{"title":"The Role of Distinguished People in Creating the Century-Old History of Pediatrics Department in the Astrakhan Medical University","authors":"E. M. Shaforostova","doi":"10.15690/rpj.v4i1.2509","DOIUrl":"https://doi.org/10.15690/rpj.v4i1.2509","url":null,"abstract":"<jats:p>.</jats:p>","PeriodicalId":52396,"journal":{"name":"Russian Journal of Pediatric Hematology and Oncology","volume":"49 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-02-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"87362758","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"There is such outpatient clinic... To the 30th anniversary of the children’s outpatient clinic of the budgetary institution of the Khanty-Mansiisk autonomous district — Ugra “Surgut City Outpatient Clinic № 2”","authors":"E. A. Mikhailyova","doi":"10.15690/rpj.v4i1.2514","DOIUrl":"https://doi.org/10.15690/rpj.v4i1.2514","url":null,"abstract":"<jats:p>.</jats:p>","PeriodicalId":52396,"journal":{"name":"Russian Journal of Pediatric Hematology and Oncology","volume":"81 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-02-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"79144823","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Penza Regional Children's Hospital named after N.F. Filatov","authors":"L. A. Musatova, L. I. Krasnova, A. I. Chernova","doi":"10.15690/rpj.v4i1.2512","DOIUrl":"https://doi.org/10.15690/rpj.v4i1.2512","url":null,"abstract":"<jats:p>.</jats:p>","PeriodicalId":52396,"journal":{"name":"Russian Journal of Pediatric Hematology and Oncology","volume":"39 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-02-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"87227773","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"History of the Children Diseases Department of Ryazan State Medical University: the Way from the Past to the Present","authors":"A. V. Dmitriev, N. Fedina, V. Petrova","doi":"10.15690/rpj.v4i1.2510","DOIUrl":"https://doi.org/10.15690/rpj.v4i1.2510","url":null,"abstract":"<jats:p>.</jats:p>","PeriodicalId":52396,"journal":{"name":"Russian Journal of Pediatric Hematology and Oncology","volume":"31 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-02-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"75621373","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 : 2023-02-05DOI: 10.21682/2311-1267-2022-9-4-107-113
Y. S. Korkina, T. Valiev, K. Kirgizov, S. Varfolomeeva
Early T-cell precursor leukemia (early T-cell precursor, ETP-ALL) is a new subvariant of acute lymphoblastic leukemia (ALL). Tumor blasts have a unique phenotype, including signs of both stem and myeloid cells. This fact significantly complicates differential diagnosis. Cytogenetic and molecular biological features of cells in ETP-ALL are the most important diagnostic criteria. Nowadays the leading scientific international groups of pediatric leukemia conduct researches to develop new treatment protocols for ETP-ALL or to optimize existing chemotherapy regimens by including targeted drugs (bortezomib, ruxolitinib, venetoclax). Currently, hematopoietic stem cell transplantation is a mandatory option in the treatment of ETP-ALL. Targeted drugs and CAR-T-cell (chimeric antigen receptor of T-cells) therapy are the most perspective ways of posable treatment. In this article there are summarized data on diagnosis and therapy and a description of a successful treatment of a patient with ETP-ALL.
