malignancies

恶性肿瘤
  • 文章类型: Journal Article
    由于诊断延迟等因素,癌症被广泛认为是全球死亡率的主要原因。预后不良,复发的可能性很高。FGD5转录因子G反义RNA1(FGD5-AS1),一种新发现的长链非编码RNA,已经成为一种有希望的预后生物标志物,恶性肿瘤预后。这项荟萃分析旨在评估FGD5-AS1在各种癌症中的预后意义。
    通过五个电子数据库进行了系统搜索,以确定研究FGD5-AS1表达作为癌症预后因素的作用的研究。FGD5-AS1在恶性肿瘤中的价值通过比值比(ORs)和风险比(HRs)以及相应的95%置信区间(CIs)来估计。此外,GEPIA数据库用于进一步补充我们的结果.
    这项分析包括12项研究,涵盖了8种癌症类型的642例病例。高FGD5-AS1表达与不良总生存期(OS)显着相关(HR=2.04,95CI[1.72,2.42],P<0.00001),晚期肿瘤分期(OR=3.47,95CI[2.34,5.14],P<0.00001),淋巴结转移(LNM)(OR=1.79,95%CI[1.20,2.67],P=0.004),和较大的肿瘤大小(OR=5.25,95CI[2.68,10.30],P<0.00001)。此外,如使用GEPIA在线基因分析工具所证实的,FGD5-AS1表达在6种恶性肿瘤中显著上调.
    这项荟萃分析的结果表明,FGD5-AS1高表达与不同癌症类型的不良预后显着相关,提示FGD5-AS1可能是预测癌症预后的一个有前景的生物标志物。
    https://www.约克。AC.uk/inst/crd,标识符CRD42024552582。
    UNASSIGNED: Cancer is widely recognized as a prominent contributor to global mortality due to factors such as delayed diagnosis, unfavorable prognosis, and high likelihood of recurrence. FGD5 transcription factor G antisense RNA 1(FGD5-AS1), a newly identified long non-coding RNA, has emerged as a promising prognostic biomarker, for malignancy prognosis. This meta-analysis aimed to assess the prognostic significance of FGD5-AS1 in various carcinomas.
    UNASSIGNED: A systematic search was performed through five electronic databases to identify studies that investigating the role of FGD5-AS1 expression as a prognostic factor in carcinomas. The value of FGD5-AS1 in malignancies was estimated by odds ratios (ORs) and hazard ratios (HRs) with a corresponding 95% confidence intervals (CIs). Furthermore, the GEPIA database was used to further supplement our results.
    UNASSIGNED: This analysis included 12 studies with 642 cases covering eight cancer types. High FGD5-AS1 expression exhibited a significant correlation with poor overall survival(OS) (HR = 2.04, 95%CI [1.72, 2.42], P < 0.00001), advanced tumor stage (OR = 3.47, 95%CI [2.34, 5.14], P < 0.00001), lymph node metastasis(LNM) (OR = 1.79, 95% CI [1.20,2.67], P = 0.004), and larger tumor size (OR= 5.25, 95%CI [2.68, 10.30], P < 0.00001). Furthermore, the FGD5-AS1 expression was notably upregulated in six types of malignancies as verified using the GEPIA online gene analysis tool.
    UNASSIGNED: The findings of this meta-analysis indicated that high FGD5-AS1 expression was significantly associated with poor prognosis in diverse cancer types, suggesting that FGD5-AS1 may be a promising biomarker for predicting cancer prognosis.
    UNASSIGNED: https://www.york.ac.uk/inst/crd, identifier CRD42024552582.
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  • 文章类型: Journal Article
    ShaukatKhanum纪念信托基金已经开始运作,自1990年2月。第一个ShaukatKhanum纪念癌症医院和研究中心(SKMCH&RC)于1994年12月29日在拉合尔开始运作。SKMCH&RC,白沙瓦,2015年12月开始运营。该研究旨在概述在SKMCH&RC注册的癌症病例超过28年。
    本研究包括在信托附属中心登记后输入医院信息系统的患者数据。根据性别和年龄类别(儿童[≤18岁]和成人[>18岁])对恶性肿瘤进行分层。
    乳腺肿瘤,下胃肠道(GI),嘴唇和口腔在所有年龄段和两性中都很普遍;在成年女性中,乳房肿瘤,卵巢和子宫附件,嘴唇和口腔;在成年男性中,低GI系统,前列腺,嘴唇和口腔;在儿童中,霍奇金淋巴瘤,急性淋巴细胞白血病,以非霍奇金淋巴瘤为主。
    在医院登记处登记的病例很重要。当与其他设施的信息结合时,他们可以估计人口水平的统计数据。这可以改善该国的癌症监测,以有效预防疾病,control,和管理。
    UNASSIGNED: The Shaukat Khanum Memorial Trust has been operational, since February 1990. The first Shaukat Khanum Memorial Cancer Hospital and Research Center (SKMCH&RC) started functioning in Lahore on December 29, 1994. SKMCH&RC, Peshawar, started its operation in December 2015. The study aimed to give an overview of the cancer cases registered at SKMCH&RC over 28 years.
