Pub Date : 2023-06-01DOI: 10.24110/0031-403x-2023-102-3-189-196
A.S. Klyut, T.T. Valiev, T.S. Belysheva, O.A. Malikhova, T.V. Nasedkina, O.A. Gusarova, K.I. Kirgizov, S.R. Varfolomeeva
Reactive blood changes are a heterogeneous group of pathological conditions that can occur in response to various conditions: viral, bacterial infections, helminth toxins, allergic agents. These reactions often require differential diagnosis with malignant neoplasms (MN) of the blood and hematopoietic organs (leukemias, lymphomas). It is important to note that only with a comprehensive examination of the patient with the involvement of a pediatric oncologist and hematologist, it is possible to exclude MN and carry out etiotropic treatment that contributes to the normalization of hemogram parameters. Article represents current data on the etiopathogenesis of leukemoid reactions, reactive thrombocytosis, secondary erythrocytosis and a clinical case observation of the development of a pseudoblast leukemoid reaction and reactive thrombocytosis against the background of an infectious process in a patient with Peutz-Jeghers syndrome, which will be of use to a wide range of pediatric practitioners.
{"title":"REACTIVE BLOOD CHANGES IN THE PRACTICE OF PEDIATRIC PHYSICIAN, ONCOLOGIST AND HEMATOLOGIST: BIBLIOGRAPHICAL REVIEW AND CLINICAL CASE","authors":"A.S. Klyut, T.T. Valiev, T.S. Belysheva, O.A. Malikhova, T.V. Nasedkina, O.A. Gusarova, K.I. Kirgizov, S.R. Varfolomeeva","doi":"10.24110/0031-403x-2023-102-3-189-196","DOIUrl":"https://doi.org/10.24110/0031-403x-2023-102-3-189-196","url":null,"abstract":"Reactive blood changes are a heterogeneous group of pathological conditions that can occur in response to various conditions: viral, bacterial infections, helminth toxins, allergic agents. These reactions often require differential diagnosis with malignant neoplasms (MN) of the blood and hematopoietic organs (leukemias, lymphomas). It is important to note that only with a comprehensive examination of the patient with the involvement of a pediatric oncologist and hematologist, it is possible to exclude MN and carry out etiotropic treatment that contributes to the normalization of hemogram parameters. Article represents current data on the etiopathogenesis of leukemoid reactions, reactive thrombocytosis, secondary erythrocytosis and a clinical case observation of the development of a pseudoblast leukemoid reaction and reactive thrombocytosis against the background of an infectious process in a patient with Peutz-Jeghers syndrome, which will be of use to a wide range of pediatric practitioners.","PeriodicalId":39654,"journal":{"name":"Pediatriya - Zhurnal im G.N. Speranskogo","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135626601","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 : 2023-06-01DOI: 10.24110/0031-403x-2023-102-3-71-78
S. Kulyova, R. I. Khabarova
This research was aimed to developing the scoring system for evaluating indications for wide excision of the Spitz/Read nevus in children and adolescents, the use of which would be of help in reducing the risk of underdiagnosis of skin melanoma and determining the management tactics of patients with spitzoid formations. The purpose of the research was to create a scoring system for evaluating indications for wide excision of the Spitz/Reed nevus in children and adolescents. Materials and methods used: a single-center (N.N. Petrov National Medical Research Center for Oncology, Saint Petersburg, Russia) retrospective (2016-2022) cohort study included 65 childhood and adolescent patients with skin neoplasms of melanocytic and vascular origin. Clinical and dermatoscopic examinations of the skin neoplasm were performed at the pre-invasive stage, which was then followed by its removal and morphological verification. The study group consisted of children and adolescents aged 0 to 18 y/o (median age 14 (12.0; 15.0) y/o) with complex (n=22 or 33.8%), dermal (n=30/46.1%), blue (n=2/3.1%) nevi, Spitz/Reed nevus (n=4/6.2%) and capillary-lobular hemangioma (n=7/10.8%) Results: correlation analysis of 77 factors characterizing the demographic, clinical and dermatoscopic patterns had allowed the Authors to identify signs that are statistically significantly correlated with the Spitz/Reed nevus variable. In the block with demographic data parameters, the main position was occupied by the patient’s age of over 11 y/o (p˂0.001); formation area ≥0.38 cm2 (p=0.010) topped in the block with clinical examination parameters; and starburst pattern (p=0.003), irregular borders (p˂0.001), white-blue veil (p˂0.001), multicomponent (p=0.001), reticular network (p˂0.001), globules (p˂0.001) and vascular structures in the form of a comma (p˂0.001) among dermatoscopic ones. The value of the prognostic coefficient determined the score of each factor, which contributed to the selection of large and small patterns. The gradation of the sum of scores of the identified predictors made it possible to create a scoring system for analyzing indications for wide excision of the Spitz/Read nevus in children and adolescents and to develop a practical BASE-SCRAG scale. The sensitivity of the scale was 100%, the specificity was 62% (95% CI 57-64%). Conclusions: the Spitz/Reed scoring system for evaluating indications for wide excision of a nevus and the BASE-SCRAG practical scale contribute to the stratification of pediatric and adolescent patients in the group for possible dynamic observation and the group of mandatory removal and verification of the pathological skin process.
