tbuckley/cabot-search huggingface dataset:
Normalizing hepcidin predicts TMPRSS6 mutation status in patients with chronic iron deficiency
Authors: Matthew M. Heeney, Dongjing Guo, Luigia De Falco, Dean R. Campagna, Gordana Olbina, Paige P.-C. Kao, Klaus Schmitz-Abe, Fedik Rahimov, Patrick Gutschow, Keith Westerman, Vaughn Ostland, Tracy Jackson, Robert J. Klaassen, Kyriacos Markianos, Karin E. Finberg, Achille Iolascon, Mark Westerman, Wendy B. London, Mark D. Fleming
Journal: Blood (2018)
Selective FcγR Co-engagement on APCs Modulates the Activity of Therapeutic Antibodies Targeting T Cell Antigens
Authors: Jeremy D. Waight, Dhan Chand, Sylvia Dietrich, Randi Gombos, Thomas Horn, Ana M. Gonzalez, Mariana Manrique, Lukasz Swiech, Benjamin Morin, Christine Brittsan, Antoine Tanne, Belinda Akpeng, Ben A. Croker, Jennifer S. Buell, Robert Stein, David A. Savitsky, Nicholas S. Wilson
Journal: Cancer Cell (2018)
Risk stratification of smoldering multiple myeloma incorporating revised IMWG diagnostic criteria
Authors: Arjun Lakshman, S. Vincent Rajkumar, Francis K. Buadi, Moritz Binder, Morie A. Gertz, Martha Q. Lacy, Angela Dispenzieri, David Dingli, Amie L. Fonder, Suzanne R. Hayman, Miriam A. Hobbs, Wilson I. Gonsalves, Yi Lisa Hwa, Prashant Kapoor, Nelson Leung, Ronald S. Go, Yi Lin, Taxiarchis V. Kourelis, Rahma Warsame, John A. Lust, Stephen J. Russell, Steven R. Zeldenrust, Robert A. Kyle, Shaji K. Kumar
Journal: Blood Cancer Journal (2018)

Abstract In 2014, the International Myeloma Working Group reclassified patients with smoldering multiple myeloma (SMM) and bone marrow-plasma cell percentage (BMPC%) ≥ 60%, or serum free light chain ratio (FLCr) ≥ 100 or &gt;1 focal lesion on magnetic resonance imaging as multiple myeloma (MM). Predictors of progression in patients currently classified as SMM are not known. We identified 421 patients with SMM, diagnosed between 2003 and 2015. The median time to progression (TTP) was 57 months (CI, 45–72). BMPC% &gt; 20% [hazard ratio (HR): 2.28 (CI, 1.63–3.20); p &lt; 0.0001]; M-protein &gt; 2g/dL [HR: 1.56 (CI, 1.11–2.20); p = 0.01], and FLCr &gt; 20 [HR: 2.13 (CI, 1.55–2.93); p &lt; 0.0001] independently predicted shorter TTP in multivariate analysis. Age and immunoparesis were not significant. We stratified patients into three groups: low risk (none of the three risk factors; n = 143); intermediate risk (one of the three risk factors; n = 121); and high risk (≥2 of the three risk factors; n = 153). The median TTP for low-, intermediate-, and high-risk groups were 110, 68, and 29 months, respectively ( p &lt; 0.0001). BMPC% &gt; 20%, M-protein &gt; 2 g/dL, and FLCr &gt; 20 at diagnosis can be used to risk stratify patients with SMM. Patients with high-risk SMM need close follow-up and are candidates for clinical trials aiming to prevent progression.
Radiomics Signature on Magnetic Resonance Imaging: Association with Disease-Free Survival in Patients with Invasive Breast Cancer
Authors: Hyunjin Park, Yaeji Lim, Eun Sook Ko, Hwan-ho Cho, Jeong Eon Lee, Boo-Kyung Han, Eun Young Ko, Ji Soo Choi, Ko Woon Park
Journal: Clinical Cancer Research (2018)