    UNASSIGNED: This study comprised patient data entered into the hospital information system after registration at the centers affiliated with the trust. The malignancies were stratified according to sex and age category (children [≤18 years] and adults [>18 years]).
    UNASSIGNED: Neoplasms of the breast, lower gastrointestinal (GI) tract, and lip and oral cavity were prevalent in all ages and both sexes combined; in adult females, neoplasms of the breast, ovary and uterine adnexa, and lip and oral cavity; in adult males, lower GI system, prostate, and lip and oral cavity; and in children, Hodgkin lymphoma, acute lymphoblastic leukemia, and non-Hodgkin lymphoma were predominant.
    UNASSIGNED: Cases registered in a hospital-based registry are important. When combined with information from other facilities, they can estimate population-level statistics. This can improve cancer surveillance in the country for effective disease prevention, control, and management.
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  • 文章类型: Journal Article
    目的:特定类型的恶性肿瘤与随后的痴呆风险之间的关系仍然未知。
    方法:一项基于台湾国民健康保险研究数据库数据的基于人群的回顾性队列研究。
    方法:我们在1998年至2011年间招募了32,250名恶性肿瘤患者和322,500名对照患者,并随访至2013年底。
    方法:痴呆(包括阿尔茨海默病(AD),血管性痴呆(VaD),和未指明的痴呆症)在随访期间进行。校正潜在混杂因素后进行Cox回归分析。进行敏感性分析以排除前驱痴呆患者。
    结果:癌症幸存者更容易患上AD(风险比[HR]:1.68,95%置信区间[CI]:1.38-2.06),未指明的痴呆(HR:1.19,95%CI:1.07-1.32),和任何痴呆(HR:1.26,95%CI:1.16-1.37)与对照组相比,校正了潜在的混杂因素。重要的是,消化和泌尿生殖器官的癌症似乎与AD有关,未指明的痴呆症,和任何痴呆症,而只有大脑的恶性肿瘤更有可能发展为VaD。排除前3年或5年观察后以及排除2009年或2007年之前的病例登记后的敏感性分析显示了一致的结果。
    结论:癌症幸存者患后续痴呆的风险更高。不同类型的癌症幸存者可能导致特定痴呆的风险可变。需要进一步的研究来研究癌症幸存者和痴呆症患者的潜在机制。
    OBJECTIVE: The association between specific types of malignancies and the subsequent risk of dementia remains unknown.
    METHODS: A retrospective population-based cohort study based on data from Taiwan National Health Insurance Research Database.
    METHODS: We recruited 32,250 patients who survived malignancies and 322,500 controls between 1998 and 2011 and followed them up until the end of 2013.
    METHODS: Diagnoses of dementia (including Alzheimer\'s disease (AD), vascular dementia (VaD), and unspecified dementia) was made during the follow-up period. Cox regression analyses were performed after adjusting for potential confounders. A sensitivity analysis was conducted to exclude patients with prodromal dementia.
    RESULTS: Cancer survivors were more likely to develop AD (hazard ratio [HR]: 1.68, 95% confidence interval [CI]: 1.38-2.06), unspecified dementia (HR: 1.19, 95% CI: 1.07-1.32), and any dementia (HR: 1.26, 95% CI: 1.16-1.37) compared with controls after adjusting for potential confounders. Importantly, cancers of the digestive and genitourinary organs seem to be associated with AD, unspecified dementia, and any dementia, whereas only malignant neoplasms of the brain are more likely to develop into VaD. Sensitivity analyses after exclusion of the first three or five years of observation and after exclusion of case enrollment before 2009 or 2007 showed consistent findings.
