tbuckley/cabot-search huggingface dataset:
Duration of Electrocardiographic Monitoring of Emergency Department Patients With Syncope
Authors: Venkatesh Thiruganasambandamoorthy, Brian H. Rowe, Marco L.A. Sivilotti, Andrew D. McRae, Kirtana Arcot, Marie-Joe Nemnom, Longlong Huang, Muhammad Mukarram, Andrew D. Krahn, George A. Wells, Monica Taljaard
Journal: Circulation (2019)

The optimal duration of cardiac rhythm monitoring after emergency department (ED) presentation for syncope is poorly described. We sought to describe the incidence and time to arrhythmia occurrence to inform decisions regarding duration of monitoring based on ED risk stratification.We conducted a prospective cohort study with enrolled adult patients (≥16 years old) presenting within 24 hours of syncope at 6 EDs. We collected baseline characteristics, time of syncope and ED arrival, and the Canadian Syncope Risk Score (CSRS) risk category. We followed subjects for 30 days, and our adjudicated primary outcome was serious arrhythmic conditions (arrhythmias, interventions for arrhythmias, and unexplained death). After excluding patients with an obvious serious condition on ED presentation and those with missing CSRS predictors, we used Kaplan-Meier analysis to describe the time to serious arrhythmic outcomes.A total of 5581 patients (mean age, 53.4 years; 54.5% females; 11.6% hospitalized) were available for analysis, including 346 (6.2%) for whom the 30-day follow-up was incomplete and who were censored at the last follow-up time. A total of 417 patients (7.5%) experienced serious outcomes, 207 of which (3.7%; 95% CI, 3.3%-4.2%) were arrhythmic (161 arrhythmias, 30 cardiac device implantations, 16 unexplained deaths). Overall, 4123 (73.9%) were classified as CSRS low risk, 1062 (19.0%) medium risk, and 396 (7.1%) high risk. The CSRS accurately stratified subjects as low risk (0.4% risk for 30-day arrhythmic outcome), medium risk (8.7% risk), and high risk (25.3% risk). One-half of arrhythmic outcomes were identified within 2 hours of ED arrival in low-risk patients and within 6 hours in medium- and high-risk patients, and the residual risk after these cut points were 0.2% for low-risk, 5.0% for medium-risk, and 18.1% for high-risk patients. Overall, 91.7% of arrhythmic outcomes among medium- and high-risk patients, including all ventricular arrhythmias, were identified within 15 days. None of the low-risk patients experienced ventricular arrhythmia or unexplained death, whereas 0.9% of medium-risk patients and 6.3% of high-risk patients experienced them ( P<0.0001).Serious underlying arrhythmia was often identified within the first 2 hours of ED arrival for CSRS low-risk patients and within 6 hours for CSRS medium- and high-risk patients. Outpatient cardiac rhythm monitoring for 15 days for selected medium-risk patients and all high-risk patients discharged from the hospital should also be considered.
AB0180 Identification of lncrna lncmkln1 contributed to abnormal activation of type i interferon pathway in systemic lupus erythematosus
Authors: Z. Ye, Y. Tang, Z. Yin, X. Chen, Y. Chen
Journal: Annals of the Rheumatic Diseases (2018)

