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Foramen magnum stenosis in patients with mucopolysaccharidoses: diagnosis and surgical treatment. Review of literature 粘多糖病患者的枕骨大孔狭窄:诊断和手术治疗。文献综述
Pub Date : 2024-06-11 DOI: 10.18705/2782-3806-2024-4-2-96-105
A. V. Stepanenko, K. A. Denisova, V. V. Stepanenko, A. S. Shapovalov, K. V. Sysoev, A. V. Kim
Cervico-medullary compression and atlantoaxial dislocation syndromes are the dominant clinical manifestations of mucopolysaccharidoses at the craniovertebral level. The review provides an analysis of international literary sources concerning modern aspects of diagnosis and neurosurgical correction of the foramen magnum stenosis in patients with different types of mucopolysaccharidoses. The existing surgical approaches to determining the indications and choosing the method of surgical treatment and some aspects of the use of enzyme replacement therapy and hematopoietic stem cell transplantation are presented. A variety of options for surgical correction of pathology at the craniovertebral level have been demonstrated in patients with different types of mucopolysaccharidosis, however the described recommendations can be considered from the point of view of traditions of the hospitals or personal experience of surgeons, but not as recognized standards of treatment this pathology. Further accumulation of individual observations or clinical series is required to conduct a comparative analysis of the effectiveness of various approaches on a sufficiently large and homogeneous material to determine standards for the diagnosis and treatment of craniovertebral junction pathology in patients with mucopolysaccharidoses.
颈髓压迫和寰枢脱位综合征是粘多糖病在颅椎水平的主要临床表现。本综述分析了有关不同类型粘多糖病患者枕骨大孔狭窄的现代诊断和神经外科矫正方面的国际文献资料。文章介绍了确定手术治疗适应症和选择手术治疗方法的现有手术方法,以及酶替代疗法和造血干细胞移植的一些应用方面。对不同类型的粘多糖病患者进行颅椎骨水平病理手术矫正的方案多种多样,但所述建议可从医院传统或外科医生个人经验的角度加以考虑,而不能作为治疗这种病理的公认标准。需要进一步积累个人观察结果或临床系列资料,才能对各种方法在足够大的同质材料中的有效性进行比较分析,从而确定诊断和治疗粘多糖病患者颅椎间隙病变的标准。
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引用次数: 0
Special methods of magnetic resonance in diagnosis of multiple sclerosis. 磁共振诊断多发性硬化症的特殊方法。
Pub Date : 2024-03-22 DOI: 10.18705/2782-3806-2024-4-1-12-18
I. A. Turchinskaya, S. R. Sheriev, A. Efimtsev, G. E. Trufanov
The article is devoted to the review of the most promising MRI techniques in the diagnosis of multiple sclerosis as part of personalized medicine. A brief description of the current state of the problem is provided. Examples of promising MR research techniques are considered. The issues of clinical importance and expediency of using these techniques are discussed. The existing approaches in the diagnosis of multiple sclerosis are summarized. In conclusion, the prospects and importance of the considered methods in the structure of healthcare are summarized.