    CONCLUSIONS: Cancer survivors are at higher risk of subsequent dementia. Different types of cancer survivors may contribute to variable risks of specific dementias. Further studies are necessary to investigate the underlying mechanisms in cancer survivors and patients with dementia.
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  • 文章类型: Journal Article
    继发性噬血细胞性淋巴组织细胞增生症(sHLH)历来被定义为细胞因子风暴综合征(CSS),发生在导致强烈和失调的免疫激活的触发条件下,没有已知的遗传偏好。然而,最近的研究表明,现有的潜在遗传因素可能与特定的疾病和/或环境触发因素(包括感染,自身免疫性/自身炎症性疾病,某些生物疗法,或恶性转化),导致SHLH。随着基因检测技术的进步,更多的患者检查原发性HLH(pHLH)相关基因的遗传变异,包括全外显子组和全基因组测序。这种不断扩大的遗传和基因组证据表明,HLH是一种更复杂的现象,由于具有易感遗传背景的患者的特定免疫挑战。而不是一个简单的,pHLH和sHLH的二进制定义,HLH代表一系列疾病,从常见感染的严重并发症(EBV,流感)只能通过移植治愈的早发性家族性疾病。
    Secondary hemophagocytic lymphohistiocytosis (sHLH) has historically been defined as a cytokine storm syndrome (CSS) occurring in the setting of triggers leading to strong and dysregulated immunological activation, without known genetic predilection. However, recent studies have suggested that existing underlying genetic factors may synergize with particular diseases and/or environmental triggers (including infection, autoimmune/autoinflammatory disorder, certain biologic therapies, or malignant transformation), leading to sHLH. With the recent advances in genetic testing technology, more patients are examined for genetic variations in primary HLH (pHLH)-associated genes, including through whole exome and whole genome sequencing. This expanding genetic and genomic evidence has revealed HLH as a more complex phenomenon, resulting from specific immune challenges in patients with a susceptible genetic background. Rather than a simple, binary definition of pHLH and sHLH, HLH represents a spectrum of diseases, from a severe complication of common infections (EBV, influenza) to early onset familial diseases that can only be cured by transplantation.
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  • 文章类型: Journal Article
    软组织肉瘤(STSs)是一种罕见且多样化的癌症,由80多种不同的种类组成,每个都表现出独特的间充质分化,如世界卫生组织(WHO)所述。诊断时的预后因素主要取决于大小,深度,和淋巴受累的组织学分级。改善的预后指标对于确定可能从辅助治疗中受益的高风险患者和可能避免治疗相关副作用的低风险患者是必要的。在五年的时间里,相当多的患者经历了肿瘤在同一区域的复发或疾病转移到身体的其他部位,即使通过手术完全切除局部肿瘤。为了进一步个性化和集中药物,提高预测准确性和发现新的治疗靶点至关重要.考虑到与转移性STS相关的高死亡率和发病率,这一点尤为重要。STS的显着多样性给理解其病理生物学和将生物学进展转化为治疗应用带来了困难。这项前瞻性队列研究是在一家主要的大学医院进行的,目的是确定在2018年至2023年期间被诊断为四肢STS的成年患者。纳入标准为18岁或以上的STS组织学诊断的个体。排除标准包括STS以外的恶性肿瘤患者和基本因素数据不足的患者。进行了彻底的评估以分析患者的人口统计学,肿瘤特征(包括部位,尺寸,深度,神经血管或骨侵入),和组织学类型和分级(根据法国癌症中心联合会(FNCLCC)分级系统)。目的是寻找预测标志物并评估结果。该结果与先前的研究一致,并增强了我们对STS预后的了解。转移和死亡风险的关键预后标志物包括肿瘤大小大于5厘米,组织学分级,和肉瘤亚型.根治性外科手术,如截肢或脱节,没有表现出任何生存优势,可能是因为它们用于疾病已经在局部发展并且在血管中具有显著受累的情况。组织学分级已被确定为预测成人STS转移可能性的最重要因素。研究发现,大多数肿瘤是高级别肿瘤,肿瘤分级之间有统计学上显著的关联,Ki67级别,和总体生存率。一小部分患者的寿命超过五年,强调早期检测之间的联系,严重程度较低的肿瘤,和增强的结果。这些观察结果强调了准确的预后评估和定制的治疗方法在STS治疗中的重要性。
    Soft-tissue sarcomas (STSs) are an uncommon and diverse group of cancers, consisting of more than 80 different kinds, each showing unique mesenchymal differentiation as described by the World Health Organization (WHO). The prognostic factors at the time of diagnosis mostly depend on the size, depth, and histological grade of the lymphatic involvement. Improved prognostic indicators are necessary to identify patients at high risk who may derive advantages from adjuvant therapy and those at low risk who might avoid treatment-related side effects. Over a period of five years, a considerable number of patients experience the recurrence of the tumor in the same area or the metastasis of the disease to other parts of the body, even after the complete removal of the localized tumor through surgery. To further personalize and focus medicines, it is critical to enhance prediction