Rambam Maimonides Medical Journal最新文献_第5页

Idiopathic Eosinophilic Vasculitis: Case Presentation and Literature Review. 特发性嗜酸性血管炎:病例报告及文献回顾。

IF 1.5

Rambam Maimonides Medical Journal Pub Date : 2023-04-30 DOI: 10.5041/RMMJ.10498

Alexandra Balbir-Gurman, Adi Broyde, Ori Elkayam

{"title":"Idiopathic Eosinophilic Vasculitis: Case Presentation and Literature Review.","authors":"Alexandra Balbir-Gurman, Adi Broyde, Ori Elkayam","doi":"10.5041/RMMJ.10498","DOIUrl":"https://doi.org/10.5041/RMMJ.10498","url":null,"abstract":"Objective: Idiopathic eosinophilic vasculitis has been described in previous case series as a possible manifestation of hypereosinophilic syndrome (HES) in asthma-free patients. A rare disease, it can be classified as an eosinophilic-rich, necrotizing, systemic form of vasculitis that affects vessels of various sizes in these patients. This report shares our experience with the treatment of a patient with eosinophilic vasculitis.Case presentation: We present the case of a 45-year-old man who suffered from idiopathic HES manifesting as digital ulcers and peripheral ischemia of both the upper and lower limbs without the involvement of other systems. Diagnosis was made after excluding the primary and secondary causes of eosinophilia. The patient responded well to both corticosteroids and mepolizumab, an interleukin-5 inhibitor, as a corticosteroid-sparing therapy.Conclusion: Our case of HES-associated vasculitis in an asthma-free patient supports previous reports describing this rare diagnosis of idiopathic eosinophilic vasculitis in recent years. We describe a good response to mepolizumab (interleukin-5 inhibitor) in our patient.","PeriodicalId":46408,"journal":{"name":"Rambam Maimonides Medical Journal","volume":"14 2","pages":""},"PeriodicalIF":1.5,"publicationDate":"2023-04-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10147401/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9366583","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Dual Biologic Therapy in Patients with Rheumatoid Arthritis and Psoriatic Arthritis. 类风湿性关节炎和银屑病关节炎的双重生物治疗。

IF 1.5

Rambam Maimonides Medical Journal Pub Date : 2023-04-30 DOI: 10.5041/RMMJ.10494

Victoria Furer, Ori Elkayam

{"title":"Dual Biologic Therapy in Patients with Rheumatoid Arthritis and Psoriatic Arthritis.","authors":"Victoria Furer, Ori Elkayam","doi":"10.5041/RMMJ.10494","DOIUrl":"https://doi.org/10.5041/RMMJ.10494","url":null,"abstract":"Treatment with biological agents has become standard of care in treatment of immune-mediated diseases (IMD), including rheumatoid arthritis and psoriatic arthritis. Yet, a significant proportion of patients experience loss of response to biologics, need treatment escalation, or develop side effects. During the past decade, new biologic agents with different targeted molecular pathways have been approved for treatment of IMD, introducing the possibility of concomitant dual biologic therapy. The role of dual biologic therapy targeting different inflammatory pathways has become an area of great interest in the field of IMD, addressing the unmet clinical need of patients with refractory diseases and treatment of comorbidities, such as osteoporosis, asthma, atopic dermatitis, and urticaria. Despite the increasing use of biologics as a dual therapy across different indications, there is a paucity of data concerning the safety of the simultaneous use of more than one biological agents. The purpose of this review is to summarize the current literature on the use of dual biologics in patients with rheumatoid arthritis and psoriatic arthritis, addressing the potential adverse effects associated with combination therapy, and highlighting future directions in the use of this novel therapeutic modality.","PeriodicalId":46408,"journal":{"name":"Rambam Maimonides Medical Journal","volume":"14 2","pages":""},"PeriodicalIF":1.5,"publicationDate":"2023-04-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10147400/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9366588","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 2

The Emerging Role of Mitochondrial Dysfunction in the Pathogenesis of Idiopathic Inflammatory Myopathies. 线粒体功能障碍在特发性炎性肌病发病机制中的新作用。

IF 1.5

Rambam Maimonides Medical Journal Pub Date : 2023-04-30 DOI: 10.5041/RMMJ.10493

Alexandra Balbir-Gurman, Jorge Armando Gonzalez-Chapa, Marina Barguil Macêdo, Christian Lood

