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Efficacy of disease-modifying antirheumatic drugs in primary Sjögren's syndrome-related interstitial lung disease 改变病情抗风湿药物对原发性斯约格伦综合征相关间质性肺病的疗效
Pub Date : 2024-11-20 DOI: 10.1016/j.medcle.2024.06.007
Funda Erbasan , Tahir Saygın Öğüt , Melis Dilbil , Mine Nokay , Mustafa Ender Terzioğlu , Veli Yazısız

Objectives

To evaluate the treatment modalities and their effects in primary Sjögren's syndrome (pSS) patients with interstitial lung disease (ILD).

Methods

In this chart review study, patients diagnosed with pSS-related ILD (pSS-ILD) between January 2004 and August 2022 were screened. Glucocorticoid use and administered disease-modifying antirheumatic drugs (DMARDs) were determined. The difference between forced vital capacity (FVC) and diffusion capacity of the lungs for carbon monoxide (DLCO) before and after treatment was evaluated.

Results

ILD was present in 44 of 609 patients (7.2%) diagnosed with pSS. In 27 patients included in the study, steroid usage was 81.5%. There was a statistically insignificant increase in FVC% (from 80.20 ± 22.1 to 81.6 ± 23.0) and a decrease in DLCO% (53.7 ± 15.3–52.2 ± 19.3) with DMARD treatment (p = 0.434 and p = 0.652, respectively). There was no significant difference between the treatment groups (azathioprine [AZA], mycophenolate mofetil [MMF], and rituximab [RTX]) in terms of the change in FVC% and DLCO% compared with baseline levels. The effect of treatment on FVC and DLCO was similar in UIP and NSIP patterns.

Conclusions

AZA, MMF, and RTX have similar effects on pulmonary functions in pSS-ILD and provide disease stabilization.
目的 评估原发性斯约格伦综合征(pSS)间质性肺病(ILD)患者的治疗方法及其效果。方法 在这项病历回顾研究中,筛选了 2004 年 1 月至 2022 年 8 月期间诊断为 pSS 相关 ILD(pSS-ILD)的患者。研究确定了糖皮质激素的使用情况和所服用的改善病情抗风湿药物(DMARDs)。评估了治疗前后用力肺活量(FVC)和一氧化碳肺弥散容量(DLCO)之间的差异。在609名确诊为pSS的患者中,有44人(7.2%)存在结果ILD。在纳入研究的 27 名患者中,类固醇使用率为 81.5%。DMARD治疗后,FVC%增加(从80.20 ± 22.1增至81.6 ± 23.0),DLCO%下降(53.7 ± 15.3-52.2 ± 19.3),差异无统计学意义(分别为p = 0.434和p = 0.652)。与基线水平相比,各治疗组(硫唑嘌呤[AZA]、霉酚酸酯[MMF]和利妥昔单抗[RTX])的 FVC% 和 DLCO% 变化无明显差异。结论AZA、MMF和RTX对pSS-ILD患者的肺功能具有相似的效果,并能稳定病情。
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引用次数: 0
Recurrent Guillain Barré 复发性吉兰巴雷
Pub Date : 2024-11-20 DOI: 10.1016/j.medcle.2024.06.008
Carlos Piquero Fernández, Carolina Saenz Lafourcada, Ana Pinel González
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引用次数: 0
Segmental arterial mediolysis mimicking polyarteritis nodosa 模仿结节性多动脉炎的节段性动脉内膜溶解症
Pub Date : 2024-11-20 DOI: 10.1016/j.medcle.2024.05.023
Cristina Calomarde-Gómez , Andrés García-Gamez , Ivette Casafont-Solé
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引用次数: 0
Utility of 18F-FDG PET/CT in the diagnosis and follow-up of a patient with eosinophilic granulomatosis with polyangiitis and large vessel vasculitis 18F-FDG PET/CT 在诊断和随访一名嗜酸性粒细胞肉芽肿伴多血管炎和大血管炎患者中的作用
Pub Date : 2024-11-20 DOI: 10.1016/j.medcle.2024.05.022
Montserrat Negre Busó , Gemma Alvarez Martínez , Antoni Rubió Rodríguez
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引用次数: 0
Deactivation of cardiac implantable electronic devices in palliative patients: When and how 停用姑息治疗患者的心脏植入式电子装置:何时以及如何
Pub Date : 2024-11-20 DOI: 10.1016/j.medcle.2024.04.032
Tatiana Oliveira, Nuno Ferreira Monteiro, Patrícia Cipriano
Cardiac implantable electronic devices have transformed medicine as they improve quality of life and prevent premature death. In palliative care settings, deactivation of these devices must be discussed, particularly at end-of-life. In terminally ill patients it is consensual to recommend implantable cardioverter defibrillator deactivation once shocks are frequent and painful. Concerning pacemakers, the decision to deactivate is controversial and it usually is not an option at patients’ end-of-life, since in pacing-dependent patients, such low heart rates might induce symptoms of bradycardia, with no impact on survival. Regarding cardiac resynchronization therapy, deactivation is not recommended as it can worsen symptoms. Left ventricular assistance device deactivation at end-of-life is a well-accepted practice, since it has the benefit of ending the physical burden associated with the device. Advance care planning should be encouraged and patients should be informed that deactivation is possible.
心脏植入式电子设备改善了生活质量并防止了过早死亡,从而改变了医学。在姑息治疗环境中,必须讨论停用这些设备的问题,尤其是在生命末期。对于临终病人,一旦电击频繁且疼痛难忍,建议停用植入式心律转复除颤器是一致同意的。关于心脏起搏器,停用的决定存在争议,通常在患者临终时不会选择停用,因为对起搏依赖的患者来说,这种低心率可能会诱发心动过缓症状,但对生存没有影响。关于心脏再同步化治疗,不建议停用,因为这会加重症状。在生命末期停用左心室辅助装置是一种广为接受的做法,因为这样做的好处是可以结束与该装置相关的身体负担。应鼓励预先制定护理计划,并告知患者可以停用该装置。
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引用次数: 0
Risk stratification for CNS infection: A potential tool to avoid unwarranted lumbar punctures – An observational study 中枢神经系统感染的风险分层:避免不必要腰椎穿刺的潜在工具 - 一项观察性研究
Pub Date : 2024-11-20 DOI: 10.1016/j.medcle.2024.07.009
Cristina Kirkegaard-Biosca , Carmen Moreno-Blas , Marta Lluch-Álvarez , Anna Falcó-Roget , Paula Salmerón , Clara Ramírez-Serra , Júlia Sellarès-Nadal , Joaquín Burgos , Núria Fernández-Hidalgo

