tbuckley/cabot-search huggingface dataset:
AB0778 Clinical characteristics and prognosis of polymyositis and dermatomyositis associated with malignancy: a 25-year retrospective study
Authors: K. Motomura, H. Yamashita, S. Yamada, Y. Takahashi, H. Kaneko
Journal: Annals of the Rheumatic Diseases (2018)

<h3>Background</h3> Previous studies indicate that malignancies in polymyositis (PM) or dermatomyositis (DM) patients are associated with high mortality. Hence, it becomes important clinically to identify patients who are at high risk of developing malignancy. However, previous studies to characterise such risk factors for malignancy in patients with PM and DM were small size or the findings ware not conclusive. <h3>Objectives</h3> This study investigated factors predictive of malignancy and prognosis in patients with PM and DM. <h3>Methods</h3> We conducted a retrospective study of PM and DM patients who were inpatients at our hospital between January 1992 and September 2016. The diagnosis of PM or DM was made according to Bohan and Peter criteria. The collected data included gender, age at onset, laboratory test results at presentation, clinical features and complications in all progress. Past history of hypertension or diabetes mellitus was determined upon diagnosis of myositis. All patients in this study were followed from the baseline visit until loss of follow-up or death or otherwise until censor date 1 September 2017. Fisher exact test and Mann-Whitney U test were used for group comparisons. Univariate and multivariate analysis of the predictors of malignancy associated with PM or DM were performed by logistic regression to identify independent risk factors. Patient survival was analysed by using Kaplan-Meier curve and log-rank test. <h3>Results</h3> Among 134 patients, 29 had cancer diagnosed between 2 years prior and 3 years following identification of PM or DM. Esophageal cancer (n=5, 14.7%) and lung cancer (n=5, 14.7%) were the most frequent. Univariate analysis showed that male sex (55.2% vs 23.8%, p=0.003), older age (64.6±11.3 vs 53.4±16.5, p&lt;0.001), past history of diabetes mellitus (28.6% vs 2.9%, p&lt;0.001), dysphagia (41.4% vs 17.3%, p=0.01) and absence of interstitial lung disease (37.9% vs 64.8%, p=0.01), arthralgia (17.2% vs 47.1%, p=0.005) and the Raynaud phenomenon (3.4% vs 23.1%, p=0.02) were associated with increased malignancy. Multivariate analysis showed that independent factors included male sex (OR=3.65, p=0.03), older age (OR=1.05, p=0.02), past history of diabetes mellitus (OR=10.4, p=0.005) and absence of interstitial lung disease (OR=0.25, p=0.03) (table 1). Survival was significantly lower in patients with malignancy than in patients without malignancy (p&lt;0.001). <h3>Conclusions</h3> This study is the first to associate a past history of diabetes mellitus with malignancy; 28.6% of PM or DM patients with malignancy had diabetes mellitus whereas 7.3 percent of cancer patients had diabetes mellitus. <h3>Reference</h3> [1] J.wang, G.Guo, G. Chen, et al. Br J Dermatol. 2013; 169(4): 838–847 <h3>Disclosure of Interest</h3> None declared
WINDOW consortium: A path towards increased therapy efficacy against glioblastoma
Authors: Kulsoom U. Abdul, Megan Houweling, Fredrik Svensson, Ravi S. Narayan, Fleur M.G. Cornelissen, Asli Küçükosmanoglu, Emmanouil Metzakopian, Colin Watts, David Bailey, Tom Wurdinger, Bart A. Westerman
Journal: Drug Resistance Updates (2018)
Is Culprit-Lesion-Only PCI in Cardiogenic Shock Still Better at 1 Year?
Authors: Tom Adriaenssens, Frans Van de Werf
Journal: New England Journal of Medicine (2018)