accuracy and uncover new therapy targets. This is particularly important considering the high mortality and morbidity rate associated with metastatic STS. The significant diversity of STS poses difficulty in comprehending its pathobiology and in converting biological progress into therapeutic application. This prospective cohort study was carried out at a major university hospital to ascertain adult patients who were diagnosed with STS of the extremities between the period from 2018 to 2023. The inclusion criteria were individuals who were 18 years of age or older with a histological diagnosis of STS. The exclusion criteria encompassed individuals with malignancies other than STS and those with inadequate data on essential factors. Thorough assessments were conducted to analyze patient demographics, tumor features (including site, size, depth, neurovascular or bone invasion), and histologic type and grade (according to the French Federation of Cancer Centers (FNCLCC) grading system). The purpose was to find predictive markers and evaluate results. The results are consistent with previous research and enhance our current knowledge of STS prognosis. Key prognostic markers for metastasis and mortality risk include tumor size larger than 5 cm, histologic grade, and sarcoma subtype. Radical surgical procedures, such as amputation or disarticulation, did not show any survival advantage, probably because they were used in situations where the disease had already progressed locally and had significant involvement in the blood vessels. Histologic grading has been identified as the most important factor in predicting the likelihood of metastasis in adult STSs. The study found that most tumors were of high grade, and there was a statistically significant association between tumor grade, Ki67 levels, and overall survival. A small proportion of patients experienced prolonged longevity beyond five years, emphasizing the connection between early detection, tumors of lesser severity, and enhanced results. These observations emphasize the significance of accurate prognostic assessments and customized therapeutic approaches in the treatment of STS.
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  • 文章类型: Journal Article
    近年来,特异性生物标志物在实体器官恶性肿瘤诊断和预后中的作用日益突出。使用预测性生物标志物的相关性,预测癌症对特定治疗形式的反应,比以往任何时候都扮演了更重要的角色,因为它会影响诊断和治疗的开始,监测治疗的疗效和副作用,并调整长期治疗方案。在当前的审查中,我们探索了预测生物标志物在实体器官恶性肿瘤治疗中的应用,包括结直肠癌等常见癌症,乳腺癌,肺癌,前列腺癌,与高死亡率相关的癌症,比如胰腺癌,肝癌,肾癌和中枢神经系统癌症。我们还使用预测性生物标志物分析了个性化治疗的目标和类型,以及使用预测性生物标志物对各种类型的实体器官恶性肿瘤的管理及其迄今为止在临床环境中的相对效力。
    In recent years, the influence of specific biomarkers in the diagnosis and prognosis of solid organ malignancies has been increasingly prominent. The relevance of the use of predictive biomarkers, which predict cancer response to specific forms of treatment provided, is playing a more significant role than ever before, as it affects diagnosis and initiation of treatment, monitoring for efficacy and side effects of treatment, and adjustment in treatment regimen in the long term. In the current review, we explored the use of predictive biomarkers in the treatment of solid organ malignancies, including common cancers such as colorectal cancer, breast cancer, lung cancer, prostate cancer, and cancers associated with high mortalities, such as pancreatic cancer, liver cancer, kidney cancer and cancers of the central nervous system. We additionally analyzed the goals and types of personalized treatment using predictive biomarkers, and the management of various types of solid organ malignancies using predictive biomarkers and their relative efficacies so far in the clinical settings.
