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https://www.readbyqxmd.com/read/28535617/epidemiology-and-clinical-characteristics-of-sarcoidosis-an-update-from-a-population-based-cohort-study-from-olmsted-county-minnesota
#1
P Ungprasert, C S Crowson, E L Matteson
Information about the epidemiology, clinical manifestations and comorbidities of sarcoidosis among Caucasians is relatively scarce. This review focuses primarily on the data from a recently published Caucasianpredominant population-based cohort from Olmsted County, Minnesota. Overall, the incidence rate was 10.0 per 100,000 population, which suggested that sarcoidosis is less common in Caucasians than in Blacks, but is more common in Caucasians than in Asians. Intrathoracic involvement was seen in the vast majority of patients, but less than half have respiratory symptoms...
May 22, 2017: Reumatismo
https://www.readbyqxmd.com/read/28533834/-splenic-marginal-zone-lymphoma
#2
Sinaa Mohamed
Splenic marginal zone lymphoma (SMZL) is a rare B-cell lymphoma, well defined according to the 2016 WHO classification of tumors of the hematopoietic tissue, which can raise diagnostic problems. We report the case of a 72-year old patient, hospitalised for exploration of a massive splenomegaly confirmed by computed tomography (CT). Laboratory tests were normal. The patient underwent splenectomy. Microscopic examination of the surgical specimen showed widespread lymphomatous proliferation in small cells associated with CD20 marker...
2017: Pan African Medical Journal
https://www.readbyqxmd.com/read/28527249/-sociodemographic-and-clinical-characteristics-of-patients-infected-with-leptospira-spp-treated-at-four-hospitals-in-medell%C3%A3-n-colombia-2008-2013
#3
Lina María Echeverri-Toro, Sara Penagos, Laura Castañeda, Pablo Villa, Santiago Atehortúa, Faiver Ramírez, Carlos Restrepo, Sigifredo Ospina, Yuli Agudelo, Alicia Hidrón, Paola Agudelo, Eliana Valderrama, Carlos Andrés Agudelo
INTRODUCTION: Leptospirosis remains a significant health problem in tropical regions including Latin America, where its presentation is 100 times higher than that observed in other regions of the world. Mortality reaches 10% in severe cases. Its diagnosis is challenging because clinical manifestations during the initial phase are non-specific and because of limited availability of diagnostic tests. OBJECTIVE: To describe the demographic and clinical characteristics and the outcomes in hospitalized patients with leptospirosis...
January 24, 2017: Biomédica: Revista del Instituto Nacional de Salud
https://www.readbyqxmd.com/read/28522274/relationship-between-exposure-to-malaria-and-haemoglobin-level-of-children-2-9-years-old-in-low-malaria-transmission-settings
#4
Zewdie Birhanu, Yemane Ye-Ebiyo Yihdego, Daniel Emana, Damtew Feyissa, Silashi Kenate, Estifanos Kebede, Kefelegn Getahun, Delenasaw Yewhalaw
In the context of reduced transmission of malaria, it is essential to examine the association between exposure to malaria and haemoglobin level. This study measured the Haemoglobin level of children 2-9 years of age and examined its association with malariometric indices. A cross sectional study was conducted, during June 2016, on 763 children 2-9 years old, recruited from ten sites representing different malaria transmission settings in Ethiopia. Haemoglobin concentration was determined using HemoCue analyzer...
May 15, 2017: Acta Tropica
https://www.readbyqxmd.com/read/28518222/the-effect-of-initial-molecular-profile-on-response-to-recombinant-interferon-%C3%AE-rifn%C3%AE-treatment-in-early-myelofibrosis
#5
Richard T Silver, Ariella C Barel, Elena Lascu, Ellen K Ritchie, Gail J Roboz, Paul J Christos, Attilio Orazi, Duane C Hassane, Wayne Tam, Nicholas C P Cross
BACKGROUND: Although recombinant interferon-α (rIFNα) effectively treats patients with early myelofibrosis, the effect of driver and high molecular risk (HMR) mutations has not been considered. In this phase 2 study, for the first time, the authors correlate response to rIFNα treatment with driver and HMR mutations. METHODS: Patients were diagnosed using World Health Organization or International Working Group for Myeloproliferative Neoplasms Research and Treatment criteria...
