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: 10.20953/1817-7646-2023-1-8-15
O. N. Solodovnikova, A. Dyagileva, A. A. Erovichenkov, D. V. Troshansky, O. Gosteva, A. Nurpeisova, Y. Khlypovka, А. Tsygankov, А.К. Shakaryan, N.S. Ipatov, I. N. Tyurin, D. Protsenko
In recent years, healthcare professionals are actively developing new systemic approaches to monitor vital signs at the bedside of a sick child by a nurse. One of these approaches is the Pediatric Early Warning Systems (PEWS). Objective. To standardize approaches to early detection of risks and timely prevention of clinical deterioration in children treated in hospitals for infectious diseases using the PEWS scale. Patients and methods. This study was conducted between September 28 and October 29, 2021 in the Pediatric Department for Infectious Diseases, Central Clinical Hospital No 40 and included 749 patients aged from 1 month to 19 years. The majority of patients were under 6 years of age. MS Excel 2021 was used for statistical analysis. Fisher's exact test with Yate's correction was used to identify significant changes. Results. As many as 352 patients (47.0 ± 1.8%) had no risk for poor prognosis (PEWS 0). We found no significant differences between the groups of children studied (acute upper respiratory infection, unspecified, n = 412; other and unspecified gastroenteritis and colitis of infectious origin, n = 233) in the frequency of PEWS scores. Thirteen out of 25 patients with PEWS ≥5 were admitted to the intensive care unit, whereas the remaining 12 patients received timely adequate care in specialized departments. Conclusion. Our first experience with the PEWS scale allows us to recommend it for pediatric hospitals. The PEWS scale is aimed at early detection of clinical signs associated with a risk of possibly deterioration in children treated in the departments of infectious diseases and helps to identify patients that require more extensive examination and treatment. Key words: children, infection, monitoring, risks, condition, scale, deterioration
{"title":"Standardization of approaches to early detection of risks for clinical deterioration in children treated in hospitals for infectious diseases","authors":"O. N. Solodovnikova, A. Dyagileva, A. A. Erovichenkov, D. V. Troshansky, O. Gosteva, A. Nurpeisova, Y. Khlypovka, А. Tsygankov, А.К. Shakaryan, N.S. Ipatov, I. N. Tyurin, D. Protsenko","doi":"10.20953/1817-7646-2023-1-8-15","DOIUrl":"https://doi.org/10.20953/1817-7646-2023-1-8-15","url":null,"abstract":"In recent years, healthcare professionals are actively developing new systemic approaches to monitor vital signs at the bedside of a sick child by a nurse. One of these approaches is the Pediatric Early Warning Systems (PEWS). Objective. To standardize approaches to early detection of risks and timely prevention of clinical deterioration in children treated in hospitals for infectious diseases using the PEWS scale. Patients and methods. This study was conducted between September 28 and October 29, 2021 in the Pediatric Department for Infectious Diseases, Central Clinical Hospital No 40 and included 749 patients aged from 1 month to 19 years. The majority of patients were under 6 years of age. MS Excel 2021 was used for statistical analysis. Fisher's exact test with Yate's correction was used to identify significant changes. Results. As many as 352 patients (47.0 ± 1.8%) had no risk for poor prognosis (PEWS 0). We found no significant differences between the groups of children studied (acute upper respiratory infection, unspecified, n = 412; other and unspecified gastroenteritis and colitis of infectious origin, n = 233) in the frequency of PEWS scores. Thirteen out of 25 patients with PEWS ≥5 were admitted to the intensive care unit, whereas the remaining 12 patients received timely adequate care in specialized departments. Conclusion. Our first experience with the PEWS scale allows us to recommend it for pediatric hospitals. The PEWS scale is aimed at early detection of clinical signs associated with a risk of possibly deterioration in children treated in the departments of infectious diseases and helps to identify patients that require more extensive examination and treatment. Key words: children, infection, monitoring, risks, condition, scale, deterioration","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":"67750781","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: 10.20953/1817-7646-2023-1-136-143
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: 10.20953/1817-7646-2023-3-56-66
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}
Pub Date : 2023-01-01DOI: 10.20953/1817-7646-2023-3-67-74
S. M. Dehghani, N. Rezvan, A. Shamsaeefar, I. Shahramian, M. Ataollahi, SH.Rakhshani Тasab, M. Tahani
Liver transplantation (LT) is the treatment of choice for end-stage liver disease in children, but donor shortage is still a central problem in this age group. Objective. To evaluate the rate of complications and mortality in cirrhotic children awaiting transplantation. Patients and methods. We analyzed medical records of 113 children under 18 years of age who were listed for LT, but the organ was unavailable between 2013 and 2018. The outcome was assessed from their records or follow-up data. Results. Among the children (mean age: 3.79 years; 50.4% boys and 49.6% girls) awaiting LT, the common causes of cirrhosis were biliary atresia (38.9%) and progressive familial intrahepatic cholestasis (17.7%). While awaiting transplantation, 82 (72.6%) children died. The most common complications were encephalopathy (34.5%), esophageal bleeding (26.5%), infections (23.0%), and spontaneous bacterial peritonitis (13.3%). The mean duration of hospital admission was 2.72 ± 3.10 days. Conclusion. About two-thirds of children listed for LT developed complications, and about three-quarters died without any LT. It seems that split liver transplantation and the introduction of a live-related transplant program in our center will provide many benefits to pediatric patients. Key words: liver transplantation, mortality, cirrhosis, pediatrics, children
{"title":"Complications and mortality in cirrhotic children awaiting liver allografts: a single-center report from Iran","authors":"S. M. Dehghani, N. Rezvan, A. Shamsaeefar, I. Shahramian, M. Ataollahi, SH.Rakhshani Тasab, M. Tahani","doi":"10.20953/1817-7646-2023-3-67-74","DOIUrl":"https://doi.org/10.20953/1817-7646-2023-3-67-74","url":null,"abstract":"Liver transplantation (LT) is the treatment of choice for end-stage liver disease in children, but donor shortage is still a central problem in this age group. Objective. To evaluate the rate of complications and mortality in cirrhotic children awaiting transplantation. Patients and methods. We analyzed medical records of 113 children under 18 years of age who were listed for LT, but the organ was unavailable between 2013 and 2018. The outcome was assessed from their records or follow-up data. Results. Among the children (mean age: 3.79 years; 50.4% boys and 49.6% girls) awaiting LT, the common causes of cirrhosis were biliary atresia (38.9%) and progressive familial intrahepatic cholestasis (17.7%). While awaiting transplantation, 82 (72.6%) children died. The most common complications were encephalopathy (34.5%), esophageal bleeding (26.5%), infections (23.0%), and spontaneous bacterial peritonitis (13.3%). The mean duration of hospital admission was 2.72 ± 3.10 days. Conclusion. About two-thirds of children listed for LT developed complications, and about three-quarters died without any LT. It seems that split liver transplantation and the introduction of a live-related transplant program in our center will provide many benefits to pediatric patients. Key words: liver transplantation, mortality, cirrhosis, pediatrics, children","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":"67753043","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}
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