{"title":"SCORING SYSTEM FOR EVALUATING INDICATIONS FOR WIDE EXCISION OF SPITZ/REED NEVUS IN CHILDREN AND ADOLESCENTS","authors":"S. Kulyova, R. I. Khabarova","doi":"10.24110/0031-403x-2023-102-3-71-78","DOIUrl":"https://doi.org/10.24110/0031-403x-2023-102-3-71-78","url":null,"abstract":"This research was aimed to developing the scoring system for evaluating indications for wide excision of the Spitz/Read nevus in children and adolescents, the use of which would be of help in reducing the risk of underdiagnosis of skin melanoma and determining the management tactics of patients with spitzoid formations. The purpose of the research was to create a scoring system for evaluating indications for wide excision of the Spitz/Reed nevus in children and adolescents. Materials and methods used: a single-center (N.N. Petrov National Medical Research Center for Oncology, Saint Petersburg, Russia) retrospective (2016-2022) cohort study included 65 childhood and adolescent patients with skin neoplasms of melanocytic and vascular origin. Clinical and dermatoscopic examinations of the skin neoplasm were performed at the pre-invasive stage, which was then followed by its removal and morphological verification. The study group consisted of children and adolescents aged 0 to 18 y/o (median age 14 (12.0; 15.0) y/o) with complex (n=22 or 33.8%), dermal (n=30/46.1%), blue (n=2/3.1%) nevi, Spitz/Reed nevus (n=4/6.2%) and capillary-lobular hemangioma (n=7/10.8%) Results: correlation analysis of 77 factors characterizing the demographic, clinical and dermatoscopic patterns had allowed the Authors to identify signs that are statistically significantly correlated with the Spitz/Reed nevus variable. In the block with demographic data parameters, the main position was occupied by the patient’s age of over 11 y/o (p˂0.001); formation area ≥0.38 cm2 (p=0.010) topped in the block with clinical examination parameters; and starburst pattern (p=0.003), irregular borders (p˂0.001), white-blue veil (p˂0.001), multicomponent (p=0.001), reticular network (p˂0.001), globules (p˂0.001) and vascular structures in the form of a comma (p˂0.001) among dermatoscopic ones. The value of the prognostic coefficient determined the score of each factor, which contributed to the selection of large and small patterns. The gradation of the sum of scores of the identified predictors made it possible to create a scoring system for analyzing indications for wide excision of the Spitz/Read nevus in children and adolescents and to develop a practical BASE-SCRAG scale. The sensitivity of the scale was 100%, the specificity was 62% (95% CI 57-64%). Conclusions: the Spitz/Reed scoring system for evaluating indications for wide excision of a nevus and the BASE-SCRAG practical scale contribute to the stratification of pediatric and adolescent patients in the group for possible dynamic observation and the group of mandatory removal and verification of the pathological skin process.","PeriodicalId":39654,"journal":{"name":"Pediatriya - Zhurnal im G.N. Speranskogo","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"75834549","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 : 2023-06-01DOI: 10.24110/0031-403x-2023-102-3-188-193
E.O. Bezdolnova, M. S. Kubirov, A.A. Kirillov, V. V. Gorev, E. Kumirova
Juvenile granulosa cell tumor of the ovaries is a rare disease in pediatric oncology. Mostly seen in girls and young women in premenarchal age (median age 10 y/o) and is more aggressive than the adult type, but has a favorable prognosis, especially in its early stages. Large reviews and studies include patients with bot adult and juvenile types granulosa cell tumor. Due to the limited cohort of pediatric patients, there are no standards for the treatment of juvenile granulosa cell tumor in pediatric practice. Therefore, diagnosis, treatment, and long-term follow-up for this disease are based on recommendations for an adult cohort. Adjuvant chemotherapy as a curative option is used in advanced stages above the FIGO IC. For the IC stage, the issue of prescribing adjuvant chemotherapy remains debatable. Article represents analysis of the bibliographical data of the largest studies on the treatment of this disease as well as clinical case of a 15 y/o patient with juvenile granulosa cell tumor of the right ovary who had received complex treatment in the Oncological Department of the Morozov Children’s City Clinical Hospital (Moscow, Russia).
