tbuckley/cabot-search huggingface dataset:
SP0180 FOOT ORTHOSES IN RA
Authors: Jim Woodburn
Journal: Annals of the Rheumatic Diseases (2019)

<h3></h3> In this presentation I will: (1) Provide a general overview of the indications and use of foot orthoses in rheumatoid arthritis (RA) <sup>1</sup>, (2) Explore mechanisms of action with respect to preserving, maintaining and restoring foot biomechanics<sup> 2</sup>, (3) Summarise current evidence on the use of foot orthoses for managing foot pain, disability, deformity and quality of life by drawing on recently published systematic reviews and meta-analyses<sup>3</sup>. Further I will direct delegates to national, European and International evidence-based clinical guidelines where they exist<sup>1,4</sup>, and (4) Introduce new technology advances with regards to materials and digital design and manufacturing concepts<sup>2</sup>. I will conclude the presentation by setting out future directions and priorities for both clinical practice and research and innovation. <h3>References:</h3> [1] Hennessy K, Woodburn J, Steultjens M. Clinical practice guidelines for the foot and ankle in rheumatoid arthritis: a critical appraisal. J Foot Ankle Res. 2016 Aug 19;9:31. [2] Gibson KS, Woodburn J, Porter D, Telfer S. Functionally optimized orthoses for early rheumatoid arthritis foot disease: a study of mechanisms and patient experience. Arthritis Care Res (Hoboken). 2014 Oct;66(10):1456-64. [3] Hennessy K, Woodburn J, Steultjens MP. Custom foot orthoses for rheumatoid arthritis: A systematic review. Arthritis Care Res (Hoboken). 2012 Mar;64(3):311-20. [4] Chapman LS, Redmond AC, Landorf KB, Rome K, Keenan AM, Waxman R, Alcacer-Pitarch B, Siddle HJ, Backhouse MR. Foot orthoses for people with rheumatoid arthritis: a survey of prescription habits among podiatrists. J Foot Ankle Res. 2019 Jan 25;12:7. <h3>Disclosure of Interests:</h3> None declared
UFBoot2: Improving the Ultrafast Bootstrap Approximation
Authors: Diep Thi Hoang, Olga Chernomor, Arndt von Haeseler, Bui Quang Minh, Le Sy Vinh
Journal: Molecular Biology and Evolution (2017)

The standard bootstrap (SBS), despite being computationally intensive, is widely used in maximum likelihood phylogenetic analyses. We recently proposed the ultrafast bootstrap approximation (UFBoot) to reduce computing time while achieving more unbiased branch supports than SBS under mild model violations. UFBoot has been steadily adopted as an efficient alternative to SBS and other bootstrap approaches. Here, we present UFBoot2, which substantially accelerates UFBoot and reduces the risk of overestimating branch supports due to polytomies or severe model violations. Additionally, UFBoot2 provides suitable bootstrap resampling strategies for phylogenomic data. UFBoot2 is 778 times (median) faster than SBS and 8.4 times (median) faster than RAxML rapid bootstrap on tested data sets. UFBoot2 is implemented in the IQ-TREE software package version 1.6 and freely available at http://www.iqtree.org.
PB2316 EFFECT OF VITAMIN D SUPPLEMENTATION ON CLINICAL OUTCOMES OF SICKLE CELL DISEASE
Authors: A. Adly, F. Ebeid, S. Sallam, A. Gohar
Journal: HemaSphere (2019)