Objective

Central nervous system (CNS) infection poses a diagnostic challenge especially in elderly patients who frequently exhibit atypical symptoms. Our study aimed to identify patients with a low risk of CNS infection, in whom lumbar puncture (LP) could be avoided.

Methods

Observational study of consecutive adult patients who underwent a LP in the emergency room (ER) of Hospital Universitari Vall d’Hebron between January 2017 and December 2021. We performed a univariate and multivariate analysis to identify factors associated with non-CNS infection. These factors were used to create a combined variable, and its diagnostic positive predictive value and specificity to detect patients without CNS infections were calculated.

Results

We included 489 patients of which 77 (15.7%) were diagnosed with CNS infection. Median age was 62 years (IQR 41–78) and 240 (49.1%) were male. In the multivariate analysis, variables associated with non-CNS infection were female sex (OR 1.89; 95% CI 1.12–3.20), age older than 80 years (OR 3.14; 95% CI 1.20–8.19), previous cognitive impairment (OR 3.91; 95% CI 1.18–13.01), and clinical presentation without meningitis triad (fever, headache and neck stiffness) (OR 4.12; 95% CI 1.72–9.85). A composite variable encompassing age older than 80, cognitive impairment, and the absence of the meningitis triad was used as a diagnostic tool to identify patients with non-CNS infection, exhibiting a 98% positive predictive value and 99% specificity.

Conclusions

This study identifies factors associated with a low risk of CNS infection. Thus, a more precise clinical approach could help clinicians to detect patients who would not benefit from a LP.
目的中枢神经系统(CNS)感染是一项诊断难题,尤其是对于经常表现出非典型症状的老年患者。我们的研究旨在确定中枢神经系统感染风险较低的患者,避免对其进行腰椎穿刺(LP)。方法对 2017 年 1 月至 2021 年 12 月期间在瓦尔德希伯伦大学医院急诊室(ER)接受腰椎穿刺的连续成年患者进行观察研究。我们进行了单变量和多变量分析,以确定与非中枢神经系统感染相关的因素。这些因素被用来创建一个综合变量,并计算其诊断阳性预测值和特异性,以检测无中枢神经系统感染的患者。结果我们纳入了489名患者,其中77人(15.7%)被诊断为中枢神经系统感染。中位年龄为 62 岁(IQR 41-78),男性 240 人(49.1%)。在多变量分析中,与非中枢神经系统感染相关的变量有女性(OR 1.89;95% CI 1.12-3.20)、年龄大于 80 岁(OR 3.14;95% CI 1.20-8.19)、既往认知障碍(OR 3.91;95% CI 1.18-13.01)和临床表现无脑膜炎三联征(发热、头痛和颈部僵硬)(OR 4.12;95% CI 1.72-9.85)。这项研究确定了与中枢神经系统感染低风险相关的因素。因此,更精确的临床方法可以帮助临床医生发现那些不会从 LP 中获益的患者。
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引用次数: 0
Pulmonary arterial hypertension associated with systemic lupus erythematous: a case series 与系统性红斑狼疮相关的肺动脉高压:一个病例系列
Pub Date : 2024-11-15 DOI: 10.1016/j.medcle.2024.05.013
Rossio Gardenia Ortuño Lobo, Marina Garcia Carrasco, Gustavo Medina
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引用次数: 0
Temporal variability of Lp(a) in clinically stable patients: Implications for cardiovascular risk assessment 临床病情稳定患者脂蛋白(a)的时间变化:对心血管风险评估的影响
Pub Date : 2024-11-15 DOI: 10.1016/j.medcle.2024.05.018
Maria G. Matta , Laura Schreier , Augusto Lavalle-Cobo , Sebastian Garcia-Zamora , Agustina Ferraresi , Angeles Madsen , Sofia Bellini , Guadalupe Ramos , Paula Roubicek , Pablo Corral