{"title":"JUVENILE GRANULOSA CELL TUMOR OF THE OVARY IN A CHILD: CLINICAL CASE AND BIBLIOGRAPHICAL REVIEW","authors":"E.O. Bezdolnova, M. S. Kubirov, A.A. Kirillov, V. V. Gorev, E. Kumirova","doi":"10.24110/0031-403x-2023-102-3-188-193","DOIUrl":"https://doi.org/10.24110/0031-403x-2023-102-3-188-193","url":null,"abstract":"Juvenile granulosa cell tumor of the ovaries is a rare disease in pediatric oncology. Mostly seen in girls and young women in premenarchal age (median age 10 y/o) and is more aggressive than the adult type, but has a favorable prognosis, especially in its early stages. Large reviews and studies include patients with bot adult and juvenile types granulosa cell tumor. Due to the limited cohort of pediatric patients, there are no standards for the treatment of juvenile granulosa cell tumor in pediatric practice. Therefore, diagnosis, treatment, and long-term follow-up for this disease are based on recommendations for an adult cohort. Adjuvant chemotherapy as a curative option is used in advanced stages above the FIGO IC. For the IC stage, the issue of prescribing adjuvant chemotherapy remains debatable. Article represents analysis of the bibliographical data of the largest studies on the treatment of this disease as well as clinical case of a 15 y/o patient with juvenile granulosa cell tumor of the right ovary who had received complex treatment in the Oncological Department of the Morozov Children’s City Clinical Hospital (Moscow, Russia).","PeriodicalId":39654,"journal":{"name":"Pediatriya - Zhurnal im G.N. Speranskogo","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"75445371","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 : 2023-06-01DOI: 10.24110/0031-403x-2023-102-3-33-42
O. M. Romantsova, D. Khestanov, A. Dzampaev, V. Khairullova, M. M. Efimova, T. Gorbunova, K. Kirgizov, S. Varfolomeeva
Ewing's sarcoma (ES) is a highly malignant tumor of children and adolescents that affects bones and soft tissues with the same frequency. The origins of ES are the subject to many discussions. Differential diagnosis is complicated and requires a full range of immunohistochemical and molecular genetic studies. The prognostic value for extraskeletal and bone ES under current chemotherapy protocols is unknown and requires further analysis. The purposes of this research were a comparative analysis of clinical characteristics, therapeutic approaches and outcomes of the disease in patients with extraskeletal and bone localizations of ES. Materials and methods used: a single-center retrospective cohort study was conducted, which included 330 patients (237 (71.8%) boys/93 (28.2%) girls) aged 0 to 18 y/o (median 11 [7; 14] y/o) with confirmed diagnosis of ES who received treatment in 2008-2022, of which 280 (84.85%) with primary bone localization (group of bone ES - BES), and 50 (15.15%) with soft tissue localization (group of extraskeletal ES - ESES). Comparative analysis of survival rates for primary tumor localization in the area of bones and soft tissues was performed. The median follow-up time for all patients was 35.5 [18.2; 68.5] months, 37.0 [18.0; 71.0] months with BES, and 29.5 [16.8; 65.5] months with ESES. All patients received treatment according to the protocols adopted at the Research Institute of Pediatric Oncology and Hematology named after Academician L.A. Durnov with the N.N. Blokhin Russian Cancer Research Center (Moscow, Russia): MMES-99, ES-2017. Overall survival (OS) was calculated with the Kaplan-Meier estimator. Results: the selected groups differed statistically significantly by gender (74% of boys in the BES group, and 60% of boys the ESES group, p=0.035) and age (10.5 [8; 15] years in the BES group, and 8.5 [4; 12] y/o in the ESES group, p=0.001). BES was diagnosed statistically significantly more often in older age groups than ESES (p=0.004). Compared with BES, in ESES the tumor was statistically significantly more often located in the region of the axial skeleton and visceral organs (24.0% vs. 56%, p<0.001). Disseminated form of the disease in the BES group was recorded in 110 (39.3%) patients, and in 15 (30.0%) in the ESES group. Authors did not find statistically significant differences in overall 5-year OS for localized forms of BES and ESES (79% and 78.5%, respectively), the median OS in these groups was not reached. The OS of patients with disseminated stages of BES and ESES was statistically significantly lower than in the group of localized forms. At the same time, the 5-year OS was 41.2% and 40.6%, the median OS was 46.9 and 28.4 months (p=0.001, respectively). Differences in 5-year progression-free survival (PFS) for localized forms were 71.6% for BES and 75.6% for ESES (p=0.001), for disseminated forms - 32.4% vs. 44.9% (p=0.036, respectively). In the disseminated stage of BES, progression/relapse was detected in 50
{"title":"Bone and extraskeletal Ewing's sarcoma: comparative characteristics of the course and outcomes of the disease. Experience of the Research Institute of Pediatric Oncology and Hematology named after Academician L.A. Durnov with the N.N. Blokhin Russian Cancer Research Center (Moscow, Russia)","authors":"O. M. Romantsova, D. Khestanov, A. Dzampaev, V. Khairullova, M. M. Efimova, T. Gorbunova, K. Kirgizov, S. Varfolomeeva","doi":"10.24110/0031-403x-2023-102-3-33-42","DOIUrl":"https://doi.org/10.24110/0031-403x-2023-102-3-33-42","url":null,"abstract":"Ewing's sarcoma (ES) is a highly malignant tumor of children and adolescents that affects bones and soft tissues with the same frequency. The origins of ES are the subject to many discussions. Differential diagnosis is complicated and requires a full range of immunohistochemical and molecular genetic studies. The prognostic value for extraskeletal and bone ES under current chemotherapy protocols is unknown and requires further analysis. The purposes of this research were a comparative analysis of clinical characteristics, therapeutic approaches and outcomes of the disease in patients with extraskeletal and bone localizations of ES. Materials and methods used: a single-center retrospective cohort study was conducted, which included 330 patients (237 (71.8%) boys/93 (28.2%) girls) aged 0 to 18 y/o (median 11 [7; 14] y/o) with confirmed diagnosis of ES who received treatment in 2008-2022, of which 280 (84.85%) with primary bone localization (group of bone ES - BES), and 50 (15.15%) with soft tissue localization (group of extraskeletal ES - ESES). Comparative analysis of survival rates for primary tumor localization in the area of bones and soft tissues was performed. The median follow-up time for all patients was 35.5 [18.2; 68.5] months, 37.0 [18.0; 71.0] months with BES, and 29.5 [16.8; 65.5] months with ESES. All patients received treatment according to the protocols adopted at the Research Institute of Pediatric Oncology and Hematology named after Academician L.A. Durnov with the N.N. Blokhin Russian Cancer Research Center (Moscow, Russia): MMES-99, ES-2017. Overall survival (OS) was calculated with the