Abstract Purpose: To develop a radiomics signature based on preoperative MRI to estimate disease-free survival (DFS) in patients with invasive breast cancer and to establish a radiomics nomogram that incorporates the radiomics signature and MRI and clinicopathological findings. Experimental Design: We identified 294 patients with invasive breast cancer who underwent preoperative MRI. Patients were randomly divided into training (n = 194) and validation (n = 100) sets. A radiomics signature (Rad-score) was generated using an elastic net in the training set, and the cutoff point of the radiomics signature to divide the patients into high- and low-risk groups was determined using receiver-operating characteristic curve analysis. Univariate and multivariate Cox proportional hazards model and Kaplan–Meier analysis were used to determine the association of the radiomics signature, MRI findings, and clinicopathological variables with DFS. A radiomics nomogram combining the Rad-score and MRI and clinicopathological findings was constructed to validate the radiomic signatures for individualized DFS estimation. Results: Higher Rad-scores were significantly associated with worse DFS in both the training and validation sets (P = 0.002 and 0.036, respectively). The radiomics nomogram estimated DFS [C-index, 0.76; 95% confidence interval (CI); 0.74–0.77] better than the clinicopathological (C-index, 0.72; 95% CI, 0.70–0.74) or Rad-score–only nomograms (C-index, 0.67; 95% CI, 0.65–0.69). Conclusions: The radiomics signature is an independent biomarker for the estimation of DFS in patients with invasive breast cancer. Combining the radiomics nomogram improved individualized DFS estimation. Clin Cancer Res; 24(19); 4705–14. ©2018 AACR.
Abstract 65: Anemia-related Heart Failure in Sub-saharan African Sickle Cell Disease Patients
Authors: Adebayo C Atanda, Yahya Aliyu, Oluwafunmilayo Atanda, Aliyu Babadoko, Aisha Suleiman, Aisha Mamman, Zakari Aliyu
Journal: Circulation Research (2017)

Introduction: Anemia has been implicated in heart failure. Existing literatures, involving predominantly African-Americans, suggests that Sickle Cell Disease (SCD) maybe linked to various cardiovascular complications including pulmonary hypertension and left venticular dysfunction. Peculiarly, our study involves exclusively Sub-Saharan population. Method: We conducted a cross sectional observational study of 208 hydroxyurea-naive consecutive SCD patients aged 10-52 years at steady state and 94 healthy non-matched controls who were studied in an out patient clinic in Sub-Saharan Africa. SCD patients were required to have electrophoretic or liquid chromatography documentation of major sickling phenotypes. Control group was required to have non-sickling phenotypes. Cardiac measurements were performed with TransThoracic Echo according to American Society of Echocardiography guidelines. Hemoglobin level was also obtained. Results: Hemoglobin level in SCD group (8.5+/- 1.5) was significant (P&lt;0.001) compared to control (13.8+/- 1.7). Although SCD group had significantly higher values of left ventricular (LV) size, there was no qualitative evidence of LV dysfunction. SCD group had higher values of Ejection Fraction but not statistically significant. There was no evidence of LV wall stiffening to impair proper filling in SCD group, with the ratio of early to late ventricular filling velocities, E/A ratio elevated (1.7+/-0.4 compared to 1.6+/- 0.4; P=0.010). Right ventricular systolic pressure was determined using the formula of 4x Tricuspid Reugurgitant jet (TRV) square as an indirect measurement of Pulmonary arterial systolic pressure. SCD patients had significantly higher mean±SD values for tricuspid regurgitant jet velocity than did the controls (2.1±0.6 vs. 1.8±0.5; p= 0.001). Within the SCD group, there was no clear pattern of worsening diastolic function with increased TRV. Furthermore, E/A had a significant positive relationship with jet velocity in bivariate analysis (R=0.20; P=0.013). Conclusions: We were unable to demonstrate existence of anemia-associated left ventricular dysfunction in Sub-Saharan African with SCD. Further studies is required to highlight the reason behind this finding.
Review: In CAD, CABG reduced 5-year mortality more than PCI in multivessel but not left main disease
Authors: Eric R. Bates
Journal: Annals of Internal Medicine (2018)

