tbuckley/cabot-search huggingface dataset:
OC-035 The UK Multicentre Audit of Management and Outcome of Autoimmune Hepatitis (AIH). Overall Outcome and Its Associations
Authors: VM Gordon
Journal: Gut (2016)

<h3>Introduction</h3> There are limited data on outcome of AIH outside large centres. We have conducted an outcome audit of AIH in 28 UK centres of varying size. We report overall outcome results in this cohort. <h3>Methods</h3> We attempted to collect all prevalent cases since 2000 and all incident cases since 2007 by searching electronic patient letters, histology databases and hospital coding. Validation was by 1999 IAIHG diagnostic criteria. Information, on diagnosis, initial severity, treatment and outcome was entered into a web-based data collection system. <h3>Results</h3> There were 1267 patients (80% female, age at diagnosis (median (range) 55 years (8–86)). IAIHG criteria were not met in 79 (6%) but they were still treated as AIH. At diagnosis, cirrhosis (biopsy, varices, ascites or Fibroscan) was present in 317 (25%) patients and decompensation (ascites, oedema, encephalopathy, variceal bleed or MELD score &gt;15) at diagnosis was present in 282 (22%). Follow-up (time from diagnosis to last seen) was 4 (0–14) years. AIH relapse occurred in 371 patients (141 had ≥2 and 57 ≥ 3 relapses). De-novo cirrhosis developed in 78 (6%) patients, decompensation in 42, and hepatocellular cancer in 7. Fifteen patients died of liver disease, 28 had a liver transplant and 31 died of liver-unrelated causes. Five- and 10 year death/transplant rates were, respectively 6.4±0.1% and 10.0±0.1% (all-cause) and 3.6±0.1% and 5.8±0.1% (liver related). Factors associated with death/transplant rate by Cox regression analysis were (a) cirrhosis, and decompensation at diagnosis (p &lt; 0.001 both all-cause and liver related) (b) age and cardiovascular/respiratory comorbidity (all-cause only, p = 0.004 and 0.014) (c) failure to normalise serum transaminases (p &lt; 0.001); however because of “early” deaths/transplants, direction of cause and effect is unclear. In contrast, death/transplant rate showed no association with failure to achieve ALT normalisation at specific time points (over 1–12 months). Nor was it associated with gender, peak initial serum ALT, number of relapses or concurrent presence of PBC “overlap”, diabetes or cancer. Both all-cause (p-0.05) and liver-related (p = 0.021) death/transplant were lower in patients attending District General Hospitals (DGHs), compared to University Hospitals; these differences persisted in multivariate analysis (p = 0.017 and 0.036), adjusting for the above baseline predictive factors. <h3>Conclusion</h3> In this multicentre UK AIH Audit, disease outcome is similar to that in patients reported from large single centres. Main determinants are disease severity, age and comorbidity at presentation. Normalisation of serum ALT at specific time points may not be an accurate longer term outcome marker and better ones are needed. Outcome of AIH in UK patients attending DGHs seems at least as good as those attending University hospitals. <h3>Disclosure of Interest</h3> None Declared
Utility of Positron Emission Tomography/Computed Tomography In Extranodal Natural Killer/T-Cell Lymphoma
Authors: Hideaki Fujiwara, Yoshinobu Maeda, Yuichiro Nawa, Masayuki Yamakura, Daisuke Ennishi, Yukihiro Miyazaki, Katsuji Shinagawa, Masamichi Hara, Mitsune Tanimoto, Kosei Matsue
Journal: Blood (2010)
At Disease Presentation, Severity of the Bleeding Symptom Predicts the Following Bleeds in Patients with FVII Deficiency
Authors: Matteo ND Di Minno, Angelika Batorova, Mariasanta Napolitano, Alberto Dolce, Giovanni Di Minno, Francesco Bernardi, Jean-Francois Schved, Jørgen Ingerslev, Muriel Giansily-Blaizot, Guenter K.H. Auerswald, Guglielmo Mariani
Journal: Blood (2011)
AB0389 Methotrexate Treatment for Rheumatoid Arthritis in Poland: Retrospective Analysis of Patients in Routine Clinical Practice
Authors: J. Świerkot, B. Batko, M. Stajszczyk, M. Jedrzejewski, P. Wiland
Journal: Annals of the Rheumatic Diseases (2016)

