tbuckley/cabot-search huggingface dataset:
SAT0271 Hepatosplenomegaly: A Complication of Persistent Subclinical Inflammation in Children with Familial Mediterranean Fever?: Table 1.
Authors: E. Comak, G. Duygulu, E. Isıyel, F. Kılıçbay, M. Koyun, S. Akman
Journal: Annals of the Rheumatic Diseases (2016)

<h3>Background</h3> Familial Mediterranean Fever (FMF) is a hereditary autoinflammatory disorder characterized by attacks of painful inflammation. Some patients with FMF have subclinical inflammation persisting between the attacks. This subclinical inflammation may leads to many important clinical complications such as anemia, splenomegaly, decreased bone density, and amyloidosis. <h3>Objectives</h3> The aims of this study were to evaluate sonographic findings in patients with familial Mediterranean fever (FMF) during attack-free periods and examine whether there is an association between hepatosplenomegaly and <i>inflammatory</i> markers in FMF. <h3>Methods</h3> Medical records of children with FMF were reviewed during attack-free periods. Demographic features, clinical and laboratory findings, hematological data (leukocyte, hemoglobin and platelet counts),er<i>ythrocyte</i> sedimentation rate (ESR), SAA and C-reactive protein (CRP) levels, type of MEFV mutation, family history of FMF were collected from the hospital9s computerized database following approval by the local ethics committee. <h3>Results</h3> A total of 136 patients, 74 (54.4%) girls were included in this cross sectional study during non-attack period. Median age of 11.25 years (2–18 years) and median follow-up period were 18 months (3–129 months) at time of sonographic evaluation. Thirty one patients (22.8%) had parental consanguinity and 472 (52.9%) patients had family history of FMF. Thirty three patients (24.3%) patients were homozygous, 61 (44.9%) patients were compound heterozygous and 42 (30.9%) patients were heterozygous for MEFV gene. Hepatomegaly and splenomegaly were found in 22 (16.2%) and 32 patients (23.5%), respectively. Ten patients had both hepatomegaly and splenomegaly. Spleen size had a negative correlation with platelet count and a positive correlation with ESR, CRP and SAA (all p&lt;0.05). Also, there was significant positive correlation between liver size and CRP levels (p&lt;0.05). White blood cell count, hemoglobin, urinary protein-creatinine ratio were not associated with spleen and liver size. And also, patients with splenomegaly had significantly higher SAA, CRP and ESR levels than the patients with normal spleen size (Table 1). <h3>Conclusions</h3> Hepatomegaly and splenomegaly are not uncommon in FMF, which might be due to the chronic inflammatory characteristics of the disease. We speculate that FMF is a chronic inflammatory disease rather than episodic illness. <h3>Disclosure of Interest</h3> None declared
PTH-036 Single Centre Experience of The Use of Needle Knife Papillotomy During Difficult ERCP: Is It Safe and Should It Be Used By, and Taught to Trainees
Authors: N Rabb, C Beckett, S Jowett, S Moreea
Journal: Gut (2016)

<h3>Introduction</h3> Despite previous concerns regarding the safety of needle knife papillotomy (NKP) there is data to support its efficacy and safety in the context of increasing bile duct cannulation at ERCP.<sup>1</sup> This data predominantly originates from high volume tertiary centres outside the UK. We audited our experiences of using NKP in a secondary care centre where wire-guided cannulation is the standard method for accessing the bile duct and the overall ERCP complication rate is &lt;5%, focusing on success of bile duct cannulation in relation to pathology, safety of the procedure and complication rate. <h3>Methods</h3> Electronic databases and medical records were interrogated to retrieve data for analysis on all ERCP cases using NKP between 2010–2015. The following data was collected: patient (pt) demographics, indications for ERCP and subsequent complications. <h3>Results</h3> 1843 ERCP were performed on 1362 pts (817 female (F), 545 male (M), age