tbuckley/cabot-search huggingface dataset:
FRI0497 The Interleukin-1 Inhibitor Canakinumab for Familial Mediterranean Fever: Long-Term Beneficial Effect in A Cohort of 14 Patients
Authors: K. Laskari, P. Boura, G.N. Dalekos, A. Garyfallos, D. Karokis, D. Pikazis, L. Settas, G. Skarantavos, E. Tsitsami, P.P. Sfikakis
Journal: Annals of the Rheumatic Diseases (2016)

<h3>Background</h3> Interleukin-1 (IL-1) is a major mediator of the inflammatory cascade in Familial Mediterranean Fever (FMF) and an established therapeutic target (1). <h3>Objectives</h3> To assess the efficacy and safety of the IL-1 inhibitor Canakinumab in adult and adolescent patients with refractory FMF. <h3>Methods</h3> Fourteen patients (7 men) with genetically confirmed FMF, fulfilling the Tel Hashomer criteria, aged 38.5 years (median, range 13–70), with median disease duration of 14 years and active disease despite colchicine (n=9) or both colchicine and anakinra (n=5), received Canakinumab 150mg subcutaneously (sc) every 4 (n=7) or 6 (n=2) or 8 weeks (n=5) for a median of 18 months (range 7–53). Canakinumab was given as monotherapy in 8; 6 patients received concomitant treatment with colchicine and/or corticosteroids. <h3>Results</h3> Eleven out of 14 patients (79%) achieved complete clinical remission (median time 2 months), while normalization of all laboratory parameters associated with inflammation occurred in 92% of patients (median time 3 months). The remaining patients achieved partial responses. Response was maintained until the last visit in all but 4 patients. Reducing the Canakimumab administration interval led to suppression of disease activity in three cases, whereas in another two patients drug administration intervals could be increased without disease exacerbation. The corticosteroid dose was significantly reduced during follow up. The FMF50 score (2) was achieved by 50% and 86% of patients at 1 and 12 months, respectively. Canakinumab was well tolerated; one patient experienced an urinary tract infection and another one a viral gastroenteritis. <h3>Conclusions</h3> The rapid and sustained response to Canakinumab in the majority of our patients, together with the favorable safety profile, encourages its further use in FMF. <h3>References</h3> Ter Haar N et al. Ann Rheum Dis. 2013;72(5):678–85. Ozen S et al. Ann Rheum Dis. 2014;73(5):897–901. <h3>Disclosure of Interest</h3> None declared
Characteristics of Medicare Payments to Dermatologists in 2013
Authors: Adewole S. Adamson, Stacie B. Dusetzina
Journal: JAMA Dermatology (2016)

Characteristics of Medicare Payments to Dermatologists in 2013Dermatologists provide many types of medical and procedural services, yet little is known about how Medicare payments to dermatologists vary by the type of service performed.In April 2014, the Centers for Medicare & Medicaid Services released the Medicare Provider Utilization and Payment Data: Physician and Other Supplier Public Use File (POSPUF). 1For the first time, the public had access to Medicare payments to health care professionals.Although these payments are not sufficient to determine value, physician payments are part of the value equation and will be a continued source of scrutiny.This study examines the characteristics of Medicare payments to dermatologists.
Prolonged Survival of Chronic Lymphocytic Leukemia Cells Induced by Sphingosine 1-Phosphate Is Mediated by the G Protein-Coupled Receptor S1P1 and Involves Erk/MAP Kinase, but Not Akt Signaling.
Authors: Gabriele Seitz, Sedat Yildirim, Andreas Boehmler, Lothar Kanz, Robert Mohle
Journal: Blood (2006)
THU0137 Non-Adherence To Subcutaneous Biological Therapy in Patients with Rheumatoid Arthritis. Arco Study
Authors: J. Calvo-Alén, C. Marras Fernandez-Cid, I. Monteagudo, G. Salvador Alarcόn, T.R. Vázquez-Rodríguez, J.V. Tovar Beltrán, P. Vela, F. Maceiras, M.S. Bustabad Reyes, J.A. Román Iborra, E. Peirό Callizo, L. Cea-Calvo, M.J. Arteaga, S. Fernández, J. Rosas, E. Raya Alvarez
Journal: Annals of the Rheumatic Diseases (2016)