Background: Sickle cell disease (SCD) is one of the most common hereditary hemoglobinopathy. Vitamin D deficiency has emerged as a public health focus in recent years and it is one of the most common nutritional conditions among persons with SCD and there are characteristics specific to SCD that may contribute to this phenomenon including decreased appetite, inability to absorb nutrients due to damage to the intestinal mucosa, as well as an increased basal metabolic rate and higher nutritional demands to sustain normal physiologic functioning. Aims: Assess the level of vitamin D in pediatric SCD patients. Assess the effect of vitamin D supplementation on its clinical outcome including; frequency of vaso‐occlusive crisis and hospital admission and rate of infections. Methods: The current study is a follow‐up prospective study that included 50 children and adolescents with SCD recruited from the regular attendants Hematology/Oncology Clinic in Pediatric Hospital, Ain Shams University. Patients with SCD as confirmed by qualitative and quantitative analysis of hemoglobin using high performance liquid chromatography (HPLC) at their steady state were recruited. Patients with any inflammatory condition within one month prior to sample collection and those on vitamin D and/or calcium supplementation in the last 6 months were excluded. All patients subjected to clinical assessment and laboratory assessment including complete blood count, liver and kidney function tests and serum 25‐OH vitamin D level. Patients were divided into two groups according to their serum vitamin D level above and below 30 nmol and those with insufficient level were treated for three months and prospectively followed up for clinical outcome and had their vitamin D reassessed Results: There was a high prevalence of vitamin D deficiency (46%); 23 patients had vitamin D level less than 30nmol (Group 1) and 37 patients had vitamin D level more than 30 nmol (Group 2). Upon comparison between group 1 and group 2, there were no significant differences as regard clinic‐demographic data and treatment modalities. Patients with SCD and vitamin D deficiency had statistically significant higher history of fracture than those of with normal vitamin D level. There were statistically significant lower frequencies of joint and bone pain, sickle crisis, hospital admission and infections in patients with normal level of vitamin D. There was statistically significant improvement in vitamin D level after supplementation. There was statistically significant improvement as regard number of crisis after 3 months of vitamin D therapy (P = 0.043), with no statistically significant difference other studied parameters. There was statistically significant negative correlation between vitamin D level and number of crisis after 3 months of vitamin D therapy Summary/Conclusion: The high prevalence of vitamin D deficiency in Egyptian SCD patients highlights the importance of early diagnosis and management on the clinical outcome of those patients
Water balloon-induced orbital fracture in an aviator
Authors: Timothy E. Holland, David M. Smith, Guy N. Gibson, Jared G. Brinkerhoff
Journal: Military Medical Research (2019)

Orbital fractures are common injuries found in facial trauma. Typical etiologies of orbital fractures include motor vehicle collisions and assault. We report the case of a 32-year-old male who suffered an orbital fracture from a water balloon. Additionally, we describe the aeromedical complications that may result from this injury. Finally, we attempt to answer the question of when a patient may return to flying after sustaining such an injury through review of the literature. A 32-year-old male pilot with the United States Air Force was at an outdoor event with his unit when he was struck with a water balloon launched from a sling shot into his left orbit. Shortly afterwards, he had an onset of subcutaneous emphysema and was escorted to a nearby Emergency Department. Computed tomography identified an orbital fracture with associated orbital and subcutaneous emphysema. The patient was evaluated by a plastic surgeon and was determined not to be a surgical candidate. Four weeks later, he returned to flying status. Water balloons are thought to be safe and harmless toys. However, when coupled with slingshots, water balloons can become formidable projectiles capable of significant orbital injury including orbital fractures. These injuries are concerning to aviators, as the most common sites for fractures of the orbit are the thin ethmoid and maxillary bones adjacent to the sinuses. At altitude, gases in the sinuses may expand and enter the orbit through these fractures, which may suddenly incapacitate the flyer. It is important for flight surgeons to identify and assess these individuals to determine suitability for flying.
AB0668 ASSOCIATION OF ANTI-RNA POLYMERASE III ANTIBODY AND BREAST IMPLANTS RUPTURE IN ITALIAN PATIENTS WITH SYSTEMIC SCLEROSIS
Authors: Maria Grazia Lazzaroni, Cristian Caimmi, Eugenia Bertoldo, Franco Franceschini, Angela Tincani, Paolo Airò
Journal: Annals of the Rheumatic Diseases (2019)

