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。分别对婴儿(1岁，58/12%)、幼儿(1-3岁，99/20%)和3-18岁儿童(335/68%)进行分析。结果:婴儿组的特点是白细胞增多、髓外病变发生率较高，单核细胞/单核细胞(63%)和巨核细胞(24%)亚变异体占主导地位，涉及11q23位点(KMT2A基因)的染色体畸变(53%)。“CBF白血病”在婴儿中几乎不存在(t(8;21)(q22;q22.1)/RUNX1::RUNX1T1为0%，inv(16)(p13.1q22)/CBF::MYH11为2%)，并且随着年龄的增长而增加。FLT3基因的激活突变极为罕见(2% vs. 13.5%)。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.K. Ignatova, I.I. Kalinina, D.A. Venyov, D.A. Evseev, T.Yu. Salimova, M.N. Sadovskaya, O.V. Goronkova, E.V. Suntsova, V.E. Matveev, U.N. Petrova, K.S. Antonova, D.D. Baydildina, K.A. Voronin, P.A. Levin, M.E. Dubrovina, T.V. Konyukhova, Yu.V. Olshanskaya, E.A. Zerkalenkova, M.V. Gaskova, A.M. Popov, S.A. Kashpor, M.A. Maschan, A.A. Maschan","doi":"10.24110/0031-403x-2023-102-3-8-15","DOIUrl":"https://doi.org/10.24110/0031-403x-2023-102-3-8-15","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":"135623950","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}
The article is about the experience in treating of pediatric patients with tumors of the pancreas who have undergone the gastro-pancreatoduodenal resection (GPDR) and pancreatoduodenal resection (PDR) 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). These kinds of surgical interventions are performed both with local neoplasms of the pancreatic head and with the spread of the tumor process to the duodenum, distal part of the stomach, and parapancreatic tissue. Materials and methods used: 13 GPDR, PDR surgical interventions were performed in children aged 5 to 16 y/o in 2010-2022. The results of treatment of these patients were assessed as satisfactory despite the complications that arose in some patients in the early and late postoperative periods. All patients are alive. GPDR surgical interventions were performed in 6 patients, and PDR in 7. The most frequently verified were solid pseudopapillary tumor of the pancreatic head (in 10 cases), neuroendocrine tumor (in 2 cases) and paraganglioma in a single case. Discussion and conclusions: bleeding from the pancreatic branch of the splenic artery and acute pancreatitis were noted among the early postoperative complications. Recurrent phenomenon of enzyme evasion and syndrome of excessive bacterial growth were revealed in the late postoperative period. Despite the complications arisen all patients are alive without signs of recurrence of the disease. The prognosis for this category of patients with radically performed surgical intervention is favorable.
本文是关于N.N. Blokhin俄罗斯癌症研究中心(Moscow, Russia)以L.A. Durnov院士命名的儿童肿瘤学和血液学研究所(academy of L.A. Durnov)对行胃胰十二指肠切除术(GPDR)和胰十二指肠切除术(PDR)的儿童胰腺肿瘤患者的治疗经验。这些类型的手术干预既适用于胰腺头部的局部肿瘤，也适用于肿瘤扩散到十二指肠、胃远端和胰腺旁组织的肿瘤。采用的材料和方法:2010-2022年对5 ~ 16岁儿童进行了13例GPDR、PDR手术干预。尽管一些患者在术后早期和后期出现并发症，但这些患者的治疗结果令人满意。所有病人都还活着。GPDR手术干预6例，PDR手术干预7例。最常见的是胰头实性假乳头状瘤(10例)、神经内分泌瘤(2例)和副神经节瘤(1例)。讨论与结论:术后早期并发症以脾动脉胰支出血和急性胰腺炎为主。术后后期出现酶逃避和细菌过度生长综合征反复出现。尽管出现了并发症，但所有患者都存活，没有疾病复发的迹象。这类患者经根治性手术干预后预后良好。
{"title":"Possibility for gastro-pancreatoduodenal and pancreatoduodenal surgical resections in children with pancreatic tumors","authors":"D.V. Rybakova, P.A. Kerimov, D.V. Podluzhny, Y.I. Patyutko, M.A. Rubansky, A.S. Temnyy, E.A. Petrash, S.R. Varfolomeeva, I.S. Stilidi","doi":"10.24110/0031-403x-2023-102-3-57-62","DOIUrl":"https://doi.org/10.24110/0031-403x-2023-102-3-57-62","url":null,"abstract":"The article is about the experience in treating of pediatric patients with tumors of the pancreas who have undergone the