clinical characteristics

临床特征
  • 文章类型: Journal Article
    OBJECTIVE: To analyze gender-related differences in patient and care characteristics and in toxicology findings in suspected cases of drug facilitated crime (DFC).
    METHODS: Observational cross-sectional study of all patients in suspected DFC cases attended in the emergency department of Hospital Clínico San Carlos and of their blood or urine samples analyzed by the National institute of Toxicology and Forensics in Madrid between March 1, 2015, and March 1, 2023. We analyzed variables from patient records and the toxicology reports according to gender.
    RESULTS: A total of 514 suspected DFC episodes were studied; 101 (19.6%) were proactive crimes, 61 (11.9%) opportunistic, and 352 (68.5%) mixed. The median (interquartile range) age was 25 years (21-34 years), and 370 (72%) were women. Eighty-three percent of the patients had amnesia, and 48% of the cases involved sexual assault or robbery. Toxicology identified substances in 78% of the patients (alcohol, 53%; street drugs, 37%; and/or psychopharmaceuticals or opioids, 23%). Independent variables associated with female gender in the multivariate analysis, according to adjusted odds ratio (aORs) were age less than 25 years (aOR, 2.73; 95% CI, 1.75 4.24; P < .001); physician-referred emergency (aOR, 1.77; 95% CI, 1.12-2.80; P = .03); robbery (aOR, 0.25; 95% CI, 0.15-0.41; P < .001); alcohol-positive test result (aOR, 1.91; 95% CI, 1.21-3.00; P = .01); and a drug-positive result (aOR, 0.43; 95% CI, 0.28-0.64; P < .001). Police and a forensic physician intervened in 13% of the cases, and in such cases the victim was more likely to be female (aOR, 3.97; 95% CI, 1.41-11.13; P < .001). Toxicology identified the presence of an unknown substance in 39%, and a woman was less likely to be involved in such cases (aOR, 0.43; 95% CI, 0.28-0.67; P < .001).
    CONCLUSIONS: The majority of victims of DFCs were female, and the crimes were mixed, involving involve alcohol, psychopharmaceuticals or street drugs. Female victims were more likely to be under the age of 25 years, be referred to the emergency service by a physician, be attended by a forensic physician for sexual assault, and have an alcoholpositive toxicology report. Women were also less likely to report a robbery or have a toxicology report identifying drugs or an unknown substance.
    OBJECTIVE: Analizar las diferencias en las características de los pacientes atendidos por sospecha de sumisión química (SQ) y en los resultados del análisis toxicológico (AT) en función del sexo.
    METHODS: Estudio observacional transversal retrospectivo que incluyó a todos los casos con SQ atendidos en el servicio de urgencias del Hospital Clínico San Carlos y las muestras (sangre o orina) para el AT en el Instituto Nacional de Toxicología y Ciencias Forenses de Madrid entre el 1 de marzo de 2015 y el 1 de marzo de 2023. Se analizan variables de la historia clínica y del AT según el sexo.
    RESULTS: Se incluyeron 514 episodios con sospecha de SQ [101 (19,6%) proactiva, 61 (11,9%) oportunista y 352 (68,5%) mixta] en pacientes con una mediana de 25 años (RIC: 21-34), 370 (72%) de sexo femenino. El 83% presentó amnesia y el 48% asoció agresión sexual o robo. En el 78% se identificó alguna sustancia en el AT (53% alcohol etílico, 37% drogas y/o 23% psicofármaco u opiáceos). En el análisis multivariado las variables que se asociaron de manera independiente con el sexo femenino fueron la edad menor de 25 años con ORa de 2,73 (IC 95%: 1,75-4,24; p < 0,001), con médico deriva a urgencias con ORa de 1,77 (IC 95%: 1,12-2,80; p = 0,03), delito de robo con de ORa 0,25 (IC 95%: 0,15-0,41; p < 0,001), alcohol etílico en el AT con ORa 1,91 (IC 95%: 1,21-3,00; p = 0,01) y alguna droga en el AT con ORa 0,43 (IC 95%: 0,28-0,64; p < 0,001). En el 13% de casos hubo intervención policial y médico-forense y fue más probable que fuera a una mujer, con ORa 3,97 (IC 95%: 1,41-11,13; p < 0,001). En el 39% de AT se identificó alguna sustancia desconocida y fue menos probable que fuera mujer, con ORa de 0,43 (IC 95%: 0,28-0,67; p < 0,001).
