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Russian Journal of Pediatric Hematology and Oncology最新文献

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Hemophilic Infection 嗜血的感染
Q4 Medicine Pub Date : 2023-04-25 DOI: 10.15690/rpj.v4i2.2548
A. Editorial
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引用次数: 0
Chickenpox 水痘
Q4 Medicine Pub Date : 2023-04-25 DOI: 10.15690/rpj.v4i2.2547
Article Editorial
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{"title":"Chickenpox","authors":"Article Editorial","doi":"10.15690/rpj.v4i2.2547","DOIUrl":"https://doi.org/10.15690/rpj.v4i2.2547","url":null,"abstract":"<jats:p>.</jats:p>","PeriodicalId":52396,"journal":{"name":"Russian Journal of Pediatric Hematology and Oncology","volume":"87 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-04-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"73359602","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}
引用次数: 0
Meningococcal Infection 脑膜炎球菌感染
Q4 Medicine Pub Date : 2023-04-25 DOI: 10.15690/rpj.v4i2.2549
A. Editorial
.
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引用次数: 0
Dynamics of indicators characterizing the health of children in the territory of military conflict. The impact of rehabilitation measures: cohort study 军事冲突地区儿童健康指标的动态变化。康复措施的影响:队列研究
Q4 Medicine Pub Date : 2023-04-25 DOI: 10.15690/rpj.v4i2.2535
A. M. Levchin, I. B. Ershova, A. G. Rogovtsova
Background. There are still understudied issues concerning the health indicators of children who are in conditions of conducting warfare in primary school age. Objective. The aim of the study is to evaluate comprehensive rehabilitation measures to optimize health indicators (physical, functional and mental capacity parameters) of primary school children in conditions of military conflict.Methods. An examination of 419 elementary school children was conducted in the period from March 2022 to September 2022 including. The main group: 128 children, who lived in the territory of military operations. Control group: 291 children who lived peaceful territories. The indicators (body mass index, Robinson index, vital index, strength index, V.A. Shapovalova power index and mental capacity of children according to the results of V.Ya. Anfimov correction task) were compared before the start of the developed comprehensive rehabilitation program and 6 months after the start of its implementation.Results. It was found that all elementary school children who lived in locations of military conflict had a decrease in physical and functional parameters of varying degrees of severity, as well as indicators of mental capacity. The comprehensive rehabilitation program promoted to the normalization of the studied indicators in evacuated (57/86, 56%) children in comparison with children who remained in the locations of military operations (43/70, 49%). The studied indicators had the highest recovery rates at the age of 7-8 years. The most vulnerable was a subgroup of 9-10 years old boys who permanently living in conditions of conducting warfare. The development and implementation of the comprehensive integration program using individual rehabilitation routes had an optimizing effect on the studied indicators of physical and functional conditions, as well as cognitive performance in 101 children of the main subgroups, which amounted to 78.9%.Conclusion. Under the influence of the comprehensive integration rehabilitation program, optimization of health indicators was observed in children living in locations of conducting warfare. The most pronounced effect was observed in children who left the territories of shelling as a result of evacuation to Rostov-on-Don.
背景。关于处于作战条件下的小学学龄儿童的健康指标,仍有未充分研究的问题。目标。本研究旨在评价军事冲突条件下小学生综合康复措施对其身体、功能和心理健康指标的优化效果。在2022年3月至2022年9月期间,对419名小学生进行了测试。主要群体是128名儿童,他们生活在军事行动的领土上。对照组:291名生活在和平地区的儿童。根据V.Ya的结果,对儿童的体质指数、Robinson指数、生命指数、力量指数、va . Shapovalova功率指数和心智能力等指标进行分析。比较综合康复方案实施前和实施后6个月的安菲莫夫矫正任务。研究发现,生活在军事冲突地区的所有小学生在不同程度的身体和功能参数以及心理能力指标方面都有所下降。与留在军事行动地点的儿童(43/70,49%)相比,综合康复方案促进了撤离儿童(57/ 86,56%)所研究指标的正常化。各指标在7 ~ 8岁时恢复率最高。最脆弱的是一群9-10岁的男孩,他们长期生活在战争的环境中。综合康复方案的制定和实施对主要亚组101例儿童的身体和功能状况指标及认知表现均有优化效果,达到78.9%。在综合融入康复方案的影响下,观察到生活在战争地区的儿童的健康指标得到优化。在撤离到顿河畔罗斯托夫后离开炮击地区的儿童中观察到的影响最为明显。
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引用次数: 0
Modern methods of therapy in the development of life-threatening complications in a patient after repeated allogeneic hematopoietic stem cell transplantation: review of the literature and clinical case 重复异基因造血干细胞移植后危及生命的并发症的现代治疗方法:文献和临床病例的回顾
Q4 Medicine Pub Date : 2023-04-18 DOI: 10.21682/2311-1267-2023-10-1-57-67
There is no doubt that allogeneic hematopoietic stem cell transplantation (allo-HSCT) is one of the most effective treatments for many serious diseases. However, despite significant progress, allo-HSCT is still associated with a high rate of complications and mortality in the posttransplant period due to the toxicity of conditioning regimens, infectious and immune conditions. Acute complications such as endothelial injury, acute and chronic graft-versus-host disease (GVHD) remain the main causes of mortality after allo-HSCT. In our clinical case, we demonstrated an example of the development of such life-threatening complications as transplant-associated thrombotic microangiopathy and GVHD in a patient after repeated allo-HSCT, as well as the successful relief of these complications by modern therapeutic methods, including the introduction of closely related donor mesenchymal stem cells and the complement blocker eculizumab.
