• 文章类型: Journal Article
    背景:对于早期非小细胞肺癌(NSCLC)患者,肺段切除术和肺叶切除术是否具有相似的生存结果的问题仍存在争议。
    方法:纳入队列研究和随机对照试验,比较肺段切除术和肺叶切除术,通过利用计算机访问Pubmed,WebofScience,和Cochrane图书馆数据库,直到2022年7月。Cochrane协作工具用于评估随机对照试验,而纽卡斯尔-渥太华量表(NOS)用于评估队列研究。还进行了敏感性分析。
    结果:分析纳入了17篇文献研究,包括一项随机对照试验和16项队列研究,根据患者接受的手术类型,分为节段切除术组(n=2081)和肺叶切除术组(n=2395)。每个研究都在手术后27个月至130.8个月进行随访。过度生存(OS):HR=1.14,95CI(0.97,1.32),P=0.10;无病生存率(DFS):HR=1.13,95CI(0.91,1.41),P=0.27;无复发生存率(RFS):HR=0.95,95CI(0.81,1.12),P=0.54。
    结论:研究结果表明,肺段切除术组的生存结果并不劣于肺叶切除术组。因此,节段切除术应被视为早期NSCLC的治疗选择。
    BACKGROUND: The question of whether segmentectomy and lobectomy have similar survival outcomes for patients with early-stage non-small cell lung cancer (NSCLC) is a matter of debate.
    METHODS: A cohort study and randomized controlled trial were included, comparing segmentectomy and lobectomy, by utilizing computerized access to the Pubmed, Web of Science, and Cochrane Library databases up until July 2022. The Cochrane Collaboration tool was used to evaluate the randomized controlled trials, while the Newcastle-Ottawa Scale (NOS) was used to evaluate the cohort studies. Sensitivity analyses were also carried out.
    RESULTS: The analysis incorporated 17 literature studies, including one randomized controlled trial and 16 cohort studies, and was divided into a segmentectomy group (n = 2081) and a lobectomy group (n = 2395) based on the type of surgery the patient underwent. Each study was followed up from 27 months to 130.8 months after surgery. Over survival (OS): HR = 1.14, 95%CI(0.97,1.32), P = 0.10; disease-free survival (DFS): HR = 1.13, 95%CI(0.91,1.41), P = 0.27; recurrence-free survival (RFS): HR = 0.95, 95%CI(0.81,1.12), P = 0.54.
    CONCLUSIONS: The results of the study suggest that the survival outcomes of the segmentectomy group were not inferior to that of the lobectomy group. Segmentectomy should therefore be considered as a treatment option for early stage NSCLC.
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  • 文章类型: Journal Article
    背景:本研究旨在评估在根治性子宫切除术前接受新辅助化疗(NACT)的IB2/IIA2期宫颈鳞状细胞癌患者与术前未接受NACT的患者的预后。
    方法:这是一项多中心研究,包括6个妇科肿瘤科的数据。该研究得到了该机构当地伦理委员会之一的批准。根据手术干预前接受NACT的情况,将患者分为两组。分析临床病理因素和无进展生存期。
    结果:共纳入87例患者。在接受NACT的组中观察到淋巴管间隙侵犯(LVSI)为40%,而未接受NACT治疗组的比例为66.1%(p=0.036)。接受NACT的组中深部基质浸润(>50%)为56%,未接受NACT的组中为84.8%(p=0.001)。在单变量分析中,在与无病生存相关的因素中,NACT的应用具有统计学意义.因此,对无进展生存期进行了多变量分析,结合基质侵入深度等因素,LVSI的存在,以及NACT的管理。其中,只有NACT作为与无进展生存期降低相关的独立预测因子.(RR:5.88;95%CI:1.63-21.25;p=0.07)。
    结论:NACT不应在IB2/IIA2期宫颈癌根治术前常规使用。在全国妇科肿瘤大会和全国宫颈病理学和阴道镜大会(2022/TURKEY)上作为口头报告。
    BACKGROUND: This study aimed to evaluate the outcomes of patients diagnosed with stage IB2/IIA2 cervical squamous cell carcinoma who underwent neoadjuvant chemotherapy (NACT) prior to radical hysterectomy compared to those who did not receive NACT before surgery.