Cardiogenic shock occurs in approximately 5% of patients with an acute coronary syndrome — usually, but not always, after ST-segment elevation myocardial infarction. If a large amount of myocardial tissue has become ischemic or injured, pump failure and reduced blood flow to vital organs occur. Urgent percutaneous coronary intervention (PCI) of the culprit lesion is currently the only therapy associated with a significant decrease in mortality.1 No other intervention with a device or pharmacologic agent has shown a significant benefit, and as a consequence, mortality has plateaued at 50% in recent years. On angiography performed during the acute phase, clinically . . .
Abnormal Illness Behavior, Alexithymia, Demoralization, and Other Clinically Relevant Psychosocial Syndromes in Kidney Transplant Recipients: A Comparative Study of the Diagnostic Criteria for Psychosomatic Research System versus ICD-10 Psychiatric Nosology
Authors: Yuri Battaglia, Elena Martino, Giulia Piazza, Elena Cojocaru, Sara Massarenti, Luana Peron, Alda Storari, Luigi Grassi
Journal: Psychotherapy and Psychosomatics (2018)
Abstract 19133: Diabetes is associated With Unrecognized Myocardial Infarctions in African-Americans: The Jackson Heart Study
Authors: Richard B Stacey, Janice Zgibor, Michael E Hall, Douglas Schocken, Paul E Leaverton
Journal: Circulation (2017)

Background: Almost 1/2 of initial myocardial infarctions (MI) are unrecognized or clinically-silent (UMI), which forecasts future clinical events. While diabetes mellitus (DM) increases the risk fo...
Thrombogenic and Arrhythmogenic Roles of the Left Atrial Appendage in Atrial Fibrillation
Authors: Luigi Di Biase, Andrea Natale, Jorge Romero
Journal: Circulation (2018)

Understanding the anatomy, physiology, and arrhythmogenic and thrombogenic roles of the left atrial appendage (LAA) has become very important. The potential deleterious effects of this chamber in patients with atrial fibrillation have led to the development of specific treatments for this structure. It has been established that the LAA is the area where the vast majority of thrombi in nonvalvular atrial fibrillation are formed and that some LAA morphologies may actually facilitate thrombi formation and risk stratification for thromboembolic events in patients with low CHA 2 DS 2 -VASc scores. Likewise, clinical data supporting the role of LAA electric isolation to improve freedom from all-atrial arrhythmias are rapidly growing. Acute LAA electric isolation can be achieved in most cases of catheter ablation for atrial fibrillation. However, late LAA reconnections are frequent. Special considerations should be taken when this procedure is performed to avoid myocardial perforation, left phrenic nerve, and left circumflex coronary artery damage. LAA electric isolation is a safe procedure that does not increase the risk of stroke as long as patients remain compliant with lifelong anticoagulation regardless of CHA 2 DS 2 -VASc score. Finally, ligation of the LAA has recently been associated with modulation of the renin-angiotensin-aldosterone system with clinical implications in blood pressure control.
Benefits and Harms of Antihypertensive Treatment in Low-Risk Patients With Mild Hypertension
Authors: James P. Sheppard, Sarah Stevens, Richard Stevens, Una Martin, Jonathan Mant, F. D. Richard Hobbs, Richard J. McManus
Journal: JAMA Internal Medicine (2018)

Evidence to support initiation of pharmacologic treatment in low-risk patients with mild hypertension is inconclusive, with previous trials underpowered to demonstrate benefit. Clinical guidelines across the world are contradictory.
Early ACL reconstruction is required to prevent additional knee injury: a misconception not supported by high-quality evidence
Authors: Stephanie Rose Filbay
Journal: British Journal of Sports Medicine (2018)