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  • 文章类型: Journal Article
    结直肠癌(CRC)是一个重大的全球健康问题,在匈牙利是一个重大的公共卫生挑战。在欧盟,它的发病率和死亡率最高。地中海饮食已被建议减少CRC的发病率。但需要来自不同研究设计的综合证据来证实这种效应.在PubMed进行了系统的文献检索,ClinicalTrials.gov,中部,和WebofScience确定2008年至2024年的随机对照试验和人体临床试验,以确定相关研究。使用https://metaanalysisonline.comWeb应用程序使用随机效应模型进行统计分析,以估计合并危险率(HR)。森林地块,漏斗图,和Z分数图用于可视化结果。我们确定了15项临床试验和9项病例对照研究,包括总共2,217,404个科目。汇总分析表明,坚持地中海饮食显着降低了CRC的患病率(HR=0.84,95%CI=0.78-0.91,p<0.01)。这种保护作用在不同性别之间是一致的,男性的HR为0.85(95%CI=0.75-0.97,p=0.01),女性为0.88(95%CI=0.79-0.99,p=0.03)。病例对照研究显示了显著的效果(HR=0.51,95%CI=0.38-0.68,p<0.01)。在研究中观察到显著的异质性,然而,先验信息大小大大低于累积样本量,确保有足够的数据得出可靠的结论。这项荟萃分析的结果加强了地中海饮食对CRC的保护作用。这项荟萃分析的结果将为旨在减轻CRC风险的饮食干预提供信息。这是在Semmelweis研究的框架内进行的,Semmelweis大学正在进行的一项综合队列研究,旨在探索匈牙利不健康衰老的多方面原因。这些干预措施旨在探索地中海饮食模式在减少匈牙利人口中CRC发病率方面的实际应用。
    Colorectal cancer (CRC) is a major global health concern and represents a significant public health challenge in Hungary, where it exhibits some of the highest morbidity and mortality rates in the European Union. The Mediterranean diet has been suggested to reduce the incidence of CRC, but comprehensive evidence from diverse study designs is needed to substantiate this effect. A systematic literature search was conducted in PubMed, ClinicalTrials.gov, CENTRAL, and the Web of Science to identify randomized controlled trials and human clinical trials from 2008 to 2024 to identify relevant studies. Statistical analysis was performed using the https://metaanalysisonline.com web application using a random effects model to estimate the pooled hazard rates (HRs). Forest plots, funnel plots, and Z-score plots were utilized to visualize results. We identified 15 clinical trials and 9 case-control studies, encompassing a total of 2,217,404 subjects. The pooled analysis indicated that adherence to the Mediterranean diet significantly reduced the prevalence of CRC (HR = 0.84, 95% CI = 0.78-0.91, p < 0.01). This protective effect was consistent across sexes, with HRs of 0.85 (95% CI = 0.75-0.97, p = 0.01) for males and 0.88 (95% CI = 0.79-0.99, p = 0.03) for females. Case-control studies specifically showed a substantial effect (HR = 0.51, 95% CI = 0.38-0.68, p < 0.01). Notable heterogeneity was observed across studies, yet the a priori information size was substantially below the cumulative sample size, ensuring sufficient data for reliable conclusions. The findings from this meta-analysis reinforce the protective role of the Mediterranean diet against CRC. The results of this meta-analysis will inform dietary interventions designed to mitigate CRC risk, which are conducted within the framework of the Semmelweis Study, an ongoing comprehensive cohort study at Semmelweis University, designed to explore the multifaceted causes of unhealthy aging in Hungary. These interventions aim to explore the practical application of Mediterranean dietary patterns in reducing CRC incidence among the Hungarian population.