文章主要回顾了在多发性硬化症诊断中最有前途的磁共振成像技术,这些技术是个性化医疗的一部分。文章简要介绍了这一问题的现状。文中列举了一些有前景的磁共振成像研究技术。讨论了使用这些技术的临床重要性和便捷性问题。总结了诊断多发性硬化症的现有方法。最后,总结了所考虑的方法在医疗保健结构中的前景和重要性。
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引用次数: 0
The level of blood neutrophils primary reactive oxygen species production in Graves’ disease patients undergoing conservative therapy with thiamazole. 接受噻马唑保守治疗的巴塞杜氏病患者血液中的中性粒细胞原发性活性氧生成水平。
Pub Date : 2024-03-22 DOI: 10.18705/2782-3806-2024-4-1-58-6
M. Dudina, S. Dogadin, A. A. Savchenko, I. Gvozdev
Background. Conservative therapy with thiamazole in Graves’ disease helps to reduce the level of oxidative stress by restoring euthyroidism and the total reactive antioxidant potential of plasma. However, until now, in patients with Graves’ disease, the radical-producing ability of neutrophils remains unexplored at the initial stage of free radical conversion at different durations of drug-induced euthyroidism, which seems significant in revealing the mechanisms of disease recurrence. The aim is to study the level of production of primary reactive oxygen species in peripheral blood neutrophils depending on the duration of drug — induced euthyroidism in patients with Graves’ disease. Materials and methods. A single-center, prospective, controlled study was conducted with the participation of patients with laboratory-confirmed Graves’ disease. Subgroups of patients with Graves’ disease were formed depending on the duration of achieved drug-induced euthyroidism with continuous conservative therapy with thiamazole. The level of ROS production in peripheral blood neutrophils was determined by chemiluminescence. The assessment of spontaneous and zymosan-induced chemiluminescence was carried out for 90 minutes on a 36-channel chemiluminescence analyzer BLM-3607 (Medbiotech LLC, Krasnoyarsk). Results. The study included 102 Graves’ disease women with an average age of 47.54 ± 15.47 years. Of these, 75 (73.5 %) with relapse and 27 (26.47 %) with newly diagnosed disease. The total synthesis of (S) primary ROS increased statistically significantly relative to the control values in the examined patients with a duration of the euthyroid state of 5–8 and 9–12 months. It was found that in patients with Graves’ disease with stable drug-induced euthyroidism for 5–8 and 9–12 months, the S index of zymosan-induced lucigenin-dependent chemiluminescence of neutrophils increased statistically significantly, respectively, by 10.7 and 7.4 times relative to the control values. Conclusion. The restoration and maintenance of drug-induced euthyroidism for more than 12 months against the background of conservative thiamazole therapy in patients with Graves’ disease reduces the load of systemic oxidative stress on the immune system. However, the high total production of primary ROS in neutrophils of patients with Graves’ disease in a state of drug-induced euthyroidism with conservative thiamazole therapy may play a significant role in disconnecting the functioning of the NADPH oxidase complex and determine the functional capabilities of neutrophils in case of disease recurrence.
