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https://www.readbyqxmd.com/read/28540277/noncirrhotic-portal-vein-thrombosis-presenting-with-hepatic-encephalopathy-due-to-polycythemia-vera-and-mri-findings
#1
Burcu Gökçe Çokal, Tahir Kurtuluş Yoldaş, Selda Keskin Güler, Murat Kekilli, Faruk Pirinçcioğlu, Hafize Nalan Güneş
No abstract text is available yet for this article.
April 2017: Iranian Journal of Public Health
https://www.readbyqxmd.com/read/28536669/disparate-presentations-of-localized-cystic-disease-of-kidney-a-review-with-an-objective-of-correct-approach-for-accurate-treatment-plan
#2
Sachin Khanduri, Mriganki Chaudhary, Tushar Sabharwal, Aakshit Goyal, Gaurav Katyal
BACKGROUND: Localized cystic disease of the kidney is a rare, non-familial condition. Its imaging and clinical features are unique and need to be differentiated from autosomal dominant polycystic kidney disease and focal cystic masses such as multicystic nephroma and cystic renal cell carcinoma. It is always restricted to one kidney and is characterized by multiple cysts of varying sizes separated by residual normal renal tissue. MATERIALS AND METHODS: This study reports 12 cases of localized cystic disease of the kidney based on imaging findings and clinical histories...
April 22, 2017: Curēus
https://www.readbyqxmd.com/read/28533538/proteomic-analysis-of-jak2v617f-induced-changes-identifies-potential-new-combinatorial-therapeutic-approaches
#3
S Pearson, A J K Williamson, R Blance, T C P Somervaille, S Taylor, N Azadbakht, A D Whetton, A Pierce
In excess of 90% of patients with polycythemia vera express a mutated form of JAK2, JAK2V617F. Such aberrant proteins offer great potential for the treatment of these diseases however inhibitors to JAK2 have had limited success in the clinic in terms of curing the disease. To understand the effects of this oncogene in hematopoietic cells with the aim of improving treatment strategies we undertook a systematic evaluation of the effects of JAK2V617F expression using proteomics. The effects of JAK2V617F on over 5000 proteins and 2000 nuclear phosphopeptides sites were relatively quantified using either SILAC or eight channel iTRAQ mass spectrometry...
May 23, 2017: Leukemia: Official Journal of the Leukemia Society of America, Leukemia Research Fund, U.K
https://www.readbyqxmd.com/read/28532193/glycated-albumin-level-during-late-pregnancy-as-a-predictive-factor-for-neonatal-outcomes-of-women-with-diabetes
#4
Daisuke Sugawara, Atsuko Kawano, Kohei Nagai, Hiroaki Sato, Ko Ichihashi
PURPOSE: To investigate the association between glycated albumin (GA) in diabetic mothers and complications in their children, and determine GA cut-off values for predicting complications in infants. MATERIALS AND METHODS: This hospital-based case-control study involved 71 Japanese diabetic mothers and their children. Mean GA values were compared between mothers of infants with and without complications, and relationship with number of complications was analyzed by Pearson's correlation...
May 22, 2017: Journal of Maternal-fetal & Neonatal Medicine
https://www.readbyqxmd.com/read/28526632/erythrocytosis-following-testosterone-therapy
#5
REVIEW
Samuel J Ohlander, Bibin Varghese, Alexander W Pastuszak
INTRODUCTION: A rapid increase in awareness of androgen deficiency has led to substantial increases in prescribing of testosterone therapy (TTh), with benefits of improvements in mood, libido, bone density, muscle mass, body composition, energy, and cognition. However, TTh can be limited by its side effects, particularly erythrocytosis. This review examines the literature on testosterone-induced erythrocytosis and polycythemia. AIM: To review the available literature on testosterone-induced erythrocytosis, discuss possible mechanisms for pathophysiology, determine the significance of formulation, and elucidate potential thromboembolic risk...
