tbuckley/cabot-search huggingface dataset:
A2.08 Extrathymic autoimmune regulator (AIRE) expression can be induced in dendritic cells by CD40-mediated activation of noncanonical NF-κb signalling, but is impaired in primary sjögren’s syndrome
Authors: LFA Huitema, B Helder, AR Noort, MC Lebre, SW Tas
Journal: Annals of the Rheumatic Diseases (2016)

<h3>Background</h3> The nuclear factor (NF)-κB family of transcription factors has a key role in the regulation of inflammation. We have previously demonstrated that activation of noncanonical NF-κB signalling in dendritic cells (DC) via CD40 stimulation is important for the induction of the immunoregulatory enzyme indoleamine 2,3-dioxygenase (IDO). This pathway is also involved in the expression of Autoimmune Regulator (AIRE) in the thymus, which is essential central tolerance. Peripheral extrathymic AIRE-expressing cells (mainly antigen presenting cells, including DC) can also induce tolerance. However, stimuli that induce extrathymic AIRE expression in DC are unknown. Therefore, we studied whether activation of noncanonical NF-κB signalling induces AIRE expression in monocyte-derived DC (moDC) and whether this induction is altered in moDC derived from patients with autoimmunity, using primary Sjögren's syndrome (pSS) as a prototypic systemic autoimmune disease. <h3>Objective</h3> To study whether activation of noncanonical NF-κB signalling induces AIRE expression in moDC from healthy donors and pSS. <h3>Methods</h3> MoDC were generated by culturing monocytes for 6 days with GM-CSF and IL-4. Maturation was induced by addition of the noncanonical NF-κB stimulus anti-CD40 for 48 h. Cleavage of p100 into p52, IDO and AIRE protein was analysed by Western blot. Immunofluorescence staining for RelB was performed on cytospins of moDC. RelB protein was visualised by confocal microscopy. siRNA-mediated silencing of NF-κB-inducing kinase (NIK), the main activating kinase of the noncanonical pathway, was accomplished by neon electroporation. <h3>Results</h3> Anti-CD40 stimulation of moDC from healthy donors induced cleavage of p100 and nuclear translocation of RelB, which is indicative of active noncanonical NF-κB signalling. This was accompanied by upregulation of IDO and AIRE expression, which was inhibited by siRNA-mediated silencing of NIK. Anti-CD40 stimulation of moDC derived from pSS patients also resulted in activation of noncanonical NF-κB signalling and upregulation of IDO. However, AIRE expression was to a much lesser extent upregulated in moDC derived from pSS patients compared to healthy donor DC. <h3>Conclusion</h3> Extrathymic AIRE expression can be induced in moDC via CD40-stimulation and is regulated by noncanonical NF-κB signalling. This mechanism is impaired in pSS patients. The functional consequences of this potentially defective immunoregulatory program are currently investigated.
Abstract 25: Pfetin as a risk factor of recurrence in gastrointestinal stromal tumors.
Authors: Hajime Orita, Tomoaki Ito, Mutsumi Sakurada, Tomoyuki Kushida, Hiroshi Maekawa, Ryo Wada, Yoshiyuki Suehara, Daisuke Kubota, Tadashi Kondo, Koichi Sato
Journal: Cancer Research (2013)

