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https://www.readbyqxmd.com/read/29342998/-x-linked-immunodeficiency-with-magnesium-defect-epstein-barr-virus-infection-and-neoplasia-report-of-a-family-and-literature-review
#1
T Y He, Y Xia, C G Li, C R Li, Z X Qi, J Yang
Objective: To investigate the clinical features and genetic characteristics of cases with X-linked immunodeficiency with magnesium defect, Epstein-Barr virus (EBV) infection, and neoplasia (XMEN). Methods: Characteristics of clinical material, immunological data and gene mutation of two cases with XMEN in the same family in China were retrospectively analyzed. The related reports literature were searched by using search terms'MAGT1 gene'or'XMEN'. Results: The proband, a 2-year-eight-month old boy, was admitted due to 'Urine with deepened color for two days and yellow stained skin for one day'...
January 2, 2018: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/29338606/utility-of-mean-sphered-cell-volume-and-mean-reticulocyte-volume-for-the-diagnosis-of-hereditary-spherocytosis
#2
Rahul Darshan Arora, Jasmita Dass, Seema Maydeo, Vandana Arya, Jyoti Kotwal, Manorama Bhargava
INTRODUCTION: Hereditary spherocytosis (HS) is the most common congenital hemolytic anemia, characterized by anemia, jaundice, and splenomegaly. The diagnosis of HS relies on symptoms of hemolysis, a family history of HS, and a positive laboratory test which is usually the osmotic fragility test (OFT). We conducted a study to assess the utility of mean corpuscular hemoglobin concentration (MCHC), mean corpuscular volume (MCV), mean sphered cell volume (MSCV), and mean reticulocyte volume (MRV) in the diagnosis of HS and if these are helpful in distinguishing cases of HS from immune hemolytic anemia...
January 16, 2018: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/29324864/safety-of-single-low-dose-primaquine-in-glucose-6-phosphate-dehydrogenase-deficient-falciparum-infected-african-males-two-open-label-randomized-safety-trials
#3
Guido J H Bastiaens, Alfred B Tiono, Joseph Okebe, Helmi E Pett, Sam A Coulibaly, Bronner P Gonçalves, Muna Affara, Alphonse Ouédraogo, Edith C Bougouma, Guillaume S Sanou, Issa Nébié, John Bradley, Kjerstin H W Lanke, Mikko Niemi, Sodiomon B Sirima, Umberto d'Alessandro, Teun Bousema, Chris Drakeley
BACKGROUND: Primaquine (PQ) actively clears mature Plasmodium falciparum gametocytes but in glucose-6-phosphate dehydrogenase deficient (G6PDd) individuals can cause hemolysis. We assessed the safety of low-dose PQ in combination with artemether-lumefantrine (AL) or dihydroartemisinin-piperaquine (DP) in G6PDd African males with asymptomatic P. falciparum malaria. METHODS AND FINDINGS: In Burkina Faso, G6PDd adult males were randomized to treatment with AL alone (n = 10) or with PQ at 0...
2018: PloS One
https://www.readbyqxmd.com/read/29306055/trimethoprim-sulfamethoxazole-treatment-increases-the-pig-a-mutant-frequency-in-peripheral-blood-from-severely-malnourished-rats
#4
M Monserrat Pacheco-Martínez, Elsa Cervantes-Ríos, María Del Carmen García-Rodríguez, Rocío Ortiz-Muñiz
Severe malnutrition is a complex condition that increases susceptibility to infections. Thus, drugs are extensively used in malnutrition cases. In the present study, we assessed the mutagenic effects of combined trimethoprim and sulfamethoxazole (TMP-SMX) treatment in undernourished (UN) and well-nourished (WN) rats. Six-week-old UN and WN Han-Wistar rats were treated with TMP-SMX at a daily dose of 10 mg/kg/d TMP and 50 mg/kg/d SMX for 5 or 10 days. Blood was collected from the tail vein one day before (day -1) and 15, 30, and 45 days after TMP-SMX administration...
December 30, 2017: Mutation Research
https://www.readbyqxmd.com/read/29303771/short-and-medium-term-biological-variation-estimates-of-red-blood-cell-and-reticulocyte-parameters-in-healthy-subjects
#5
Sabrina Buoro, Anna Carobene, Michela Seghezzi, Barbara Manenti, Paola Dominoni, Aurelio Pacioni, Ferruccio Ceriotti, Cosimo Ottomano, Giuseppe Lippi
BACKGROUND: The integrated evaluation of traditional and innovative red blood cell (RBC) and reticulocyte parameters is a rapid, inexpensive and non-invasive diagnostic tools for differential diagnosis and follow-up of anemia and other pathological conditions needing bone marrow erythropoiesis assessment. Therefore, estimating the biological variation (BV) of these parameters is essential for evaluating the analytical performance of hematological analyzers, and for enabling accurate data interpretation and appropriate clinical management...