    CONCLUSIONS: La mayoría de casos registrados fueron mujeres con sospecha de SQ mixta por alcohol, psicofármacos o drogas de abuso. Las mujeres presentaron mayor probabilidad de tener menos de 25 años, ser derivada a urgencias por un médico, de intervención médico-forense por agresión sexual y encontrar alcohol etílico en el AT.
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  • 文章类型: Case Reports
    背景:胆管癌是胆道树最常见的恶性肿瘤,预后不良。腺癌是胆管癌最常见的病理类型,但罕见的鳞状,腺鳞状,据报道,粘液性变体没有足够的临床数据。
    方法:本报告描述了一例罕见的肝内胆管原发性鳞状细胞癌(SCC)。患者入院时肝尾状叶肿瘤,无明显临床症状。检查显示乙肝表面抗原阳性,甲胎蛋白略有增加至16.34ng/mL,肝尾状叶有不规则的轻微异质性增强病变,最初被认为是肝细胞癌。进行了腹腔镜切除术,最终病理提示罕见的肝内胆管原发性SCC。免疫组织化学显示绒毛阳性,p63部分阳性,肝细胞阴性,CK7、CK8、CK19和CK20。Ki-67指数约为60%。患者接受6个周期的Tegio化疗。15个月后在肝脏中检测到新的病变。进行了手术,患者在当地医院接受了随访。迄今为止,没有观察到新的病变。
    结论:手术是可切除病变的首选,基于病理学的联合化疗对于提高总生存率至关重要。
    BACKGROUND: Cholangiocarcinoma is the most common malignancy of the biliary tree and has a poor prognosis. Adenocarcinoma is the most common pathological type of cholangiocarcinomas, but rare squamous, adenosquamous, and mucinous variants have been reported without adequate clinical data.
    METHODS: This report describes a rare case of primary squamous cell carcinoma (SCC) of the intrahepatic bile duct. The patient was admitted with a tumor in the hepatic caudate lobe with no obvious clinical symptoms. Examination revealed hepatitis B surface antigen positivity, a slight increase in alfa-fetoprotein to 16.34 ng/mL, and an irregular slightly heterogeneous enhancing lesion in the hepatic caudate lobe, which was initially thought to be hepatocellular carcinoma. Laparoscopic resection was performed, and the final pathology suggested a rare primary SCC of the intrahepatic bile duct. Immunohistochemistry indicated positivity for villin, partial positivity for p63, and negativity for hepatocyte, CK7, CK8, CK19, and CK20. The Ki-67 index was approximately 60%. The patient received six cycles of Tegio chemotherapy. A new lesion was detected in the liver after 15 months. The surgery was performed, and the patient was followed-up at a local hospital. To date, no new lesions have been observed.
    CONCLUSIONS: Surgery is the first choice for resectable lesions, and combined chemotherapy based on pathology is essential for increasing overall survival.
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  • 文章类型: Journal Article
    戊型肝炎(HE),由戊型肝炎病毒(HEV)引起,是全球急性病毒性肝炎的重要原因,也是主要的公共卫生问题,特别是在中国特定的高流行地区,具有不同的传播途径和区域差异。确定HE传播的主要危险因素对于针对弱势群体制定有针对性的干预措施至关重要。
    这项研究采用了1:1匹配的病例对照方法,使用由医疗记录补充的标准化问卷进行数据验证。
    在442例HE病例和428例健康对照中,与对照组相比,病例组的疲劳(46.21%)和食欲不振(43.84%)的患病率更高。此外,肝功能指标明显高于病例组,平均丙氨酸氨基转移酶(ALT)水平为621.94U/L,天冬氨酸氨基转移酶(AST)水平为411.53U/L。重度HE患者以男性为主,ALT和AST水平显著升高,分别达到1443.81U/L和862.31U/L,伴随着更高的发生率疲劳(90%)和食欲不振(75%)。多因素分析表明,经常外出就餐(OR=2.553,95CI:1.686-3.868),卫生条件差(OR=3.889,95CI:1.399-10.807),合并慢性病(OR=2.275,95CI:1.616-3.202)是HE感染的危险因素;相反,良好的卫生习惯是HE感染的保护因素(OR=0.698,95CI:0.521~0.934)。
    总而言之,浙江省HE感染与饮食习惯和环境卫生密切相关,患有慢性疾病或合并感染的个体面临更高的风险。这凸显了有针对性的健康教育以减少这些人群中HE的发病率的必要性。
    UNASSIGNED: Hepatitis E (HE), caused by the Hepatitis E virus (HEV), is a significant cause of acute viral hepatitis globally and a major public health concern, particularly in specific high-prevalence areas in China, which have diverse transmission routes and regional differences. Identifying the primary risk factors for HE transmission is essential to develop targeted interventions for vulnerable populations.
