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hemolytic anemias

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https://www.readbyqxmd.com/read/28550351/diagnostic-and-treatment-guidelines-for-thrombotic-thrombocytopenic-purpura-ttp-2017-in-japan
#1
Masanori Matsumoto, Yoshihiro Fujimura, Hideo Wada, Koichi Kokame, Yoshitaka Miyakawa, Yasunori Ueda, Satoshi Higasa, Takanori Moriki, Hideo Yagi, Toshiyuki Miyata, Mitsuru Murata
Thrombotic thrombocytopenic purpura (TTP) can rapidly progress into a life-threatening condition, thus the importance of appropriate diagnosis and treatment cannot be overstated. Until recently, TTP has mainly been diagnosed by clinical findings such as thrombocytopenia and non-immune hemolytic anemia. In addition to these clinical findings, however, reduced activity of a disintegrin-like and metalloprotease with thrombospondin type 1 motif 13 (ADAMTS13) below 10% has been accepted internationally as a diagnostic criterion for TTP...
May 26, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/28550188/recommendations-for-splenectomy-in-hereditary-hemolytic-anemias
#2
Achille Iolascon, Immacolata Andolfo, Wilma Barcellini, Francesco Corcione, Loïc Garçon, Lucia De Franceschi, Claudio Pignata, Giovanna Graziadei, Dagmar Pospisilova, David C Rees, Mariane de Montalembert, Stefano Rivella, Antonella Gambale, Roberta Russo, Leticia Ribeiro, Jules Vives-Corrons, Patricia Aguilar-Martinez, Antonis Kattamis, Beatrice Gulbis, Maria Domenica Cappellini, Irene Roberts, Hannah Tamary
Hereditary hemolytic anemias are a group of disorders including red cell membrane defects, red blood cells enzyme disorders, congenital dyserythropoietic anemias, thalassemia syndromes and haemoglobinopathies. As damaged red blood cells passing through the spleen red pulp are efficiently removed by splenic macrophages, splenectomy is one possible therapeutic approach to the management of severely affected patients. However, except for hereditary spherocytosis for which the effectiveness of splenectomy has been well documented, the efficacy of splenectomy in other anemias within this group has yet to be determined and there are concerns regarding short- and long-term infectious and thrombotic complications...
May 26, 2017: Haematologica
https://www.readbyqxmd.com/read/28549435/molecular-characterization-of-hemotropic-mycoplasmas-mycoplasma-ovis-and-candidatus-mycoplasma-haemovis-in-sheep-and-goats-in-china
#3
Xiaoxing Wang, Yanyan Cui, Yan Zhang, Ke Shi, Yaqun Yan, Fuchun Jian, Longxian Zhang, Rongjun Wang, Changshen Ning
BACKGROUND: Hemotropic mycoplasmas (hemoplasmas) are emerging zoonotic pathogens with a worldwide distribution that can cause mild to severe hemolytic anemia, icterus, ill-thrift, infertility, and poor weight gain. However, understanding of the molecular epidemiology of hemoplasmas (Mycoplasma ovis and 'Candidatus Mycoplasma haemovis') is limited in sheep and goats, and the hemoplasma strain/species/variant 'Candidatus M. haemovis' was poorly studied throughout the world and had never been detected in China until now...
May 26, 2017: BMC Veterinary Research
https://www.readbyqxmd.com/read/28542880/suicidal-death-of-erythrocytes-in-cancer-and-its-chemotherapy-a-potential-target-in-the-treatment-of-tumor-associated-anemia
#4
REVIEW
Elisabeth Lang, Rosi Bissinger, Syed M Qadri, Florian Lang
In analogy to apoptosis of nucleated cells, erythrocytes may enter eryptosis characterized by cell shrinkage and cell membrane scrambling. Eryptotic erythrocytes are rapidly cleared from circulating blood and may adhere to the vascular wall. Stimulation of eryptosis thus impairs microcirculation and leads to anemia as soon as the loss of erythrocytes cannot be fully compensated by enhanced erythropoiesis. Signaling stimulating eryptosis includes increase of cytosolic Ca(2+) -activity, ceramide, caspases, calpain, p38-kinase, protein-kinase C, Janus-activated kinase 3, casein-kinase 1α, and cyclin-dependent kinase 4...
May 23, 2017: International Journal of Cancer. Journal International du Cancer
https://www.readbyqxmd.com/read/28538406/dexmedetomidine-based-intravenous-anesthesia-of-a-pediatric-patient-with-glucose-6-phosphate-dehydrogenase-g6pd-deficiency-a-case-report
#5
Nanae Takahashi, Takashi Ogawa, Zen'ichiro Wajima, Akibumi Omi
RATIONALE: Glucose-6-phosphate dehydrogenase (G6PD) deficiency is the most common human enzyme defect, resulting in deficits in nicotinamide adenine dinucleotide phosphate production, an important intracellular antioxidant enzyme. G6PD-deficient subjects present with a susceptibility of erythrocytes to oxidative stress and hemolysis, and should avoid drugs or stressors that have oxidative actions. Dexmedetomidine is an anesthetic agent with antioxidant actions. PATIENT CONCERNS AND DIAGNOSES: A 5-year-old boy with G6PD deficiency...