    METHODS: This is a multicenter study including data of 6 gynecological oncology departments. The study is approved from one of the institution\'s local ethics committee. Patients were stratified into two cohorts based on the receipt of NACT preceding their surgical intervention. Clinico-pathological factors and progression-free survival were analyzed.
    RESULTS: Totally 87 patients were included. Lymphovascular space invasion (LVSI) was observed as 40% in the group receiving NACT, while it was 66.1% in the group not receiving NACT (p = 0.036). Deep stromal invasion (> 50%) was 56% in the group receiving NACT and 84.8% in the group not receiving NACT (p = 0.001). In the univariate analysis, application of NACT is statistically significant among the factors that would be associated with disease-free survival. Consequently, a multivariate analysis was conducted for progression-free survival, incorporating factors such as the depth of stromal invasion, the presence of LVSI, and the administration of NACT. Of these, only the administration of NACT emerged as an independent predictor associated with decreased progression-free survival. (RR:5.88; 95% CI: 1.63-21.25; p = 0.07).
    CONCLUSIONS: NACT shouldn\'t be used routinely in patients with stage IB2/IIA2 cervical cancer before radical surgery. Presented as oral presentation at National Congress of Gynaecological Oncology & National Congress of Cervical Pathologies and Colposcopy (2022/ TURKEY).
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  • 文章类型: Journal Article
    COVID-19大流行深刻地影响了我们生活的各个方面。通过实时监测和快速实施疫苗,我们成功地抑制了疾病的传播并减轻了其后果。最后,结论可以总结和得出。这里,我们以波兰为例,受到大流行的严重影响。与其他国家相比,波兰在检测或疫苗接种方面都没有取得令人印象深刻的成果,这可以解释其高死亡率(病死率,CFR1.94%)。通过对COVID-19数据门户波兰收集的数据进行回顾性分析,我们发现执行的测试数量存在显著的区域差异,发现的病例数,与COVID-19相关的死亡人数,和疫苗接种率。Masovian,大波兰,和波美拉尼亚省,该国的领导人在疫苗接种,报告病例数量高,但死亡率低。相比之下,波兰东部和南部的省(Subcarpathian,Podlaskie,卢布林,Opole),记录了低疫苗接种水平和低病例数,COVID-19相关死亡率较高。CFR与波兰接种疫苗的人口比例之间的强烈负相关支持疫苗接种的有效性。为了深入了解病毒的进化,我们对500多个基因组进行了测序,并分析了波兰诊断中心保存在GISAID的近8万份SARS-CoV-2基因组序列.我们表明,随着时间的推移,SARS-CoV-2变体在波兰的分布反映了欧洲的分布。单倍型网络分析使我们能够跟踪病毒传播途径,并在每次大流行浪潮中识别潜在的超级传播者。
    The COVID-19 pandemic has profoundly affected all aspects of our lives. Through real-time monitoring and rapid vaccine implementation, we succeeded in suppressing the spread of the disease and mitigating its consequences. Finally, conclusions can be summarized and drawn. Here, we use the example of Poland, which was seriously affected by the pandemic. Compared to other countries, Poland has not achieved impressive results in either testing or vaccination, which may explain its high mortality (case fatality rate, CFR 1.94%). Through retrospective analysis of data collected by the COVID-19 Data Portal Poland, we found significant regional differences in the number of tests performed, number of cases detected, number of COVID-19-related deaths, and vaccination rates. The Masovian, Greater Poland, and Pomeranian voivodeships, the country\'s leaders in vaccination, reported high case numbers but low death rates. In contrast, the voivodeships in the eastern and southern parts of Poland (Subcarpathian, Podlaskie, Lublin, Opole), which documented low vaccination levels and low case numbers, had higher COVID-19-related mortality rates. The strong negative correlation between the CFR and the percentage of the population that was vaccinated in Poland supports the validity of vaccination. To gain insight into virus evolution, we sequenced more than 500 genomes and analyzed nearly 80 thousand SARS-CoV-2 genome sequences deposited in GISAID by Polish diagnostic centers. We showed that the SARS-CoV-2 variant distribution over time in Poland reflected that in Europe. Haplotype network analysis allowed us to follow the virus transmission routes and identify potential superspreaders in each pandemic wave.