{"title":"The Emerging Role of Mitochondrial Dysfunction in the Pathogenesis of Idiopathic Inflammatory Myopathies.","authors":"Alexandra Balbir-Gurman, Jorge Armando Gonzalez-Chapa, Marina Barguil Macêdo, Christian Lood","doi":"10.5041/RMMJ.10493","DOIUrl":"https://doi.org/10.5041/RMMJ.10493","url":null,"abstract":"Increasing evidence points towards mitochondria as crucial players in the initiation and progression of auto-immune and degenerative disorders, to which impaired cell metabolism is but a facet of the subjacent etiopathogenesis. This review aims to introduce the reader to essential concepts of mitochondrial abnormalities in idiopathic inflammatory myopathy (IIM), underscoring inclusion-body myositis and dermatomyositis. Far surpassing the initial simplistic view of being responsible for energy generation, mitochondria have gathered attention regarding their role in inflammatory processes, being able to fuel autoimmunity, as shown by the presence of anti-mitochondrial antibodies (AMAs) in up to 10% of IIM patients. As cellular respiration takes place, mitochondrial metabolites might help to shape the pro-inflammatory milieu in affected muscle, beyond generating reactive oxygen species, which are well-recognized inducers of damage-associated molecular patterns. A series of mitochondrial components might facilitate the sterile activation of pro-inflammatory cells and the production of several cytokines responsible for enhancing auto-immune responses. Marked variation in the mitochondrial genome has also been reported in IIM patients. As such, we summarize key historical and recent advances linking aberrations and instabilities of mitochondrial DNA to impaired muscle function. Besides discussing mitochondrial dysfunction as an essential part of IIM development, we also highlight possible associations between presence of AMAs and a particular phenotype of IIM, with its own characteristic clinical and radiological pattern. Finally, we present promising treatment approaches targeting mitochondria, while briefly discussing experimental models for gaining deeper insight into the disease process, and ultimately leading to novel drug development.","PeriodicalId":46408,"journal":{"name":"Rambam Maimonides Medical Journal","volume":"14 2","pages":""},"PeriodicalIF":1.5,"publicationDate":"2023-04-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10147396/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9373333","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 2

A 25-Hour Fast Among Quiescent Hereditary Coproporphyria and Variegate Porphyria Patients is Associated With a Low Risk of Complications. 静止遗传性比例卟啉症和多样性卟啉症患者禁食25小时与并发症风险低相关

IF 1.5

Rambam Maimonides Medical Journal Pub Date : 2023-01-29 DOI: 10.5041/RMMJ.10490

Yonatan Edel, Rivka Mamet, Iftach Sagi, Igor Snast, Ran Kaftory, Tomer Mimouni, Assi Levi

{"title":"A 25-Hour Fast Among Quiescent Hereditary Coproporphyria and Variegate Porphyria Patients is Associated With a Low Risk of Complications.","authors":"Yonatan Edel, Rivka Mamet, Iftach Sagi, Igor Snast, Ran Kaftory, Tomer Mimouni, Assi Levi","doi":"10.5041/RMMJ.10490","DOIUrl":"https://doi.org/10.5041/RMMJ.10490","url":null,"abstract":"Objective: In patients with acute hepatic porphyria (AHP), prolonged fasting is a known trigger of AHP attacks. Despite this, some Jewish AHP patients-mainly hereditary coproporphyria (HCP) and variegate porphyria (VP) patients-fast for 25 consecutive hours during the traditional Jewish holy day known as Yom Kippur. In this study, we evaluated the effect of the fast on these patients.Methods: A retrospective study and survey of AHP patients in Israel was carried out. Patients were asked whether they have fasted and whether any symptoms were induced by this fast. Patients' medical records were reviewed for an emergency department (ED) visit following Yom Kippur between 2007 and 2019. Only 3 acute intermittent porphyria (AIP) patients reported fasting; they were excluded from analysis.Results: A total of 21 HCP patients and 40 VP patients completed the survey; 30 quiescent patients reported they fast, while 31 did not fast. The majority of fasting patients (96.67%) reported no symptoms following a fast. We found no statistically significant association between ED visits 1 week (0.26% in both fasting and non-fasting patients) or 1 month (2.1% visits in non-fasting versus 0.78% in fasting patients) following Yom Kippur. Of the symptomatic ED visits following a fast, none were defined as severe attacks.Conclusion: A 25-hour fast in stable HCP and VP patients did not increase the risk of an acute attack and can probably be regarded as safe.","PeriodicalId":46408,"journal":{"name":"Rambam Maimonides Medical Journal","volume":"14 1","pages":""},"PeriodicalIF":1.5,"publicationDate":"2023-01-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9888486/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10673903","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Is Cirrhotic Cardiomyopathy Related to Cirrhosis Severity? 肝硬化心肌病与肝硬化严重程度有关吗?