    UNASSIGNED: This study employed a 1:1 matched case-control methodology, using a standardized questionnaire complemented by medical records for data validation.
    UNASSIGNED: Among the 442 HE cases and 428 healthy controls, the case group had a higher prevalence of fatigue (46.21%) and loss of appetite (43.84%) compared to the control group. Furthermore, liver function indicators were significantly higher in the case group, with an average alanine aminotransferase (ALT) level of 621.94 U/L and aspartate aminotransferase (AST) level of 411.53 U/L. Severe HE patients were predominantly male, with significantly increased ALT and AST levels reaching 1443.81 U/L and 862.31 U/L respectively, along with a higher incidence of fatigue (90%) and loss of appetite (75%). Multifactorial analysis indicated that frequent dining out (OR = 2.553, 95%CI:1.686-3.868), poor hygiene conditions (OR = 3.889, 95%CI:1.399-10.807), and comorbid chronic illnesses (OR = 2.275, 95%CI:1.616-3.202) were risk factors for HE infection; conversely, good hygiene practices were protective factors against HE infection (OR = 0.698, 95%CI:0.521-0.934).
    UNASSIGNED: In conclusion, HE infection in Zhejiang Province is closely associated with dietary habits and environmental hygiene, and individuals with chronic diseases or co-infections are at increased risk. This highlights the need for targeted health education to reduce the incidence of HE among these populations.
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  • 文章类型: Journal Article
    目的:系统回顾伊朗报道的克雅氏病(CJD)病例。
    方法:使用PubMed®对伊朗的CJD病例进行了全面的文献综述,Scopus®和GoogleScholar数据库。此外,搜索了伊朗数据库MagIran的波斯语报告。病例选择使用以下标准:(i)伊朗血统的患者;(ii)在同行评审的期刊或信誉良好的医学数据库中发表;(iii)根据已建立的诊断标准对CJD进行明确诊断。
    结果:本系统综述包括12例报告中的13例病例。大多数病例为女性(13人中有11人;84.6%)。入院时患者的平均±SD年龄为59.38±7.44岁。病例审查的结果表明,伊朗的CJD患病率尚未完全确定。CJD可能与其他临床体征一起被误诊。该疾病最普遍的早期适应症本质上是精神病学和神经学。在某些情况下,发现诊断有相当大的延迟,并且缺乏脑部尸检记录。
    结论:努力提高诊断能力,提高认识和建立监测系统对于管理在伊朗提供CJD早期诊断的挑战是必要的。
    OBJECTIVE: To systematically review the reported cases of Creutzfeldt-Jakob disease (CJD) in Iran.
    METHODS: A comprehensive literature review of CJD cases in Iran was undertaken using the PubMed®, Scopus® and Google Scholar databases. In addition, the Iranian database MagIran was searched for Persian language reports. Case selection used the following criteria: (i) patients of Iranian origin; (ii) publication in peer-reviewed journals or reputable medical databases; (iii) a definitive diagnosis of CJD based on established diagnostic criteria.
    RESULTS: Thirteen cases from twelve reports were included in this systematic review. The majority of the cases were female (11 of 13; 84.6%). The mean ± SD age of patients at hospital admission was 59.38 ± 7.44 years. The findings of the case review suggested that the prevalence of CJD in Iran is not fully established. CJD may be misdiagnosed alongside other clinical signs. The most prevalent early indications of the disease were psychiatric and neurological in nature. A considerable delay in diagnosis was observed in some cases and there was a shortage of brain autopsy records.
    CONCLUSIONS: Efforts to improve diagnostic capabilities, promote awareness and establish monitoring systems are necessary for managing the challenges of providing an early diagnosis of CJD in Iran.
