tbuckley/cabot-search huggingface dataset:
Successful single-session cricopharyngeal and Zenker’s diverticulum peroral endoscopic myotomy
Authors: Olaya Brewer Gutierrez, Robert Moran, Juliana Yang, Omid Sanaei, Anthony Kalloo, Vivek Kumbhari, Mouen Khashab
Journal: Endoscopy (2018)

Therapy of symptomatic Zenker’s diverticulum can be accomplished with open surgery, rigid endoscopy, or flexible endoscopy [1]. Peroral endoscopic myotomy (POEM) can be used to treat Zenker’s diverticulum using the principles of submucosal tunneling. Zenker’s POEM has potential advantages over traditional endoscopic septotomy, including complete septum division and mucosal integrity during septotomy. Importantly, Zenker’s POEM may be associated with a decreased risk of symptom recurrence, because of the complete exposure and dissection of the septum ([Table 1]). We describe a patient with a hypertensive cricopharyngeus muscle and Zenker’s diverticulum successfully treated with POEM in the same session.
THU0289 Burden of psoriatic arthritis in different definitions of disease activity: comparing minimal disease activity and disease activity index for psoriatic arthritis
Authors: K. Wervers, M. Vis, I. Tchetverikov, A.H. Gerards, M.R. Kok, C.W. Appels, W.L. van der Graaff, J.H. van Groenendael, L.A. Korswagen, J.J. Veris-Van Dieren, J.M. Hazes, J.J. Luime
Journal: Annals of the Rheumatic Diseases (2018)

<h3>Background</h3> Treat-to-target strategies have improved outcomes in rheumatic diseases. In psoriatic arthritis (PsA), the proposed targets are the multidimensional target Minimal Disease Activity (MDA) and the articular target Disease Activity index for PsA (DAPSA). <h3>Objectives</h3> We aimed to compare burden of PsA in patients with low disease activity according to the two definitions MDA and DAPSA-Low Disease Activity (DAPSA-LDA), one year after diagnosis. <h3>Methods</h3> We obtained data on MDA, DAPSA-LDA and disease burden one year after diagnosis from patients included in the Dutch southwest early PsA cohort. Disease burden was assessed in two domains: ‘Body Functions’, including Short Form 36 Bodily Pain (SF36-BP), and ‘Activity’, including Health Assessment Questionnaire (HAQ). <h3>Results</h3> Of the 292 patients included, 48% achieved MDA and 64% DAPSA-LDA. Average ‘Body Functions’ and ‘Activity’ were similar in patients in MDA and patients in DAPSA-LDA. However the scores were significantly better in the 44% of patients in both MDA and DAPSA-LDA than in the 20% of patients only in DAPSA-LDA. The average SF36-BP was higher in patients achieving both targets (74.7, 95% CI 72.0–77.4) than in patients only in DAPSA-LDA (58.7, 95% CI 54.8–62.6). Similarly, mean HAQ scores measuring ‘Activity’ were 0.19 (95%CI 0.14–0.25) and 0.61 (95%CI 0.48–0.73) respectively. <h3>Conclusions</h3> Of patients newly diagnosed with PsA, 48% achieved MDA and 64% DAPSA-LDA after one year receiving usual care. Average disease burden was similar in patients in MDA and in patients in DAPSA-LDA. However, those that only achieved DAPSA-LDA reported worse outcomes than those also achieving MDA. <h3>Disclosure of Interest</h3> None declared
Clinical trials for treating recurrent head and neck cancer with boron neutron capture therapy using the Tsing‐Hua Open Pool Reactor
Authors: Ling‐Wei Wang, Yen‐Wan Hsueh Liu, Fong‐In Chou, Shiang‐Huei Jiang
Journal: Cancer Communications (2018)