<h3>Background</h3> Lack of adherence to medication in rheumatoid arthritis (RA) can worsen disease activity, but data on adherence to biological drugs is scarce. <h3>Objectives</h3> To evaluate RA patients adherence to the subcutaneous (SC) biological treatment prescribed by the physician during the first year of treatment. <h3>Methods</h3> Retrospective study in RA patients ≥18 years-old from 42 Spanish hospitals, which had been prescribed a SC biological drug 12 to 18 months prior to the study visit. Data were collected from patients9 clinical records and hospital pharmacy drug-dispensing logs. Adherence was evaluated through the Medication Possession Ratio (MPR) (actual number of days covered by the taken medication/theoretical number of days covered by the prescribed medication). The number of days theoretically covered was calculated considering the interval of administration of the prescribed biological drug, if induction dose was prescribed, and if there were suspension periods or changes in the dose. The actual number of days covered was calculated with the number of vials that the patient had taken from the hospital pharmacy during the study period. Non-adherence was diagnosed if MPR was ≤80%. <h3>Results</h3> 364 patients were included (age 54.9 years [12.5]; 77.5% women, median RA duration 7.8 years). The initial interval of administration of the biological drug was weekly (44.2%), biweekly (39.1%) and monthly (17.3%); the median duration of the period studied was 14.8 months. For the primary analysis, 363 patients were valid, of which 52 patients were non-adherent (MPR ≤80%) to the prescribed schedule (14.3%; 95% CI: 11.1–18.3). There were no differences in adherence by age ranges, gender or other socio-demographic factors, such as level of education, smoking habit or alcohol intake, employment status, comorbidities or polypharmacy (all, p= NS). Non-adherence was less frequent in patients with a monthly administration schedule (6.4%) than in patients with a weekly (17.4%, p=0.034) or biweekly administration (14.4%, p=0.102), and slightly more frequent in those receiving induction (21.6% vs 12.5% without induction, p=0.068). No differences were observed between patients who used syringes or pen devices, or in patients who had periods of treatment interruption. <h3>Conclusions</h3> In RA patients, 14.3% were non-adherent to the prescribed SC biological medication during the first year of treatment, with no differences related to socio-demographic variables. Adherence was higher in those patients with monthly subcutaneous administration. <h3>Acknowledgement</h3> The ARCO study was funded by Merck Sharp &amp; Dohme of Spain. <h3>Disclosure of Interest</h3> None declared
PTH-147 A More Restrictive Transfusion Policy for Upper Gastrointestinal Haemorrhage Would Save Resources Without Compromising Clinical Outcome
Authors: L Summers, E Wesley, S Prasad
Journal: Gut (2016)

<h3>Introduction</h3> Upper GI bleeding (UGIB) is the most common indication for transfusion in England, comprising 14% of all transfusions.<sup>1</sup> Evidence has demonstrated a restrictive as opposed to liberal transfusion policy improves clinical outcome.<sup>2</sup> In addition to the known clinical benefits, our aim was to asssess whether a more restrictive transfusion policy would save significant resources. <h3>Methods</h3> We conducted a prospective audit of consecutive patients presenting to the Royal Devon and Exeter hospital with UGIB during a 9 week period from 7.12.12. The case notes and laboratory data were reviewed. Unstable patients, those with evidence of massive UGIB and those not receiving blood transfusion were excluded. Liberal transfusion was defined as haemoglobin &gt;80 g/L pre-transfusion or a post transfusion haemoglobin &gt;90 g/L. We calculated the number of additional units transfused beyond these parameters. <h3>Results</h3> 79 patients presented with an UGIB. 42 patients received a total of 170 units of red blood cells. 7 patients were transfused 1 unit, 33 patients were transfused 2 units, and 28 patients received ≥3 units. 30/42 were transfused in a liberal manner, resulting in the potentially unnecessary use of 68 additional units at a cost of £8,302. <h3>Conclusion</h3> A significant number of patients received transfusions according to our definition of a liberal transfusion strategy, usually receiving several units at a time. A restrictive transfusion policy has been proven to be safe and would also result in significant cost savings. <h3>References</h3> 1 Wallis JP, Wells AW, Chapman CE. Changing indications for red cell transfusion from 2000 to 2004 in the North of England. <i>Transfus Med</i>. 2006;<b>16</b>:411–417. 2 Villanueva C, Colomo A, Bosch A, Concepcion M, Hernandez-Gea V, Aracil C Transfusion strategies for acute upper gastrointestinal bleeding. <i>N Engl J Med</i>. 2013;<b>368</b>:11–21. <h3>Disclosure of Interest</h3> None Declared
PTH-154 Gastrointestinal Late Effects of Cancer Care – Is There an Unmet Need in Primary Care?
Authors: R Stacey, H Ludlow, J Turner, J Green
Journal: Gut (2016)