毫无疑问,同种异体造血干细胞移植是治疗许多严重疾病最有效的方法之一。然而,尽管取得了重大进展,由于调节方案、感染和免疫条件的毒性,同种异体造血干细胞移植仍然与移植后时期的高并发症和死亡率相关。急性并发症,如内皮损伤、急性和慢性移植物抗宿主病(GVHD)仍然是同种异体造血干细胞移植后死亡的主要原因。在我们的临床病例中,我们展示了一个患者在重复同种异体造血干细胞移植后发生的危及生命的并发症,如移植相关的血栓性微血管病和GVHD,以及通过现代治疗方法成功缓解这些并发症,包括引入密切相关的供体间充质干细胞和补体阻滞剂eculizumab。
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引用次数: 0
Acne disease in germ cell tumors. A brief review of the literature and a description of our own clinical observation 生殖细胞肿瘤中的痤疮病。简要回顾文献和描述我们自己的临床观察
Q4 Medicine Pub Date : 2023-04-18 DOI: 10.21682/2311-1267-2023-10-1-73-80
D. Martynov, Т. S. Belysheva, Т. T. Valiev, O. A. Kapkova, E. Sharapova, A. Prokofyev, Y. Vishnevskaya, E. Mikhailova, T. R. Panferova, S. Varfolomeeva
malignant neoplasms (MNP). The frequency of PNS in MNP ranges from 1 to 20 % according to different authors. One of the manifestations of PNS is acne, which occurs against the background of malignant neoplasm due to hormonal disorders.The presented article describes a clinical case of a patient with a mixed germ cell tumor and metastases in the retroperitoneal lymph nodes. The germ cell tumor produced human beta-chorionic gonadotropin, which led to the development of acne as a PNS.Antitumor treatment (surgical removal of the tumor and polychemotherapy) normalized the levels of human beta-chorionic gonadotropin and serum testosterone, which in turn led to regression of acne without acne therapy.
恶性肿瘤(MNP)。根据不同的作者,MNP中PNS的频率从1%到20%不等。PNS的表现之一是痤疮,它发生在恶性肿瘤的背景下,由于激素紊乱。本文描述了一个临床病例的病人与混合生殖细胞肿瘤和转移在腹膜后淋巴结。生殖细胞肿瘤产生人-绒毛膜促性腺激素,导致痤疮作为PNS的发展。抗肿瘤治疗(手术切除肿瘤和多种化疗)使人β -绒毛膜促性腺激素和血清睾酮水平正常化,这反过来又导致痤疮在没有痤疮治疗的情况下消退。
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引用次数: 0
Synovial sarcoma of the median nerve of the right shoulder (case report) 右肩正中神经滑膜肉瘤(附1例报告)
Q4 Medicine Pub Date : 2023-04-18 DOI: 10.21682/2311-1267-2023-10-1-68-72
D. Khestanov, M. M. Efimova, O. M. Romantsova, V. Khairullova, A. Malakhova, E. Mikhailova, A. Dzampaev, S. Varfolomeeva
Synovial sarcoma (SS) is a soft tissue tumor that occurs among a group of tumors other than rhabdomyosarcoma (RMS) in childhood and adolescence in 4 % of cases. In terms of incidence among children, SS is second only to RMS and most often occurs in young and mature patients (15–35 years), with a slight predominance in men. The localization of SS in the soft tissues of the extremities reaches 80 %; to a lesser extent, these tumors occur in the head and neck, trunk, retroperitoneal space, and joints (no more than 5 %). Cases of the appearance of SS in the mediastinum, peripheral nerves, skin and visceral organs are also described.This article presents a rare clinical case of the development of SS of the median nerve of the right shoulder in a 15-year-old patient.