Source Citation Head SJ, Milojevic M, Daemen J, et al. Mortality after coronary artery bypass grafting versus percutaneous coronary intervention with stenting for coronary artery disease: a pooled analysis of individual patient data. Lancet. 2018;391:939-48. 29478841
SAT0537 Is balance affected in behcet’s disease and what are the factors that effect the balance?
Authors: A. Celİk, M. A. Melİko&gbreve;lu
Journal: Annals of the Rheumatic Diseases (2018)

Objectives: The aim of this EULAR Task Force was to identify a core set of data items which can easily be collected from clinicians and facilitates examination of disease course and outcome.Methods: A multidisciplinary EULAR task force group of 20 experts including rheumatologists, epidemiologists and patient representatives was assembled and breakout groups formed for a meeting at which items from a previously compiled collection of core parameters for a GCA registry were evaluated.Results were presented to the other group members following a structured process for discussion and consensus finding.The meeting was followed by several rounds of discussions to achieve consensus.Results: A total of 95 items were identified, subdivided into the following categories: General, Demographics, GCA-related signs and symptoms, Other medical conditions, and Treatment.Suitable instruments and assessment intervals were determined for documentation of each item.To facilitate implementation of the recommendations in both primary care and scientifically oriented registers, a minimum core set of parameters was distilled, with supplemental items that can be added optionally depending on the designated purpose of individual registers.
Increased Expression of Programmed Death-Ligand 1 in Infiltrating Immune Cells in Hepatocellular Carcinoma Tissues after Sorafenib Treatment
Authors: Li-Chun Lu, Yi-Hsuan Lee, Chun-Jung Chang, Chia-Tung Shun, Chih-Yeu Fang, Yu-Yun Shao, Tsung-Hao Liu, Ann-Lii Cheng, Chih-Hung Hsu
Journal: Liver Cancer (2018)