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  • 文章类型: Journal Article
    黑色素瘤是最常见的致命类型的皮肤癌,在美国和世界范围内,这是一个重要且日益严重的公共卫生问题。幸运的是,年轻人的发病率正在下降,稳定中年人,并且在老年人中增加。在这里,作者进一步描述了黑色素瘤发病率和死亡率的趋势,回顾有关风险因素的文献,并提供全人群筛查和黑色素瘤筛查中使用的新技术的最新评估。
    Melanoma is the most commonly fatal type of skin cancer, and it is an important and growing public health problem in the United States and worldwide. Fortunately, incidence rates are decreasing in young people, stabilizing in middle-aged people, and increasing in older individuals. Herein, the authors further describe trends in melanoma incidence and mortality, review the literature on risk factors, and provide an up-to-date assessment of population-wide screening and new technology being utilized in melanoma screening.
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  • 文章类型: Journal Article
    背景:在试验中,亚组分析用于检查治疗效果是否因患者的重要特征而不同.然而,哪些亚组最常报告尚未得到全面描述。
    方法:使用美国临床试验注册(ClinicalTrials.gov)中确定的一组试验,我们描述了一系列疾病和药物类别的每个报告亚组.
    方法:我们通过ClinicalTrials.gov数据库的综合分析从ClinicalTrials.gov获得了试验特征。随后,我们获得了所有相应的PubMed索引论文,并筛选了这些论文的亚组报告。使用医学主题标题和WHO解剖学治疗化学代码提取并标准化报告的亚组的表格和文本。通过逻辑和泊松回归模型,我们确定了结果报告(任何vs无)和亚组报告(任何vs无和计数)的独立预测因子。然后,我们按索引条件总结了分组报告,并通过基于网络的交互式热图(https://ihwph-hehta)呈现了所有试验的所有分组。shinyapps.io/subgroup_reporting_app/)。
    结果:在2235项符合条件的试验中,23%(524项试验)报告亚组。随访时间(OR,95CI:1.13,1.04-1.24),入学率(每10倍增加,3.48,2.25-5.47),试验开始年(1.07、1.03-1.11)和具体指标条件(例如,高胆固醇血症,高血压,以哮喘为参考,OR范围从0.15到10.44),预测报告,赞助来源和武器数量没有。在对任何结果报告进行建模时,结果相似(除了武器数量,1.42、1.15-1.74)和子组总数。年龄(51%)性别(45%),种族组(28%)是最常见的亚组.经常报告与指标状况相关的特征(严重程度/持续时间/类型等)(例如,69%的心肌梗死试验报告了其严重程度/持续时间/类型)。然而,关于共病/虚弱(5项试验)和心理健康(4项试验)的报告很少.
    结论:除了年龄,性别,种族、民族或地理位置以及与指标状况相关的特征,关于治疗效果变化的信息很少。
    CRD42018048202。
    BACKGROUND: In trials, subgroup analyses are used to examine whether treatment effects differ by important patient characteristics. However, which subgroups are most commonly reported has not been comprehensively described.
    METHODS: Using a set of trials identified from the US clinical trials register (ClinicalTrials.gov), we describe every reported subgroup for a range of conditions and drug classes.
    METHODS: We obtained trial characteristics from ClinicalTrials.gov via the Aggregate Analysis of ClinicalTrials.gov database. We subsequently obtained all corresponding PubMed-indexed papers and screened these for subgroup reporting. Tables and text for reported subgroups were extracted and standardised using Medical Subject Headings and WHO Anatomical Therapeutic Chemical codes. Via logistic and Poisson regression models we identified independent predictors of result reporting (any vs none) and subgroup reporting (any vs none and counts). We then summarised subgroup reporting by index condition and presented all subgroups for all trials via a web-based interactive heatmap (https://ihwph-hehta.shinyapps.io/subgroup_reporting_app/).
