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B K Singleton, M Ahmed, C A Green, H Heimpel, M J Woźniak, L Ranjha, F Seeney, A Bomford, P Mehta, A Guest, R Mushens, M-J King
BACKGROUND: Bone marrow examination has been the confirmatory test for congenital dyserythropoietic anemia type II (CDAII). Occasional spherocytes on peripheral blood smear can confound the diagnosis. Since a screening test is still unavailable, we explored the feasibility of using flow cytometry as a preliminary screening method. METHODS: Thirteen monoclonal antibodies with specificities for eight erythrocyte membrane proteins were used in FACS analysis to probe the cellular features of red cells from CDAII, normal adults, hereditary spherocytosis (HS), and cord red cells...
October 26, 2016: Cytometry. Part B, Clinical Cytometry
Adriano M Pellicelli, Valeria Pace Palitti, Pascal Vignally, Francesca Ceccherini-Silberstain, Massimo Siciliano, Valerio Giannelli, Alessandra Moretti, Pierluigi Tarquini, Gaetano Scifo, Vincenzo Messina, Antonio Ascione, Antonio Izzi, Massimo Marignani, Cecilia D'Ambrosio, Lucia Fondacaro, Giuseppe M Ettorre, Pasquale Ialongo, Rodolfo Sacco, Carlo Federico Perno, Giorgio Barbarini
BACKGROUND AND AIMS: The proportion of HCV infected patients over age 65 years in Western countries is increasing. This growth and the advent of new antiviral therapy bring into the question the real world efficacy and safety of the combination of Sofosbuvir (SOF) and Simeprevir (SMV) plus a flat dose of 800 mg/d ribavirin (RBV) in elderly patients with liver cirrhosis compared to younger patients. METHODS: Retrospective observational multicenter real life investigation study of SOF/SMV/RBV for a duration of 12 weeks in HCV genotype 1-infected patients with liver cirrhosis...
October 26, 2016: Liver International: Official Journal of the International Association for the Study of the Liver
Katherine D Westmoreland, Christopher C Stanley, Nathan D Montgomery, Bongani Kaimila, Edwards Kasonkanji, Nader Kim El-Mallawany, Peter Wasswa, Idah Mtete, Mercy Butia, Salama Itimu, Mary Chasela, Mary Mtunda, Maria Chikasema, Victor Makwakwa, Coxcilly Kampani, Bal M Dhungel, Marcia K Sanders, Robert Krysiak, Tamiwe Tomoka, N George Liomba, Dirk P Dittmer, Yuri Fedoriw, Satish Gopal
BACKGROUND: Contemporary descriptions of classical Hodgkin lymphoma (cHL) are lacking from sub-Saharan Africa where human immunodeficiency virus (HIV) and Epstein-Barr virus (EBV) are prevalent. METHODS: We describe a prospective cHL cohort in Malawi enrolled from 2013 to 2015. Patients received standardized treatment and evaluation, including HIV status and EBV testing of tumors and plasma. RESULTS: Among 31 patients with confirmed cHL, the median age was 19 years (range, 2-51 years) and 22 (71%) were male...
October 26, 2016: Pediatric Blood & Cancer
Alexander G Raufi, Shruti Scott, Omar Darwish, Kevin Harley, Kanwarpal Kahlon, Sheetal Desai, Yuxin Lu, Minh-Ha Tran
Among the spectrum of disease manifestations associated with systemic lupus erythematosus, lupus nephritis is particularly concerning due to the potential for renal failure. This autoimmune attack may not, however, be limited to the kidney and is increasingly being recognized as a trigger for atypical Hemolytic Uremic Syndrome (aHUS). Atypical HUS falls under the spectrum of the thrombotic microangiopathies (TMAs) - a group of disorders characterized by microangiopathic hemolytic anemia, thrombocytopenia, and end organ damage...