It is evident that meniscus injury is associated with increased rates of osteoarthritis and joint replacement surgery, and that preserving the meniscus should be a key priority when managing ACL-injured individuals. A common belief within the orthopaedic community is that early ACL reconstruction is necessary to prevent additional meniscus and cartilage injury.1 The studies referenced to support this argument (table 1) are limited by selection and indication bias. While these studies highlight the importance of managing ACL injury early to preserve joint health, they do not provide evidence that early reconstruction is superior to ‘evidence-based rehabilitation’2 in reducing subsequent meniscus or cartilage injury rates. Referenced studies share a similar conclusion that people who present for early reconstruction shortly after ACL injury have less meniscus or cartilage damage than those who present for ACL reconstruction months or years after injury (table 1). This is not surprising; individuals who are chronically ACL deficient may have been incorrectly diagnosed or exposed themselves to sport or other high-risk activities for months or years, without undertaking appropriate rehabilitation. Importantly, studies that found more meniscus or cartilage damage in patients presenting for a delayed reconstruction (following unknown treatment/no treatment) compared with patients presenting for an early reconstruction should not be used to justify recommending early ACL reconstruction over management of an acute ACL injury with evidence-based rehabilitation.

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Table 1 
Studies commonly referenced to support the belief …
Abstract 19548: Comparison of a Novel Four Dimensional CT Myocardial Strain Analyzing Technique With MRI, TTE, and Endomyocardial Biopsy
Authors: Hiroyuki Takaoka, Nobusada Funabashi, Koya Ozawa, Takashi Nakayama, Yoshio Kobayashi
Journal: Circulation (2015)

Introduction: Myocardial strain is a new indicator of myocardial characteristics and function, mainly evaluated by transthoracic echocardiography (TTE) or magnetic resonance imaging (MRI) techniques that rely on dynamic two dimensional images. Hypothesis: We hypothesized that utilizing the full capability of four dimensional (4D) volumetric 320 slice CT image data with retrospective ECG gating we could provide additional information on myocardial morphology, characteristics and function on Novel 4D CT myocardial stain analysis (developed with PhyZioDynamics, Zio). Method: We retrospectively analyzed ten subjects with various rare and un- typical myocardial diseases diagnosed by 320 slice CT using retrospective ECG gating and dose modulation. Some diagnoses were confirmed using MRI, TTE or endomyocardial biopsy (EMB). 4D CT myocardial strain analysis confirmed their diagnosis (see fig). Areas with maximum strain are colored red and decreasing strain, yellow, green, blue, and finally no color (=no strain). Results: Case 1 had a large, idiopathic fat deposit in the left ventricle (LV) that could be observed using enhanced CT with a low CT attenuation area. On 4D CT strain analysis, the fat deposit showed no strain. Case 2 had cardiac amyloidosis confirmed by EMB and Case 3 had type 2 hypertrophic cardiomyopathy (HCM). MRI showed late gadolinium enhancements (LGE) in both cases. At corresponding sites, no strain was observed by 4D CT. Other cases analyzed were dilated cardiomyopathy with focal LGE on MRI, subendocardial myocardial infarction with enlarged LV myocardium, etc. Conclusion: This is the first report worldwide of a 4D CT myocardial strain analyzing technique that utilizes full volumetric data. Our new technique uses ordinary cardiac CT data acquired with retrospective ECG gating, to provide detailed additional information on myocardial characteristics and function in addition to LV ejection fraction volume data and information on coronary arteries.
酒色巨唇中 GNAQ 突变
Authors: G. Ma, Z. Yu, F. Liu, L. Wang, W. Yu, J. Zhu, H. Gu, Y. Liu, R. Cai, X. Lin
Journal: British Journal of Dermatology (2018)

