rapid progression

快速进展
  • 文章类型: Journal Article
    血管球瘤是一种罕见的肿瘤,通常在四肢下发现,并充当专门的神经血管器官。血管球瘤极为罕见的部位是乳腺,只有少数病例报告。乳腺血管球瘤有三种典型的临床症状:隐痛,病灶压痛,冷敏感性。所有血管球瘤中不到10%是恶性的。我们在此介绍一例起源于乳腺的恶性血管球瘤。远处转移被排除,肿瘤完全切除了.然而,患者意外出现快速全身转移,肿瘤切除后5周检测。尽管有镇痛药和靶向治疗,患者1个月后死亡。在治疗未确诊的乳腺肿瘤患者时,临床医生应注意无法解释和反复报告的症状,并考虑罕见疾病的可能性。我们的文献检索显示没有起源于乳腺的恶性血管球瘤病例,使这个案子成为同类案件中的第一个。
    The glomus tumor is a rare neoplasm that is typically found subungually in the extremities and functions as a specialized neurovascular organ. An extremely rare site for glomus tumors is the breast, with only a few reported cases. Breast glomus tumors present with three typical clinical signs: dull pain, focal tenderness, and cold sensitivity. Less than 10% of all glomus tumors are malignant. We herein present a case of a malignant glomus tumor originating in the breast. Distant metastasis was ruled out, and the tumor was completely resected. However, the patient unexpectedly developed rapid systemic metastasis, detected 5 weeks after tumor removal. Despite the administration of analgesics and targeted therapy, the patient died 1 month later. When treating patients with undiagnosed breast tumors, clinicians should pay attention to unexplained and repeatedly reported symptoms and consider the possibility of a rare disease. Our literature search revealed no cases of malignant glomus tumors originating in the breast, making this case the first of its kind.
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  • 文章类型: Journal Article
    冠状动脉非靶病变的快速进展对于确定未来的心血管事件至关重要。预测非靶病变快速进展的临床因素尚不清楚。这项研究的目的是确定冠状动脉非靶病变快速进展和血运重建的临床预测因素。
    连续进行两次冠状动脉造影的冠心病患者被纳入研究。在两种程序中都识别并评估了所有冠状动脉非靶病变。采用多变量Cox回归分析探讨冠状动脉非靶病变快速进展或血运重建的临床危险因素。
    共纳入1255例患者和1670个病灶。在这群患者中,239(19%)进展迅速,186(14.8%)进行了血运重建。在病变级别,251例(15.0%)进展迅速,194例(11.6%)接受血运重建。进展迅速的患者,病变血运重建和心肌梗死的发生率明显较高。在多变量分析中,高血压(危险比[HR],0.76;95%置信区间[95%CI],0.58-1.00;p=0.049),ST段抬高型心肌梗死(STEMI)(HR,1.46;95%CI,1.03-2.07;p=0.035),糖化血红蛋白(HR,1.16;95%CI,1.01-1.33;p=0.039)和病变分类(B2/C与A/B1)(HR,1.73;95%CI,1.27-2.35;p=0.001)是与快速进展相关的显著因素。甘油三酯的水平(HR,1.10;95%CI,1.00-1.20;p=0.040)和病变分类(B2/C与A/B1)(HR,1.53;95%CI,1.09-2.14;p=0.014)是病变血运重建的预测因子。
    高血压,STEMI,糖化血红蛋白和病变分类可作为冠状动脉非靶病变快速进展的预测因子。甘油三酯水平和病变分类可以预测非靶病变的血运重建。为了预防未来的心血管事件,应更加重视这些因素的患者。
    UNASSIGNED: Rapid progression of coronary non-target lesions is essential for the determination of future cardiovascular events. Clinical factors that predict rapid progression of non-target lesions are unclear. The purpose of this study was to identify the clinical predictors of rapid progression and revascularization of coronary non-target lesions.