<h3>Background</h3> Methotrexate (MTX) is a folic acid antagonist which has both an antiproliferative and an anti-inflammatory action. It is the most commonly prescribed DMARD (disease-modifying antirheumatic drug) and is considered the "anchor drug" in rheumatoid arthritis (RA). <h3>Objectives</h3> Our study reviews RA treatment with MTX in Poland. <h3>Methods</h3> Presented study was based on a retrospective analysis of a cohort of 1957 patients with RA in routine clinical practice. The study was conducted among 100 rheumatologists from different centers that have been selected to reflect the structure of the treatment of RA in Poland. Each physician received 20 questionnaires that were filled on the nearest visit of consecutive 20 patients with RA. Data was collected using pre-defined questionnaire. <h3>Results</h3> MTX was taken by 91% of patients and 80% of them continued the treatment both as monotherapy (65%) or concomitantly with other DMARDs. In general, the therapy was initiated within 6 months after diagnosis in 60% of cases. However, MTX was introduced at the very beginning of the treatment for the majority of patients diagnosed after 2009. Methotrexate was given mostly orally, nevertheless in case of 18% of patients it was administered subcutaneously. During MTX treatment dose modifications could be observed in 76% cases and were contingent on different factors. Lack of efficacy was the major reason for increase, while the most common cause of reduction the dose was intolerance of MTX or presence of adverse events, like the most prevalent nausea and vomiting, weakness, increase in activity of liver enzymes and hair loss. Initial dose of MTX was mostly 10 or 15 mg/week. An increase in dosage to 25 mg/week was observed in 36% cases and was continued for 27% of patients. Medium maximum dose that was achieved during treatment was 20.2 mg/week (median 19.3 mg/week). Patients who currently take MTX primarily use a dose of 25 mg/week (27%), 20 mg/week (24%) or 15 mg/week (21%). Treatment interruption was noted in 21% patients predominantly due to intolerance to MTX, however in 13% of cases it was patient9s decision. More than half of patients (61%) treated for 3–6 months with MTX achieved remission or low disease activity based on DAS-28. <h3>Conclusions</h3> MTX when used according to current recommendations is a effective drug, taking into account the efficacy and adverse effects. To optimize the MTX therapy regular control visits are required <h3>Disclosure of Interest</h3> J. Świerkot Grant/research support from: supported by Roche, B. Batko Grant/research support from: supported by Roche, M. Stajszczyk Grant/research support from: supported by Roche, M. Jedrzejewski Grant/research support from: supported by Roche, P. Wiland Grant/research support from: supported by Roche
Greater Precision in Melanoma Prevention
Authors: Monika Janda, Peter Soyer
Journal: JAMA Dermatology (2016)

Our website uses cookies to enhance your experience. By continuing to use our site, or clicking "Continue," you are agreeing to our Cookie Policy | Continue JAMA Dermatology HomeNew OnlineCurrent IssueFor Authors Podcast Publications JAMA JAMA Network Open JAMA Cardiology JAMA Dermatology JAMA Health Forum JAMA Internal Medicine JAMA Neurology JAMA Oncology JAMA Ophthalmology JAMA Otolaryngology–Head & Neck Surgery JAMA Pediatrics JAMA Psychiatry JAMA Surgery Archives of Neurology & Psychiatry (1919-1959) JN Learning / CMESubscribeJobsInstitutions / LibrariansReprints & Permissions Terms of Use | Privacy Policy | Accessibility Statement 2023 American Medical Association. All Rights Reserved Search All JAMA JAMA Network Open JAMA Cardiology JAMA Dermatology JAMA Forum Archive JAMA Health Forum JAMA Internal Medicine JAMA Neurology JAMA Oncology JAMA Ophthalmology JAMA Otolaryngology–Head & Neck Surgery JAMA Pediatrics JAMA Psychiatry JAMA Surgery Archives of Neurology & Psychiatry Input Search Term Sign In Individual Sign In Sign inCreate an Account Access through your institution Sign In Purchase Options: Buy this article Rent this article Subscribe to the JAMA Dermatology journal
SAT0230 Progression of Left Ventricular Myocardial Dysfunction in Systemic Sclerosis: Using Speckle Tracking Strain Echocardiography To Identify Patients at Risk
Authors: J.K. De Vries-Bouwstra, S.E. van Wijngaarden, S. Ben Said-Bouyeri, M.K. Ninaber, J.J. Bax, V. Delgado, N. Ajmone Marsan
Journal: Annals of the Rheumatic Diseases (2016)

