tbuckley/cabot-search huggingface dataset:
Altered Hepcidin Regulation in Hereditary Hemochromatosis and Dysmetabolic Hyperferritinemia.
Authors: Elizabeta Nemeth, Olivier Loreal, Caroline Le Lan, Martine Ropert, Marie-Bérengère Troadec, Lénaïck Detivaud, Christine Jard, Michele Pouchard, André Le Treut, Yves Deugnier, Tomas Ganz, Pierre Brissot
Journal: Blood (2005)
Impact of IL-10 Polymorphisms in Survival of Lymphoid Neoplasms.
Authors: Eva Domingo-Domenech, Yolanda Benavente, Eva Gonzalez-Barca, Carlos Montalban, Josep Guma, Sophia S. Wang, Denise Whitby, Alberto Fernandez de Sevilla, Nathaniel Rothman, Silvia de Sanjose
Journal: Blood (2006)
P08.09 Axitinib for the treatment of patients with recurrent glioblastoma, final results from a randomized phase II clinical trial
Authors: J. Duerinck, S. Du Four, F. Bouttens, V. Verschaeve, C. Andre, F. Van Fraeyenhove, C. Chaskis, N. D’haene, I. Salmon, B. Neyns
Journal: Neuro-Oncology (2016)
Sleep is an important factor when considering rugby union player load
Authors: Ian C Dunican, Peter R Eastwood
Journal: British Journal of Sports Medicine (2016)

We read with great interest the paper by Dr Kenneth Quarrie, et al 1 . The review provides an excellent summary of elite player physical demands, and the nature and measurement of the physical and non-physical loads that underpin these demands. However, we believe that the lack of acknowledgement of sleep as an important contributor to non-physical loads is an oversight. This is because sleep has been shown in many studies to be important for optimal athletic performance (physical and cognitive components) and is recognised as an essential component of recovery.2 Indeed, it is considered by some to be one of a number …
AB0418 Secondary Sjögren's Syndrome and Sicca Syndrome in Systemic Sclerosis in Mexican Population
Authors: O. Vera-Lastra, J. Pelaez-Montiel, F. Treviño-Tello, M.D.P. Cruz-Dominguez
Journal: Annals of the Rheumatic Diseases (2016)

<h3>Background</h3> Systemic sclerosis (SSc) and Sjögren9s syndrome (SS) are autoimmune diseases, which can coexist with sicca syndrome due to accumulation of collagen or chronic inflammation of the exocrine glands (salivary and endocrine). The prevalence of secondary SS to SSc ranges from 4 to 60%. <h3>Objectives</h3> To determine the prevalence of SS and sicca syndrome in Mexican patients with SSc. <h3>Methods</h3> Cross-sectional study. Seventy seven patients with SSc were studied at a tertiary hospital. SS and sicca syndrome were investigated according to the 2002 revised international criteria for Sjögren9s syndrome by the American European Consensus Group (AECG). All patients underwent minor salivary gland biopsy, and the results of fibrosis andinflammation were classified according to Tarpley9s classification with a score ≥1. Anti-Ro/SSA and Anti-La/SSB antibodies were quantified in 34 patients with positive histological findings for SS. All patients with ocular symptoms were also performed Schirmer9s I test to evaluate the ocular manifestations. <h3>Results</h3> Infiltration characteristic of SSc, and 21 (27%) revealed SS lymphocytic infiltrate. Isolatedxerostomia was found in 17 (22%) patients, and isolated xerophthalmia in 5 (7%) patients.Five (6.5%) patients referred parotid glands swelling. Anti-Ro/SSA and/or anti-La/SSBautoantibodies were found in 28.5% (6/21) of patients with SS. 21 (27%) patients met the2002 revised criteria for classification for SS by the AECG, Schirmer9s I test revealed dryeye severity level 2 in 4 (19%) patients, level 3 in 12 (57%) patients and level 4 in 5 (23.8%) patients. <h3>Conclusions</h3> The prevalence of sicca syndrome was 71%, 44 per cent related to systemic sclerosis9s collagen infiltration, and 27% due to Sjögren9s syndrome9s lymphocytic infiltrate. It is important to give a treatment to improve symptoms and considering the high frequency of non Hodgkin lymphoma in patients with SSc and SS. <h3>References</h3> Kobak S, Oksel F, Aksu K, Kabasakal Y. The frequency of sicca symptoms and Sjögren9s syndrome in patients with systemic sclerosis. Int J Rheum Dis. 2013;16:88–92. Baldini C, Mosca M, Della Rossa A, Pepe P, Notarstefano C, Ferro F, Luciano N, Talarico R, Tani C, Tavoni AG, Bombardieri S. Overlap of ACA-positive systemic sclerosis and Sjögren9s syndrome: a distinct clinical entity with mild organ involvement but at high risk of lymphoma. Clin Exp Rheumatol. 2013;31:272–80. Avouac J, Sordet C, Depinay C, et al. Systemic Sclerosis - Associated Sjögren9s Syndrome and Relationship to the Limited Cutaneous Subtype. Arthritis Rheum. 2006; 54: 2243–2249. <h3>Disclosure of Interest</h3> None declared
New Medical and Surgical Insights Into Neonatal Necrotizing Enterocolitis
Authors: Brandy L. Frost, Biren P. Modi, Tom Jaksic, Michael S. Caplan
Journal: JAMA Pediatrics (2016)

Necrotizing enterocolitis (NEC) has long remained a significant cause of morbidity and mortality in neonatal intensive care units. While the mainstay of treatment for this devastating condition remains largely supportive, research efforts continue to be directed toward understanding pathophysiology as well as how best to approach surgical management when indicated.In this review, we first examine recent medical observations, including overviews on the microbiome and a brief review of the use of probiotics. Next, we discuss the use of biomarkers and how clinicians may be able to use them in the future to predict the course of disease and, perhaps, the need for surgical intervention. We then provide an overview on the use of exclusive human milk feeding and the utility of this approach in preventing NEC. Finally, we discuss recent developments in the surgical management of NEC, beginning with indications for surgery and following with a section on technical surgical considerations, including peritoneal drain vs laparotomy. The review concludes with outcomes from infants with surgically treated NEC.Although medical treatment options for NEC are largely unchanged, understanding of the disease continues to evolve. As new research methods are developed, NEC pathophysiology can be more completely understood. In time, it is hoped that data from ongoing and planned clinical trials will allow us to routinely add targeted preventive measures in addition to human milk, such as prebiotics and probiotics, to the management of high-risk infants. In addition, the discovery of novel biomarkers may not only prove useful in predicting severity of illness but also will hopefully allow for identification of the disease prior to onset of clinical signs. Finally, continued investigation into optimizing surgical outcomes is essential in this population of infants, many of whom require long-term parenteral therapy and intestinal rehabilitation.