    UNASSIGNED: Consecutive patients with coronary heart disease who had undergone two serial coronary angiograms were enrolled. All coronary non-target lesions were identified and evaluated at both procedures. Multivariable Cox regression analysis was used to investigate the clinical risk factors associated with rapid progression or revascularization of coronary non-target lesions.
    UNASSIGNED: A total of 1255 patients and 1670 lesions were enrolled. In this cohort of patients, 239 (19%) had rapid progression and 186 (14.8%) underwent revascularization. At the lesion level, 251 (15.0%) had rapid progression and 194 (11.6%) underwent revascularization. The incidence of lesion revascularization and myocardial infarction was significantly higher in patients with rapid progression. In multivariable analyses, hypertension (hazard ratio [HR], 0.76; 95% confidence interval [95% CI], 0.58-1.00; p = 0.049), ST-segment elevation myocardial infarction (STEMI) (HR, 1.46; 95% CI, 1.03-2.07; p = 0.035), glycosylated hemoglobin (HR, 1.16; 95% CI, 1.01-1.33; p = 0.039) and lesion classification (B2/C versus A/B1) (HR, 1.73; 95% CI, 1.27-2.35; p = 0.001) were significant factors associated with rapid progression. The level of triglycerides (HR, 1.10; 95% CI, 1.00-1.20; p = 0.040) and lesion classification (B2/C versus A/B1) (HR, 1.53; 95% CI, 1.09-2.14; p = 0.014) were predictors of lesion revascularization.
    UNASSIGNED: Hypertension, STEMI, glycosylated hemoglobin and lesion classification may be used as predictors of rapid progression of coronary non-target lesions. The level of triglyceride and lesion classification may predict the revascularization of non-target lesions. In order to prevent future cardiovascular events, increased attention should be paid to patients with these factors.
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  • 文章类型: Letter
    暂无摘要。
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  • 文章类型: Case Reports
    阿尔茨海默病(AD)是老年人最常见的认知障碍类型。在这份报告中,我们介绍了一例52岁女性患者在6个月内病情进展迅速.根据临床症状和神经心理学评估结果,她被诊断为轻度痴呆。根据脑脊液中神经病理学蛋白的结果,头颅磁共振成像,和正电子发射断层扫描/计算机断层扫描,患者显示存在β淀粉样蛋白沉积,病理性tau伴神经变性[A+T+(N+)],指示AD。全外显子组测序显示染色体17q23(rs762056936)上血管紧张素转化酶(ACE)基因外显子25的核苷酸3,755(c.3755C>T)的杂合C-T错义突变。
    Alzheimer\'s disease (AD) is the most common type of cognitive impairment in the elderly. In this report, we presented a case of a 52-year-old woman with rapid disease progression within 6 months. She was diagnosed with mild dementia according to the clinical symptoms and neuropsychological assessment results. Based on the results of neuropathological proteins in cerebrospinal fluid, cranial magnetic resonance imaging, and positron emission tomography/computed tomography, the patient showed the presence of β amyloid deposition, pathologic tau along with neurodegeneration [A+T+(N+)], indicative of AD. Whole exome sequencing revealed a heterozygous C-to-T missense mutation of nucleotide 3,755 (c.3755C > T) in exon 25 of the angiotensin converting enzyme (ACE) gene on chromosome 17q23 (rs762056936).