Abstract Background: Despite complete resection, gastrointestinal stromal tumors (GIST) may recur or metastasize, depending on tumor grade. As such, a sensitive and specific marker is sought to select patients at high risk for recurrence. Previously our group reported Pfetin as prognostic predictors by use of proteomics technology. We used microarray of IHC to identify Pfetin as a statistically significant predictor of prognosis in resected GIST. In this project, we evaluated the role of Pfetin expression by IHC, controlled for clinicopathological data, as a predictor for recurrence. Methods: 45 patients with primary GIST were treated with complete (R0) resection at our institution between 1995 and 2009. Tumors originated in the stomach (38 cases), small intestine (6 cases), and rectum (1 case). Results: There were 8 recurrences, with the following characteristics. (1) Pfetin negative cases were significantly related to recurrence (p=0.002). (2) By univariate analysis, tumor size, mitoses, dissemination (tumor explosion), invasion (other organ) as well as Pfetin were significant predictors. According to multivariate analysis using these 5 factors, tumor mitosis and Pfetin were the most significant risks (p=0.059). (3) All 8 cases of recurrence were grouped as high risk according to Fletcher's classification. This demonstrated high accuracy. Conclusions: We determined that tumor mitoses and Pfetin under-expression correlate with recurrence. These markers may have utility in selecting patients for adjuvant therapy. Citation Format: Hajime Orita, Tomoaki Ito, Mutsumi Sakurada, Tomoyuki Kushida, Hiroshi Maekawa, Ryo Wada, Yoshiyuki Suehara, Daisuke Kubota, Tadashi Kondo, Koichi Sato. Pfetin as a risk factor of recurrence in gastrointestinal stromal tumors. [abstract]. In: Proceedings of the 104th Annual Meeting of the American Association for Cancer Research; 2013 Apr 6-10; Washington, DC. Philadelphia (PA): AACR; Cancer Res 2013;73(8 Suppl):Abstract nr 25. doi:10.1158/1538-7445.AM2013-25
Clip or loop? Which modality can we call “A stitch in time saves nine”?
Authors: Mo Thoufeeq
Journal: Endoscopy (2014)

The article by Ji et al. was read with interest [1]. It is reassuring to note that clip application is as good as the endoloop in reducing the risk of postpolypectomy bleeds. Placement of prophylactic endoscopic clips after polypectomy has been found to be a cost-effective strategy for patients who receive antiplatelet or anticoagulation therapy [2].
1081 NEW DIAGNOSTIC AND PROGNOSTIC PLASMA BIOMARKERS FOR PATIENTS WITH HEPATOCELLULAR CARCINOMA IDENTIFIED BY PROTEIN PROFILING
Authors: I. Serra, J.M. Estanyol, M.-R. Sarrias, S. Ragull, C. Huertas, M. Miquel, S. Coll, F. Pons, A. Rubin, R. Bartolí, A. Barriocanal, G. òdena, X. Aldeguer, R. Morillas, O. Bachs, R. Planas, M. Sala, C. Armengol
Journal: Journal of Hepatology (2013)
A MULTICENTER STUDY OF THE ETIOLOGY AND OUTCOME OF CARDIAC TAMPONADE IN THE CONTEMPORARY ERA
Authors: Luke Cunningham, Toug Tanavin, Mahboob Alam, Salim Virani, Biykem Bozkurt, Anita Deswal, Vijay Nambi, Hani Jneid
Journal: Journal of the American College of Cardiology (2016)
Treatment Choice for Diabetic Macular Edema
Authors: Daniel F. Martin, Maureen G. Maguire
Journal: New England Journal of Medicine (2015)

Macular edema is a common cause of vision loss in patients with diabetes. Chronic elevation of serum glucose levels leads to capillary damage that results in microaneurysm formation in the retina. Leakage from these microaneurysms leads to vision loss if the fluid involves the center of the fovea. The mainstay of therapy for more than 25 years was focal laser photocoagulation applied to or near the microaneurysms.1 However, results from clinical trials of drugs that block vascular endothelial growth factor (VEGF) for the treatment of diabetic macular edema have led to a dramatic shift away from laser therapy to primary . . .
Liver abscess and septic shock as an unusual complication after endoscopic ampullectomy
Authors: S. Gruttadauria, S. Li Petri, G. Echeverri, C. Ricotta, I. Tarantino, M. Traina, B. Gridelli
Journal: Endoscopy (2011)

