Pub Date : 2023-01-01DOI: 10.20953/1817-7646-2023-1-55-61
N. Odinaeva, E. Kondratyeva, T. Maksimycheva
In recent years, great progress has been associated with the use of targeted therapy (since 2012 in world practice, since 2021 in the Russian Federation) in the treatment of cystic fibrosis. The use of pathogenetic therapy improves the nutritional status and functional state of the lungs. However, the problem of nutritional insufficiency in a number of patients with cystic fibrosis still persists. Objective. To evaluate the dynamics of anthropometric indicators and body composition using bioimpedance analysis in children with cystic fibrosis against the background of dietary correction using a semi-elemental hypercaloric product. Material and methods. The analysis of the results of the examination of patients meeting the inclusion criteria was carried out retrospectively according to the primary medical documentation. The program included only the primary medical documentation of patients, containing all the data necessary for the assessment of primary and secondary points. Design: single-center, retrospective in one group. Results. The results of observation of 20 patients with a BMI of less than 50 percentile showed that the use of Peptamen Junior 1.5 formula for 90 ± 7 days increased BMI by an average of 0.6 kg/m2 and the proportion of skeletal muscle mass in lean body weight (SMM, %) by 1.3%. Conclusion. A retrospective analysis of the use of a semi-elemental liquid enteral nutrition product Peptamen Junior 1.5 for the correction of nutritional deficiency in children with cystic fibrosis aged 5 to 10 years indicates its clinical effectiveness for this category of patients. Key words: cystic fibrosis, children, semi-elemental formula, enteral nutrition, intestinal syndrome, Peptamen Junior 1.5, malabsorption
{"title":"The first experience of using a ready-to-use liquid form of a hypercaloric semi-elemental product for enteral nutrition in children with cystic fibrosis","authors":"N. Odinaeva, E. Kondratyeva, T. Maksimycheva","doi":"10.20953/1817-7646-2023-1-55-61","DOIUrl":"https://doi.org/10.20953/1817-7646-2023-1-55-61","url":null,"abstract":"In recent years, great progress has been associated with the use of targeted therapy (since 2012 in world practice, since 2021 in the Russian Federation) in the treatment of cystic fibrosis. The use of pathogenetic therapy improves the nutritional status and functional state of the lungs. However, the problem of nutritional insufficiency in a number of patients with cystic fibrosis still persists. Objective. To evaluate the dynamics of anthropometric indicators and body composition using bioimpedance analysis in children with cystic fibrosis against the background of dietary correction using a semi-elemental hypercaloric product. Material and methods. The analysis of the results of the examination of patients meeting the inclusion criteria was carried out retrospectively according to the primary medical documentation. The program included only the primary medical documentation of patients, containing all the data necessary for the assessment of primary and secondary points. Design: single-center, retrospective in one group. Results. The results of observation of 20 patients with a BMI of less than 50 percentile showed that the use of Peptamen Junior 1.5 formula for 90 ± 7 days increased BMI by an average of 0.6 kg/m2 and the proportion of skeletal muscle mass in lean body weight (SMM, %) by 1.3%. Conclusion. A retrospective analysis of the use of a semi-elemental liquid enteral nutrition product Peptamen Junior 1.5 for the correction of nutritional deficiency in children with cystic fibrosis aged 5 to 10 years indicates its clinical effectiveness for this category of patients. Key words: cystic fibrosis, children, semi-elemental formula, enteral nutrition, intestinal syndrome, Peptamen Junior 1.5, malabsorption","PeriodicalId":38157,"journal":{"name":"Voprosy Prakticheskoi Pediatrii","volume":"1 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"67750461","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}
Pub Date : 2023-01-01DOI: 10.20953/1817-7646-2023-2-65-75
E. Dolgova, N. Bodunova, A. I. Khavkin, M. Litvinova
Significant changes have taken place in cardiology over the past 15 years within the framework of a personalized approach to the management of patients with cardiovascular diseases. This review provides up-to-date information on the genetic aspects of the development of various cardiovascular diseases, including heart and vascular malformations, cardiomyopathy, cardiac arrhythmias, dyslipidemia, arterial hypertension, thrombosis, and some others. In the current paper cardiovascular pathologies are divided according to the etiological principle into the chromosomal, monogenic and polygenic diseases. The problems of introducing intensively developing genetic technologies and medical genetic counseling into the practice of cardiologists, as well as the appropriate and rational usage and interpretation of genetic tests, remain actual issue. The necessity of a multidisciplinary approach in the management of cardiological patients with close cooperation of a cardiologist and a clinical geneticist is shown. Key words: cardiogenetics, hereditary diseases, cardiology, cardiovascular diseases, chromosomal diseases, gene diseases, congenital heart defects, cardiomyopathy, cardiac arrhythmia, cardiomyopathy, arterial hypertension, thrombosis, personalized medicine, mutations, polymorphisms