<h3>Background</h3> Cardiac involvement is a main cause of mortality in systemic sclerosis (SSc)<sup>1</sup>. Its detection remains challenging using conventional echocardiographic parameters and little is known about which patients are at risk for deterioration of cardiac function. Speckle-tracking strain analysis of echocardiographic images can detect subtle myocardial dysfunction in patients with SSc, and decreased strain has been shown to associate with lower functional capacity and rhythm disturbances<sup>2</sup> <h3>Objectives</h3> This study evaluated: 1. changes in cardiac performance over time in SSc patients including echocardiographic myocardial speckle tracking strain analysis, and 2. baseline characteristics associated with deterioration of cardiac function. <h3>Methods</h3> 205 SSc patients (171 female, 52±14 years) were evaluated at baseline and follow-up (2.25years IQR1.3–3.8), including complete physical examination and screening for organ involvement with at least HRCT-thorax, pulmonary function test, cardiopulmonary exercise test, electrocardiography and echocardiography. <h3>Results</h3> Left ventricular (LV) ejection fraction did not change significantly (61%±7 vs 61%±8,p=0.148) while global longitudinal strain (LS) decreased significantly (-21%±2 vs -19%±3, p&lt;0.001). 39 patients showed progression of LV dysfunction as defined by ≥15% decline in LS. These patients showed significant worsening of LV diastolic function (figure) and TAPSE (22mm±4 vs 19mm±4, p=0.003) and 8% developed pulmonary hypertension. Baseline parameters associated with progression of LV dysfunction were proximal muscle weakness (OR: 4.264 IQR: 1.473–12.342, p=0.007), diffusing capacity of carbon monoxide (DLCO, OR: 0.972 IQR: 0.948–0.995, p=0.020) and LV diastolic dysfunction (OR: 2.245 IQR: 1.019–4.946, p=0.045). <h3>Conclusions</h3> In SSc patients, progression of LV systolic dysfunction was detected by speckle tracking strain analysis but not by LV ejection fraction. Proximal muscle weakness, DLCO and LV diastolic function may identify patients at higher risk and in need of closer cardiac monitoring. <h3>References</h3> Rubio-Rivas M, Royo C et al. Mortality and survival in systemic sclerosis: systematic review and meta-analysis. Semin Arthritis Rheum 2014 Oct;44(2):208–19. Yiu KH, Schouffoer AA, et al. Left ventricular dysfunction assessed by speckle-tracking strain analysis in patients with systemic sclerosis: relationship to functional capacity and ventricular arrhythmias. Arthritis Rheum 2011 Dec;63(12):3969–78. <h3>Disclosure of Interest</h3> None declared
Sixty seconds on . . . soda taxes
Authors: Anne Gulland
Journal: BMJ (2016)

I know, but soda sounds much snappier than sugar sweetened beverages. Anyway, the NHS has launched a consultation on whether to introduce a levy on the sale of sugary drinks or to ban them completely from NHS premises. NHS England’s chief executive, Simon Stevens, said he did not want hospitals to be “marketing joints for junk food and unhealthy sodas.”

Nor should staff and visitors, …
Treatment With Ledipasvir–Sofosbuvir for 12 or 24 Weeks in Kidney Transplant Recipients With Chronic Hepatitis C Virus Genotype 1 or 4 Infection
Authors: Massimo Colombo, Alessio Aghemo, Hong Liu, Jie Zhang, Hadas Dvory-Sobol, Robert Hyland, Chohee Yun, Benedetta Massetto, Diana M. Brainard, John G. McHutchison, Marc Bourlière, Markus Peck-Radosavljevic, Michael Manns, Stanislas Pol
Journal: Annals of Internal Medicine (2016)