<h3>Background:</h3> Several epidemiological studies have investigated the link between silicone breast implants (SBI) and Systemic Sclerosis (SSc). Although discordant data were reported, a recent analysis of SBI followed by United States Food and Drug administration post approval studies, including nearly 100,000 individuals, described an association of SBI with a higher rate of SSc (Standardized incidence ratio 7.00), compared with normative data<sup>1</sup>. The analysis of clinical associations in patients with SSc is complicated by the heterogeneity of the disease, both on immunological and clinical terms. Interestingly, a specific association of anti-RNA polymerase III antibody (anti-RNAP3) and SBI in Japanese patients with SSc was described in a single-center cohort<sup>2</sup>. Moreover, an association of anti-RNAP3 with breast cancer, particularly when synchronous with SSc onset, was also demonstrated<sup>3,4</sup>. <h3>Objectives:</h3> To evaluate the association of SBI with SSc in Italian patients classified according to their SSc-related autoantibodies. <h3>Methods:</h3> 562 consecutive women with SSc, classified according the 2013 aCR/EULAR criteria, were evaluated for the presence of SSc-specific autoantibodies (anti-Topoisomerase-I (anti-Topo-I), anticentromere (ACA), and anti-RNAP3) in two Italian University centres. For each patient, history of breast cancer and SBI were recorded. <h3>Results:</h3> Anti-RNAP3 antibodies were found in 4.6%, anti-Topo-I in 22.2% and aCA in 57.3% of total SSc women (Table 1). Comparing anti-RNAP3+ vs anti-RNAP3- patients, a significantly higher frequency of breast cancer (15.3% vs 4.1%; p=0.026), SBI (11.5% vs 1.1%; p=0.006) and SBI rupture (11.5% vs 0.2%; p=0.0003) was found. Notably a higher rate of SBI rupture without breast cancer in anti-RNAP3+ vs anti-RNAP3- was also noted (2/22 (9.1%) vs 0/514 (0%); p=0.0016). <h3>Conclusion:</h3> In this large Italian cohort, a higher prevalence of SBI and SBI rupture in anti-RNAP3+ compared to anti-RNAP3- SSc patients was confirmed, regardless of the history of breast cancer. This preliminary observation should be confirmed in multicentre cohorts, particularly regarding the connection between SSc and SBI rupture. <h3>References</h3> [1] Coroneos CJ, et al. Ann Surg2019;269:30-6. [2] Saigusa R, et al. J Dermatol2016; 43:808-10. [3] Moinzadeh P, et al. Arthritis Res ther. 2014;16(1):R53. [4] Lazzaroni MG, et al. J Rheumatol. 2017;44(5):639-47. <h3>Disclosure of interests:</h3> Maria Grazia Lazzaroni: None declared, Cristian Caimmi: None declared, Eugenia Bertoldo: None declared, Franco Franceschini: None declared, angela Tincani Consultant for: UCB, Pfizer, abbvie, BMS, Sanofi, Roche, GSK, alphaSigma, Lillly, Jannsen, Cellgene, Novartis, Paolo airò: None declared
Abstract 1838: Mechanisms of the Maintenance of Atrial Fibrillation: Role of the Complex Fractionated Atrial Electrograms Region Assessed by Non-contact Mapping
Authors: Hiroshige Yamabe, Kenji Morihisa, Yasuaki Tanaka, Takashi Uemura, Hisao Ogawa
Journal: Circulation (2007)

Background: Complex fractionated atrial electrograms (CFAE) have been implicated as the substrate of atrial fibrillation (AF). However, its role for the maintenance of AF has not been well clarifie...
Abstract 13492: Usefulness of Aortic Valve Area Measurement Obtained by Single-Beat Recording of Double-Envelope Technique in Patients With Aortic Stenosis and Atrial Fibrillation
Authors: Hiroki Oe, Nobuhisa Watanabe, Yuko Ohno, Rika Takemoto, Madoka Ikeda, Hitomi Sakamoto, Kunihisa Kohno, Kazufumi Nakamura, Hiroshi Morita, Hiroshi Ito
Journal: Circulation (2014)