Tumors of the ampulla of Vater represent about 5 % of gastrointestinal neoplasms and are more often identified during upper gastrointestinal endoscopy [1], most are adenomas forming part of the adenoma–carcinoma sequence [2]. The first endoscopic resection of a duodenal adenoma was performed in the 1970 s [3], since when this procedure has become increasingly common, and the management has shifted from surgical resection to endoscopic procedures [1] [2] [3] [4]. We present here the first report in the literature in English, of a major septic complication after endoscopic ampullectomy.
INTRA-STENT THROMBUS FORMATION FOLLOWING BIOLIMUS A-9 ELUTING STENT IMPLANTATION IN PATIENTS WITH SHORTER AND PROLONGED DUAL ANTI-PLATELET THERAPY: OPTICAL COHERENCE TOMOGRAPHY SUB-STUDY OF NIPPON TRIAL
Authors: Koji Kuroda, Masato Nakamura, Hiroyoshi Yokoi, Shinsuke Nanto, Kazuaki Mitsudo, Shigeru Saito, Junya Ako, Raisuke Iijima, Toshiro Shinke, Hiromasa Otake, Tomofumi Takaya, Masaru Kuroda, Hachidai Takahashi, Daisuke Terashita, Kenzo Uzu, Yuto Shinkura, Daiji Kashiwagi, Natsuko Tahara, Yoshinori Nagasawa, Ken-Ichi Hirata
Journal: Journal of the American College of Cardiology (2016)
Ideas and Leaders in Contentious Politics: One Parish Priest in El Salvador's Popular Movement
Authors: PETER M. SANCHEZ
Journal: Behavioral and Brain Sciences (2014)

Abstract This paper examines the actions of one Salvadorean priest – Padre David Rodríguez – in one parish – Tecoluca – to underscore the importance of religious leadership in the rise of El Salvador's contentious political movement that began in the early 1970s, when the guerrilla organisations were only just beginning to develop. Catholic leaders became engaged in promoting contentious politics, however, only after the Church had experienced an ideological conversion, commonly referred to as liberation theology. A focus on one priest, in one parish, allows for generalisation, since scores of priests, nuns and lay workers in El Salvador followed the same injustice frame and tactics that generated extensive political mobilisation throughout the country. While structural conditions, collective action and resource mobilisation are undoubtedly necessary, the case of religious leaders in El Salvador suggests that ideas and leadership are of vital importance for the rise of contentious politics at a particular historical moment.
Traitement endoscopique de l'empierrement et des gros calculs cholédociens: taux de succès et de complications et facteurs associés
Authors: H Seddik, A Frej, H Massit, FZ El Hamdi, A Benkirane
Journal: Endoscopy (2012)

La sphinctérotomie biliaire endoscopique est efficace dans le traitement de la lithiase de la voie biliaire principale permettant d'obtenir une vacuité complète de celle-ci dans 85 à 90% des cas. Toutefois ces résultats peuvent être limités par La présence de gros calculs ou d'un empirement cholédocien. Objectif: évaluer le taux de succès et les facteurs influençant celui-ci ainsi que les complications du traitement endoscopique dans la prise en charge de l'empierrement et des gros calculs cholédociens .
OP0225 Mefv and TNFRSF1A Gene Mutations in Patients with Inflammatory Myopathy with Abundant Macrophages
Authors: K. Fujikawa, K. Migita, M. Umeda, F. Nonaka, S.-Y. Kawashiri, N. Iwamoto, K. Ichinose, M. Tamai, H. Nakamura, A. Mizokami, T. Tsukada, T. Origuchi, Y. Ueki, M. Yasunami, A. Kawakami, K. Eguchi
Journal: Annals of the Rheumatic Diseases (2014)

<h3>Background</h3> Inflammatory myopathy with abundant macrophages (IMAM) is a new clinical condition characterized by diffuse infiltration of macrophages in fascia [1], but the mechanism remains unclear. <h3>Objectives</h3> The objective of this study was to investigate the <i>MEFV</i> and <i>TNFRSF1A</i> mutations in patients with IMAM. <h3>Methods</h3> Nine patients with IMAM were enrolled. Clinical characteristics, MRI, and pathologic findings were analyzed. Mutation analysis was performed by genomic sequencing of <i>MEFV</i> and <i>TNFRSF1A.</i> <h3>Results</h3> The patients with IMAM had clinical symptoms such as myalgia, muscle weakness, erythema, fever, and arthralgia. STIR images in MRI showed thickening of fascia in all patients. In biopsied specimens from 8 patients, a number of CD68+ macrophages were found apparently in the fascia in all samples. In gene analysis, 7 of 9 patients had <i>MEFV</i> polymorphisms (<i>G304R, R202R, E148Q, E148Q-L110P</i>, and <i>P369S-R408Q</i>) and one patient had <i>TNFRSF1A</i> mutation (<i>C43R</i>). <h3>Conclusions</h3> These results suggest that <i>MEFV</i> gene polymorphisms and <i>TNRRSF1A</i> mutations are susceptibility and modifier genes in IMAM. <h3>References</h3> Bassez G, Authier F-J, Lechapt-Zalcman E et al. Inflammatory myopathy with abundant macrophages (IMAM): a condition sharing similarities with cytophagic histiocytic panniculitis and distinct from macrophagic myofasciitis. J Neuropathol Exp Neurol 2003; 62: 464-74. <h3>Disclosure of Interest</h3> None declared <h3>DOI</h3> 10.1136/annrheumdis-2014-eular.1474
P18.05 * TREATMENT OF ACUTE ISCHEMIC STROKE IN CANCER PATIENTS
Authors: S. Rosenberg, A. Meirrovitz, R. Leker, A. Mordechai, E. Linetzky, J. Cohen, T. Peretz, A. Lossos
Journal: Neuro-Oncology (2014)