毛细血管畸形 (CMs) 也被称为酒色斑,是一种胎记,在每 1000 名新生儿中影响大约 3 至 5 人。酒色巨唇 (PWM) 是同时导致唇增大的 CMs。据报告,酒色斑是由一种称为 GNAQ 的蛋白突变导致的,这项由中华人民共和国研究者进行的研究旨在探索不同 (PWM) 组织[如皮肤、粘膜(例如口腔和鼻腔内的组织)]、腺体和肌肉中此突变的发生频率。作者们发现,这种称为 GNAQ (R183Q) 的突变不存在于腺体标本中,但分别在 90% 的皮肤、95% 的粘膜和 95% 的肌肉样本中检出。这项研究还发现,GNAQ 突变频率和临床特征之间呈正相关,意味着突变与症状有关,尤其对于粘膜而言。因此,作者们假设,唇粘膜中的 GNAQ 突变(无论皮肤有无基础酒色斑)可被用作早期 PWM 的预测标志物。作者们指出,如果医生认为患者可能有基础酒色斑,进行粘膜活检(对一小块皮肤标本进行检查)可能有助于更早发现 PWM 和更早开始治疗。活检是实用方便的,愈合迅速,不会留疤。 Linked Article:Ma et al. Br J Dermatol 2018; 179:1109–1114
Phase 1 Cohort Expansion of Flotetuzumab, a CD123×CD3 Bispecific Dart® Protein in Patients with Relapsed/Refractory Acute Myeloid Leukemia (AML)
Authors: Geoffrey L. Uy, Michael P. Rettig, Norbert Vey, John Godwin, Matthew C Foster, David A. Rizzieri, Martha L. Arellano, Max S. Topp, Gerwin Huls, Mojca Jongen-Lavrencic, Giovanni Martinelli, Stefania Paolini, Fabio Ciceri, Matteo Giovanni Carrabba, Kendra L. Sweet, Farhad Ravandi, Sarah E. Church, Jayakumar Vadakekolathu, Sergio Rutella, Jichao Sun, Kang Yang, Jan Baughman, Teia Curtis, Erin Timmeny, Kerri Cali, Kathy Tran, John Muth, Ross La Motte-Mohs, Camille Poirot, Athanasios Pallis, Alessandra Cesano, Ezio Bonvini, Jon Wigginton, Bob Lowenberg, Jan K Davidson-Moncada, John F. DiPersio
Journal: Blood (2018)
Biallelic mutations in DNA ligase 1 underlie a spectrum of immune deficiencies
Authors: Patrick Maffucci, Jose Chavez, Thomas J. Jurkiw, Patrick J. O’Brien, Jordan K. Abbott, Paul R. Reynolds, Austen Worth, Luigi D. Notarangelo, Kerstin Felgentreff, Patricia Cortes, Bertrand Boisson, Lin Radigan, Aurélie Cobat, Chitra Dinakar, Mohammad Ehlayel, Tawfeg Ben-Omran, Erwin W. Gelfand, Jean-Laurent Casanova, Charlotte Cunningham-Rundles
Journal: Journal of Clinical Investigation (2018)

We report the molecular, cellular, and clinical features of 5 patients from 3 kindreds with biallelic mutations in the autosomal LIG1 gene encoding DNA ligase 1. The patients exhibited hypogammaglobulinemia, lymphopenia, increased proportions of circulating γδT cells, and erythrocyte macrocytosis. Clinical severity ranged from a mild antibody deficiency to a combined immunodeficiency requiring hematopoietic stem cell transplantation. Using engineered LIG1-deficient cell lines, we demonstrated chemical and radiation defects associated with the mutant alleles, which variably impaired the DNA repair pathway. We further showed that these LIG1 mutant alleles are amorphic or hypomorphic, and exhibited variably decreased enzymatic activities, which lead to premature release of unligated adenylated DNA. The variability of the LIG1 genotypes in the patients was consistent with that of their immunological and clinical phenotypes. These data suggest that different forms of autosomal recessive, partial DNA ligase 1 deficiency underlie an immunodeficiency of variable severity.
Hemosiderotic Fibrolipomatous Tumor on FDG PET/CT
Authors: Fanhui Yang, Fei Chen, Lingzhi Cao, Shiyu Lin, Suping Li
Journal: Clinical Nuclear Medicine (2018)