gastro-pancreatoduodenal resection (GPDR) and pancreatoduodenal resection (PDR) 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). These kinds of surgical interventions are performed both with local neoplasms of the pancreatic head and with the spread of the tumor process to the duodenum, distal part of the stomach, and parapancreatic tissue. Materials and methods used: 13 GPDR, PDR surgical interventions were performed in children aged 5 to 16 y/o in 2010-2022. The results of treatment of these patients were assessed as satisfactory despite the complications that arose in some patients in the early and late postoperative periods. All patients are alive. GPDR surgical interventions were performed in 6 patients, and PDR in 7. The most frequently verified were solid pseudopapillary tumor of the pancreatic head (in 10 cases), neuroendocrine tumor (in 2 cases) and paraganglioma in a single case. Discussion and conclusions: bleeding from the pancreatic branch of the splenic artery and acute pancreatitis were noted among the early postoperative complications. Recurrent phenomenon of enzyme evasion and syndrome of excessive bacterial growth were revealed in the late postoperative period. Despite the complications arisen all patients are alive without signs of recurrence of the disease. The prognosis for this category of patients with radically performed surgical intervention is favorable.","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":"135623947","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-2023-102-3-137-144
Y. Dinikina
Intensification of antitumor treatment using high-dose chemotherapy (HDCT) with autologous hematopoietic stem cell transplantation (aHSCT) has been an effective option for many years in a number of nosological forms of central nervous system (CNS) tumors in children as the first-choice therapy. However, its benefits in relapsing forms of the disease are not obvious, which has led to ongoing discussions. Studies on the comparative efficacy of HDCT in recurrent CNS tumors are few. The heterogeneity of the studied patient cohorts should be noted additionally, which in its turn does not allow reaching a consensus on indications for its use. The Article represents current bibliographical data on the use of HDCT with aHSCT in pediatric patients with relapses of various nosological forms of CNS tumors in terms of its possible effectiveness and toxicity.
{"title":"HIGH-DOSE CHEMOTHERAPY WITH AUTOLOGOUS HEMATOPOIETIC STEM CELL TRANSPLANTATION IN RELAPSED CENTRAL NERVOUS SYSTEM TUMORS: CHALLENGES AND RESULTS","authors":"Y. Dinikina","doi":"10.24110/0031-403x-2023-102-3-137-144","DOIUrl":"https://doi.org/10.24110/0031-403x-2023-102-3-137-144","url":null,"abstract":"Intensification of antitumor treatment using high-dose chemotherapy (HDCT) with autologous hematopoietic stem cell transplantation (aHSCT) has been an effective option for many years in a number of nosological forms of central nervous system (CNS) tumors in children as the first-choice therapy. However, its benefits in relapsing forms of the disease are not obvious, which has led to ongoing discussions. Studies on the comparative efficacy of HDCT in recurrent CNS tumors are few. The heterogeneity of the studied patient cohorts should be noted additionally, which in its turn does not allow reaching a consensus on indications for its use. The Article represents current bibliographical data on the use of HDCT with aHSCT in pediatric patients with relapses of various nosological forms of CNS tumors in terms of its possible effectiveness and toxicity.","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":"77704295","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-2023-102-3-58-64