背景。使用噻马唑对巴塞杜氏病进行保守治疗有助于通过恢复甲状腺功能和血浆总活性抗氧化潜能来降低氧化应激水平。然而,迄今为止,在巴塞杜氏病患者中,中性粒细胞在药物诱导甲状腺功能亢进不同持续时间的自由基转化初期产生自由基的能力仍未得到研究,而这对于揭示疾病复发的机制似乎意义重大。本文旨在研究巴塞杜氏病患者外周血中性粒细胞中初级活性氧的生成水平取决于药物诱导甲状腺功能亢进的持续时间。材料与方法在实验室确诊的巴塞杜氏病患者中开展了一项单中心、前瞻性、对照研究。根据持续使用噻马唑保守治疗后药物诱导甲状腺功能亢进的持续时间,对巴塞杜氏病患者进行分组。通过化学发光法测定外周血中性粒细胞产生的 ROS 水平。在 36 通道化学发光分析仪 BLM-3607 (Medbiotech LLC,克拉斯诺亚尔斯克)上对自发化学发光和紫霉素诱导化学发光进行了 90 分钟的评估。研究结果研究对象包括 102 名患巴塞杜氏病的女性,平均年龄(47.54 ± 15.47)岁。其中,75人(73.5%)病情复发,27人(26.47%)新近确诊。在甲状腺状态持续时间为 5-8 个月和 9-12 个月的受检患者中,(S) 原发性 ROS 的总合成量与对照值相比有显著的统计学增长。研究发现,在药物诱导甲状腺功能稳定达5-8个月和9-12个月的巴塞杜氏病患者中,紫杉醇诱导的中性粒细胞荧光素依赖性化学发光的S指数在统计学上比对照值分别显著增加了10.7倍和7.4倍。结论在保守治疗噻马唑的背景下,巴塞杜氏病患者恢复并维持药物诱导的甲状腺功能亢进超过12个月,可减少全身氧化应激对免疫系统造成的负荷。然而,巴塞杜氏病患者的中性粒细胞在噻马唑保守疗法的药物诱导甲状腺功能亢进状态下产生的原发性ROS总量较高,这可能会对NADPH氧化酶复合物的功能发挥重要作用,并在疾病复发时决定中性粒细胞的功能能力。
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引用次数: 0
Progression-free survival after first recurrence in patients with glioblastoma. 胶质母细胞瘤患者首次复发后的无进展生存期。
Pub Date : 2024-03-22 DOI: 10.18705/2782-3806-2024-4-1-29-43
S. M. Sklyar, N. E. Voinov, A. Ulitin, M. Matsko
Relevance. Despite all the treatment glioblastoma recurs as an aggressive and therapy-resistant tumor, and patients quickly die from these neoplasms. The study of glioblastoma recurrence processes and search for prognostic factors of the disease should lead to the improvement of survival rates of patients with this pathology. Purpose of the study. To study the influence of clinical and molecular-genetic factors on the median second recurrence-free period. Materials and methods. Progression-free survival after first recurrence in 34 patients aged 28 to 81 years with recurrent glioblastoma was analyzed. The diagnosis was established according to the WHO 2021 classification of CNS tumors. In each observation we studied such clinical parameters as patient’s age, functional status according to the Karnovsky scale pre- and postoperatively, peculiarities of neuroimaging picture (prevalence of tumor process, localization of recurrence, tumor volume), conducted treatment and molecular-genetic characteristics of the tumor (determination of mRNA expression level of genes: MGMT, VEGF, PDGFRA, β-tubulin III, ERCC-1, TOP2A). Results. Among the clinical and demographic characteristics, the median of the survival was influenced by the patients’ age and functional status after surgery. The median of the survival was more than 2 times higher in the group of patients under 50 years old, compared to patients over 50 years old (18.5 vs 8 weeks). The dependence of the median of the survival on the post- operative functional status (according to the Karnovsky scale) was determined (p = 0.001). The median of the survival in case of a single brain lobe lesion was more than 5 times higher than in case of widespread tumor process, though without statistical reliability (p = 0.09, 21.5 vs 4 weeks). Survival rates were higher when recurrence was localized within 2 cm of the area of removal of the primary neoplasm. After disease progression, the MGMT gene lost its predictive value. Patients with low expression of the TOR2A gene had a higher survival rate than those with medium and high expression (47.5 vs 3 weeks, p = 0.001; 47.5 vs 22.5 weeks, p = 0.06). The median of survival was higher than at high levels at low and medium PDGFRA gene expression levels (29 vs 0 weeks, p = 0.04; 21 vs 0 weeks; p = 0.05, respectively). Maximum survival rates