May 18, 2017: Cancer
https://www.readbyqxmd.com/read/28515254/nccn-debuts-new-guidelines-for-myeloproliferative-neoplasms
#6
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/28512865/efficacy-and-safety-of-ruxolitinib-in-intermediate-1-ipss-risk-myelofibrosis-patients-results-from-an-independent-study
#7
Francesca Palandri, Mario Tiribelli, Giulia Benevolo, Alessia Tieghi, Francesco Cavazzini, Massimo Breccia, Micaela Bergamaschi, Nicola Sgherza, Nicola Polverelli, Monica Crugnola, Alessandro Isidori, Gianni Binotto, Florian H Heidel, Francesco Buccisano, Bruno Martino, Roberto Latagliata, Marco Spinsanti, Lydia Kallenberg, Giuseppe Alberto Palumbo, Elisabetta Abruzzese, Luigi Scaffidi, Antonio Cuneo, Michele Cavo, Nicola Vianelli, Massimiliano Bonifacio
Patients with myelofibrosis at intermediate-1 risk according to the International Prognostic Score System are projected to a relatively long survival; nonetheless, they may carry significant splenomegaly and/or systemic constitutional symptoms that hamper quality of life and require treatment. Since registrative COMFORT studies included only patients at intermediate-2/high International Prognostic Score System risk, safety and efficacy data in intermediate-1 patients are limited. We report on 70 intermediate-1 patients treated with ruxolitinib according to standard clinical practice that were evaluated for response using the 2013 IWG-MRT criteria...
May 17, 2017: Hematological Oncology
https://www.readbyqxmd.com/read/28510795/liver-stiffness-assessed-by-shear-wave-elastography-predicts-postoperative-liver-failure-in-patients-with-hepatocellular-carcinoma
#8
Yinghao Shen, Chenhao Zhou, Guodong Zhu, Guoming Shi, Xiaodong Zhu, Cheng Huang, Jian Zhou, Jia Fan, Hong Ding, Ning Ren, Hui-Chuan Sun
BACKGROUND: Cirrhosis increases a patient's risk of developing postoperative liver failure (PLF). Liver stiffness (LS), assessed by two-dimensional shear wave elastography (SWE), indicates liver fibrosis with high accuracy. Whether LS is superior to portal hypertension (PHT) in predicting PLF remains to be studied. METHODS: The study enrolled 280 patients who underwent hepatectomy for hepatocellular carcinoma from July 2015 to July 2016. All patients received preoperative assessments for LS, PHT, and serum markers of liver fibrosis in addition to other clinicopathological tests...
May 16, 2017: Journal of Gastrointestinal Surgery: Official Journal of the Society for Surgery of the Alimentary Tract
https://www.readbyqxmd.com/read/28508023/an-unusual-presentation-of-lcat-deficiency-as-nephrotic-syndrome-with-normal-serum-hdl-c-level
#9
Manish R Balwani, Vijaykumar A Ghodela, Vivek B Kute, Pankaj R Shah, Himanshu V Patel, Dinesh N Gera, Aruna Vanikar, Hargovind L Trivedi
Clinical and biochemical manifestations of lecithin-cholesterol acyltransferase (LCAT) deficiency include an abnormal lipid profile (characterized by hypercholesterolemia with markedly decreased high-density lipoprotein cholesterol [HDL-C] and hypertriglyceridemia), corneal opacities, hematologic abnormalities (normochromic anemia of varying intensity), splenomegaly, variable early coronary artery disease and nephropathy (initially proteinuria followed by progressive deterioration of renal function). We presented a patient with nephrotic syndrome, which renal biopsy revealed classic features of LCAT deficiency...
2017: Journal of Nephropharmacology
https://www.readbyqxmd.com/read/28504123/erythropoietin-and-small-molecule-agonists-of-the-tissue-protective-erythropoietin-receptor-increase-fxn-expression-in-neuronal-cells-in-vitro-and-in-fxn-deficient-kiko-mice-in-vivo
#10
James L Miller, Myriam Rai, Normand L Frigon, Massimo Pandolfo, Juha Punnonen, Jeffrey R Spencer
Friedreich's ataxia (FRDA) is a progressive neurodegenerative disease caused by reduced levels of the mitochondrial protein frataxin (FXN). Recombinant human erythropoietin (rhEPO) increased FXN protein in vitro and in early clinical studies, while no published reports evaluate rhEPO in animal models of FRDA. STS-E412 and STS-E424 are novel small molecule agonists of the tissue-protective, but not the erythropoietic EPO receptor. We find that rhEPO, STS-E412 and STS-E424 increase FXN expression in vitro and in vivo...