M. Natrusova, G. Bronin, B.M. Kurmanov, V. Konstantinova, A. V. Lifshits, E. A. Zhuravel, N. V. Bronina, E. A. Burtsev, E. Kumirova
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. Natrusova, G. Bronin, B.M. Kurmanov, V. Konstantinova, A. V. Lifshits, E. A. Zhuravel, N. V. Bronina, E. A. Burtsev, E. Kumirova","doi":"10.24110/0031-403x-2023-102-3-58-64","DOIUrl":"https://doi.org/10.24110/0031-403x-2023-102-3-58-64","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":"88569218","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-2023-102-3-79-94
A. Glotov, A. Kiselev, M. Maretina, L.M. Shchyugareva, S. Shtykalova, N. Maslyanyuk, M.V. Plokhikh, T.V. Kabakova, A. Andreeva, L. Koroleva, O.B. Sokolova, T.A. Antsiferova, M.E. Orlovskaya, A. Kyanksep, O. Nevmerzhitskaya, E. S. Kuznetsova, E.N. Slastenova, S. Vinogradova, Y. Pavlenko, M. Kruchina, N. A. Filatova, V. Bezhenar, O.Yu. Krishtofik, M.B. Fedotova, S.A. Fomin, S. V. Moiseev, I. Sosnina, A. Koroteev, Yuu V Gorelik, O. Bespalova, I. Kogan, V. Baranov
Spinal muscular atrophy (SMA) is one of the most frequent and severe hereditary diseases that leads to the destruction of motor neurons in the spinal cord and muscle weakness and is resulted from mutations in the SMN1 gene. The purpose of this research was to identify homo- and heterozygous mutations in the SMN1 gene in newborns. Materials and methods used: a multicenter prospective open continuous screening study of newborns at the age of 4 DoL (for full-term)/7 DoL (for preterm). The screening was carried out in Dec. 30, 2021-Nov. 17, 2022 on the basis of 21 obstetrics facilities located within the City of Saint Petersburg (Russia). Results: 36,140 participants (17,687 (49%) boys/18,453 (51%) girls) of the neonatal screening; 77 (0.21%) parents/caretakers have refused to participate. A technique for detecting homo- and heterozygous deletions in the SMN1 gene was developed based on rapid DNA extraction from dried blood spots followed by the real-time PCR. The project had identified 4 patients with SMA, two of whom had two and the other two had three copies of the SMN2 gene. The period of time needed to set the diagnosis ranged from 7th to 14th DoL. All of these patients have received the gene replacement therapy for a period of 3 to 5 months. There was a positive trend as a result of the therapy in all of the cases. 772 SMA carriers were also identified during the screening. 51% of their parents/caretakers have been followingly consulted on the possible risks of the disease. The frequency of SMA in the City of Saint Petersburg was 1 in 9035 newborns with a carrier frequency of 1 in 47. The sensitivity and specificity of the test were determined, which were 99.74% and 100%, respectively. Conclusion: SMA screening has been tested and put into practice. The conducted project of neonatal screening for SMA in the City of Saint Petersburg has demonstrated the readiness of its residents and the municipal healthcare system, the practitioners and organizers to this type of medical solution. The described developed methodology has demonstrated its effectiveness in terms of early detection of patients and carriers of this disease.