滑膜肉瘤(SS)是一种软组织肿瘤,发生在儿童期和青春期的一组肿瘤中,而不是横纹肌肉瘤(RMS),发病率为4%。在儿童发病率方面,SS仅次于RMS,最常见于年轻和成熟患者(15-35岁),男性略占优势。SS在四肢软组织的定位达到80%;在较小程度上,这些肿瘤发生在头颈部、躯干、腹膜后间隙和关节(不超过5%)。在纵隔,周围神经,皮肤和内脏器官出现的病例也被描述。这篇文章提出一个罕见的临床病例发展SS的右肩正中神经在一个15岁的病人。
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引用次数: 0
Tandem high-dose chemotherapy and autologous hematopoietic stem cell transplantation in pediatric patients with high-risk neuroblastoma: single-center experience 串联大剂量化疗和自体造血干细胞移植在高危神经母细胞瘤患儿中的应用:单中心经验
Q4 Medicine Pub Date : 2023-04-17 DOI: 10.21682/2311-1267-2023-10-1-25-32
S. Kulyova, A. A. Abadjeva, E. Mikhailova, M. A. Kulyov, Y. G. Fedukova, R. I. Khabarova
Currently, the use high doses chemotherapy (HDC) supported by autologous peripheral blood stem cells in consolidation is a necessary therapeutic option in patients with high-risk neuroblastoma (NB). Conditioning regimens and schemes of HDC remain the subject of debate. In recent years, the evidence base of the advantages of tandem myeloablative procedures in comparison with single-transplantation has been actively accumulated in clinical practice. This article presents our own experience of tandem-transplantation with stem cell rescue in the N.N. Petrov National Medical Research Centre of Oncology (Saint-Petersburg) in four patients with initially stratified high-risk group NB; two of them were NMYC amplified. Tandem consolidation included TC ([T]hiotepa, [C]yclophosphamide) and CEM ([C]arboplatin, [E]toposide, [M]elphalan) regimens. The acceptable toxicity of the tandem-transplantation is proved.
目前,使用自体外周血干细胞支持的高剂量化疗(HDC)巩固是高风险神经母细胞瘤(NB)患者的必要治疗选择。HDC的调理方案和方案仍然是争论的主题。近年来,与单次骨髓移植相比,串联清髓手术优势的证据基础在临床实践中得到了积极积累。本文介绍了我们在N.N. Petrov国家肿瘤医学研究中心(圣彼得堡)对4例初始分层高危组NB患者进行串联移植与干细胞抢救的经验;其中两个是NMYC扩增的。串联巩固包括TC ([T]hiotepa, [C]环磷酰胺)和CEM ([C]arboplatin, [E]toposide, [M]elphalan)方案。证明了串联移植的可接受毒性。
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引用次数: 0
Approaches to the treatment of a multisystem form of juvenile xanthogranuloma with central nervous system lesion 多系统形式的青少年黄色肉芽肿伴中枢神经系统病变的治疗方法
Q4 Medicine Pub Date : 2023-04-17 DOI: 10.21682/2311-1267-2023-10-1-41-48
M. V. Natrusova, E. A. Burtsev, N. V. Bronina, D. S. Osipova, D. A. Evseev, E. V. Seliverstova, G. O. Bronin, M. A. Maschan, Е. V. Kumirova
Relevance. Juvenile xanthogranuloma (JXG) is the most common form of non-Langerhans cell histiocytic disorder. Cutaneous forms of the disease spontaneously regress within a few years, while systemic forms of JXG require treatment and may pose a threat to the lives of patients. Due to the lack of unified approach to the treatment of multisystem forms of JXG, the question of effective therapy tactics remains unresolved. The most common approach is to use Langerhans cell histiocytosis (LCH) treatment regimens for JXG. With the understanding of the leading role of mutations in the MEK-ERK signaling pathway in the pathogenesis of JXG, targeted therapy, BRAF- and MEK-inhibitors, are increasingly being considered in the treatment of JXG. Clinical cases. We present two cases of multisystem JXG with central nervous system (CNS) lesions. The first patient with CNS and skin lesions was treated with chemotherapy, developed for the treatment of multisystem LCH, which allowed us to obtain an effect “active disease better” (AD better). The second JXG patient with brain, lungs, bones, and adrenal gland lesions, combined targeted therapy with BRAF- and MEKinhibitors, vemurafenib and cobimetinib, resulted in a “non active disease” (NAD) effect. Conclusion. Multisystem form of JXG with CNS involvement is a rare oncological disease, the therapy of which has not been developed. With the introduction of molecular genetic profiling technology, it became possible to obtain NAD effect using targeted therapy.