BACKGROUND: Intravenous thrombolysis using tissue plasminogen activator (iv-tPA) and endovascular clot retrieval have revolutionized treatment of acute ischemic stroke. Cancer patients are at increased risk of ischemic stroke because of inherent hypercoagulability. However, the benefit of active thrombolysis in this clinical setting is unknown and treatment with iv-tPA is often avoided because of possible hemorrhagic complications. METHODS: Retrospective screening of the institutional medical records for cancer patients presenting with acute ischemic stroke during the year 2011. Description of 4 representative clinical cases. RESULTS: During the one-year period, 33 cancer patients (16 men and 17 women) presented with and were evaluated for acute ischemic stroke at a mean age of 69 (45-92) years. Of these, 17 (52%) had lacunar strokes, 14 (42%) had large artery strokes and 2 (6%) presented with multiple cardioembolic strokes. At the time of presentation, 14/33 (42%) patients had evidence of systemic metastatic disease and 12/33 (36%) had active locally advanced disease. Most common malignancies included breast cancer, prostatic carcinoma, colorectal and urinary bladder tumors. According to the commonly accepted criteria for intravenous thrombolysis, 3/33 (9%) patients were treated with iv- tPA, and 1/33 (3%) underwent endovascular clot retrieval. No hemorrhagic complications were observed in these 4 patients and all had marked improvement of the neurological disability following treatment. CONCLUSION: Selected cancer patients with acute ischemic stroke may benefit from intravenous thrombolysis and endovascular clot retrieval. Application of the currently accepted clinical criteria for iv-tPA reduces the risk of hemorrhagic complications in this group of patients. Awareness to the available early therapeutic options in this clinical setup may improve outcome of cancer patients with acute ischemic stroke.
P44 * CLINICAL AUDIT: ASSESSMENT OF USE AND BENEFIT OF IMMUNOHISTOCHEMISTRY WITH THE NOVEL ANTIBODY ISOCITRATE DEHYDROGENASE 1 (R132H) IN ASTROCYTIC BRAIN TUMOUR PATHOLOGY
Authors: T. O. Millner, U. Pohl
Journal: Neuro-Oncology (2014)

INTRODUCTION: Astrocytic gliomas are the most common primary brain tumours. We assessed the diagnostic and prognostic utility of our new IDH1 antibody (R132H) in astrocytic gliomas. The use of the antibody is recommended by the London Cancer Network, however it is costly. METHOD: We recorded demographics, diagnosis, grade, IDH1 status and survival status/date of death for all patients with a histological diagnosis of astrocytic tumour within a 12 month period. We then quantified requests, results, documentation and interpretation for all IDH1 immunohistochemistry. We also reviewed all cases and examined the correlation between diagnosis and IDH1 result. RESULTS: Our sample consisted of 108 patients. IDH1 was requested in 63% (54% of glioblastomas) and was positive in 18% of all cases. IDH1 status was documented in all cases when requested, but interpreted in the pathology report in only 59% of all cases (65% in glioblastomas). Mean survival time declined as WHO grade increased, with IDH1 positive cases surviving longer. In grade III gliomas mortality was lower for IDH1 positive cases. CONCLUSION: In our laboratory, percentages of tumours with IDH1 mutation are similar to those reported in the literature and the IDH1 positive cases had longer survival times, confirming IDH1 as a prognostic marker. As expected, mean survival time decreased as WHO grade increased and for grade III gliomas mortality was lower with IDH1 positivity. We then made recommendations for our clinical practice: 1) Increase the proportion of cases for which IDH1 is requested 2) Always comment on its prognostic value 3) Continue to use IDH1 and discuss its prognostic value at the neuropathology MDM 4) Re-audit with more cases, especially grade IV tumours.
Case 29-2006
Authors: Karen H. Costenbader, Panos Fidias, Matthew D. Gilman, Anwer Qureshi, Rosemary H. Tambouret
Journal: New England Journal of Medicine (2006)