May 16, 2017: Sexual Medicine Reviews
https://www.readbyqxmd.com/read/28522758/regulation-of-erythropoiesis-after-normoxic-return-from-chronic-sustained-and-intermittent-hypoxia
#6
Jihyun Song, Krishna Sundar, Radhika Gangaraju, Josef T Prchal
Hypoxia increases erythropoiesis mediated by hypoxia-inducible transcription factors (HIF) which regulate erythropoietin (EPO) transcription. Neocytolysis is a physiological mechanism that corrects polycythemia from chronic sustained hypoxemia (CSH) by transient, preferential destruction of young RBCs after normoxia is restored. We showed that neocytolysis is caused by excessive mitochondrial-derived reactive oxygen species (ROS) in reticulocytes mediated by down-regulation of HIF-controlled BNIP3L regulated mitophagy and a decrease in RBC antioxidant catalase (CAT) in hypoxia-produced erythrocytes...
May 18, 2017: Journal of Applied Physiology
https://www.readbyqxmd.com/read/28515254/nccn-debuts-new-guidelines-for-myeloproliferative-neoplasms
#7
Ruben A Mesa
For the first time, NCCN has published guidelines specifically geared toward treating myeloproliferative neoplasms (MPNs). The first set of guidelines was developed for myelofibrosis (MF), and was presented at the NCCN 22nd Annual Conference. Future guidelines will be issued for polycythemia vera, essential thrombocytopenia, and atypical MPNs. Patients with MF can have an unpredictable course, one that is largely dependent on the presence of certain molecular alterations. Models are currently emerging that take into account molecular factors...
May 2017: Journal of the National Comprehensive Cancer Network: JNCCN
https://www.readbyqxmd.com/read/28500663/obesity-and-related-risk-of-myeloproliferative-neoplasms-among-israeli-adolescents
#8
Adi Leiba, Adrian Duek, Arnon Afek, Estela Derazne, Merav Leiba
OBJECTIVE: Obesity has been associated with various malignancies, but a clear association between overweight and myeloproliferative neoplasms (MPN) has not been established. METHODS: This study assessed the association between adolescent obesity and future risk for MPN. Data on 2,516,256 Israeli adolescents, who underwent a compulsory general health examination at ages 16 to 19, between 1967 and 2011, were linked to the National Cancer Registry in this nationwide, population-based cohort study...
May 12, 2017: Obesity
https://www.readbyqxmd.com/read/28500170/jak2-inhibitors-for-myeloproliferative-neoplasms-what-is-next
#9
Prithviraj Bose, Srdan Verstovsek
Since its approval in 2011, the Janus kinase (JAK) 1/2 inhibitor ruxolitinib has evolved to become the centerpiece of therapy for myelofibrosis (MF), and its use in patients with hydroxyurea resistant/intolerant polycythemia vera (PV) is steadily increasing. A number of other JAK2 inhibitors have entered clinical testing, but none has been approved yet, and many discontinued. Importantly, the activity of these agents is not restricted to patients with JAK2 V617F or exon 12 mutations. Although JAK2 inhibitors provide substantial clinical benefit, their disease-modifying activity is limited, and rational combinations with other targeted agents are needed, particularly in MF, where survival is short...
May 12, 2017: Blood
https://www.readbyqxmd.com/read/28499938/allogeneic-stem-cell-transplantation-in-myelofibrosis
#10
REVIEW
Tania Jain, Ruben A Mesa, Jeanne M Palmer
Myeloproliferative neoplasm is a category in the World Health Organization classification of myeloid tumors. BCR-ABL1-negative MPN is a subcategory that includes primary myelofibrosis (MF), post-essential thrombocythemia MF, and post-polycythemia vera MF. These disorders are characterized by stem cell-derived clonal myeloproliferation. Clinically, these diseases present with anemia and splenomegaly, as well as significant constitutional symptoms, such as severe fatigue, symptoms associated with an enlarged spleen and liver, pruritus, fevers, night sweats, and bone pain...
May 9, 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/28497563/frequency-of-polycythemia-in-individuals-with-normal-complete-blood-cell-counts-according-to-the-new-2016-who-classification-of-myeloid-neoplasms
#11
A F Sandes, M V Gonçalves, M de L Chauffaille
INTRODUCTION: Polycythemia vera (PV) is a disorder characterized by clonal proliferation of myeloid cells and increased red blood cell mass. Recently, the revised 2016 WHO classification of myeloid neoplasms decreased the threshold levels of hemoglobin and hematocrit for the diagnosis of PV. However, the new proposed cutoffs have remarkable overlap with the normal reference values reported and the clinical impact of these new cutoffs has not been widely assessed in the general population...