<h3>Background</h3> Dysregulation or dysfunction of some key moleculars in signalling pathway is involved in disease pathogenesis. Type I interferon pathway abnormal activation has been identified as major molecular phenotype of lupus patients. Long non-coding RNA (lncRNA), as a regulator of gene expression, plays great role in signalling pathway. <h3>Objectives</h3> We hypothesised that dysregulation of lncRNA may involve in key signalling pathway of SLE. <h3>Methods</h3> Deep sequencing of human renal samples to screen differential expression of lncRNAs between LN patients and healthy donors. RNA-FISH was used to identify subcellular location of lncRNA. Stimulation in human renal mesangial cells (HRMC) by all kinds of TLR ligands, IFNs, and TNFα, and transfection in HRMC cells by antisense oligonucleotides (ASOs), and quantitative real-time polymerase chain reaction (RT-qPCR), enzyme linked immunosorbent assay (ELISA) were used to analyse the relative genes expression. LncMKLN1 transcription was activated or inhibited through CRISPR-dCas9 system in Hela cell line. RNA-seq was executed to examine the gene expression profile after changing lncMKLN1 expression, and western blot was applied to determine the key signalling molecules of IFN pathway. <h3>Results</h3> LncMKLN1, dominantly located in nucleus, was up-regulated in lupus patients compared to healthy donors, and could be induced by IFNα and TLR ligands in HRMC. Silencing lncMKLN1 significantly reduced the expression of a group of interferon-inducible genes, including IFIT3, OAS1, CXCL10, etc. We used lose-of-function and gain-of-function strategy through CRSIPR system to confirm that lncMKLN1 positively regulated type I interferon pathway. Furthermore, it was identified the involvement of lncMKLN1 in interferon signalling pathway was through regulating the expression of STAT1, IRF9 and phosphorylation of IRF9 and STAT1 although its mechanism is also needed to investigate. <h3>Conclusions</h3> Upregulated lncMKLN1 expression contributed to abnormal activation of interferon pathway of SLE. <h3>Disclosure of Interest</h3> None declared
Elusive case of dysphagia: Zenker’s diverticulum masquerading as a cervical neoplasm
Authors: Ben Singh, Irina Cazacu, Osman Ahmed, John Stroehlein, Manoop Bhutani
Journal: Endoscopy (2019)

A 73-year-old man with previous skin cancer presented with a year long history of dysphagia and regurgitation. When evaluating the dysphagia 5 months earlier, esophagogastroduodenoscopy (EGD) was generally unremarkable, only revealing a possible short-segmented Barrett’s esophagus, with no esophageal lesion noted. Magnetic resonance imaging (MRI) of the cervical spine (during follow-up from neck surgery) described a prevertebral soft tissue mass lesion measuring 2.2 × 1.7 cm. The patient was then referred to a tertiary cancer center for a suspected cervical neoplasm.
SP0143 Armful hidden telangiectasia
Authors: C. Frantz
Journal: Annals of the Rheumatic Diseases (2018)

<h3></h3> Patients with systemic sclerosis (SSc) develop a broad spectrum of vascular manifestations including the almost universal Raynaud's phenomenon, commonly digital ulceration and more rarely critical digital ischaemia. In parallel, within this very heterogeneous disease, some patients will develop vascular related organ damages leading to heart or kidney failure. In addition, SSc patients commonly exhibit telangiectasia that are visible macular, dilated superficial blood vessels. They can develop near the surface of the skin or the mucous membranes. Cutaneous telangiectasia are now included in the classification criteria and may be a marker of more aggressive vascular phenotype. Furthermore, telangiectasia can occur in the gut and promote the development of more structured damages like vascular ectasias and sometimes the very specific gastric antral vascular ectasia. All these vascular lesions induce severe and recurrent chronic iron-deficiency anaemia that can require specific local treatments to stop the bleeding. They also occur in subsets of SSc patients at risk of other SSc complications that will be highlighted in the clinical cases to be presented. <h3>Disclosure of Interest</h3> None declared
FRI0370 Clinical features and outcomes of lupus nephritis with podocyte injury in patients with systemic lupus erythematosus
Authors: K. Suzuki, Y. Kaneko, R. Anan, H. Takei, T. Takeuchi
Journal: Annals of the Rheumatic Diseases (2018)