{"title":"Utility of personalized medicine in cardiology practice","authors":"E. Dolgova, N. Bodunova, A. I. Khavkin, M. Litvinova","doi":"10.20953/1817-7646-2023-2-65-75","DOIUrl":"https://doi.org/10.20953/1817-7646-2023-2-65-75","url":null,"abstract":"Significant changes have taken place in cardiology over the past 15 years within the framework of a personalized approach to the management of patients with cardiovascular diseases. This review provides up-to-date information on the genetic aspects of the development of various cardiovascular diseases, including heart and vascular malformations, cardiomyopathy, cardiac arrhythmias, dyslipidemia, arterial hypertension, thrombosis, and some others. In the current paper cardiovascular pathologies are divided according to the etiological principle into the chromosomal, monogenic and polygenic diseases. The problems of introducing intensively developing genetic technologies and medical genetic counseling into the practice of cardiologists, as well as the appropriate and rational usage and interpretation of genetic tests, remain actual issue. The necessity of a multidisciplinary approach in the management of cardiological patients with close cooperation of a cardiologist and a clinical geneticist is shown. Key words: cardiogenetics, hereditary diseases, cardiology, cardiovascular diseases, chromosomal diseases, gene diseases, congenital heart defects, cardiomyopathy, cardiac arrhythmia, cardiomyopathy, arterial hypertension, thrombosis, personalized medicine, mutations, polymorphisms","PeriodicalId":38157,"journal":{"name":"Voprosy Prakticheskoi Pediatrii","volume":"1 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"67751367","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}
Pub Date : 2023-01-01DOI: 10.20953/1817-7646-2023-1-62-71
D. Polyakov, A. Voronkova, E. Savlevich, A. Gerasimov, E. Kondratyeva
One of the main factors determining the severity of the course and prognosis in cystic fibrosis (CF) is the severity of the infectious and inflammatory process and the species composition of the microflora in the lower respiratory tract (LRT). Studies show the important role of the nasal cavity and paranasal sinuses as a source and reservoir of microflora. Objective. To assess the species composition and correlation of microflora from the various parts of the upper and lower respiratory tract in children with cystic fibrosis. Patients and methods. 94 patients aged 2 to 18 years diagnosed with chronic rhinosinusitis with cystic fibrosis which had surgical intervention were included. The species composition of microflora was obtained from paranasal sinuses and nasal cavity, evaluated intraoperatively, from trachea during intubation in comparison with the actual (1-3 weeks before the intervention ) results of bacteriological test of induced sputum and / or material from the posterior pharyngeal wall, as well as the most "clinically significant" microflora in the anamnesis. Statistical analysis was carried out in order to search for correlations of the species composition of the bacterial flora between these parts of the respiratory tract. Results. The analysis showed no correlation between the microflora of different parts, except for the correlation between the most "clinically significant" microflora in anamnesis and obtained intraoperatively from paranasal sinuses in relation to two prognostically unfavorable species of bacteria: Sternotrophomonas maltophilia ( p = 0.001) and Pseudomonas aeruginosa (p = 0.047). In 13 (81.3%) of the 16 patients which had in anamnesis P. aeruginosa and was isolated from the maxillary sinus intraoperatively, its eradication was ascertained. Conclusion. The revealed positive correlation between the isolation of P. aeruginosa ever in anamnesis from low respiratory tract (LRT) and its presence in the paranasal sinuses during intraoperative sampling of the material indicates the important role of paranasal sinuses as a reservoir of pathogenic microflora and the possibility of long-term preservation of infectious agents after eradication courses of treatment and the achievement of eradication. Keywords: cystic fibrosis, children, chronic rhinosinusitis, microflora, Pseudomonas aeruginosa, paranasal sinuses
{"title":"Microbiological characteristics of chronic rhinosinusitis in children with cystic fibrosis comparing with other parts of the respiratory tract","authors":"D. Polyakov, A. Voronkova, E. Savlevich, A. Gerasimov, E. Kondratyeva","doi":"10.20953/1817-7646-2023-1-62-71","DOIUrl":"https://doi.org/10.20953/1817-7646-2023-1-62-71","url":null,"abstract":"One of the main factors determining the severity of the course and prognosis in cystic fibrosis (CF) is the severity of the infectious and inflammatory process and the species composition of the microflora in the lower respiratory tract (LRT). Studies show the important role of the nasal cavity and paranasal sinuses as a source and reservoir of microflora. Objective. To assess the species composition and correlation of microflora from the various parts of the upper and lower respiratory tract in children with cystic fibrosis. Patients and methods. 94 patients aged 2 to 18 years diagnosed with chronic rhinosinusitis with cystic fibrosis which had surgical intervention were included. The species composition of microflora was obtained from paranasal sinuses and nasal cavity, evaluated intraoperatively, from trachea during intubation in comparison with the actual (1-3 weeks before the intervention ) results of bacteriological test of induced sputum and / or material from the posterior pharyngeal wall, as well as the most \"clinically significant\" microflora in the anamnesis. Statistical analysis was carried out in order to search for correlations of the species composition of the bacterial flora between these parts of the respiratory tract. Results. The analysis showed no correlation between the microflora of different parts, except for the correlation between the most \"clinically significant\" microflora in anamnesis and obtained intraoperatively from paranasal sinuses in relation to two prognostically unfavorable species of bacteria: Sternotrophomonas maltophilia ( p = 0.001) and Pseudomonas aeruginosa (p = 0.047). In 13 (81.3%) of