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  • 文章类型: Journal Article
    建议外科脑血管重建术治疗小儿烟雾病(MMD)。然而,单侧联合旁路手术是否会导致对侧疾病进展尚不确定.该研究旨在调查成功的单侧联合旁路手术后小儿MMD患者的血管结构和局部脑血流量(rCBF)状况,并确定可能的危险因素。
    入选2019年至2021年期间诊断为MMD并入住宣武医院接受联合搭桥手术的儿科患者。进行数字减影血管造影(DSA)和带有动脉自旋标记(ASL)的磁共振成像(MRI),以研究手术期间和短期随访中的血管结构和rCBF。使用铃木的血管造影分期和烟雾血管分级系统。进展定义为单侧手术后检测到的铃木分期或烟雾血管等级增加。所有分析均采用常规统计方法进行。
    共有27名连续患者,中位年龄为8[四分位距(IQR),确定了5-14岁。在非操作(非OP)侧,11例(40.7%)患者出现进展,所有这些都显示出烟雾船等级的增加,和5还显示了铃木在中位数4.7期间的阶段进展(IQR,3.7-5.7)个月随访。然而,与术前相比,非OP侧的rCBF几乎没有变化[术前:中位数,49.6,(IQR,42.9-61.1)毫升/100克/分钟;术后:中位数,50.2,(IQR,43.5-59.3)mL/100g/min;P=0.445]。
    联合旁路手术可能会加速对侧的放射学进展,这发生在rCBF下降之前。非OP侧铃木期较早的MMD患者在单侧联合血运重建后容易快速进展。
    UNASSIGNED: Surgical cerebral revascularization is recommended for treating pediatric moyamoya disease (MMD). However, whether unilateral combined bypass surgery can cause disease progression on the contralateral side is uncertain. The study aimed to investigate the vascular architecture and regional cerebral blood flow (rCBF) status of patients with pediatric MMD after successful unilateral combined bypass surgery and to identify the possible risk factors.
    UNASSIGNED: Pediatric patients diagnosed with MMD and admitted to Xuanwu Hospital who underwent combined bypass surgery between 2019 and 2021 were enrolled. Digital subtraction angiography (DSA) and magnetic resonance imaging (MRI) with arterial spin labeling (ASL) were performed to investigate the vascular architecture and rCBF during surgery and at short-term follow-up. Suzuki\'s angiographic staging and moyamoya vessel grading system were both used. Progression was defined as an increase in either Suzuki stage or moyamoya vessel grade detected after unilateral surgery. All analyses were performed with conventional statistic methods.
    UNASSIGNED: A total of 27 successive patients with a median age of 8 [interquartile range (IQR), 5-14] years old were identified. On the non-operated (non-OP) side, 11 (40.7%) patients demonstrated progression, all of whom showed an increase in the moyamoya vessel grade, and 5 also displayed Suzuki stage progression during the median 4.7 (IQR, 3.7-5.7) months follow-up. However, rCBF barely changed on the non-OP side compared to preoperation [preoperation: median, 49.6, (IQR, 42.9-61.1) mL/100 g/min; postoperation: median, 50.2, (IQR, 43.5-59.3) mL/100 g/min; P=0.445].
    UNASSIGNED: Combined bypass surgery might accelerate the radiological progression on the contralateral side, which occurs before the decline of rCBF. Those with earlier Suzuki stage MMD of the non-OP side are prone to rapid progression after unilateral combined revascularization.
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  • 文章类型: Journal Article
    本研究旨在确定不同方法在常染色体显性遗传多囊肾病(ADPKD)的亚太患者中预测快速进展者(RP)及其临床特征的实用性。
    这是一项针对亚太地区ADPKD患者的多国回顾性观察队列研究。来自澳大利亚的五家医院,中国,韩国,台湾,土耳其参加了这项研究。RP由欧洲肾脏协会-欧洲透析和移植协会(ERA-EDTA)指南定义,并与缓慢进展者(SP)进行比较。
    在768名患者中,426例患者为RP。三百六位患者仅满足1项标准,而120位患者满足RP的多项标准。历史估计的肾小球滤过率(eGFR)下降符合210例患者的RP标准。五名患者符合身高调整后的总肾脏体积(TKV)历史增加的标准。210例患者满足基于肾体积的标准。在后续期间,囊肿感染,囊肿出血,和蛋白尿更频繁发生在RP中;13.9%和2.1%的RP和SP,分别,进展为终末期肾病(ESKD)。基于历史eGFR下降的RP标准与2年随访期间eGFR变化的相关性最强。
    在随访期间,在现实临床实践中,应使用各种评估策略来识别亚太ADPKD患者的RP,囊肿感染,囊肿出血,蛋白尿的发生频率更高;与SPs相比,更多的患者在RP中进展为ESKD。
    UNASSIGNED: This study aimed to determine the utility of different methods to predict rapid progressors (RPs) and their clinical characteristics in Asia-Pacific patients with autosomal dominant polycystic kidney disease (ADPKD).