May 11, 2017: Neuropharmacology
https://www.readbyqxmd.com/read/28501742/differences-in-the-imaging-features-and-distribution-of-non-indolent-and-indolent-mastocytosis-a-single-institution-experience-of-29-patients
#11
Yan Epelboym, Abhishek R Keraliya, Sree Harsha Tirumani, Jason L Hornick, Nikhil H Ramaiya, Atul B Shinagare
PURPOSE: Compare imaging features of indolent and non-indolent mastocytosis. METHODS: For 29 patients, imaging features, imaging indications, and distribution of indolent and non-indolent mastocytosis subtypes were analyzed. RESULTS: 16/29 (55%) patients had three distinct patterns of osseous abnormality, not significantly differing between cohorts. Non-indolent disease was more likely to present with hepatomegaly (p=0.0004), splenomegaly (p=0...
May 6, 2017: Clinical Imaging
https://www.readbyqxmd.com/read/28501004/adipose-derived-mesenchymal-stem-cells-slow-disease-progression-of-acute-on-chronic-liver-failure
#12
Carlos Gilsanz, Maria-Angeles Aller, Sherezade Fuentes-Julian, Isabel Prieto, Alejandro Blázquez-Martinez, Salvador Argudo, Jorge Fernández-Delgado, Jose Beleña, Jaime Arias, María P De Miguel
A serious complication of chronic hepatic insufficiency is acute-on-chronic liver failure, a recognized syndrome characterized by acute decompensation of cirrhosis and organ/system failure. We investigated the use of adipose-derived mesenchymal stem cells (AD-MSCs) in an experimental model of acute-on-chronic liver failure, developed by microsurgical extrahepatic cholestasis in rats. Rats undergoing microsurgical extrahepatic cholestasis were treated by intraparenchymal liver injection of human or rat AD-MSCs, undifferentiated or previously differentiated in vitro toward the hepatocyte lineage...
May 10, 2017: Biomedicine & Pharmacotherapy, Biomédecine & Pharmacothérapie
https://www.readbyqxmd.com/read/28499938/allogeneic-stem-cell-transplantation-in-myelofibrosis
#13
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/28494931/hemophagocytic-lymphohistiocytosis-in-acute-african-swine-fever-clinic
#14
Z R Karalyan, Z R Ter-Pogossyan, N Yu Karalyan, Z B Semerjyan, M R Tatoyan, S A Karapetyan, E M Karalova
INTRODUCTION: Hemophagocytic lymphohistiocytosis (HLH) usually has been defined as the combination of a proliferation of cytologically benign, actively phagocytic macrophages in bone marrow, spleen, lymph nodes, etc. in association with fever, cytopenia, splenomegaly, and hypertriglyceridemia. HLH is often triggered by viral infection. The aim of this study was to ascertain the features of HLH involvement in African swine fever virus (ASFV) (genotype II) pathogenesis. METHODS: The serum levels of macrophage colony-stimulating factor (MCSF) and granulocyte-macrophage colony-stimulating factor (GMCSF), as well as the histological constitution (for hemophagocytic macrophages detection) of various organs of pigs infected with ASFV genotype II were investigated...
May 2017: Veterinary Immunology and Immunopathology
https://www.readbyqxmd.com/read/28493472/turf-wars-exploring-splenomegaly-in-sickle-cell-disease-in-malaria-endemic-regions
#15
REVIEW
Venée N Tubman, Julie Makani
Sickle cell disease (SCD) is a group of recessively inherited disorders of erythrocyte function that presents an ongoing threat to reducing childhood and adult morbidity and mortality around the world. While decades of research have led to improved survival for SCD patients in wealthy countries, survival remains dismal in low- and middle-income countries. Much of the early mortality associated with SCD is attributed to increased risk of infections due to early loss of splenic function. In the West, bacterial infections with encapsulated organisms are a primary concern...