Programmed death-ligand 1 (PD-L1) expression in the tumor microenvironment (TME) has been reported to be related to prognosis in patients with hepatocellular carcinoma (HCC) after hepatectomy. The impact of sorafenib on PD-L1 expression in the TME of advanced HCC is unclear.Patients with HCC who received sorafenib for advanced disease at National Taiwan University Hospital, Taipei, Taiwan, and who had paired HCC tissues obtained before and after sorafenib treatment were included in the study group. HCC patients not treated with sorafenib who had paired primary and recurrent or metastatic tissues were identified as the reference group. The membrane PD-L1 staining, detected by immunohistochemistry (IHC) using SP142 antibody, was semiquantitatively scored in tumor cells (TCs) or tumor-infiltrating immune cells (ICs). Additional IHC assays were employed to characterize the PD-L1-expressing ICs.Twenty-three advanced HCC patients with pre- and post-sorafenib paired HCC tissues were included in the study group. The median duration of sorafenib treatment was 4.3 months (range: 1.3-18.7). PD-L1 expression in ICs was significantly higher in post-sorafenib HCC tissues than in pre-sorafenib HCC tissues (pre-sorafenib vs. post-sorafenib IHC 0/1/2/3: 11/5/5/2 vs. 5/5/2/11, p = 0.016). However, PD-L1 expression in TCs was not significantly different between pre- and post-sorafenib tissues (IHC 0/1/2/3: 19/2/0/2 vs. 14/5/0/4, p = 0.094). In the reference group of 44 patients not treated with sorafenib, PD-L1 expression in ICs and TCs was not significantly different between the paired primary and metastatic HCC tissues. By performing IHC double staining with PD-L1 and CD68, we found the PD-L1-expressing ICs were mainly CD68-positive macrophages. PD-L1 expression levels of pre- and post-sorafenib tissues were not associated with patients' overall survival or duration of sorafenib treatment.PD-L1 expression in ICs was significantly increased in post-sorafenib HCC tissues. The mechanisms and clinical significance of this observation warrants further investigation.
Inherited p40phox deficiency differs from classic chronic granulomatous disease
Authors: Annemarie van de Geer, Alejandro Nieto-Patlán, Douglas B. Kuhns, Anton T.J. Tool, Andrés A. Arias, Matthieu Bouaziz, Martin de Boer, José Luis Franco, Roel P. Gazendam, John L. van Hamme, Michel van Houdt, Karin van Leeuwen, Paul J.H. Verkuijlen, Timo K. van den Berg, Juan F. Alzate, Carlos A. Arango-Franco, Vritika Batura, Andrea R. Bernasconi, Barbara Boardman, Claire Booth, Siobhan O. Burns, Felipe Cabarcas, Nadine Cerf Bensussan, Fabienne Charbit-Henrion, Anniek Corveleyn, Caroline Deswarte, María Esnaola Azcoiti, Dirk Foell, John I. Gallin, Carlos Garcés, Margarida Guedes, Claas H. Hinze, Steven M. Holland, Stephen M. Hughes, Patricio Ibañez, Harry L. Malech, Isabelle Meyts, Marcela Moncada-Velez, Kunihiko Moriya, Esmeralda Neves, Matias Oleastro, Laura Perez, Vimel Rattina, Carmen Oleaga-Quintas, Neil Warner, Aleixo M. Muise, Jeanet Serafín López, Eunice Trindade, Julia Vasconcelos, Séverine Vermeire, Helmut Wittkowski, Austen Worth, Laurent Abel, Mary C. Dinauer, Peter D. Arkwright, Dirk Roos, Jean-Laurent Casanova, Taco W. Kuijpers, Jacinta Bustamante
Journal: Journal of Clinical Investigation (2018)

Biallelic loss-of-function (LOF) mutations of the NCF4 gene, encoding the p40phox subunit of the phagocyte NADPH oxidase, have been described in only 1 patient. We report on 24 p40phox-deficient patients from 12 additional families in 8 countries. These patients display 8 different in-frame or out-of-frame mutations of NCF4 that are homozygous in 11 of the families and compound heterozygous in another. When overexpressed in NB4 neutrophil-like cells and EBV-transformed B cells in vitro, the mutant alleles were found to be LOF, with the exception of the p.R58C and c.120_134del alleles, which were hypomorphic. Particle-induced NADPH oxidase activity was severely impaired in the patients' neutrophils, whereas PMA-induced dihydrorhodamine-1,2,3 (DHR) oxidation, which is widely used as a diagnostic test for chronic granulomatous disease (CGD), was normal or mildly impaired in the patients. Moreover, the NADPH oxidase activity of EBV-transformed B cells was also severely impaired, whereas that of mononuclear phagocytes was normal. Finally, the killing of Candida albicans and Aspergillus fumigatus hyphae by neutrophils was conserved in these patients, unlike in patients with CGD. The patients suffer from hyperinflammation and peripheral infections, but they do not have any of the invasive bacterial or fungal infections seen in CGD. Inherited p40phox deficiency underlies a distinctive condition, resembling a mild, atypical form of CGD.
AB1076 Complex regional pain syndrome type 1: which treatment?
Authors: M. Sghir, S. Salah, A. Haj Salah, W. Kessomtini, Z. Ben Salah
Journal: Annals of the Rheumatic Diseases (2018)