<h3>Introduction</h3> Gastrointestinal late effects of pelvic cancer treatment are common and these patients are recognised as having an unmet need within the health service at present. We have recently set up a dedicated clinic to deal with late effects of cancer care in Cardiff, however noted that all our referrals were received from secondary care oncology. We did not receive any primary care referrals. We aimed to assess the current knowledge of general practitioners in Wales and assess their referral practice. <h3>Methods</h3> An online questionnaire comprising of 22 questions were sent via email to all GP practices in Wales. 63 GPs replied and completed the questionnaire. The results were collected anonymously using survey monkey online software. <h3>Results</h3> 63 GPs replied (39 GP Partners, 9 Salaried GPS, 1 GP locum, 14 GP registrars) from all regions of Wales. 78.33% reported having previously seen patients with new gastrointestinal symptoms after treatment for pelvic cancer, (e.g. after radiotherapy) in their practice. The prevalence of late effects of cancer care was underestimated by 96.55% and the impact on quality of life was also underestimated by 39.65% as described in the literature.<sup>1</sup> Most GPs reported that although they were aware of the condition, they only saw less than 1 case per month. 25.86% of GPs felt confident with basic cases, but would need sub-specialty advise with more complex cases felt that they needed expert help for most cases. All other respondents felt either were not confident/ had no experience or somewhat confident, but have minimal experience. 84.48% wanted more education on this topic. <h3>Conclusion</h3> GPs appear to underestimate the prevalence and impact of late effects on patients. They recognise that expertise is needed to treat these patients and that they lack both this training and frequent exposure to these patients, but also are keen for further education. The treatment of GI late effects has benefitted in secondary care within the last 5 years due to increased exposure, but this has yet to filter down into primary care. There is almost certainly an unmet need in primary care which needs to be addressed via better communication of services available in secondary care and current practice. Clinicians, NHS trusts and supportive charities should aim to improve the awareness of this under recognised condition. <h3>Reference</h3> 1. Andreyev J. Gastrointestinal symptoms after pelvic radiotherapy: a new understanding to improve management of symptomatic patients. <i>Lancet Oncol</i> 2007 Oct 25;<b>8</b>(11):1007–17. <h3>Disclosure of Interest</h3> None Declared
PWE-052 Auditing The Diagnostic Process of Coeliac Disease in Paediatrics
Authors: H Jarad, S McCann
Journal: Gut (2016)

<h3>Introduction</h3> The management of coeliac disease (CD) is clear, however agreement on paediatric diagnosis is debated. NICE dictates that TTG testing should be used front line, with EMA added if TTG is borderline, and referral to a specialist for a final diagnosis. ESPGHAN states that symptomatic patients with TTG &gt; 10 times the upper limit (ULN) will most likely have villous atrophy and recommends the use of HLA DQ2/DQ8 as a rule in test to obviate the need for a biopsy. This audit reviewed our adherence to these guidelines over 4 years and investigated the reliability of serological and genetic testing as indicators of CD. <h3>Methods</h3> TTG positive data (&gt;7.0 kU/L) in those &lt;16 years was collected from the Telepath Database System, from 02/11/11 to 11/11/15. Only data on newly suspected CD patients was used, n = 34. Monitored CD patients or those managed elsewhere were excluded. TTG testing was carried out on the Thermo Fisher ImmunoCAP 250. HLA results, biopsy results, and referral times were collated from the Advantis Document System. <h3>Results</h3> Of the 34 patients, 28 were diagnosed with CD. 13 underwent a biopsy; 12 were positive and 1 was negative, most likely due to a GFD. 11 nonbiopsied patients underwent HLA testing; all 11 were positive. 4 had neither biopsy or HLA testing; 2 were diagnosed on positive TTG and EMA results, 1 was diagnosed on symptoms and a high TTG, and 1 was diagnosed on TTG alone, due to a biopsy refusal. The average dietician referral time post initial TTG was 164 days; 122 days for those undergoing HLA testing and 220 days for those undergoing a biopsy. Of the remaining patients, 4 were not diagnosed with CD and their average TTG = 13.4 kU/L; 2 had normal EMA results, 1 had a negative biopsy, and 1 had a normal repeat TTG. Of all patients, 24 had TTG &gt; 10 ULN; 22 were diagnosed with CD, and 2 had early CD, but a GFD was not instigated. 1 was asymptomatic and did not consent to a biopsy, and 1 had a positive EMA and was awaiting HLA testing. 10 patients had TTG &lt; 10 ULN; 2 were tested for EMA, 1 tested for HLA, and 5 had a biopsy. <h3>Conclusion</h3> TTG was always used front line, but EMA was not assessed in all borderline cases. Symptomatic patients with TTG &gt; 10 ULN, regardless of biopsy or HLA testing, were diagnosed with CD or early CD. HLA testing was not always utilised, with 46% of cases having a biopsy, however in all instances of HLA testing this occurred to obviate a biopsy. There is no indication of a minimum referral time to a dietician in the guidelines, but referral times are longer in those who underwent a biopsy, with the need to maintain a normal diet. The diagnostic process of CD is becoming streamlined as more consultants opt for serological and genetic testing, allowing for quicker management. <h3>Disclosure of Interest</h3> None Declared
Intestinal Marginal Zone B-Cell Lymphoma of MALT Type: Clinical Manifestation and Outcome of a Rare Disease.
Authors: Sung Yong Oh, Won Seog Kim, Baek-Yeol Ryoo, Hye Jin Kang, Yeon Hee Park, Kihyun Kim, Young Hye Ko, Hyuk-Chan Kwon, Eunmi Nam, Jae-Hoon Lee, Jung Han Kim, Hyo-Jin Kim
Journal: Blood (2006)
A Phase I Dose Escalation Study of RP6530, a Novel, Dual PI3K-γδ Inhibitor, for Patients with Relapsed or Refractory Hematologic Malignancies
Authors: Lydia Scarfò, Prajak J Barde, Claudia Fazi, Uday Kumar, Srikant Viswanadha, Swaroop Vakkalanka, Paolo Ghia, Andres J.M. Ferreri
Journal: Blood (2014)
SP0077 Differential Diagnosis between Primary and Secondary Raynaud's Phenomenon
Authors: V. Smith
Journal: Annals of the Rheumatic Diseases (2016)