May 12, 2017: International Journal of Laboratory Hematology
https://www.readbyqxmd.com/read/28494714/is-the-hepatic-factor-a-mirna-that-maintains-the-integrity-of-pulmonary-microvasculature-by-inhibiting-the-vascular-endothelial-growth-factor
#12
Joseph John Vettukattil
The "hepatic factor," a molecule or group of molecules present in the hepatic venous blood, essential for the prevention of the development of pulmonary arteriovenous malformations (PAVMs) and right-to-left shunting has been a conceptual enigma in the understanding of many related conditions. Patients with various forms of liver diseases including acute hepatic failure, and others with normal hepatic function like hereditary hemorrhagic telangiectasia (HHT), inflammatory and parasitic disorders, cardiogenic hepatopulmonary syndrome (cHPS) and skin disorders like Dyskeratosis congenita are all known to cause PAVMs...
May 9, 2017: Current Cardiology Reviews
https://www.readbyqxmd.com/read/28482711/the-presence-of-monoclonal-gammopathy-in-ph-negative-myeloproliferative-neoplasms-is-associated-with-a-detrimental-effect-on-outcomes
#13
Lenaïg Le Clech, Mehdi Sakka, Ahmed Meskar, Helene Kerspern, Jean-Richard Eveillard, Christian Berthou, Caroline Buors, Eric Lippert, Gaelle Guillerm, Isabelle Quintin-Roué, Jean-Luc Carré, Jean-Christophe Ianotto
Many case reports have indicated the occurrence of monoclonal gammopathy of uncertain significance (MGUS) or multiple myeloma (MM) in patients with Ph-negative myeloproliferative neoplasms (MPN), but few cohorts of patients have been published. This study concerns 667 patients newly diagnosed with polycythemia vera (PV) or essential thrombocythemia (ET) who were tested for monoclonal (M) protein at diagnosis (13.9% of patients). The overall survival of patients with M protein was dramatically lower than that of patients without M protein (12...
May 9, 2017: Leukemia & Lymphoma
https://www.readbyqxmd.com/read/28479073/-macroscopic-description-of-placental-vascular-anastomoses-after-dye-injection-for-the-comprehension-of-monochorionic-pregnancy-complications
#14
A Atallah, P-A Bolze, A Buenerd, S Marino, J Massardier, P Gaucherand, M Massoud
OBJECTIVES: The aim of the study is to compare placental monochorionic angioarchitecture complicated with twin-oligohydramnios-polyhydramnios sequence (TOPS), twin anemia polycythemia sequence (TAPS), twin reversed arterial perfusion (TRAP) and selective intra uterine growth restriction (sIUGR) to normal uneventful monochorionic placenta. METHODS: Between December 2012 and December 2015, monochorionic placenta has been studied at the multiple pregnancy care center of the Femme-Mère-Enfant Hospital in Lyon...
May 2017: Gynecologie, Obstetrique, Fertilite & Senologie
https://www.readbyqxmd.com/read/28474128/-chronic-hypoxia-and-cardiovascular-risk-clinical-significance-of-different-forms-of-hypoxia
#15
REVIEW
U Koehler, O Hildebrandt, J Krönig, W Grimm, J Otto, W Hildebrandt, R Kinscherf
It is of fundamental importance to differentiate whether chronic hypoxia occurs intermittently or persistently. While chronic intermittent hypoxia (CIH) is found typically in patients with obstructive sleep apnea (OAS), chronic persistent hypoxia (CPH) is typically diagnosed in patients with chronic lung disease. Cardiovascular risk is markedly increased in patients with CIH compared to patients with CPH. The frequent change between oxygen desaturation and reoxygenation in patients with CIH is associated with increased hypoxic stress, increased systemic inflammation, and enhanced adrenergic activation followed by endothelial dysfunction and increased arteriosclerosis...
