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Polycythemia

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https://www.readbyqxmd.com/read/29332220/perinatal-outcomes-of-unplanned-out-of-hospital-deliveries-a-case-control-study
#1
Yael Pasternak, Eliana Muskin Wintner, Gil Shechter-Maor, Yehonatan Pasternak, Netanella Miller, Tal Biron-Shental
PURPOSE: To compare the pregnancy and perinatal outcomes of unplanned home or car births vs. in-hospital deliveries. METHODS: A retrospective, case-control study of women who underwent unplanned out-of-hospital deliveries vs. in-hospital deliveries from 2004 through 2014. Matching was based on gestational age and parity in a ratio of 2:1. RESULTS: There were no significant differences between the groups regarding demographic criteria, prenatal care and delivery complications...
January 13, 2018: Archives of Gynecology and Obstetrics
https://www.readbyqxmd.com/read/29330800/evaluation-of-erythrocyte-changes-after-normoxic-return-from-hypoxia
#2
Jihyun Song, Josef T Prchal
Hypoxia increases erythropoiesis by hypoxia-inducible factors (HIF), principally by HIF-2, which upregulates erythropoietin transcription. This results in an increase of red blood cell (RBC) production and delivery of more oxygen to tissues. Upon rapid return to normoxia, hypoxia-induced polycythemia is overcorrected by neocytolysis, a transient destruction of preferentially young RBCs bearing low catalase (downregulated by hypoxia-stimulated microRNA(miR)-21) caused by reactive oxygen species (ROS) from expanded mitochondria...
2018: Methods in Molecular Biology
https://www.readbyqxmd.com/read/29323541/development-of-a-targeted-next-generation-sequencing-assay-to-detect-diagnostically-relevant-mutations-of-jak2-calr-and-mpl-in-myeloproliferative-neoplasms
#3
Thomas Frawley, Cathal P O'Brien, Eibhlin Conneally, Elisabeth Vandenberghe, Melanie Percy, Stephen E Langabeer, Karl Haslam
BACKGROUND: The classical Philadelphia chromosome-negative myeloproliferative neoplasms (MPNs), consisting of polycythemia vera, essential thrombocythemia, and primary myelofibrosis, are a heterogeneous group of neoplasms that harbor driver mutations in the JAK2, CALR, and MPL genes. The detection of these mutations has been incorporated into the recent World Health Organization (WHO) diagnostic criteria for MPN. Given a pressing clinical need to screen for these mutations in a routine diagnostic setting, a targeted next-generation sequencing (NGS) assay for the detection of MPN-associated mutations located in JAK2 exon 14, JAK2 exon 12, CALR exon 9, and MPL exon 10 was developed to provide a single platform alternative to reflexive, stepwise diagnostic algorithms...
January 11, 2018: Genetic Testing and Molecular Biomarkers
https://www.readbyqxmd.com/read/29321547/polycythemia-vera-treatment-algorithm-2018
#4
REVIEW
Ayalew Tefferi, Alessandro M Vannucchi, Tiziano Barbui
Recently reported mature survival data have confirmed the favorable prognosis in polycythemia vera (PV), with an estimated median survival of 24 years, in patients younger than age 60 years old. Currently available drugs for PV have not been shown to prolong survival or alter the natural history of the disease and are instead indicated primarily for prevention of thrombosis. Unfortunately, study endpoints that are being utilized in currently ongoing clinical trials in PV do not necessarily target clinically or biologically relevant outcomes, such as thrombosis, survival, or morphologic remission, and are instead focused on components of disease palliation...
January 10, 2018: Blood Cancer Journal
https://www.readbyqxmd.com/read/29321520/essential-thrombocythemia-treatment-algorithm-2018
#5
REVIEW
Ayalew Tefferi, Alessandro M Vannucchi, Tiziano Barbui
Current drug therapy for myeloproliferative neoplasms, including essential thrombocythemia (ET) and polycythemia vera (PV), is neither curative nor has it been shown to prolong survival. Fortunately, prognosis in ET and PV is relatively good, with median survivals in younger patients estimated at 33 and 24 years, respectively. Therefore, when it comes to treatment in ET or PV, less is more and one should avoid exposing patients to new drugs that have not been shown to be disease-modifying, and whose long-term consequences are suspect (e...