IF 1.5

Rambam Maimonides Medical Journal Pub Date : 2023-01-29 DOI: 10.5041/RMMJ.10488

Subhash Chandra Dash, Beeravelli Rajesh, Suresh Kumar Behera, Naba Kishore Sundaray, Praveen Patil

{"title":"Is Cirrhotic Cardiomyopathy Related to Cirrhosis Severity?","authors":"Subhash Chandra Dash, Beeravelli Rajesh, Suresh Kumar Behera, Naba Kishore Sundaray, Praveen Patil","doi":"10.5041/RMMJ.10488","DOIUrl":"https://doi.org/10.5041/RMMJ.10488","url":null,"abstract":"Objective: Cirrhotic cardiomyopathy (CCM) is associated with increased morbidity and mortality in patients with liver cirrhosis. Yet, it remains an under-diagnosed entity. Further, its relation to the severity of cirrhosis is contradictory. We conducted this study on an Indian population to determine the cardiac dysfunctions in cirrhosis of the liver and correlations with etiologies and cirrhosis severity.Methods: This study enrolled patients with diagnosed liver cirrhosis without any cardiac disease or conditions affecting cardiac function. All participants were evaluated clinically, electrocardiographically, and echocardiographically. Cirrhosis severity was assessed by scores from the Model for End-stage Liver Disease (MELD) and Child-Turcotte-Pugh (CTP) tests. Cirrhotic cardiomyopathy was defined as diastolic dysfunction and/or systolic dysfunction with QT prolongation.Results: Ninety-six patients were evaluated, and CTP-A stage of cirrhosis was found in 23 (24%), CTP-B in 42 (43.8%), and CTP-C in 31 (32.3%) cases. Systolic dysfunction was most frequent (P=0.014), and left ventricular ejection fraction was significantly reduced (P=0.001) in CTP-C stage of cirrhosis. Cirrhotic cardiomyopathy was found in 39.6% (n=38) of patients; CCM patients had significantly higher CTP scores (9.6±2.6 versus 8.3±2.3, P=0.012) as well as MELD scores (19.72±4.9 versus 17.41±4.1, P=0.015) in comparison to patients without CCM.Conclusion: Cirrhotic cardiomyopathy has a positive relationship with the severity of cirrhosis. Systolic function declines with the severity of cirrhosis, and overt systolic dysfunction can be present, particularly in the advanced stage of cirrhosis of the liver.","PeriodicalId":46408,"journal":{"name":"Rambam Maimonides Medical Journal","volume":"14 1","pages":""},"PeriodicalIF":1.5,"publicationDate":"2023-01-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9888483/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9212646","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Fresh Frozen Plasma Increases Hemorrhage in Blunt Traumatic Brain Injury and Uncontrolled Hemorrhagic Shock. 新鲜冷冻血浆增加钝性外伤性脑损伤和失血性休克的出血。

IF 1.5

Rambam Maimonides Medical Journal Pub Date : 2023-01-29 DOI: 10.5041/RMMJ.10489

Hilla Abergel, Miri Bidder, Itamar Ashkenazi, Leonid Reytman, Ricardo Alfici, Michael M Krausz

{"title":"Fresh Frozen Plasma Increases Hemorrhage in Blunt Traumatic Brain Injury and Uncontrolled Hemorrhagic Shock.","authors":"Hilla Abergel, Miri Bidder, Itamar Ashkenazi, Leonid Reytman, Ricardo Alfici, Michael M Krausz","doi":"10.5041/RMMJ.10489","DOIUrl":"https://doi.org/10.5041/RMMJ.10489","url":null,"abstract":"Background: Blunt traumatic brain injury (bTBI) and uncontrolled hemorrhagic shock (UCHS) are common causes of mortality in polytrauma. We studied the influence of fresh frozen plasma (FFP) resuscitation in a rat model with both bTBI and UCHS before achieving hemorrhage control.Methods: The bTBI was induced by an external weight drop (200 g) onto the bare skull of anesthetized male Lewis (Lew/SdNHsd) rats; UCHS was induced by resection of two-thirds of the rats' tails. Fifteen minutes following trauma, bTBI+UCHS rats underwent resuscitation with FFP or lactated Ringer's solution (LR). Eight groups were evaluated: (1) Sham; (2) bTBI; (3) UCHS; (4) UCHS+FFP; (5) UCHS+LR; (6) bTBI+UCHS; (7) bTBI+UCHS+FFP; and (8) bTBI+UCHS+LR. Bleeding volume, hematocrit, lactate, mean arterial pressure (MAP), heart rate, and mortality were measured.Results: The study included 97 rats that survived the immediate trauma. Mean blood loss up to the start of resuscitation was similar among UCHS only and bTBI+UCHS rats (P=0.361). Following resuscitation, bleeding was more extensive in bTBI+UCHS+FFP rats (5.2 mL, 95% confidence interval [CI] 3.7, 6.6) than in bTBI+UCHS+LR rats (2.5 mL, 95% CI 1.2, 3.8) and bTBI+UCHS rats (1.9 mL, 95% CI 0, 3.9) (P=0.005). Overall mortality increased if bleeding was above 4.5 mL (92.3% versus 8%; P<0.001). Mortality was 83.3% (10/12) in bTBI+UCHS+FFP rats, 41.7% (5/12) in bTBI+UCHS+LR rats, and 64.3% (9/14) in bTBI+UCHS rats.Conclusion: The bTBI did not exacerbate bleeding in rats undergoing UCHS. Compared to LR, FFP resuscitation was associated with a significantly increased blood loss in bTBI+UCHS rats.","PeriodicalId":46408,"journal":{"name":"Rambam Maimonides Medical Journal","volume":"14 1","pages":""},"PeriodicalIF":1.5,"publicationDate":"2023-01-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9888485/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10673901","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