range (AR) 16–103). NKP was performed in 69 cases (3.7% 37F 32 M, AR 21–98). In the papillotomy cohort indications for ERCP were stone disease (40); stricturing disease (22); others including unexplained pancreatitis (4), bile leak (2), sphincter of Oddi dysfunction (1). Successful bile duct cannulation was achieved during the index procedure in 50 cases (25M 25 F). Of the remaining 19 cases, 11 (5M 6 F) had bile duct cannulation achieved during a repeat procedure via the papillotomy: global success rate 88%. The remaining 6 cases (4F 2 M) were managed with alternative interventions (5 PTC, 1 surgical). NKP was more successful for stone disease: an initial procedure success rate of 73% vs. 64% for non-stone disease, increasing to 95% with repeat procedure. 5/6 (83%) cases requiring alternative interventions had non-stone disease. Complications: 4/69 (5.8%) cases were complicated by pancreatitis; all were managed conservatively with no associated mortality. 3/4 (75%) required hospitalisation for &lt;3 days and 1 for &gt;10 days. There were 4 cases of bleeding (5.8%) with 2/4 (50%) requiring blood transfusion and endoscopic therapy to achieve haemostasis. Perforation occurred in 2 cases (2.8%); both were managed conservatively, 1 case died. <h3>Conclusion</h3> The global success rate for bile duct cannulation after pre-cut NKP was 88% which supports its efficacy for obtaining bile duct access in otherwise difficult ERCP cases. The overall complication rate of 13% associated with the technique is high, though comparable to other published data. Acknowledgement that NKP may be less fruitful in cases of non-stone related disease may help to guide our practice further, particularly by helping identify cases that may be more appropriate for trainees to tackle. <h3>Reference</h3> 1 Baillie J. <i>GIE</i><b>79</b>(5): 2014. <h3>Disclosure of Interest</h3> None Declared
Polyomavirus Reactivation and Immune Responses to Kidney-Specific Self-Antigens in Transplantation
Authors: Michael E. Seifert, Muthukumar Gunasekaran, Timothy A. Horwedel, Reem Daloul, Gregory A. Storch, Thalachallour Mohanakumar, Daniel C. Brennan
Journal: Journal of the American Society of Nephrology (2016)

Humoral immune responses against donor antigens are important determinants of long-term transplant outcomes. Reactivation of the polyomavirus BK has been associated with de novo antibodies against mismatched donor HLA antigens in kidney transplantation. The effect of polyomavirus reactivation (BK viremia or JC viruria) on antibodies to kidney-specific self-antigens is unknown. We previously reported excellent 5-year outcomes after minimization of immunosuppression for BK viremia and after no intervention for JC viruria. Here, we report the 10-year results of this trial ( n =193) along with a nested case-control study ( n =40) to explore associations between polyomavirus reactivation and immune responses to the self-antigens fibronectin (FN) and collagen type-IV (Col-IV). Consistent with 5-year findings, subjects taking tacrolimus, compared with those taking cyclosporin, had less acute rejection (11% versus 22%, P =0.05) and graft loss (9% versus 22%, P =0.01) along with better transplant function (eGFR 65±19 versus 50±24 ml/min per 1.73 m 2 , P &lt;0.001) at 10 years. Subjects undergoing immunosuppression reduction for BK viremia had 10-year outcomes similar to those without viremia. In the case-control study, antibodies to FN/Col-IV were more prevalent during year 1 in subjects with polyomavirus reactivation than in those without reactivation (48% versus 11%, P= 0.04). Subjects with antibodies to FN/Col-IV had more acute rejection than did those without these antibodies (38% versus 8%, P =0.02). These data demonstrate the long-term safety and effectiveness of minimizing immunosuppression to treat BK viremia. Furthermore, these results indicate that polyomavirus reactivation associates with immune responses to kidney-specific self-antigens that may increase the risk for acute rejection through unclear mechanisms.