    UNASSIGNED: This was a multinational retrospective observational cohort study of patients with ADPKD in the Asia-Pacific region. Five hospitals from Australia, China, South Korea, Taiwan, and Turkey participated in this study. RP was defined by European Renal Association-European Dialysis and Transplantation Association (ERA-EDTA) guidelines and compared to slow progressors (SPs).
    UNASSIGNED: Among 768 patients, 426 patients were RPs. Three hundred six patients met only 1 criterion and 120 patients satisfied multiple criteria for RP. Historical estimated glomerular filtration rate (eGFR) decline fulfilled the criteria for RP in 210 patients. Five patients met the criteria for a historical increase in height-adjusted total kidney volume (TKV). The 210 patients satisfied the criteria for based on kidney volume. During the follow-up period, cyst infections, cyst hemorrhage, and proteinuria occurred more frequently in RP; and 13.9% and 2.1% of RPs and SPs, respectively, progressed to end-stage kidney disease (ESKD). RP criteria based on historical eGFR decline had the strongest correlation with eGFR change over a 2-year follow-up.
    UNASSIGNED: Various assessment strategies should be used for identifying RPs among Asian-Pacific patients with ADPKD in real-world clinical practice during the follow-up period, cyst infections, cyst hemorrhage, and proteinuria occurred more frequently; and more patients progressed to ESKD in RPs compared with SPs.
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  • 文章类型: Journal Article
    未经证实:坏死性小肠结肠炎(NEC)是一种破坏性的胃肠道急症,具有显著的死亡率和发病率。一部分患者进展迅速,并在短时间内接受了手术干预。本研究旨在建立预测早产儿NEC快速进展的模型。
    UNASSIGNED:2015年12月至2019年4月在广州市妇女儿童医疗中心进行了一项回顾性研究,以评估NEC新生儿。快速进展的NEC被定义为需要手术干预或在NEC发病48小时内死亡。患者分为两组:快速进行性NEC(RP-NEC)和非快速进行性NEC(nRP-NEC)。人口统计数据,围产期特征,考试变量,收集发病时的影像学检查结果。
    UNASSIGNED:共216例NEC早产儿被纳入研究,其中64个有RP-NEC,152个有NRP-NEC。RP-NEC和nRP-NEC患者的死亡率分别为32.8%和3.28%,分别。男性(p值,调整后的赔率比[95%置信区间]:0.002,3.43[1.57,7.53]),门静脉气体(0.000,8.82[3.73,20.89]),中性粒细胞<2.0×109/L(0.005,4.44[1.59,12.43]),pH<7.3(7.2≤pH<7.3)(0.041,2.95[1.05,8.31]),NEC发病时pH<7.2(0.000,11.95[2.97,48.12])被确定为RP-NEC的独立危险因素。包括四个危险因素的已建立模型呈现0.801的曲线下面积,具有83%的特异性和66%的灵敏度。
    未经证实:在患有NEC的早产儿中,在进展迅速的患者中观察到显著较高的死亡率.建议对这些患者进行密切监测,我们相信我们建立的模型可以有效地预测这种快速发展过程。
    UNASSIGNED: Necrotizing enterocolitis (NEC) is a devastating gastrointestinal emergency with significant mortality and morbidity rates. A subset of patients progressed rapidly and underwent surgical intervention within a short period. This study aimed to establish a model to predict the rapid progression of NEC in preterm neonates.
    UNASSIGNED: A retrospective study was conducted to review neonates with NEC between December 2015 and April 2019 at the Guangzhou Women and Children\'s Medical Center. Rapidly progressive NEC was defined as the need for surgical intervention or death within 48 h of NEC onset. Patients were divided into two groups: rapidly progressive NEC (RP-NEC) and non-rapidly progressive NEC (nRP-NEC). Data on demographics, perinatal characteristics, examination variables, and radiographic findings at onset were collected.