A 74-year-old woman with a history of melanoma 10 years ago presented worsening swelling pain associated with a lump in her inner left thigh. Metastatic disease was suspected and for this reason, FDG PET/CT was performed. The images demonstrated peripherally increased FDG activity in the lump with a centrally photopenic region. Postsurgical pathological examination showed a hemosiderotic fibrolipomatous tumor.
Local Delivery and Sustained‐Release of Nitric Oxide Donor Loaded in Mesoporous Silica Particles for Efficient Treatment of Primary Open‐Angle Glaucoma
Authors: Chunchun Hu, Jianguo Sun, Yu Zhang, Jian Chen, Yuan Lei, Xinghuai Sun, Yonghui Deng
Journal: Advanced Healthcare Materials (2018)

Abstract Nitric oxide (NO) donors are ideal drug candidates for reducing intraocular pressure in the treatment of glaucoma. However, poor cornea penetration, short duration of efficacy, and narrow therapeutic index of most NO donors obstruct their clinical applications in glaucoma treatment. This study reports a novel NO donor delivery system based on mesoporous silica nanoparticles that can readily overcome the above difficulties and deliver the NO‐donating drug sodium nitroprusside to the target tissues (trabecular meshwork and Schlemm's canal). Mesoporous silica nanoparticles loaded with sodium nitroprusside can produce more exogenous NO and sustain higher NO concentration in animal eye models, which significantly extend the duration of intraocular pressure reduction from 3 to 48 h with only 1/40 of the dose of sodium nitroprusside solution. These findings open up the possibility of mesoporous silica nanoparticles loading sodium nitroprusside for effective management of ocular hypertension.
Genetic Predisposition to Renal Cell Carcinoma: Implications for Counseling, Testing, Screening, and Management
Authors: Brian Shuch, Jin Zhang
Journal: Journal of Clinical Oncology (2018)

In many individuals with renal cell carcinoma (RCC), a hereditary cause may have contributed to cancer development. Various risk factors can be suggestive of a genetic contribution, including early disease onset, multifocal or bilateral tumors, family history of RCC, and personal/family history of other benign or malignant tumors. Genetic counseling and understanding of the entire family tree are the first steps in evaluation and will determine if the patient should proceed with testing. Methods of testing have changed to next-generation sequencing, which allows multiple genes to be evaluated together. The results of testing have significant implications for the individual and his or her family members. Screening of the kidney and at-risk organs ensues, with most algorithms focused on early diagnosis and intervention to limit morbidity and mortality of disease manifestations. A comprehensive clinical program that can offer multidisciplinary care is useful for several complex cancer syndromes. Management of localized and advanced hereditary kidney cancers may differ from the sporadic forms of RCC. Knowledge of the genetics can have significant management implications and if necessary genetic evaluation can be expedited to allow treatment decisions.
Sputum Microbiome Is Associated with 1-Year Mortality after Chronic Obstructive Pulmonary Disease Hospitalizations
Authors: Fernando Sergio Leitao Filho, Nawaf M. Alotaibi, David Ngan, Sheena Tam, Julia Yang, Zsuzsanna Hollander, Virginia Chen, J. Mark FitzGerald, Corey Nislow, Janice M. Leung, S. F. Paul Man, Don D. Sin
Journal: American Journal of Respiratory and Critical Care Medicine (2018)