AB1120-HPR Application of The Innovative Rheumatoid Arthritis Medication Assement Tool within A Pharmaceutical Care Model
Authors: L. Grech, V. Ferrito, L. Camilleri, A. Serracino Inglott, L.M. Azzopardi
Journal: Annals of the Rheumatic Diseases (2016)

<h3>Background</h3> Pharmacists must adapt and evolve in order to face challenges and sustain the profession to continuously provide optimum personalised patient care to rheumatoid arthritis patients. Rheumatoid arthritis medication assessment tool, RhMAT is an innovative evidence-based instrument developed specifically to assess pharmacetherapy adherence to rheumatoid arthritis evidence-based guidelines and recommendations. <h3>Objectives</h3> The objective of the study was to use the RhMAT within an outpatient rheumatology clinic to identify pharmaceutical care issues and assess therapeutic management of rheumatoid arthritis patients. <h3>Methods</h3> Rheumatoid arthritis patients regularly attending the rheumatology clinic at Mater Dei Hospital eligible to participate in the study were offered a pharmaceutical care session. The pharmacist screened each patients9 pharmacetherapy using the RhMAT at two time points, Phase 1 (time =0 months) and Phase 2 (time = approximately 12 months).The RhMAT adherence rate for each patient was calculated at both time points. A score of 50% of less was determined as low adherence, a score between 51% and 74% showed intermediate adherence and a score of 75% or more was determined as high adherence to the RhMAT.Gaps in non-adherence to the RhMAT which led to pharmaceutical care issues were documented and discussed with the clinician. Data analysis of the mean score at both phases was analysed using Wilcoxon signed rank test, SPSS verion 22. <h3>Results</h3> A total of 78 patients (52 females) participated in the study. The mean age was 64 years (SD 12).The majority of the patients (55%) were prescribed at least one traditional disease modifying anti-rheumatic drug (tDMARD) whereas 45% were on biologic disease modifying anti-rheumatic drug (bDMARD). The most common tDMARD prescribed was methotrexate (n=52 patients). The mean RhMAT adherence rate at phase 1 was 81.7%. Following the pharmacist9s contribution whereby the identified gaps leading to non-adherence such as lack of appropriate monitoring or lack of drug therapy counselling, were discussed as necessary with the respective clinician and the patient, the mean RhMAT adherence rate at the follow up session at Phase 2 statistically significantly increased to 85.2% (Wilcoxon signed rank test p value &lt;0.05). <h3>Conclusions</h3> The RhMAT is an innovative tool which aids pharmacists to identify adherence of prescribed pharmacotherapy in accordance to established guidelines. At the same time, the RhMAT allows pharmacists to identify gaps in individual patients care which lead to pharmaceutical care issues. Through the RhMAT, pharmacists further improve the quality of care offered to rheumatoid arthritis patients. <h3>Disclosure of Interest</h3> None declared
Recruitment of Infants with Sickle Cell Anemia to a Phase III Trial:Data from the BABY HUG Study.
Authors: Lynn W Wynn, Lane Faughnan, Daner Li, Winfred Wang, Brenda Martin, Tracy Kelly, Caroline Reed, Phillip Seaman, Joan Marasciulo, Elizabeth Rackoff, Scott T. Miller, Tally Hustace, Glenda Thomas, Jennifer Marshall, Jennifer McDuffie, MaryLou MacDermott, Ellen Debenham, Mary Murphy, Renee C. Rees
Journal: Blood (2008)
Determinants for Iron Overload-Related Disease in Siblings of Probands with Clinically Detected HFE Hereditary Hemochromatosis: The Hemochromatosis Family Study.