Abstract Head and neck (HN) cancer is an endemic disease in Taiwan, China. Locally recurrent HN cancer after full‐dose irradiation poses a therapeutic challenge, and boron neutron capture therapy (BNCT) may be a solution that could provide durable local control with tolerable toxicity. The Tsing‐Hua Open Pool Reactor (THOR) at National Tsing‐Hua University in Hsin‐Chu, provides a high‐quality epithermal neutron source for basic and clinical BNCT research. Our first clinical trial, entitled “A phase I/II trial of boron neutron capture therapy for recurrent head and neck cancer at THOR”, was carried out between 2010 and 2013. A total of 17 patients with 23 recurrent HN tumors who had received high‐dose photon irradiation were enrolled in the study. The fructose complex of l ‐boronophenylalanine was used as a boron carrier, and a two‐fraction BNCT treatment regimen at 28‐day intervals was used for each patient. Toxicity was acceptable, and although the response rate was high (12/17), re‐recurrence within or near the radiation site was common. To obtain better local control, another clinical trial entitled “A phase I/II trial of boron neutron capture therapy combined with image‐guided intensity‐modulated radiotherapy (IG‐IMRT) for locally recurrent HN cancer” was initiated in 2014. The first administration of BNCT was performed according to our previous protocol, and IG‐IMRT was initiated 28 days after BNCT. As of May 2017, seven patients have been treated with this combination. The treatment‐related toxicity was similar to that previously observed with two BNCT applications. Three patients had a complete response, but locoregional recurrence was the major cause of failure despite initially good responses. Future clinical trials combining BNCT with other local or systemic treatments will be carried out for recurrent HN cancer patients at THOR.
Phase III randomized study of second line ADI-PEG 20 plus best supportive care versus placebo plus best supportive care in patients with advanced hepatocellular carcinoma
Authors: G.K. Abou-Alfa, S. Qin, B.-Y. Ryoo, S.-N. Lu, C.-J. Yen, Y.-H. Feng, H.Y. Lim, F. Izzo, M. Colombo, D. Sarker, L. Bolondi, G. Vaccaro, W.P. Harris, Z. Chen, R.A. Hubner, T. Meyer, W. Sun, J.J. Harding, E.M. Hollywood, J. Ma, P.J. Wan, M. Ly, J. Bomalaski, A. Johnston, C.-C. Lin, Y. Chao, L.-T. Chen
Journal: Annals of Oncology (2018)
AB0077 High-mobility group box 1 mediated monosodium urate crystal-induced nlrp3 inflammasome activation in human macrophages
Authors: S.-K. Kim, J.-Y. Choe, S.-H. Park, H. Lee
Journal: Annals of the Rheumatic Diseases (2018)

<h3>Background</h3> High-mobility group box 1 (HMGB1) was identified originally as a highly conserved non-histone DNA-binding factor and recently noted as a potent inflammatory mediator under inflammatory conditions. <h3>Objectives</h3> This study is to investigate the inflammatory cascade between HMGB1 protein and activation of NLRP3 inflammasome in human macrophage under uric acid-induced inflammation. <h3>Methods</h3> The study used human U937 macrophage cell line under stimulation with monosodium urate (MSU) crystal or HMGB1. Total reactive oxygen species (ROS) were measured by flow cytometry. Interleukin-1b (IL-1b), NLRP3, TXNIP, HMGB1, NF-kB, IkBa, and caspase-1 protein expression was detected using western blotting. IL-1b, IL-18, caspase-1, and HMGB1 gene expression were assessed by quantitative real-time polymerase chain reaction. Intracellular HMGB1 expression was assessed by immunofluorescent staining with MitoTracker Red. <h3>Results</h3> MSU crystals induced HMGB1 and ROS production by activation of NF-kB signal pathway in human macrophages. HMGB1 mRNA expression was markedly attenuated under stimulation using TXNIP siRNA. Enhanced release of IL-1 was noted through increased HMGB1 expression and TXNIP-mediated NLRP3 infammasome activation under stimulation of MSU. Combination of MSU and HMGB1 augmented NLRP3 inflammasome, compared to either MSU or HMGB1 stimulation. <h3>Conclusions</h3> This study demonstrated that HMGB1 is a crucial molecule for ROS-mediated TXNIP and NLRP3 inflammasome activation in uric acid-induced inflammation. <h3>Disclosure of Interest</h3> None declared
The locus coeruleus drives disinhibition in the midline thalamus via a dopaminergic mechanism
Authors: B. Sofia Beas, Brandon J. Wright, Miguel Skirzewski, Yan Leng, Jung Ho Hyun, Omar Koita, Nicholas Ringelberg, Hyung-Bae Kwon, Andres Buonanno, Mario A. Penzo
Journal: Nature Neuroscience (2018)
Reversible De-differentiation of Mature White Adipocytes into Preadipocyte-like Precursors during Lactation
Authors: Qiong A. Wang, Anying Song, Wanze Chen, Petra C. Schwalie, Fang Zhang, Lavanya Vishvanath, Lei Jiang, Risheng Ye, Mengle Shao, Caroline Tao, Rana K. Gupta, Bart Deplancke, Philipp E. Scherer
Journal: Cell Metabolism (2018)
Evidence of the high prevalence of neurological disorders in nonsyndromic X-linked recessive ichthyosis: a retrospective case series
Authors: B. Rodrigo-Nicolás, E. Bueno-Martínez, A. Martín-Santiago, J. Cañueto, A. Vicente, A. Torrelo, L. Noguera-Morel, A. Duat-Rodríguez, C. Jorge-Finnigan, I. Palacios-Álvarez, J.L. García-Hernández, D.F. Sebaratnam, R. González-Sarmiento, A. Hernández-Martín
Journal: British Journal of Dermatology (2018)