Background: Use of interferon and ribavirin to treat chronic hepatitis C virus (HCV) infection in kidney transplant recipients is limited because of the risk for allograft rejection and poor tolerability. Objective: To evaluate the safety and efficacy of the interferon- and ribavirin-free regimen ledipasvir–sofosbuvir in kidney transplant recipients with chronic genotype 1 or 4 HCV infection. Design: Randomized, phase 2, open-label study. (ClinicalTrials.gov: NCT02251717) Setting: 5 sites in Europe. Patients: Treatment-naive or -experienced kidney transplant recipients with chronic genotype 1 or 4 HCV infection, with or without compensated cirrhosis, and with an estimated glomerular filtration rate (eGFR) of 40 mL/min or greater were randomly assigned 1:1 to receive ledipasvir (90 mg) and sofosbuvir (400 mg) for 12 or 24 weeks. Measurements: The primary end point was sustained virologic response at 12 weeks after therapy ended (SVR12). Results: Among 114 patients, the median age was 53 years, 58% were male, 91% had genotype 1 infection, 69% were treatment naive, and 15% had compensated cirrhosis. The median eGFR was 56 mL/min (range, 35 to 135 mL/min). One hundred percent of patients (57 of 57) treated for 12 weeks (95% CI, 94% to 100%) and 100% of those (57 of 57) treated for 24 weeks (CI, 94% to 100%) achieved SVR12. Serious adverse events were reported in 13 patients (11%). Of these, 3 events—syncope, pulmonary embolism, and serum creatinine increase—in 3 patients were determined to be treatment related. One patient permanently discontinued treatment because of an adverse event (syncope). The most frequent adverse events overall were headache (n = 22 [19%]), asthenia (n = 16 [14%]), and fatigue (n = 11 [10%]). Limitations: The study was open label, no inferential statistics were planned, and only patients with genotype 1 or 4 infection were included. Few patients with HCV genotype 1a and cirrhosis were enrolled. Conclusion: Treatment with ledipasvir–sofosbuvir for 12 or 24 weeks was well-tolerated and seemed to have an acceptable safety profile among kidney transplant recipients with HCV genotype 1 or 4 infection, all of whom achieved SVR12. Primary Funding Source: Gilead Sciences.
Recurrence and return to play after shoulder instability events in young and adolescent athletes: a systematic review and meta-analysis
Authors: Jason L Zaremski, Juan Galloza, Fernando Sepulveda, Terrie Vasilopoulos, William Micheo, Daniel C Herman
Journal: British Journal of Sports Medicine (2016)

Background Shoulder instability in athletics is a common occurrence. However, there is controversy as to whether non-operative versus operative management is the most effective treatment in youth athletes. We systematically reviewed recurrence and return to play (RTP) after shoulder instability events in youth athletes based on surgical versus non-surgical intervention. Design The systematic review concerned studies published before August 2016. Statistical analysis was performed to compare rates of recurrence for each extracted risk factor. Pooled ORs were analysed using random-effects meta-analysis. Results 17 studies comprising 654 total shoulder instability events met the criteria for inclusion (438 men and 158 women; 507 cases traumatic and 20 atraumatic). Patients were grouped by whether they received non-operative or operative treatment. The primary non-operative group was more likely to have recurrence compared to the primary operative group (OR=13.41; 99% CI 3.60 to 49.93, p&lt;0.001). Patients &lt;14 years old in the primary non-operative group were less likely to recur compared to those aged ≥14 years (OR=0.16; 99% CI 0.06 to 0.43, p&lt;0.001). The rate of recurrence in patients aged &lt;14 years was high (44.44%). For RTP, there is evidence that RTP rates were higher for primary operative patients (95.3%) versus primary non-operative (41.3%, Z=6.12, p&lt;0.001) and secondary operative patients (77.6%, Z=2.66, p=0.008). Conclusions This meta-analysis summarises a mix of 17 acceptable quality level II and III prospective and retrospective cohort studies. Given the superior rates of recurrence and RTP, primary operative treatment for shoulder instability should be considered in youth athletes aged ≥14 years. Additionally, the recurrence rate in athletes aged &lt;14 years is significant.
The Molecular and Cytokine Profile of Triple-Negative (JAK2 V617F, JAK2 exon 12, MPL negative) Myelofibrosis, a Myeloproliferative Neoplasm with Distinct Clinico-Pathologic Characteristics
Authors: Ali Tabarroki, Heesun J. Rogers, Valeria Visconte, Edy Hasrouni, Anjali Advani, Mikkael A. Sekeres, Hien K. Duong, Matt Kalaycio, Edward A. Copelan, Brady L Stein, Ramon V. Tiu
Journal: Blood (2012)
Hematopoietic stem cell transplant in patients with activated PI3K delta syndrome
Authors: Zohreh Nademi, Mary A. Slatter, Christopher C. Dvorak, Benedicte Neven, Alain Fischer, Felipe Suarez, Claire Booth, Kanchan Rao, Alexandra Laberko, Julia Rodina, Yves Bertrand, Sylwia Kołtan, Robert Dębski, Terence Flood, Mario Abinun, Andrew R. Gennery, Sophie Hambleton, Stephan Ehl, Andrew J. Cant
Journal: Journal of Allergy and Clinical Immunology (2016)
Zoledronic Acid Increases Bone Mineral Density in Patients with Thalassemia Intermedia-Induced Osteoporosis Regardless of the Incessant Bone Marrow Expansion.
Authors: Ersi Voskaridou, Dimitrios Christoulas, Lito Antoniadou, Panagiotis Tsaftaridis, Eleni Plata, Evangelos Terpos
Journal: Blood (2007)
Mechanisms and Medicines for Remyelination
Authors: Mark Bothwell
Journal: Annual Review of Medicine (2016)