were recorded in the group of patients after total and subtotal removal of tumor recurrence (22 and 18.5 weeks, p = 0.05). Administration of second-line chemotherapy with temozolomide statistically significantly increased the median of the second BRS (p = 0.01). Conclusion. Recurrent glioblastomas are characterized by an extremely aggressive course. Therefore, such prognostic factors as patient age, degree of tumor resection, tumor process prevalence, degree of tumor resection and 2nd line chemotherapy come to the forefront. It should be noted that the MGMT gene loses its predictive value during disease progression, while the TOR2A gene
相关性。尽管接受了各种治疗,胶质母细胞瘤仍会复发,这是一种具有侵袭性和耐药性的肿瘤,患者很快就会死于这种肿瘤。对胶质母细胞瘤复发过程的研究以及对该疾病预后因素的探索,将有助于提高该病症患者的生存率。研究目的研究临床和分子遗传因素对第二次无复发期中位数的影响。材料和方法。分析了 34 名年龄在 28 至 81 岁之间的复发性胶质母细胞瘤患者首次复发后的无进展生存期。诊断是根据世界卫生组织 2021 年中枢神经系统肿瘤分类确定的。在每次观察中,我们都研究了患者的年龄、术前和术后的功能状态(根据卡诺夫斯基量表)、神经影像的特殊性(肿瘤过程的发生率、复发的位置、肿瘤体积)、进行的治疗和肿瘤的分子遗传学特征(基因 mRNA 表达水平的测定)等临床参数:MGMT、VEGF、PDGFRA、β-tubulin III、ERCC-1、TOP2A)。结果在临床和人口统计学特征中,生存期中位数受患者年龄和术后功能状态的影响。与 50 岁以上的患者相比,50 岁以下患者组的生存期中位数高出 2 倍多(18.5 周对 8 周)。存活期中位数与术后功能状态(根据卡诺夫斯基量表)的关系已被确定(p = 0.001)。单个脑叶病变患者的生存期中位数是广泛肿瘤病变患者的5倍多,但没有统计学可靠性(P = 0.09,21.5周对4周)。当复发部位位于原发肿瘤切除区域的 2 厘米范围内时,生存率更高。疾病进展后,MGMT 基因失去了预测价值。TOR2A基因低表达患者的生存率高于中、高表达患者(47.5 vs 3周,p = 0.001;47.5 vs 22.5周,p = 0.06)。PDGFRA基因低表达水平和中表达水平组的生存中位数高于高表达水平组(分别为29周 vs 0周,p = 0.04;21周 vs 0周,p = 0.05)。肿瘤复发全切除和次全切除组患者的生存率最高(分别为 22 周和 18.5 周,p = 0.05)。使用替莫唑胺进行二线化疗可显著提高第二次 BRS 的中位数(p = 0.01)。结论复发性胶质母细胞瘤的特点是病程极具侵袭性。因此,患者年龄、肿瘤切除程度、肿瘤过程发生率、肿瘤切除程度和二线化疗等预后因素显得尤为重要。值得注意的是,MGMT 基因在疾病进展过程中失去了预测价值,而 TOR2A 基因和 PDGFRA 基因则成为预后标志。
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引用次数: 0
Radiology diagnostics of neonatal pneumonia. 新生儿肺炎的放射诊断。
Pub Date : 2024-03-22 DOI: 10.18705/2782-3806-2024-4-1-6-11
I. A. Turchinskaya, L. G. Konstantinova, G. E. Trufanov
The article is dedicated to reviewing the role of radiological diagnosis in “Identifying neonatal pneumonia”. It includes a brief historical and terminological overview, as well as a description of the current state of the issue. The article discusses the clinical importance and feasibility of using radiological diagnostic methods, summarizes existing approaches in the diagnosis and treatment of the most significant nosological forms, and concludes with an overview of the prospects and significance of these methods in the healthcare system.
本文旨在回顾放射诊断在 "识别新生儿肺炎 "中的作用。文章包括简短的历史和术语概述,以及对该问题现状的描述。文章讨论了使用放射诊断方法的临床重要性和可行性,总结了诊断和治疗最重要的病理形式的现有方法,最后概述了这些方法在医疗系统中的前景和意义。
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引用次数: 0
4D FLOW in assessing blood flow in aorta. 评估主动脉血流的 4D FLOW。
Pub Date : 2024-03-22 DOI: 10.18705/2782-3806-2024-4-1-19-24
S. R. Sheriev, I. A. Turchinskaya, V. A. Fokin, A. V. Ryzhkov
The article is dedicated to reviewing the role of the 4D FLOW technique in assessing blood flow in the aorta. A brief historical and terminological overview is provided, along with a description of the current state of the issue. The clinical relevance and appropriateness of using this radiological diagnostic method are discussed. The scanning and image post-processing process is summarized. Finally, the prospects and significance of the reviewed technique in healthcare are outlined.