May 11, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28491877/nodular-regenerative-hyperplasia-a-case-of-rare-prognosis
#16
Mindy Lee, Manhal Izzy, Ashwin Akki, Kathryn Tanaka, Harmit Kalia
Introduction: Nodular regenerative hyperplasia (NRH) is a known etiology of noncirrhotic portal hypertension. Cases of biopsy-proven NRH in human immunodeficiency virus (HIV)-positive patients have been described. While these patients often have normal synthetic liver function, several reports described disease progression to liver failure. Case: We here present a 26-year-old woman with history of congenital HIV on antiretroviral therapy complicated by Pneumocystis carinii pneumonia at age 14. CD4 counts have been >300 with undetectable viral load...
January 2017: Journal of Investigative Medicine High Impact Case Reports
https://www.readbyqxmd.com/read/28486007/concomitant-laparoscopic-perisplenic-artery-ligation-facilitates-surgery-for-hepatobiliary-disease-coexisting-with-portal-hypertension
#17
Youlong Wang, Fei Chen, Yajuan Chu, Xiaofang Tan, Jingwang Tan, Wenbin Ji
BACKGROUND: Although liver cirrhosis with portal hypertension (PH) contributes significantly to morbidity and mortality in abdominal surgery, many authors still consider this disease as an indication for surgery. In many reports, however, numerous treatment modalities focus on hypersplenism secondary to PH, irrespective of splenomegaly and PH. The proven benefits of laparoscopy seem especially applicable to patients with this complex disease. This study evaluates the safety and efficacy of laparoscopic perisplenic artery ligation (SAL) in patients with hepatobiliary disease and PH...
May 9, 2017: Journal of Laparoendoscopic & Advanced Surgical Techniques. Part A
https://www.readbyqxmd.com/read/28484156/bcr-abl1-positive-chronic-myeloid-leukemia-emerging-in-a-patient-with-secondary-myelofibrosis-harboring-the-jak2-v617f-mutation
#18
Ayae Amemiya, Yoshikazu Ito, Yasunori Ishibashi, Yuu Saito, Seiichiro Katagiri, Tamiko Suguro, Michiyo Asano, Seiichiro Yoshizawa, Daigo Akahane, Yuko Tanaka, Hiroaki Fujimoto, Seiichi Okabe, Moritaka Gotoh, Tetsuzo Tauchi, Kazuma Ohyashiki
A 53-year-old woman with a 27-year history of myeloproliferative neoplasms came to our hospital because of a marked white blood cell count increase and progressive anemia. Clinical examination demonstrated positivity for BCR-ABL1 and JAK2-V617F mutations. She was given a diagnosis of chronic myeloid leukemia. Using the international scale, a molecular response (MR) (4.5) was achieved after treatment with dasatinib, despite the persistence of marked splenomegaly. The pathological findings of myelofibrosis were demonstrated by bone marrow biopsy...
2017: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/28482820/the-changing-epidemiology-and-clinical-features-of-infective-endocarditis-a-retrospective-study-of-196-episodes-in-a-teaching-hospital-in-china
#19
Wan Zhu, Qian Zhang, Jingping Zhang
BACKGROUND: Infective endocarditis is an uncommon but life-threatening infectious disease. To our knowledge, current investigations of the characteristics of infective endocarditis in our region are scarce. In this study, we aimed to investigate the changes in the epidemiology and clinical features of infective endocarditis. METHODS: A retrospective analysis of clinical data was performed using 196 infective endocarditis cases diagnosed between June 2004 and December 2012 at The First Affiliated Hospital of China Medical University...
May 8, 2017: BMC Cardiovascular Disorders
https://www.readbyqxmd.com/read/28482758/lesions-caused-by-virulent-aeromonas-hydrophila-in-farmed-catfish-ictalurus-punctatus-and-i-punctatus-%C3%A3-i-furcatus-in-mississippi
#20
Wes A Baumgartner, Lorelei Ford, Larry Hanson
In 2009, a virulent strain of Aeromonas hydrophila caused severe disease and high mortalities (motile aeromonad septicemia variant [MASv]) in farmed channel catfish ( Ictalurus punctatus) and hybrid catfish ( I. punctatus × I. furcatus) in eastern Mississippi and Alabama. As is common in MAS, there is severe hemorrhagic dermatitis with ulceration, as well as abdominal hyperemia, petechiation, and mild ascites. Additional findings in MASv cases include panophthalmitis and orbital cellulitis, leading to ocular rupture, and brains are often hyperemic with mild random acute hemorrhage...
May 1, 2017: Journal of Veterinary Diagnostic Investigation
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