X-linked recessive ichthyosis (XLI) is a relatively common type of ichthyosis caused by a deficiency in the steroid sulfatase (STS) enzyme. It is the only type of ichthyosis that can be both syndromic and nonsyndromic. Typical clinical features include dark-brown scale of variable size favouring the extensor surfaces of the extremities.To characterize clinically nonsyndromic XLI, with a particular focus on extracutaneous manifestations.This was a multicentre retrospective review of clinical findings from a case series of patients with a clinical and genetic diagnosis of XLI.We identified 30 patients with XLI belonging to 25 different families carrying a deletion in the STS locus. All patients had dark scales of variable size on the extensor surfaces of the extremities. Lack of flexural involvement and pruritus were common but inconsistent findings, whereas palmoplantar hyperlinearity was absent in all but one patient. A history of orchiopexy was present in 10% and thus was more common than expected vs. the general population (3%). Neurological disorders including epilepsy (13%) and attention deficit hyperactivity disorder (ADHD; 30%) were over-represented in patients with XLI.This was a retrospective study with a limited number of patients. In the absence of confirmatory genetic testing and family history of the disease, dark-brown scale of the extensor surfaces and the absence of palmoplantar hyperlinearity appear to be the most reliable clinical findings supporting a diagnosis of XLI. Dermatologists should be aware of the high prevalence of ADHD and epilepsy in patients with nonsyndromic XLI.
Specific Inhibition of Hepatic Lactate Dehydrogenase Reduces Oxalate Production in Mouse Models of Primary Hyperoxaluria
Authors: Chengjung Lai, Natalie Pursell, Jessica Gierut, Utsav Saxena, Wei Zhou, Michael Dills, Rohan Diwanji, Chaitali Dutta, Martin Koser, Naim Nazef, Rachel Storr, Boyoung Kim, Cristina Martin-Higueras, Eduardo Salido, Weimin Wang, Marc Abrams, Henryk Dudek, Bob D. Brown
Journal: Molecular Therapy (2018)

Primary hyperoxalurias (PHs) are autosomal recessive disorders caused by the overproduction of oxalate leading to calcium oxalate precipitation in the kidney and eventually to end-stage renal disease. One promising strategy to treat PHs is to reduce the hepatic production of oxalate through substrate reduction therapy by inhibiting liver-specific glycolate oxidase (GO), which controls the conversion of glycolate to glyoxylate, the proposed main precursor to oxalate. Alternatively, diminishing the amount of hepatic lactate dehydrogenase (LDH) expression, the proposed key enzyme responsible for converting glyoxylate to oxalate, should directly prevent the accumulation of oxalate in PH patients. Using RNAi, we provide the first in vivo evidence in mammals to support LDH as the key enzyme responsible for converting glyoxylate to oxalate. In addition, we demonstrate that reduction of hepatic LDH achieves efficient oxalate reduction and prevents calcium oxalate crystal deposition in genetically engineered mouse models of PH types 1 (PH1) and 2 (PH2), as well as in chemically induced PH mouse models. Repression of hepatic LDH in mice did not cause any acute elevation of circulating liver enzymes, lactate acidosis, or exertional myopathy, suggesting further evaluation of liver-specific inhibition of LDH as a potential approach for treating PH1 and PH2 is warranted. Primary hyperoxalurias (PHs) are autosomal recessive disorders caused by the overproduction of oxalate leading to calcium oxalate precipitation in the kidney and eventually to end-stage renal disease. One promising strategy to treat PHs is to reduce the hepatic production of oxalate through substrate reduction therapy by inhibiting liver-specific glycolate oxidase (GO), which controls the conversion of glycolate to glyoxylate, the proposed main precursor to oxalate. Alternatively, diminishing the amount of hepatic lactate dehydrogenase (LDH) expression, the proposed key enzyme responsible for converting glyoxylate to oxalate, should directly prevent the accumulation of oxalate in PH patients. Using RNAi, we provide the first in vivo evidence in mammals to support LDH as the key enzyme responsible for converting glyoxylate to oxalate. In addition, we demonstrate that reduction of hepatic LDH achieves efficient oxalate reduction and prevents calcium oxalate crystal deposition in genetically engineered mouse models of PH types 1 (PH1) and 2 (PH2), as well as in chemically induced PH mouse models. Repression of hepatic LDH in mice did not cause any acute elevation of circulating liver enzymes, lactate acidosis, or exertional myopathy, suggesting further evaluation of liver-specific inhibition of LDH as a potential approach for treating PH1 and PH2 is warranted.
Convection-enhanced delivery for diffuse intrinsic pontine glioma: a single-centre, dose-escalation, phase 1 trial
Authors: Mark M Souweidane, Kim Kramer, Neeta Pandit-Taskar, Zhiping Zhou, Sofia Haque, Pat Zanzonico, Jorge A Carrasquillo, Serge K Lyashchenko, Sunitha B Thakur, Maria Donzelli, Ryan S Turner, Jason S Lewis, Nai-Kong V Cheung, Steven M Larson, Ira J Dunkel
Journal: The Lancet Oncology (2018)
Impact of the driving pressure on mortality in obese and non-obese ARDS patients: a retrospective study of 362 cases
Authors: Audrey De Jong, Jeanne Cossic, Daniel Verzilli, Clément Monet, Julie Carr, Mathieu Conseil, Marion Monnin, Moussa Cisse, Fouad Belafia, Nicolas Molinari, Gérald Chanques, Samir Jaber
Journal: Intensive Care Medicine (2018)
Measuring Low-Density Lipoprotein Cholesterol
Authors: Neil J. Stone
Journal: JAMA Cardiology (2018)