A 43-year-old woman was hospitalized because of painful nodules on the fingertips, shortness of breath, and fatigue. She had had systemic lupus erythematosus for 12 years, associated with Raynaud's phenomenon. Two weeks before admission, painful nodules developed on the fingers; 1 week later, shortness of breath and fatigue developed. On admission, computed tomography of the chest showed mediastinal and hilar lymphadenopathy and two nodules in the lung. A diagnostic procedure was performed.
EXPERIENCIA SOBRE LA UTILIZACION DE LAS PROTESIS AUTOEXPANDIBLES METALICAS (PAM) EN EL CANCER COLORRECTAL OBSTRUCTIVO
Authors: AB García Sánchez, L Vázquez Pedreño, JM Ruiz Ruiz, F Melgarejo Cordero, FJ Rando Muñoz, MC Ortiz Correro, B Jucha Taybi, R Manteca González
Journal: Endoscopy (2011)

Introducción: Las prótesis autoexpandibles metálicas han supuesto una alternativa válida en el tratamiento de urgencia de la obstrucción maligna colorrectal. Permiten tanto la posibilidad de una cirugía diferida de la neoplasia intestinal con menor morbimortalidad (prótesis puente) como su colocación definitiva paliativa en los casos no candidatos a cirugía.
Infliximab for the Treatment of Fistulas in Patients with Crohn's Disease
Authors: Daniel H. Present, Paul Rutgeerts, Stephan Targan, Stephen B. Hanauer, Lloyd Mayer, R.A. van Hogezand, Daniel K. Podolsky, Bruce E. Sands, Tanja Braakman, Kimberly L. DeWoody, Thomas F. Schaible, Sander J.H. van Deventer
Journal: New England Journal of Medicine (1999)

Enterocutaneous fistulas are a serious complication of Crohn's disease and are difficult to treat. Infliximab, a chimeric monoclonal antibody to tumor necrosis factor alpha, has recently been developed as a treatment for Crohn's disease. We conducted a randomized, multicenter, double-blind, placebo-controlled trial of infliximab for the treatment of fistulas in patients with Crohn's disease.The study included 94 adult patients who had draining abdominal or perianal fistulas of at least three months' duration as a complication of Crohn's disease. Patients were randomly assigned to receive one of three treatments: placebo (31 patients), 5 mg of infliximab per kilogram of body weight (31 patients), or 10 mg of infliximab per kilogram (32 patients); all three were to be administered intravenously at weeks 0, 2, and 6. The primary end point was a reduction of 50 percent or more from base line in the number of draining fistulas observed at two or more consecutive study visits. A secondary end point was the closure of all fistulas.Sixty-eight percent of the patients who received 5 mg of infliximab per kilogram and 56 percent of those who received 10 mg per kilogram achieved the primary end point, as compared with 26 percent of the patients in the placebo group (P=0.002 and P=0.02, respectively). In addition, 55 percent of the patients assigned to receive 5 mg of infliximab per kilogram and 38 percent of those assigned to 10 mg per kilogram had closure of all fistulas, as compared with 13 percent of the patients assigned to placebo (P=0.001 and P=0.04, respectively). The median length of time during which the fistulas remained closed was three months. More than 60 percent of patients in all the groups had adverse events. For patients treated with infliximab, the most common were headache, abscess, upper respiratory tract infection, and fatigue.Infliximab is an efficacious treatment for fistulas in patients with Crohn's disease.
AB0918 Efficacy and Safety of Treatment of Children with Traps and Hids with IL1 Blocker (CANAKINUMAB)
Authors: A. Kozlova, E. Mamzerova, A. Maschan, A. Shcherbina
Journal: Annals of the Rheumatic Diseases (2014)