{"title":"Aftermath of the pilot project on neonatal screening for spinal muscular atrophy in the City of Saint Petersburg, Russia. Organizational, diagnostic and clinical aspects","authors":"A. Glotov, A. Kiselev, M. Maretina, L.M. Shchyugareva, S. Shtykalova, N. Maslyanyuk, M.V. Plokhikh, T.V. Kabakova, A. Andreeva, L. Koroleva, O.B. Sokolova, T.A. Antsiferova, M.E. Orlovskaya, A. Kyanksep, O. Nevmerzhitskaya, E. S. Kuznetsova, E.N. Slastenova, S. Vinogradova, Y. Pavlenko, M. Kruchina, N. A. Filatova, V. Bezhenar, O.Yu. Krishtofik, M.B. Fedotova, S.A. Fomin, S. V. Moiseev, I. Sosnina, A. Koroteev, Yuu V Gorelik, O. Bespalova, I. Kogan, V. Baranov","doi":"10.24110/0031-403x-2023-102-3-79-94","DOIUrl":"https://doi.org/10.24110/0031-403x-2023-102-3-79-94","url":null,"abstract":"Spinal muscular atrophy (SMA) is one of the most frequent and severe hereditary diseases that leads to the destruction of motor neurons in the spinal cord and muscle weakness and is resulted from mutations in the SMN1 gene. The purpose of this research was to identify homo- and heterozygous mutations in the SMN1 gene in newborns. Materials and methods used: a multicenter prospective open continuous screening study of newborns at the age of 4 DoL (for full-term)/7 DoL (for preterm). The screening was carried out in Dec. 30, 2021-Nov. 17, 2022 on the basis of 21 obstetrics facilities located within the City of Saint Petersburg (Russia). Results: 36,140 participants (17,687 (49%) boys/18,453 (51%) girls) of the neonatal screening; 77 (0.21%) parents/caretakers have refused to participate. A technique for detecting homo- and heterozygous deletions in the SMN1 gene was developed based on rapid DNA extraction from dried blood spots followed by the real-time PCR. The project had identified 4 patients with SMA, two of whom had two and the other two had three copies of the SMN2 gene. The period of time needed to set the diagnosis ranged from 7th to 14th DoL. All of these patients have received the gene replacement therapy for a period of 3 to 5 months. There was a positive trend as a result of the therapy in all of the cases. 772 SMA carriers were also identified during the screening. 51% of their parents/caretakers have been followingly consulted on the possible risks of the disease. The frequency of SMA in the City of Saint Petersburg was 1 in 9035 newborns with a carrier frequency of 1 in 47. The sensitivity and specificity of the test were determined, which were 99.74% and 100%, respectively. Conclusion: SMA screening has been tested and put into practice. The conducted project of neonatal screening for SMA in the City of Saint Petersburg has demonstrated the readiness of its residents and the municipal healthcare system, the practitioners and organizers to this type of medical solution. The described developed methodology has demonstrated its effectiveness in terms of early detection of patients and carriers of this disease.","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":"88726033","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-2023-102-3-50-57
N. Martynova, N. Vorobyov, K. Boyko, Yu. V. Gutsalo, M. Linnik, K. Suprun, G. Andreev, A. Lyubinskiy, E. Spiridenko, A. Kalesnik
Repeated craniospinal irradiation (CSR) is one of the options in the treatment of multifocal recurrences of medulloblastomas (MB), however, it is prescribed to a limited extent due to its toxicity. The use of proton therapy can reduce the toxicity of treatment. Materials and methods used: a single-center retrospective cohort study was conducted in 25 patients (18 (72%) boys/7 (28%) girls) aged 7 to 18 y/o (median 13 (10-14) y/o) in 2019-2023. Repeated CSR was performed for leptomeningeal MB recurrence using a proton beam at the Treatment and Diagnostic Center of the International Institute of Biological Systems named after Sergey Berezin (Saint Petersburg, Russia). The median dose of primary CSR was 35.2 Gy (24-35.2). The median dose of repeated CSR was 30.6 Gy (24-35.2). 23 patients after CSR were given a local boost on the area of metastatic lesions and local recurrence (if any). 7 had previously received courses of repeated radiation therapy for recurrent MB. Results: during the course of the treatment thrombocytopenia was noted with a statistically significant decrease by the 3rd week of treatment (M=97x109/l, p<0.001). The median follow-up was 12.5 months (8-19.5). In 8 patients, progression of the disease was observed, which resulted in the death of 7 of them. In one patient, the appearance of radiation necrosis in the local boost area (left cerebellar peduncle) was noted 5 months after the completion of the repeated irradiation course. The median progression period was 17 months (95% CI 10.8-23.1). Conclusions: proton therapy makes it possible to deliver therapeutic doses for CSR with an acceptable level of hematological toxicity. The obtained data on relapse-free and overall survival indicate the need for further study of the role of repeated CSR and the development of a methodology for calculating the permissible maximum effective dose during the repeated exposure.