PTU-023 The 2015 “Be Clear On Cancer” Campaign: Its Impact on Gastroenterology Referral Rates and Cancer Diagnosis: A Twin-Centre Review
Authors: K Siau, S Hingley, A Veitch, N Fisher
Journal: Gut (2016)

<h3>Introduction</h3> The “Be Clear on Cancer” campaign was launched by Public Health England to raise awareness of gastro-oesophageal cancers, and ran for four weeks from January to February 2015.<sup>1</sup> The key message advertised on television was: ‘Having heartburn, most days, for 3 weeks or more, could be a sign of cancer – tell your doctor.’ We aimed to measure how this campaign affected the following: 1) 2 WW gastroscopy referrals, 2) incidence of target diagnoses (Barrett’s and gastro-oesophageal cancer, 3) stage of cancers at diagnosis, 4) cancer survival. <h3>Methods</h3> We performed a retrospective study using pooled data from 2 NHS trusts (Royal Wolverhampton and Dudley Group NHS Trust), with a combined population of 699000 in 2014 and 798000 in 2015. Patients referred for 2 WW open access endoscopy for 3 months after campaign start (Feb to April 2015) were identified from Trust clinical databases. Diagnoses, endoscopy, staging, and 9 month survival were compared with data from corresponding months in 2014. Analyses were performed using Fisher’s exact and t-test. <h3>Results</h3> 832 referrals were received in the 3 months of 2015, compared with 519 in 2014. After adjusting for population, 2 WW endoscopy demand had increased by 40.4% (p &lt; 0.03). 63 cases of Barrett’s and carcinoma were diagnosed in 2015, compared with 50 cases in 2015. Overall, there was 10.4% increase in detection of significant diagnoses (<i>p</i> = 0.6). 23 cases of oesophageal malignancy were detected, compared with 19 in 2014. The incidence of gastro-oesophageal cancer was 11.5 per 100,000 in 2015, compared with 10.8 per 100,000 population in 2014 (relative increase of 6%, p = 0.85). Due to the increase in gastroscopies performed, the yield of significant diagnoses fell from 8.0% in 2014 to 6.7% in 2015. 8/20 patients had presented with T2 or earlier stage of oesophageal cancer, compared with 3/16 in 2014 (odds ratio 2.9 for ≤T2 disease, p = 0.28). 8/20 had localised disease (N0 and M0) in 2015, with 6/18 in 2014 (OR 1.33 for localised disease, p = 0.74). The unadjusted 9 month mortality rates were 38% (10/28) in 2015 and 52% (12/23) (OR for 9 month survival in 2015 was 0.57, p = 0.39). <h3>Conclusion</h3> The “Be Clear on Cancer” campaign significantly increased the demand for 2 WW gastroscopies. A trend towards a higher incidence of Barrett’s or cancer diagnosis was noted, although the overall yield of endoscopy procedures was lower. The odds ratios appear promising for diagnostic yield, earlier presentation and 9 month survival but this was not statistically significant. Larger studies are required to validate our findings. <h3>Reference</h3> 1 https://www.england.nhs.uk/wp-content/uploads/2014/09/bcoc-tri-partite-lett-0914.pdf (accessed 25/2/16). <h3>Disclosure of Interest</h3> None Declared
Final Results of the Phase II SAPHIRE Trial of Resminostat (4SC-201) in Patients with Relapsed/Refractory Hodgkin Lymphoma
Authors: Jan Walewski, Ewa Paszkiewicz-Kozik, Agnieszka Warszewska, Gabriela Borsaru, Andreea Moicean, Andrzej Hellmann, Jiri Mayer, Bernhard Hauns, Anna Mais, Stefan W. Henning, Bernd Hentsch
Journal: Blood (2011)
Epigenetic Control Of Cell Cycle-Promoting Genes By Ikaros and Casein Kinase II In B-Cell Acute Lymphoblastic Leukemia
Authors: Sinisa Dovat, Chunhua Song, Xiaokang Pan, Yali Ding, Chandrika S. Gowda, Marie S Bulathsinghala, Mansi Sachdev, Haijun Wang, Kimberly J Payne
Journal: Blood (2013)
AML with Multilineage Dysplasia (MLD) Correlates with MDS-Related Cytogenetic Abnormalities and a Prior History of MDS or MDS/MPN but Has No Independent Prognostic Relevance: A Comparison of 461 Cases Classified as “AML Not Otherwise specified” or “AML with Myelodysplasia-Related changes” as Defined by the 2008 WHO Classification.