Clinical rheumatologists frequently get patients with Raynaud9s phenomenon (discoloration of extremities upon exposure to cold or stress) referred. The challenge is to distinguish patients with a primary Raynaud9s phenomenon (not connected to any connective tissue disease) from patients with a secondary Raynaud9s phenomenon (due to a connective tissue disease). A large 20-year follow-up study (Koenig Arthritis Rheum 2008) of patients presenting at baseline with merely the Raynaud9s phenomenon and no sign of a connective tissue disease has taught the scientific community that if a CTD is to develop in such a population then it is systemic sclerosis. Subsequently it makes sense to screen for SSc in a Raynaud9s phenomenon population. In 1992 LeRoy and Medsger proposed criteria to distinguish primary from secondary Raynaud9s phenomenon. Retrospective validation of these criteria allow correct classification of a patient in one clinical consultation in 89%. Amongst other criteria, a "normal" capillaroscopy is obligatory to be able to speak of a "primary" Raynaud9s phenomenon. Conversely patients with a secondary RP due to SSc have the combination of a SSc-specific antibody and a scleroderma pattern on capillaroscopy. These criteria have prospectively been validated. The triade of RP, SSc-specific antibody and scleroderma pattern on capillaroscopy has a high positive predictive value for the development of SSc, whilst the absence of SSc-specific antibodies and a scleroderma pattern has a high negative predictive value for the non-occurrence of SSc. <h3>Disclosure of Interest</h3> None declared
FRI0401 Safety of Resuming Tumor Necrosis Factor Inhibitors in Ankylosing Spondylitis Patients Concomitant with The Treatment of Active Tuberculosis: A Retrospective Nationwide Registry of The Korean Society of Spondyloarthritis Research
Authors: H.W. Kim, S.-H. Lee, H.J. Baek, T.-H. Kim, Y.-G. Kim
Journal: Annals of the Rheumatic Diseases (2016)