本文专门回顾了 4D FLOW 技术在评估主动脉血流中的作用。文章提供了一个简短的历史和术语概述,并描述了该问题的现状。讨论了使用这种放射诊断方法的临床意义和适宜性。总结了扫描和图像后处理过程。最后,概述了该技术在医疗保健领域的前景和意义。
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引用次数: 0
Identifying Immunological Markers for Bowel Cancer. 确定肠癌的免疫标记物
Pub Date : 2024-03-22 DOI: 10.18705/2782-3806-2024-4-1-25-28
N. S. Bayramova
The role of immunological processes in the pathophysiology of colorectal cancer has received a lot of attention lately and has been extensively researched. Immune processes are significantly regulated by cytokines and antimicrobial peptides (AMP). Numerous studies have demonstrated the significance of cytokines in the prognosis and surveillance of malignant oncological illnesses. In group II, the blood serum concentration of Calprotectin was found to be greater in 13 patients (92.0 %) and lower in 1 patient (8.0 %) compared to control limits (χ = 24.27, p < 0.001). The obtained results show that, in comparison to the control group, the concentration of Calprotectin rises statistically consistently by 3.5 times, with a coefficient of integrity of p < 0.001. According to the data, there is a statistically significant rise in Calprotectin concentration (3.5 times higher than in the control group) with a p-value of less than 0.001. The average mathematical density of this indicator is 3.42 ± 0.48 pg/ml, the minimum density is 0.7 pg/ml, and the maximum thickness is 6.1 pg/ml.
免疫过程在结直肠癌病理生理学中的作用近来受到广泛关注和研究。免疫过程受到细胞因子和抗菌肽(AMP)的重要调控。大量研究表明,细胞因子对恶性肿瘤疾病的预后和监测具有重要意义。与对照组相比(χ = 24.27,P < 0.001),第二组 13 名患者(92.0%)和 1 名患者(8.0%)的血清中钙蛋白浓度分别升高和降低。结果显示,与对照组相比,钙黏蛋白的浓度在统计学上持续上升了 3.5 倍,完整性系数 p < 0.001。数据显示,钙黏蛋白浓度在统计学上有显著的上升(比对照组高 3.5 倍),P 值小于 0.001。该指标的平均数学密度为 3.42 ± 0.48 pg/ml,最小密度为 0.7 pg/ml,最大厚度为 6.1 pg/ml。
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引用次数: 0
Tumors of the third ventricle in children (literature review). 儿童第三脑室肿瘤(文献综述)。
Pub Date : 2024-03-22 DOI: 10.18705/2782-3806-2024-4-1-68-72
M. S. Nikolaenko, N. Samochernykh
Tumors of the third ventricle are rare and account for 0.6–0.9 % of all pediatric brain tumors. Tumors of the third ventricle are divided into primary tumors, such as choroid plexus papillomas and ependymomas, and secondary tumors, such as craniopharyngiomas, optic nerve gliomas, pineal tumors, and meningiomas. Choroid papilloma is a common tumor, the treatment of which is mainly based on microsurgical resection. Apart from the many treatment options for craniopharyngiomas, microsurgery is the preferred treatment option. Ependymomas also have several treatment options, with microsurgical removal considered the first line of treatment.
第三脑室肿瘤非常罕见,占所有小儿脑肿瘤的 0.6%-0.9%。第三脑室肿瘤分为原发性肿瘤(如脉络丛乳头状瘤和上皮瘤)和继发性肿瘤(如颅咽管瘤、视神经胶质瘤、松果体瘤和脑膜瘤)。脉络膜乳头状瘤是一种常见肿瘤,其治疗主要以显微外科切除术为主。颅咽管瘤的治疗方法很多,显微外科手术是首选治疗方法。外胚窦瘤也有多种治疗方法,显微手术切除被认为是第一线治疗方法。
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引用次数: 0
Achievements and prospects for further development of photodynamic therapy technology in the treatment of cerebral tumors 光动力疗法技术在治疗脑肿瘤方面的成就和进一步发展前景
Pub Date : 2024-03-22 DOI: 10.18705/2782-3806-2024-4-1-44-57
K. K. Kukanov, A. Nechaeva, A. Ulitin, S. M. Sklyar, A. E. Vershinin, M. V. Dikonenko, A. O. Politova, G. V. Papayan, V. Olyushin
Literature review reflects the current status and development status of intraoperative photodynamic therapy in neurooncology and discusses the results of the most important studies on photodynamic therapy (PDT). We searched the Pubmed, EMBASE, Cochrane Library and eLibrary databases for publications published between January 2000 and December 2022. Found 204 publications in foreign sources and 59 publications in domestic editions, dealing with the issues of photodynamic therapy in neurooncology. An analysis of the literature has shown that intraoperative PDT in neurooncology is an important tool that contributes to increasing the radicality of the operation and local control. The basic rationale for the effectiveness of PDT lies in the study of the pathways leading to the complete devitalization of a malignant tumor, the study of the mechanisms of the local and systemic immune response. In addition, subcellular targets in PDT are determined by the properties of photosensitizers (PS). Second generation PSs have already been introduced into clinical practice. The effectiveness of PDT using photoditazine, 5-aminolevulinic acid has been demonstrated. The mechanisms of action and targets of these PS have been established. In Russia, a number of studies have repeatedly shown and proved the clinical effectiveness of PDT in groups of neurooncological patients with glial tumors and secondary metastatic tumors, but so far, the method has not been included in the clinical guidelines for the provision of high-tech neurosurgical care. There is certainly a need for further development of PTD techniques in neurooncology, especially in patients at high risk of recurrence and aggressive CNS tumors.