反复颅脊髓照射(CSR)是治疗髓母细胞瘤(MB)多灶性复发的选择之一，但由于其毒性，其使用范围有限。使用质子治疗可以减少治疗的毒性。使用的材料和方法:在2019-2023年对25例患者(18例(72%)男孩/7例(28%)女孩)进行了单中心回顾性队列研究，年龄为7 - 18岁(中位数为13例(10-14)岁)。在Sergey Berezin(圣彼得堡，俄罗斯)命名的国际生物系统研究所治疗和诊断中心，使用质子束对脑膜轻脑膜MB复发进行重复CSR。原发性CSR的中位剂量为35.2 Gy(24-35.2)。重复CSR的中位剂量为30.6 Gy(24-35.2)。23例患者在CSR后给予局部增强转移灶和局部复发(如果有的话)的面积。结果:治疗过程中血小板减少，治疗第3周明显减少(M=97x109/l, p<0.001)。中位随访时间为12.5个月(8-19.5)。在8例患者中，观察到疾病进展，导致7例死亡。1例患者在重复放射治疗完成5个月后，局部增强区(左小脑脚)出现放射性坏死。中位进展期为17个月(95% CI 10.8-23.1)。结论:质子治疗使CSR的治疗剂量达到可接受的血液学毒性水平成为可能。获得的无复发和总生存期数据表明，需要进一步研究重复CSR的作用，并制定一种计算重复暴露期间允许的最大有效剂量的方法。
{"title":"REPEATED CRANIOSPINAL IRRADIATION IN PEDIATRIC PATIENTS WITH RECURRENT MEDULLOBLASTOMA: PROTON THERAPY EXPERIENCE AT THE TREATMENT AND DIAGNOSTIC CENTER OF THE INTERNATIONAL INSTITUTE OF BIOLOGICAL SYSTEMS NAMED AFTER SERGEY BEREZIN","authors":"N. Martynova, N. Vorobyov, K. Boyko, Yu. V. Gutsalo, M. Linnik, K. Suprun, G. Andreev, A. Lyubinskiy, E. Spiridenko, A. Kalesnik","doi":"10.24110/0031-403x-2023-102-3-50-57","DOIUrl":"https://doi.org/10.24110/0031-403x-2023-102-3-50-57","url":null,"abstract":"Repeated craniospinal irradiation (CSR) is one of the options in the treatment of multifocal recurrences of medulloblastomas (MB), however, it is prescribed to a limited extent due to its toxicity. The use of proton therapy can reduce the toxicity of treatment. Materials and methods used: a single-center retrospective cohort study was conducted in 25 patients (18 (72%) boys/7 (28%) girls) aged 7 to 18 y/o (median 13 (10-14) y/o) in 2019-2023. Repeated CSR was performed for leptomeningeal MB recurrence using a proton beam at the Treatment and Diagnostic Center of the International Institute of Biological Systems named after Sergey Berezin (Saint Petersburg, Russia). The median dose of primary CSR was 35.2 Gy (24-35.2). The median dose of repeated CSR was 30.6 Gy (24-35.2). 23 patients after CSR were given a local boost on the area of metastatic lesions and local recurrence (if any). 7 had previously received courses of repeated radiation therapy for recurrent MB. Results: during the course of the treatment thrombocytopenia was noted with a statistically significant decrease by the 3rd week of treatment (M=97x109/l, p<0.001). The median follow-up was 12.5 months (8-19.5). In 8 patients, progression of the disease was observed, which resulted in the death of 7 of them. In one patient, the appearance of radiation necrosis in the local boost area (left cerebellar peduncle) was noted 5 months after the completion of the repeated irradiation course. The median progression period was 17 months (95% CI 10.8-23.1). Conclusions: proton therapy makes it possible to deliver therapeutic doses for CSR with an acceptable level of hematological toxicity. The obtained data on relapse-free and overall survival indicate the need for further study of the role of repeated CSR and the development of a methodology for calculating the permissible maximum effective dose during the repeated exposure.","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":"85981250","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-2023-102-3-25-32