January 10, 2018: Blood Cancer Journal
https://www.readbyqxmd.com/read/29313725/prognostic-risk-model-for-patients-with-high-risk-polycythemia-vera-and-essential-thrombocythemia
#6
Abhishek A Mangaonkar, Katherine P Hoversten, Naseema Gangat
Polycythemia Vera (PV) and essential thrombocythemia (ET) are the most frequent Philadelphia chromosome-negative myeloproliferative neoplasms, the other entity being myelofibrosis. Management of patients with PV and ET is fraught with difficulties as they have an inherent tendency to cause thrombotic and hemorrhagic events. There are no curative treatment options therefore it is important that a risk-adapted treatment approach is applied. Areas covered: This review discusses existing literature about prognosis in PV and ET, and addresses critical aspects related to defining 'high-risk' disease...
January 9, 2018: Expert Review of Hematology
https://www.readbyqxmd.com/read/29311459/-low-continuity-rate-of-sucroferric-oxyhydroxide-among-japanese-hemodialysis-patients-with-high-phosphate-binder-pill-burden
#7
Satoru Mitsuboshi, Hitoshi Yamada, Kazuhiko Nagai, Hideo Okajima
 This prospective observational study was conducted to evaluate the continuity, efficacy, and tolerability of sucroferric oxyhydroxide (SO) among hemodialysis (HD) patients who switched to SO from sevelamer hydrochloride (SH) or bixalomer (BX). Participants were 9 HD patients in Kaetsu Hospital who had been receiving more than 9 tablets/d of SH or BX and were switched to SO 750 mg/d. All the participants were men. Over a 6-month observational period, 6 of the 9 patients (67%) discontinued SO because of adverse events, including diarrhea, atheroma, and polycythemia...
2018: Yakugaku Zasshi: Journal of the Pharmaceutical Society of Japan
https://www.readbyqxmd.com/read/29290171/health-care-costs-and-thromboembolic-events-in-hydroxyurea-treated-patients-with-polycythemia-vera
#8
Shreekant V Parasuraman, Nianwen Shi, Dilan C Paranagama, Machaon Bonafede
BACKGROUND: Patients with polycythemia vera (PV) are at increased risk of thromboembolic events (TEs), which are key contributors to reduced overall survival compared with the age- and sex-matched general population. In addition to aspirin and phlebotomy to maintain hematocrit level < 45%, many patients receive cytoreduction with hydroxyurea (HU), which is associated with improved survival and may reduce the risk of cardiovascular events and TEs. However, 1 in 4 patients become resistant to or intolerant of HU...
January 2018: Journal of Managed Care & Specialty Pharmacy
https://www.readbyqxmd.com/read/29286929/correlation-of-hematocrit-and-apgar-scores-in-newborns-of-women-with-hypertensive-disorders-in-pregnancy
#9
Helen Chioma Okoye, Benedict Nwogoh, Odutola Israel Odetunde
OBJECTIVES: To compare the incidence of polycythemia in newborns of women with hypertensive disorders in pregnancy (HDP) with those of normotensive mothers, to determine the incidence of perinatal stress using Apgar scores and to correlate hematocrit with Apgar scores in these newborn. STUDY DESIGN: This was a hospital-based comparative study conducted in the University of Port Harcourt Teaching Hospital, Nigeria. MAIN OUTCOME MEASURES: Apgar scores of 200 newborns- 100 from mothers with HDP (case group) and 100 from normotensive mothers (control group)- were taken at 1st and 5th minute of birth and cord blood samples collected to determine hematocrit...
2017: Journal of Neonatal-perinatal Medicine
https://www.readbyqxmd.com/read/29285580/bone-marrow-findings-in-blast-phase-of-polycythemia-vera
#10
Juliana E Hidalgo López, Adrian Carballo-Zarate, Srdan Verstovsek, Sa A Wang, Shimin Hu, Shaoying Li, Jie Xu, Wenli Zuo, Zhenya Tang, C Cameron Yin, L Jeffrey Medeiros, Carlos E Bueso-Ramos, Guilin Tang
Approximately 10% of patients with polycythemia vera (PV) transform to acute leukemia (blast phase) at 10 years after initial diagnosis of PV. The bone marrow pathologic, cytogenetic, and molecular features of blast phase have not been well characterized. In this study, we reviewed 422 PV patients over a period of 11 years and identified 58 patients who developed acute myeloid leukemia (blast phase) during the course of disease. We found that blast phase of PV was characterized by overt myelodysplasia (n = 51, 88%); moderate to severe myelofibrosis (33 of 45, 73%); an abnormal karyotype (n = 51, 88%) that was often complex karyotype (n = 42, 72%); and gene mutations involving TP53 (55%), TET2 (27%), and DNMT3A (25%)...