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  • 文章类型: Journal Article
    肿瘤免疫学和肾移植的相互作用预示着医学科学的变革时代。这种融合,虽然有希望,提出了重大挑战。其中最主要的是免疫抑制增强针对恶性肿瘤的免疫力,同时抑制移植物存活的二分法。此外,关于新疗法的临床数据有限,影响反应的遗传变异,经济问题,移植后恶性肿瘤的狭窄治疗窗口需要战略解决。相反,机会比比皆是,包括个性化的免疫监测,靶向治疗,最小化的免疫抑制,改善患者生活质量。强调协作研究和跨学科合作,这些领域的合并提供了提高移植物存活率和降低移植后恶性肿瘤风险的潜力。当我们利用现代技术并促进以患者为中心的护理时,对未来肾移植的愿景越来越有希望,为更个性化和有效的治疗铺平道路。本文旨在阐明平衡免疫抑制以同时对抗恶性肿瘤并确保移植物存活的关键挑战。它解决了新疗法临床数据的匮乏,遗传变异对治疗反应的影响,以及管理移植后恶性肿瘤的经济和治疗问题。此外,它探索了精准医学提供的机会,如个性化的免疫监测,靶向治疗,减少免疫抑制,这可以显著改善患者的预后。强调合作研究和跨学科努力的重要性,本文旨在证明提高移植物存活率和降低移植后恶性肿瘤风险的潜力.通过利用现代技术并优先考虑以患者为中心的护理,它设想了一个肾移植更加个性化和有效的未来,为这一领域的进步提供了希望。
    The interplay of onco-immunology and kidney transplantation heralds a transformative era in medical science. This integration, while promising, presents significant challenges. Chief among these is the dichotomy of immunosuppression-boosting immunity against malignancies while suppressing it for graft survival. Additionally, limited clinical data on novel therapies, genetic variations influencing responses, economic concerns, and the narrow therapeutic window for post-transplant malignancies necessitate strategic addressal. Conversely, opportunities abound, including personalized immune monitoring, targeted therapies, minimized immunosuppression, and improved patient quality of life. Emphasizing collaborative research and interdisciplinary cooperation, the merging of these fields offers the potential for enhanced graft survival and reduced post-transplant malignancy risks. As we harness modern technology and promote patient-centric care, the vision for the future of kidney transplantation becomes increasingly hopeful, paving the way for more personalized and effective treatments. The article aims to elucidate the critical challenge of balancing immunosuppression to simultaneously combat malignancies and ensure graft survival. It addresses the scarcity of clinical data on novel therapies, the impact of genetic variations on treatment responses, and the economic and therapeutic concerns in managing post-transplant malignancies. Furthermore, it explores the opportunities precision medicine offers, such as personalized immune monitoring, targeted therapies, and reduced immunosuppression, which could significantly improve patient outcomes. Highlighting the importance of collaborative research and interdisciplinary efforts, the article seeks to demonstrate the potential for enhanced graft survival and reduced post-transplant malignancy risks. By leveraging modern technology and prioritizing patient-centric care, it envisions a future where kidney transplantation is more personalized and effective, offering hope for advancements in this field.
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  • 文章类型: Journal Article
    目的:这项研究旨在检查印度白血病和其他癌症的影响,并观察随时间的变化。
    方法:详细估计发病率,患病率,死亡率,作为全球疾病负担(GBD)研究的一部分,从1990年到2019年,对印度30种癌症的残疾调整寿命年(DALYs)进行了29年的分析。来自所有可用来源的数据用于收集有关印度疾病总体负担的信息。
    结果:癌症是全球死亡的主要原因,印度的发病率不同,使预防和治疗成为挑战。因为癌症在印度不是可报告的疾病,总体负担估计仍在进行中。这项研究分析了白血病和其他癌症在印度的影响,包括发病率趋势,DALYs,和死亡率与所有癌症和各种恶性肿瘤有关。还探讨了印度白血病的病因。
    结论:该研究发现了占大多数白血病相关和癌症相关DALYs的癌症类型的趋势,死亡,患病率,以及印度的发病率。在四种最常见的恶性肿瘤中,比如白血病,根据年龄有显著差异。在过去的29年里,慢性粒细胞白血病(CML)和急性淋巴细胞白血病(ALL)的死亡率有所下降,而急性髓细胞性白血病(AML)和慢性淋巴细胞性白血病(CLL)的死亡人数稳步增加。
    OBJECTIVE: This study aimed to examine the impact of leukemia and other cancers in India and to observe any changes over time.
    METHODS: Detailed estimates of incidence, prevalence, mortality, and disability-adjusted life years (DALYs) for 30 types of cancers in India were analyzed for 29 years from 1990 to 2019 as part of the Global Burden of Diseases (GBD) study. Data from all available sources were used to gather information on the overall burden of disease in India.
    RESULTS: Cancer is a leading cause of death worldwide, with varying rates of incidence in India, making prevention and treatment a challenge. Because cancer is not a reportable disease in India, the overall burden estimate is still a work in progress. This study analyzed the impact of leukemia and other cancers in India, including trends in incidence, DALYs, and mortality related to all cancers and various malignancies. The causes of leukemia in India were also explored.
    CONCLUSIONS: The study found the trends of cancer types that account for the majority of leukemia-related and cancer-related DALYs, death, prevalence, and incidence in India. Among the four most frequent malignancies, such as leukemia, there was significant variation based on age. Over the last 29 years, mortality from chronic myelogenous leukemia (CML) and acute lymphocytic leukemia (ALL) has decreased, while deaths from acute myelogenous leukemia (AML) and chronic lymphocytic leukemia (CLL) have increased steadily.