January 5, 2018: Clinical Chemistry and Laboratory Medicine: CCLM
https://www.readbyqxmd.com/read/29302006/transferrin-receptor-1-is-a-reticulocyte-specific-receptor-for-plasmodium-vivax
#6
Jakub Gruszczyk, Usheer Kanjee, Li-Jin Chan, Sébastien Menant, Benoit Malleret, Nicholas T Y Lim, Christoph Q Schmidt, Yee-Foong Mok, Kai-Min Lin, Richard D Pearson, Gabriel Rangel, Brian J Smith, Melissa J Call, Michael P Weekes, Michael D W Griffin, James M Murphy, Jonathan Abraham, Kanlaya Sriprawat, Maria J Menezes, Marcelo U Ferreira, Bruce Russell, Laurent Renia, Manoj T Duraisingh, Wai-Hong Tham
Plasmodium vivax shows a strict host tropism for reticulocytes. We identified transferrin receptor 1 (TfR1) as the receptor for P. vivax reticulocyte-binding protein 2b (PvRBP2b). We determined the structure of the N-terminal domain of PvRBP2b involved in red blood cell binding, elucidating the molecular basis for TfR1 recognition. We validated TfR1 as the biological target of PvRBP2b engagement by means of TfR1 expression knockdown analysis. TfR1 mutant cells deficient in PvRBP2b binding were refractory to invasion of P...
January 5, 2018: Science
https://www.readbyqxmd.com/read/29296203/the-pathogenesis-of-diclofenac-induced-immunoallergic-hepatitis-in-a-canine-model-of-liver-injury
#7
Saravanakumar Selvaraj, Jung-Hwa Oh, Reinhard Spanel, Florian Länger, Hyoung-Yun Han, Eun-Hee Lee, Seokjoo Yoon, Jürgen Borlak
Hypersensitivity to non-steroidal anti-inflammatory drugs is a common adverse drug reaction and may result in serious inflammatory reactions of the liver. To investigate mechanism of immunoallergic hepatitis beagle dogs were given 1 or 3 mg/kg/day (HD) oral diclofenac for 28 days. HD diclofenac treatment caused liver function test abnormalities, reduced haematocrit and haemoglobin but induced reticulocyte, WBC, platelet, neutrophil and eosinophil counts. Histopathology evidenced hepatic steatosis and glycogen depletion, apoptosis, acute lobular hepatitis, granulomas and mastocytosis...
December 8, 2017: Oncotarget
https://www.readbyqxmd.com/read/29295661/neonatal-hyperbilirubinemia-secondary-to-combined-anti-e-and-anti-c-isoimmunisation-a-literature-review
#8
Deepak Sharma, Nazanin Farahbakhsh
A term male infant was admitted at 48 hours of postnatal life to the neonatal unit for jaundice. The investigation showed total serum bilirubin (TSB) of 17.1 mg/dl, haemoglobin of 11 g/dl, reticulocyte count of 9.5% and peripheral smear was suggestive of macrocytic, normochromic red blood cell (RBC) with target cells and multiple spherocytes with occasional nucleated RBC. The infant's blood group was B positive. Direct antiglobulin test was strongly positive by gel method (3+). Mother's blood group was B positive and indirect antiglobulin test was positive when tested postnatally...
January 2, 2018: Journal of Maternal-fetal & Neonatal Medicine
https://www.readbyqxmd.com/read/29287218/high-serum-adiponectin-is-associated-with-anemia-development-in-chronic-kidney-disease-the-results-from-the-know-ckd-study
#9
Hyoungnae Kim, Hae-Ryong Yun, Seohyun Park, Jong Hyun Jhee, Jung Tak Park, Tae-Hyun Yoo, Kyu-Beck Lee, Yeong-Hoon Kim, Su-Ah Sung, Joongyub Lee, Shin-Wook Kang, Kyu Hun Choi, Curie Ahn, Seung Hyeok Han
BACKGROUND: Adiponectin is an adipokine secreted by adipocytes. A low adiponectin level is a significant risk factor of diabetes mellitus and cardiovascular disease. Recent studies have shown that adiponectin is negatively associated with hematopoiesis and predicts the development of anemia in the general population. In chronic kidney disease (CKD) patients, circulating adiponectin level is paradoxically elevated and the role of adiponectin is complex. Therefore, we evaluated the relationship between adiponectin and anemia in these patients...