the 16 patients which had in anamnesis P. aeruginosa and was isolated from the maxillary sinus intraoperatively, its eradication was ascertained. Conclusion. The revealed positive correlation between the isolation of P. aeruginosa ever in anamnesis from low respiratory tract (LRT) and its presence in the paranasal sinuses during intraoperative sampling of the material indicates the important role of paranasal sinuses as a reservoir of pathogenic microflora and the possibility of long-term preservation of infectious agents after eradication courses of treatment and the achievement of eradication. Keywords: cystic fibrosis, children, chronic rhinosinusitis, microflora, Pseudomonas aeruginosa, paranasal sinuses","PeriodicalId":38157,"journal":{"name":"Voprosy Prakticheskoi Pediatrii","volume":"1 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"67750077","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}
Pub Date : 2023-01-01DOI: 10.20953/1817-7646-2023-1-136-143
D. Ivanov, Y. Petrenko, A. Zavyalova
The spectrum of neonatal diagnostic methods for severe hereditary disorders has been expanding since December 31, 2022. In most cases, causal therapy, including diet therapy, can prevent early death and disability in children. X-linked adrenoleukodystrophy (X-ALD) is an inherited disorder of peroxisomal fatty acid oxidation characterized by accumulation of very long-chain saturated fatty acids. There are three clinical forms of this disease. The first one is infantile cerebral form accounting for 48% of all cases. Its onset is usually observed at the age of 2.5–10 years after normal development of a child, manifesting with progressive decortication. The second form is adrenomyelopathy (25% of cases) which starts with adrenal insufficiency at the age of 12–55 years, followed by neurological symptoms. The adult form (3% of cases) manifests after the age of 30 years and combines clinical phenotypes resembling schizophrenia with dementia. A low-fat diet with Lorenzo oil is believed to be effective, because it normalizes plasma levels of C24:0 and C26:0 in X-ALD patients; however, it does not prevent or alleviate neurological symptoms if they have already developed. Diet therapy increases plasma levels of C22:1 and C24:1 fatty acids, although their effect on X-ALD is yet to be elucidated. This review describes the mechanism of Lorenzo oil action, its side effects, case series, and analysis of dietary correction in various groups of patients. A modified diet with dietary fats replaced by Lorenzo oil is effective only in people with asymptomatic X-ALD, as a preventive measure for asymptomatic boys at risk of developing the cerebral form. Lorenzo oil is capable of alleviating and preventing ALD progression in patients without brain lesions. However, in symptomatic patients, the restriction of dietary fats and their replacement by Lorenzo oil is associated with a high risk of life-threatening complications. In this case, nutrition should meet special requirements of a patient, for example, the ability to chew and swallow, while the therapy should be primarily substitutive and symptomatic. Key words: adrenoleukodystrophy, diet therapy, Lorenzo oil
{"title":"Lorenzo oil: a panacea, palliative care, or marketing. Opinion of a neonatologist, pediatrician, and nutritionist","authors":"D. Ivanov, Y. Petrenko, A. Zavyalova","doi":"10.20953/1817-7646-2023-1-136-143","DOIUrl":"https://doi.org/10.20953/1817-7646-2023-1-136-143","url":null,"abstract":"The spectrum of neonatal diagnostic methods for severe hereditary disorders has been expanding since December 31, 2022. In most cases, causal therapy, including diet therapy, can prevent early death and disability in children. X-linked adrenoleukodystrophy (X-ALD) is an inherited disorder of peroxisomal fatty acid oxidation characterized by accumulation of very long-chain saturated fatty acids. There are three clinical forms of this disease. The first one is infantile cerebral form accounting for 48% of all cases. Its onset is usually observed at the age of 2.5–10 years after normal development of a child, manifesting with progressive decortication. The second form is adrenomyelopathy (25% of cases) which starts with adrenal insufficiency at the age of 12–55 years, followed by neurological symptoms. The adult form (3% of cases) manifests after the age of 30 years and combines clinical phenotypes resembling schizophrenia with dementia. A low-fat diet with Lorenzo oil is believed to be effective, because it normalizes plasma levels of C24:0 and C26:0 in X-ALD patients; however, it does not prevent or alleviate neurological symptoms if they have already developed. Diet therapy increases plasma levels of C22:1 and C24:1 fatty acids, although their effect on X-ALD is yet to be elucidated. This review describes the mechanism of Lorenzo oil action, its side effects, case series, and analysis of dietary correction in various groups of patients. A modified diet with dietary fats replaced by Lorenzo oil is effective only in people with asymptomatic X-ALD, as a preventive measure for asymptomatic boys at risk of developing the cerebral form. Lorenzo oil is capable of alleviating and preventing ALD progression in patients without brain lesions. However, in symptomatic patients, the restriction of dietary fats and their replacement by Lorenzo oil is associated with a high risk of life-threatening complications. In this case, nutrition should meet special requirements of a patient, for example, the ability to chew and swallow, while the therapy should be primarily substitutive and symptomatic. Key words: adrenoleukodystrophy, diet therapy, Lorenzo oil","PeriodicalId":38157,"journal":{"name":"Voprosy Prakticheskoi Pediatrii","volume":"1 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"67749732","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}