    RESULTS: Among 2235 eligible trials, 23% (524 trials) reported subgroups. Follow-up time (OR, 95%CI: 1.13, 1.04-1.24), enrolment (per 10-fold increment, 3.48, 2.25-5.47), trial starting year (1.07, 1.03-1.11) and specific index conditions (eg, hypercholesterolaemia, hypertension, taking asthma as the reference, OR ranged from 0.15 to 10.44), predicted reporting, sponsoring source and number of arms did not. Results were similar on modelling any result reporting (except number of arms, 1.42, 1.15-1.74) and the total number of subgroups. Age (51%), gender (45%), racial group (28%) were the most frequently reported subgroups. Characteristics related to the index condition (severity/duration/types etc) were frequently reported (eg, 69% of myocardial infarction trials reported on its severity/duration/types). However, reporting on comorbidity/frailty (five trials) and mental health (four trials) was rare.
    CONCLUSIONS: Other than age, sex, race ethnicity or geographic location and characteristics related to the index condition, information on variation in treatment effects is sparse.
    UNASSIGNED: CRD42018048202.
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  • 文章类型: Journal Article
    背景:糖尿病足溃疡是一种主要的医学疾病,社会,和经济问题,也是导致住院的主要原因,发病率增加,和死亡率。尽管事件有所增加,关于发病率及其预测因素的数据缺乏。
    目的:在埃塞俄比亚中部的糖尿病随访诊所中评估糖尿病患者糖尿病足溃疡的发生率和预测因素。
    方法:回顾性随访研究设计。
    方法:从2012年1月1日至2022年12月31日,共418例新诊断的糖尿病患者。使用计算机生成的简单随机抽样方法来选择研究参与者。使用结构化数据提取检查表收集数据。将收集的数据输入EpiInfoV.7.2,并输出到STATAV.14进行分析。为了估计生存时间,采用Kaplan-Meier法,生存差异用对数秩检验检验。
    方法:拟合Cox比例风险模型以确定糖尿病足溃疡发展的预测因子。使用具有95%置信区间(CI)的调整后的风险比(AHR)来估计关联的强度,在p<0.05时宣布有统计学意义。
    结果:糖尿病足溃疡的总发生率为每100人年观察1.51例(95%CI1.03至2.22)。10年内累积发生率为6.2%(95%CI4.1%至8.6%)。中位随访时间为45个月(IQR21~73)。舒张压为90mmHg或以上(AHR2.91,95%CI1.25至6.77),服用联合药物(AHR3.24,95%CI1.14~9.19)和患有外周动脉疾病(AHR5.26,95%CI1.61~17.18)是糖尿病足溃疡发展的统计学显著预测因子.
    结论:糖尿病足溃疡的发生风险相对较高。舒张压水平,联合用药和外周动脉疾病是糖尿病足溃疡发展的独立预测因素.因此,密切监测和适当干预至关重要。
    BACKGROUND: Diabetic foot ulcer is a major medical, social, and economic problem, and a leading cause of hospitalisations, increased morbidity, and mortality. Despite a rising occurrence, there is a dearth of data on the incidence and its predictors.
    OBJECTIVE: To assess the incidence and predictors of diabetic foot ulcers among patients with diabetes mellitus in a diabetic follow-up clinic in Central Ethiopia.
    METHODS: Retrospective follow-up study design.
    METHODS: A total of 418 newly diagnosed diabetes mellitus patients from 1 January 2012 to 31 December 2022. A computer-generated simple random sampling method was used to select the study participants. Data were collected using a structured data extraction checklist. The collected data were entered into Epi Info V.7.2 and exported to STATA V.14 for analysis. To estimate survival time, the Kaplan-Meier method was used, and the survival difference was tested using a log-rank test.