Kaplan-Meier estimator. Results: the selected groups differed statistically significantly by gender (74% of boys in the BES group, and 60% of boys the ESES group, p=0.035) and age (10.5 [8; 15] years in the BES group, and 8.5 [4; 12] y/o in the ESES group, p=0.001). BES was diagnosed statistically significantly more often in older age groups than ESES (p=0.004). Compared with BES, in ESES the tumor was statistically significantly more often located in the region of the axial skeleton and visceral organs (24.0% vs. 56%, p<0.001). Disseminated form of the disease in the BES group was recorded in 110 (39.3%) patients, and in 15 (30.0%) in the ESES group. Authors did not find statistically significant differences in overall 5-year OS for localized forms of BES and ESES (79% and 78.5%, respectively), the median OS in these groups was not reached. The OS of patients with disseminated stages of BES and ESES was statistically significantly lower than in the group of localized forms. At the same time, the 5-year OS was 41.2% and 40.6%, the median OS was 46.9 and 28.4 months (p=0.001, respectively). Differences in 5-year progression-free survival (PFS) for localized forms were 71.6% for BES and 75.6% for ESES (p=0.001), for disseminated forms - 32.4% vs. 44.9% (p=0.036, respectively). In the disseminated stage of BES, progression/relapse was detected in 50","PeriodicalId":39654,"journal":{"name":"Pediatriya - Zhurnal im G.N. Speranskogo","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"81735881","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 : 2023-06-01DOI: 10.24110/0031-403x-2023-102-3-17-24
D. Akhaladze, G. Rabaev, I. V. Tverdov, N. Merkulov, N. Uskova, S. Talypov, A. A. Krivonosov, N. S. Grachev
The purpose of this research was to develop a scale for risk, difficulty and complexity assessment of laparoscopic surgical interventions for neurogenic tumors of abdominal localization in children taking into account IDRF, tumor size and other criteria that affect the surgical outcome. Materials and methods used: 124 patients (68 (55%) boys/56 (45%) girls) aged 0 to 18 y/o (median age of 20.5 [5-50.5] months old) who have undergone laparoscopic surgery for neurogenic tumors of abdominal localization in Jan. 2018-Apr. 2022 were included in a single-center retrospective cohort study. An analysis was made of the relationship between the 24 criteria for the complexity of the surgical intervention and the parametric evaluation of its results (duration of surgical intervention; intraoperative blood loss; intraoperative blood transfusion; intraoperative complications and access conversion). As for the quantitative variables, the Spearman's rank correlation coefficient was used, and the eta coefficient for qualitative variables. Cluster analysis was used to distribute points by the difficulty levels. The ROC curve analysis with sensitivity and specificity for high level of complexity was used to check the scale of complexity. Results: median duration of surgical intervention was 105 [75; 150] min, volume of intraoperative blood loss - 5 [5; 20] ml. Blood transfusion was required in 15 (12%) cases, median blood transfusion was 0 [0; 0] ml. Intraoperative complications developed in 4 (3%) cases. Conversions were performed in 4 (3%) patients. 10 complexity criteria were selected: IDRF F1, F2, F3, F4, F5, location of the tumor center, extension beyond the midline, previous open surgery, contact of the tumor with the inferior vena cava, and the tumor volume (ml)/height (m) ratio of > or
{"title":"PILOT VERSION OF SCORING SYSTEM FOR LAPAROSCOPIC SURGERY OF ABDOMINAL NEUROBLASTOMA IN CHILDREN","authors":"D. Akhaladze, G. Rabaev, I. V. Tverdov, N. Merkulov, N. Uskova, S. Talypov, A. A. Krivonosov, N. S. Grachev","doi":"10.24110/0031-403x-2023-102-3-17-24","DOIUrl":"https://doi.org/10.24110/0031-403x-2023-102-3-17-24","url":null,"abstract":"The purpose of this research was to develop a scale for risk, difficulty and complexity assessment of laparoscopic surgical interventions for neurogenic tumors of abdominal localization in children taking into account IDRF, tumor size and other criteria that affect the surgical outcome. Materials and methods used: 124 patients (68 (55%) boys/56 (45%) girls) aged 0 to 18 y/o (median age of 20.5 [5-50.5] months old) who have undergone laparoscopic surgery for neurogenic tumors of abdominal localization in Jan. 2018-Apr. 2022 were included in a single-center retrospective cohort study. An analysis was made of the relationship between the 24 criteria for the complexity of the surgical intervention and the parametric evaluation of its results (duration of surgical intervention; intraoperative blood loss; intraoperative blood transfusion; intraoperative complications and access conversion). As for the quantitative variables, the Spearman's rank correlation coefficient was used, and the eta coefficient for qualitative variables. Cluster analysis was used to distribute points by the difficulty levels. The ROC curve analysis with sensitivity and specificity for high level of complexity was used to check the scale of complexity. Results: median duration of surgical intervention was 105 [75; 150] min, volume of intraoperative blood loss - 5 [5; 20] ml. Blood transfusion was required in 15 (12%) cases, median blood transfusion was 0 [0; 0] ml. Intraoperative complications developed in 4 (3%) cases. Conversions were performed in 4 (3%) patients. 10 complexity criteria were selected: IDRF F1, F2, F3, F4, F5, location of the tumor center, extension beyond the midline, previous open surgery, contact of the tumor with the inferior vena cava, and the tumor volume (ml)/height (m) ratio of > or </= 28. A pilot difficulty scale with three levels of difficulty has been developed. Sensitivity for a high level of complexity (6 or over pts) was 60% (95% CI 23.1-88.2%), specificity was 98.3% (95% CI 94.1-99.5%). Conclusion: different IDRFs contribute differently to the complexity and risk of laparoscopic surgery. The ratio of tumor size to growth is more significant criterion of complexity than the absolute size of the neoplasm. Other criteria of complexity also play an important role, such as previous open surgeries and tumor localization, in particular. The Authors do not recommend this scale for clinical use because the results obtained in this research would form the basis for further prospective study aiming to the development of a more accurate scale of the complexity of laparoscopic operations in neurogenic tumors of the abdominal localization.","PeriodicalId":39654,"journal":{"name":"Pediatriya - Zhurnal im G.N. Speranskogo","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"84851436","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 : 2023-06-01DOI: 10.24110/0031-403x-2023-102-3-16-24
S. Chechelnitskaya, E. Zhukovskaya, A.V. Baerbah, D. Zhuk, Y. Saraykin, A. F. Karelin