Introduction: In patients with aortic stenosis (AS) and atrial fibrillation (AF), the conventional continuity equation to evaluate aortic valve area (AVA) is cumbersome, because 5 to 10 cycles are required to ensure accuracy of results. Double-envelope (DE) is obtained by a single continuous-wave Doppler envelope with double density velocity profiles; the inner envelope represents flow across the left ventricular outflow tract and outer envelope represents flow across the aortic valve orifice. Hypothesis: The aim of this study to evaluate the usefulness of the AVA calculated from single-beat recorded inner and outer envelopes in patients with AS and AF. Methods: Eighteen AS patients (74.6±7.2 years old) with AF were examined by transthoracic echocardiography. The conventional AVA (PW/CW technique) was calculated from nonsimultaneously measured LVOT flow and AVO flow in randomly picked up 8 cardiac cycles, respectively. The AVA (DE technique) was calculated from the inner and outer envelopes simultaneously recorded. A single-beat AVA was calculated from simultaneously recorded inner and outer envelopes (by DE technique) when the preceding RR interval/pre-preceding RR interval = 1. Bland-Altman analysis was used to compare the results of two techniques and estimate the reproducibility. Results: DE profiles were successfully obtained in all patients, and mean AVA (PW/CW technique) was 1.09±0.19 cm2. AVA by Single-beat DE technique showed good correlation with that by PW/CW technique (r=0.90), and the mean bias in the AVA measurements between by PW/CW technique and by DE technique was 0.048 cm2. (Figure) Conclusions: AVA in patients with AS and AF obtained by Single-beat DE technique was feasible and in good agreement with that by PW/CW technique. We suggest that DE technique should be considered to estimate the subtle temporal change of AVA in patient with AS and AF more simply and accurately.
OP0050 ADDITIVE VALUE AND DIAGNOSTIC ACCURACY OF DUAL-ENERGY CT FOR THE DIAGNOSIS OF GOUT: A PROSPECTIVE STUDY IN SUBJECTS WITH UNCLASSIFIED MONO OR OLIGOARTHRITIS
Authors: Mihaela Gamala, Johannes W. G. Jacobs, Suzanne Linn-Rasker, Maarten Nix, Ben Heggelman, Pieternel Pasker, Jacob M. van Laar, Ruth Klaasen
Journal: Annals of the Rheumatic Diseases (2019)

<h3>Background:</h3> The latest diagnostic technique to visualize monosodium uric acid (MSU) depositions is Dual Energy CT scan (DECT).<sup>1</sup> <h3>Objectives:</h3> To assess the additive value and accuracy of DECT in diagnosing gout in a prospective study in subjects with unclassified arthritis. <h3>Methods:</h3> We included 100 consecutive patients with acute unclassified mono or oligo arthritis who presented to the outpatient clinic of the Department of Rheumatology of Meander Medical Centre, Amersfoort, the Netherlands, ClinicalTrials.gov NCT03038386. Eleven patients dropped out, see Figure. Patients underwent aspiration of the arthritic joint (see Table) and a DECT scan of hands and wrists, knees, ankles and feet bilaterally. The 2015 EULAR/ACR gout classification criteria were used to score the subjects, with and without DECT result, to determine the additive value of DECT. Sensitivity and specificity of DECT for diagnosing gout were calculated separately for at the joint and at individual persons' level, using as reference standards microscopy of synovial fluid (SF) and the gout classification criteria. <h3>Results:</h3> For demographic and clinical characteristics of the patients see Table 1, and for study data and outcomes see Figure and Table 2. Sixty-eight of 89 patients (76.4%) fulfilled the 2015 EULAR/ACR criteria for gout. Of the 98 patients with SF aspiration, 55 (56%) were positive for MSU; of these, 89 subjects underwent DECT, of whom 38 had a negative microscopy result. Of these 38 subjects, 14 (37%) met the gout classification criteria only after a positive DECT result. <h3>Conclusion:</h3> Our findings suggested that DECT could have an additive value to clinical algorithms in subjects with undifferentiated arthritis when microscopy of SF fails to demonstrate the presence of MSU crystals. <h3>References:</h3> [1] Choi HK, et al. Dual energy CT in gout: a prospectieve validation study. Ann Rheum Dis2012; 71:1466-71. <h3>Disclosure of Interests:</h3> Mihaela Gamala: None declared, Johannes W. G. Jacobs Grant/research support from: Roche, Consultant for: Roche, Suzanne Linn-Rasker: None declared, Maarten Nix: None declared, Ben Heggelman: None declared, Pieternel Pasker: None declared, Jacob M. van Laar Grant/research support from: Genentech, Consultant for: F. Hoffmann-La Roche, Ruth Klaasen: None declared
Abstract 13073: Epicardial YAP/TAZ Regulate an Immune Suppressive Response Following Myocardial Infarction
Authors: Vimal Ramjee, Manvendra K Singh, Feiyan Liu, Kurt A Engleka, Lauren J Manderfield, Eric N Olson, Jonathan A Epstein
Journal: Circulation (2015)

