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Stiff Person Syndrome: A Rare Presentation of a Rare Disorder 僵硬人综合症:一种罕见疾病的罕见表现
Pub Date : 2023-08-14 DOI: 10.53785/2769-2779.1162
Muhammad H. Sharif, B. G. Verghese
Abstract Stiff Person Syndrome (SPS) is a rare autoimmune disease that is caused by the lack of inhibition to excitatory neurotransmitters in the central nervous system (CNS) which then leads to inappropriate and excessive motor unit firing causing stiffness, a characteristic feature of the disease. SPS has an incidence of one case in a million and occurs in the middle-aged population with a female predominance. SPS mostly occurs in the background of autoimmune disorders like type 1 diabetes, thyroid disorders, pernicious anemia, and less often, vitiligo.. The pathophysiology is not completely understood; however, there is a strong correlation between high titers of anti-glutamic acid decarboxylase antibody (anti-GAD Ab) and the disease. We present an 82 years old man who complained of stiffness and weakness, mostly on the right side, with multiple negative workups. He was then eventually diagnosed with SPS based on the characteristic history and physical examination findings and being positive for anti-GAD Ab. He was treated with a combination of baclofen, gabapentin, intravenous immunoglobulins (IVIG), and physical therapy. We review the case presentation which was unusual in terms of age and sex, and treatment options in the context of a severe presentation of this disabling disease.
僵直人综合征(SPS)是一种罕见的自身免疫性疾病,是由于中枢神经系统(CNS)缺乏对兴奋性神经递质的抑制,从而导致不适当和过度的运动单元放电导致僵硬,这是该疾病的一个特征。SPS的发病率为百万分之一,发生在中年人群中,以女性为主。SPS主要发生在自身免疫性疾病的背景下,如1型糖尿病、甲状腺疾病、恶性贫血,以及较少出现的白癜风。病理生理学尚不完全清楚;然而,高滴度的抗谷氨酸脱羧酶抗体(抗gad Ab)与疾病有很强的相关性。我们报告一位82岁的男性,主诉僵硬和虚弱,主要在右侧,多次阴性检查。根据特征性病史和体格检查结果,最终诊断为SPS,抗广域网域网广域网域网抗体阳性。患者接受巴氯芬、加巴喷丁、静脉注射免疫球蛋白(IVIG)和物理治疗联合治疗。我们回顾的情况下,这是不寻常的年龄和性别方面的表现,并在严重的情况下,这种致残疾病的治疗方案。
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引用次数: 0
Pseudo-Wellens syndrome: A rare entity associated with cocaine use 假性韦伦斯综合征:一种与可卡因使用有关的罕见症状
Pub Date : 2023-08-14 DOI: 10.53785/2769-2779.1164
Mohamed Salah Mohamed, Syed Hamza Waheed, Amir Mahmoud, Anas Hashem, Bipul Baibhav, Abdullah M Firoze Ahmed
Wellens syndrome usually indicates critical left anterior descending artery (LAD) occlusion. Pseudo-Wellens syndrome consists of criteria of Wellens syndrome in the absence of critical LAD occlusion. We report a case of Pseudo-Wellens syndrome related to cocaine use. A 52-year-old male with a medical history of hypertension and diabetes, presented with acute retrosternal chest pain of 3 days duration. Physical examination was unremarkable. EKG on presentation showed deep T-wave inversions in leads V2 to V5. Highly sensitive troponin was elevated. The patient admitted to using cocaine daily for the past two months. Due to concerns for Wellens syndrome, the patient had an immediate coronary angiography which revealed mild disease of the LAD (< 30%) only. Inpatient echocardiogram revealed preserved left ventricular ejection fraction and no segmental wall motion abnormalities. Subsequent EKG at the cardiology clinic showed improvement in T-wave inversion. The patient was advised to abstain from using cocaine. As Pseudo-Wellens syndrome is a diagnosis of exclusion, patients with a history of recent cocaine use presenting with acute chest pain history, evidence of myocardial injury, and EKG findings suggestive of Wellens syndrome should undergo an emergent coronary angiogram to exclude critical LAD occlusion.