May 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28535517/coupled-plasma-filtration-adsorption-application-for-liver-and-thyroid-toxins
#6
Gabriele Donati, Irene Capelli, Anna Laura Croci Chiocchini, Nicolò Natali, Anna Scrivo, Gaetano La Manna
Coupled plasma filtration and adsorption (CPFA) is a detoxification system that combines a plasma adsorption circuit and a continuous renal replacement therapy circuit. Its main application is for sepsis and septic shock with or without acute renal failure. Several recent studies have suggested that CPFA can reduce the mortality when the volume of plasma absorbed on the styrenic resin is at least >0.18 L/kg/day. At present, new applications for CPFA are under investigation, also in patients without significant kidney failure...
2017: Contributions to Nephrology
https://www.readbyqxmd.com/read/28533306/bendamustine-plus-rituximab-for-chronic-cold-agglutinin-disease-results-of-a-nordic-prospective-multicenter-trial
#7
Sigbjørn Berentsen, Ulla Randen, Markku Oksman, Henrik Birgens, Tor Henrik Anderson Tvedt, Jakob Dalgaard, Eivind Galteland, Einar Haukås, Robert Brudevold, Jon Hjalmar Sørbø, Inger Anne Næss, Agnieszka Malecka, Geir E Tjønnfjord
Primary chronic cold agglutinin disease (CAD) is a well-defined clinicopathologic entity in which a bone marrow clonal B-cell lymphoproliferation results in autoimmune hemolytic anemia and cold-induced circulatory symptoms. Rituximab monotherapy or fludarabine-rituximab in combination are documented treatment options. In a prospective, non-randomized multicenter trial, 45 eligible patients received rituximab 375 mg/m(2) day 1 and bendamustine 90 mg/m(2) day 1 and 2 for 4 cycles at 28 days interval. Thirty-two patients (71%) responded; 18 (40%) achieved complete response (CR) and 14 (31%) partial response (PR)...
May 22, 2017: Blood
https://www.readbyqxmd.com/read/28533197/incomplete-antibodies-may-reduce-abo-cross-match-incompatibility-a-pilot-study
#8
Mehmet Özen, Soner Yılmaz, Tülin Özkan, Yeşim Özer, Aliye Aysel Pekel, Asuman Sunguroğlu, Günhan Gürman, Önder Arslan
OBJECTIVE: Any erythrocyte transfusion among humans having type A or B blood groups is impossible due to antibodies causing fatal transfusion complications. A cross-match test is performed to prevent immune transfusion complications before transfusion. Our hypothesis is that the Fragment antibody (Fab) part of antibody (incomplete antibody) may be used to prevent an immune stimulus related to complete antibody. Therefore, we designed a pilot study to evaluate the effectiveness of these incomplete antibodies with using cross-match tests...
May 23, 2017: Turkish Journal of Haematology: Official Journal of Turkish Society of Haematology
https://www.readbyqxmd.com/read/28531196/co-inheritance-of-glucose-6-phosphate-dehydrogenase-deficiency-mutations-and-hemoglobin-e-in-a-kachin-population-in-a-malaria-endemic-region-of-southeast-asia
#9
Zeshuai Deng, Fang Yang, Yao Bai, Lijun He, Qing Li, Yanrui Wu, Lan Luo, Hong Li, Limei Ma, Zhaoqing Yang, Yongshu He, Liwang Cui
Glucose-6-phosphate dehydrogenase (G6PD) deficiency and hemoglobin E (HbE, β26 Glu-Lys) are two common red cell disorders in Southeast Asia. G6PD deficiency produces hemolytic anemia, which can be triggered by certain drugs or infections. HbE is asymptomatic or is manifested as microcytic, minimally hemolytic anemia. The association between G6PD deficiency and HbE is little understood. This study aimed to investigate G6PD deficiency and HbE in a Kachin ethnic group in the China-Myanmar border area. G6PD enzyme activity was measured using a quantitative G6PD assay, G6PD variants genotyped by the SNaPshot assay, and an HbE gene mutation identified by an amplification refractory mutation system and subsequently confirmed by using a reverse dot blot hybridization assay from 100 unrelated individuals in the study area...