<h3>Background</h3> Patients who develop an active tuberculosis infection (TI) during tumor necrosis factor (TNF) inhibitor treatment typically discontinue TNF inhibitor and receive standard anti-tuberculosis treatment. However, there is currently insufficient information on patient outcomes following resumption of TNF inhibitor treatment during ongoing anti- tuberculosis treatment. <h3>Objectives</h3> Our study designed to investigate the safety of resuming TNF inhibitors in ankylosing spondylitis (AS) patients who developed TI as a complication of the use of TNF inhibitors. <h3>Methods</h3> Through the nationwide registry of the Korean Society of Spondyloarthritis Research, 3929 AS patients were recruited who were prescribed TNF inhibitors between June 2003 and June 2014 at fourteen referral hospitals. Clinical information about the patients who experienced TI after exposure to TNF inhibitors was analyzed. The clinical features of resumers and non-resumers of TNF inhibitor were compared and the outcome of TI was surveyed individually. <h3>Results</h3> Fifty-six AS patients were treated for TI associated with TNF inhibitors. Among them, 23 patients resumed TNF inhibitors and these patients were found to be exposed to TNF inhibitors for a longer period of time and experienced more frequent disease flare up after discontinuation of TNF inhibitors compared with those who did not resume. Fifteen patients resumed TNF inhibitors during anti-tuberculosis treatment (early resumers) and 8 after completion of TI treatment (late resumers). Median time to resuming TNF inhibitor from TI was 3.3 and 9.0 months in the early and late resumers, respectively. TI was treated successfully in all resumers and did not relapse in any of them during follow-up (median 33.8 (IQR; 20.8–66.7) months). <h3>Conclusions</h3> TI were treated successful in our AS patients, even when given concomitantly with TNF inhibitors. We suggest that early resuming TNF inhibitors in AS patients could be safe under effective coverage of the TI. <h3>Disclosure of Interest</h3> None declared
Placenta Growth Factor Induces 5-Lipoxygenase-Activating Protein Via Hypoxia-Inducible Factor-1α and Contributes to Increased Leukotrienes in Sickle Cell Disease.
Authors: Vijay K Kalra, Nitin Patel, Caryn Gonsalves, Minyang Yang, Punam Malik
Journal: Blood (2008)
Validation of the prognostic value of histologic scoring systems in primary sclerosing cholangitis: An international cohort study
Authors: Elisabeth M. G. de Vries, Manon de Krijger, Martti Färkkilä, Johanna Arola, Peter Schirmacher, Daniel Gotthardt, Benjamin Goeppert, Palak J. Trivedi, Gideon M. Hirschfield, Henriette Ytting, Ben Vainer, Henk R. van Buuren, Katharina Biermann, Maren H. Harms, Olivier Chazouilleres, Dominique Wendum, Astrid D. Kemgang, Roger W. Chapman, Lai Mun Wang, Kate D. Williamson, Annette S. H. Gouw, Valerie Paradis, Christine Sempoux, Ulrich Beuers, Stefan G. Hübscher, Joanne Verheij, Cyriel Y. Ponsioen
Journal: Hepatology (2016)

Histologic scoring systems specific for primary sclerosing cholangitis (PSC) are not validated. We recently determined the applicability and prognostic value of three histological scoring systems in a single PSC cohort. The aim of this study was to validate their prognostic use and reproducibility across a multicenter PSC cohort. Liver biopsies from PSC patients were collected from seven European institutions. Histologic scoring was performed using the Nakanuma, Ishak, and Ludwig scoring systems. Biopsies were independently scored by six liver pathologists for interobserver agreement. The prognostic value of clinical, biochemical, and all three histologic scoring systems on predicting composite endpoints 1 (PSC‐related death and liver transplantation), 2 (liver transplantation), and 3 (liver‐related events), was assessed using univariable and multivariable Cox proportional hazards modeling. A total of 119 PSC patients were identified, and the median follow‐up was 142 months. During follow‐up, 31 patients died (20 PSC‐related deaths), 31 patients underwent liver transplantation, and 35 patients experienced one or more liver‐related events. All three staging systems were independent predictors of endpoints 2 and 3 (Nakanuma system: hazard ratio [HR], 3.16 [95% confidence interval (CI), 1.49‐6.68] for endpoint 2 and HR, 2.05 [95% CI, 1.17‐3.57] for endpoint 3; Ishak system: HR, 1.55 [95% CI, 1.10‐2.18] for endpoint 2 and HR, 1.43 [95% CI, 1.10‐1.85] for endpoint 3; Ludwig system: HR, 2.62 [95% CI, 1.19‐5.80] for endpoint 2 and HR, 2.06 [95% CI, 1.09‐3.89] for endpoint 3). Only the Nakanuma staging system was independently associated with endpoint 1: HR, 2.14 (95% CI, 1.22‐3.77). Interobserver agreement was moderate for Nakanuma stage (κ = 0.56) and substantial for Nakanuma component fibrosis (κ = 0.67), Ishak stage (κ = 0.64), and Ludwig stage (κ = 0.62). Conclusion : We confirm the independent prognostic value and demonstrate for the first time the reproducibility of staging disease progression in PSC using the Nakanuma, Ishak, and Ludwig staging systems. The Nakanuma staging system—incorporating features of chronic biliary disease—again showed the strongest predictive value. (H epatology 2017;65:907‐919).
A Novel Fusion of RARA to the PRKAR1A Gene, Encoding the Regulatory Subunit Type-I α of Cyclic AMP Dependent Protein Kinase A, in a Variant Acute Promyelocytic Leukaemia.
Authors: Alberto Catalano, Mark A. Dawson, Karthiga Somana, Stephen S. Opat, Lynda J. Campbell, Anthony P. Schwarer, Harry J. Iland
Journal: Blood (2006)