoriev, L. Kharitonova, T. G. Plakhuta","doi":"10.21508/1027-4065-2023-68-3-107-116","DOIUrl":"https://doi.org/10.21508/1027-4065-2023-68-3-107-116","url":null,"abstract":"April 17 marks World Hemophilia Day. The date is dedicated to the birthday of Frank Schnabel, the founder of the World Federation of Hemophilia. And on this day and on other days, the medical community is called upon to draw attention to the problems of people suffering from blood clotting disorders — one of the central ones in hematology. Hemophilia, although one of the most common genetically determined blood coagulation disorders, is considered a RARE (orphan) disease. The disease manifests itself in early childhood, clinically has two of the most common variants of the disease: hemophilia A and hemophilia B. Diagnosis requires mandatory laboratory confirmation of the genetic absence or decrease in the concentration of FVIII for hemophilia A and FIX for hemophilia B. In recent years, the introduction of domestic recombinant coagulation factors into practice has made it possible to significantly optimize replacement therapy in children with hemophilia, and in the context of increasing Western sanctions, to ensure children of Russia with the necessary means of urgent care and prevention, which are not inferior to the best foreign analogues. New therapy principles that are actually changing the principles of treatment include non-factor drugs and gene therapy options for both hemophilia A and hemophilia B.","PeriodicalId":21550,"journal":{"name":"Rossiyskiy Vestnik Perinatologii i Pediatrii (Russian Bulletin of Perinatology and Pediatrics)","volume":"57 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-07-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"73313227","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":"Epigenetic markers of podocyte repair in children with primary nephrotic syndrome","authors":"S. L. Morozov, V. Dlin, V. Voinova, A. V. Smirnova, O. R. Piruzieva","doi":"10.21508/1027-4065-2023-68-3-68-76","DOIUrl":"https://doi.org/10.21508/1027-4065-2023-68-3-68-76","url":null,"abstract":"In terms of a glomerular injury research model, idiopathic nephrotic syndrome is the most prominent example. Worldwide, nephrotic syndrome is a socially significant disease and is considered one of the most common glomerulopathies in childhood, leading to the development of chronic kidney disease, and in the case of progression of the disease, to the formation of terminal chronic renal failure. This article presents data from our own study and discusses epigenetic markers of podocyte repair in children with primary nephrotic syndrome. We noted that in patients with steroid-sensitive variants of nephrotic syndrome, the expression of the WT1 (podocyte transcription factor) and NPHS1 (the gene encoding nephrin — a transmembrane protein that is a structural component of the slit diaphragm of the podocyte) genes is significantly higher than in the control group and in children with steroid resistant nephrotic syndrome. The data obtained open up prospects for the development of a personalized approach to the management of children with primary nephrotic syndrome by determining the markers of the staging and/or severity of the pathological process occurring in the glomeruli. Currently, genomic and post-genomic technologies are increasingly being used in clinical practice, which will eventually lead to the development of personalized diagnostic panels based on innovative technologies.","PeriodicalId":21550,"journal":{"name":"Rossiyskiy Vestnik Perinatologii i Pediatrii (Russian Bulletin of Perinatology and Pediatrics)","volume":"17 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-07-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"73638317","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":"Food allergy and anaphylaxis to walnut","authors":"A. A. Ivanov, N. Esakova, S. Zimin, V. V. Gorev, A. Pampura","doi":"10.21508/1027-4065-2023-68-3-117-123","DOIUrl":"https://doi.org/10.21508/1027-4065-2023-68-3-117-123","url":null,"abstract":"Among the wide variety of tree nuts, walnut is an allergen that deserves special attention in the context of acute allergic reactions, due to their severity, low inducing trigger dose, and minimal likelihood of developing tolerance. The overall consumption of walnut has been steadily increasing, while the importance of this allergen as a trigger for anaphylaxis is underestimated due to its often “hidden” presence in foods and the difficulty of trigger verification. This article presents the epidemiological aspects of walnut food allergy, provides current data on the molecular characteristics and properties of various allergen proteins, and their clinical significance for the development of anaphylaxis. The article is supplemented with two clinical cases of food anaphylaxis to walnuts from own clinical practice.","PeriodicalId":21550,"journal":{"name":"Rossiyskiy