tbuckley/cabot-search huggingface dataset:
Adult‐onset cystic fibrosis liver disease: Diagnosis and characterization of an underappreciated entity
Authors: Christopher Koh, Sasan Sakiani, Pallavi Surana, Xiongce Zhao, Jason Eccleston, David E. Kleiner, David Herion, T. Jake Liang, Jay H. Hoofnagle, Milica Chernick, Theo Heller
Journal: Hepatology (2017)

Cystic fibrosis (CF) liver disease (CFLD), a leading cause of death in CF, is mostly described in pediatric populations. Adult‐onset CFLD lacks sufficient characterization and diagnostic tools. A cohort of CF patients without CFLD during childhood were followed for up to 38 years with serologic testing, imaging, and noninvasive fibrosis markers. Historical CFLD diagnostic criteria were compared with newly proposed CFLD criteria. Thirty‐six CF patients were followed for a median of 24.5 years (interquartile range 15.6‐32.9). By the last follow‐up, 11 (31%) had died. With conventional criteria, 8 (22%) patients had CFLD; and by the new criteria, 17 (47%) had CFLD at a median age of 36.6 years (interquartile range 26.5‐43.2). By the new criteria, those with CFLD had higher median alanine aminotransferase (42 versus 27, P = 0.005), aspartate aminotransferase (AST; 26 versus 21, P = 0.01), direct bilirubin (0.13 versus 0.1, P = 0.01), prothrombin time (14.4 versus 12.4, P = 0.002), and AST‐to‐platelet ratio index (0.31 versus 0.23, P = 0.003) over the last 2 years of follow‐up. Subjects with a FibroScan &gt;6.8 kPa had higher alanine aminotransferase (42 versus 28U/L, P = 0.02), AST (35 versus 25U/L, P = 0.02), AST‐to‐platelet ratio index (0.77 versus 0.25, P = 0.0004), and Fibrosis‐4 index (2.14 versus 0.74, P = 0.0003) and lower platelet counts (205 versus 293, P = 0.02). One CFLD patient had nodular regenerative hyperplasia. Longitudinally, mean platelet counts significantly declined in the CFLD group (from 310 to 230 U/L, P = 0.0005). Deceased CFLD patients had lower platelet counts than those alive with CFLD (143 versus 258 U/L, P = 0.004) or those deceased with no CFLD (143 versus 327U/L, P = 0.006). Conclusion : Adult‐onset CFLD may be more prevalent than previously described, which suggests a later wave of CFLD that impacts morbidity; routine liver tests, radiologic imaging, noninvasive fibrosis markers, and FibroScan can be used algorithmically to identify adult CFLD; and further evaluation in other CF cohorts should be performed for validation. (H epatology 2017;66:591–601).
Large-scale analyses of common and rare variants identify 12 new loci associated with atrial fibrillation
Authors: Ingrid E Christophersen, Michiel Rienstra, Carolina Roselli, Xiaoyan Yin, Bastiaan Geelhoed, John Barnard, Honghuang Lin, Dan E Arking, Albert V Smith, Christine M Albert, Mark Chaffin, Nathan R Tucker, Molong Li, Derek Klarin, Nathan A Bihlmeyer, Siew-Kee Low, Peter E Weeke, Martina Müller-Nurasyid, J Gustav Smith, Jennifer A Brody, Maartje N Niemeijer, Marcus Dörr, Stella Trompet, Jennifer Huffman, Stefan Gustafsson, Claudia Schurmann, Marcus E Kleber, Leo-Pekka Lyytikäinen, Ilkka Seppälä, Rainer Malik, Andrea R V R Horimoto, Marco Perez, Juha Sinisalo, Stefanie Aeschbacher, Sébastien Thériault, Jie Yao, Farid Radmanesh, Stefan Weiss, Alexander Teumer, Seung Hoan Choi, Lu-Chen Weng, Sebastian Clauss, Rajat Deo, Daniel J Rader, Svati H Shah, Albert Sun, Jemma C Hopewell, Stephanie Debette, Ganesh Chauhan, Qiong Yang, Bradford B Worrall, Guillaume Paré, Yoichiro Kamatani, Yanick P Hagemeijer, Niek Verweij, Joylene E Siland, Michiaki Kubo, Jonathan D Smith, David R Van Wagoner, Joshua C Bis, Siegfried Perz, Bruce M Psaty, Paul M Ridker, Jared W Magnani, Tamara B Harris, Lenore J Launer, M Benjamin Shoemaker, Sandosh Padmanabhan, Jeffrey Haessler, Traci M Bartz, Melanie