Demyelination of central nervous system axons, associated with traumatic injury and demyelinating diseases such as multiple sclerosis, causes impaired neural transmission and ultimately axon degeneration. Consequently, extensive research has focused on signaling systems that promote myelinating activity of oligodendrocytes or promote production of new oligodendrocytes from oligodendrocyte progenitor cells. Many receptor systems, notably including growth factor receptors and G protein–coupled receptors, control myelination. A number of recent clinical trials target these receptor signaling pathways.
The Predictive Value of FCGR3A Polymorphism on Clinical Outcomes of Patients with Diffuse Large B-Cell Lymphoma Treated with R-CHOP Chemotherapy
Authors: Steven I. Park, Amy S. Lucas, Michael Kingberg, Dominic Moore, Michael Chiu, Anne M.D. Misher, Janelle Hoskins, Cherie H. Dunphy, Howard L. McLeod, Kristy L. Richards
Journal: Blood (2011)
PTH-107 Chronic Hepatitis B Management The UK: A National Survey of Current Practice Following Nice Guidance
Authors: N Hansi, P Troke, US Gill, K Agarwal, M Aldersley, S Al-Shamma, A Brown, J Cobbold, P Collins, A Fowell, W Gelson, A Holt, S McPherson, M Phillips, M Prince, P Richardson, S Ryder, S Singhal, B Stone, A Ustianowski, J Vilar, L Walker, T Wong, P Kennedy
Journal: Gut (2016)

<h3>Introduction</h3> National Institute for Health and Care Excellence (NICE) published guidance on the management of chronic hepatitis B (CHB) in June 2013 (NICE 165). Where antiviral therapy was indicated, NICE recommended 48 weeks Peg-interferon alfa-2a (PegIFNα) as first-line treatment in compensated disease and early discontinuation of PegIFNα based on stopping rules. Nucleos(t)ide analogues were recommended as second-line therapies. This survey was undertaken to capture current CHB practice management across the UK following NICE 165 and report PegIFNα use, and review the availability/utility of quantitative HBsAg. <h3>Methods</h3> This was a UK-wide national multicentre study, where 25 CHB treatment centres were invited to complete a qualitative questionnaire of CHB practice. Clinical practice in the 12 months preceding NICE 165 was compared with CHB management in the immediate 12 months post publication. <h3>Results</h3> All centres participating undertook a multi-disciplinary approach to the treatment of CHB patients, with 75% of centres adopting joint consultant-nurse led clinics. Interim analysis of the data revealed that 73% of centres consider PegIFNα as first line therapy in eAg+ disease, compared with 40% in eAg- disease. Importantly, there was no difference in the use of PegIFNα, irrespective of eAg status, prior to and following the publication of NICE 165 (p = 0.82). Of those patients treated with PegIFNα as first line-therapy, 63% of them, to date, required second line therapy with NUCs due to treatment failure. <h3>Conclusion</h3> This UK survey demonstrates that current practice has not significantly changed following NICE 165. While most centres adopt a multi-disciplinary approach, the UK guidance on CHB has not been widely adopted. Barriers to this may include the limited availablilty of qHBsAg in UK centres, but more likely this relates to Physician preference for the continued use of NUCs as first-line therapy of choice. <h3>Disclosure of Interest</h3> N. Hansi: None Declared, P. Trok, None Declared, U. Gill: None Declared, K. Agarwal: None Declared, M. Aldersley: None Declared, S. Al-Shamma: None Declared, A. Brown: None Declared, J. Cobbold: None Declared, P. Collins: None Declared, A. Fowell: None Declared, W. Gelson: None Declared, A. Holt: None Declared, S. McPherson: None Declared, M. Phillips: None Declared, M. Prince: None Declared, P. Richardson: None Declared, S. Ryder: None Declared, S. Singhal: None Declared, B. Stone: None Declared, A. Ustianowski: None Declared, J. Vilar: None Declared, L. Walker: None Declared, T. Wong: None Declared, P. Kennedy Grant/research support from: Gilead Sciences
Significant Improvement of the Survival of Patients with Multiple Myeloma Presenting with Severe Renal Impairment After the Introduction of Novel Agents
Authors: Meletios A Dimopoulos, Sosana Delimpasi, Eirini Katodritou, Eleftheria Hatzimichael, Marie-Christine Kyrtsonis, Panagiotis Repousis, Zafiris Kartasis, Agapi Parcharidou, Michalis Michael, Eurydiki Michalis, Dimitra Gika, Argiris Symeonidis, Konstantinos Zervas, Evangelos Terpos, Efstathios Kastritis
Journal: Blood (2012)