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Red cell pathology

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https://www.readbyqxmd.com/read/29050073/-clinicopathologic-features-of-three-cases-of-erythropoietic-protoporphyria-with-liver-involvement
#1
J Shi, X Q Li, Z H Lu
Objective: To investigate the clinicopathologic features of the erythropoietic protoporphyria (EPP) with liver involvement. Methods: The clinical findings and hepatic biopsy of 3 cases of EPP diagnosed between July, 2011 to August, 2014 with liver involvement were reviewed, with relevant literature review. Results: All patients presented with persistent and refractory abdominal pain, with obvious jaundice and deranged liver function. Imaging showed homogeneous hepatomegaly in all patients. Histologically, the hepatocytes were edematous, and contained numerous cytoplasmic globular brown pigments and bile pigments, which were also found in Kupffer cells, in the bile canaliculi and in some of dilated sinusoid...
October 8, 2017: Zhonghua Bing Li Xue za Zhi Chinese Journal of Pathology
https://www.readbyqxmd.com/read/29045442/a-semi-synthetic-glycosaminoglycan-analogue-inhibits-and-reverses-plasmodium-falciparum-cytoadherence
#2
Mark A Skidmore, Khairul Mohd Fadzli Mustaffa, Lynsay C Cooper, Scott E Guimond, Edwin A Yates, Alister G Craig
A feature of mature Plasmodium falciparum parasitized red blood cells is their ability to bind surface molecules of the microvascular endothelium via the parasite-derived surface protein Plasmodium falciparum erythrocyte membrane protein 1 (PfEMP1). This ligand is associated with the cytoadherence pathology observed in severe malaria. As pRBC treated with effective anti-malarial drugs are still able to cytoadhere, there is therefore a need to find an adjunct treatment that can inhibit and reverse the adhesion process...
2017: PloS One
https://www.readbyqxmd.com/read/29042979/mechanism-of-apoptosis-involved-in-gastric-mucosal-lesions-in-tibetans-with-high-altitude-polycythemia
#3
Kang Li, Luobu Gesang, Chaohui He
The Tibetan high plateau is a low-oxygen environment, which may cause the pathogenesis of high-altitude polycythemia (HAPC). Gastric mucosal lesions (GML) are a common complication of HAPC. The molecular mechanisms involved in HAPC-induced GML have remained to be fully elucidated and were therefore investigated in the present study. Gastric tissues of patients with heavy, HAPC-induced GML and healthy controls were assessed by ultrastructural and histopathological analysis. In addition, terminal deoxynucleotidyl transferase-mediated deoxyuridine triphosphate nick-end labeling and reverse transcription-quantitative polymerase chain reaction (RT-qPCR) analysis were used to detect cell apoptosis in the gastric mucosa tissues...
October 2017: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/29024480/osmotic-gradient-ektacytometry-a-valuable-screening-test-for-hereditary-spherocytosis-and-other-red-blood-cell-membrane-disorders
#4
E Llaudet-Planas, J L Vives-Corrons, V Rizzuto, P Gómez-Ramírez, J Sevilla Navarro, M T Coll Sibina, M García-Bernal, A Ruiz Llobet, I Badell, P Velasco-Puyó, J L Dapena, M M Mañú-Pereira
INTRODUCTION: New generation osmotic gradient ektacytometry has become a powerful procedure for measuring red blood cell deformability and therefore for the diagnosis of red blood cell membrane disorders. In this study, we aim to provide further support to the usefulness of osmotic gradient ektacytometry for the differential diagnosis of hereditary spherocytosis by measuring the optimal cutoff values of the parameters provided by this technique. METHODS: A total of 65 cases of hereditary spherocytosis, 7 hereditary elliptocytosis, 3 hereditary xerocytosis, and 171 normal controls were analyzed with osmotic gradient ektacytometry in addition to the routine red blood cell laboratory techniques...