Pub Date : 2023-01-01DOI: 10.20953/1817-7646-2023-1-80-88
L. Klimov, V. A. Kuryaninova, A. Yagupova, E. A. Cherkasova, I. Zakharova, S. Dolbnya, T. A. Ivenskaya, L. Abramskaya, S. Kashnikova, M. Stoyan, G. Utkina, A. A. Karaikozova, I. A. Gavrisheva
Objective. To analyze the effectiveness of correction of hypovitaminosis D and the dynamics of bone metabolism indicators in children with celiac disease against the background of exogenous dosing with cholecalciferol preparations. Patients and methods. This study included 105 children with celiac disease aged 2–17 years. All patients were divided into two groups: group 1 received vitamin D3 supplements and group 2 did not receive any therapy. The levels of vitamin D, osteocalcin, C-terminal telopeptide (CTX), and parathyroid hormone (PTH) were assessed in all children. Results. An increase in median vitamin D levels up to 32.4 [23.3; 39.7] ng/mL was observed against the background of taking cholecalciferol supplements, which was 2.5 times higher than in the same patients before treatment – 11.1 [5.9; 17.3] ng/mL (p < 0.001); meanwhile, the number of children with optimal calcidiol levels increased to 54.3% (p < 0.001), and the proportion of children with deficiency conditions decreased to 14.3% (p < 0.001). Positive dynamics of serum 25(OH)D levels were observed in 100% of cases. Against the background of vitamin D therapy, there was a decrease in CTX and PTH levels and an increase in osteocalcin levels. In the absence of therapy, a decrease in vitamin D levels from baseline of 25.7 [17.2; 35.1] ng/mL (p < 0.001) was noted. The proportion of patients with normal vitamin D levels decreased to 5.7% (p < 0.001); the number of children with deficiency increased to 85.7% (p < 0.001). There was a decrease in osteocalcin levels and an increase in CTX and PTH levels. Conclusion. Against the background of decreasing vitamin D levels and in the absence of its supplementation in children with celiac disease, osteoresorption processes prevail. Children with celiac disease may receive cholecalciferol supplementation in higher doses, which will avoid the development of osteopenia symptoms and significantly improve the quality of life. Key words: vitamin D, bone tissue, bone mineralization disorders, osteocalcin, osteoporosis, parathyroid hormone, C-terminal telopeptide
{"title":"Dynamics of parathyroid hormone levels and bone metabolism indicators against the background of hypovitaminosis D treatment in children with celiac disease","authors":"L. Klimov, V. A. Kuryaninova, A. Yagupova, E. A. Cherkasova, I. Zakharova, S. Dolbnya, T. A. Ivenskaya, L. Abramskaya, S. Kashnikova, M. Stoyan, G. Utkina, A. A. Karaikozova, I. A. Gavrisheva","doi":"10.20953/1817-7646-2023-1-80-88","DOIUrl":"https://doi.org/10.20953/1817-7646-2023-1-80-88","url":null,"abstract":"Objective. To analyze the effectiveness of correction of hypovitaminosis D and the dynamics of bone metabolism indicators in children with celiac disease against the background of exogenous dosing with cholecalciferol preparations. Patients and methods. This study included 105 children with celiac disease aged 2–17 years. All patients were divided into two groups: group 1 received vitamin D3 supplements and group 2 did not receive any therapy. The levels of vitamin D, osteocalcin, C-terminal telopeptide (CTX), and parathyroid hormone (PTH) were assessed in all children. Results. An increase in median vitamin D levels up to 32.4 [23.3; 39.7] ng/mL was observed against the background of taking cholecalciferol supplements, which was 2.5 times higher than in the same patients before treatment – 11.1 [5.9; 17.3] ng/mL (p < 0.001); meanwhile, the number of children with optimal calcidiol levels increased to 54.3% (p < 0.001), and the proportion of children with deficiency conditions decreased to 14.3% (p < 0.001). Positive dynamics of serum 25(OH)D levels were observed in 100% of cases. Against the background of vitamin D therapy, there was a decrease in CTX and PTH levels and an increase in osteocalcin levels. In the absence of therapy, a decrease in vitamin D levels from baseline of 25.7 [17.2; 35.1] ng/mL (p < 0.001) was noted. The proportion of patients with normal vitamin D levels decreased to 5.7% (p < 0.001); the number of children with deficiency increased to 85.7% (p < 0.001). There was a decrease in osteocalcin levels and an increase in CTX and PTH levels. Conclusion. Against the background of decreasing vitamin D levels and in the absence of its supplementation in children with celiac disease, osteoresorption processes prevail. Children with celiac disease may receive cholecalciferol supplementation in higher doses, which will avoid the development of osteopenia symptoms and significantly improve the quality of life. Key words: vitamin D, bone tissue, bone mineralization disorders, osteocalcin, osteoporosis, parathyroid hormone, C-terminal telopeptide","PeriodicalId":38157,"journal":{"name":"Voprosy Prakticheskoi Pediatrii","volume":"1 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"67750098","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}
Pub Date : 2023-01-01DOI: 10.20953/1817-7646-2023-3-7-12
E. I. Kleshchenko, E. Shimchenko