Yu.Yu. Kozel, D.Yu. Yurchenko, E.P. Ulyanova, A.B. Sagakyants, G.A. Mkrtchyan, E.E. Pak, V.V. Dmitrieva, M.V. Starzhetskaya, O.V. Kozyuk, A.I. Bespalova, O.P. Popovyan, V.A. Kovalenko, L.B. Kushtova
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
本研究的目的是考虑IDRF、肿瘤大小等影响手术结果的标准，制定儿童腹部定位神经源性肿瘤腹腔镜手术干预的风险、难度和复杂性评估量表。材料与方法:2018年1月- 4月，124例0 ~ 18岁(中位年龄20.5[5-50.5]个月)行腹腔镜下腹腔定位神经源性肿瘤手术的患者(男孩68例(55%)/女孩56例(45%))。2022例纳入单中心回顾性队列研究。分析了手术干预复杂性的24项标准与手术结果的参数评价(手术干预时间;术中出血量;术中输血;术中并发症和通路转换)。定量变量采用Spearman等级相关系数，定性变量采用eta系数。采用聚类分析方法根据难度等级来分配分数。采用高复杂程度敏感性和特异性的ROC曲线分析来检验复杂程度。结果:手术干预的中位时间为105 [75];150] min，术中出血量- 5 [5];15例(12%)需要输血，输血中位数为0 [0];4例(3%)出现术中并发症。4例(3%)患者进行了转换。选取10项复杂性标准:IDRF F1、F2、F3、F4、F5、肿瘤中心位置、中线外延伸、既往开放手术、肿瘤与下腔静脉接触、肿瘤体积(ml)/高度(m)比>或者</= 28。已经开发了一个具有三个难度等级的试点难度量表。高度复杂性(6分或以上)的敏感性为60% (95% CI 23.1-88.2%)，特异性为98.3% (95% CI 94.1-99.5%)。结论:不同的IDRFs对腹腔镜手术的复杂性和风险有不同的影响。与肿瘤的绝对大小相比，肿瘤大小与生长的比值是判断复杂性更重要的标准。复杂性的其他标准也起着重要的作用，例如以前的开放手术和肿瘤定位。作者不推荐该量表用于临床，因为本研究获得的结果将为进一步的前瞻性研究奠定基础，旨在制定更准确的腹部定位神经源性肿瘤腹腔镜手术复杂性量表。
{"title":"PILOT VERSION OF SCORING SYSTEM FOR LAPAROSCOPIC SURGERY OF ABDOMINAL NEUROBLASTOMA IN CHILDREN","authors":"Yu.Yu. Kozel, D.Yu. Yurchenko, E.P. Ulyanova, A.B. Sagakyants, G.A. Mkrtchyan, E.E. Pak, V.V. Dmitrieva, M.V. Starzhetskaya, O.V. Kozyuk, A.I. Bespalova, O.P. Popovyan, V.A. Kovalenko, L.B. Kushtova","doi":"10.24110/0031-403x-2023-102-3-25-32","DOIUrl":"https://doi.org/10.24110/0031-403x-2023-102-3-25-32","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":"135626576","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-2023-102-3-201-207
V.S. Fominykh, N. Batmanova, T. Valiev, I. Nazarenko, K. Kirgizov, S. Varfolomeeva
Langerhans cell histiocytosis (LCH) is the most common histiocytic disorder in children and is characterized by clonal expansion of myeloid precursors. The pathological cells are morphologically and phenotypically similar to Langerhans cells. LCH is considered as a sporadic disease as yet with undescribed hereditary factors for its development. However, hereditary predisposition to the development of this pathology cannot be excluded. There are cases of the LCH development in twins described, both in monozygotic and dizygotic. Article represents bibliographical review on the familial forms of Langerhans cell histiocytosis and clinical case of the Langerhans cell histiocytosis development in twin girls.