<h3>Background</h3> The hyperimmunoglobulinemia D syndrome (HIDS),so-called mevalonate kinase deficiency, is caused by recessive mutations in the gene MVK encoding mevalonate kinase enzyme.HIDS is characterized by recurrent fever attacks of 3-7 days that begin in infancy and recur every 4-6 weeks. The febrile period is accompanied by lymphadenopathy, arthralgia, abdominal pain, diarrhea, aphthous ulcers and varying degree of skin involvement. Tumor Necrosis Factor Receptor Associated Periodic Syndrome (TRAPS) is a hereditary autoinflammatory syndrome characterized by recurrent attacks of high spiking fevers, usually of two to three weeks duration. The fever is typically accompanied by gastro-intestinal disturbances (abdominal pain, vomiting, and diarrhea), painful red skin rash, muscle pain and periorbital swelling. Amyloidosis is the most severe life-threatening complication of TRAPS, occurring in 14% of patients. We report two pediatric cases of HIDS and TRAPS resistant to TNF alpha inhibitor therapy. <h3>Objectives</h3> Efficacy and safety of therapy with IL1 blocker (canakinumab) in children with TRAPS, HIDS. <h3>Methods</h3> 2 patients with HIDS and 2 patients with TRAPS were followed over several years9 period. All patients suffered from typical clinical features:bouts of fever, lymphadenitis and high laboratory inflammatory activity starting from first months of life. Attacks were often accompanied by rash and severe stomach ache and diarrhea. In all patients their respective diagnoses were confirmed genetically (mutations in MVK and TNFRSFIA genes). All patients were treated originally with steroids, then with various TNF-blockers (etanercept, infliximab) with no or minimal response. <h3>Results</h3> Patients were initiated on IL-1 inhibitors therapy with Anakinra. Complete or almost complete clinical and laboratory remission was achieved in all four patients. Later 2 patients were switched to canakinumab therapy, with no side effects and continuous stable remission. <h3>Conclusions</h3> In our group of patients with non-CAPS autoinflamatory syndromes IL-1 inhibitors (anakinra and canakinumab) demonstrated better efficacy compared to TNF inhibitors and is a safe treatment modality. <h3>Disclosure of Interest</h3> None declared <h3>DOI</h3> 10.1136/annrheumdis-2014-eular.3697
Pulmonary Artery Angiosarcoma on 18F-FDG PET/CT Masquerading as Pulmonary Embolism
Authors: Alexander Kessler, Hongju Son
Journal: Clinical Nuclear Medicine (2014)

A 53-year old woman suspicious for having pulmonary embolism on enhanced chest CT was proven to have metabolically active tumor in her pulmonary arteries on F-FDG PET/CT. Through biopsy and surgery, this turned out to be a pulmonary artery angiosarcoma. This case demonstrates that PET/CT is very useful in differentiating an aggressive malignant tumor from a bland pulmonary embolus in the pulmonary vasculature.
3D-EUS in staging and accurate localisation of early oesophageal cancer prior to treatment
Authors: S Inglis, S Paterson-Brown, D Patel, J Plevris
Journal: Endoscopy (2006)

Introduction: EUS is routinely used in the staging of oesophageal cancer. Most tumours are advanced, but when the disease is diagnosed early there are several treatment options available depending on patient fitness, age and treatment preference.
Asymptomatic Enlargement of the Parotid Glands
Authors: John J. Duggan, Earle N. Rothbell
Journal: New England Journal of Medicine (1957)

ASYMPTOMATIC enlargement of the parotid glands has been noted in this country in patients with liver disease.1 , 2 On search, it has become evident that similarly enlarged glands may be found in a wider variety of patients. Indeed, this swelling is the commonest disturbance of these glands in adults, and a useful physical sign. The clinical settings in which it occurs have come to be familiar even though its pathogenesis is not clear. This presentation is concerned with 50 patients with noninflammatory parotid enlargement, 6 of whom were described previously as patients with liver disease.1 Method of StudyOver a two-year . . .