Waldenberger, Peter Lichtner, Marina Arendt, Jose E Krieger, Mika Kähönen, Lorenz Risch, Alfredo J Mansur, Annette Peters, Blair H Smith, Lars Lind, Stuart A Scott, Yingchang Lu, Erwin B Bottinger, Jussi Hernesniemi, Cecilia M Lindgren, Jorge A Wong, Jie Huang, Markku Eskola, Andrew P Morris, Ian Ford, Alex P Reiner, Graciela Delgado, Lin Y Chen, Yii-Der Ida Chen, Roopinder K Sandhu, Man Li, Eric Boerwinkle, Lewin Eisele, Lars Lannfelt, Natalia Rost, Christopher D Anderson, Kent D Taylor, Archie Campbell, Patrik K Magnusson, David Porteous, Lynne J Hocking, Efthymia Vlachopoulou, Nancy L Pedersen, Kjell Nikus, Marju Orho-Melander, Anders Hamsten, Jan Heeringa, Joshua C Denny, Jennifer Kriebel, Dawood Darbar, Christopher Newton-Cheh, Christian Shaffer, Peter W Macfarlane, Stefanie Heilmann-Heimbach, Peter Almgren, Paul L Huang, Nona Sotoodehnia, Elsayed Z Soliman, Andre G Uitterlinden, Albert Hofman, Oscar H Franco, Uwe Völker, Karl-Heinz Jöckel, Moritz F Sinner, Henry J Lin, Xiuqing Guo, Martin Dichgans, Erik Ingelsson, Charles Kooperberg, Olle Melander, Ruth J F Loos, Jari Laurikka, David Conen, Jonathan Rosand, Pim van der Harst, Marja-Liisa Lokki, Sekar Kathiresan, Alexandre Pereira, J Wouter Jukema, Caroline Hayward, Jerome I Rotter, Winfried März, Terho Lehtimäki, Bruno H Stricker, Mina K Chung, Stephan B Felix, Vilmundur Gudnason, Alvaro Alonso, Dan M Roden, Stefan Kääb, Daniel I Chasman, Susan R Heckbert, Emelia J Benjamin, Toshihiro Tanaka, Kathryn L Lunetta, Steven A Lubitz, Patrick T Ellinor
Journal: Nature Genetics (2017)
The Use of Estrogens and Progestins and the Risk of Breast Cancer in Postmenopausal Women
Authors: Graham A. Colditz, Susan E. Hankinson, David J. Hunter, Walter C. Willett, JoAnn E. Manson, Meir J. Stampfer, Charles Hennekens, Bernard Rosner, Frank E. Speizer
Journal: New England Journal of Medicine (1995)

The effect of adding progestins to estrogen therapy on the risk of breast cancer in postmenopausal women is controversial.
TCTAP C-284 Magnesium Bioresorbable Scaffold in A Bifurcation Lesion
Authors: Mohd Kamal Mohd Arshad, Hafisyatul Aiza Zainal Abidin, Effarezan Abdul Rahman, Rizmy Najme Khir, Johan Rizwal Ismail, Nicholas Yul Chye Chua, Khairul Shafiq Ibrahim, Chiao Wen Lim, Zubin Ibrahim, Sazzli Kasim
Journal: Journal of the American College of Cardiology (2017)
Genomic analyses of primitive, wild and cultivated citrus provide insights into asexual reproduction
Authors: Xia Wang, Yuantao Xu, Siqi Zhang, Li Cao, Yue Huang, Junfeng Cheng, Guizhi Wu, Shilin Tian, Chunli Chen, Yan Liu, Huiwen Yu, Xiaoming Yang, Hong Lan, Nan Wang, Lun Wang, Jidi Xu, Xiaolin Jiang, Zongzhou Xie, Meilian Tan, Robert M Larkin, Ling-Ling Chen, Bin-Guang Ma, Yijun Ruan, Xiuxin Deng, Qiang Xu
Journal: Nature Genetics (2017)

The emergence of apomixis-the transition from sexual to asexual reproduction-is a prominent feature of modern citrus. Here we de novo sequenced and comprehensively studied the genomes of four representative citrus species. Additionally, we sequenced 100 accessions of primitive, wild and cultivated citrus. Comparative population analysis suggested that genomic regions harboring energy- and reproduction-associated genes are probably under selection in cultivated citrus. We also narrowed the genetic locus responsible for citrus polyembryony, a form of apomixis, to an 80-kb region containing 11 candidate genes. One of these, CitRWP, is expressed at higher levels in ovules of polyembryonic cultivars. We found a miniature inverted-repeat transposable element insertion in the promoter region of CitRWP that cosegregated with polyembryony. This study provides new insights into citrus apomixis and constitutes a promising resource for the mining of agriculturally important genes.