December 28, 2017: Annals of Hematology
https://www.readbyqxmd.com/read/29284718/cohort-profile-study-of-transition-outcomes-and-gender-strong-to-assess-health-status-of-transgender-people
#11
Virginia P Quinn, Rebecca Nash, Enid Hunkeler, Richard Contreras, Lee Cromwell, Tracy A Becerra-Culqui, Darios Getahun, Shawn Giammattei, Timothy L Lash, Andrea Millman, Brandi Robinson, Douglas Roblin, Michael J Silverberg, Jennifer Slovis, Vin Tangpricha, Dennis Tolsma, Cadence Valentine, Kevin Ward, Savannah Winter, Michael Goodman
PURPOSE: The Study of Transition, Outcomes and Gender (STRONG) was initiated to assess the health status of transgender people in general and following gender-affirming treatments at Kaiser Permanente health plans in Georgia, Northern California and Southern California. The objectives of this communication are to describe methods of cohort ascertainment and data collection and to characterise the study population. PARTICIPANTS: A stepwise methodology involving computerised searches of electronic medical records and free-text validation of eligibility and gender identity was used to identify a cohort of 6456 members with first evidence of transgender status (index date) between 2006 and 2014...
December 27, 2017: BMJ Open
https://www.readbyqxmd.com/read/29282357/risk-factors-for-arterial-versus-venous-thrombosis-in-polycythemia-vera-a-single-center-experience-in-587-patients
#12
S Cerquozzi, D Barraco, T Lasho, C Finke, C A Hanson, R P Ketterling, A Pardanani, N Gangat, A Tefferi
In a recent International Working Group on Myeloproliferative Neoplasms Research and Treatment (IWG-MRT) study, prior arterial events and hypertension were predictors of subsequent arterial thrombosis whereas prior venous events and age ≥65 years predicted venous thrombosis in polycythemia vera (PV). In the current study, we sought to validate the above findings and identify additional predictors of arterial versus venous thrombosis. At a median follow up of 109 months, thrombosis after diagnosis occurred in 128 (22%) patients; 82 (14%) arterial and 57 (10%) venous events...
December 27, 2017: Blood Cancer Journal
https://www.readbyqxmd.com/read/29279869/bilateral-renal-anastomosing-hemangiomas-a-tale-of-two-kidneys
#13
Hamid Abboudi, Benjamin Tschobotko, Christopher Carr, Ranan DasGupta
Background: Renal anastomosing hemangioma (RAH) is an extremely rare benign vascular tumor first described in 2009. Making this diagnosis is fraught with challenges. Radiologically they share features consistent with renal cell carcinomas (RCCs). Their vascular nature poses risks if considering preoperative biopsy and histologically they share characteristics akin to angiosarcomas. The few reports published in the literature suggest presentation with hematuria, flank pain, and polycythemia although the majority are diagnosed at postnephrectomy histologic examination...
2017: Journal of Endourology Case Reports
https://www.readbyqxmd.com/read/29277359/soho-state-of-the-art-update-and-next-questions-mpn
#14
REVIEW
Prithviraj Bose, Jason Gotlib, Claire N Harrison, Srdan Verstovsek
The discovery of the activating Janus kinase (JAK)2V617F mutation in 2005 in most patients with the classic Philadelphia chromosome-negative myeloproliferative neoplasms (MPN) spurred intense interest in research into these disorders, culminating in the identification of activating mutations in MPL in 2006 and indels in the gene encoding calreticulin (CALR) in 2013, thus providing additional mechanistic explanations for the universal activation of JAK-signal transducer and activator of transcription (JAK-STAT) observed in these conditions, and the success of the JAK1/2 inhibitor ruxolitinib, which first received regulatory approval in 2011...
January 2018: Clinical Lymphoma, Myeloma & Leukemia
https://www.readbyqxmd.com/read/29249336/pulsatile-flow-of-non-newtonian-blood-fluid-inside-stenosed-arteries-investigating-the-effects-of-viscoelastic-and-elastic-walls-arteriosclerosis-and-polycythemia-diseases
#15
A Abbas Nejad, Z Talebi, D Cheraghali, A Shahbani-Zahiri, M Norouzi
BACKGROUND AND OBJECTIVE: In this study, the interaction of pulsatile blood flow with the viscoelastic walls of the axisymmetric artery is numerically investigated for different severities of stenosis. The geometry of artery is modeled by an axisymmetric cylindrical tube with a symmetric stenosis in a two-dimensional case. The effects of stenosis severity on the axial velocity profile, pressure distribution, streamlines, wall shear stress, and wall radial displacement for the viscoelastic artery are also compared to the elastics artery...