Ischemic heart disease constitutes the most prevalent type of heart disease in the US. Remodeling post-myocardial infarction (MI) is a multifaceted process driven by a strong immune response. Regulatory T-cells, a subset of CD4+ T-cells, have been shown to suppress the innate and adaptive immune response following myocardial injury to allow for less deleterious remodeling. To date, the precise mechanism by which injured myocardium recruits these suppressive immune cells remains unknown. Here, we show a novel role for the epicardium in suppressing the post-infarct inflammation response through recruitment of T-regulatory cells. The Hippo pathway is a signal transduction pathway, which has gained importance in determining organ size and is implicated as a critical regulator of tissue regeneration. Mice deficient in epicardial YAP/TAZ, two core effectors of the Hippo signaling pathway, develop profound pericardial inflammation, thoracic adhesions and myocardial fibrosis post-MI, with resultant cardiomyopathy and death. These mice demonstrate fewer suppressive T-regulatory cells in the injured myocardium, due to a deficiency of interferon-gamma, a known inducer of these T-cells. Collectively, these results suggest a novel role for Hippo signaling in immune regulation. In addition, our data suggest that the epicardium plays an important role in homing suppressive T-regulatory cells to injured myocardium so that the inflammation response may be attenuated following MI.
Abstract O.20: Deep RNA Sequencing Reveals a Transcriptional Profile of Cytotoxic T Lymphocyte Activation, Antigen Presentation, Immunoglobulin Production, and Type I Interferon Response in Kawasaki Disease Arteritis
Authors: Anne H Rowley, Kristine Wylie, Kwang-Youn A Kim, Adam J Pink, Rebecca Reindel, Susan C Baker, Stanford T Shulman, Amy Yang, Jan M Orenstein, Mark W Lingen, George Weinstock, Todd Wylie
Journal: Circulation (2015)