May 4, 2017: Herz
https://www.readbyqxmd.com/read/28473622/characteristics-and-clinical-significance-of-cytogenetic-abnormalities-in-polycythemia-vera
#16
Guilin Tang, Juliana E Hidalgo Lopez, Sa A Wang, Shimin Hu, Junsheng Ma, Sherry Pierce, Wenli Zuo, Adrian Alejandro Carballo-Zarate, C Cameron Yin, Zhenya Tang, Shaoying Li, L Jeffrey Medeiros, Srdan Verstovsek, Carlos E Bueso-Ramos
UP to 20% of patients with polycythemia vera have karyotypic abnormalities at time of initial diagnosis. However, the cytogenetic abnormalities in polycythemia vera have not been well-characterized and their prognostic impact is largely unknown. In this study, we aimed to address these questions using a large cohort of polycythemia vera patients with cytogenetic information available. The study included 422 patients, 271 in polycythaemic phase, 112 post-polycythaemic myelofibrosis, 11 accelerated phase, and 28 blast phase...
May 4, 2017: Haematologica
https://www.readbyqxmd.com/read/28472278/dihydrotestosterone-biochemistry-physiology-and-clinical-implications-of-elevated-blood-levels
#17
Ronald S Swerdloff, Robert E Dudley, Stephanie T Page, Christina Wang, Wael A Salameh
This review on dihydrotestosterone (DHT) biochemistry, physiology, and clinical implications of elevated levels in blood clarifies concepts that are important in clinical practice. Benefits associated with lowered serum DHT levels after 5α-reductase inhibitor (5AR-I) therapy in men have contributed to a misconception that circulating DHT levels are an important stimulus for androgenic action in target tissues (e.g., prostate). Yet evidence from clinical studies indicate that intracellular concentrations of androgens (particularly in androgen-sensitive tissues) are essentially independent of circulating levels...
May 2, 2017: Endocrine Reviews
https://www.readbyqxmd.com/read/28471901/right-hand-weakness-and-headache-during-ascent-to-mount-everest-a-case-of-cerebral-venous-infarction
#18
Jeong Hee Kim, Soo Jeong Kim, Hahn Young Kim
INTRODUCTION: The increasing popularity of trekking in alpine regions has drawn attention to high altitude-associated health concerns. CASE REPORT: Here, we report a case of cerebral venous infarction as a consequence of a hypercoagulable state induced by secondary polycythemia as an adaptation to high altitude. CONCLUSIONS: When patients present focal neurological symptoms such as hemiparesis in addition to symptoms of acute mountain sickness or high-altitude cerebral edema such as headache, nausea, vomiting, and dizziness, cerebral venous infarction should be considered...
May 2017: Neurologist
https://www.readbyqxmd.com/read/28460542/hematological-disorders-at-birth-in-complicated-monochorionic-twins
#19
Lianne Verbeek, Femke Slaghekke, Marieke Sueters, Johanna M Middeldorp, Frans J Klumper, Monique C Haak, Dick Oepkes, Enrico Lopriore
Monochorionic twins are at risk of severe complications including twin-twin transfusion syndrome (TTTS), twin anemia-polycythemia sequence (TAPS) and acute peripartum TTTS. The pathophysiology is based on inter-twin blood transfusion through placental vascular anastomoses. Areas covered: This review focuses on the incidence, management and outcome of neonatal hematological complications at birth in TTTS, TAPS and acute peripartum TTTS. Expert commentary: Hematological disorders are often present at birth in monochorionic twins and include acute or chronic anemia, polycythemia and thrombocytopenia...
May 10, 2017: Expert Review of Hematology
https://www.readbyqxmd.com/read/28456851/ruxolitinib-reduces-jak2-p-v617f-allele-burden-in-patients-with-polycythemia-vera-enrolled-in-the-response-study
#20
Alessandro Maria Vannucchi, Srdan Verstovsek, Paola Guglielmelli, Martin Griesshammer, Timothy C Burn, Ahmad Naim, Dilan Paranagama, Mahtab Marker, Brian Gadbaw, Jean-Jacques Kiladjian
In patients with polycythemia vera (PV), an elevated JAK2 p.V617F allele burden is associated with indicators of more severe disease (e.g., leukocytosis, splenomegaly, and increased thrombosis risk); however, correlations between allele burden reductions and clinical benefit in patients with PV have not been extensively evaluated in a randomized trial. This exploratory analysis from the multicenter, open-label, phase 3 Randomized Study of Efficacy and Safety in Polycythemia Vera With JAK Inhibitor INCB018424 Versus Best Supportive Care trial evaluated the long-term effect of ruxolitinib treatment on JAK2 p...
April 30, 2017: Annals of Hematology
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