Article represents the results of the research undertaken to prove the relationship between the physical conditions of patients treated for oncological diseases (OD) and the decrease in their health-related quality of life (HRQOL). Materials and methods used: 1564 children aged 6 to 18 y/o who have undergone rehabilitation at the “Russkoye pole” Clinical Rehabilitation Research Center for patients in remission with the National Scientific and Practical Center for Pediatric Hematology, Oncology and Immunology named after Dmitry Rogachev located in Grishenki village, Chekhovsky District, Moscow Oblast, Russia, after the complex treatment for OD from 2018 to 2021 participated in the research, of which 864 boys and 700 girls. The average age of the patients was 10.6+/-3.4 y/o, the average remission period was 5.4+/-2.7 years. The comparison group consisted of healthy siblings aged 6 to 18 y/o who stayed at the same facility and during the same period, a total of 238, of which 111 boys and 127 girls. The average age of the examined siblings was 10.2+/-2.4 y/o. The indicators of physical condition were tested as follows: anthropometry, strength endurance of muscles, tolerance to physical loads, development of motor skills. The PedsQL questionnaire was used for the HRQOL assessment. In order to build the mathematical models and to evaluate the statistically significant influence of factors, the classical linear regression models were used. The analysis of the relationship between groups of variables was carried out with the canonical correlation analysis. Comparison of children treated for OD and healthy children was carried out using the Welch's t-test. Results: all of the HRQOL indicators in all gender and age subgroups in children with OD were statistically significantly lower compared to healthy siblings. The most significant differences were obtained in the scale of physical functioning (p=0.001). A direct moderate relationship was found between HRQOL and a factor indicator for the physical condition (rcc=0.53), a direct moderate-weak relationship with a generalized measure of strength endurance (rcc=0.315) and a direct moderate relationship with motor skills (rcc=0.5). It was also found that a lag in the formation of basic motor skills had the greatest negative impact on the HRQOL of children treated for OD. Conclusion: the results of the research do confirm the role of physical condition in improving the patients’ quality of life. Continued research would allow developing a new tool for assessment of the effectiveness of physical rehabilitation for patients in this category.
{"title":"PHYSICAL AND MOTOR DEVELOPMENT OF CHILDREN AFTER CANCER TREATMENT AS A FACTOR INFLUENCING HEALTH-RELATED QUALITY OF LIFE","authors":"S. Chechelnitskaya, E. Zhukovskaya, A.V. Baerbah, D. Zhuk, Y. Saraykin, A. F. Karelin","doi":"10.24110/0031-403x-2023-102-3-16-24","DOIUrl":"https://doi.org/10.24110/0031-403x-2023-102-3-16-24","url":null,"abstract":"Article represents the results of the research undertaken to prove the relationship between the physical conditions of patients treated for oncological diseases (OD) and the decrease in their health-related quality of life (HRQOL). Materials and methods used: 1564 children aged 6 to 18 y/o who have undergone rehabilitation at the “Russkoye pole” Clinical Rehabilitation Research Center for patients in remission with the National Scientific and Practical Center for Pediatric Hematology, Oncology and Immunology named after Dmitry Rogachev located in Grishenki village, Chekhovsky District, Moscow Oblast, Russia, after the complex treatment for OD from 2018 to 2021 participated in the research, of which 864 boys and 700 girls. The average age of the patients was 10.6+/-3.4 y/o, the average remission period was 5.4+/-2.7 years. The comparison group consisted of healthy siblings aged 6 to 18 y/o who stayed at the same facility and during the same period, a total of 238, of which 111 boys and 127 girls. The average age of the examined siblings was 10.2+/-2.4 y/o. The indicators of physical condition were tested as follows: anthropometry, strength endurance of muscles, tolerance to physical loads, development of motor skills. The PedsQL questionnaire was used for the HRQOL assessment. In order to build the mathematical models and to evaluate the statistically significant influence of factors, the classical linear regression models were used. The analysis of the relationship between groups of variables was carried out with the canonical correlation analysis. Comparison of children treated for OD and healthy children was carried out using the Welch's t-test. Results: all of the HRQOL indicators in all gender and age subgroups in children with OD were statistically significantly lower compared to healthy siblings. The most significant differences were obtained in the scale of physical functioning (p=0.001). A direct moderate relationship was found between HRQOL and a factor indicator for the physical condition (rcc=0.53), a direct moderate-weak relationship with a generalized measure of strength endurance (rcc=0.315) and a direct moderate relationship with motor skills (rcc=0.5). It was also found that a lag in the formation of basic motor skills had the greatest negative impact on the HRQOL of children treated for OD. Conclusion: the results of the research do confirm the role of physical condition in improving the patients’ quality of life. Continued research would allow developing a new tool for assessment of the effectiveness of physical rehabilitation for patients in this category.","PeriodicalId":39654,"journal":{"name":"Pediatriya - Zhurnal im G.N. Speranskogo","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"85055820","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 : 2023-06-01DOI: 10.24110/0031-403x-2023-102-3-124-130
A. Nechesnyuk, A. Loginova, A. Rumyantsev, S.V. Kazyukov
New technologies in radiation therapy (RT) are rapidly developing and entering the clinical practice of leading clinics. Pediatricians, pediatric oncologists, hematologists and other physicians are in need of understanding of the main trends in the development of modern RT in pediatric practice. The Article describes the main modern methods of photon radiation therapy (PRT) and of RT with intensity modulation and image control in particular, considers the methods of adaptive and stereotaxic RT. The urgent need for a reference evaluation of irradiation of pediatric patients’ plans is demonstrated as well. Thus, according to the reference evaluation results of 270 plans from 40 clinics, it was found that gross errors in target contouring were found in 39% of cases, the plan that could not be accepted for treatment in 40%. Modern PRT has wide technological capabilities, however, for its more effective use for the benefit of patients it is necessary to create a unified national database of RT plans. Only an analysis of the new data can make it possible to take more balanced approach to planning exposure in children, to provide dose reduction/escalation in patients of various prognostic groups taking into account the likelihood of undesirable late effects.