<h3>Background</h3> Lupus podocytopathy is a recently recognised, new class of lupus nephritis (LN) characterised by diffuse foot process effacement (FPE) without capillary wall immune deposits and glomerular proliferation. However, the frequency, clinical features and treatment response to glucocorticoid and additional immunosuppressive agents of podocyte injury has not been well investigated. <h3>Objectives</h3> To clarify clinical characteristics of podocyte injury and its association with therapeutic response in patients with LN. <h3>Methods</h3> Consecutive patients with LN with available electronic microscopic findings in our institute were included. Patients were divided into 2 groups according to the presence or absence of FPE (FPE positive group and FPE negative group), and patient characteristics and laboratory data and pathological classification were compared. <h3>Results</h3> Twenty-seven patients with LN with electronic microscopic findings were enrolled. The mean age was 43.7 years old, and 22 (81.5%) were female. The ISN/RPS classification of lupus nephritis were 2 of unclassified, 1 class II, 3 class III, 3 class IV, 9 class V, 6 class III+V, and 3 class IV+V. Two cases were identified as lupus podocytopathy and their renal tissues showed focal segmental glomerulosclerosis pattern. Eighteen patients (67%) were identified to have FPE (FPE positive group) and the remaining 9 patients (33%) did not show FPE in their renal tissues (FPE negative group). The mean age was comparable (42.1 vs 41.2 y/o, p=0.84). The mean estimated glomerular filtration rate (eGFR) at LN diagnosis was 77.5 and 70.9 ml/min/1.73 m<sup>2</sup> (p=0.39), and the mean urine protein was 1.35 and 2.41 g/day (p=0.11). During the mean observation period of 50.1 months, 19 patients (70%) achieved complete response (CR, urinary protein/urine creatinine ratio &lt;0.5 mg/gcr or proteinuria by dipstick test &lt;±). While the dose of prednisolone at treatment initiation and subsequent immunosuppressive therapy was similar in both groups (prednisolone dose, 34.8 vs 39.2 mg/day, p=0.57), the rate of CR achievement was significantly higher in the FPE positive group (15 cases, 83%) than the FPE negative group (four cases, 44%, p=0.04). The duration to CR achievement was also significantly shorter in the FPE positive group (median, 8 vs 2 months, p=0.05). <h3>Conclusions</h3> This study showed that podocyte damage was common in LN and pathological types and FPE was associated with renal prognosis in LN. We should be more aware of findings of electronic microscopic in patients with LN. <h3>Disclosure of Interest</h3> None declared
FRI0702-HPR Treatment satisfaction in patients with juvenile idiopathic arthritis
Authors: A. Tollisen, A.M. Selvaag, T. Ingebrigtsen, A. Aasland, A. Lerdal, B. Flatø
Journal: Annals of the Rheumatic Diseases (2018)

<h3>Background</h3> Limited information exists regarding treatment satisfaction in patients with Juvenile Idiopathic Arthritis (JIA). <h3>Objectives:</h3> The aim of this study was to investigate satisfaction with synthetic and biologic disease-modifying anti-rheumatic drugs (sDMARDs and bDMARDs) in adults with JIA. <h3>Methods:</h3> Patients with JIA who attended Oslo University Hospital from 1995–2000 with &lt;18 months disease duration were invited to participate. From a cohort of 96 patients, 52 (54%) used DMARDs. Patients treated with Methotrexate (MTX) or biologics were assessed with the 14-item Treatment Satisfaction Questionnaire for Medication (TSQM, one questionnaire for each medication). The TSQM covers 4 domains (effectiveness, side effects, convenience and global satisfaction) with a score range from 0 – 100 and with higher score representing higher satisfaction on the domain. <h3>Results:</h3> The mean age of the 52 participants was 25.1 (4.6) years, 75% were female and 33 (63%) patients had polyarticular couse JIA. The following DMARDs were used; MTX (n=29), biologics [n=37 (etanercept, n=13; adalimumab, n=8; tocilizumab, n=6; infliximab, n=3; certolizumab, n=3; golimumab,n=2; anakinra, n=1 and rituximab, n=1)] and sulfasalazine (n=5). 19 patients used a combination of sDMARDs and bDMARDs or 2 sDMARDs. A correlation was found between the domain side effects and age in patients using MTX (rs 0.368, p=0.049). No other associations were found between TSQM domains and age, gender, disease duration or polyarticular disease course. <h3>Conclusions:</h3> Patients reported higher treatment satisfaction with biologics compared to MTX in the domains effectiveness, side effects and global satisfaction. An association was found between age and the TSQM domain side effects in patients using MTX. Other domains of TSQM were not related to patient or disease characteristics in JIA. In order to ensure good health care, information of patients' treatment satisfaction should be incorporated in the process of treatment decision-making. <h3>Acknowledgements:</h3> This project was supported by the Norwegian Foundation for Health and Rehabilitation <h3>Disclosure of Interest:</h3> None declared
Circulating Exosomes Isolated during Acute Chest Syndrome Disrupt Endothelial Integrity
Authors: Gabrielle Lapping-Carr, Joanna Gemel, Yifan Mao, Gianna Sparks, Margaret Harrington, Rebecca Sturey, Shelby Gruntorad, Wendy S Darlington, Radhika Peddinti, Eric C. Beyer
Journal: Blood (2018)
Ocular adhesives: Design, chemistry, crosslinking mechanisms, and applications
Authors: Grissel Trujillo-de Santiago, Roholah Sharifi, Kan Yue, Ehsan Shrizaei Sani, Sara Saheb Kashaf, Mario Moisés Alvarez, Jeroen Leijten, Ali Khademhosseini, Reza Dana, Nasim Annabi
Journal: Biomaterials (2019)
SAT0496 Incidence, cardiovascular events and mortality of adult inflammatory myopathies in south korea: a nationwide population-based study
Authors: K.H. Jung, H.J. Kim, W. Park, S.R. Kwon, H.S. Ahn
Journal: Annals of the Rheumatic Diseases (2018)