October 10, 2017: International Journal of Laboratory Hematology
https://www.readbyqxmd.com/read/29021902/significantly-elevated-foetal-haemoglobin-levels-in-individuals-with-glucose-6-phosphate-dehydrogenase-disease-and-or-sickle-cell-trait-a-cross-sectional-study-in-cape-coast-ghana
#5
Patrick Adu, Essel K M Bashirudeen, Florence Haruna, Edward Morkporkpor Adela, Richard K D Ephraim
BACKGROUND: Previously published data have demonstrated that sickle red blood cells produce twice as much reactive oxygen species (ROS) suggesting that co-inheritance of sickle cell disease (SCD) and glucose 6-phosphate dehydrogenase (G6PD) enzymopathy could lead to more severe anaemia during sickling crises. Elevated foetal haemoglobin (Hb F) levels have been shown to have positive modulatory effects on sickling crises and disease outcomes. This study sought to assess how inheritance of G6PD enzymopathy affects the level of Hb F and haemoglobin concentration in adults in steady state...
2017: BMC Hematology
https://www.readbyqxmd.com/read/29020888/clinical-haematology-and-biochemistry-profiles-of-cattle-naturally-infected-with-theileria-orientalis-ikeda-type-in-new-zealand
#6
K E Lawrence, S F Forsyth, B Vaatstra, A M J McFadden, D Pulford, K Govindaraju, W Pomroy
AIMS: To present the haematology and biochemistry profiles for cattle in New Zealand naturally infected with Theileria orientalis Ikeda type and investigate if the results differed between adult dairy cattle and calves aged <6 months. METHODS: Haematology and biochemistry results were obtained from blood samples from cattle which tested positive for T. orientalis Ikeda type by PCR, that were submitted to veterinary laboratories in New Zealand between October 2012 and November 2014...
October 11, 2017: New Zealand Veterinary Journal
https://www.readbyqxmd.com/read/28989659/a-multi-signal-fluorescent-probe-for-simultaneously-distinguishing-and-sequentially-sensing-cysteine-homocysteine-glutathione-and-hydrogen-sulfide-in-living-cells
#7
Longwei He, Xueling Yang, Kaixin Xu, Xiuqi Kong, Weiying Lin
Biothiols, which have a close network of generation and metabolic pathways among them, are essential reactive sulfur species (RSS) in the cells and play vital roles in human physiology. However, biothiols possess highly similar chemical structures and properties, resulting in it being an enormous challenge to simultaneously discriminate them from each other. Herein, we develop a unique fluorescent probe (HMN) for not only simultaneously distinguishing Cys/Hcy, GSH, and H2S from each other, but also sequentially sensing Cys/Hcy/GSH and H2S using a multi-channel fluorescence mode for the first time...
September 1, 2017: Chemical Science
https://www.readbyqxmd.com/read/28988798/differential-carbonylation-of-proteins-in-end-stage-human-fatty-and-nonfatty-nash
#8
Colin T Shearn, Laura M Saba, James R Roede, David J Orlicky, Alisabeth H Shearn, Dennis R Petersen
OBJECTIVE: In the liver, a contributing factor in the pathogenesis of non-alcoholic fatty liver disease is oxidative stress leading to the accumulation of highly reactive electrophilic α/β unsaturated aldehydes. The objective of this study was to determine if significant differences were evident when evaluating carbonylation in human end-stage fatty nonalcoholic steatohepatitis (fNASH) compared to end-stage nonfatty NASH (nfNASH). METHODS: Using hepatic tissue obtained from healthy humans and patients diagnosed with end stage nfNASH or fNASH, overall carbonylation was assessed by immunohistochemistry (IHC) and LC-MS/MS followed by bioinformatics...