The Joles Jewish Hospital: A Short-lived Dutch Small City Hospital With an Unusual Resurrection. 乔尔斯犹太医院:一个短暂的荷兰小城市医院与一个不寻常的复活。

IF 1.5

Rambam Maimonides Medical Journal Pub Date : 2023-01-29 DOI: 10.5041/RMMJ.10492

Jack Y Vanderhoek, Dick van de Kamp

引用次数: 0

Global Vaccinations: New Urgency to Surmount a Triple Threat of Illness, Antiscience, and Anti-Semitism. 全球疫苗接种:克服疾病、反科学和反犹太主义三重威胁的新紧迫性。

IF 1.5

Rambam Maimonides Medical Journal Pub Date : 2023-01-29 DOI: 10.5041/RMMJ.10491

Peter J Hotez

引用次数: 2

Three Decades of Cannabis Research: What are the Obstacles? 大麻研究三十年：障碍是什么？

IF 1.4

Rambam Maimonides Medical Journal Pub Date : 2022-10-27 DOI: 10.5041/RMMJ.10486

Michael Dor

引用次数: 0

Comparing Sublingual and Inhaled Cannabis Therapies for Low Back Pain: An Observational Open-Label Study. 比较舌下和吸入大麻治疗腰痛:一项开放标签观察性研究。

IF 1.5

Rambam Maimonides Medical Journal Pub Date : 2022-10-27 DOI: 10.5041/RMMJ.10485

Dror Robinson, Sivan Ritter, Mustafa Yassin

{"title":"Comparing Sublingual and Inhaled Cannabis Therapies for Low Back Pain: An Observational Open-Label Study.","authors":"Dror Robinson, Sivan Ritter, Mustafa Yassin","doi":"10.5041/RMMJ.10485","DOIUrl":"https://doi.org/10.5041/RMMJ.10485","url":null,"abstract":"Background and objective: Medical cannabis is becoming an acceptable treatment modality in medicine, especially for pain relief. Concurrently, cannabis use is becoming more prevalent worldwide, a public demand-driven trend despite the lack of established scientific basis. This observational open-label study sought to investigate the effectiveness of cannabis therapy for alleviating low back pain symptoms.Methods: Two types of cannabis treatment modalities were sequentially administered to chronic low back pain patients. After an initial 1-month washout period (WO1), the first modality was cannabidiol (CBD)-rich sublingual extract treatment administered for 10 months. Following another washout period, the second modality, Δ9-tetrahydrocannabinol (THC)-rich smoked inflorescence (whole dried cannabis flowers) was administered for 12 months.Results: Enrolled in the study were 24 patients whose advanced imaging studies (i.e. computerized tomography or magnetic resonance imaging of the lumbar spine) revealed disc herniation or spinal stenosis. Three patients dropped out of extract therapy treatment but resumed study participation to receive THC-rich smoking therapy. After a minimum of 2 years, cannabis therapy had reduced lower back pain symptoms, as assessed by Oswestry Disability Index, the SF-12 patient-reported outcome questionnaire, and the visual analogue scale. Pain reduction was not significant during the extract treatment part of the study; however, pain reduction was significant during the inhaled therapy part of the study.Conclusions: Our findings indicate that inhaled THC-rich therapy is more effective than CBD-rich sublingual extract therapy for treating low back pain and that cannabis therapy is safe and effective for chronic low back pain.","PeriodicalId":46408,"journal":{"name":"Rambam Maimonides Medical Journal","volume":"13 4","pages":""},"PeriodicalIF":1.5,"publicationDate":"2022-10-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9622393/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40496002","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 2