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AB0393 Electrocardiographic disturbances in patients with rheumatoid arthritis using antimalarial drugs
Authors: P. Rodriguez Henriquez, F.J. Reyes-Muciño, L.M. Amezcua-Guerra
Journal: Annals of the Rheumatic Diseases (2018)

<h3>Background</h3> Cardiac involvement is present in 7% to 35% of patients with rheumatoid arthritis (RA). Anecdotal case series have associated the use of antimalarial drugs with the presence of electrocardiographic disturbances in patients with RA <h3>Objectives</h3> To assess whether the use of antimalarial drugs is associated with the presence of electrocardiographic abnormalities in RA patients. The main clinical associations were also evaluated <h3>Methods</h3> A single-centre, case-control (1:1 proportion) medical chart review study was performed. A total of 144 patients with RA (ACR/EULAR 2010 criteria) were studied, and grouped as follows: 72 with a history of antimalarial drug use (case) and 72 who never used antimalarial drugs (control). We excluded individuals with a history of ischaemic heart disease or an already diagnosed disturbance of the cardiac rhythm/conduction. <h3>Results</h3> A total of 37 cases of electrocardiographic disturbances were found in patients receiving antimalarials (51%), but only 18 cases (25%) in the control group (p=0.001), giving an odds ratio (OR) of 3.17 (95% CI, 1.57 to 6.42). Rhythm disturbances observed in the antimalarial group were: left anterior branch hemiblock (n=12, 32.4%), right branch block (n=10; 27.02%), 1st degree AV block (n=5; 13.5%), bifascicular block (n=4; 10.8%), posterior hemiblock of the left branch (n=3; 8.1%), incomplete left bundle branch block (n=2, 5.4%), and left bundle branch block (n=1, 2.7%). Right bundle branch block was the only statistically significant conduction disorder (27.02% vs 5.5%with p value of 0.004 [OR 11.45 1.43–92]). The average dose of hydroxychloroquine was 3.02±0.41 mg/kg, while the dose of chloroquine was 2.23±0.13 mg/kg). A history of prednisone consumption also was associated with an increased risk to present an electrical cardiac disturbance (p=0.005; OR 9.73, from 2.00 to 47.32). <h3>Conclusions</h3> In this study, the use of antimalarial drugs was associated with an increased risk to present disturbances of the cardiac rhythm/conduction in patients with established RA. <h3>References</h3> [1] Seferović PM, et al. Cardiac arrhythmias and conduction disturbances in autoimmune rheumatic diseases. Rheumatology (Oxford)2006;45:iv39–42. [2] Coskun S, et al. Cardiac involvement in patients with rheumatoid arthritis. Journal of Rheumatology2005;8:23–31. [3] Costedoat-Chalumeau N, et al. Heart conduction disorders related to antimalarials toxicity: an analysis of electrocardiograms in 85 patients treated with hydroxychloroquine for connective tissue diseases. Rheumatology2007;46:808–810. <h3>Disclosure of Interest</h3> None declared
Abstract 15575: Polymorphism at the Telomerase Ribonucleic Acid Component Gene Interacts With Mediterranean Diet to Influence Inflammation Status and its Relationship With Telomere Attrition, From the CORDIOPREV Clinical Trial
Authors: Francisco Gomez-Delgado, Antonio Garcia-Rios, Isabel Perez-Corral, Andreea Corina, Javier Delgado-Lista, Oriol Rangel, Juan Francisco Alcala-Diaz, Jose Lopez-Miranda, Pablo Perez-Martinez
Journal: Circulation (2016)