Objective. To determine the diagnostic criteria of morphological changes in children with perinatal hypoxic brain injury. Patients and Methods. The study included 58 full-term newborns with severe hypoxic brain damages who, according to MRI of brain performed on 2–10 days of life, had low rates of diffusion processes of the white matter of the cerebral hemispheres – diffusion coefficient (Ds) of less than 1.35 × 10–3 mm2/s. Children received treatment in the intensive care unit of the regional perinatal center of Krasnodar Children's Clinical Hospital. Follow-up MRI was performed at the 4th week of life. Observation of children was carried out up to 2 years of age. Results. Denominated destructive changes in the nervous tissue with multicystic encephalomalacia were identified in 23 (40%) newborns (group 1). The range of values of Ds of the white matter of cerebral hemispheres in children of the 1st group was 0.60–1.11 × 10–3 m2/s. In the remaining 35 (60%) children (group 2), hypoxic brain damage was manifested by the expansion of the subarachnoid space, the interhemispheric fissure, and the lateral ventricles. These changes were reflected in a decrease in the volume of brain parenchyma with the persistent neurological deficiency in the future (disorders of psycho-speech development, cerebral palsy, structural epilepsy). The range of values of the Ds of the white matter of the cerebral hemispheres in children of the second group was 1.14–1.35 × 10–3 mm2/s. Conclusions. Using the modern high-tech methods of neuroimaging makes it possible to determine the diagnostic criteria for damaging effects of hypoxia in children with perinatal brain injury and in early stage to identify morphological changes in brain tissue. Key words: hypoxia, brain, children, magnetic resonance imaging, neurological outcome
{"title":"Diagnostic criteria of morphological changes in children with perinatal hypoxic brain injury","authors":"E. I. Kleshchenko, E. Shimchenko","doi":"10.20953/1817-7646-2023-3-7-12","DOIUrl":"https://doi.org/10.20953/1817-7646-2023-3-7-12","url":null,"abstract":"Objective. To determine the diagnostic criteria of morphological changes in children with perinatal hypoxic brain injury. Patients and Methods. The study included 58 full-term newborns with severe hypoxic brain damages who, according to MRI of brain performed on 2–10 days of life, had low rates of diffusion processes of the white matter of the cerebral hemispheres – diffusion coefficient (Ds) of less than 1.35 × 10–3 mm2/s. Children received treatment in the intensive care unit of the regional perinatal center of Krasnodar Children's Clinical Hospital. Follow-up MRI was performed at the 4th week of life. Observation of children was carried out up to 2 years of age. Results. Denominated destructive changes in the nervous tissue with multicystic encephalomalacia were identified in 23 (40%) newborns (group 1). The range of values of Ds of the white matter of cerebral hemispheres in children of the 1st group was 0.60–1.11 × 10–3 m2/s. In the remaining 35 (60%) children (group 2), hypoxic brain damage was manifested by the expansion of the subarachnoid space, the interhemispheric fissure, and the lateral ventricles. These changes were reflected in a decrease in the volume of brain parenchyma with the persistent neurological deficiency in the future (disorders of psycho-speech development, cerebral palsy, structural epilepsy). The range of values of the Ds of the white matter of the cerebral hemispheres in children of the second group was 1.14–1.35 × 10–3 mm2/s. Conclusions. Using the modern high-tech methods of neuroimaging makes it possible to determine the diagnostic criteria for damaging effects of hypoxia in children with perinatal brain injury and in early stage to identify morphological changes in brain tissue. Key words: hypoxia, brain, children, magnetic resonance imaging, neurological outcome","PeriodicalId":38157,"journal":{"name":"Voprosy Prakticheskoi Pediatrii","volume":"170 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"67753050","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}
Pub Date : 2023-01-01DOI: 10.20953/1817-7646-2023-1-90-94
A.A. Sukhodolsky, I. V. Poddubny, V. V. Sytkov, A. V. Fedulov
The selection of surgical options for hydronephrosis correction and urinary diversion methods remains a relevant problem nowadays despite the widespread use of minimally invasive procedures. This paper summarizes our own long-term experience of using various drainage methods during ureteropelvic junction endoscopic surgery in children. Objective. To investigate the safety problems and assess the effiсacy of using pyelostomy during laparoscopic pyeloplasty in children. Patients and methods. Between 2011 and 2021, we performed Anderson–Hynes–Kucera laparoscopic pyeloplasty with partial resection of the renal pelvis and affected ureter area in 109 patients aged 3 months to 18 years. Among those, 15 children had grade 2 hydronephrosis, 59 children had grade 3 hydronephrosis, and 35 patients had grade 4 hydronephrosis. Only patients with primary ureteropelvic junction stricture causing hydronephrosis were included in the study. Children in whom hydronephrosis aroused due to other reasons (aberrant vessel, ureteric calculi, etc.) were not included in the statistics. The follow-up period ranged from 6 months to 2 years after surgery. Results. Pyelostomy as a method of renal pelvis drainage was performed in 77 patients. The mean duration of pyelostomy was 5 minutes. The mean duration of pyeloplasty with the use of pyelostomy was 90 minutes. Retrograde and antegrade double J stent (JJ/DJ stent) insertion took significantly more time. The mean surgery duration with the use of those methods was 115 and 120 minutes, respectively. Pyelostomy was successfully performed in all patients (100%). Retrograde stent insertion was unsuccessful in 3 patients (14%), and antegrade stent insertion – in 2 patients (18%). In the long-term follow-up period, 2 patients (2.6%) after pyelostomy and 1 patient (4.8%) after retrograde stenting developed ureteropelvic junction stricture, which required repeated pyeloplasty. Conclusion. Pyelostomy is an effective, sparing, and relatively easy to perform method of renal pelvis drainage during laparoscopic pyeloplasty. It allows to significantly reduce the time of surgical intervention, as well complication rate. The presence of pyelostoma in patients does not affect the length of hospital stay. Key words: hydronephrosis, children, laparoscopic pyeloplasty, pyelostomy, urinary tract drainage, DJ stent / JJ stent.