<h3>Background</h3> The inflammatory myopathies (IMs), including dermatomyositis (DM) and polymyositis (PM), are rare autoimmune diseases characterised by progressive proximal muscle weakness, elevated muscle enzyme and various organ involvement. Studies on epidemiology and mortality of IMs at the national level are rare. <h3>Objectives</h3> We conducted a nationwide population study of incidence, cardiovascular events (especially acute myocardial infarction, ischaemic stroke, haemorrhagic stroke) and survival of IMs in South Korea over the course of 11 years. <h3>Methods</h3> We used data from the Rare Intractable Disease (RID) registry and Health Insurance Review and Assessment (HIRA) service, which include information on all IMs patients diagnosed based on uniform criteria between 2005 and 2015. Survival data from Statistics Korea linked to HIRA-RID database were used in our survival analysis. <h3>Results</h3> In this study, total incident cases between 2005 and 2015 were 3014 (1,860 DM patients, 1,154 PM patients) and the mean annual incidence rate was 7.16/10<sup>6</sup>/year (DM was 4.42/10<sup>6</sup>/year, PM was 2.74/10<sup>6</sup>/year). The female to male ratio of DM was 2.2:1 and that of PM was 1.9:1. Cardiovascular events occurred in 155 patients during the study period. Of the patients with DM, 25 were diagnosed with AMI (SIR 1.3, 95% CI 0.9–1.9), 39 with ischaemic stroke (SIR 1.4, 95% CI 1.0–1.9), 18 with haemorrhagic stroke (SIR 1.3, 95% CI 0.8–2.0). The results of PM were similar with those of DM. Of 155 patients, 63 (40.6%) died and had a high mortality rate. 640 of 3014 patients with IMs died during the study period; 290 and 350 were male and female, respectively. The survival curve for IMs is shown in Fig.1. The DM patients showed a 5 year survival rate (YSR) of 76.8%, and a 10-YSR of 68.9%, and the PM patients showed a 5-YSR of 79.3% and a 10-YSR of 68.3%. <h3>Conclusions</h3> This is the first study of incidence and mortality of IMs over 3000 cases by the national registry in South Korea. In this nationwide population-based study of IMs, we found still high mortality and low survival and increased cardiovascular events related mortality compared than general population. <h3>Disclosure of Interest</h3> None declared
SAT0627 Fabry disease: diagnostic errors in rheumatology practice
Authors: S. Moiseev, P. Novikov, N. Bulanov, E. Karovaikina, A. Moiseev, V. Fomin
Journal: Annals of the Rheumatic Diseases (2018)