    UNASSIGNED: A total of 216 preterm neonates with NEC were included in the study, of which 64 had RP-NEC and 152 had nRP-NEC. The mortality rates of patients with RP-NEC and nRP-NEC were 32.8% and 3.28%, respectively. Male sex (p-value, adjusted odds ratio [95% confidence interval]: 0.002, 3.43 [1.57, 7.53]), portal venous gas (0.000, 8.82 [3.73, 20.89]), neutrophils <2.0 × 109/L (0.005, 4.44 [1.59, 12.43]), pH <7.3 (7.2 ≤ pH < 7.3) (0.041, 2.95 [1.05, 8.31]), and pH <7.2 (0.000, 11.95 [2.97, 48.12]) at NEC onset were identified as independent risk factors for RP-NEC. An established model that included the four risk factors presented an area under the curve of 0.801 with 83% specificity and 66% sensitivity.
    UNASSIGNED: Among preterm neonates with NEC, a significantly higher mortality rate was observed in those with rapid progression. It is recommended that close surveillance be performed in these patients, and we are confident that our established model can efficiently predict this rapid progression course.
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  • 文章类型: Case Reports
    背景:据报道,在这种情况下,经过10个月的随访,在免疫治疗后,气管支架的保护作用可以缓解气道阻塞并减少恶性气管食管瘘(MTEF)快速进展的副作用。
    方法:在一名58岁男性的主气道中放置两种硅胶支架,以缓解晚期食管癌引起的气道阻塞。然后,患者接受了四剂托里帕利单抗。随后,快速,发现原始瘘管的进行性恶化。尽管免疫治疗后瘘管迅速扩大,它仍然被完全覆盖,可能正因为如此,他的病情保持稳定。因此,免疫疗法可以继续治疗原发性肿瘤。尽管做出了这些努力,病人死于食道癌的进展。
    结论:适当大小的气管支架置入联合免疫检查点抑制剂可改善MTEF患者的生活质量和生存率。
    BACKGROUND: The protective effect of tracheal stents is reported to relieve airway obstruction and reduce side effects of rapid progression of malignant tracheoesophageal fistula (MTEF) after immunotherapy in this case with 10 mo follow-up.
    METHODS: Two kinds of silicone stents were placed in the main airway of a 58-year-old male to relieve the airway obstruction caused by advanced esophageal carcinoma. The patient then received four doses of toripalimab. Subsequently, rapid, progressive deterioration of the original fistula was found. Although the fistula enlarged rapidly after immunotherapy, it remained covered completely, and likely because of this, his condition remained stable. Therefore, immunotherapy could be continued to treat the primary tumor. Despite these efforts, the patient died of the advancement of his esophageal cancer.
    CONCLUSIONS: Appropriately-sized tracheal stent placement combined with immune checkpoint inhibitors may improve the quality of life and survival of patients with MTEF.
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  • 文章类型: Case Reports
    癌症患者对免疫检查点抑制剂(ICIs)的反应存在异质性,包括过度进展,免疫治疗后肿瘤进展非常迅速,和伪进程,这是最初的增加,然后是肿瘤负荷或肿瘤病变数量的减少。这种异质性使临床决定复杂化,因为过早退出治疗或长期无效治疗会损害患者。我们介绍了两名接受ICIs治疗的患者,其反应具有异质性。一名患者右大腿患有默克尔细胞癌,另一个患有鼻咽鳞癌。第一名患者接受辛替利玛治疗,第二名接受辛替利玛联合abraxane治疗。在第一个病人中,在使用sintilimab的第一个治疗周期后,皮下病变大幅增加。第二个病人,sintilmab联合abraxane治疗第二周期后,皮下病变逐渐增加。在这两种情况下,活检证实新出现的病灶是原发肿瘤的转移。这两个病例提醒临床医生,当使用ICIs治疗后出现皮下结节时,需要进行病理活检以确定病程的性质-假性进展或快速进展。
    There is heterogeneity in cancer patients\' responses to immune checkpoint inhibitors (ICIs), including hyperprogression, which is very rapid tumor progression following immunotherapy, and pseudoprogression, which is an initial increase followed by a decrease in tumor burden or in the number of tumor lesions. This heterogeneity complicates clinical decisions because either premature withdrawal of the treatment or prolonged ineffective treatment harms patients. We presented two patients treated with ICIs with heterogeneous responses. One patient had Merkel cell carcinoma in the right thigh, and the other had nasopharyngeal squamous carcinoma. The first patient was treated with sintilimab and the second with sintilimab combined with abraxane. In the first patient, subcutaneous lesions grew substantially after the first cycle of treatment with sintilimab. In the second patient, subcutaneous lesions grew gradually after the second cycle of treatment with sintilimab combined with abraxane. In both cases, biopsy examination confirmed that newly emerged lesions were metastases of the primary tumor. These two cases remind clinicians that when subcutaneous nodules appear after treatment with ICIs, pathological biopsy is needed to determine the nature-pseudoprogression or rapid progression-of the disease course.