September 28, 2016: Hematology Reports
Pieter Martens, Frederik Verbrugge, Petra Nijst, Matthias Dupont, W H Wilson Tang, Wilfried Mullens
Iron deficiency is prevalent in heart failure with reduced ejection fraction and relates to symptomatic status, readmission, and all-cause mortality. The relation between iron status and response to cardiac resynchronization therapy (CRT) remains insufficiently elucidated. This study assesses the impact of iron deficiency on clinical response and reverse cardiac remodeling and outcome after CRT. Baseline characteristics, change in New York Heart Association functional class, reverse cardiac remodeling on echocardiography, and clinical outcome (i...
September 29, 2016: American Journal of Cardiology
Manish K Yadav, Nandini M Manoli, SubbaRao V Madhunapantula
BACKGROUND: Megaloblastic anemia (MBA), also known as macrocytic anemia, is a type of anemia characterized by decreased number of RBCs as well as the presence of unusually large, abnormal and poorly developed erythrocytes (megaloblasts), which fail to enter blood circulation due to their larger size. Lack of vitamin-B12 (VB12) and / or folate (Vitamin-B9, VB9) with elevated homocysteine is the key factor responsible for megaloblastic anemia. Prior studies have demonstrated the induction of apoptosis in these abnormal under-developed erythrocytes...
2016: PloS One
Rong Fu, Yinping Meng, Yihao Wang, Hui Liu, Yi Liu, Lijuan Li, Shaoxue Ding, Guojin Wang, Jia Song, Zonghong Shao
INTRODUCTION: Thrombosis is a dangerous complication of paroxysmal nocturnal hemoglobinuria (PNH) and has a high mortality rate. However, the mechanism underlying the development of thrombosis in PNH remains unclear. To explore this, platelet function and serum complement activity were investigated in 14 patients with classical PNH, 11 with PNH aplastic anemia (AA) and 30 healthy controls. MATERIAL AND METHODS: Serum concentrations of the terminal complement complex (sC5b-9) were determined by enzyme-linked immunofluorescence assay (ELISA), and the levels of C5b-9, CD61 and CD62p on platelet membranes were determined by flow cytometry...
July 22, 2016: Thrombosis Research
Lawrence Tim Goodnough, Josep Comin-Colet, Santiago Leal-Noval, Sherri Ozawa, Jacqueline Takere, David Henry, Mazyar Javidroozi, Benjamin Hohmuth, Elvira Bisbe, Irwin Gross, Aryeh Shander
Anemia is an independent risk factor for adverse patient outcomes. There are no guidelines for management of anemia in patients withcongestive heart failure (CHF), despite itshigh incidence. Four objectives were defined by the International Anemia Management and Clinical Outcomes Expert Panel (AMCO), a multinational group of interdisciplinary experts identified by the Society for the Advancement of Blood Management (SABM) to: determine the prevalence of anemia in outpatients; to determine the prevalence of hospital-acquired anemia; to assess the impact of anemia management on clinical outcomes such as quality of life and functional status; and to provide recommendations for primary care physicians and specialists for the diagnosis, evaluation, and management of anemia in patients with CHF...
October 25, 2016: American Journal of Hematology
Margit Juhász, Megan Rogge, Mary Chen, Annette Czernik, Soo Jung Kim, Lauren Geller
This is a case report of a 16-year-old girl recently diagnosed with systemic lupus erythematosus (SLE) who presented with multiple blisters on the face, hands, arms, legs, trunk, and vaginal and oral mucosa. Skin biopsy was consistent with bullous SLE (BSLE). Dapsone is often the first-line treatment option for BSLE, but the patient's history of anemia and leukopenia and long-term immunosuppression requirement for her systemic symptoms raised concerns about dapsone and bone marrow toxicity, especially hemolytic anemia and agranulocytosis...
October 25, 2016: Pediatric Dermatology
Farahnaz Golriz, Lane F Donnelly, Sridevi Devaraj, Raj Krishnamurthy
BACKGROUND: Until recently scurvy has been viewed in developed countries as a disease of the past. More recently there have been reports of case series of children with scurvy who have had a delayed diagnosis after an extensive diagnostic workup that included imaging. Most of these children have had underlying neurologic conditions such as autism. OBJECTIVE: To review the medical records of children diagnosed with vitamin C (ascorbic acid) deficiency based on serum ascorbic acid levels at a large pediatric health care system, to determine imaging findings and utility of imaging in management, and to identify at-risk pediatric populations...