{"title":"Langerhans cell histiocytosis in twins: bibliographical review and clinical case","authors":"V.S. Fominykh, N. Batmanova, T. Valiev, I. Nazarenko, K. Kirgizov, S. Varfolomeeva","doi":"10.24110/0031-403x-2023-102-3-201-207","DOIUrl":"https://doi.org/10.24110/0031-403x-2023-102-3-201-207","url":null,"abstract":"Langerhans cell histiocytosis (LCH) is the most common histiocytic disorder in children and is characterized by clonal expansion of myeloid precursors. The pathological cells are morphologically and phenotypically similar to Langerhans cells. LCH is considered as a sporadic disease as yet with undescribed hereditary factors for its development. However, hereditary predisposition to the development of this pathology cannot be excluded. There are cases of the LCH development in twins described, both in monozygotic and dizygotic. Article represents bibliographical review on the familial forms of Langerhans cell histiocytosis and clinical case of the Langerhans cell histiocytosis development in twin girls.","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":"77966519","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-2023-102-3-95-106
S. Deryabina, I. Tuzankina, D. A. Cheremokhin, V. Shershnev, D. Kudlay, M. Litvinova, E. Vlasova
Sverdlovsk Oblast has become one of the first Russia regions to participate in a pilot project of neonatal screening for the detection of severe forms of primary immunodeficiency (PID) in newborns yet back in 2021, prior to the nationwide rollout of the Expanded Screening Program. The purpose of this research was to evaluate preliminary the effectiveness of identifying children with severe combined immunodeficiencies as well as other life-threatening forms of lymphopenia at the stage of possible impact on the course and outcome of the disease. Materials and methods used: quantitative determination of TREC/KREC in dry blood spots was carried out in 5044 children born in the region in July-Sept. 2021. The testing was performed using the domestic “IMMUNO-BIT” kit by “ABV-TEST,” LTD (Skolkovo Innovation Center, Mozhaysky District, Moscow, Russia). Cutoffs suggested in the instructions were used: 500 copies per 105 leukocytes for TREC and 300 copies per 105 leukocytes for KREC. Results: the risk group included 157 newborns (68 girls and 89 boys) with low TREC and/or KREC values (3.1%), no children were found with a complete absence of markers. After testing the re-requested samples, 104 of them were determined to be false positive, and 53 families were invited for an immunologist’s appointment. 28 children received in-depth examination with the advisory. At the time of appointment, the condition of most of them was assessed as clinically stable, however, according to the results of laboratory and instrumental diagnostic examinations, the presence of immunological abnormalities and developmental features was demonstrated: changes in the thymus (50%), neutropenia (32%), lymphopenia and thrombocytopenia (28%), hypogammaglobulinemia (84%), congenital malformations (30%). Conclusion: for the correct understanding of the phenomenon of reduced TREC/KREC values (but not zero values) in some newborns and predicting outcomes, it is necessary to monitor the health status of these patients, which can be carried out through the formation of a dynamic observation group, which is impossible without active identification of such children through wide screening program.