October 6, 2017: Free Radical Biology & Medicine
https://www.readbyqxmd.com/read/28984339/a-unique-immunofluorescence-protocol-to-detect-protein-expression-in-vascular-tissues-tacking-a-long-standing-pathological-hitch
#9
Puneet Gandhi, Richa Khare
OBJECTIVE: Autofluorescence induced interference is one of the major drawbacks in immunofluorescence analysis of formalin-fixed paraffin-embedded tissues, as it decreases the signal-to-noise ratio of specific labeling. Apart from aldehyde-fixation induced artifacts; collagen and elastin, red blood cells and endogenous fluorescent pigment lipofuscin are prime sources of autofluorescence in vascular and aging tissues. We describe herein, an optimized indirect-immunofluorescence method for archival formalin-fixed paraffin-embedded tissues tissues and cryo sections, using a combination of 3-reagents in a specific order, to achieve optimal fluorescence signals and imaging...
October 6, 2017: Türk Patoloji Dergisi
https://www.readbyqxmd.com/read/28978568/impact-of-extracellular-fluid-tonicity-on-sickle-red-blood-cell-deformability-and-adhesion
#10
Marcus A Carden, Meredith E Fay, Xinran Lu, Robert G Mannino, Yumiko Sakurai, Jordan C Ciciliano, Caroline E Hansen, Satheesh Chonat, Clinton H Joiner, David K Wood, Wilbur A Lam
Abnormal sickle red blood cell (sRBC) biomechanics, including pathological deformability and adhesion, correlate with clinical severity in sickle cell disease (SCD). Clinical intravenous fluids (IVFs) of various tonicities are often used during treatment of vaso-occlusive pain episodes (VOE), the major cause of morbidity in SCD. However, evidence-based guidelines are lacking and there is no consensus regarding which IVFs to use during VOE. Further, it is unknown how altering extracellular fluid tonicity with IVFs affects sRBC biomechanics in the microcirculation, where vaso-occlusion takes place...
October 4, 2017: Blood
https://www.readbyqxmd.com/read/28966701/in-vitro-particulate-analogue-fluids-for-experimental-studies-of-rheological-and-hemorheological-behavior-of-glucose-rich-rbc-suspensions
#11
Diana Pinho, Laura Campo-Deaño, Rui Lima, Fernando T Pinho
Suspensions of healthy and pathological red blood cells (RBC) flowing in microfluidic devices are frequently used to perform in vitro blood experiments for a better understanding of human microcirculation hemodynamic phenomena. This work reports the development of particulate viscoelastic analogue fluids able to mimic the rheological and hemorheological behavior of pathological RBC suspensions flowing in microfluidic systems. The pathological RBCs were obtained by an incubation of healthy RBCs at a high concentration of glucose, representing the pathological stage of hyperglycaemia in diabetic complications, and analyses of their deformability and aggregation were carried out...
September 2017: Biomicrofluidics
https://www.readbyqxmd.com/read/28962187/the-therapeutic-effect-of-zerumbone-on-chronic-gastritis-via-antioxidant-mechanisms
#12
Liqing Li, Liang Kong, Hongquan Song
Effects of zerumbone on chronic gastritis remain unclear. The purpose of this study was to investigate the mechanism of the protective effect of zerumbone on the treatment of chronic gastritis in rats. The animal models of chronic gastritis in rats were established, and the surface damage of gastric mucosa was observed by gross anatomy; the changes of gastric mucosal tissue and surface morphology were observed by pathological sections of gastric mucosal tissues; the expressions of heme oxygenase-1 (HO-1) and nuclear factor E2-related factor 2 (Nrf-2) proteins of gastric mucosal tissues in each group were detected by western blot analysis; the activities of superoxide dismutase (SOD) and catalase (CAT) as well as the contents of reduced glutathione (GSH) and malondialdehyde (MDA) in gastric mucosal tissues were detected by kits...