Rationale: Lung dysbiosis promotes airway inflammation and decreased local immunity, potentially playing a role in the pathogenesis of acute exacerbations of chronic obstructive pulmonary disease (AECOPD). Objectives: We sought to determine the relationship between sputum microbiome at the time of AECOPD hospitalization and 1-year mortality in a COPD cohort. Methods: We used sputum samples from 102 patients hospitalized because of AECOPD. All subjects were followed for 1 year after discharge. The microbiome profile was assessed through sequencing of 16S rRNA gene. Microbiome analyses were performed according to 1-year mortality status. To investigate the effect of α-diversity measures and taxon features on time to death, we applied Cox proportional hazards regression models and obtained hazard ratios (HRs) associated with these variables. Measurements and Main Results: We observed significantly lower values of α-diversity (richness, Shannon index, evenness, and Faith's Phylogenetic Diversity) among nonsurvivors (n = 19, 18.6%) than survivors (n = 83, 81.4%). β-Diversity analysis also demonstrated significant differences between both groups (adjusted permutational multivariate ANOVA, P = 0.010). The survivors had a higher relative abundance of Veillonella; in contrast, nonsurvivors had a higher abundance of Staphylococcus. The adjusted HRs for 1-year mortality increased significantly with decreasing α-diversity. We also observed lower survival among patients in whom sputum samples were negative for Veillonella (HR, 13.5; 95% confidence interval, 4.2-43.9; P < 0.001) or positive for Staphylococcus (HR, 7.3; 95% confidence interval, 1.6-33.2; P = 0.01). Conclusions: The microbiome profile of sputum in AECOPD is associated with 1-year mortality and may be used to identify subjects with a poor prognosis at the time of hospitalization.
Imetelstat Is Effective Treatment for Patients with Intermediate-2 or High-Risk Myelofibrosis Who Have Relapsed on or Are Refractory to Janus Kinase Inhibitor Therapy: Results of a Phase 2 Randomized Study of Two Dose Levels
Authors: John Mascarenhas, Rami S. Komrokji, Michele Cavo, Bruno Martino, Dietger Niederwieser, Andreas Reiter, Bart L Scott, Maria R. Baer, Ronald Hoffman, Olatoyosi Odenike, Jacqueline Bussolari, Eugene Zhu, Fei Huang, Esther Rose, Laurie Sherman, Souria Dougherty, Faye M. Feller, Jean-Jacques Kiladjian
Journal: Blood (2018)
Interventions for Preventing Thromboembolic Events in Patients With Atrial Fibrillation
Authors: Angela Lowenstern, Sana M. Al-Khatib, Lauren Sharan, Ranee Chatterjee, Nancy M. Allen LaPointe, Bimal Shah, Ethan D. Borre, Giselle Raitz, Adam Goode, Roshini Yapa, J. Kelly Davis, Kathryn Lallinger, Robyn Schmidt, Andrzej S. Kosinski, Gillian D. Sanders
Journal: Annals of Internal Medicine (2018)

Background: The comparative safety and effectiveness of treatments to prevent thromboembolic complications in atrial fibrillation (AF) remain uncertain. Purpose: To compare the effectiveness of medical and procedural therapies in preventing thromboembolic events and bleeding complications in adults with nonvalvular AF. Data Sources: English-language studies in several databases from 1 January 2000 to 14 February 2018. Study Selection: Two reviewers independently screened citations to identify comparative studies of treatments to prevent stroke in adults with nonvalvular AF who reported thromboembolic or bleeding complications. Data Extraction: Two reviewers independently abstracted data, assessed study quality and applicability, and rated strength of evidence. Data Synthesis: Data from 220 articles were included. Dabigatran and apixaban were superior and rivaroxaban and edoxaban were similar to warfarin in preventing stroke or systemic embolism. Apixaban and edoxaban were superior and rivaroxaban and dabigatran were similar to warfarin in reducing the risk for major bleeding. Treatment effects with dabigatran were similar in patients with renal dysfunction (interaction P > 0.05), and patients younger than 75 years had lower bleeding rates with dabigatran (interaction P < 0.001). The benefit of treatment with apixaban was consistent in many subgroups, including those with renal impairment, diabetes, and prior stroke (interaction P > 0.05 for all). The greatest bleeding risk reduction was observed in patients with a glomerular filtration rate less than 50 mL/min/1.73 m2 (P = 0.003). Similar treatment effects were observed for rivaroxaban and edoxaban in patients with prior stroke, diabetes, or heart failure (interaction P > 0.05 for all). Limitation: Heterogeneous study populations, interventions, and outcomes. Conclusion: The available direct-acting oral anticoagulants (DOACs) are at least as effective and safe as warfarin for patients with nonvalvular AF. The DOACs had similar benefits across several patient subgroups and seemed safe and efficacious for a wide range of patients with nonvalvular AF. Primary Funding Source: Patient-Centered Outcomes Research Institute. (PROSPERO: CRD42017069999)