Authors: Miriam Miesner, Tamara Weiss, Katja Macijewski, Ulrike Bacher, Susanne Schnittger, Claudia Haferlach, Wolfgang Kern, Torsten Haferlach
Journal: Blood (2009)
SAT0029 Dectin-1 Mediates Aberrant Innate and Adaptive Immune Response in Patients with Systemic Lupus Erythematosus
Authors: M.Y. Mok, I.K. Lam, Y. Lo, D. Luk, W.C. Lau, W.K. Chan
Journal: Annals of the Rheumatic Diseases (2016)

<h3>Background</h3> Dectin-1 is a c-type lectin receptor that signals via syk and is involved in anti-fungal immunity. Dectin-1 was found to trigger experimental inflammatory arthritis, and likely play a role in the pathogenesis of some autoimmune diseases. <h3>Objectives</h3> To examine (1) dectin-1 expression on circulating CD14+ monocytes in patients with systemic lupus erythematosus (SLE), (2) the effects of dectin-1 stimulation in ROS production by lupus monocytes and (3) syk signaling and cytokine profile of dectin-1 stimulated lupus monocyte-derived dendritic cells (MDDCs). <h3>Methods</h3> SLE patients with active and inactive diseases and healthy subjects were recruited. MDDCs were derived from peripheral monocytes in the presence of IL-4 and GM-CSF. Dectin-1 agonists including curdlan, zymosan and toll-like receptor agonists Pam<sub>3</sub>CSK<sub>4</sub> (TLR2) and LPS (TLR4) were used to stimulate monocytes and/or MDDCs. Dectin-1, ROS and phosphorylated-syk (p-syk) were measured by flow cytometry. Cytokine profile was measured by and multi-bead immunoassay. <h3>Results</h3> The percentage of dectin-1 expressing monocytes was significantly lower in active SLE patients (64.5±24.3%) compared to inactive patients (89.6±7.2%) and healthy controls (91.7±9.5%) (both p&lt;0.001). The absolute count of dectin-1 expressing monocytes correlated significantly and inversely with SLEDAI (r=-0.40, p&lt;0.001), anti-dsDNA antibody level (r=-0.29, p=0.004), C3 (r=0.35, p=0.001) and C4 (r=0.24, p=0.02). Despite this, ROS production upon stimulation by dectin-1 agonists was comparable between these 3 groups. Stimulation of dectin-1 led to activation and maturation of MDDCs with upregulation of HLA-DR and CD86 (all p&lt;0.001). SLE MDDCs showed higher p-syk activation compared to normal MDDCs upon dectin-1 stimulation (6.0±2.0 vs 2.8±1.0%, p&lt;0.001). Curdlan-stimulated MDDCs produced higher levels of IL-1β, IL-23 and TNF-α. Adding TLR2 agonist to curdlan, SLE MDDCs produced significantly higher level of IL-1β (327.1±51.5 vs 125.3±88.5, p=0.009) and IL-6 (median 39.8 vs 21.7, p=0.03) compared to normal MDDCs. Combination of TLR4 agonist and curdlan induced IL-12 and TNF-α in normal MDDCs whereas lupus MDDCs produced predominant IL-6 and TNF-α. <h3>Conclusions</h3> Active SLE patients had significantly lower circulating dectin-1 expressing CD14+ monocytes which produced comparable level of ROS upon stimulation compared to inactive patients and healthy subjects. Dectin-1 agonists led to activation, maturation and higher p-syk activation in SLE MDDCs. Concomitant dectin-1 and TLR2 stimulation induced production of Th17 promoting cytokines, among which IL-1β and IL-6 were significantly higher in SLE compared to normal MDDCs. <h3>Disclosure of Interest</h3> None declared
Prognostic Impact of Wilms Tumor 1 Single Nucleotide Polymorphism rs16754 In Older Patients with Acute Myeloid Leukemia
Authors: Aline Renneville, Nicolas Boissel, Nathalie Helevaut, Olivier Nibourel, Christine Terré, Cécile Pautas, Claude Gardin, Xavier Thomas, Pascal Turlure, Oumedaly Reman, Céline Berthon, Hervé Dombret, Sylvie Castaigne, Claude Preudhomme
Journal: Blood (2010)
Assessment of a Revised Management Strategy for Patients With Intraductal Papillary Mucinous Neoplasms Involving the Main Pancreatic Duct
Authors: Motokazu Sugimoto, Irmina A. Elliott, Andrew H. Nguyen, Stephen Kim, V. Raman Muthusamy, Rabindra Watson, O. Joe Hines, David W. Dawson, Howard A. Reber, Timothy R. Donahue