文献综述反映了术中光动力疗法在神经肿瘤学中的现状和发展状况,并讨论了最重要的光动力疗法(PDT)研究结果。我们检索了 Pubmed、EMBASE、Cochrane Library 和 eLibrary 数据库中 2000 年 1 月至 2022 年 12 月间发表的出版物。发现204篇国外文献和59篇国内文献涉及神经肿瘤学中的光动力疗法问题。文献分析表明,术中光动力疗法在神经肿瘤学中是一种重要工具,有助于提高手术的根治性和局部控制。光动力疗法之所以有效,其基本原理在于对导致恶性肿瘤完全消失的途径的研究,以及对局部和全身免疫反应机制的研究。此外,PDT 的亚细胞目标是由光敏剂(PS)的特性决定的。第二代光敏剂已被引入临床实践。使用光二甲嗪、5-氨基乙酰丙酸进行局部光疗的有效性已得到证实。这些 PS 的作用机制和靶点已经确定。在俄罗斯,多项研究反复显示并证明了光动力疗法对神经肿瘤患者中的胶质瘤和继发性转移瘤群体的临床疗效,但迄今为止,该方法尚未被纳入提供高科技神经外科治疗的临床指南中。当然,在神经肿瘤学领域,尤其是在复发风险高和侵袭性中枢神经系统肿瘤患者中,PTD 技术还需要进一步发展。
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引用次数: 0
Case of successful surgical treatment of the aorto-bifemoral re-occlusion with both lower limbs dry gangrene formation 手术治疗双侧主动脉再闭塞伴双下肢干性坏疽的成功案例
Pub Date : 2023-09-22 DOI: 10.18705/2782-3806-2023-3-4-96-105
P. D. Puzdriak, V. V. Shlomin, E. F. Rzaev, K. V. Zhdanovich, M. A. Shatil, M. A. Voskresensky, P. B. Bondarenko
The paper demonstrates a clinical case of open surgical re-interventional on the abdominal aorta 8 years later after primary aortofemoral bypass through the right and left retroperitoneal accesses. Severe ischemic damage to the tissues of both lower extremities required repeated vascular reconstruction. The tactics of repeated retroperitoneal intervention on the aorta was chosen, while the access could be expanded to thoracophrenolumbotomy. Due to the pronounced cicatricial process in the retroperitoneal space and intraoperative damage to the ureter, the branches of the prosthesis were performed in an atypical manner.
本文报道1例经左、右腹膜后通道行原发性股主动脉绕道术后8年腹主动脉再介入手术的临床病例。下肢严重的缺血性损伤需要反复的血管重建。选择反复腹膜后主动脉介入治疗策略,同时可扩大入路至胸脑叶切除术。由于腹膜后间隙明显的瘢痕过程和术中对输尿管的损伤,假体的分支以非典型的方式进行。
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引用次数: 0
期刊
Russian Journal for Personalized Medicine
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