Low expression of hexokinase-2 is associated with false-negative FDG–positron emission tomography in multiple myeloma
Authors: Leo Rasche, Edgardo Angtuaco, James E. McDonald, Amy Buros, Caleb Stein, Charlotte Pawlyn, Sharmilan Thanendrarajan, Carolina Schinke, Rohan Samant, Shmuel Yaccoby, Brian A. Walker, Joshua Epstein, Maurizio Zangari, Frits van Rhee, Tobias Meissner, Hartmut Goldschmidt, Kari Hemminki, Richard Houlston, Bart Barlogie, Faith E. Davies, Gareth J. Morgan, Niels Weinhold
Journal: Blood (2017)
When the goal is not cure: A randomized trial of a patient decision aid in advanced colorectal cancer
Authors: N. B. Leighl, H. Shepherd, P. Butow, S. J. Clarke, M. McJannett, P. Beale, N. Wilcken, M. J. Moore, E. X. Chen, M. H. Tattersall, D. Goldstein
Journal: Journal of Clinical Oncology (2007)

6509 Background: With improvements in treatment and supportive care, decisions in advanced cancer are increasingly complex. To facilitate decision-making, we developed a decision aid (DA) for patients considering first-line chemotherapy for incurable colorectal cancer, reviewing treatment options, prognostic information and toxicities. We examined its impact on patient understanding, consultation and decision- making satisfaction, anxiety, decisional conflict, quality of life, information preferences, and treatment decisions made. Methods: 207 newly diagnosed advanced colorectal cancer patients at 5 cancer centers in Australia and Canada were randomized to receive either a standard medical oncology consultation, or the same plus the DA, (take-home booklet with audio-recording, selected review by oncologist). Results: 100 were randomized to the control arm, 107 to receive the DA. Sample characteristics: median age 62 years, 58% male, 89% PS 0/1, 36% prior adjuvant chemotherapy. Patients in the DA arm demonstrated a greater increase in understanding of prognosis, options, benefits and toxicities (+19% vs +5.6%, p 0.001), with higher overall understanding (72% vs 60%, p&lt;0.0001). Decision and consultation satisfaction, decisional conflict and quality of life were similar between groups. Anxiety, measured serially over 4–6 weeks, was similar and decreased over time. Most arrived at a decision during the first consultation; 80% chose chemotherapy, 7.5% supportive care alone, 10.5% a wait and watch strategy, with no differences between arms. 87% wanted as much information as possible, 82% wished to share decision-making with the physician, and only 15% felt the doctor alone should make the decision. More patients who received the decision aid felt they received all possible details about therapy, (72% vs 63%). 90% felt the decision was shared in part between physician and patient. Conclusion: This first randomized trial of a decision aid in advanced cancer patients shows that its use in advanced colorectal cancer improved patient understanding of prognosis, treatment options, risks and benefits without increasing anxiety. Decision aids can improve informed consent and decision-making, and can be tested through randomized trials even in the setting of advanced cancer. No significant financial relationships to disclose.
Morbid Risks of Unnecessary Red Blood Cell Transfusion in Stable Coronary Artery Bypass Graft Patients.