Authors: Esther M.G. Jacobs, Jan C.M. Hendriks, Herman G. Kreeftenberg, Richard A. de Vries, Joannes J.M. Marx, Cees Th. B. M. van Deursen, Anton F.H. Stalenhoef, Andre L.M. Verbeek, Dorine W. Swinkels
Journal: Blood (2008)
PTH-087 Severity of Bile Acid Malabsorption Does Not Correlate with Length of Ileal Resection or Response to Bile Salt Sequestrant Therapy in Crohn’s Disease
Authors: K Lo, H Riyat, Y Prasad, S Subramanian
Journal: Gut (2016)

<h3>Introduction</h3> Bile acid malabsorption (BAM) is a common cause of diarrhoea in Crohn’s disease (CD) patients with ileal resection and can lead to complications such as renal and biliary stone disease. BAM is usually diagnosed by selenium labelled homo-taurocholic acid test (<sup>75</sup> SeHCAT) but its availability is limited. Thus, large proportions of resected CD patients either remain undiagnosed or subject to empirical therapy. There is a paucity of studies examining the correlation between length of ileal resection and severity of BAM which will be of particular use to clinicians with no recourse to diagnostic testing for BAM. <h3>Methods</h3> We identified all CD patients with a prior surgical resection who underwent <sup>75</sup> SeHCAT testing at our institute. Testing was based on the treating clinician’s discretion. The length of resected ileum was recorded from histopathology report. Correlation between length of resected ileum and percentage retention on <sup>75</sup> SeHCAT was assessed using a Spearman’s correlation test. Response to treatment with bile salt sequestrant and <sup>75</sup> SeHCAT retention values (using a cut point of 5%) was analysed using Fisher’s exact test. <h3>Results</h3> A total of 40 patients were identified with a mean age of 45 (SD±13), of which 6 (15%) were men. The median length of resected ileum was 22.8 cms (range 5.8–61.0 cms) with a median of 1 resection (range 1–4). Overall, 38 patients (95%) had <sup>75</sup> SeHCAT retention values of &lt;10% and 36 (90%) had retention of &lt;5%. There was no correlation between <sup>75</sup> SeHCAT retention and length of ileal resection (Spearman’s rho: -0.19, P = 0.24). Data on response to treatment was available for 30 patients, of who 21 (70%) responded and 9 (30%) failed to respond to bile salt sequestrant therapy. Three of the responders (14%) had <sup>75</sup> SeHCAT values of &gt;5% and the remaining 18 (86%) had values &lt;5%. All of the non-responders had <sup>75</sup> SeHCAT retention values &lt;5% (Fisher’s exact, two tailed P = 0.53). <h3>Conclusion</h3> There was no correlation between length of ileal resection and severity of BAM as defined by <sup>75</sup> SeHCAT retention values. Response to bile salt sequestrant therapy was not dependent on <sup>75</sup> SeHCAT retention values though overall clinical response was good. <h3>Disclosure of Interest</h3> K. Lo: None Declared, H. Riyat: None Declared, Y. Prasad: None Declared, S. Subramanian Speaker bureau with: MSD, Abbvie, Shire, Ferring, Actavis, Conflict with: Advisory board Boehringer-Ingelheim, Vifor pharma, MSD, Abbvie, Shire
Increased Plasma Levels of NGAL and IL-18 Correlate with Reduced GFR Providing Two Novel Biomarkers for Kidney Injury Assessment: Evidence of Early Tubuloglomerular Defect in Patients with HbS/b-Thalassemia
Authors: Ioannis Papassotiriou, Alexandra Margeli, Eugenia Hantzi, Evangelos Terpos, Ersi Voskaridou
Journal: Blood (2008)
Organotypic Epigenetic Signature Predicts Bone and Marrow Niche Forming Capacity of Stromal Progenitors in a Novel Mouse Model in Vivo.