    METHODS: The Cox proportional hazard model was fitted to identify the predictors of diabetic foot ulcer development. The strength of the association was estimated using an adjusted hazard ratio (AHR) with a 95% confidence interval (CI), and statistical significance was proclaimed at a p<0.05.
    RESULTS: The overall incidence of diabetic foot ulcer was 1.51 cases (95% CI 1.03 to 2.22) per 100 person-years of observation. The cumulative incidence was 6.2% (95% CI 4.1% to 8.6%) over 10 years. The median time of follow-up was 45 months (IQR 21-73). Diastolic blood pressure of 90 mm Hg or above (AHR 2.91, 95% CI 1.25 to 6.77), taking combined medication (AHR 3.24, 95% CI 1.14 to 9.19) and having a peripheral arterial disease (AHR 5.26, 95% CI 1.61 to 17.18) were statistically significant predictors of diabetic foot ulcer development.
    CONCLUSIONS: The risk of occurrence of diabetic foot ulcer was relatively high. Diastolic blood pressure level, combined medication and peripheral arterial disease were independent predictors of diabetic foot ulcer development. Hence, close monitoring and proper interventions are essential.
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  • 文章类型: Journal Article
    目的:苏格兰世代(GS)是一项基于大型家庭的队列研究,是遗传研究的纵向资源,生活方式和身心健康的环境决定因素。它包括广泛的遗传,苏格兰志愿者的社会人口统计学和临床数据。
    方法:共有24084名成年参与者,包括5501个家庭,在2006年至2011年期间招募。在队列中,59%(约14209)是女性,招募平均年龄为49岁。参与者填写了一份健康问卷,并参加了面对面的诊所访问,收集了关于生活方式信息的详细基线数据,认知功能,人格特质和身心健康。基因型阵列数据可用于2026名(83%)参与者,和18869名(78%)参与者的基于血液的DNA甲基化(DNAm)数据。与常规国家卫生服务数据集的联系已成为可能的93%(n=22402)的队列,创建包括初级保健在内的纵向资源,医院就诊,处方和死亡率记录。多峰脑成像在1069个人中可用。
    结果:GS已被世界各地的研究人员广泛用于研究常见复杂疾病的遗传和环境基础。使用GS数据发表了350多篇同行评审论文,有助于研究领域,如老龄化,癌症,心血管疾病和心理健康。Recontact研究建立在GS队列上,以收集更多的前瞻性数据来研究慢性疼痛,抑郁症和COVID-19。
    要创建一个更大的,更富有,纵向资源,“下一代苏格兰”于2022年5月启动,以扩大现有队列,目标是增加2万名志愿者,现在包括任何12岁以上的人。新参与者完成在线同意书和问卷调查,并提供邮政唾液样本,基因型和唾液DNAm阵列数据将从中产生。最新的队列信息以及如何访问数据可以在GS网站上找到(www。generationscotland.org)。
    OBJECTIVE: Generation Scotland (GS) is a large family-based cohort study established as a longitudinal resource for research into the genetic, lifestyle and environmental determinants of physical and mental health. It comprises extensive genetic, sociodemographic and clinical data from volunteers in Scotland.
    METHODS: A total of 24 084 adult participants, including 5501 families, were recruited between 2006 and 2011. Within the cohort, 59% (approximately 14 209) are women, with an average age at recruitment of 49 years. Participants completed a health questionnaire and attended an in-person clinic visit, where detailed baseline data were collected on lifestyle information, cognitive function, personality traits and mental and physical health. Genotype array data are available for 20 026 (83%) participants, and blood-based DNA methylation (DNAm) data for 18 869 (78%) participants. Linkage to routine National Health Service datasets has been possible for 93% (n=22 402) of the cohort, creating a longitudinal resource that includes primary care, hospital attendance, prescription and mortality records. Multimodal brain imaging is available in 1069 individuals.
    RESULTS: GS has been widely used by researchers across the world to study the genetic and environmental basis of common complex diseases. Over 350 peer-reviewed papers have been published using GS data, contributing to research areas such as ageing, cancer, cardiovascular disease and mental health. Recontact studies have built on the GS cohort to collect additional prospective data to study chronic pain, major depressive disorder and COVID-19.