Journal: JAMA Surgery (2016)

According to the 2012 International Consensus Guidelines, the diagnostic criterion of intraductal papillary mucinous neoplasms (IPMNs) involving the main duct (MD IPMNs) or the main and branch ducts (mixed IPMNs) of the pancreatic system is a main pancreatic duct (MPD) diameter of 5.0 mm or greater on computed tomography (CT) or magnetic resonance imaging (MRI). However, surgical resection is recommended for patients with an MPD diameter of 10.0 mm or greater, which is characterized as a high-risk stigma. An MPD diameter of 5.0 to 9.0 mm is not an indication for immediate resection.To determine an appropriate cutoff (ie, one with high sensitivity and negative predictive value) of the MPD diameter on CT or MRI as a prognostic factor for malignant disease and to propose a new management algorithm for patients with MD or mixed IPMNs.This retrospective cohort study included 103 patients who underwent surgical resection for a preoperative diagnosis of MD or mixed IPMN and in whom IPMN was confirmed by surgical pathologic findings at a single institution from July 1, 1996, to December 31, 2015.Malignant disease was defined as high-grade dysplasia or invasive adenocarcinoma on results of surgical pathologic evaluation. An appropriate MPD diameter on preoperative CT or MRI to predict malignant disease was determined using a receiver operating characteristic curve analysis. The prognostic value of the new management algorithm that incorporated the new MPD diameter cutoff was evaluated.Among the 103 patients undergoing resection for an MD or mixed IPMN (59 men [57.3%]; 44 women [42.7%]; median [range] age, 71 [48-86] years), 64 (62.1%) had malignant disease. Diagnostic accuracy for malignant neoplasms was highest at an MPD diameter cutoff of 7.2 mm (area under the receiver operating characteristic curve, 0.70; 95% CI, 0.59-0.81). An MPD diameter of 7.2 mm or greater was also an independent prognostic factor for malignant neoplasms (odds ratio, 12.76; 95% CI, 2.43-66.88; P = .003) on logistic regression analysis after controlling for preoperative variables. The new management algorithm, which included an MPD diameter of 7.2 mm or greater as one of the high-risk stigmata, had a higher sensitivity (100%), negative predictive value (100%), and accuracy (66%) for malignant disease than the 2012 version of the International Consensus Guidelines (95%, 57%, and 63%, respectively).In this single-center, retrospective analysis, an MPD diameter of 7.2 mm was identified as an optimal cutoff for a prognostic factor for malignant disease in MD or mixed IPMN. These data support lowering the accepted criteria for MPD diameter when selecting patients for resection vs surveillance so as not to overlook cancer in IPMN.
Telehematology: Expanding Comprehensive Care for Pediatric Patients with Blood Disorders through Telemedicine at Several Delivery Sites (Medical Homes, Hospital Clinics, Home Setting and Hemophilia Treatment Centers (HTC)) and with Interdisciplinary Providers
Authors: Roshni Kulkarni, Laura Carlson, Rebecca A Malouin, Marcia L Bird, Colleen Vallad-Hix, Michelle Witkop, Ajovi Scott-Emuakpor, Renuka Gera, Steve Gualdoni
Journal: Blood (2015)
Combined Autophagy and Proteasome Inhibition for Multiple Myeloma: Final Results of a Phase 1 Trial of Hydroxychloroquine and Standard Dose Bortezomib for Patients with Relapsed or Refractory Myeloma
Authors: Dan T. Vogl, Edward A. Stadtmauer, James Bradner, Lisa Davis, Thomas M. Paul, Emma C. Scott, Charles W. Nichols, David L. Porter, Martin Carroll, Janeen Kaplan, Gayle Mallon, Cezary R. Swider, Patricia A. Mangan, Brenda K. Shelly, Ravi K. Amaravadi
Journal: Blood (2011)
FRI0379 Survival Analysis in Antineutrophil Cytoplasmic Antibody-Associated Vasculitis
Authors: L. Petelytska, O. Iaremenko