{"title":"The use of pyelostomy during laparoscopic pyeloplasty in children","authors":"A.A. Sukhodolsky, I. V. Poddubny, V. V. Sytkov, A. V. Fedulov","doi":"10.20953/1817-7646-2023-1-90-94","DOIUrl":"https://doi.org/10.20953/1817-7646-2023-1-90-94","url":null,"abstract":"The selection of surgical options for hydronephrosis correction and urinary diversion methods remains a relevant problem nowadays despite the widespread use of minimally invasive procedures. This paper summarizes our own long-term experience of using various drainage methods during ureteropelvic junction endoscopic surgery in children. Objective. To investigate the safety problems and assess the effiсacy of using pyelostomy during laparoscopic pyeloplasty in children. Patients and methods. Between 2011 and 2021, we performed Anderson–Hynes–Kucera laparoscopic pyeloplasty with partial resection of the renal pelvis and affected ureter area in 109 patients aged 3 months to 18 years. Among those, 15 children had grade 2 hydronephrosis, 59 children had grade 3 hydronephrosis, and 35 patients had grade 4 hydronephrosis. Only patients with primary ureteropelvic junction stricture causing hydronephrosis were included in the study. Children in whom hydronephrosis aroused due to other reasons (aberrant vessel, ureteric calculi, etc.) were not included in the statistics. The follow-up period ranged from 6 months to 2 years after surgery. Results. Pyelostomy as a method of renal pelvis drainage was performed in 77 patients. The mean duration of pyelostomy was 5 minutes. The mean duration of pyeloplasty with the use of pyelostomy was 90 minutes. Retrograde and antegrade double J stent (JJ/DJ stent) insertion took significantly more time. The mean surgery duration with the use of those methods was 115 and 120 minutes, respectively. Pyelostomy was successfully performed in all patients (100%). Retrograde stent insertion was unsuccessful in 3 patients (14%), and antegrade stent insertion – in 2 patients (18%). In the long-term follow-up period, 2 patients (2.6%) after pyelostomy and 1 patient (4.8%) after retrograde stenting developed ureteropelvic junction stricture, which required repeated pyeloplasty. Conclusion. Pyelostomy is an effective, sparing, and relatively easy to perform method of renal pelvis drainage during laparoscopic pyeloplasty. It allows to significantly reduce the time of surgical intervention, as well complication rate. The presence of pyelostoma in patients does not affect the length of hospital stay. Key words: hydronephrosis, children, laparoscopic pyeloplasty, pyelostomy, urinary tract drainage, DJ stent / JJ stent.","PeriodicalId":38157,"journal":{"name":"Voprosy Prakticheskoi Pediatrii","volume":"1 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"67750907","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}
Pub Date : 2023-01-01DOI: 10.20953/1817-7646-2023-2-91-101
G. Filippova, O. Morozova, E.Yu. Kirichek, A. A. Eliseeva, O. Efimenko, T. Raba, F. Raupov, Z. M. Kulieva, T.G. Eyvazov
This article discusses the problem of acute viral gastroenteritis (AVGE), including aspects of immunopathogenesis that are significant for effective therapy and prevention. It also summarizes the results of randomized clinical trials assessing the efficacy of technologically processed purified antibodies (Anaferon® for children, Ergoferon®). We describe our own experience with Ergoferon® evaluated in a an open-label study involving 94 AVGE patients aged 1 to 10 years. Children who received Ergoferon® demonstrated rapid resolution of symptoms and normalization of body temperature 3–3.5 days after treatment initiation. Resolution of vomiting was achieved during the first 2 days of therapy in most of the patients. Median duration of diarrhea was 3.5–4 days, with a trend to a little bit longer diarrhea period in patients in patients aged 1–3 years. None of the patients developed complications or aggravation by the moment of discharge from the hospital (day 5–6 of treatment); all children were cured. The therapy was well tolerated by patients; none of them reported adverse events. Our findings suggest that drugs based on technologically processed affine purified antibodies are effective are effective and can be used in comprehensive treatment for acute viral gastroenteritis in children to ensure active defense against viruses, support the immune system, and alleviate symptoms via the anti-inflammatory effect. Key words: acute viral gastroenteritis, Ergoferon, Anaferon for children, treatment