Wellens综合征通常提示严重左前降支闭塞。假性韦伦斯综合征由韦伦斯综合征的标准组成,没有严重的LAD闭塞。我们报告一例假性韦伦斯综合征与可卡因的使用。52岁男性,有高血压和糖尿病病史,急性胸骨后胸痛3天。体格检查无明显异常。心电图显示V2至V5导联深t波反转。高度敏感的肌钙蛋白升高。病人承认在过去的两个月里每天都吸食可卡因。由于对Wellens综合征的担忧,患者立即进行了冠状动脉造影,仅发现LAD轻度病变(< 30%)。住院超声心动图显示左心室射血分数保留,无节段性壁运动异常。随后的心脏科门诊心电图显示t波倒置改善。病人被建议戒除可卡因。假性韦伦斯综合征是一种排除性诊断,近期有可卡因使用史且有急性胸痛史、心肌损伤证据、心电图提示韦伦斯综合征的患者应进行紧急冠状动脉造影,以排除严重的LAD闭塞。
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引用次数: 0
Quality Improvement Project to increase pneumococcal vaccination rates in adults with inflammatory bowel diseases (IBD) being treated with immunosuppressants. 提高接受免疫抑制剂治疗的炎症性肠病(IBD)成人肺炎球菌疫苗接种率的质量改进项目。
Pub Date : 2023-06-01 DOI: 10.53785/2769-2779.1141
L. Naing, Sheza Malik, Jay Bapaye, Nagesh Jadhav
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引用次数: 0
Radiologic and Histologic Findings of Locally Advanced Mucoepidermoid Carcinoma Managed with Total Surgical Excision 局部晚期黏液表皮样癌全手术切除的放射学和组织学表现
Pub Date : 2023-06-01 DOI: 10.53785/2769-2779.1155
Christopher R Huber, Don Truong, Joseph Hatem, Joel Thompson, Bryan Scott
Abstract The present case report provides images from computed tomography and histology of a patient with a salivary gland neoplasm consistent with mucoepidermoid carcinoma.
摘要:本病例报告提供了一个涎腺肿瘤与粘液表皮样癌一致的计算机断层扫描和组织学图像。
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引用次数: 0
Pedagogy of Problems 问题教学法
Pub Date : 2023-06-01 DOI: 10.53785/2769-2779.1158
Ryan Babineau
N owadays, medical students are learning in a myriad of ways e some more traditional than others. For example, some students elect to attend class in person, while others take lectures online at 2x speed. While I am one of the only students in my class who still uses the analog pencil and paper, my iPad-laden counterparts and I converge for sessions involving group problem solving. Most American medical schools employ some variation of this idea in their curriculum, and common to academia, institutions often have a proprietary name for their version of it. During a problem-based learning (PBL) session, students are tasked with completing a case-based discussion with their team using pre-existing knowledge in their small group with the help of a preceptor. A recent systematic review of PBL pedagogy by Trull as et al. even asserted that one of the main benefits, in addition to learning objectives, is that this design promotes the development of other professional competencies required of healthcare professionals. In the four meta-analyses included in their review, objective metrics of knowledge acquisition from PBL sessions appear as good or better than traditional methods, but integrating self-learning with communication, social, and problem-solving skills could pay dividends later. Also of note, Trull as found that student satisfaction tended to be higher for PBL sessions compared to lecture-based classes e and I agree. Interestingly, PBL's superiority is unclear when compared to simulation-based activities, where learners apply clinical judgement and skills. Simulations can be even more resource-intensive than PBL methods. My school often uses team-based learning (TBL) sessions, where we similarly engage in solving a problem, now using the information we were assigned to learn beforehand. Unlike a traditional lecture, the learning is done prior to class time, and Q6 during each session, teams are working on applying the new information to a tricky patient vignette (our recent session on SLE was a doozie). Typical to TBL, students complete individual and group evaluations before entering the case time, with peer evaluations at the end (Burgess). These may have an impact on the degree of preparation students arrive with, which is key for getting the most out of the session. Actively engaging with the material is the crux of why these designs are proposed to work. When evaluating hypothetical patient cases, we are encouraged to use outside resources, including recent literature and diagnostic guidelines attempting to fill in gaps in our knowledge. Working with other students in a PBL or TBL session aids in preparing for times when learners will have to have similar discussions on the wards or when presenting a patient. These sessions on the calendar ensure that I take ownership of the material; if no other evaluation lies between myself and the test, it'd be much easier to learn it when I am comfortable, but this compels me to be ready and contribu
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引用次数: 0
Extrinsic Allergic Alveolitis: A Systematic Review of HLA-DR in Pigeon Breeder’s Disease 外源性过敏性肺泡炎:HLA-DR在鸽子饲养者病中的系统评价
Pub Date : 2023-06-01 DOI: 10.53785/2769-2779.1150
Dylan Thibaut, Ryan A Witcher, Anitha Kunnath, James Toldi
Abstract Abstract Abstract IntroductionIntroduction: Pigeon Breeder’s Pneumonitis (PBP) results due to a complex pathophysiology that includes exposure to avian antigens. Susceptibility has been linked to human leukocyte antigen (HLA) class II, though consensus has not been reached. The goal of this systematic review is to further elucidate the association between PBP and HLA-DR subtypes. MethodsMethods: Databases utilized included PubMed, Google Scholar, ScienceDirect, and Cochrane Library. Inclusion required a minimum of three studies in English presenting HLA-DR alleles of PBP and control subgroups. Exclusion was due to insufficient data or non-feasible control groups. Forest plots were created for HLA-DR subtypes’ association with PBP. The NIH Bias assessment tool and LFK index assessed bias. ResultsResults: 4 studies were included in the meta-analysis. HLA-DR3 was associated with the pooled PBP subgroups (OR=1.86 [1.13, 3.05], p DiscussionDiscussion: Limitations included limited sources, with multiple study methodology unacceptable to this review. These findings expand on previous research on HLA variants and PBP frequency, and offer further clarity supporting novel approaches in treatment of PBP.