{"title":"Modern trends in photon radiation therapy in pediatric practice","authors":"A. Nechesnyuk, A. Loginova, A. Rumyantsev, S.V. Kazyukov","doi":"10.24110/0031-403x-2023-102-3-124-130","DOIUrl":"https://doi.org/10.24110/0031-403x-2023-102-3-124-130","url":null,"abstract":"New technologies in radiation therapy (RT) are rapidly developing and entering the clinical practice of leading clinics. Pediatricians, pediatric oncologists, hematologists and other physicians are in need of understanding of the main trends in the development of modern RT in pediatric practice. The Article describes the main modern methods of photon radiation therapy (PRT) and of RT with intensity modulation and image control in particular, considers the methods of adaptive and stereotaxic RT. The urgent need for a reference evaluation of irradiation of pediatric patients’ plans is demonstrated as well. Thus, according to the reference evaluation results of 270 plans from 40 clinics, it was found that gross errors in target contouring were found in 39% of cases, the plan that could not be accepted for treatment in 40%. Modern PRT has wide technological capabilities, however, for its more effective use for the benefit of patients it is necessary to create a unified national database of RT plans. Only an analysis of the new data can make it possible to take more balanced approach to planning exposure in children, to provide dose reduction/escalation in patients of various prognostic groups taking into account the likelihood of undesirable late effects.","PeriodicalId":39654,"journal":{"name":"Pediatriya - Zhurnal im G.N. Speranskogo","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"83269136","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 : 2023-06-01DOI: 10.24110/0031-403x-2023-102-3-63-70
A. Ignatova, I. Kalinina, D. Venyov, D. Evseev, T. Salimova, M. Sadovskaya, O. Goronkova, E. Suntsova, V. E. Matveev, U. Petrova, K. Antonova, D. D. Baydildina, K. Voronin, P. A. Levin, M. Dubrovina, T. Konyukhova, Y. Olshanskaya, E. Zerkalenkova, M. Gaskova, A. Popov, S. Kashpor, M. Maschan, A. A. Maschan
Infants of the first year of life represent a unique group of patients with acute myeloid leukemia (AML). Materials and methods of the research: the characteristics of 492 patients with newly diagnosed AML aged 10 DoL-18 y/o who received intensive chemotherapy according to the AML-MM-2006 and AML-MRD-2018 guidelines in Apr. 2007-Apr. 2021 were analyzed. The analysis was carried out separately for infants (<1 y/o, 58/12%), young children (1-3 y/o, 99/20%) and children aged 3-18 y/o (335/68%). Results: the infant group was characterized by a higher incidence of hyperleukocytosis, extramedullary lesions, the predominance of monoblast/monocytic (63%) and megakaryoblast (24%) subvariants and chromosomal aberrations involving the 11q23 locus (KMT2A gene) (53%). “CBF leukemias” were virtually non-existent in infants (0% for t(8;21)(q22;q22.1)/RUNX1::RUNX1T1 and 2% for inv(16)(p13.1q22)/CBF::MYH11) and the rates increased with age. Activating mutations in the FLT3 gene were extremely rare (2% vs. 13.5% in patients >/=3 y/o). Patients of the 1-3 y/o group were similar in morphological and cytogenetic characteristics to the infants’ group but had a smaller tumor mass and higher survival rates. The 5-year overall survival of infants was statistically significantly lower than in older patients, 52% vs. 67% (p<0.001), primarily due to high mortality prior to remission, which in its turn was caused by complications associated with hyperleukocytosis. Conclusions: given the vulnerability of this category of patients, it is reasonable to transfer them to larger medical facilities with extensive experience in the field in order to conduct the remission induction stage (especially cytoreduction) within the intensive care unit.