September 2017: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/28959409/a-universal-fluorogenic-switch-for-fe-ii-ion-based-on-n-oxide-chemistry-permits-the-visualization-of-intracellular-redox-equilibrium-shift-towards-labile-iron-in-hypoxic-tumor-cells
#13
Tasuku Hirayama, Hitomi Tsuboi, Masato Niwa, Ayaji Miki, Satoki Kadota, Yukie Ikeshita, Kensuke Okuda, Hideko Nagasawa
Iron (Fe) species play a number of biologically and pathologically important roles. In particular, iron is a key element in oxygen sensing in living tissue where its metabolism is intimately linked with oxygen metabolism. Regulation of redox balance of labile iron species to prevent the generation of iron-catalyzed reactive oxygen species (ROS) is critical to survival. However, studies on the redox homeostasis of iron species are challenging because of a lack of a redox-state-specific detection method for iron, in particular, labile Fe(2+)...
July 1, 2017: Chemical Science
https://www.readbyqxmd.com/read/28958290/lenalidomide-myelodysplastic-syndromes-with-del-5q-and-beyond
#14
REVIEW
Chetasi Talati, David Sallman, Alan List
Myelodysplastic syndrome (MDS) with deletion 5q (del(5q)) is a distinct clinical and pathological disease subset that is exquisitely sensitive to lenalidomide for the treatment of red blood cell transfusion-dependent anemia. Although lenalidomide has erythropoeitic promoting activity in MDS without del(5q) (non-del(5q) MDS), the frequency of response to treatment is lower and relates to biologically separate drug effects. In del(5q) MDS, lenalidomide suppresses the malignant clone to restore effective erythropoiesis by virtue of synthetic lethality, arising from cereblon-dependent degradation of haplodeficient proteins encoded within the commonly deleted region of the chromosome 5q deletion...
July 2017: Seminars in Hematology
https://www.readbyqxmd.com/read/28947137/hint2-triggers-mitochondrial-ca-2-influx-by-regulating-the-mitochondrial-ca-2-uniporter-mcu-complex-and-enhances-gemcitabine-apoptotic-effect-in-pancreatic-cancer
#15
Linghui Chen, Qiang Sun, Dongkai Zhou, Wei Song, Qifan Yang, Bingjie Ju, Lufei Zhang, Haiyang Xie, Lin Zhou, Zhenhua Hu, Hangping Yao, Shusen Zheng, Weilin Wang
In early studies, it was shown that HINT2, which sensitizes cells to mitochondrial apoptosis, is down-regulated in hepatocellular carcinoma (HCC) cells (Martin et al., 2006). However, the molecular mechanism of this effect is unknown. Immunohistochemistry revealed that HINT2 expression is relatively low in pancreatic cancer tissues, compared to that in adjacent tissues (P < 0.05). Furthermore, its expression was related to pathological grade and lymph node metastasis (P = 0.0161 and 0.0108, respectively); in addition, down-regulation of HINT2 was found to be associated with relatively poor prognosis in pancreatic cancer patients...
September 23, 2017: Cancer Letters
https://www.readbyqxmd.com/read/28940216/zeroing-in-on-red-blood-cell-unit-expiry
#16
Fathima Ayyalil, Greg Irwin, Bryony Ross, Michael Manolis, Anoop K Enjeti
BACKGROUND: Expiry of red blood cell (RBC) units is a significant contributor to wastage of precious voluntary donations. Effective strategies aimed at optimal resource utilization are required to minimize wastage. STUDY DESIGN AND METHODS: This retrospective study analyzed the strategic measures implemented to reduce expiry of RBC units in an Australian tertiary regional hospital. The measures, which included inventory rearrangement, effective stock rotation, and the number of emergency courier services required during a 24-month period, were evaluated...