December 26, 2017: Cytokine
https://www.readbyqxmd.com/read/29277545/in-vitro-reconstitution-of-translational-arrest-pathways
#10
Qing Feng, Sichen Shao
Protein translation is tightly regulated to ensure high-fidelity expression of genetic information. Various conditions cause ribosomes to stall while synthesizing new proteins. Different types of translational arrest initiate specific mRNA surveillance, protein quality control, and stress response pathways that directly impact gene expression and protein homeostasis. Our understanding of these pathways is greatly enhanced by reconstituting these processes in cell-free systems. The high degree of biochemical manipulability of in vitro systems facilitates the identification of key machineries, mechanistic dissection of their functional roles, and structural analysis of intermediate complexes...
December 22, 2017: Methods: a Companion to Methods in Enzymology
https://www.readbyqxmd.com/read/29261547/intravenous-iron-for-treatment-of-anemia-in-the-3-perisurgical-phases-a-review-and-analysis-of-the-current-literature
#11
Frank Peters, Ines Ellermann, Andrea U Steinbicker
Anemia is a common comorbidity throughout the entire hospital stay. Treatment options include intravenous (IV) iron, oral iron, erythropoietin, and red blood cell (RBC) transfusions. IV iron has gained in popularity with the implementation of patient blood management programs. A variety of studies have been performed to investigate the use of IV iron in preoperative, perioperative, and postoperative settings. An objective review on these studies has yet to be performed. The current narrative review provides an overview of trials investigating IV iron use in the preoperative, perioperative, and postoperative settings...
December 19, 2017: Anesthesia and Analgesia
https://www.readbyqxmd.com/read/29251012/enhancing-effect-of-hydroxyurea-on-hb-f-in-sickle-cell-disease-ten-year-egyptian-experience
#12
Ilham Youssry, Amina Abdel-Salam, Rania Ismail, Rayan Bou-Fakhredin, Rania Mohamed Samy, Fatma Ezz El-Deen, Ali T Taher
Patients with sickle cell disease experience hemolytic anemia and vaso-occlusions that result in pain, organ injury, and premature mortality. Several prospective studies have verified the efficacy and tolerability of hydroxyurea (HU), and demonstrated its efficacy in reducing painful vaso-occlusive crises (VOCs) in addition to its ability to increase Hb F levels. We aimed to evaluate the long-term effects of HU therapy on Hb F and assess its long term efficacy and safety in sickle cell disease patients. A retrospective study on 60 sickle cell disease patients was conducted...
December 18, 2017: Hemoglobin
https://www.readbyqxmd.com/read/29247172/host-iron-status-and-erythropoietic-response-to-iron-supplementation-determines-susceptibility-to-the-rbc-stage-of-falciparum-malaria-during-pregnancy
#13
Morgan M Goheen, Amat Bah, Rita Wegmüller, Hans Verhoef, Bakary Darboe, Ebrima Danso, Andrew M Prentice, Carla Cerami
Anaemia and malaria are both common in pregnant women in Sub-Saharan Africa. Previous evidence has shown that iron supplementation may increase malaria risk. In this observational cohort study, we evaluated P. falciparum pathogenesis in vitro in RBCs from pregnant women during their 2nd and 3rd trimesters. RBCs were collected and assayed before (n = 327), 14 days (n = 82), 49 days (n = 112) and 84 days (n = 115) after iron supplementation (60 mg iron as ferrous fumarate daily). P. falciparum erythrocytic stage growth in vitro is reduced in anaemic pregnant women at baseline, but increased during supplementation...
December 15, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29240037/fetal-anemia-causing-hydrops-fetalis-from-an-alpha-globin-variant-homozygous-hemoglobin-constant-spring
#14
Patcharee Komvilaisak, Ratana Komvilaisak, Arunee Jetsrisuparb, Surapon Wiangnon, Junya Jirapradittha, Pakaphan Kiatchoosakun, Goonapa Fucharoen
BACKGROUND: Fetal anemia is often assumed to be due to red cell alloimmunization and Parvovirus infection, and can lead to hydrops fetalis and death in utero. Other causes, such as mutations of hemoglobin alpha, are less commonly considered. METHODS: We report 7 cases with fetal anemia causing hydrops fetalis. Serial Doppler ultrasound for measurement peak systolic velocity (PSV) of middle cerebral artery (MCA) was used for evaluation of fetal anemia. Fetal anemia is suggested if the MCA/PSV ratio is >1...
December 12, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29236334/post-transfusional-changes-in-serum-hepcidin-and-iron-parameters-in-preterm-infants
#15
Fotini Stripeli, John Kapetanakis, Dimitris Gourgiotis, Antonis Drakatos, Maria Tsolia, Lydia Kossiva
BACKGROUND: Packed red blood cells (PRBCs) transfusions are common in preterm neonates. Hepcidin acts as a negative feedback iron regulator. Iron parameters such as immature reticulocyte fraction (IRF) and high light scatter reticulocytes (HLR) are used to clarify iron metabolism. Very little is known about the regulation of hepcidin in the preterms as most of reports evaluated the prohepcidin. The aim of the study was to evaluate serum hepcidin and established hematological parameters in preterms after the transfusion...