{"title":"Early T-cell precursor leukemia: questions of diagnosis, treatment and description of own clinical case","authors":"Y. S. Korkina, T. Valiev, K. Kirgizov, S. Varfolomeeva","doi":"10.21682/2311-1267-2022-9-4-107-113","DOIUrl":"https://doi.org/10.21682/2311-1267-2022-9-4-107-113","url":null,"abstract":" Early T-cell precursor leukemia (early T-cell precursor, ETP-ALL) is a new subvariant of acute lymphoblastic leukemia (ALL). Tumor blasts have a unique phenotype, including signs of both stem and myeloid cells. This fact significantly complicates differential diagnosis. Cytogenetic and molecular biological features of cells in ETP-ALL are the most important diagnostic criteria. Nowadays the leading scientific international groups of pediatric leukemia conduct researches to develop new treatment protocols for ETP-ALL or to optimize existing chemotherapy regimens by including targeted drugs (bortezomib, ruxolitinib, venetoclax). Currently, hematopoietic stem cell transplantation is a mandatory option in the treatment of ETP-ALL. Targeted drugs and CAR-T-cell (chimeric antigen receptor of T-cells) therapy are the most perspective ways of posable treatment. In this article there are summarized data on diagnosis and therapy and a description of a successful treatment of a patient with ETP-ALL.","PeriodicalId":52396,"journal":{"name":"Russian Journal of Pediatric Hematology and Oncology","volume":"22 10 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-02-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"88255096","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 : 2023-02-05DOI: 10.21682/2311-1267-2022-9-4-114-118
M. E. Prokofiev, I. Kostareva, K. Sergeenko, N. Stepanyan, N. Sidorova, K. Kirgizov
Fanconi anemia (AF) is a hereditary genetic disease characterized by developmental abnormalities, progressive bone marrow failure, hypersensitivity to alkylating agents, and a tendency to hematological and solid tumors throughout life. The only curative option in the treatment of bone marrow failure in patients with AF is allogeneic hematopoietic stem cell transplantation (allo-HSCT). There are no detailed descriptions of allo-HSCT in patients with AF in the Russian-language literature. On the example of a clinical case with AF at the onset of myelodysplastic syndrome, a choose of method for treating bone marrow failure is presented.
{"title":"Clinical case: allogeneic transplantation of hematopoietic stem cells for Fanconi anemia in the onset of refractory anemia with excess blasts","authors":"M. E. Prokofiev, I. Kostareva, K. Sergeenko, N. Stepanyan, N. Sidorova, K. Kirgizov","doi":"10.21682/2311-1267-2022-9-4-114-118","DOIUrl":"https://doi.org/10.21682/2311-1267-2022-9-4-114-118","url":null,"abstract":" Fanconi anemia (AF) is a hereditary genetic disease characterized by developmental abnormalities, progressive bone marrow failure, hypersensitivity to alkylating agents, and a tendency to hematological and solid tumors throughout life. The only curative option in the treatment of bone marrow failure in patients with AF is allogeneic hematopoietic stem cell transplantation (allo-HSCT). There are no detailed descriptions of allo-HSCT in patients with AF in the Russian-language literature. On the example of a clinical case with AF at the onset of myelodysplastic syndrome, a choose of method for treating bone marrow failure is presented.","PeriodicalId":52396,"journal":{"name":"Russian Journal of Pediatric Hematology and Oncology","volume":"7 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-02-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"89001306","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 : 2023-02-05DOI: 10.21682/2311-1267-2022-9-4-119-121
T. Belysheva, S. Varfolomeeva, I. Zakharova, A. Zuev, N. P. Katukova, Y. Polyaev, V. K. Fedyaeva, K. I. Kyrgyzov, G. B. Sagoyan
On October 19, 2022, the Council of Experts considered and discussed a number of issues related to the treatment of patients with infantile hemangioma in the Russian Federation.