Introduction: Subacute/chronic arteritis (SA/C) and luminal myofibroblastic proliferation are the pathologic processes that can continue in Kawasaki Disease (KD) coronary arteries after the second week of illness, when acute neutrophilic necrotizing arteritis has subsided. The specific dysregulated immune pathways in SA/C arteritis have been unknown, hampering the development of effective immunomodulatory therapies for patients not responding to intravenous gammaglobulin therapy. Controversy exists as to whether the arteritis is lymphocyte or macrophage-mediated. Hypothesis: Based on our histopathologic studies, we hypothesized that the immune transcriptome of SA/C KD arteritis was primarily lymphocyte- rather than macrophage-mediated. Methods: RNA was isolated from paraffin-embedded CA tissues, and samples passing quality control assays were subjected to deep RNA sequencing (101 nt paired-end reads, 40-100million reads/sample). Reads were aligned to the human genome, and HTseq used to determine read counts. DESeq was used to test for differential gene expression in CA tissues from 8 KD children (median age=7 mo, median 4 wks and all &gt;2 weeks after onset) compared to 7 controls with normal CAs (median age=5 mo). Pathways analysis was performed using Ingenuity iReport™. Results: 1074 differentially expressed genes were identified with &gt;1.5-fold change and q-value &lt;0.05. The most significantly upregulated pathways included cytotoxic CD8 T lymphocyte, dendritic cell and toll-like receptor (TLR) signaling, and antigen presentation. Immunoglobulin and type I interferon-stimulated genes were significantly upregulated. 50 immune response genes encoding secreted proteins were upregulated and are candidate biomarkers of KD arteritis. Conclusions: The immune transcriptional profile in KD CA tissues after the second week of illness is primarily lymphocyte-mediated and compatible with an antiviral immune response. This first report of the KD CA transcriptome identifies specific dysregulated immune response pathways that can inform the development of new therapies for and biomarkers of KD vasculitis, and provides direction for future etiologic studies of this potentially fatal childhood illness.
PF739 SICKLE CELL DISEASE IN GERMANY IS HETEROGENEOUS AND RESULTS IN SEVERE MORBIDITY: ANALYSES FROM A NATIONAL REGISTRY
Authors: J. Kunz, S. Lobitz, J. Andrea, G. Regine, H. Cario, L. Oevermann, D. Hakimeh, L. Le Cornet, D. Beck, I. Karapanagiotou‐Schenkel, L. Tagliaferri, A. Kulozik
Journal: HemaSphere (2019)

Background: Sickle Cell Disease (SCD) in Germany exclusively affects immigrants and their descendants. Its increasing prevalence results in challenges for medical care for these patients. Aims: The registry aims at describing the epidemiology, clinical course and treatment of patients with SCD in Germany. Methods: To date, 367 patients have been included in the registry by 36 mainly pediatric institutions. At initial registration, basic data including date of birth, date and circumstances of diagnosis, genotype, country of origin, blood type and complications in the patient's history are reported. Annual follow‐ups document details on complications, laboratory parameters and treatment. Data of 327 patients were analyzed for this abstract. Results: Genotypes of registry patients were homozygous SCD in 75% of patients, HbSC disease in 12%, HbS/β 0 thal in 6% and HbS/β + thal in 7%. The main regions of origin were sub‐Saharan Africa (in total 69% of patients, including Ghana 19%, Nigeria 19%, Togo 9%) and the Middle East (in total 21%, including Lebanon 6%, Turkey 5%, Syria 4%). Although pilot projects for newborn screening for SCD were in place in some of the centers, only 35% of patients were diagnosed during their first year of life, 85% before the age of six. SCD was diagnosed on the occasion of presenting symptoms in 60% of patients. The mean age at registration was 12 years (median 10), with 10% of patients being older than 21 years at registration. The most frequent complications in the patients’ prior history were pain crises with hospitalization (67% of patients), acute chest syndrome (32%), splenic sequestration (15%), aplastic crisis (10%), stroke (4%) and sepsis (4%). During the first year of observation, 47% of the registry patients were hospitalized, 11% required treatment on an intensive care unit. Red blood cell transfusions were given to 27% of patients. 96% of patients with homozygous SCD or with HbS/β 0 thal at an age of 2 years or older were prescribed hydroxycarbamide, 76% of these patients under the age of 18 were annually examined by transcranial Doppler ultrasound (TCD). Summary/Conclusion: Patients with SCD in Germany are genetically and ethnically highly heterogeneous and suffer from severe morbidity. The diagnosis is frequently made too late because of the lack of a routine newborn screening for SCD. While the high rate of hydroxycarbamide prescription indicates good care at the institutions that contribute to the registry, the insufficient proportion of patients receiving routine TCD checks indicates an incomplete availability of the diagnostic infrastructure required for the optimal care of this challenging group of patients.