    UNASSIGNED: To create a larger, richer, longitudinal resource, \'Next Generation Scotland\' launched in May 2022 to expand the existing cohort by a target of 20 000 additional volunteers, now including anyone aged 12+ years. New participants complete online consent and questionnaires and provide postal saliva samples, from which genotype and salivary DNAm array data will be generated. The latest cohort information and how to access data can be found on the GS website (www.generationscotland.org).
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  • 文章类型: Journal Article
    放射性慢性疼痛的流行病学和预后,尤其是慢性神经性疼痛(CNP),是头颈癌(HNC)幸存者放疗(RT)后的研究不足的领域。这项研究旨在估计这种慢性疼痛的患病率,探索他们与心理健康的关系,睡眠障碍,认知功能,以及这些患者的生活质量(QOL)。这项研究包括RT后的HNC幸存者。辐射引起的慢性疼痛和CNP的测定符合国际疼痛研究协会(IASP)概述的指南。采用多元回归分析探讨疼痛与焦虑之间的关系,抑郁症,睡眠障碍,认知功能,和QOL。共有1071名RT后HNC幸存者被纳入本研究。放射性慢性疼痛的患病率为67.1%,与RT相关的CNP的患病率为38.3%,.与那些报告没有疼痛的人相比,放射性慢性疼痛患者焦虑风险显著增加,抑郁症和睡眠障碍(所有p<0.001)。慢性疼痛和生活质量之间存在显著的负相关(p<0.001),心理(p<0.001),社会关系(p=0.001)和环境(p=0.009)领域。与非CNP相比,RT相关CNP患者出现焦虑(p=0.027)和睡眠障碍(p=0.013)的风险较高.在CNP和生理之间发现了显著的负相关(p=0.001),WHOQOL-BREF的心理评分(p=0.012)和社会评分(p=0.035)。这项研究强调了慢性疼痛的大量流行,特别是CNP,以及它们对心理健康的潜在影响,睡眠,RT后HNC幸存者的生活质量。观点:这项研究强调了辐射引起的慢性疼痛和CNP的高患病率,以及它们对焦虑的潜在影响,抑郁症,HNC幸存者的睡眠和生活质量。临床上,这些发现对于改善HNC幸存者的护理和结局具有重要意义.
    The epidemiology and prognosis of radiation-induced chronic pain, especially chronic neuropathic pain (CNP), are the understudied domain among head and neck cancer (HNC) survivors after radiotherapy (RT). This study aimed to estimate the prevalence of such chronic pain, and explore their correlations with mental health, sleep disorders, cognitive function, and quality of life (QOL) within these patients. This research encompassed HNC survivors post-RT. The determination of radiation-induced chronic pain and CNP adhered to the guidelines outlined by the International Association for the Study of Pain (IASP). Multivariable regression analyses were employed to explore the relationship between pain and anxiety, depression, sleep disturbances, cognitive function, and QOL. A total of 1071 HNC survivors post-RT were included in this study. The prevalence of radiation-induced chronic pain was 67.1%, and the prevalence of RT-associated CNP was 38.3%,. Compared with those reporting no pain, patients with radiation-induced chronic pain had a significantly increased risk of anxiety, depression and sleep disorders (all p < 0.001). And there was a significantly negative association between chronic pain and QOL across physiological (p < 0.001), psychological (p < 0.001), social relationships (p = 0.001) and environmental (p = 0.009) domains. Compared with non-CNP, patients with RT-related CNP had a higher risk of anxiety (p= 0.027) and sleep disorders (p= 0.013). The significantly negative associations were found between CNP and the physiological (p = 0.001), psychological (p = 0.012) and social score (p = 0.035) in WHOQOL-BREF. This study underscores the substantial prevalence of chronic pain, particularly CNP, and their potential impact on the mental health, sleep, and QOL among HNC survivors post-RT. PERSPECTIVE: This study highlights the high prevalence of radiation-induced chronic pain and CNP, and their potential impacts on anxiety, depression, sleep and QOL among the HNC survivors. Clinically, these findings have important implications for improving the care and outcomes of HNC survivors.