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引用次数: 0
Journal Club: Glymphatic System in Neurodegenerative Diseases 杂志俱乐部:神经退行性疾病中的淋巴系统
Pub Date : 2023-06-01 DOI: 10.53785/2769-2779.1156
Ronald G Lott, Xavier Zonna, Samuel Wlasowicz
Neurodegenerative diseases such as Alzheimer's Disease impact a multitude of individuals worldwide. The neural Glymphatic system is a relatively newly discovered cellular transport pathway within the brain that has been implicated in neurodegenerative diseases. This article serves to provide a journal club review of current literature on the Glymphatic system to raise awareness of this system and its potential for future treatment and prevention of neurodegenerative conditions.
神经退行性疾病,如阿尔茨海默病,影响着全世界许多人。神经淋巴系统是脑内一个相对较新发现的细胞运输途径,与神经退行性疾病有关。这篇文章提供了一个杂志俱乐部回顾当前的文献关于淋巴系统,以提高对该系统的认识,以及它在未来治疗和预防神经退行性疾病的潜力。
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引用次数: 0
Comparing Pleural Fluid Removal Volume and Oxygenation Change in Therapeutic Thoracentesis: Is There a Relationship? 比较胸腔穿刺治疗性胸腔积液排出量与氧合变化:是否有关系?
Pub Date : 2023-06-01 DOI: 10.53785/2769-2779.1149
Hannah E. Zazulak, Ryan Burke, Andrea M Bodine
Background: During therapeutic thoracentesis (TT), pleural fluid is typically drained to completion. Fluid removal improves oxygenation, and the amount of fluid removed is directly associated with the risk of serious complications. A relationship between the amount of fluid removed during TT and the change in oxygenation has not been found in the literature. A direct relationship was hypothesized in this study. Differences in the change in oxygenation between sexes, age, and pre-procedure oxygen support were secondarily hypothesized. This information would assist in the guidance of future TT protocols. Methods: Subjects of this retrospective cohort study were males and females aged 26-74 years. Of the 166 patients who underwent inpatient TT between February 4, 2020, and December 10, 2022, at Berkshire Medical Center (BMC), 16 met the inclusion criteria. They were identified using CPT codes in the electronic medical record (EMR). Demographics, amount of fluid removed, and type of oxygen support were recorded. Pre and post-TT arterial oxygen partial pressure (PaO2) and fraction of inspired oxygen (FiO2) values were collected to calculate the P/F, a metric for oxygenation status. T-test and correlation coefficient were calculated to analyze the change in the P/F ratio versus the amount of pleural fluid removed. Correlation coefficients or ANOVA were calculated to compare oxygenation changes to sex, age, and supplemental oxygen types. Results: There was no statistical significance (p=0.87) in the linear relationship between the amount of pleural fluid drained (mean=660 mL, range=150-1500 mL) and the change in oxygenation (mean=162, range=34-300). There were no statistically significant differences in oxygenation changes between sex (p=0.60), age (p=0.81), or types of oxygen support pre-procedure (p=0.07). There was a statistically significant difference in pre and post-procedure P/F ratio (p<0.001). Discussion: We found a statistically significant change in oxygenation before and after TT, with no evidence of a direct relationship between amount of fluid removed and improvement in oxygenation. There were no complications when removing up to 1500 mL of fluid. A protocol that halts pleural fluid drainage once it is complete or when the amount removed reaches 1500 mL, whichever occurs first, may be optimal.