Authors: Patrick Moehnle, Stephanie A. Snyder-Ramos, Yi-Shin Weng, Alexander Kulier, Bernd W. Boettiger, Shirley Wang, Jack Levin, Dennis T. Mangano
Journal: Blood (2005)
TCTAP A-057 The Relationship Between Carina Bifurcation Angle and Side Branch Occlusion in Elective Percutaneous Coronary Bifurcation Lesions Intervention
Authors: Bogie Putra Palinggi, Doni Firman, Sunarya Soerianata, Amir Aziz Alkatiri, Nur Haryono
Journal: Journal of the American College of Cardiology (2017)
Abstract 18830: Transmission of Serious Disseminated Nontuberculous Mycobacterium (NTM) Infections to Patients During Cardiothoracic Surgery: Epidemiology and Potential Remedies
Authors: Keith B Allen, David Yuh, Suzanne B Schwartz, Richard Lange, Richard A Hopkins, Kelly Bauer, Julie Marders, Jose D Donayre, Nicole Milligan, Catherine Wentz
Journal: Circulation (2016)

Introduction: Heater-cooler devices (HCDs) used in cardiac procedures provide temperature-controlled water to heat exchangers used in an extracorporeal circuit. The FDA has received reports of atyp...
A Strong and Simple Heart
Authors: Stephen E. Wilkinson
Journal: Annals of Internal Medicine (2017)

On Being a Doctor18 April 2017A Strong and Simple HeartStephen E. Wilkinson, MDStephen E. Wilkinson, MDFrom Medical College of Wisconsin, Milwaukee, Wisconsin.Search for more papers by this authorAuthor, Article, and Disclosure Informationhttps://doi.org/10.7326/M16-1521 Audio Reading - “A Strong and Simple Heart” Audio. Michael A. LaCombe, MD, Annals Associate Editor, reads "A Strong and Simple Heart," by S.E. Wilkinson. Your browser does not support the audio element. Audio player progress bar Step backward in current audio track Play current audio trackPause current audio track Step forward in current audio track Mute current audio trackUnmute current audio track 00:00/ SectionsAboutFull TextPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail It was surprising how quickly the answer came to mind. I was with a group of friends at dinner, none involved in health care, when one threw out the question, “What's the most incredible thing you've seen at the hospital?” The image of an elderly woman's face bolted to the forefront of my mind. I had not thought of that woman in ages. Perhaps my friend was thinking of guts and gore. My mind went to grace and gratitude.Mrs. S was a European immigrant. I was an intern on the cardiology service when she presented to our emergency department ... Author, Article, and Disclosure InformationAffiliations: From Medical College of Wisconsin, Milwaukee, Wisconsin.Corresponding Author: Stephen E. Wilkinson, MD, Medical Education Office, Suite 730, 999 North 92nd Street, Milwaukee, WI 53226. PreviousarticleNextarticle Advertisement Audio Reading - “A Strong and Simple Heart” Audio. Michael A. LaCombe, MD, Annals Associate Editor, reads "A Strong and Simple Heart," by S.E. Wilkinson. Your browser does not support the audio element. Audio player progress bar Step backward in current audio track Play current audio trackPause current audio track Step forward in current audio track Mute current audio trackUnmute current audio track 00:00/ FiguresReferencesRelatedDetails Metrics 18 April 2017Volume 166, Issue 8Page: 603-604KeywordsCatheterizationDyspneaEmergency departmentGratitudeHeartIschemiaNursesOxygenRunningSurgery ePublished: 18 April 2017 Issue Published: 18 April 2017 Copyright & PermissionsCopyright © 2017 by American College of Physicians. All Rights Reserved.PDF downloadLoading ...