一岁的婴儿是急性髓性白血病(AML)患者的一个独特群体。研究材料与方法:2007年4月至4月，492例10 dol18 y/o新诊断AML患者根据AML- mm -2006和AML- mrd -2018指南接受强化化疗的特点。2021年进行了分析。对婴儿(/=3岁/岁)单独进行分析。1-3岁组患者的形态学和细胞遗传学特征与婴儿组相似，但肿瘤体积较小，生存率较高。婴儿的5年总生存率在统计学上显著低于老年患者，52% vs. 67% (p<0.001)，这主要是由于缓解前的高死亡率，而缓解前的高死亡率又由白细胞增多症相关的并发症引起。结论:鉴于这类患者的脆弱性，合理的做法是将他们转移到在该领域有丰富经验的大型医疗机构，以便在重症监护病房内进行缓解诱导阶段(特别是细胞减少)。
{"title":"CLINICAL FEATURES AND RESULTS OF THERAPY IN INFANTS AND CHILDREN AGED 1 TO 3 YEARS OLD WITH ACUTE MYELOID LEUKEMIA","authors":"A. Ignatova, I. Kalinina, D. Venyov, D. Evseev, T. Salimova, M. Sadovskaya, O. Goronkova, E. Suntsova, V. E. Matveev, U. Petrova, K. Antonova, D. D. Baydildina, K. Voronin, P. A. Levin, M. Dubrovina, T. Konyukhova, Y. Olshanskaya, E. Zerkalenkova, M. Gaskova, A. Popov, S. Kashpor, M. Maschan, A. A. Maschan","doi":"10.24110/0031-403x-2023-102-3-63-70","DOIUrl":"https://doi.org/10.24110/0031-403x-2023-102-3-63-70","url":null,"abstract":"Infants of the first year of life represent a unique group of patients with acute myeloid leukemia (AML). Materials and methods of the research: the characteristics of 492 patients with newly diagnosed AML aged 10 DoL-18 y/o who received intensive chemotherapy according to the AML-MM-2006 and AML-MRD-2018 guidelines in Apr. 2007-Apr. 2021 were analyzed. The analysis was carried out separately for infants (<1 y/o, 58/12%), young children (1-3 y/o, 99/20%) and children aged 3-18 y/o (335/68%). Results: the infant group was characterized by a higher incidence of hyperleukocytosis, extramedullary lesions, the predominance of monoblast/monocytic (63%) and megakaryoblast (24%) subvariants and chromosomal aberrations involving the 11q23 locus (KMT2A gene) (53%). “CBF leukemias” were virtually non-existent in infants (0% for t(8;21)(q22;q22.1)/RUNX1::RUNX1T1 and 2% for inv(16)(p13.1q22)/CBF::MYH11) and the rates increased with age. Activating mutations in the FLT3 gene were extremely rare (2% vs. 13.5% in patients >/=3 y/o). Patients of the 1-3 y/o group were similar in morphological and cytogenetic characteristics to the infants’ group but had a smaller tumor mass and higher survival rates. The 5-year overall survival of infants was statistically significantly lower than in older patients, 52% vs. 67% (p<0.001), primarily due to high mortality prior to remission, which in its turn was caused by complications associated with hyperleukocytosis. Conclusions: given the vulnerability of this category of patients, it is reasonable to transfer them to larger medical facilities with extensive experience in the field in order to conduct the remission induction stage (especially cytoreduction) within the intensive care unit.","PeriodicalId":39654,"journal":{"name":"Pediatriya - Zhurnal im G.N. Speranskogo","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"84842325","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 : 2023-06-01DOI: 10.24110/0031-403x-2023-102-3-166-173
A. Kugushev, A. Lopatin, N. S. Grachev, I. Kletskaya, K.D. Starokokova
Thymus ectopia is a rare solid neck lesion in children. The location of the formation along the path of thymus migration (thymopharyngeal duct), the echo pattern and the intensity of the MR signal, which is identical to the normally developed thymus, help in making a confident diagnosis. Materials and methods of the research: 14 pediatric patients were treated against the cervical thymus ectopia in 2018-2022 in the Russian Children's Clinical Hospital with the Pirogov Russian National Research Medical University of the Ministry of Healthcare of Russia and the National Scientific and Practical Center for Pediatric Hematology, Oncology and Immunology named after Dmitry Rogachev (both located in Moscow, Russia). The diagnosis was set only after the histological examination results in all cases. A differential diagnosis was made with other cystic and solid formations, taking into account its location and distribution according to ultrasound, computed tomography, and magnetic resonance imaging in each case. Results: stable, relapse-free treatment result was achieved after more than a year of follow-up in all cases. The correct diagnosis wasn’t set in every case until the biopsy results due to the rarity and diverse clinical and radiological picture of the disease. Conclusion: cervical thymus ectopia is a benign and relatively rare condition in children that is rarely diagnosed preoperatively by the visualization only. The conservative management may be appropriate in some cases when the thymus is in the chest cavity only, but surgical correction is more often needed in cases with the neck deforming formations.