October 24, 2016: Pediatric Radiology
Mohammad Abu-Hishmeh, Alamgir Sattar, Fnu Zarlasht, Mohamed Ramadan, Aisha Abdel-Rahman, Shante Hinson, Caroline Hwang
BACKGROUND Thrombotic thrombocytopenic purpura (TTP) is one of the thrombotic microangiopathic (TMA) syndromes, caused by severely reduced activity of the vWF-cleaving protease ADAMTS13. Systemic lupus erythematosus (SLE), on the other hand, is an autoimmune disease that affects various organs in the body, including the hematopoietic system. SLE can present with TMA, and differentiating between SLE and TTP in those cases can be very challenging, particularly in patients with no prior history of SLE. Furthermore, an association between these 2 diseases has been described in the literature, with most of the TTP cases occurring after the diagnosis of SLE...
October 25, 2016: American Journal of Case Reports
Xiaofei Gao, Hsiang-Ying Lee, Edroaldo Lummertz da Rocha, Cheng Zhang, Yi-Fen Lu, Dandan Li, Yuxiong Feng, Jideofor Ezike, Russell R Elmes, M Inmaculada Barrasa, Patrick Cahan, Hu Li, George Q Daley, Harvey F Lodish
Burst-forming unit erythroid progenitors (BFU-Es) are so-named based on their ability to generate in methylcellulose culture large colonies of erythroid cells that consist of "bursts" of smaller erythroid colonies derived from the later CFU-E Epo- dependent progenitors. "Early" BFU-E cells forming large BFU-E colonies presumably have higher capacities for self-renewal than do "late" BFU-E forming small colonies but the mechanism underlying this heterogeneity remains unknown. We show that the type III transforming growth factor (TGF)-β receptor (TβRIII) is a marker that distinguishes "early" and "late" BFU-Es...
October 24, 2016: Blood
Guillermo J Pons-Estel, Laura Andreoli, Francesco Scanzi, Ricard Cervera, Angela Tincani
The antiphospholipid syndrome (APS) is an autoimmune disease characterized by the occurrence of venous and/or arterial thrombosis and pregnancy morbidity in the presence of pathogenic autoantibodies known as antiphospholipid antibodies (aPL). APS may be associated with other diseases, mainly systemic lupus erythematosus (SLE). The presence or absence of SLE might modify the clinical or serological expression of APS. Apart from the classical manifestations, APS patients with associated SLE more frequently display a clinical profile with arthralgias, arthritis, autoimmune hemolytic anemia, livedo reticularis, epilepsy, glomerular thrombosis, and myocardial infarction...
October 21, 2016: Journal of Autoimmunity
Jacquelyn M Powers, Mark Shamoun, Timothy L McCavit, Leah Adix, George R Buchanan
OBJECTIVE: To assess the benefits and risks of intravenous (IV) ferric carboxymaltose (FCM) in children with iron deficiency anemia (IDA). STUDY DESIGN: In a retrospective cohort study of patients seen at our center, we identified all FCM infusions in children with IDA over a 12-month period through a query of pharmacy records. Clinical data, including hematologic response and adverse effects, were extracted from the electronic medical record. RESULTS: A total of 116 IV FCM infusions were administered to 72 patients with IDA refractory to oral iron treatment (median age, 13...
October 21, 2016: Journal of Pediatrics
David P Steensma
The term "refractory anemia" was used in 1937 by Cornelius Parker Rhoads to describe patients whose anemia did not improve after treatment with liver extract or iron salts, and this term has been used to denote patients with certain subtypes of myelodysplastic syndromes (MDS) since the 1976 and 1982 French-American-British (FAB) classifications of acute leukemias and MDS. In 2016, the World Health Organization (WHO) proposed elimination of "refractory anemia" in a more general proposal for reclassification of myeloid neoplasia...