Introduction:  Telomere attrition has been associated with several age-related diseases such as cardiovascular disease. Telomerase RNA Component (TERC) genetic variants have been associated with leukocyte telomere length (LTL); whereas fatty acids are key metabolic regulators that can interact with genetic factors and influence in age-related diseases.   Hypothesis:  To evaluate whether genetic variability at the TERC gene interacts with fatty acid profile and two healthy diets (low-fat diet,    Methods:  Inflammation status (high sensitivity C-reactive protein (hsCRP)), glucose metabolism (glucose, insulin, glycated haemoglobin (HbA1c) and homeostasis model assessment of insulin resistance (HOMA-IR)), fatty acids, LTL and single nucleotide polymorphisms (SNPs) of the TERC gene ( rs12696304, rs16847897  and  rs3772190 ) were determined in 1,002 patients from the CORDIOPREV clinical trial (NCT00924937).   Results:  We report an interaction between TERC  rs12696304  SNP and plasma total MUFA levels affecting hsCRP ( P  =0.03) and LTL ( P  =0.01). Among subjects with MUFA levels above the median, carriers of the CC genotype showed lower hsCRP and higher LTL than G-allele carriers (GG + CG). After 12 months of dietary intervention, we found also a significant gene–diet interaction between TERC  rs12696304  SNP and Mediterranean diet ( P  =0.03). Specifically, CC subjects displayed a greater decrease in hsCRP than G-allele carriers.   Conclusions:  Our findings suggest that the TERC  rs12696304  SNP interacts with MUFA improving inflammation status and telomere attrition related with CHD.  Moreover, Mediterranean diet intervention induced a better inflammatory profile in CC subjects compared with the G-allele carriers. Our results add further support to the concept of personalised nutritional advice tailored to the genetic make-up of CHD patients.
SP0011 Is there an universal translator? which (ANIMAL) models tell us most?
Authors: J. Distler
Journal: Annals of the Rheumatic Diseases (2018)

<h3></h3> We will review the key features of currently available preclinical models of SSc, highlight their strengths and limitations and analyse, which subsets of SSc patients individual models mimic. We will also discuss how to employ models to evaluate drug candidates with different modes of actions and how to combine different models for an optimal preclinical portfolio. <h3>Disclosure of Interest</h3> J. Distler Shareholder of: 4D Science, Grant/research support from: Anamar, Active Biotech, Array Biopharma, aTyr, BMS, Bayer Pharma, Boehringer Ingelheim, Celgene, Galapagos, GSK, Inventiva, Novartis, Sanofi-Aventis, RedX, UCB, Consultant for: Actelion, Active Biotech, Anamar, Bayer Pharma, Boehringer Ingelheim, Celgene, Galapagos, GSK, Inventiva, JB Therapeutics, Medac, Pfizer, RuiYi and UCB
SP0004 Extra spinal manifestations in axial spondyloarthritis
Authors: M. Dougados
Journal: Annals of the Rheumatic Diseases (2018)

<h3></h3> The extra-spinal manifestations in axial spondyloarthritis can be categorised in 2 mains sub-groups: the extra-spinal rheumatological manifestations on one hand and the extra rheumatological manifestations in the other hand. The extra spinal manifestations comprise synovitis, dactylitis and enthesitis and extra rheumatological manifestations comprise mainly skin psoriasis, uveitis and inflammatory bowel disease. In terms of diagnostic approach in case of a patient presenting with axial symptoms, the probability to detect any extra-spinal manifestations in the past-history of the patients and/or concomitantly to the axial symptoms is very high (&gt;75% at the baseline visit of the DESIR cohort including patients with recent onset axial symptoms suggestive of spondyloarthritis). These manifestations (based on data from different databases: for example the DESIR cohort and the ASAS-COMOSPA cross-sectional study) will be described The long term follow-up of patients presenting with axial spondyloarthritis emphasises the possibility to observe these extra-spinal manifestations overtime (incident cases). These data are strong arguments to Justify the inclusion of these extra–spinal manifestations in the set of classification criteria of axial spondyloarthritis Justify specific investigations (mainly interview and physical exam ) of the patients at the time of the diagnosis Justify a systematic monitoring of these different extra–spinal manifestations during the entire course of the disease <h3>Disclosure of Interest</h3> None declared