{"title":"CERVICAL THYMUS ECTOPIA IN CHILDREN","authors":"A. Kugushev, A. Lopatin, N. S. Grachev, I. Kletskaya, K.D. Starokokova","doi":"10.24110/0031-403x-2023-102-3-166-173","DOIUrl":"https://doi.org/10.24110/0031-403x-2023-102-3-166-173","url":null,"abstract":"Thymus ectopia is a rare solid neck lesion in children. The location of the formation along the path of thymus migration (thymopharyngeal duct), the echo pattern and the intensity of the MR signal, which is identical to the normally developed thymus, help in making a confident diagnosis. Materials and methods of the research: 14 pediatric patients were treated against the cervical thymus ectopia in 2018-2022 in the Russian Children's Clinical Hospital with the Pirogov Russian National Research Medical University of the Ministry of Healthcare of Russia and the National Scientific and Practical Center for Pediatric Hematology, Oncology and Immunology named after Dmitry Rogachev (both located in Moscow, Russia). The diagnosis was set only after the histological examination results in all cases. A differential diagnosis was made with other cystic and solid formations, taking into account its location and distribution according to ultrasound, computed tomography, and magnetic resonance imaging in each case. Results: stable, relapse-free treatment result was achieved after more than a year of follow-up in all cases. The correct diagnosis wasn’t set in every case until the biopsy results due to the rarity and diverse clinical and radiological picture of the disease. Conclusion: cervical thymus ectopia is a benign and relatively rare condition in children that is rarely diagnosed preoperatively by the visualization only. The conservative management may be appropriate in some cases when the thymus is in the chest cavity only, but surgical correction is more often needed in cases with the neck deforming formations.","PeriodicalId":39654,"journal":{"name":"Pediatriya - Zhurnal im G.N. Speranskogo","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"83103733","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}
Neuroblastoma (NB) is the most common extracranial solid tumor in childhood. Prior to the introduction of intensive multimodal therapy, overall survival (OS) in patients with high-risk NB was below 15%. The addition of immunotherapy with dinutuximab beta in post-consolidation to multimodal treatment can increase the 5-year event-free survival rate for patients with high-risk NB up to 56.6%, and the 5-year OS up to 73.2%. The purpose of the research was to study the efficacy and safety of dinutuximab beta therapy in high-risk NB patients. Materials and methods used: the study included 8 pediatric patients with high-risk NB who had been treated with dinutuximab beta immunotherapy during postconsolidation on the basis of the Morozov Children’s City Clinical Hospital (Moscow, Russia) in 2020 to 2022. In case a patient's vital parameters normalization coupled with the absence of the need for accompanying therapy for 24 hours in a hospital, the patient was then considered as a candidate for outpatient treatment with possible no need for admission in the hospital. Results: 6 patients (75%) completed the full course of therapy with dinutuximab beta. The course of immunotherapy was not completed in 2 patients due to further progression of the disease. The most common adverse events were recorded as follows: pain (100%), fever (100%), diarrhea (62.5%), edematous syndrome (37.5%), allergic reactions (25%), hematological toxicity (25%). The period of time to becoming the candidates for outpatient treatment was 10.38±2.56 days for the 1st cycle of therapy and 2.67±1.63 days for the last, 5th cycle of the immunotherapy. In 6 out of 8 cases described the immunotherapy with dinutuximab beta allowed maintaining the response to treatment achieved after the end of consolidation preventing further development of the disease. The overall good tolerance to the drug made it possible to carry out the immunotherapy on an outpatient basis using microinfusing pump. Conclusion: an adequate route of administration and accompanying treatment make dinutuximab beta toxicity profile sufficient for outpatient management of patients with high-risk NB.
{"title":"IMMUNOTHERAPY WITH ANTI-GD2 MONOCLONAL ANTIBODIES IN HIGH-RISK NEUROBLASTOMA PATIENTS ON AN OUTPATIENT BASIS","authors":"M.V. Natrusova, G.O. Bronin, B.M. Kurmanov, V.V. Konstantinova, A.V. Lifshits, E.A. Zhuravel, N.V. Bronina, E.A. Burtsev, E.V. Kumirova","doi":"10.24110/0031-403x-2023-102-3-50-56","DOIUrl":"https://doi.org/10.24110/0031-403x-2023-102-3-50-56","url":null,"abstract":"Neuroblastoma (NB) is the most common extracranial solid tumor in childhood. Prior to the introduction of intensive multimodal therapy, overall survival (OS) in patients with high-risk NB was below 15%. The addition of immunotherapy with dinutuximab beta in post-consolidation to multimodal treatment can increase the 5-year event-free survival rate for patients with high-risk NB up to 56.6%, and the 5-year OS up to 73.2%. The purpose of the research was to study the efficacy and safety of dinutuximab beta therapy in high-risk NB patients. Materials and methods used: the study included 8 pediatric patients with high-risk NB who had been treated with dinutuximab beta immunotherapy during postconsolidation on the basis of the Morozov Children’s City Clinical Hospital (Moscow, Russia) in 2020 to 2022. In case a patient's vital parameters normalization coupled with the absence of the need for accompanying therapy for 24 hours in a hospital, the patient was then considered as a candidate for outpatient treatment with possible no need for admission in the hospital. Results: 6 patients (75%) completed the full course of therapy with dinutuximab beta. The course of immunotherapy was not completed in 2 patients due to further progression of the disease. The most common adverse events were recorded as follows: pain (100%), fever (100%), diarrhea (62.5%), edematous syndrome (37.5%), allergic reactions (25%), hematological toxicity (25%). The period of time to becoming the candidates for outpatient treatment was 10.38±2.56 days for the 1st cycle of therapy and 2.67±1.63 days for the last, 5th cycle of the immunotherapy. In 6 out of 8 cases described the immunotherapy with dinutuximab beta allowed maintaining the response to treatment achieved after the end of consolidation preventing further development of the disease. The overall good tolerance to the drug made it possible to carry out the immunotherapy on an outpatient basis using microinfusing pump. Conclusion: an adequate route of administration and accompanying treatment make dinutuximab beta toxicity profile sufficient for outpatient management of patients with high-risk NB.","PeriodicalId":39654,"journal":{"name":"Pediatriya - Zhurnal im G.N. Speranskogo","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135623946","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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