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  • 文章类型: Journal Article
    目的:结晶球蛋白血症是一种罕见的以单克隆免疫球蛋白(Migs)血管内结晶为特征的综合征。有关肾脏受累的数据仅限于病例报告。该系列描述了晶体球蛋白诱导的肾病(CIN)的临床病理特征。
    方法:案例系列。
    方法:从梅奥诊所和哥伦比亚大学的肾脏病理学档案中发现了19例N患者。CIN由光学(LM)和电子显微镜(EM)可见的血管内(细胞外)MIg晶体定义。
    结果:在病例中,68%为男性,65%为高加索人(中位年龄56岁)。大多数患者出现严重AKI(中位数肌酐3.5mg/dL),血尿,和轻度蛋白尿(中位数1.1g)。常见的肾外表现是宪法(67%),皮肤(56%),和风湿病(50%)。50%的病例患有低补体血症。血液系统疾病为肾意义的单克隆丙种球蛋白病(MGRS)(72%),淋巴瘤(17%),或骨髓瘤(11%),这些疾病中有65%与CIN同时发现。所有患者在SPEP/SIF上都有MIg(IgGκ占65%)。sFLC比率在40%的肾脏范围之外,骨髓活检检测到67%的相关克隆。在LM上,晶体涉及肾小球(100%)和血管(47%),常伴有炎症反应(89%)和纤维蛋白(58%)。通过EM,所有病例均表现出晶体亚结构(主要是次晶)。石蜡包埋组织上的免疫荧光(IF)比冷冻组织更敏感(92%对47%),以证明晶体组成(IgGκ为63%)。16例患者获得了随访(中位数为20个月)。百分之八十一接受了类固醇治疗,44%血浆置换,38%血液透析,69%的化疗。接受克隆指导治疗的患者中,有90%的患者实现了肾脏恢复。20%的人没有(p=0.017)。
    结论:回顾性设计,小样本量。
    结论:CIN是与淋巴浆细胞病(主要是MGRS)相关的肾病的罕见原因,通常表现为严重的AKI和肾外表现。诊断通常需要在石蜡包埋的肾组织上进行IF。迅速开始克隆导向治疗,再加上皮质类固醇和血浆置换,可能导致肾功能的恢复。
    OBJECTIVE: Crystalglobulinemia is a rare syndrome characterized by intravascular crystallization of monoclonal immunoglobulins (MIgs). Data on kidney involvement are limited to case reports. This series characterizes the clinicopathologic spectrum of crystalglobulin-induced nephropathy (CIN).
    METHODS: Case series.
    METHODS: Nineteen CIN cases were identified from the nephropathology archives of Mayo Clinic and Columbia University. CIN was defined by intravascular (extracellular) MIg crystals visible by light (LM) and electron microscopy (EM).
    RESULTS: Among the cases, 68% were male and 65% were Caucasian (median age 56 years). Most patients presented with severe AKI (median creatinine 3.5 mg/dL), hematuria, and mild proteinuria (median 1.1 g). Common extrarenal manifestations were constitutional (67%), cutaneous (56%), and rheumatologic (50%). Fifty percent of cases had hypocomplementemia. The hematologic disorders were monoclonal gammopathy of renal significance (MGRS) (72%), lymphoma (17%), or myeloma (11%), with 65% of these disorders discovered concomitantly with CIN. All patients had MIg identified on SPEP/SIF (IgGκ in 65%). The sFLC ratio was outside the renal range in 40%, and bone marrow biopsy detected the responsible clone in 67%. On LM, crystals involved glomeruli (100%) and vessels (47%), often with an inflammatory reaction (89%) and fibrin (58%). All cases exhibited crystal substructures (mostly paracrystalline) by EM. Immunofluorescence (IF) on paraffin embedded tissue was more sensitive than frozen tissue (92% versus 47%) for demonstrating the crystal composition (IgGκ in 63%). Follow up (median 20 months) was available in 16 patients. Eighty-one percent received steroids, 44% plasmapheresis, 38% hemodialysis, and 69% chemotherapy. Ninety-percent of patients who received clone-directed therapy achieved kidney recovery vs. 20% of those who did not (p=0.017).