October 17, 2016: Leukemia Research
Jessica Chapman, Stephanie Marfurt, Julie Reid
This systematic review evaluates the effectiveness of delayed cord clamping in preterm infants on reducing postdelivery complications of anemia, hemodynamic instability, and the development of intraventricular hemorrhages. Interventions included varying durations of delayed cord clamping with and without cord milking as compared with immediate cord clamping, shorter delays in cord clamping, and delayed cord clamping without cord milking. A comprehensive search of randomized controlled trials, observational, cohort, and before-after studies was conducted between 1946 and 2015 in the electronic databases of Ovid MEDLINE, Embase, and Google Scholar...
October 2016: Journal of Perinatal & Neonatal Nursing
Jana G Hashash, Claudia Ramos-Rivers, Ada Youk, Wai Kan Chiu, Kyle Duff, Miguel Regueiro, David G Binion, Ioannis Koutroubakis, Ashley Vachon, David Benhayon, Michael A Dunn, Eva M Szigethy
BACKGROUND: Fatigue is common in inflammatory bowel disease (IBD) patients and is associated with factors such as psychopathology, sleep quality, and disease activity. GOAL: To investigate the combined role of all the above factors in the burden of fatigue among IBD patients. STUDY: We conducted an observational study of adult patients enrolled in an IBD clinical research registry at a tertiary care clinic. Fatigue burden was defined by Item 1 of the Short-form IBD Questionnaire (SIBDQ), which is scored on a 7-point Likert scale...
October 21, 2016: Journal of Clinical Gastroenterology
Konstantinos Triantafyllou, Paraskevas Gkolfakis, Nikos Viazis, Panagiotis Tsibouris, Athanasios Tsigaridas, Periklis Apostolopoulos, John Anastasiou, Eleni Hounda, Ioannis Skianis, Konstantina Katopodi, Xhoela Ndini, George Alexandrakis, Demetrios G Karamanolis
INTRODUCTION: Since its introduction, small bowel video capsule endoscopy (VCE) use has evolved considerably. AIM: Evaluation of the temporal changes of small bowel VCE utilization in three tertiary centers in Greece in Era 1 (2002-2009) and Era 2 (2010-2014) and the development a forecast model for future VCE use during 2015-2017. MATERIALS AND METHODS: Data from all small bowel VCE examinations were retrieved and analyzed in terms of the annual number of the performed examinations, their indications and the significance of their findings...
October 21, 2016: European Journal of Gastroenterology & Hepatology
Seon Ha Kim, Hyeon Jeong Lee, Minsu Ock, Dun Sol Go, Hyun Joo Kim, Jin Yong Lee, Min Woo Jo
Maternal and child health is an important issue throughout the world. Given their impact on maternal and child health, nutritional issues need to be carefully addressed. Accordingly, the effect of maternal, child, and nutritional disorders on disability-adjusted life years (DALYs) should be calculated. The present study used DALYs to estimate the burden of disease of maternal, neonatal, and nutritional disorders in the Korean population in 2012. For this purpose, we used claim data of the Korean National Health Insurance Service, DisMod II, and death data of the Statistics Korea and adhered to incidence-based DALY estimation methodology...
November 2016: Journal of Korean Medical Science
Elisabeth Bezine, Yann Malaisé, Aurore Loeuillet, Marianne Chevalier, Elisa Boutet-Robinet, Bernard Salles, Gladys Mirey, Julien Vignard
The Cytolethal Distending Toxin (CDT), produced by many bacteria, has been associated with various diseases including cancer. CDT induces DNA double-strand breaks (DSBs), leading to cell death or mutagenesis if misrepaired. At low doses of CDT, other DNA lesions precede replication-dependent DSB formation, implying that non-DSB repair mechanisms may contribute to CDT cell resistance. To address this question, we developed a proliferation assay using human cell lines specifically depleted in each of the main DNA repair pathways...
October 24, 2016: Scientific Reports
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