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  • 文章类型: Journal Article
    未经证实:大量证据表明,主动脉瓣狭窄(AS)的快速进展与不良预后显著相关。糖尿病是否加速AS的进展仍存在争议。
    未经批准:本研究的目的是调查糖尿病是否与AS的快速进展有关。
    UNASSIGNED:我们回顾性分析了276例AS患者,这些患者在2016年1月至2021年6月期间接受了至少两次经胸超声心动图检查,最大间隔≥180天。AS严重程度由主动脉射流峰值速度(Vmax)和/或平均压力梯度的特定阈值定义。Vmax≥0.3m/s/年的增加被定义为快速进展。使用二元Logistic回归模型来确定糖尿病与AS快速进展之间的关联。
    UNASSIGNED:中位超声心动图随访时间为614天,Vmax的年增长率为0.16(0.00-0.41)m/s。与那些没有快速发展的人相比,进展迅速的患者年龄较大,更容易患糖尿病(分别为P=0.040和P=0.010).在单变量二元Logistic回归分析中,糖尿病与AS的快速进展相关(OR=2.02,P=0.011)。在基于模型2和模型3的多变量分析中,这种相关性仍然显著(OR=1.93,P=0.018;OR=1.93,P=0.022)。根据Vmax进行倾向评分匹配后,糖尿病还与AS的快速进展相关(OR=2.57,P=0.045)。
    未经证实:糖尿病与AS的快速进展密切相关。
    UNASSIGNED: Mounting evidence indicates that rapid progression of aortic stenosis (AS) is significantly associated with poor prognosis. Whether diabetes accelerates the progression of AS remains controversial.
    UNASSIGNED: The purpose of the present study was to investigate whether diabetes was associated with rapid progression of AS.
    UNASSIGNED: We retrospectively analyzed 276 AS patients who underwent transthoracic echocardiography at least twice with a maximum interval ≥ 180 days from January 2016 to June 2021. AS severity was defined by specific threshold values for peak aortic jet velocity (Vmax) and/or mean pressure gradient. An increase of Vmax ≥ 0.3 m/s/year was defined as rapid progression. The binary Logistic regression models were used to determine the association between diabetes and rapid progression of AS.
    UNASSIGNED: At a median echocardiographic follow-up interval of 614 days, the annual increase of Vmax was 0.16 (0.00-0.41) m/s. Compared with those without rapid progression, patients with rapid progression were older and more likely to have diabetes (P = 0.040 and P = 0.010, respectively). In the univariate binary Logistic regression analysis, diabetes was associated with rapid progression of AS (OR = 2.02, P = 0.011). This association remained significant in the multivariate analysis based on model 2 and model 3 (OR = 1.93, P = 0.018; OR = 1.93, P = 0.022). After propensity score-matching according to Vmax, diabetes was also associated rapid progression of AS (OR = 2.57, P = 0.045).
    UNASSIGNED: Diabetes was strongly and independently associated with rapid progression of AS.
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