February 2018: Computer Methods and Programs in Biomedicine
https://www.readbyqxmd.com/read/29226426/significance-of-thrombocytopenia-in-patients-with-primary-and-post-essential-thrombocythemia-polycythemia-vera-myelofibrosis
#16
Lucia Masarova, Ahmad Alhuraiji, Prithviraj Bose, Naval Daver, Naveen Pemmaraju, Jorge Cortes, Sherry Pierce, Hagop Kantarjian, Srdan Verstovsek
Severe thrombocytopenia (platelets <50 x109 /L) is associated with very poor outcome of patients with myelofibrosis (MF). Since patients with primary myelofibrosis (PMF) differ from patients with post-essential thrombocythemia (PET-MF) and post-polycythemia vera myelofibrosis (PPV-MF), we aimed to evaluate the significance of low platelets among these patients. We present clinical characteristics and outcome of patients with either PMF, PPV-MF, or PET-MF, and thrombocytopenia who presented to our institution between 1984 and 2015...
December 11, 2017: European Journal of Haematology
https://www.readbyqxmd.com/read/29223988/prevalence-of-physical-problems-detected-by-the-distress-thermometer-and-problem-list-in-patients-with-myeloproliferative-disorders
#17
Daniel C McFarland, Kelly M Shaffer, Heather Polizzi, John Mascarenhas, Marina Kremyanskaya, Jimmie Holland, Ronald Hoffman
Background: Patients with myeloproliferative neoplasms (MPNs) can have a severe physical symptom burden over an extended disease trajectory that contributes to decreased quality of life. Few studies, however, have characterized which patients most frequently consider physical symptoms a problem. This study describes the physical symptoms of patients with MPNs and the relationship of these symptoms to patient characteristics. Methods: Patients with MPNs (N=117) completed questionnaires in a dedicated academic medical center MPN clinic...
December 2017: Journal of the National Comprehensive Cancer Network: JNCCN
https://www.readbyqxmd.com/read/29222296/what-are-the-current-treatment-approaches-for-patients-with-polycythemia-vera-and-essential-thrombocythemia
#18
REVIEW
Alessandro M Vannucchi, Paola Guglielmelli
Polycythemia vera (PV) and essential thrombocythemia (ET) are chronic myeloproliferative neoplasms that are characterized by thrombohemorrhagic complications, symptom burden, and impaired survival mainly due to thrombosis, progression to myelofibrosis, and transformation to acute leukemia. In this manuscript, we will review the most recent changes in diagnostic criteria, the improvements in risk stratification, and the "state of the art" in the daily management of these disorders. The role of conventional therapies and novel agents, interferon α and the JAK2 inhibitor ruxolitinib, is critically discussed based on the results of a few basic randomized clinical studies...
December 8, 2017: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/29217833/calr-mutational-status-identifies-different-disease-subtypes-of-essential-thrombocythemia-showing-distinct-expression-profiles
#19
Roberta Zini, Paola Guglielmelli, Daniela Pietra, Elisa Rumi, Chiara Rossi, Sebastiano Rontauroli, Elena Genovese, Tiziana Fanelli, Laura Calabresi, Elisa Bianchi, Simona Salati, Mario Cazzola, Enrico Tagliafico, Alessandro M Vannucchi, Rossella Manfredini
Polycythemia vera (PV) and essential thrombocythemia (ET) are Philadelphia-negative myeloproliferative neoplasms (MPNs) characterized by erythrocytosis and thrombocytosis, respectively. Approximately 95% of PV and 50-70% of ET patients harbor the V617F mutation in the exon 14 of JAK2 gene, while about 20-30% of ET patients carry CALRins5 or CALRdel52 mutations. These ET CALR-mutated subjects show higher platelet count and lower thrombotic risk compared to JAK2-mutated patients. Here, we showed that CALR-mutated and JAK2V617F-positive CD34+ cells display different gene and miRNA expression profiles...
December 8, 2017: Blood Cancer Journal
https://www.readbyqxmd.com/read/29208240/redness-discordance-in-monochorionic-twin-placentas-correlation-with-clinical-and-placental-findings
#20
Monique E De Paepe, Füsun Gündoğan, Quanfu Mao, Sharon Chu, Svetlana Shapiro
INTRODUCTION/OBJECTIVES: Recent studies suggest redness (color) discordance of the placental basal plate may be a marker for twin anemia-polycythemia sequence (TAPS), a recently described complication of diamniotic-monochorionic twinning characterized by marked intertwin hemoglobin (Hb) discordance in the absence of oligohydramnios-polyhydramnios. In this study, we determined the clinicoplacental and choriovascular correlates of basal plate color discordance in monochorionic twin placentas, and assessed its value as postnatal indicator of TAPS...
December 2017: Placenta
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