2022年10月19日，专家委员会审议并讨论了与俄罗斯联邦婴儿血管瘤患者治疗相关的一些问题。
{"title":"Resolution on the results of the Council of Experts on the topic: “Infantile hemangioma is an interdisciplinary problem that has a unified solution: from detection/diagnosis and treatment to reimbursement”","authors":"T. Belysheva, S. Varfolomeeva, I. Zakharova, A. Zuev, N. P. Katukova, Y. Polyaev, V. K. Fedyaeva, K. I. Kyrgyzov, G. B. Sagoyan","doi":"10.21682/2311-1267-2022-9-4-119-121","DOIUrl":"https://doi.org/10.21682/2311-1267-2022-9-4-119-121","url":null,"abstract":" On October 19, 2022, the Council of Experts considered and discussed a number of issues related to the treatment of patients with infantile hemangioma in the Russian Federation.","PeriodicalId":52396,"journal":{"name":"Russian Journal of Pediatric Hematology and Oncology","volume":"44 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-02-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"84220262","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 : 2023-02-05DOI: 10.21682/2311-1267-2022-9-4-125-128
T. Valiev
The success in pediatric oncology and hematology diseases treatment opened the way to cure more than 90 % of patients with leukemias and lemphomas. The ability to share an achievements in modern paediatric oncology and hematology, to find an actual directions of development, to permit the specialists from every corner of the world to communicate and discuss the most difficult aspects in diagnosis and treatment of paediatric malignancies – the basis of the International Society of Paediatric Oncology (SIOP) work. In the current issue it is presented the thesis of reports of 54th SIOP Congress, which took place in Spain (Barcelona) from September 28 to October 1, 2022. New target drugs (blinatumomab, inotuzumab ozogamicin, dinutuximab, venetoclax, daratumumab, asciminib), cellular (CAR-T) and immune approaches for malignancies treatment, new achievements in supportive care, psychological and social aspects of medical care organization for paediatric patients with malignancies – only a few topics, that were discussed of the event.
{"title":"Innovative advances in pediatric oncology and hematology: data of 54th Congress of the International Society of Paediatric Oncology (SIOP)","authors":"T. Valiev","doi":"10.21682/2311-1267-2022-9-4-125-128","DOIUrl":"https://doi.org/10.21682/2311-1267-2022-9-4-125-128","url":null,"abstract":" The success in pediatric oncology and hematology diseases treatment opened the way to cure more than 90 % of patients with leukemias and lemphomas. The ability to share an achievements in modern paediatric oncology and hematology, to find an actual directions of development, to permit the specialists from every corner of the world to communicate and discuss the most difficult aspects in diagnosis and treatment of paediatric malignancies – the basis of the International Society of Paediatric Oncology (SIOP) work. In the current issue it is presented the thesis of reports of 54th SIOP Congress, which took place in Spain (Barcelona) from September 28 to October 1, 2022. New target drugs (blinatumomab, inotuzumab ozogamicin, dinutuximab, venetoclax, daratumumab, asciminib), cellular (CAR-T) and immune approaches for malignancies treatment, new achievements in supportive care, psychological and social aspects of medical care organization for paediatric patients with malignancies – only a few topics, that were discussed of the event.","PeriodicalId":52396,"journal":{"name":"Russian Journal of Pediatric Hematology and Oncology","volume":"13 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-02-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"88169209","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 : 2023-02-04DOI: 10.21682/2311-1267-2022-9-4-96-101
D. Akhaladze, L. L. Rabaeva, A. A. Krivonosov, N. Merkulov, S. Talypov, I. V. Tverdov, N. Uskova, G. Rabaev, N. S. Grachev
Peritoneal sarcomatosis/carcinomatosis is a rare observation in pediatric practice. It occurs, as a rule, with various sarcomas, sarcomatoid tumors, desmoplastic small round cell tumors. Cytoreductive surgery followed by abdominal chemoperfusion has established itself as an effective method of treating such conditions in adult practice and is gaining popularity in pediatric practice. Laparoscopic cytoreduction followed by hyperthermic chemoperfusion used in adult patients has a number of advantages: early recovery after surgery, reduced hospital days, timely initiation of adjuvant chemotherapy, however, the experience of using a minimally invasive technique in children is limited. The article presents the first clinical observation in Russian practice of a 17-year-old patient with embryonal testicular rhabdomyosarcoma and peritoneal sarcomatosis of the abdominal cavity, which underwent laparoscopic cytoreductive surgery with hyperthermic chemoperfusion.