{"title":"Acute viral gastroenteritis: a brief review of current research and experience with antiviral drugs based on technologically processed antibodies","authors":"G. Filippova, O. Morozova, E.Yu. Kirichek, A. A. Eliseeva, O. Efimenko, T. Raba, F. Raupov, Z. M. Kulieva, T.G. Eyvazov","doi":"10.20953/1817-7646-2023-2-91-101","DOIUrl":"https://doi.org/10.20953/1817-7646-2023-2-91-101","url":null,"abstract":"This article discusses the problem of acute viral gastroenteritis (AVGE), including aspects of immunopathogenesis that are significant for effective therapy and prevention. It also summarizes the results of randomized clinical trials assessing the efficacy of technologically processed purified antibodies (Anaferon® for children, Ergoferon®). We describe our own experience with Ergoferon® evaluated in a an open-label study involving 94 AVGE patients aged 1 to 10 years. Children who received Ergoferon® demonstrated rapid resolution of symptoms and normalization of body temperature 3–3.5 days after treatment initiation. Resolution of vomiting was achieved during the first 2 days of therapy in most of the patients. Median duration of diarrhea was 3.5–4 days, with a trend to a little bit longer diarrhea period in patients in patients aged 1–3 years. None of the patients developed complications or aggravation by the moment of discharge from the hospital (day 5–6 of treatment); all children were cured. The therapy was well tolerated by patients; none of them reported adverse events. Our findings suggest that drugs based on technologically processed affine purified antibodies are effective are effective and can be used in comprehensive treatment for acute viral gastroenteritis in children to ensure active defense against viruses, support the immune system, and alleviate symptoms via the anti-inflammatory effect. Key words: acute viral gastroenteritis, Ergoferon, Anaferon for children, treatment","PeriodicalId":38157,"journal":{"name":"Voprosy Prakticheskoi Pediatrii","volume":"1 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"67752366","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}
Pub Date : 2023-01-01DOI: 10.20953/1817-7646-2023-3-56-66
N. Kondratieva, T. Dvoryakovskaya, T. Kriulina, K. Isaeva, A. Chomakhidze, O. Lomakina, K. Chibisova, I. Kriulin, E. Krekhova, I. Tsulukiya, M.S. Botova, M. Shingarova, Marta Kokina, E. Alexeeva
Juvenile idiopathic arthritis (JIA) is arthritis of unknown cause lasting more than 6 weeks, which starts in children under 16 years old with the exclusion of other joint disorder. According to the recommendations for the treatment of JIA, patients who does not responded to with antirheumatic therapy, we prescribe biological therapy, primarily TNF-alpha inhibitors. Despite their high effectiveness, in some patients, the TNF-alpha inhibitors has no proper therapeutic effect, which requires studying the possibility of using other therapy, in particular- rituximab. Objective. To evaluate the efficacy and safety of rituximab in patients with JIA without systemic manifestations Patients and methods. Retrospective cohort study included 33 patients who received rituximab. Treatment was initiated in the Department of Rheumatology, National Medical Research Center for Children's Health (Moscow) between December 2006 and April 2022. All patients were treated with non-steroid anti-inflammatory drugs (NSAIDs), non-biologic disease-modifying antirheumatic drugs (DMARDs), corticosteroids, biological agents with other mechanism of action. There was ineffectiveness of previous antirheumatic therapy in all patients. Patients were divided into two groups: with RF-positive polyarthritis (n = 30) and other non-systemic JIA (n = 13). Efficacy was evaluated in 1, 3, 6, 9, 12 months after first rituximab infusion and every 6 months till discontinuation or 18 years old achievement. Inactive disease/remission was registered according to Juvenile Arthritis Disease Activity Score (JADAS71) <1, С.Wallace criteria (2011) and The American College of Rheumatology (ACR) Pediatric criteria for assessing joint status (ACR Pedi 30, 50, 70, and 90). Results. Among 16 patients with RF-positive JIA 8 (50%) achieved 90% improvement according to ACR Pedi, inactive disease/remission according to С.Wallace was registered in 12 (75%) 12 months after rituximab initiation. In patients with other nonsystemic JIA (n = 13) 90% improvement according to ACR Pedi was registered in 5 (45%) patients, inactive disease/remission according to С.Wallace was registered in 6 (54, 5%) patients. Conclusion. Rituximab is highly effective in patients with JIA without systemic manifestation, who failed to previous treatment with NSAIDs, DMARDs and biological agents with other mechanism of action and it could be considered as next escalation therapy. Key words: rituximab, juvenile idiopathic arthritis, efficiency of therapy
{"title":"Efficacy of rituximab therapy in patients with non-systemic juvenile idiopathic arthritis","authors":"N. Kondratieva, T. Dvoryakovskaya, T. Kriulina, K. Isaeva, A. Chomakhidze, O. Lomakina, K. Chibisova, I. Kriulin, E. Krekhova, I. Tsulukiya, M.S. Botova, M. Shingarova, Marta Kokina, E. Alexeeva","doi":"10.20953/1817-7646-2023-3-56-66","DOIUrl":"https://doi.org/10.20953/1817-7646-2023-3-56-66","url":null,"abstract":"Juvenile idiopathic arthritis (JIA) is arthritis of unknown cause lasting more than 6 weeks, which starts in children under 16 years old with the exclusion of other joint disorder. According to the recommendations for the treatment of JIA, patients who does not responded to with antirheumatic therapy, we prescribe biological therapy, primarily TNF-alpha inhibitors. Despite their high effectiveness, in some patients, the TNF-alpha inhibitors has no proper therapeutic effect, which requires studying the possibility of using other therapy, in particular- rituximab. Objective. To evaluate the efficacy and