{"title":"Pilot regional project of neonatal screening for primary immunodeficiencies in Sverdlovsk Oblast of Russia. From screening to prophylactic medical checkup","authors":"S. Deryabina, I. Tuzankina, D. A. Cheremokhin, V. Shershnev, D. Kudlay, M. Litvinova, E. Vlasova","doi":"10.24110/0031-403x-2023-102-3-95-106","DOIUrl":"https://doi.org/10.24110/0031-403x-2023-102-3-95-106","url":null,"abstract":"Sverdlovsk Oblast has become one of the first Russia regions to participate in a pilot project of neonatal screening for the detection of severe forms of primary immunodeficiency (PID) in newborns yet back in 2021, prior to the nationwide rollout of the Expanded Screening Program. The purpose of this research was to evaluate preliminary the effectiveness of identifying children with severe combined immunodeficiencies as well as other life-threatening forms of lymphopenia at the stage of possible impact on the course and outcome of the disease. Materials and methods used: quantitative determination of TREC/KREC in dry blood spots was carried out in 5044 children born in the region in July-Sept. 2021. The testing was performed using the domestic “IMMUNO-BIT” kit by “ABV-TEST,” LTD (Skolkovo Innovation Center, Mozhaysky District, Moscow, Russia). Cutoffs suggested in the instructions were used: 500 copies per 105 leukocytes for TREC and 300 copies per 105 leukocytes for KREC. Results: the risk group included 157 newborns (68 girls and 89 boys) with low TREC and/or KREC values (3.1%), no children were found with a complete absence of markers. After testing the re-requested samples, 104 of them were determined to be false positive, and 53 families were invited for an immunologist’s appointment. 28 children received in-depth examination with the advisory. At the time of appointment, the condition of most of them was assessed as clinically stable, however, according to the results of laboratory and instrumental diagnostic examinations, the presence of immunological abnormalities and developmental features was demonstrated: changes in the thymus (50%), neutropenia (32%), lymphopenia and thrombocytopenia (28%), hypogammaglobulinemia (84%), congenital malformations (30%). Conclusion: for the correct understanding of the phenomenon of reduced TREC/KREC values (but not zero values) in some newborns and predicting outcomes, it is necessary to monitor the health status of these patients, which can be carried out through the formation of a dynamic observation group, which is impossible without active identification of such children through wide screening program.","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":"76371274","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-2023-102-3-115-123
M.M. Dunaykin, E. D. Kireeva, Kailash Chaurasiya, Y. Likar
Prescriptions for the positron emission tomography-computed tomography (PET-CT) in adult cancer patients are based on the results of multicenter studies, systematic reviews, and meta-analyses. Thus, published international guidelines help nuclear medicine specialists, oncologists and radiologists in determining the most appropriate diagnostic algorithms for each patient with the purpose of selecting the most appropriate treatment as well as allowing the introduction of the new prognostic factors. Compared with adult patients, the use of PET-CT in pediatric oncology is somewhat limited due to the lack of systematic, large multicenter studies and, therefore, precise and clear guidelines. The reasons for the lack of the large studies may be the relatively low incidence of malignant neoplasms in pediatric practice and the fear for the delayed effects of exposure of ionizing radiation on the growing child's body. The purpose of this bibliographical review was to provide a summary of the use and the role of PET-CT in children with the most frequent malignancies.
{"title":"Positron emission tomography-computed tomography in pediatric oncology","authors":"M.M. Dunaykin, E. D. Kireeva, Kailash Chaurasiya, Y. Likar","doi":"10.24110/0031-403x-2023-102-3-115-123","DOIUrl":"https://doi.org/10.24110/0031-403x-2023-102-3-115-123","url":null,"abstract":"Prescriptions for the positron emission tomography-computed tomography (PET-CT) in adult cancer patients are based on the results of multicenter studies, systematic reviews, and meta-analyses. Thus, published international guidelines help nuclear medicine specialists, oncologists and radiologists in determining the most appropriate diagnostic algorithms for each patient with the purpose of selecting the most appropriate treatment as well as allowing the introduction of the new prognostic factors. Compared with adult patients, the use of PET-CT in pediatric oncology is somewhat limited due to the lack of systematic, large multicenter studies and, therefore, precise and clear guidelines. The reasons for the lack of the large studies may be the relatively low incidence of malignant neoplasms in pediatric practice and the fear for the delayed effects of exposure of ionizing radiation on the growing child's body. The purpose of this bibliographical review was to provide a summary of the use and the role of PET-CT in children with the most frequent malignancies.","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":"82392328","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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