Authors: Andreas Reinisch, Nathalie Etchart, Nicole A Hofmann, Margareta Frühwirth, Anna Ortner, Daniela Thaler, Birgit Feilhauer, Udo F Hartwig, Christine Beham-Schmid, Slave Trajanosky, Wolfgang Wagner, Frauke Alves, Katharina Schallmoser, Michael Andreeff, Dirk Strunk
Journal: Blood (2012)
Diagnostic accuracy of the Ottawa Ankle and Midfoot Rules: a systematic review with meta-analysis
Authors: Paula R Beckenkamp, Chung-Wei Christine Lin, Petra Macaskill, Zoe A Michaleff, Chris G Maher, Anne M Moseley
Journal: British Journal of Sports Medicine (2016)

Objective To review the diagnostic accuracy of the Ottawa Ankle and Midfoot Rules and explore if clinical features and/or methodological quality of the study influence diagnostic accuracy estimates. Design Systematic review with meta-analysis. Data sources MEDLINE, EMBASE, CINAHL, SPORTDiscus and Cochrane Library. Eligibility criteria for selecting studies Primary diagnostic studies reporting the accuracy of the Rules in people with ankle and/or midfoot injury were retrieved. Diagnostic accuracy estimates, overall and for subgroups (patient’s age, profession of the assessor and setting of application), were made. Sensitivity analyses included studies with a low risk of bias and studies where all patients received radiographs. Results 66 studies were included. Ankle and Midfoot Rules presented similar accuracies, which were homogeneous and high for sensitivity and negative likelihood ratios and poor and heterogeneous for specificity and positive likelihood ratios (mean, 95% CI pooled sensitivity of Ankle Rules: 99.4%, 97.9% to 99.8%; specificity: 35.3%, 28.8% to 42.3%). Sensitivity of the Ankle Rules was higher in adults than in children, but the profession of the assessor did not appear to influence accuracy. Specificity was higher for Midfoot than for Ankle Rules. There were not enough studies to allow comparison according to setting of application. Studies with a low risk of bias and where all patients received radiographs provided lower accuracy estimates. Specificity heterogeneity was not explained by assessor training, use of imaging in all patients and low risk of bias. Conclusions Study features and the methodological quality influence estimates of the diagnostic accuracy of the Ottawa Ankle and Midfoot Rules.
Cell Death Mechanisms Induced by INNO-406, a Novel Tyrosine Kinase Inhibitor for Imatinib-Resistant Ph+ Leukemia.
Authors: Yuri Kamitsuji, Souichi Adachi, Motonobu Watanabe, Hiroshi Matsubara, Yasuhiro Mizushima, Yoshimi Hiraumi, Ken-Ichiro Watanabe, Hideo Tanaka, Junya Kuroda, Eishi Ashihara, Shinya Kimura, Taira Maekawa, Tatsutoshi Nakahata
Journal: Blood (2006)
Autologous 111In-Labeled Platelet Sequestration Studies in Patients with Primary Immune Thrombocytopenia (ITP): A Report From the United Kingdom Registry.
Authors: Ameet Sarpatwari, Drew Provan, Ravin Sobnack, Sebhat Erqou, F. W. David Tai, Simon Sanderson, Adrian Newland
Journal: Blood (2009)
ATPS-48A HIGH-THROUGHPUT SCREEN IDENTIFIES microRNA-1300 AS A POTENTIAL THERAPEUTIC microRNA CAUSING CYTOKINESIS FAILURE AND APOPTOSIS IN GLIOBLASTOMA CELLS
Authors: Marjorie Boissinot, Matthew Adams, Julie Higgins, Henry King, Lynette Steele, Ruth Morton, Josie Hayes, Alastair Droop, Jacqueline Bond, Darren Tomlinson, Susan Short, Sean Lawler
Journal: Neuro-Oncology (2015)

INTRODUCTION: There is a pressing need for new therapeutic approaches for the treatment of glioblastoma (GBM). MicroRNAs are single-stranded 22-24nt non-coding RNAs which function by reducing translation or causing degradation of target mRNAs. MiR-1300 was identified in a high-throughput miRNA mimic library screen as a promising therapeutic candidate, inducing cell death. METHOD: To validate the cytotoxic effect of miR-1300, a panel of four established cell lines (U251,LN229,U373,KNS42)and two patient-derived stem-like cell lines were utilised for RTqPCR, imaging and flow-cytometry to assess endogenous expression levels, cellular morphology, apoptosis, and cell cycle delay respectively. Bioformatic approaches identified potential miR-1300 targets as mediators of this effect and validation of this involvement in the cytotoxic phenotype using siRNA knock-down is being investigated. Validation of the direct targeting by miR-1300 is being performed using 3' UTR Luciferase assays. RESULTS: Expression, of miR-1300, by transfection of oligonucleotide mimics, in our panel of GBM cells consistently led to G2M arrest followed by apoptosis within 72h. The associated phenotype is binucleation, indicative of cytokinesis failure.Preliminary data suggest this may be mediated through ECT2, a predicted target of miR-1300 known to be involved in formation of the cleavage furrow. CONCLUSION: We have identified a highly potent pro-apoptotic microRNA that may be exploitable for GBM therapy. Importantly, there is no detectable endogenous miR-1300 expression in any GBM samples or cell lines. On-going work will confirm the targets of miR-1300 that drive this effect as well as exploring potential approaches to deliver this as a biotherapeutic agent.