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  • 文章类型: Journal Article
    奈梅亨破损综合征(NBS)是一种常染色体隐性遗传疾病,以小头畸形为特征,免疫缺陷,和受损的DNA修复。NBS在斯拉夫人群中最为普遍,包括乌克兰。我们的研究旨在全面评估患病率,诊断,临床资料,免疫学参数,以及乌克兰NBS患者的治疗。
    我们进行了回顾性审查,其中包括1999-2023年诊断的来自乌克兰不同地区的84名NBS患者。来自乌克兰国家统计局登记处的数据和来自治疗医生的信息,使用开发的问卷获得,用于分析。
    在84名NBS患者中,55人(65.5%)活着,25人(29.8%)死亡,4人失去了随访。患者的中位年龄为11岁,从1岁到34岁不等。大多数患者来自乌克兰西部地区(57.8%),虽然近年来,中部和东南部地区的诊断有所增加,扩大我们对NBS患病率的认识。每年诊断的患者人数平均为3.4,近年来从2.7增加到4.8。NBS诊断的中位年龄在1999-2007年为4.0岁(范围0.1-16岁),在过去6年中下降到2.7岁。在大多数10岁以下的儿童中观察到身体发育延迟。所有儿童都经历过感染,其中41.3%有反复感染。12%的死亡原因是严重感染。NBS的第二常见临床表现是恶性肿瘤(37.5%),淋巴瘤的患病率(63.3%)。恶性肿瘤是NBS患者中最常见的死亡原因(72%的病例)。在89.6%中观察到CD4+和CD19+水平降低,其次是CD3+(81.8%)和CD8+(62.5%)的减少。NK细胞的水平升高到62.5%。IgG浓度下降72.9%,IgA占56.3%。58.7%的患者接受了免疫球蛋白替代疗法。常规免疫球蛋白替代疗法有助于降低严重呼吸道感染的频率和严重程度。
    诊断方面的改进,包括产前筛查,新生儿筛查,监测,扩大治疗选择,将为NBS患者带来更好的结果。
    UNASSIGNED: Nijmegen breakage syndrome (NBS) is an autosomal recessive disorder, characterized by microcephaly, immunodeficiency, and impaired DNA repair. NBS is most prevalent among Slavic populations, including Ukraine. Our study aimed to comprehensively assess the prevalence, diagnosis, clinical data, immunological parameters, and treatment of NBS patients in Ukraine.
    UNASSIGNED: We conducted a retrospective review that included 84 NBS patients from different regions of Ukraine who were diagnosed in 1999-2023. Data from the Ukrainian Registry of NBS and information from treating physicians, obtained using a developed questionnaire, were utilized for analysis.
    UNASSIGNED: Among 84 NBS patients, 55 (65.5%) were alive, 25 (29.8%) deceased, and 4 were lost to follow-up. The median age of patients was 11 years, ranging from 1 to 34 years. Most patients originate from western regions of Ukraine (57.8%), although in recent years, there has been an increase in diagnoses from central and southeastern regions, expanding our knowledge of NBS prevalence. The number of diagnosed patients per year averaged 3.4 and increased from 2.7 to 4.8 in recent years. The median age of NBS diagnosis was 4.0 years (range 0.1-16) in 1999-2007 and decreased to 2.7 in the past 6 years. Delayed physical development was observed in the majority of children up to the age of ten years. All children experienced infections, and 41.3% of them had recurrent infections. Severe infections were the cause of death in 12%. The second most common clinical manifestation of NBS was malignancies (37.5%), with the prevalence of lymphomas (63.3%). Malignancies have been the most common cause of death in NBS patients (72% of cases). Decreased levels of CD4+ and CD19+ were observed in 89.6%, followed by a reduction of CD3+ (81.8%) and CD8+ (62.5%). The level of NK cells was elevated at 62.5%. IgG concentration was decreased in 72.9%, and IgA - in 56.3%. Immunoglobulin replacement therapy was administered to 58.7% of patients. Regular immunoglobulin replacement therapy has helped reduce the frequency and severity of severe respiratory tract infections.
    UNASSIGNED: Improvements in diagnosis, including prenatal screening, newborn screening, monitoring, and expanding treatment options, will lead to better outcomes for NBS patients.
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