December 13, 2017: Pediatrics International: Official Journal of the Japan Pediatric Society
https://www.readbyqxmd.com/read/29226507/age-is-the-only-predictor-of-small-decrease-in-lung-function-in-children-with-sickle-cell-anemia
#16
Shaina M Willen, Robyn Cohen, Mark Rodeghier, Fenella Kirkham, Susan S Redline, Carol Rosen, Jane Kirkby, Michael R DeBaun
The longitudinal pattern of lung function in children with sickle cell anemia (SCA) has shown a decrease in FEV1 % predicted, a risk factor for death in adults with SCA, but predictors for this decline are poorly characterized. In a prospective longitudinal multi-center cohort of children with SCA, we tested the hypotheses that: 1) FEV1 % predicted declines over time; and 2) SCA-specific characteristics and therapy predict this decline. At three clinical centers, children with SCA (HbSS or HbSβ0 thalassemia), unselected for respiratory disease, were enrolled in the Sleep and Asthma Cohort (SAC) study...
December 11, 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/29226393/analysis-of-abo-chimera-from-peripheral-red-cells-and-reticulocytes-by-flow-cytometry-and-micro-gel-column-technique-in-patients-post-abo-incompatible-hsct
#17
Jianhua Chen, Feng Liu
BACKGROUND: How to choose an appropriate method to monitoring dynamic ABO chimera post ABO incompatible HSCT is crucial to not only assess the status of erythroid engraftment but achieve personalized safety transfusion. METHODS: We evaluated the efficacy of micro gel column technique(MGCT) and flow cytometry(FCM) by series of artificial ABO chimera mixtures from 0.5% to 50% and by investigated 15 cases of ABO incompatible HSCT patients with longitudinally ABO blood grouping and ABO chimera from RBCs and reticulocytes...
December 11, 2017: Clinical Transplantation
https://www.readbyqxmd.com/read/29222289/what-is-the-role-of-screening-for-pulmonary-hypertension-in-adults-and-children-with-sickle-cell-disease
#18
REVIEW
Shaina M Willen, Mark T Gladwin
Patient case: An 18-year-old male patient with homozygous hemoglobin SS disease was evaluated for progressive dyspnea and elevated tricuspid regurgitant jet velocity (TRV) on echocardiography. The patient's case is described in detail in Lancet1 He had been treated with regular transfusions since childhood for stroke, had rare episodes of vaso-occlusive pain episodes, and did not take narcotic pain medications. He presented with progressive severe dyspnea on exertion and lower extremity edema. His laboratory tests were notable for a total hemoglobin level of 11...
December 8, 2017: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/29202601/hematological-parameters-outperform-plasma-markers-in-predicting-long-term-mortality-after-coronary-angiography
#19
Crystel M Gijsberts, Hester M den Ruijter, Dominique P V de Kleijn, Albert Huisman, Maarten Ten Berg, Mark de Groot, Richard H A van Wijk, Folkert W Asselbergs, Michiel Voskuil, Gerard Pasterkamp, Wouter W van Solinge, Imo E Hoefer
High-sensitivity troponin I (hsTnI) and N-terminal pro-brain natriuretic peptide (NT-pro-BNP) are predictors of coronary artery disease. Recently, routine hematological parameters emerged as mortality predictors. We examined the predictive value of hematological parameters (from the Utrecht Patient Oriented Database) and hsTnI and NT-pro-BNP for mortality in a coronary angiography population (Utrecht Coronary Biobank n = 1913). Using Cox regression, receiver operating characteristics, integrated discrimination improvement (IDI), and continuous net reclassification improvement (cNRI) analysis, we compared the predictive properties of hematological parameters with hsTnI and NT-pro-BNP for mortality...
January 1, 2017: Angiology
https://www.readbyqxmd.com/read/29195085/clinical-and-hematological-profile-in-a-newborn-cohort-with-hemoglobin-sc
#20
Paulo V Rezende, Millane V Santos, Gustavo F Campos, Laura L M Vieira, Maristela B Souza, André R Belisário, Celia M Silva, Marcos B Viana
OBJECTIVES: Hemoglobin SC is the second most common variant of sickle-cell disease worldwide, after hemoglobin SS. The objectives of the study were to describe the clinical and laboratory characteristics of hemoglobin SC disease in children from a newborn screening program and treated at a blood donor center. METHODOLOGY: This study assessed a cohort of 461 infants born between 01/01/1999 and 12/31/2012 and followed-up until 12/31/2014. Clinical events were expressed as rates for 100 patient-years, with 95% confidence intervals...
November 28, 2017: Jornal de Pediatria
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