Objectives

Lipoprotein(a) [Lp(a)] is a significant risk factor for cardiovascular disease, yet it is often overlooked in routine clinical assessments. As a primarily genetically determined risk factor, the traditional recommendation is to assess its level once in a lifetime, as the variability of Lp(a) over time is considered to be minimal. This study aims to evaluate the potential variability of Lp(a) in clinically stable patients and investigate factors contributing to the lack of stable levels.

Methods

A retrospective analysis was conducted on a sample of adult patients attending a lipid clinic. Participants with at least two Lp(a) measurements taken with a minimum interval of four months were included. Lp(a) measurements were performed using the immunoturbidimetric assay. Variability in Lp(a) values was calculated as a percentage change from baseline, with participants exceeding a 25% change classified as having hypervariable Lp(a) levels. Additional clinical and biochemical variables were assessed.

Results

61 participants with 171 Lp(a) determinations were included. Thirty-four percent exhibited a variability of 25% or higher (hypervariable). Men showed slightly greater variability than women. Changes in Lp(a) categories were observed among hypervariable patients, with some participants experiencing an increase while others showed a decrease. Menopause was present in all the women with hypervariable levels.

Conclusion

Our study suggests reconsidering the reliance on a single Lp(a) measurement for assessing cardiovascular risk. Repeat measurements, particularly in borderline cases, may be beneficial.
目的脂蛋白(a)[Lp(a)]是心血管疾病的重要危险因素,但在常规临床评估中却经常被忽视。由于脂蛋白(a)主要是由基因决定的风险因素,传统的建议是一生评估一次其水平,因为脂蛋白(a)随时间的变化被认为是最小的。本研究旨在评估临床稳定期患者脂蛋白(a)的潜在变异性,并调查导致脂蛋白(a)水平不稳定的因素。参与者至少进行过两次脂蛋白(a)测量,测量间隔至少为四个月。脂蛋白(a)测量采用免疫比浊法进行。脂蛋白(a)值的变异性按与基线相比的变化百分比计算,参与者的脂蛋白(a)水平变化超过 25% 即为变异性过高。此外,还对其他临床和生化变量进行了评估。结果61名参与者共进行了171次脂蛋白(a)测定。34%的人的变异率达到或超过 25%(高变异)。男性的变异性略高于女性。在高变异患者中观察到脂蛋白(a)类别的变化,一些参与者的脂蛋白(a)类别有所增加,而另一些参与者的脂蛋白(a)类别则有所减少。结论:我们的研究建议重新考虑依赖单次脂蛋白(a)测量来评估心血管风险的做法。重复测量,尤其是对边缘病例进行重复测量,可能会有所裨益。
{"title":"Temporal variability of Lp(a) in clinically stable patients: Implications for cardiovascular risk assessment","authors":"Maria G. Matta ,&nbsp;Laura Schreier ,&nbsp;Augusto Lavalle-Cobo ,&nbsp;Sebastian Garcia-Zamora ,&nbsp;Agustina Ferraresi ,&nbsp;Angeles Madsen ,&nbsp;Sofia Bellini ,&nbsp;Guadalupe Ramos ,&nbsp;Paula Roubicek ,&nbsp;Pablo Corral","doi":"10.1016/j.medcle.2024.05.018","DOIUrl":"10.1016/j.medcle.2024.05.018","url":null,"abstract":"<div><h3>Objectives</h3><div>Lipoprotein(a) [Lp(a)] is a significant risk factor for cardiovascular disease, yet it is often overlooked in routine clinical assessments. As a primarily genetically determined risk factor, the traditional recommendation is to assess its level once in a lifetime, as the variability of Lp(a) over time is considered to be minimal. This study aims to evaluate the potential variability of Lp(a) in clinically stable patients and investigate factors contributing to the lack of stable levels.</div></div><div><h3>Methods</h3><div>A retrospective analysis was conducted on a sample of adult patients attending a lipid clinic. Participants with at least two Lp(a) measurements taken with a minimum interval of four months were included. Lp(a) measurements were performed using the immunoturbidimetric assay. Variability in Lp(a) values was calculated as a percentage change from baseline, with participants exceeding a 25% change classified as having hypervariable Lp(a) levels. Additional clinical and biochemical variables were assessed.