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  • 文章类型: Journal Article
    背景:Billroth早在1889年就首次描述了多原发恶性肿瘤(MPMT),沃伦和盖茨于1932年发表了第一份报告。从那以后,已经报道了许多病例。对1104269例癌症患者的文献回顾显示,MPMT的发生率为0.73%至11.7%。近年来,然而,这种现象的发生率有明显上升的趋势,这可能与许多不同的因素有关,包括现代诊断程序的进步,促进更多MPMT的检查和诊断,化疗和放疗的暴露增加了癌症患者新发恶性肿瘤的风险,并延长癌症患者的生存期,从而有足够的时间发展新的原发性癌症。
    目的:分析发病率,临床特征,治疗因素,患病率,以及在单中心治疗的胃肠道MPMT患者的预后。此外,我们分析了不同的肿瘤组合,肿瘤发生的时间间隔,和分期。
    方法:这项回顾性队列研究分析了在兰州甘肃省医院接受治疗的8059例经病理证实的胃肠道恶性肿瘤患者,甘肃,2011年6月至2020年6月的中国。其中,85例患者有MPMTs。临床特征,治疗因素,患病率,并分析了后一个队列的预后。
    结果:胃肠道恶性肿瘤患者MPMTs的发生率为1.05%(85/8059),包括83例双原发恶性肿瘤和2例三原发恶性肿瘤,其中57例(67.06%)为同步MPMTs(SMPMTs),28例(32.94%)为异时MPMTs(MMPMTs)。在SMPMT类别中的直肠结肠癌和MMPMT类别中的胃结肠癌之间发现了最常见的关联。对于MMPMT,中位间期为53个月.整体1,诊断为第一原发癌的3年和5年生存率为91.36%,65.41%,和45.97%,第二原发癌的诊断分别为67.90%,29.90%,和17.37%,分别。
    结论:胃肠道中的MPMT发病率高,预后差。因此,胃肠道肿瘤患者有必要同时进行胃镜和结肠镜检查。多学科综合诊疗可提高MPMT的诊断率和治疗效率。
    BACKGROUND: Multiple primary malignant tumors (MPMTs) was first described by Billroth as early as 1889, with the first report published by Warren and Gates in 1932. Since then, numerous cases have been reported. A literature review of 1104269 patients with cancer revealed that the incidence of MPMTs ranged from 0.73 to 11.7%. In recent years, however, there has been a significant upward trend in the incidence of this phenomenon, which may be associated with many different factors, including the advancement of modern diagnostic procedures facilitating the examination and diagnosis of more MPMTs, increased exposure to chemotherapy and radiotherapy that exacerbate the risk of new malignant tumors in patients with cancer, and prolonged survival of patients with cancer allowing sufficient time for the development of new primary cancers.
    OBJECTIVE: To analyze the incidence, clinical features, treatment factors, prevalence, and prognosis of patients with MPMTs in the gastrointestinal tract treated in a single center. Additionally, we analyzed the different tumor combinations, time interval between the occurrence of tumors, and staging.
    METHODS: This retrospective cohort study analyzed 8059 patients with pathologically confirmed gastrointestinal malignant tumors treated at the Gansu Province Hospital in Lanzhou, Gansu, China between June 2011 and June 2020. Of these, 85 patients had MPMTs. The clinical features, treatment factors, prevalence, and prognosis of this latter cohort were analyzed.
    RESULTS: The incidence of MPMTs in patients with gastrointestinal malignant tumors was 1.05% (85/8059), including 83 double primary malignant tumors and two triple primary malignant tumors of which 57 (67.06%) were synchronous MPMTs (SMPMTs) and 28 (32.94%) were metachronous MPMTs (MMPMTs). The most frequent associations were found between the rectum colon cancers within the SMPMT category and the gastric-colon cancers within the MMPMT category. For the MMPMTs, the median interval was 53 months. The overall 1-, 3- and 5-year survival rates from diagnosis of the first primary cancer were 91.36%, 65.41%, and 45.97%, respectively; those from diagnosis of the second primary cancer were 67.90%, 29.90%, and 17.37%, respectively.
    CONCLUSIONS: MPMTs in the gastrointestinal tract have a high incidence and poor prognosis. Thus, it is necessary to perform both gastroscopy and colonoscopy in patients with gastrointestinal tumors. Multidisciplinary comprehensive diagnosis and treatment may improve the diagnosis rate and treatment efficiency of MPMTs.
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  • 文章类型: Case Reports
    抗硫酸盐抗体是格林-巴利综合征(GBS)诊断的关键生物标志物。然而,关于抗硫酸盐抗体相关GBS的病例报告很少,特别是非典型病例。
    一名63岁男子表现为肢体麻木和复视持续2周,在过去的4天里有明显的恶化。他的病史包括脑梗塞,糖尿病,和冠状动脉粥样硬化性心肌病。体格检查显示他的左眼活动有限,四肢感觉减弱。初始治疗包括抗血小板药物,降胆固醇药物,降血糖药,以及改善脑循环的药物.尽管如此,他的病情恶化了,导致双侧面瘫,谵妄,共济失调,下肢肌肉力量下降。静脉注射大剂量免疫球蛋白和地塞米松治疗逐渐改善。1个月的随访发现明显的神经系统后遗症。
    一名53岁妇女因子宫腺肌病入院,随后突然出现四肢无力,麻木,疼痛逐渐恶化,表现为所有四肢的感觉和肌肉力量减弱。大剂量静脉注射免疫球蛋白,服用维生素B1和甲钴胺。在1个月的随访中,患者仍然出现肢体麻木和行走困难。在这两个病人中,在脑脊液(CSF)分析中发现了白蛋白细胞解离,在CSF中检测到阳性抗硫酸盐抗体,肌电图显示周围神经损伤。
    抗硫酸盐抗体相关的GBS可出现Miller-Fisher综合征,脑干脑炎,或者两者的结合,伴随着严重的锥体束损伤和残余的神经后遗症,从而扩大了这种GBS亚型的临床概况。抗硫酸盐抗体是重要的诊断生物标志物。进一步探索病理生理机制对于精确治疗和改善预后是必要的。
    UNASSIGNED: Anti-sulfatide antibodies are key biomarkers for the diagnosis of Guillain-Barré syndrome (GBS). However, case reports on anti-sulfatide antibody-related GBS are rare, particularly for atypical cases.