September 20, 2017: Transfusion
https://www.readbyqxmd.com/read/28931864/lymphoblastoid-cell-lines-from-diamond-blackfan-anaemia-patients-exhibit-a-full-ribosomal-stress-phenotype-that-is-rescued-by-gene-therapy
#17
Anna Aspesi, Valentina Monteleone, Marta Betti, Chiara Actis, Giulia Morleo, Marika Sculco, Simonetta Guarrera, Marcin W Wlodarski, Ugo Ramenghi, Claudio Santoro, Steven R Ellis, Fabrizio Loreni, Antonia Follenzi, Irma Dianzani
Diamond Blackfan anaemia (DBA) is a congenital bone marrow failure syndrome characterised by selective red cell hypoplasia. DBA is most often due to heterozygous mutations in ribosomal protein (RP) genes that lead to defects in ribosome biogenesis and function and result in ribosomal stress and p53 activation. The molecular mechanisms underlying this pathology are still poorly understood and studies on patient erythroid cells are hampered by their paucity. Here we report that RP-mutated lymphoblastoid cell lines (LCLs) established from DBA patients show defective rRNA processing and ribosomal stress features such as reduced proliferation, decreased protein synthesis, and activation of p53 and its target p21...
September 20, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28931828/a-novel-h2s-releasing-amino-bisphosphonate-which-combines-bone-anti-catabolic-and-anabolic-functions
#18
Simona Rapposelli, Laura Gambari, Maria Digiacomo, Valentina Citi, Gina Lisignoli, Cristina Manferdini, Vincenzo Calderone, Francesco Grassi
Bisphosphonates (BPs) are the first-line treatment of bone loss resulting from various pathological conditions. Due to their high affinity to bone they have been used to develop conjugates with pro-anabolic or anti-catabolic drugs. We recently demontrated that hydrogen sulfide (H2S), promotes osteogenesis and inhibits osteoclast differentiation. Here we developed an innovative molecule, named DM-22, obtained from the combination of alendronate (AL) and the H2S-releasing moiety aryl-isothiocyanate. DM-22 and AL were assayed in vitro in the concentration range 1-33 μM for effects on viability and function of human osteoclasts (h-OCs) and mesenchymal stromal cells (h-MSCs) undergoing osteogenic differentiation...
September 20, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28928122/discovery-and-preclinical-characterization-of-gsk1278863-daprodustat-a-small-molecule-hypoxia-inducible-factor-hif-prolyl-hydroxylase-inhibitor-for-anemia
#19
Jennifer L Ariazi, Kevin J Duffy, David F Adams, Duke M Fitch, Lusong Luo, Melissa Pappalardi, Mangatt Biju, Erin Hugger DiFilippo, Tony Shaw, Ken Wiggall, Connie Erickson-Miller
Decreased erythropoietin (EPO) production, shortened erythrocyte survival, and other factors reducing the response to EPO contribute to anemia in patients with a variety of underlying pathologies such as chronic kidney disease (CKD). Treatment with recombinant human EPO (rHuEPO) at supraphysiological concentrations has proven to be efficacious. However, it does not ameliorate the condition in all patients and presents its own risks, including cardiovascular complications. The transcription factors hypoxia-inducible factor (HIF)1α and HIF2α control the physiological response to hypoxia and invoke a program of increased erythropoeisis...
September 19, 2017: Journal of Pharmacology and Experimental Therapeutics
https://www.readbyqxmd.com/read/28925802/higher-prevalence-of-spontaneous-cerebral-vasculopathy-and-cerebral-infarcts-in-a-mouse-model-of-sickle-cell-disease
#20
Hyacinth I Hyacinth, Courtney L Sugihara, Thomas L Spencer, David R Archer, Andy Y Shih
Stroke is a dramatic complication of sickle cell disease (SCD), which is associated with cerebral vasculopathies including moya moya, intravascular thrombi, cerebral hyperemia, and increased vessel tortuosity. The spontaneous occurrence of these pathologies in the sickle cell mouse model has not been described. Here, we studied Townes humanized sickle cell and age-matched control mice that were 13 months old. We used in vivo two-photon microscopy to assess blood flow dynamics, vascular topology, and evidence of cerebral vasculopathy...
January 1, 2017: Journal of Cerebral Blood Flow and Metabolism
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