    CONCLUSIONS: Retrospective design, small sample size.
    CONCLUSIONS: CIN is a rare cause of nephropathy associated with lymphoplasmacytic disorders (mostly MGRS) and typically presents with severe AKI and extrarenal manifestations. Diagnosis often requires IF performed on paraffin embedded kidney tissue. Prompt initiation of clone-directed therapy, coupled with corticosteroids and plasmapheresis, may lead to recovery of kidney function.
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  • 文章类型: Journal Article
    苏拉威西冠状猕猴(Macacanigra)(SCMs)极度濒危,并且经常在囚禁中患有慢性肠道疾病。通常,尽管常规诊断调查和肠道炎症的确认,无法确定病因,导致非特异性分类为慢性小肠结肠炎,而不是病因诊断。这项研究评估了23个SCMs的胃肠道组织的组织学特征,将具有提示慢性小肠结肠炎的临床病史的动物(n=14)与没有胃肠道临床体征的动物(n=9)进行比较。根据南希指数(NI)对组织进行分级,用于人类医学评估溃疡性结肠炎疾病活动的评分系统,人类炎症性肠病(IBD)的一种常见形式。此外,结肠固有层中的炎症细胞通过类型进行视觉识别,计数并随后在患病动物和对照动物之间进行比较。中度至重度淋巴浆细胞性炎症和结构变化在受影响的SCMs的结肠中最常见,而所有检查的小肠(n=17)和胃(n=11)组织均无组织病理学改变或轻度改变。结肠NI与临床疾病严重程度呈显著正相关,57%(n=8)有临床体征的动物NI等级≥2,与中度至重度一致。活跃的IBD。复发性直肠脱垂的SCM中有一半(n=6)的NI等级为0,这表明肠道炎症并不总是这种情况的发病机理的一部分。结肠淋巴细胞的数量,浆细胞,中性粒细胞,患病动物的巨噬细胞和白细胞总数明显较高。这项研究验证了NI在SCM中的使用,能够对该物种的结肠进行更标准化的组织病理学评估。
    Sulawesi crested macaques (Macaca nigra) (SCMs) are critically endangered and frequently suffer from chronic intestinal disease in captivity. Often, despite routine diagnostic investigations and confirmation of intestinal inflammation, an aetiology cannot be identified, leading to a non-specific categorization as chronic enterocolitis rather than an aetiological diagnosis. This study evaluates the histological features of gastrointestinal tissues from 23 SCMs, comparing animals with a clinical history suggestive of chronic enterocolitis (n = 14) with those without gastrointestinal clinical signs (n = 9). Tissues were graded according to the Nancy index (NI), a scoring system used in human medicine to evaluate disease activity in ulcerative colitis, a common form of human inflammatory bowel disease (IBD). Additionally, inflammatory cells in the colonic lamina propria were visually identified by type, counted and subsequently compared between diseased and control animals. Moderate to severe lymphoplasmacytic inflammation and structural changes were most common in the colons of affected SCMs, whereas histopathological changes were absent or mild in all examined small intestine (n = 17) and stomach (n = 11) tissues. The colonic NI had a significant positive correlation with clinical disease severity and 57% (n = 8) of animals with clinical signs had a NI grade of ≥2, consistent with moderate to severe, active IBD. Half of SCMs with recurrent rectal prolapse (n = 6) had a NI grade of 0, suggesting that intestinal inflammation is not always part of this condition\'s pathogenesis. The numbers of colonic lymphocytes, plasma cells, neutrophils, macrophages and total leucocytes were significantly higher in diseased animals. This study validated the use of the NI in SCMs, enabling a more standardized histopathological evaluation of the colon in this species.
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