<h3>Background</h3> The treatment of Complex Regional Pain Syndrome Type 1 (CRPS-I) is multidisciplinary.<sup>1</sup> It aims to minimise symptoms, pain, preserve functional ability and professional activity. Unfortunately, therapeutic possibilities are still very limited and there is no Gold standard. <h3>Objectives</h3> The purpose of our work is to evaluate the efficiency of four therapeutic modalities in the management of CRPS-I. <h3>Methods</h3> Retrospective study of 60 patients (21 men and 39 women) treated for CRPS-I. Four groups were identified according to therapeutic modalities used: rehabilitation alone (16 patients), Calcitonin associated with rehabilitation (15 patients), Bisphosphonates (BP) (Sodium Risedronate, 1 tablet per week, over an average duration of 1 month) associated with rehabilitation (20 patients) and Calcitonin in combination with BP and rehabilitation (9 patients). <h3>Results</h3> The mean age of the patients was 51±16.5 years. The average time of treatment was 2 months and the average duration of follow-ups was 7 months. Traumatic origin was found in 88.3% of cases. Distal radius fractures (DRF) were the most incriminated (40% of cases). The evolution was judged on pain reduction, vasomotor signs and on functional improvement. For all etiologies combined, no statistically significant difference was found between the different groups (p=0.462). For patients with a DRF, a favourable outcome was noted in the BP group associated with rehabilitation in 85.7% of cases while it was only 42.9% for rehabilitation alone. <h3>Conclusions</h3> Our study concludes that the different therapeutic modalities evaluated for the treatment of CRPS-I had an efficiency close to each other with a superiority of BP. Oral Sodium Risedronate could therefore be proposed as a treatment for CRPS-I without marketing authorisation (MA). <h3>Reference</h3> [1] Hernández-Porras BC, Plancarte-Sánchez R, Alarcón-Barrios S, Sámano-García M. Complex regional pain syndrome: A review. Cirugía y Cirujanos (English Edition). 1 juill 2017;85(4):366–74. <h3>Disclosure of Interest</h3> None declared
Associations Between American Board of Internal Medicine Maintenance of Certification Status and Performance on a Set of Healthcare Effectiveness Data and Information Set (HEDIS) Process Measures
Authors: Bradley Gray, Jonathan Vandergrift, Bruce Landon, James Reschovsky, Rebecca Lipner
Journal: Annals of Internal Medicine (2018)

Background: The value of the American Board of Internal Medicine's (ABIM) Maintenance of Certification (MOC) program has been questioned as a marker of physician quality. Objective: To assess whether physician MOC status is associated with performance on selected Healthcare Effectiveness Data and Information Set (HEDIS) process measures. Design: Annual comparisons of HEDIS process measures among physicians who did or did not maintain certification 20 years after initial certification. Setting: Fee-for-service Medicare. Participants: 1260 general internists who were initially certified in 1991 and provided care for 85 931 Medicare patients between 2009 and 2012. Measurements: Annual percentage of a physician's Medicare patients meeting each of 5 HEDIS annual or biennial standards and a composite indicating meeting all 3 HEDIS diabetes standards. Results: Among the 1260 physicians, 786 maintained their certification from 1991 to 2012 and 474 did not. The mean annual percentage of HEDIS-eligible diabetic patients who completed semiannual hemoglobin A1c testing was 58.4% among physicians who maintained certification and 54.4% among those who did not (regression-adjusted difference, 4.2 percentage points [95% CI, 2.0 to 6.5 percentage points]; P < 0.001). Diabetic patients of physicians who maintained certification more frequently met the annual standard for low-density lipoprotein (LDL) cholesterol measurement (83.1% vs. 80.5%; regression-adjusted difference, 2.3 percentage points [CI, 0.6 to 4.1 percentage points]; P = 0.008) and all 3 diabetic standards (46.0% vs. 41.6%; regression-adjusted difference, 3.1 percentage points [CI, 0.5 to 5.7 percentage points]; P = 0.019). The regression-adjusted difference in biennial eye examinations was statistically insignificant (P = 0.112). Measures for LDL cholesterol testing in patients with coronary heart disease and biennial mammography were also met more frequently among physicians who maintained certification (79.4% vs. 77.4% and 72.0% vs. 67.8%, respectively), with regression-adjusted differences of 1.7 percentage points (CI, 0.2 to 3.3 percentage points; P = 0.032) and 4.6 percentage points (CI, 2.9 to 6.3 percentage points; P < 0.001), respectively. Limitation: Potential confounding by unobserved patient, physician, and practice characteristics; inability to determine clinical significance of observed differences. Conclusion: Maintaining certification was positively associated with physician performance scores on a set of HEDIS process measures. Primary Funding Source: American Board of Internal Medicine.
AB1255 A review of case-mix and centre effect adjustment in early rheumatoid arthritis cohorts
Authors: M. Yates, K. Bechman, S. Norton, J. Galloway
Journal: Annals of the Rheumatic Diseases (2018)