PTH-160 ‘One Stop’ Jaundice Pathway in a District General Hospital: Evaluation of a pilot Pathway
Authors: W On, N Mirza, A Lewis, A Tang, K Koss, R Saravanan
Journal: Gut (2016)

<h3>Introduction</h3> Jaundice warrants urgent investigations to exclude significant underlying disease. The British Society of Gastroenterology (BSG) commissioned a report in 2013 highlighting the importance of having a streamlined pathway for investigation of jaundice.<sup>1</sup> Macclesfield District General Hospital (MDGH) has a catchment area of approximately 0.2 million and established a jaundice pathway in 2013. The pathway serves as a <b>‘ONE STOP INVESTIGATION’</b> for jaundiced patients who have been referred from general practice (GP) or other specialties. Initial ultrasound triage by a Consultant Radiologist with hepatopancreatobiliary interest is followed by same day cross sectional imaging as appropriate. Our aim is to evaluate the performance/outcome of this pilot pathway. <h3>Methods</h3> A retrospective review of all patients on the jaundice pathway was undertaken between March 2013 to October 2015.Data was collated and analysed from hospital electronic records and radiology database. <h3>Results</h3> Thirty six patients were identified (19 males, 17 females)on the dedicated jaundice pathway . The median age of the patients was 73.5 years (range 36–92). 86% of patients (31/36) were investigated as outpatients. The median bilirubin level of the investigated patients was 122 µmol/L (range 29–497). The aetiologies of jaundice in this cohort of patients were: malignancies (n = 12), gallstone disease (n = 11), liver cirrhosis (n = 4), parenchymal liver disease (n = 6) and miscellaneous causes (n = 5). The median time from referral to investigations was 4 days (range 0–14) and the median time from investigations to an outcome was 2 days (range 0–20). Outcome was defined by a definitive management plan made after a review by a consultant and/or discussion in a MDT meeting. Overall, the median number of days from a patient being referred to obtaining an outcome was 8 days (range 0–28). For patients with malignancies (n = 12), the median time from referral to outcome was 9.5 days (range 6–28). This exceeds the expectations for the 2 week referral (2 WW) rule for patients with suspected malignancies. The patients with malignancies proceeded to undergo: endoscopic retrograde cholangiopancreatography (n = 5), percutaneous transhepatic cholangiography (n = 3), surgery (n = 2) and palliative treatment (n = 2). <h3>Conclusion</h3> The jaundice pathway at MDGH has proven to be an effective pathway for patients to undergo investigations for jaundice. Majority of patients with jaundice <b>can be managed as outpatients.</b> Adequate resource management is needed to facilitate this dedicated pathway. This has now been <b>adopted as a Gold Standard by the Central Manchester cancer network.</b> <h3>Reference</h3> 1 http://www.bsg.org.uk/images/Commissioning_report/BSG_Acute_Jaundice.pdf <h3>Disclosure of Interest</h3> None Declared