Pregnancy and Labor Complications in Female Survivors of Childhood Cancer: The British Childhood Cancer Survivor Study
Authors: Raoul C Reulen, Chloe J Bright, David L Winter, Miranda M Fidler, Kwok Wong, Joyeeta Guha, Julie S Kelly, Clare Frobisher, Angela B Edgar, Roderick Skinner, W Hamish B Wallace, Mike M Hawkins
Journal: JNCI Journal of the National Cancer Institute (2017)

Female survivors of childhood cancer treated with abdominal radiotherapy who manage to conceive are at risk of delivering premature and low-birthweight offspring, but little is known about whether abdominal radiotherapy may also be associated with additional complications during pregnancy and labor. We investigated the risk of developing pregnancy and labor complications among female survivors of childhood cancer in the British Childhood Cancer Survivor Study (BCCSS).Pregnancy and labor complications were identified by linking the BCCSS cohort (n = 17 980) to the Hospital Episode Statistics (HES) for England. Relative risks (RRs) of pregnancy and labor complications were calculated by site of radiotherapy treatment (none/abdominal/cranial/other) and other cancer-related factors using log-binomial regression. All statistical tests were two-sided.A total of 2783 singleton pregnancies among 1712 female survivors of childhood cancer were identified in HES. Wilms tumor survivors treated with abdominal radiotherapy were at threefold risk of hypertension complicating pregnancy (relative risk = 3.29, 95% confidence interval [CI] = 2.29 to 4.71), while all survivors treated with abdominal radiotherapy were at risk of gestational diabetes mellitus (RR = 3.35, 95% CI = 1.41 to 7.93) and anemia complicating pregnancy (RR = 2.10, 95% CI = 1.27 to 3.46) compared with survivors treated without radiotherapy. Survivors treated without radiotherapy had similar risks of pregnancy and labor complications as the general population, except survivors were more likely to opt for an elective cesarean section (RR = 1.39, 95% CI = 1.16 to 1.70).Treatment with abdominal radiotherapy increases the risk of developing hypertension complicating pregnancy in Wilms tumor survivors, and diabetes mellitus and anemia complicating pregnancy in all survivors. These patients may require extra vigilance during pregnancy.
Outcome in different molecular subtypes of diffuse large B-cell lymphoma: Indian experience.
Authors: Gaurav Prakash, Atul Sharma, Vinod Raina, Lalit Kumar, Mehar C Sharma
Journal: Journal of Clinical Oncology (2012)

e18518 Background: DLBCL has emerged as a heterogeneous disease and gene expression profiling has divided it into distinct molecular subtypes of Germinal center B-Cell (GCB) and Non-GCB- cell origin. These subtypes are reported to vary in the treatment responses and outcome. Methods: We analysed 97 consecutive patients of DLBCL who had satisfactory paraffin block of baseline biopsy. After reviewing diagnosis of DLBCL, sequential immunohistochemical staining with CD10, bcl6 and MUM1 was performed in all the cases. Using these markers the cases were sub-classified into GCB and non-GCB types as per Hans’ algorithm. Baseline presentation, stage, international prognostic score and treatment outcome were recorded. Survival was assessed by Kaplan-Meier survival curves and results were compared using log-rank test. Results: Thirty-three (34%) patients were classified in GCB group while 64(66%) patients in non-GCB group. Median age in both the groups was 50 years. High IPI score2-5 (GCB-34%, non GCB-72%;p 0.005) and stage 3,4 (GCB-39%,nonGCB-61%;p 0.04) were higher in non-GCB group. All the patients were treated with 6-8 cycles of CHOP based regimen and involved field radiotherapy wherever required. Complete remission rate (GCB-52%, nonGCB-27%; p0.02) was higher in GCB group. Three year overall survival, 75% and 63 %( p0.04), and event free survival was 63% and 43 %( p0.02) in GCB and non GCB subtypes, respectively. There was no difference in between two groups for performance status, LDH level and age. Conclusions: In our study, GCB-DLBCL patients had better response rate, EFS and OS in comparison to non GCB subtype. There were higher number of patients with advanced stage and high IPI score in non-GCB subtype which might have contributed to the poorer outcome.
Final results of a phase I study of amrubicin and cyclophosphamide in patients with advanced solid organ malignancies: HOG LUN 07-130.