</div></div><div><h3>Results</h3><div>61 participants with 171 Lp(a) determinations were included. Thirty-four percent exhibited a variability of 25% or higher (hypervariable). Men showed slightly greater variability than women. Changes in Lp(a) categories were observed among hypervariable patients, with some participants experiencing an increase while others showed a decrease. Menopause was present in all the women with hypervariable levels.</div></div><div><h3>Conclusion</h3><div>Our study suggests reconsidering the reliance on a single Lp(a) measurement for assessing cardiovascular risk. Repeat measurements, particularly in borderline cases, may be beneficial.</div></div>","PeriodicalId":74154,"journal":{"name":"Medicina clinica (English ed.)","volume":"163 9","pages":"Pages 436-441"},"PeriodicalIF":0.0,"publicationDate":"2024-11-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142656084","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Parkinsonism in liver diseases or dysfunction 肝脏疾病或功能障碍导致的帕金森症
Pub Date : 2024-11-15 DOI: 10.1016/j.medcle.2024.04.031
Sichen Li, Yuxia Zhua, Xi Liu
Parkinsonism in liver diseases or dysfunction, mainly including neurological manifestations in hereditary liver diseases and neurological complications of advanced liver diseases, occur in isolation or in combination with other movement disorders, and progress along disease course. Prominent akinetic-rigidity syndrome, various onset and progression, poor levodopa response and metabolism abnormalities reflected by serum biomarkers and neuroimaging, make this atypical parkinsonism recognizable and notable in clinical practice. Different susceptibility of brain areas, especially in basal ganglia, to manganese, iron, copper, ammonia overload, together with subsequent oxidative stress, neurotransmitter alterations, disturbed glia-neuron homeostasis and eventually neurotoxicity, contribute to parkinsonism under the circumstances of insufficient liver clearance ability. These mechanisms are interrelated and may interact collectively, adding to the complexity of clinical manifestations and treatment responses. This review summarizes shared clinical features of parkinsonism in liver diseases or dysfunction, depicts their underlying mechanisms and suggests practical flowchart for differential diagnosis.
肝病或肝功能异常引起的帕金森病,主要包括遗传性肝病的神经系统表现和晚期肝病的神经系统并发症,可单独发生,也可与其他运动障碍合并发生,并随病程进展。突出的运动-僵直综合征、不同的起病和进展、左旋多巴反应差以及血清生物标志物和神经影像学反映的代谢异常,使这种非典型帕金森病在临床实践中具有可识别性和显著性。在肝脏清除能力不足的情况下,脑区(尤其是基底神经节)对锰、铁、铜、氨超载的敏感性不同,再加上随后的氧化应激、神经递质改变、胶质神经元平衡紊乱以及最终的神经毒性,都会导致帕金森病。这些机制相互关联,可能共同作用,增加了临床表现和治疗反应的复杂性。本综述总结了肝脏疾病或功能障碍导致帕金森病的共同临床特征,描述了其潜在机制,并提出了实用的鉴别诊断流程图。
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引用次数: 0
Paraneoplastic hyperthyroidism secondary to chorionic gonadotrophin-producing testicular tumor 继发于绒毛膜促性腺激素分泌性睾丸肿瘤的副肿瘤性甲状腺功能亢进症
Pub Date : 2024-11-15 DOI: 10.1016/j.medcle.2024.04.029
Fernando Guerrero-Pérez , María Pérez Prieto , Nuria Vilarrasa
{"title":"Paraneoplastic hyperthyroidism secondary to chorionic gonadotrophin-producing testicular tumor","authors":"Fernando Guerrero-Pérez ,&nbsp;María Pérez Prieto ,&nbsp;Nuria Vilarrasa","doi":"10.1016/j.medcle.2024.04.029","DOIUrl":"10.1016/j.medcle.2024.04.029","url":null,"abstract":"","PeriodicalId":74154,"journal":{"name":"Medicina clinica (English ed.)","volume":"163 9","pages":"Pages 476-477"},"PeriodicalIF":0.0,"publicationDate":"2024-11-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142656077","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
期刊
Medicina clinica (English ed.)
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