2017: PloS One
https://www.readbyqxmd.com/read/28529915/hemolytic-uremic-syndrome-in-adults-a-case-report
#10
Fabiel Gerardo Pérez-Cruz, Patricia Villa-Díaz, María Consuelo Pintado-Delgado, María Loreto Fernández Rodríguez, Ana Blasco-Martínez, María Pérez-Fernández
Thrombotic microangiopathies (TMA) are microvascular occlusive disorders characterized by platelet aggregation and mechanical damage to erythrocytes, clinically characterized by microangiopatic haemolytic anemia, thrombocytopenia and organ injury. We are reporting a case of a woman patient with severe hemolytic uremic syndrome associated to infectious diarrhoea caused by Shiga toxin-producing pathogen, who were admitted to our intensive care unit. The patient described developed as organ injury, neurological failure and acute renal failure, with need of haemodialysis technique...
May 4, 2017: World Journal of Critical Care Medicine
https://www.readbyqxmd.com/read/28524208/microfluidic-analysis-of-red-blood-cell-deformability-as-a-means-to-assess-hemin-induced-oxidative-stress-resulting-from-plasmodium-falciparum-intraerythrocytic-parasitism
#11
Kerryn Matthews, Simon P Duffy, Marie-Eve Myrand-Lapierre, Richard R Ang, Li Li, Mark D Scott, Hongshen Ma
Hemolytic anemia is one of the hallmarks of malaria and leads to an increase in oxidized heme (hemin) within the plasma of infected individuals. While scavenger proteins sequester much of the circulating heme, it has been hypothesized that extracellular heme may play a central role in malaria pathogenesis. We have previously developed the multiplex fluidic plunger (MFP) device for the measurement of red blood cell (RBC) deformability. Here, we demonstrate that the measurement of changes in RBC deformability is a sensitive method for inferring heme-induced oxidative stress...
May 19, 2017: Integrative Biology: Quantitative Biosciences From Nano to Macro
https://www.readbyqxmd.com/read/28516093/iatrogenic-supravalvular-aortic-stenosis
#12
Paolo Bosco, Antonella Ferrara, Samer A M Nashef
We describe a case of hemolytic anemia and proximal anastomotic site stenosis following emergency repair of a Type A aortic dissection. This rare complication led to a reoperation to correct the iatrogenic aortic stenosis and cure the consequent hemolysis. A "sandwich" technique (with two Teflon strips on the outside and inside of the aortic wall) was used in the initial repair to reinforce the suture line and prevent bleeding from the aortic anastomoses. At the time of reoperation, the inner Teflon strip at the proximal aortic anastomosis was found to have inverted into the aortic lumen, as suggested by the preoperative magnetic resonance imaging...
October 2016: Aorta (Stamford, Conn.)
https://www.readbyqxmd.com/read/28515936/pulse-dose-steroids-in-severe-pulmonary-arterial-hypertension-secondary-to-systemic-lupus-erythematosus
#13
Cody Lee, Jean Elwing
OBJECTIVE: The pulmonary vascular targeted treatment for systemic lupus erythematosus-associated pulmonary arterial hypertension is similar to other connective tissue disease-associated pulmonary arterial hypertension. In addition, there also appears to be a role for immunosuppression in the overall management. However, the optimal immunosuppressive regimen and what patients will respond to treatments are currently not clearly elucidated given the lack of randomized controlled trials on the subject...
2017: SAGE open medical case reports
https://www.readbyqxmd.com/read/28508588/at-the-cross-section-of-thrombotic-microangiopathy-and-atypical-hemolytic-uremic-syndrome-a-narrative-review-of-differential-diagnostics-and-a-problematization-of-nomenclature
#14
REVIEW
Alexander Åkesson, Eva Zetterberg, Jenny Klintman
Complement-mediated atypical hemolytic uremic syndrome (aHUS) is a rare disease associated with high mortality and morbidity. Renal biopsies often indicate thrombotic microangiopathy (TMA). The condition is caused by an excessive activation of the alternative pathway leading to depositions of membrane attack complexes (MAC) on host cells. It may depend on mutations in complement components and regulatory proteins, or the formation of complement-specific antibodies. Mainly, an environmental trigger (e.g. infection) is needed for the excessive response to develop...
May 16, 2017: Therapeutic Apheresis and Dialysis
https://www.readbyqxmd.com/read/28505305/efficacy-and-safety-of-ledipasvir-sofosbuvir-for-the-treatment-of-chronic-hepatitis-c-in-persons-with-sickle-cell-disease
#15
Juhi Moon, Robert H Hyland, Fangqiu Zhang, Diana M Brainard, Sophie Lanzkron, John G McHutchison, Mark Sulkowski
Patients with sickle cell disease are at high risk for chronic hepatitis C infection. Prior treatment has been limited due to the use of ribavirin causing hemolytic anemia and interferon causing cytopenias. We demonstrate the safety and efficacy of fixed dose combination, ledipasvir and sofosbuvir for 12 weeks in this population.