背景:在治疗性胸腔穿刺术(TT)中,通常将胸腔积液排出。清除液体可以改善氧合,清除液体的数量与严重并发症的风险直接相关。在TT期间移出的液体量与氧合变化之间的关系尚未在文献中发现。本研究假设两者之间存在直接关系。其次假设了不同性别、年龄和术前氧支持之间氧合变化的差异。这些信息将有助于指导今后的TT协议。方法:回顾性队列研究对象为26-74岁的男性和女性。在2020年2月4日至2022年12月10日期间,在伯克希尔医疗中心(BMC)接受住院TT治疗的166例患者中,有16例符合纳入标准。他们使用电子病历(EMR)中的CPT代码进行识别。记录人口统计、移液量和供氧类型。收集tt前和tt后的动脉氧分压(PaO2)和吸入氧分数(FiO2)值,计算P/F,这是衡量氧合状态的指标。计算t检验和相关系数,分析P/F比随胸腔积液量的变化。计算相关系数或方差分析来比较氧合变化与性别、年龄和补充氧类型的关系。结果:胸水引流量(平均660 mL,范围150 ~ 1500 mL)与氧合变化(平均162 mL,范围34 ~ 300)的线性关系无统计学意义(p=0.87)。氧合变化在性别(p=0.60)、年龄(p=0.81)和术前氧支持类型(p=0.07)之间无统计学差异。手术前后P/F比差异有统计学意义(P <0.001)。讨论:我们发现TT前后氧合变化具有统计学意义,但没有证据表明移液量与氧合改善之间存在直接关系。当取出高达1500毫升的液体时,没有并发症。一旦胸腔积液完成或积液量达到1500ml,以先发生者为准,停止胸腔积液引流可能是最佳方案。
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引用次数: 0
Rare Case of Late-onset Myasthenia Gravis in a 65-year-old Female with Prolonged Doxycycline Use 65岁女性迟发性重症肌无力伴强力霉素长期使用的罕见病例
Pub Date : 2023-06-01 DOI: 10.53785/2769-2779.1139
Angel Wong, Kayvan Mirhadi
Myasthenia gravis (MG) is a rare autoimmune condition caused by antibody-mediated disruption of acetylcholine receptors (AChR) or their associated proteins. The age of onset of MG has a bimodal distribution, with a predominance of female cases in the second and third decades and a predominance of male cases in the sixth to eighth decades. MG is often unmasked by stressors such as systemic illness, medication, surgery, and pregnancy. We present a case of late-onset MG in a 65-year-old female with a recent history of prolonged doxycycline use. However, there are aspects of her past medical history, including comorbidities involving the immune system and numerous drug intolerances that should have increased clinical suspicion for MG. Additionally, one medical event that preceded symptom onset was pneumonia treated with doxycycline. This case suggests that doxycycline should be used with caution in MG patients, and that MG should be considered as part of the differential diagnosis in older female patients presenting with neurologic symptoms and suggestive past medical history.
重症肌无力(MG)是一种罕见的自身免疫性疾病,由抗体介导的乙酰胆碱受体(AChR)或其相关蛋白的破坏引起。MG发病年龄呈双峰分布,在第二、三十年以女性病例为主,在第六、八十年以男性病例为主。MG经常被压力源(如全身性疾病、药物、手术和怀孕)所掩盖。我们提出了一例迟发性MG在65岁的女性与长期使用强力霉素最近的历史。然而,她过去的一些病史,包括涉及免疫系统的合并症和许多药物不耐受,应该增加临床对MG的怀疑。此外,在症状出现之前的一个医学事件是用强力霉素治疗肺炎。本病例提示,MG患者应谨慎使用强力霉素,对于有神经系统症状和既往病史的老年女性患者,MG应作为鉴别诊断的一部分。
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引用次数: 0
Late-onset Isolated Myositis Following Transobturator Mid-Urethral Sling: A Case Report 经闭口中尿道悬吊术后迟发性孤立性肌炎1例
Pub Date : 2023-06-01 DOI: 10.53785/2769-2779.1151
Bruce Lee, Mohamad Mahmoud
Late onset of isolated myositis following a Transobturator (TOT) mid-urethral sling (MUS) is a rare post-operative complication.
经闭锁(TOT)中尿道悬吊(MUS)后迟发性孤立性肌炎是一种罕见的术后并发症。
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引用次数: 0
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Advances in Clinical Medical Research and Healthcare Delivery
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