Authors: Shadia Ibrahim Jalal, Nasser H. Hanna, Robin Zon, Gregory A. Masters, Hossein Borghaei, Karuna Koneru, Sunil S. Badve, Nagendra K Prasad, Neeta Somaiah, Jingwei Wu, Zhangsheng Yu, Lawrence H. Einhorn
Journal: Journal of Clinical Oncology (2014)

7594 Background: Relapsed small cell lung cancer (SCLC) has limited treatment options. Anthracyclines and cyclophosphamide have shown synergy in a wide variety of tumors. Amrubicin (AMR) and cyclosphosphamide both have single agent activity in SCLC. This phase I trial evaluated the combination of AMR and cyclophosphamide in refractory solid organ malignancies and in relapsed SCLC. Methods: Eligible patients (pts) had refractory solid organ malignancies, PS 0-1, and adequate organ function. The primary endpoint was MTD and dose limiting toxicities (DLTs). Secondary endpoint was to assess response. Pts were enrolled in sequential dose escalation cohorts in a standard 3+3 design. Treatment consisted of cyclophosphamide IV at 500mg/m2 on day 1 with escalating doses of AMR IV on days 1-3 (25-40mg/m2 with increments of 5mg/m2 per cohort).Cycles were repeated every 21 days. Results: Thirty six patients (pts) were enrolled on the dose escalation portion with 18 patients with SCLC as the primary diagnosis. Remaining patients had NSCLC, extrathoracic small cell carcinoma or other malignancies. The median number of cycles was 4 (range 1-6). MTD was determined to be dose level 2 (cyclophosphamide 500mg/m2, AMR 30mg/m2) due to grade 4 thrombocytopenia. Incidence of grade 3-4 neutropenia, anemia, thrombocytopenia and febrile neutropenia was 33.3%, 19.4%, 25% and 5.56% respectively. Other grade 3-4 toxicities included nausea, vomiting, hyponatremia, hypokalemia and fatigue. Partial responses were only noted in patients with SCLC or extrathoracic small cell with PR noted in 7 pts, SD in 5 pts and PD in 7 pts. Of the pts achieving PRs on our study, 4 were platinum sensitive, 3 were refractory. The median duration of response was 4 months, median progression free survival was 3.06 months and the median overall survival was 9.1 months. Ten additional pts with relapsed SCLC were treated at the MTD on a compassionate cohort and 9 were evaluable for response. In this cohort SD was noted in 3 pts, PD in 6 pts with median OS of 6 months. Conclusions: AMR and cyclophosphamide can be safely combined with activity observed in a heavily pretreated SCLC population. Clinical trial information: NCT00890955.
The Therapeutic Efficacy of Botulinum Toxin in Treating Scleroderma-Associated Raynaud's Phenomenon: A Randomized, Double-Blind, Placebo-Controlled Clinical Trial
Authors: Ricardo J. Bello, Carisa M. Cooney, Eitan Melamed, Keith Follmar, Gayane Yenokyan, Gwendolyn Leatherman, Ami A. Shah, Fredrick M. Wigley, Laura K. Hummers, Scott D. Lifchez
Journal: Arthritis & Rheumatology (2017)

To assess the therapeutic efficacy of local injections of botulinum toxin type A (Btx-A) in improving blood flow to the hands of patients with Raynaud's phenomenon (RP) secondary to scleroderma.In this randomized, double-blind, placebo-controlled clinical trial, patients with scleroderma-associated RP received Btx-A (50 units in 2.5 ml sterile saline) in one randomly selected hand and sterile saline (2.5 ml) in the opposite hand. Follow-up at 1 and 4 months postinjection included laser Doppler imaging of hands, patient-reported outcomes, and physical examination. We compared outcomes using paired t-tests and population-average generalized models with generalized estimating equations.Of 40 patients enrolled, 25 had limited scleroderma and 15 had diffuse scleroderma. From baseline to 1-month follow-up, there was a greater reduction in average blood flow in Btx-A-treated hands compared to placebo-treated hands. The model estimated that this difference was statistically significant (average difference -30.08 flux units [95% confidence interval -56.19, -3.98], P for interaction = 0.024). This difference was mainly influenced by patients with longstanding RP and diffuse scleroderma. Change in blood flow at 4-month follow-up was not significantly different between groups. Clinical measures (QuickDASH, McCabe Cold Sensitivity Score, pain on a visual analog scale, and Raynaud's Condition Score) improved slightly for Btx-A-treated hands.Our laboratory-based laser Doppler imaging flow data do not support using Btx-A to treat RP in all scleroderma patients. The secondary clinical outcomes suggest some positive effect, but its clinical meaningfulness is questionable. The role of Btx-A in treating RP should be further studied with more homogeneous patient populations and in unique clinical situations such as acute digital ischemia.