    UNASSIGNED: A 63 years-old man presented with limb numbness and diplopia persisting for 2 weeks, with marked deterioration over the previous 4 days. His medical history included cerebral infarction, diabetes, and coronary atherosclerotic cardiomyopathy. Physical examination revealed limited movement in his left eye and diminished sensation in his extremities. Initial treatments included antiplatelet agents, cholesterol-lowering drugs, hypoglycemic agents, and medications to improve cerebral circulation. Despite this, his condition worsened, resulting in bilateral facial paralysis, delirium, ataxia, and decreased lower limb muscle strength. Treatment with intravenous high-dose immunoglobulin and dexamethasone resulted in gradual improvement. A 1 month follow-up revealed significant neurological sequelae.
    UNASSIGNED: A 53 years-old woman was admitted for adenomyosis and subsequently experienced sudden limb weakness, numbness, and pain that progressively worsened, presenting with diminished sensation and muscle strength in all limbs. High-dose intravenous immunoglobulin, vitamin B1, and mecobalamin were administered. At the 1 month follow-up, the patient still experienced limb numbness and difficulty walking. In both patients, albuminocytologic dissociation was found on cerebrospinal fluid (CSF) analysis, positive anti-sulfatide antibodies were detected in the CSF, and electromyography indicated peripheral nerve damage.
    UNASSIGNED: Anti-sulfatide antibody-related GBS can present with Miller-Fisher syndrome, brainstem encephalitis, or a combination of the two, along with severe pyramidal tract damage and residual neurological sequelae, thereby expanding the clinical profile of this GBS subtype. Anti-sulfatide antibodies are a crucial diagnostic biomarker. Further exploration of the pathophysiological mechanisms is necessary for precise treatment and improved prognosis.
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  • 文章类型: Journal Article
    目的:分析临床特点,危险因素,病原体分布,与无重症肺炎(NSP)的患者相比,原发性干燥综合征(pSS)患者的预后指标。
    方法:本病例对照研究纳入2014年6月至2023年5月苏州大学第一附属医院收治的24例合并SP和96NSP的pSS住院患者。涵盖人口统计的数据,合并症,治疗,和实验室结果进行回顾性收集。单变量和多变量回归分析,ROC曲线,使用SPSS23.0进行统计分析,评估危险因素。回顾性分析临床特点及危险因素,突出有和没有SP的pSS患者之间的不同参数。
    结果:在几个参数中观察到明显的差异:pSS活性(P<0.001),白细胞(P=0.043),淋巴细胞(P<0.001),中性粒细胞(P=0.042),C反应蛋白(P=0.042),CD8+T细胞(P=0.017)。值得注意的是,淋巴细胞计数和SS活性显示出强大的辨别能力(AUC>0.85)。C反应蛋白(CRP),降钙素原,CD4+T细胞,和IgA显示与SP显著相关;较高的CRP水平与风险增加相关,而较低的CD4+T细胞和IgA水平与风险增加相关。SS活动显著影响结果。各种生物标志物表现出不同的辨别能力,但缺乏与结果的强烈预测关联。
    结论:与那些NSP相比,患有SP的pSS患者表现出更高的疾病活动性和改变的免疫谱。淋巴细胞计数和SS活性作为稳健的鉴别器出现。较高的CRP水平与SP风险增加相关,而较低的CD4+T细胞和IgA水平与风险增加相关。SS活动显著影响患者预后。关键点•与那些NSP相比,患有SP的pSS患者表现出更高的疾病活动性和改变的免疫谱。•淋巴细胞计数和SS活性作为稳健的鉴别器出现。•较高的CRP水平与SP风险增加相关,而较低的CD4+T细胞和IgA水平与风险降低相关。•SS活动显著影响患者结果。
    OBJECTIVE: To analyze clinical characteristics, risk factors, pathogen distribution, and prognostic markers in primary Sjögren\'s syndrome (pSS) patients with severe pneumonia (SP) compared to those without severe pneumonia (NSP).