<h3>Background</h3> Observational cohort studies have been utilised extensively in early Rheumatoid Arthritis (RA), regularly conducted across multiple centres spanning regional and national boundaries. Case-mix and centre effect are considerations essential for determining comparability of results, and likely prevalence of bias. There is currently no standardised approach for case-mix and centre effect adjustment in early RA observational cohorts. <h3>Objectives</h3> Describe the spectrum of methodologies used to address case-mix and centre level effects on outcomes in multi-centre early RA observational cohort studies. <h3>Methods</h3> Inclusion criteria were cohorts recruiting from 2 or more centres with 100 or more subjects, with a Rheumatologist diagnosis of RA or EIA within the last 24 months. A systematic electronic search of publications was undertaken. Papers were reviewed by two researchers independently. Reference lists of included papers were reviewed for further relevant publications. A search of all included papers' authors was also conducted. Detail on cohort characteristics, case-mix data collection and adjustment, and consideration of centre-level effect in analyses were collected. <h3>Results</h3> 1047 papers were identified from the initial search. A total of 20 unique cohorts were identified. Reference review and author search produced 14 more, to make a total of 34 unique observational cohorts drawn from 205 papers. The cohorts were mainly conducted in Europe (24/34, 71%), With 2 (6%) from less economically developed regions. The period of data collection was between 1955 and 2017. Case-mix: All cohorts considered case-mix in some form (e.g. age and gender), but with heterogenous approaches. The figure displays the relative frequencies of sociodemographic variable consideration across all included papers. Centre effect: 18/205 (9%) of the included papers accounted for centre in their results, utilising a range methodologies. Where reported, centre had a significant impact. <h3>Conclusions</h3> The degree of case-mix reporting varied widely, and few studies addressed centre effect. Where analysed, a centre level impact was clearly apparent. A failure to incorporate centre into analyses can lead to unrecognised bias as a result of confounding by centre. It must be acknowledged that including case-mix variables and adjusting for centre substantially reduces power, and it is likely that many of the reported observations may have lost statistical significance had case-mix and centre effect been addressed more completely. This is the first systematic review of centre effect and case-mix in early RA, and highlights a challenging field deserving further research. <h3>Disclosure of Interest</h3> None declared
Endoscopic mucosal autograft for treating esophageal caustic strictures: preliminary human experience
Authors: Kexin He, Lili Zhao, Shoushan Bu, Li Liu, Xiang Wang, Min Wang, Zhining Fan
Journal: Endoscopy (2018)

Abstract Background Esophageal caustic stricture is a stubborn disease and postoperative restenosis limits the clinical efficacy of endoscopic dilation. Autologous mucosal grafts have been successfully applied in the treatment of urethral stricture and in the prevention of stricture after extensive mucosal resection. We aimed to use mucosal autografting performed endoscopically to treat refractory esophageal stricture. Methods Three patients with intractable corrosive esophageal stricture were treated endoscopically by combining dilation with autologous mucosal transplantation. Results All procedures were successful with no severe complications. Mucosal regeneration was shown at the transplanted segments. One patient was able to maintain a normal diet with complete remission after 1 year of follow-up. Intraluminal stenosis and dysphagia were significantly improved in another two patients. Conclusions Mucosal autografting can achieve esophageal re-epithelialization, inhibit undesired fibrosis, prevent restenosis, and promote functional regeneration.