的相关性。幼年黄色肉芽肿(JXG)是最常见的非朗格汉斯细胞组织细胞疾病。皮肤形式的该病在几年内自行消退,而全身形式的JXG需要治疗,并可能对患者的生命构成威胁。由于对多系统形式的肾虚缺乏统一的治疗方法,有效的治疗策略问题仍然没有解决。最常见的方法是使用朗格汉斯细胞组织细胞增多症(LCH)治疗JXG。随着对MEK-ERK信号通路突变在JXG发病机制中的主导作用的认识,靶向治疗、BRAF-和mek -抑制剂越来越多地被考虑用于JXG的治疗。临床病例。我们报告两例伴有中枢神经系统病变的多系统JXG。第一位伴有中枢神经系统和皮肤病变的患者接受了化疗,这是为治疗多系统LCH而开发的,这使我们获得了“活动性疾病更好”(AD更好)的效果。第二例JXG患者脑、肺、骨和肾上腺病变,联合BRAF-和mekininhibitors、vemurafenib和cobimetinib靶向治疗,导致“非活动性疾病”(NAD)效应。结论。累及中枢神经系统的多系统型肾小球肾小球是一种罕见的肿瘤疾病,治疗方法尚未成熟。随着分子遗传谱技术的引入,利用靶向治疗获得NAD效果成为可能。
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引用次数: 0
Efficacy and safety of direct oral anticoagulants in children in real clinical practice 儿童直接口服抗凝剂在实际临床中的疗效和安全性
Q4 Medicine Pub Date : 2023-04-17 DOI: 10.21682/2311-1267-2023-10-1-33-40
T. Y. Yafoshkina, Y. Shifrin, D. Fedorova, D. B. Florinskiy, D. Evstratov, P. Zharkov
Introduction. As well as standard anticoagulants, direct oral anticoagulants (DOAC) have been approved for treatment of thromboembolism in children, recently. Several clinical trials provide promising data on efficacy and safety of DOAC in children and young adults. But further studies aimed at evaluating the efficacy and safety of these drugs in children and adolescents are still needed.The aim of the study is to evaluate the safety and effectiveness of the use DOACs in children.Materials and methods. We have retrospectively analyzed the medical records of patients (0–17 years) of from 2013 to 2022 at our tertiary care Centre and selected patients, who were treated with apixaban, rivaroxaban or dabigatran for more than 14 days. Patients with arterial thrombosis, children, who were treated with combined anticoagulant therapy and those who were unable for follow-up were excluded. We assessed the rates of bleeding and recurrent venous thromboembolism (VTE).Results. There were 67 patients, who were treated with DOAC in our center. Patients were divided into 2 groups: those, who received prophylactic anticoagulation (group 1 – 6 patients), and patients, who received DOAC therapy after venous thromboembolism (group 2 – 61 patients). The median follow-up time was 46 (25–365) days in the group 1, and 4 months (2 weeks – 36 month) in the group 2. There were no VTE episodes and 1 minor bleeding in group 1, while 1 (2 %) major, 4 (6 %) minor bleeding episodes in group 2. There were 4 (6 %) patients with recurrent VTE in group 2.Conclusion. The majority of patients in this study received rivaroxaban (79 %). We found a moderately increased recurrence rate of VTE, which could be the result of a long follow-up period, and a comparable rate of bleeding, all of which were associated with rivaroxaban therapy. In the future, of particular interest, in our opinion, are prospective studies of DOACs as primary antithrombotic prophylaxis, as well as secondary prophylaxis in the highest-risk groups, and the results of an analysis of their use in real clinical practice.
介绍。与标准抗凝剂一样,直接口服抗凝剂(DOAC)最近已被批准用于治疗儿童血栓栓塞。几项临床试验提供了关于DOAC在儿童和年轻人中的有效性和安全性的有希望的数据。但是,还需要进一步的研究来评估这些药物在儿童和青少年中的有效性和安全性。本研究的目的是评估儿童使用doac的安全性和有效性。材料和方法。我们回顾性分析了2013年至2022年在我们三级保健中心和选定的接受阿哌沙班、利伐沙班或达比加群治疗超过14天的患者(0-17岁)的医疗记录。排除动脉血栓形成患者、儿童、联合抗凝治疗及无法随访者。我们评估了出血和静脉血栓栓塞(VTE)复发率。67例患者在我中心接受了DOAC治疗。患者分为预防性抗凝治疗组(1 ~ 6例)和静脉血栓栓塞后DOAC治疗组(2 ~ 61例)。组1的中位随访时间为46(25-365)天,组2的中位随访时间为4个月(2周- 36个月)。1组无静脉血栓栓塞(VTE)发作,1例轻度出血;2组大出血1例(2%),轻度出血4例(6%)。2组静脉血栓栓塞复发4例(6%)。在这项研究中,大多数患者接受了利伐沙班治疗(79%)。我们发现静脉血栓栓塞的复发率适度增加,这可能是长时间随访的结果,出血率也相当,所有这些都与利伐沙班治疗有关。在我们看来,在未来,特别感兴趣的是DOACs作为初级抗血栓预防的前瞻性研究,以及在最高风险人群中的二级预防,以及它们在实际临床实践中的应用分析结果。
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引用次数: 0
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Russian Journal of Pediatric Hematology and Oncology
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