Journal: Annals of the Rheumatic Diseases (2016)

<h3>Background</h3> The mortality of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis is generally greater than for other types of vasculitis. The long-term survival of patients (pts) with ANCA-associated vasculitis in Ukraine is uncertain. <h3>Objectives</h3> To determine predictors of survival of pts with ANCA-associated vasculitis. <h3>Methods</h3> Outcome data was collected for 49 pts who had been diagnosed with ANCA-associated vasculitis (27 pts with granulomatosis with polyangiitis (GPA), 10 pts with microscopic polyangiitis (MPA) and 12 pts with eosinophilic granulomatosis with polyangiitis) according to the ACR 1990 criteria or the CHCC diagnostic criteria in the two city hospitals in Kyiv between 1972 and 2013. There were 15 males and 34 women aged from 20 to 77 years (median age 48.5±13.8). The duration of disease at time of diagnosis was from 1 to 156 months (mean 22.5±34.3 months). All pts received glucocorticoids, 37 - cytotoxic drugs and 3 – rituximab according to local treatment protocols. Survival was evaluated as a function of the main demographics, clinical and laboratory parameters and Birmingham Vasculitis Activity Score (BVAS) assessed at diagnosis. Statistical analyses used the Kaplan-Meier method and the multivariate Cox proportional hazards regression model. <h3>Results</h3> The median duration of follow-up was 62 months and 6 deaths (12.2%) were recorded (4 pts with MPA and 2 pts with GPA). The predominant causes of deaths were renal failure and disease activity. Cumulative survival rates at 1, 3, 5 and 10 years was 91.8, 87.6, 87.6 and 76.7%, respectively. Comparison survival of pts with ANCA-associated vasculitis according to age at onset of the disease and sex revealed that gender and age cumulative survival function didn9t differ significantly. According to univariate analysis, a poor prognostic value was attributed to serum creatinine more than 176 umol/l (p=0.0001) and hemorrhagic alveolitis (p=0.01). Other factors (including ear, nose and throat, joints, skin, heart and nervous system involvement, increased ESR, leukocytosis, anemia, ANCA positivity, etc) didn9t have influence on the survival curves. Survival curves of groups of pts with different disease activity determined by BVAS were not significantly different (p=0.88). Multivariate analysis showed that presence of increased creatinine levels more than 176 umol/l (HR=3.04, p=0.0001) and hemorrhagic alveolitis (HR=1.94, p=0.02) were independent predictors of poor outcome in pts with GPA and MPA. <h3>Conclusions</h3> Cumulative survival of pts with ANCA-associated vasculitis at 1, 3, 5 and 10 years of observation is 92, 88, 88 and 77%, respectively. The increased creatinine levels more than 176 umol/l and presence of hemorrhagic alveolitis are independent predictors of poor survival of pts with GPA and MPA. Vasculitis activity detected by BVAS and other clinical and laboratory parameters no effect on mortality. <h3>Disclosure of Interest</h3> None declared
Inhibition of the hedgehog pathway in patients with basal-cell nevus syndrome: final results from the multicentre, randomised, double-blind, placebo-controlled, phase 2 trial
Authors: Jean Y Tang, Mina S Ally, Anita M Chanana, Julian M Mackay-Wiggan, Michelle Aszterbaum, Joselyn A Lindgren, Grace Ulerio, Melika R Rezaee, Ginny Gildengorin, Jackleen Marji, Charlotte Clark, David R Bickers, Ervin H Epstein
Journal: The Lancet Oncology (2016)
Mechanism by Which Exogenous Administration of Flt3 Ligand (FL) Expands Natural Killer Cells In Vivo.
Authors: Martin Guimond, Aharon G. Freud, Hsiaoyin C. Mao, Bradley W. Blaser, Gerritt Gerritt Lagemann, Jeffrey B. Vandeusen, Adrienne Dorrance, Amy K. Ferketich, Marta Cavalcanti, Crystal L. Mackall, Michael A. Caligiuri
Journal: Blood (2007)