    METHODS: This case-control study included 24 hospitalized pSS patients with SP and 96 NSP at the first affiliated hospital of Soochow university from June 2014 to May 2023. Data encompassing demographics, comorbidities, treatments, and laboratory results were retrospectively collected. Univariate and multivariate regression analyses, ROC curves, and statistical analyses using SPSS 23.0 assessed risk factors. The study retrospectively analyzed clinical features and risk factors, highlighting distinct parameters between pSS patients with and without SP.
    RESULTS: Marked differences were observed in several parameters: pSS activity(P < 0.001), white blood cell (P = 0.043), lymphocyte (P < 0.001), neutrophils (P = 0.042), C-reactive protein (P = 0.042), and CD8+ T cell (P = 0.017). Notably, lymphocyte count and SS activity demonstrated robust discrimination ability (AUC > 0.85). C-reactive protein (CRP), procalcitonin, CD4+ T cell, and IgA showed significant associations with SP; higher CRP levels correlated with increased risk, while lower CD4+ T cell and IgA levels associated with increased risk. SS activity significantly impacted outcomes. Various biomarkers exhibited diverse discriminatory abilities but lacked strong predictive associations with outcomes.
    CONCLUSIONS: pSS patients with SP exhibited higher disease activity and altered immune profiles compared to those NSP. Lymphocyte count and SS activity emerged as robust discriminators. Higher CRP levels correlated with increased risk of SP, while lower CD4+T cell and IgA levels associated with increased risk. SS activity significantly impacted patient outcomes. Key Points • pSS patients with SP exhibited higher disease activity and altered immune profiles compared to those NSP. • Lymphocyte count and SS activity emerged as robust discriminators. • Higher CRP levels correlated with increased risk of SP, while lower CD4+ T cell and IgA levels associated with decreased risk. • SS activity significantly impacted patient outcomes.
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  • 文章类型: Journal Article
    目的:肌炎特异性抗体(MSAs)和肌炎相关抗体(MAAs)与皮肌炎(DM)的独特临床表型相关。本研究的目的是阐明MSA阴性DM患者的临床和免疫学特征。
    方法:从2013年至2022年,共筛查了515名诊断为DM的个体,并将220名DM患者纳入该回顾性队列。对这些患者的临床和实验室资料进行分析。
    结果:MSAs阴性DM患者分为两组:MAAs阴性(MSAs(-)/MAAs(-))组和MAAs阳性(MSAs(-)/MAAs(+))组。MSAs(-)/MAAs()DM患者的雷诺现象百分比(P=0.026)高于MSAs阳性DM患者和MSAs(-)/MAAs(-)DM患者。MSAs阴性DM患者中快速进展性间质性肺病(RP-ILD)的比例低于MSAs阳性组。MSAs(-)/MAAs(+)组出现机化性肺炎和普通间质性肺炎的比例较高(P=0.011),支气管肺泡灌洗液中嗜酸性粒细胞升高(P=0.008)。MSAs阴性组的淋巴细胞计数(P=0.001)和CD16CD56自然杀伤(NK)细胞计数(P=0.012)更高。此外,CD4+TNFα+的百分比(P=0.040),CD4+IFNγ+(P=0.037),与MSA阳性DM患者相比,MSA阴性DM患者的总CD4+T细胞中CD4+IL-2+(P=0.018)细胞更高。此外,MSA阴性患者比MSA阳性患者预后更好。多元回归分析确定了发病年龄,较高水平的癌胚抗原(CEA),和RP-ILD是DM患者死亡的危险因素。
    结论:与MSAs阳性组相比,与MSAs阳性组相比,MSAs阴性DM患者的器官受累较少,并且预后更好。与MSAs阳性DM患者相比,MSAs阴性DM患者表现出明显的特征:•MSAs(-)/MAAs()DM患者表现出机化性肺炎(OP)和常规间质性肺炎(UIP)的患病率较高,支气管肺泡灌洗液中嗜酸性粒细胞计数升高。•与MSA阳性患者相比,MSA阴性患者的CEA水平较低。•在MSA阴性患者中鉴定出淋巴细胞和CD16+CD56+NK细胞计数升高。此外,CD4+TNFα+的比例,CD4+IFNγ+,与MSAs阳性DM患者相比,MSAs阴性DM患者的总CD4T细胞中的CD4IL-2细胞更高。•MSAs阴性DM患者比MSAs阳性DM患者具有更良好的预后。多元回归分析显示发病年龄晚,高CEA水平,RP-ILD是DM患者死亡的危险因素。
    OBJECTIVE: Myositis-specific antibodies (MSAs) and myositis-associated antibodies (MAAs) are associated with distinctive dermatomyositis (DM) clinical phenotypes. The aim of this study is to explicate the clinical and immunological features of MSAs-negative DM patients.