<h3>Background</h3> Fabry disease (FD) is a rare X-linked storage disease resulting from the deficient activity of the lysosomal a-galactosidase A (AGAL) and leading to a progressive accumulation of glycosphingolipids in a wide range of cell types throughout the body, including kidney, heart and nervous system. Certain manifestations of FD can mimic those of rheumatic diseases. <h3>Objectives</h3> To evaluate the occurrence and the possible causes of diagnostic errors in patients with FD in rheumatology practice. <h3>Methods</h3> We retrospectively studied the medical records of 82 adult patients with definite FD (low or absent AGAL activity, pathogenic mutation in the GLA gene, typical clinical symptoms in patient and/or his relatives, results of kidney biopsy). There were 55 males and 27 females aged of 18 to 69 years (median 38<sup>29; 48</sup> years). <h3>Results</h3> Seventy two of 82 patients (87.8%) had a history of classic phenotype of FD from childhood or adolescence. The typical manifestations included neuropathic pain that was related to heat and fever (65/82; 79%), angiokeratoma (37/82; 45%), anhydrosis/hypohydrosis (44/82; 53.6%), and cornea verticillata (40/62; 64.5%). However, there was a significant delay to diagnosis of up to 51 years (median 18<sup>9; 27</sup> years). Moreover, diagnosis was established by nationwide screening in dialysis units in 22/82 (26.8%) patients or by family screening in 34/82 (41.4%) patients. At the time of diagnosis, patients usually presented with a clinical picture of systemic disease with mild to moderate proteinuria with or without impairment of kidney function (70/82; 85.4%), left ventricular hypertrophy (56/82; 68.3%), white matter lesions on brain MRI (38/72; 52.8%), and/or a history of stroke (15/82; 18.3%). Twenty six of 82 patients (31.7%) previously had at least one diagnosis of rheumatic disease (Table 1). The common causes for referral to rheumatologist were skin rash and neuropathic pain. In 6 of 26 patients (23.1%), the latter was initially misdiagnosed as joint pain. The possible causes of diagnostic errors included also 'genuine' arthralgia (6/26; 23.1%), episodes of unexplained fever (13/26; 50%), Raynaud phenomenon (2/26; 7.7%), and the laboratory markers of inflammation (11/26; 42.3%). <h3>Conclusions</h3> Practicing rheumatologists should be aware of FD, given a high occurrence of diagnostic errors. The clues to correct diagnosis include a history of typical symptoms (i.e. neuropathic pain, angiokeratoma, hypohydrosis) from childhood or adolescence and/or the presence of typical manifestations in family members. Notably, FD can initially present as an autoinflammatory disorder with episodes of joint pain and unexplained fever associated with the laboratory markers of inflammation. <h3>Disclosure of Interest</h3> None declared
Commensal Enterobacteriaceae Protect against Salmonella Colonization through Oxygen Competition
Authors: Yael Litvak, Khin K.Z. Mon, Henry Nguyen, Ganrea Chanthavixay, Megan Liou, Eric M. Velazquez, Laura Kutter, Monique A. Alcantara, Mariana X. Byndloss, Connor R. Tiffany, Gregory T. Walker, Franziska Faber, Yuhua Zhu, Denise N. Bronner, Austin J. Byndloss, Renée M. Tsolis, Huaijun Zhou, Andreas J. Bäumler
Journal: Cell Host & Microbe (2019)
Abstract 19630: TASR Genotype is Associated With Adherence to Dietary Sodium Recommendations in Adults With Cardiovascular Disease Risk Factors
Authors: Jennifer L Smith, Steve Estus, Terry A Lennie, Debra K Moser, Misook L Chung, Gia T Mudd-Martin
Journal: Circulation (2016)

Introduction: Genetic variants in taste perception have been identified that can influence dietary intake patterns associated with cardiovascular disease (CVD) risk. TAS2R38 gene variants influence...
Antibiotics-induced monodominance of a novel gut bacterial order
Authors: Falk Hildebrand, Lucas Moitinho-Silva, Sonja Blasche, Martin T Jahn, Toni Ingolf Gossmann, Jaime Huerta-Cepas, Rajna Hercog, Mechthild Luetge, Mohammad Bahram, Anna Pryszlak, Renato J Alves, Sebastian M Waszak, Ana Zhu, Lumeng Ye, Paul Igor Costea, Steven Aalvink, Clara Belzer, Sofia K Forslund, Shinichi Sunagawa, Ute Hentschel, Christoph Merten, Kiran Raosaheb Patil, Vladimir Benes, Peer Bork
Journal: Gut (2019)