Vestnik Perinatologii i Pediatrii (Russian Bulletin of Perinatology and Pediatrics)","volume":"29 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-07-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"87336533","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":"Portal hypertension syndrome in children with autosomal recessive polycystic kidney disease with liver cysts and hepatic fibrosis","authors":"E. Andreeva, I. V. Dyug, L. G. Goryacheva, N. Savenkova","doi":"10.21508/1027-4065-2023-68-3-61-67","DOIUrl":"https://doi.org/10.21508/1027-4065-2023-68-3-61-67","url":null,"abstract":"Hepatic fibrosis, liver cysts, and portal hypertension are extrarenal manifestations that determine the prognosis of autosomal recessive polycystic kidney disease in children.Purpose. To assess the features of the manifestation and course of liver cystic disease and fibrosis, the development of portal hypertension in the follow-up medical history of children with autosomal recessive polycystic kidney disease.Material and methods. We studied 27 children with autosomal recessive polycystic kidney disease, with two children with a fatal outcome in the neonatal period excluded. 25 children 1–17 years old with autosomal recessive polycystic kidney disease were divided into 2 groups depending on the presence of portal hypertension syndrome. In the long-term follow-up 10 (40%) of 25 children with autosomal recessive polycystic kidney disease had no signs of portal hypertension (group 1), 15 (60%) children had portal hypertension syndrome (group 2). The long-term follow-up, clinical, genealogical, laboratory and instrumental (US, MRI/CT of kidney and abdominal cavity, liver elastography) research methods were used in the study. The diagnosis was confirmed by autopsy for 3 children.Results. There were no cases of changes in the liver and bile ducts characteristic of autosomal recessive polycystic kidney disease by prenatal ultrasound examination. From 27 children with autosomal recessive polycystic kidney disease, in 10 (37%) were diagnosed the liver fibrosis in the long-term follow-up, 22 (81%) had cystic enlargement of intrahepatic veins, of which 15 (68%) had polycystic liver disease, 3 (14%) had Caroli disease. All children with autosomal recessive polycystic kidney disease and portal hypertension syndrome had varicose veins of the esophagus and stomach according to esophagoduodenoscopy, 53% with indications for ligation of phlebectasia, 47% were diagnosed with thrombocytopenia, 67% with anemia, 100% with splenomegaly, 13% with esophageal-gastric bleeding.Conclusion. The differences in the initial manifestations of autosomal recessive polycystic kidney disease were revealed, while no differences in the incidence of hepatic fibrosis and liver cysts were found in 2 compared groups of children.","PeriodicalId":21550,"journal":{"name":"Rossiyskiy Vestnik Perinatologii i Pediatrii (Russian Bulletin of Perinatology and Pediatrics)","volume":"09 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-07-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"85982153","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":"Cardiovascular system damage in the late-onset Pompe disease","authors":"I. Leontieva, Yu. S. Isaeva, I. Miklashevich, S. A. Thermosesov","doi":"10.21508/1027-4065-2023-68-3-83-91","DOIUrl":"https://doi.org/10.21508/1027-4065-2023-68-3-83-91","url":null,"abstract":"The article presents a clinical case of metabolic hypertrophic cardiomyopathy against the background of a late-onset form of Pompe disease, illustrating the difficulties of differential diagnostic search for the cause of the disease. The clinical, laboratory and genetic aspects of the diagnosis of Pompe disease are highlighted. The features of laboratory diagnostics, the difficult path to the correct diagnosis and the appointment of enzyme replacement therapy are discussed. Much attention is paid to the clinical symptoms of the disease — the most significant damage to the cardiovascular system, there is no damage to the musculoskeletal sphere. Clinical picture of late Pompe disease is presented: cardiac rhythm and conduction disorders (ventricular preexcitation syndrome — multiple additional atrioventricular fenestrations), unstable ventricular tachycardia, supraventricular tachycardia, sinus node weakness syndrome. Considered approaches to the prevention of sudden cardiac death the patient underwent surgical treatment: radiofrequency ablation, endocardial implantation of a cardioverter defibrillator. Pathogenetic therapy for Pompe disease has been started.","PeriodicalId":21550,"journal":{"name":"Rossiyskiy Vestnik Perinatologii i Pediatrii (Russian Bulletin of Perinatology and Pediatrics)","volume":"5 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-07-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"74775410","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}