    METHODS: A total of 515 individuals diagnosed with DM was screened from 2013 to 2022 and 220 DM patients were enrolled in this retrospective cohort. Clinical and laboratory data of these patients were analyzed.
    RESULTS: MSAs-negative DM patients were categorized into two groups: MAAs-negative (MSAs (-)/MAAs (-)) group and MAAs-positive (MSAs (-)/MAAs (+)) group. The percentage of Raynaud\'s phenomenon (P=0.026) was higher in the MSAs (-)/MAAs (+) DM patients than the MSAs-positive DM patients and MSAs (-)/MAAs (-) DM patients. The proportion of rapidly progressive interstitial lung disease (RP-ILD) in the MSAs-negative DM patients was lower than that in the MSAs-positive group. The MSAs (-)/MAAs (+) group had a higher proportion of organizing pneumonia and usual interstitial pneumonia (P=0.011), and elevated eosinophils in their bronchoalveolar lavage fluid (P=0.008). Counts of lymphocytes (P=0.001) and CD16+CD56+ natural killer (NK) cells (P=0.012) were higher in the MSAs-negative group. Additionally, the percentage of CD4+TNFα+ (P=0.040), CD4+IFNγ+ (P=0.037), and CD4+IL-2+ (P=0.018) cells among total CD4+ T cells were higher in the MSA-negative DM patients compared with the MSAs-positive DM patients. Besides, MSAs-negative patients demonstrated a more favorable prognosis than MSAs-positive patients. Multivariable regression analysis identified advanced onset age, higher level of carcinoembryonic antigen (CEA), and RP-ILD as risk factors for mortality in DM patients.
    CONCLUSIONS: Compared with MSAs-positive group, MSAs-negative DM patients suffered less from organ involvement compared with MSAs-positive group and tend to have better prognosis. Key Points MSAs-negative DM patients exhibited distinct characteristics in comparison with MSAs-positive DM patients:   • The MSAs (-)/MAAs (+) DM patients demonstrated a higher prevalence of organizing pneumonia (OP) and usual interstitial pneumonia (UIP), and elevated eosinophil counts in bronchoalveolar lavage fluid.   • CEA levels were lower in MSAs-negative patients compared with MSAs-positive patients.   • Elevated counts of lymphocytes and CD16+CD56+ NK cells were identified in the MSAs-negative patients. Additionally, proportions of CD4+TNFα+, CD4+IFNγ+, and CD4+IL-2+ cells among total CD4+ T cells were higher in the MSAs-negative DM patients compared with DM MSAs-positive DM patients.   • MSAs-negative DM patients had a more favorable prognosis than MSAs-positive DM patients. A multivariable regression analysis revealed the advanced onset age, high CEA levels, and RP-ILD were risk factors for mortality in DM patients.
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  • 文章类型: Case Reports
    原发性肝鳞状细胞癌(SCC)极为罕见,迄今为止只报告了几十例。在组织病理学检查之前几乎不能诊断出来,这就需要排除转移性肿瘤。在这种情况下,我们介绍一名60岁女性患者,无合并症.由于实验室检查和影像学检查不能诊断,在超声(US)引导下进行肝活检,最终发现肝SCC的明确病理诊断。排除转移后,原发性肝SCC的诊断已确立,然后进行化疗和免疫治疗。此外,使用PubMed对原发性肝SCC进行了全面的文献检索,谷歌学者,和WebofScience,共检索到53篇文章,时间范围为1972年至2022年。然后进行了关键分析,以评估以前的文献,重点是临床特征,成像特征,治疗,和预后。
    Primary hepatic squamous cell carcinoma (SCC) is extremely rare, and only a few dozen cases have been reported to date. It can barely be diagnosed before histopathological examination, which necessitates the exclusion of metastatic tumors. In this case, we present a 60-year-old female patient with no comorbidity. As laboratory tests and imaging examinations were not diagnostic, ultrasonography (US)-guided liver biopsy was performed and eventually revealed a definitive pathological diagnosis of hepatic SCC. After excluding metastasis, the diagnosis of primary hepatic SCC was established, and then chemotherapy and immunotherapy were performed. Additionally, a comprehensive literature search was conducted on primary hepatic SCC using PubMed, Google Scholar, and Web of Science, and a total of 53 articles were retrieved with a time range from 1972 to 2022. A critical analysis was then performed to evaluate previous literature focusing on the clinical characteristics, imaging features, treatments, and prognosis.