safety of rituximab in patients with JIA without systemic manifestations Patients and methods. Retrospective cohort study included 33 patients who received rituximab. Treatment was initiated in the Department of Rheumatology, National Medical Research Center for Children's Health (Moscow) between December 2006 and April 2022. All patients were treated with non-steroid anti-inflammatory drugs (NSAIDs), non-biologic disease-modifying antirheumatic drugs (DMARDs), corticosteroids, biological agents with other mechanism of action. There was ineffectiveness of previous antirheumatic therapy in all patients. Patients were divided into two groups: with RF-positive polyarthritis (n = 30) and other non-systemic JIA (n = 13). Efficacy was evaluated in 1, 3, 6, 9, 12 months after first rituximab infusion and every 6 months till discontinuation or 18 years old achievement. Inactive disease/remission was registered according to Juvenile Arthritis Disease Activity Score (JADAS71) <1, С.Wallace criteria (2011) and The American College of Rheumatology (ACR) Pediatric criteria for assessing joint status (ACR Pedi 30, 50, 70, and 90). Results. Among 16 patients with RF-positive JIA 8 (50%) achieved 90% improvement according to ACR Pedi, inactive disease/remission according to С.Wallace was registered in 12 (75%) 12 months after rituximab initiation. In patients with other nonsystemic JIA (n = 13) 90% improvement according to ACR Pedi was registered in 5 (45%) patients, inactive disease/remission according to С.Wallace was registered in 6 (54, 5%) patients. Conclusion. Rituximab is highly effective in patients with JIA without systemic manifestation, who failed to previous treatment with NSAIDs, DMARDs and biological agents with other mechanism of action and it could be considered as next escalation therapy. Key words: rituximab, juvenile idiopathic arthritis, efficiency of therapy","PeriodicalId":38157,"journal":{"name":"Voprosy Prakticheskoi Pediatrii","volume":"1 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"67753000","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}
Pub Date : 2023-01-01DOI: 10.20953/1817-7646-2023-3-133-136
E. Fedulova, A. I. Khavkin, G.V. Medyansteva, V.A. Tsaryev
We present the clinical case of a 12-year-old girl with acute ulcerative colitis (UC), which was clinically manifested by blood in the stool, and genetic thrombophilia, which in turn was manifested by thrombotic changes in the veins of the left lower limb and clinically we faced with the swelling of the left leg. At the colonoscopy and histological examination of biopsies we established the pathological changes in the mucous membrane of the large intestine, on CT of the abdominal organs with contrast enhancement and ultrasound of the lower extremities found the venous thrombosis. The complexity of this clinical case is due to the difficulty of differential diagnosis of thrombotic complications of UC and hereditary thrombophilia.The main therapy for thrombosis causes a high risk of aggravation of hematological manifestations of UC, which required a long-term selection of suitable therapy that allowed the thrombotic masses to dissolve without gastrointestinal bleeding. The condition of patient was improved significantly against the background of therapy with sulfasalazine and heparin. Key words: genetic thrombophilia, heparin, colonoscopy, sulfasalazine, thrombosis, ulcerative colitis
{"title":"Ulcerative colitis, hereditary thrombophilia and coronavirus infection as links in a chain: the clinical case of comorbidity in 12-year-old girl","authors":"E. Fedulova, A. I. Khavkin, G.V. Medyansteva, V.A. Tsaryev","doi":"10.20953/1817-7646-2023-3-133-136","DOIUrl":"https://doi.org/10.20953/1817-7646-2023-3-133-136","url":null,"abstract":"We present the clinical case of a 12-year-old girl with acute ulcerative colitis (UC), which was clinically manifested by blood in the stool, and genetic thrombophilia, which in turn was manifested by thrombotic changes in the veins of the left lower limb and clinically we faced with the swelling of the left leg. At the colonoscopy and histological examination of biopsies we established the pathological changes in the mucous membrane of the large intestine, on CT of the abdominal organs with contrast enhancement and ultrasound of the lower extremities found the venous thrombosis. The complexity of this clinical case is due to the difficulty of differential diagnosis of thrombotic complications of UC and hereditary thrombophilia.The main therapy for thrombosis causes a high risk of aggravation of hematological manifestations of UC, which required a long-term selection of suitable therapy that allowed the thrombotic masses to dissolve without gastrointestinal bleeding. The condition of patient was improved significantly against the background of therapy with sulfasalazine and heparin. Key words: genetic thrombophilia, heparin, colonoscopy, sulfasalazine, thrombosis, ulcerative colitis","PeriodicalId":38157,"journal":{"name":"Voprosy Prakticheskoi Pediatrii","volume":"1 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"67753031","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}
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