May 13, 2017: Clinical Infectious Diseases: An Official Publication of the Infectious Diseases Society of America
https://www.readbyqxmd.com/read/28503958/neonatal-management-and-outcome-in-alloimmune-hemolytic-disease
#16
Isabelle M C Ree, Vivianne E H J Smits-Wintjens, Johanna G van der Bom, Jeanine M M van Klink, Dick Oepkes, Enrico Lopriore
Hemolytic disease of the fetus and newborn (HDFN) occurs when fetal and neonatal erythroid cells are destroyed by maternal erythrocyte alloantibodies, it leads to anemia and hydrops in the fetus, and hyperbilirubinemia and kernicterus in the newborn. Postnatal care consists of intensive phototherapy and exchange transfusions to treat severe hyperbilirubinemia and top-up transfusions to treat early and late anemia. Other postnatal complications have been reported such as thrombocytopenia, iron overload and cholestasis requiring specific management...
May 13, 2017: Expert Review of Hematology
https://www.readbyqxmd.com/read/28502324/favorable-long-term-outcomes-of-isolated-liver%C3%A2-transplantation-in-a-child-with-atypical-hemolytic-uremic-syndrome-caused-by-a%C3%A2-novel%C3%A2-complement-factor-h-mutation%C3%A2
#17
Heeyeon Cho, Yeonhee Lee
Atypical hemolytic uremic syndrome (aHUS) is a rare syndrome characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury that is usually caused by complement dysregulation. Complement factor H (CFH) is a regulator of the complement system produced in the liver, and CFH gene mutations are the most frequent causes of aHUS. To date, the therapeutic options for aHUS with CFH mutations have consisted of plasma infusions, plasma exchange, kidney transplantation, isolated liver transplantation, or combined liver and kidney transplantation...
May 15, 2017: Clinical Nephrology
https://www.readbyqxmd.com/read/28496185/-gardos-channelopathy-a-variant-of-hereditary-stomatocytosis-with-complex-molecular-regulation
#18
Elisa Fermo, Anna Bogdanova, Polina Petkova-Kirova, Anna Zaninoni, Anna Paola Marcello, Asya Makhro, Pascal Hänggi, Laura Hertz, Jens Danielczok, Cristina Vercellati, Nadia Mirra, Alberto Zanella, Agostino Cortelezzi, Wilma Barcellini, Lars Kaestner, Paola Bianchi
The Gardos channel is a Ca(2+) sensitive, K(+) selective channel present in several tissues including RBCs, where it is involved in cell volume regulation. Recently, mutations at two different aminoacid residues in KCNN4 have been reported in patients with hereditary xerocytosis. We identified by whole exome sequencing a new family with two members affected by chronic hemolytic anemia carrying mutation R352H in the KCNN4 gene. No additional mutations in genes encoding for RBCs cytoskeletal, membrane or channel proteins were detected...
May 11, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28484163/progress-in-diagnosis-and-treatment-of-autoimmune-hemolytic-disorders
#19
Toyomi Kamesaki
Discovery of the Coombs' test was an epoch-making event in the history of managing autoimmune hemolytic anemia (AIHA). The Coombs' test allows immune-related hemolytic anemia to be distinguished from nonimmune acquired hemolytic anemia, but also creates a complicated category; Coombs-negative AIHA. To resolve this problem, several trials have been conducted to detect immunoglobulin (Ig) G molecules on erythrocytes (RBC-IgG) that the Coombs' test cannot detect. In Japan, RBC-IgG can be quantitated using a radioimmunoassay, but this procedure is time-consuming and expensive...
2017: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/28482712/heme-mediated-cell-activation-the-inflammatory-puzzle-of-sickle-cell-anemia
#20
Caroline Conceição da Guarda, Rayra Pereira Santiago, Luciana Magalhães Fiuza, Milena Magalhães Aleluia, Júnia Raquel Dutra Ferreira, Camylla Vilas Boas Figueiredo, Setondji Cocou Modeste Alexandre Yahouedehou, Rodrigo Mota de Oliveira, Isa Menezes Lyra, Marilda de Souza Gonçalves
Hemolysis triggers the onset of several clinical manifestations of sickle cell anemia (SCA). During hemolysis, heme, which is derived from hemoglobin (Hb), accumulates due to the inability of detoxification systems to scavenge sufficiently. Heme exerts multiple harmful effects, including leukocyte activation and migration, enhanced adhesion molecule expression by endothelial cells and the production of pro-oxidant molecules. Area covered: In this review, we describe the effects of heme on leukocytes and endothelial cells, as well as the features of vascular endothelial cells related to vaso-occlusion in SCA...
May 17, 2017: Expert Review of Hematology
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