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  • 文章类型: Journal Article
    背景:狼疮肠系膜血管炎(LMV)作为初始表现很少见,尤其是儿童期发病的系统性红斑狼疮(cSLE)。这是狼疮的严重并发症。目前,以LMV为首发表现的cSLE研究较少。本研究的目的是分析中国人群中cSLE伴LMV的临床特征和预后。与非LMVcSLE相比。
    方法:对2018年7月至2021年7月的55例cSLE患者进行了回顾性病例对照研究。临床数据,实验室发现,成像,治疗,收集并比较两组cSLE合并LMV和非LMV的随访数据。非LMVcSLE患者根据LMV患者的年龄和性别进行匹配。
    结果:共纳入11例有LMV的cSLE患者作为LMV组,44例无LMV的cSLE患者作为非LMV组。平均发病年龄为12.55±1.57岁,男女比例为2:9,在LMV组中观察到高疾病活动性.腹痛在LMV中最为常见。与非LMV相比,腹痛的百分比,呕吐,腹胀,腹泻更高,和胃肠道,浆膜腔,肾,LMV组肺损害较高(P<0.05)。在腹部增强CT中,肠壁增厚的百分比,腹腔积液,肠系膜血管增强,肾积水伴输尿管扩张,肠充血,LMV组胃黏膜增厚高于非LMV组(P<0.05)。LMV中接受甲基强的松龙脉冲联合环磷酰胺脉冲治疗的百分比高于非LMV。临床症状迅速消失,LMV组无死亡病例。与非LMV组相比,24小时尿蛋白较高,补体C3较低,LMV组疾病活动度较高(P<0.05)。
    结论:LMV多见于12~13岁的cSLE患者。腹痛是最常见的,也更容易对肾脏造成损害,浆膜腔,与LMV的cSLE和肺。甲基强的松龙脉冲联合CTX脉冲治疗有效。经过上述治疗,CSLE伴LMV预后良好,但总体恢复情况比非LMV患者差。
    Lupus mesenteric vasculitis (LMV) as initial presentation is rare, especially in childhood-onset systemic lupus erythematosus (cSLE). It is a critical complication of lupus. At present, the research on cSLE with LMV as the initial presentation is few. The aim of this study was to analyze the clinical characteristics and prognosis of cSLE with LMV in the Chinese population, compared with non-LMV cSLE.
    A retrospective case-controlled study was conducted on 55 cSLE patients between July 2018 and July 2021. The clinical data, laboratory findings, imaging, treatment, and follow-up data were collected and compared between the two groups of cSLE with LMV and non-LMV. Non-LMV cSLE patients were matched according to the age and sex of LMV patients.
    A total of 11 cSLE patients with LMV as the LMV group and 44 cSLE patients without LMV as the non-LMV group were included. The average age of onset was 12.55 ± 1.57 years old, the male-to-female ratio was 2:9, and high disease activity was observed in the LMV group. Abdominal pain was most common in LMV. Compared with the non-LMV, the percentage of abdominal pain, vomiting, abdominal distension, and diarrhea was higher, and gastrointestinal tract, serous cavity, kidney, and lung damage were higher in the LMV group (P < 0.05). In abdominal-enhanced CT, the percentage of intestinal wall thickening, peritoneal effusion, mesenteric vascular enhancement, hydronephrosis with ureteral dilatation, intestinal congestion, and gastric mucosa thickening in the LMV group were higher than those in the non-LMV group (P < 0.05). The percentage of receiving methylprednisolone pulse combined with cyclophosphamide pulse therapy in LMV was higher than in non-LMV. The clinical symptoms disappeared quickly, and there were no deaths in the LMV group. Compared with the non-LMV group, the 24-h urinary protein was higher, the complement C3 was lower, and the disease activity was higher in the LMV group (P < 0.05).
    LMV often occurs in 12 ~ 13-year-old girls with high disease activity of cSLE. Abdominal pain is the most common and more susceptible to damage to the kidney, serous cavity, and lung in cSLE with LMV. Methylprednisolone pulse combined with CTX pulse therapy is effective. After the treatment above, cSLE with LMV has a good prognosis, but the overall recovery is worse than non-LMV patients.
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