{"title":"Laparoscopic cytoreductive surgery with hyperthermic chemoperfusion of the abdominal cavity for peritoneal sarcomatosis in a child with embryonal rhabdomyosarcoma. The first Russian experience in pediatric practice","authors":"D. Akhaladze, L. L. Rabaeva, A. A. Krivonosov, N. Merkulov, S. Talypov, I. V. Tverdov, N. Uskova, G. Rabaev, N. S. Grachev","doi":"10.21682/2311-1267-2022-9-4-96-101","DOIUrl":"https://doi.org/10.21682/2311-1267-2022-9-4-96-101","url":null,"abstract":" Peritoneal sarcomatosis/carcinomatosis is a rare observation in pediatric practice. It occurs, as a rule, with various sarcomas, sarcomatoid tumors, desmoplastic small round cell tumors. Cytoreductive surgery followed by abdominal chemoperfusion has established itself as an effective method of treating such conditions in adult practice and is gaining popularity in pediatric practice. Laparoscopic cytoreduction followed by hyperthermic chemoperfusion used in adult patients has a number of advantages: early recovery after surgery, reduced hospital days, timely initiation of adjuvant chemotherapy, however, the experience of using a minimally invasive technique in children is limited. The article presents the first clinical observation in Russian practice of a 17-year-old patient with embryonal testicular rhabdomyosarcoma and peritoneal sarcomatosis of the abdominal cavity, which underwent laparoscopic cytoreductive surgery with hyperthermic chemoperfusion.","PeriodicalId":52396,"journal":{"name":"Russian Journal of Pediatric Hematology and Oncology","volume":"70 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-02-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"86308809","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 : 2023-02-04DOI: 10.21682/2311-1267-2022-9-4-102-106
I. Sidorov, A. Fedorova, E. Konopleva, A. Sharlai, D. Konovalov
Ewing sarcoma (ES) is one of the most frequent primary bone tumors and has a well-studied diagnostically important genetic background. However, there are primary bone round-cell tumors with atypical morphology different from conventional ES, tumors with rearrangement of the EWSR1 gene with partner genes not from the ETS gene family, tumors with unusual changes in the EWSR1 gene (amplification or deletion), which can cause significant diagnostic difficulties. In this article, we will describe a case of a primary bone tumor with an atypical morphology similar to myoepithelial carcinoma, where an amplification of the EWSR1 gene was detected. According to morphological, immunological, genetic and clinical signs, this tumor was classified as a sarcoma from the EWSR1::non-ETS group of round-cell sarcomas, namely a sarcoma with EWSR1::NFATC2 rearrangement, first formalized in the WHO classification of soft tissue tumors in 2020.
{"title":"Sarcoma of the right femur with heterogeneous morphology similar to myoepithelial carcinoma and amplification of the EWSR1 gene in a 14-year-old boy. Clinical observation and literature review","authors":"I. Sidorov, A. Fedorova, E. Konopleva, A. Sharlai, D. Konovalov","doi":"10.21682/2311-1267-2022-9-4-102-106","DOIUrl":"https://doi.org/10.21682/2311-1267-2022-9-4-102-106","url":null,"abstract":" Ewing sarcoma (ES) is one of the most frequent primary bone tumors and has a well-studied diagnostically important genetic background. However, there are primary bone round-cell tumors with atypical morphology different from conventional ES, tumors with rearrangement of the EWSR1 gene with partner genes not from the ETS gene family, tumors with unusual changes in the EWSR1 gene (amplification or deletion), which can cause significant diagnostic difficulties. In this article, we will describe a case of a primary bone tumor with an atypical morphology similar to myoepithelial carcinoma, where an amplification of the EWSR1 gene was detected. According to morphological, immunological, genetic and clinical signs, this tumor was classified as a sarcoma from the EWSR1::non-ETS group of round-cell sarcomas, namely a sarcoma with EWSR1::NFATC2 rearrangement, first formalized in the WHO classification of soft tissue tumors in 2020.","PeriodicalId":52396,"journal":{"name":"Russian Journal of Pediatric Hematology and Oncology","volume":"6 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-02-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"81649767","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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