The composition of the healthy human adult gut microbiome is relatively stable over prolonged periods, and representatives of the most highly abundant and prevalent species have been cultured and described. However, microbial abundances can change on perturbations, such as antibiotics intake, enabling the identification and characterisation of otherwise low abundant species.Analysing gut microbial time-series data, we used shotgun metagenomics to create strain level taxonomic and functional profiles. Community dynamics were modelled postintervention with a focus on conditionally rare taxa and previously unknown bacteria.In response to a commonly prescribed cephalosporin (ceftriaxone), we observe a strong compositional shift in one subject, in which a previously unknown species, UBorkfalki ceftriaxensis, was identified, blooming to 92% relative abundance. The genome assembly reveals that this species (1) belongs to a so far undescribed order of Firmicutes, (2) is ubiquitously present at low abundances in at least one third of adults, (3) is opportunistically growing, being ecologically similar to typical probiotic species and (4) is stably associated to healthy hosts as determined by single nucleotide variation analysis. It was the first coloniser after the antibiotic intervention that led to a long-lasting microbial community shift and likely permanent loss of nine commensals.The bloom of UB. ceftriaxensis and a subsequent one of Parabacteroides distasonis demonstrate the existence of monodominance community states in the gut. Our study points to an undiscovered wealth of low abundant but common taxa in the human gut and calls for more highly resolved longitudinal studies, in particular on ecosystem perturbations.
FRI0562 Association between joint regions with ultrasound-determined synovitis and systemic inflammatory markers
Authors: D. Nakagomi, K. Ikeda, K. Kobayashi, S. Hanai, M. Yamagata, R. Koh, S. Furuta, H. Nakajima, K. Kitamura
Journal: Annals of the Rheumatic Diseases (2018)

<h3>Background</h3> It is unknown which joint arthritis the inflammatory markers such as CRP or MMP-3 reflect. <h3>Objectives</h3> We analysed the association between joint regions with ultrasound-determined synovitis and systemic inflammatory markers. <h3>Methods</h3> We enrolled 152 patients with untreated arthritis and performed musculoskeletal ultrasound on 40 joints and determined a semiquantitative grade for power Doppler (PD) signals. We analysed the associations between PD scores in 8 joint regions and CRP/MMP-3 levels using multiple linear regression models with forced entry method. <h3>Results</h3> Mean age was 55 years and 112 patients were female. Median CRP and MMP-3 were 0.36 mg/dl and 65.7 ng/ml. Median total PD score was 2. Standard regression coefficients for 8 joint regions were −0.009 for MCPs, 0.05 for PIPs, 0.372 for wrists, 0.183 for elbows, 0.628 for shoulders, 0.377 for knees, 0.261 for ankles, and −0.0131 for MTPs in a regression model to explain CRP using PD scores as dependent variables. Standard regression coefficients for the same joint regions were −5.134,–4.449, 24.061, 27.839, 22.508, 64.108, 36.501, and 2.539 to explain MMP-3. <h3>Conclusions</h3> Systemic inflammatory markers such as CRP and MMP-3 do not accurately reflect the inflammation in small joints. Conversely, it is necessary to weight the large joints for the global ultrasound synovitis score to represent the severity of systemic inflammation. <h3>References</h3> [1] Kawashiri SY, Kawakami A, Iwamoto N, et al. The power Doppler ultrasonography score from 24 synovial sites or 6 simplified synovial sites, including the metacarpophalangeal joints, reflects the clinical disease activity and level of serum biomarkers in patients with rheumatoid arthritis. Rheumatology (Oxford)2011May;50(5):962–5. [2] Gorai M, Ogasawara M, Matsuki Y, et al. Weighting with the Lansbury articular index improves the correlation of ultrasound score with serum matrix metalloproteinase-3 level in rheumatoid arthritis patients. Mod Rheumatol2014Nov;24(6):915–9. <h3>Disclosure of Interest</h3> None declared