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Hemolytic disease

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https://www.readbyqxmd.com/read/28821363/living-donor-kidney-transplantation-in-atypical-hemolytic-uremic-syndrome-a-case-series
#1
Caroline Duineveld, Jacobien C Verhave, Stefan P Berger, Nicole C A J van de Kar, Jack F M Wetzels
BACKGROUND: The development of complement inhibitors has greatly improved the outcome of patients with atypical hemolytic uremic syndrome (aHUS), making kidney transplantation a more feasible option. Although prophylactic eculizumab therapy may prevent recurrent disease after transplantation, its necessity for all transplant recipients is debated. STUDY DESIGN: A case series. SETTING & PARTICIPANTS: Patients with aHUS who underwent living donor kidney transplantation after 2011 at 2 university centers, prospectively followed up with a protocol of eculizumab therapy limited to only recipients with documented posttransplantation recurrent thrombotic microangiopathy...
August 16, 2017: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
https://www.readbyqxmd.com/read/28818402/association-of-hemolysis-with-high-dose-intravenous-immunoglobulin-therapy-in-pediatric-patients-an-open-label-prospective-trial
#2
Alkim Oden Akman, Fatma Karaca Kara, Tulin Koksal, Bahar Cuhaci Cakir, Cuneyt Karagol, Tulin Sayli
Immunoglobulin therapy can be used to treat a wide variety of diseases. However, intravenous immunoglobin products can cause several adverse reactions, including hemolysis. The objective of this study was to determine the extent of anemia and hemolysis after high dose intravenous immunoglobin (2g/kg) and its relationship to the ABO blood type system and hemolytic anemia blood parameters in pediatric patients. Incidence of 'Intravenous immunoglobulin related hemolysis' was %19 (6/31) after high dose intravenous immunoglobulin therapy...
August 1, 2017: Transfusion and Apheresis Science
https://www.readbyqxmd.com/read/28818095/frequency-distribution-and-immunologic-nature-of-infusion-reactions-in-subjects-receiving-pegloticase-for-chronic-refractory-gout
#3
Leonard H Calabrese, Arthur Kavanaugh, Anthony E Yeo, Peter E Lipsky
BACKGROUND: To assess frequency and distribution of infusion reactions (IRs) in responders and nonresponders in randomized clinical trials (RCTs) of intravenous pegloticase and the utility of the National Institute of Allergy and Infectious Disease/Food and Allergy and Anaphylaxis Network (NIAID/FAAN) criteria for identifying anaphylaxis in subjects experiencing IRs. METHODS: IRs from two RCTs of pegloticase were evaluated and categorized as anaphylaxis, hypersensitivity, or other...
August 17, 2017: Arthritis Research & Therapy
https://www.readbyqxmd.com/read/28817704/analysis-of-multidrug-resistant-group-b-streptococci-with-reduced-penicillin-susceptibility-forming-small-less-hemolytic-colonies
#4
Hirotsugu Banno, Kouji Kimura, Yosuke Tanaka, Tsuyoshi Sekizuka, Makoto Kuroda, Wanchun Jin, Jun-Ichi Wachino, Keiko Yamada, Keigo Shibayama, Yoshichika Arakawa
Group B streptococci (GBS; Streptococcus agalactiae) are the leading cause of neonatal invasive diseases and are also important pathogens for elderly adults. Until now, nearly all GBS with reduced penicillin susceptibility (PRGBS) have shown β-hemolytic activity and grow on sheep blood agar. However, we have previously reported three PRGBS clinical isolates harboring a CylK deletion that form small less hemolytic colonies. In this study, we examined the causes of small, less hemolytic colony formation in these clinical isolates...
2017: PloS One
https://www.readbyqxmd.com/read/28815695/a-distinctive-histidine-residue-is-essential-for-in-vivo-glycation-inactivation-of-human-cd59-transgenically-expressed-in-mice-erythrocytes-implications-for-human-diabetes-complications
#5
Rupam Sahoo, Pamela Ghosh, Michael Chorev, Jose A Halperin
Clinical and experimental evidences support a link between the complement system and the pathogenesis of diabetes complications. CD59, an extracellular cell membrane-anchored protein, inhibits formation of the membrane attack complex (MAC), the main effector of complement-mediated tissue damage. This complement regulatory activity of human CD59 (hCD59) is inhibited by hyperglycemia-induced ε-amino glycation of Lys(41) . Biochemical and structural analyses of glycated proteins with known three-dimensional structure revealed that glycation of ε-amino lysyl residues occurs predominantly at "glycation motives" that include lysyl/lysyl pairs or proximity of a histidyl residue, in which the imidazolyl moiety is ≈ 5Å from the ε-amino group...
August 17, 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/28811695/pai-1-study-in-thalassemia-major-patients-receiving-multiple-blood-transfusion
#6
Ashwini Kumar, H S Batra, Mithu Banerjee, S Bandyopadhyay, T K Saha, Pratibha Misra, Vivek Ambade
Thalassemia is a congenital hemolytic disease which is treated by repeated blood transfusion. Chronic iron overload is currently considered to be the primary cause of mortality in β-thalassemia, mainly due to the induction of left-sided cardiac failure. Iron overload results from a number of mechanisms associated with the disease itself. In addition to chronic iron overload thalassemic patients are more prone for procoagulant status which in turn lead to clinical thrombotic events. The hypercoagulable state in thalassemia is due to multiple elements, a combination of which is often the drive behind a clinical thromboembolic events...
July 2017: Indian Journal of Clinical Biochemistry: IJCB
https://www.readbyqxmd.com/read/28811589/patients-with-igg1-anti-red-blood-cell-autoantibodies-show-aberrant-fc-glycosylation
#7
Myrthe E Sonneveld, Masja de Haas, Carolien Koeleman, Noortje de Haan, Sacha S Zeerleder, Peter C Ligthart, Manfred Wuhrer, C Ellen van der Schoot, Gestur Vidarsson
Autoimmune hemolytic anemia (AIHA) is a potentially severe disease in which red blood cells (RBC) are destroyed by IgG anti-RBC autoantibodies which can lead to hemolysis. We recently found IgG Fc-glycosylation towards platelet and RBC alloantigens to be skewed towards decreased fucosylation, increased galactosylation and sialylation. The lowered core-fucosylation increases the affinity of the pathogenic alloantibodies to FcγRIIIa/b, and hence RBC destruction. It is known that in autoimmune diseases plasma IgG1 galactosylation and sialylation are lowered, but Fc-glycosylation of RBC-specific autoantibodies has never been thoroughly analyzed...
August 15, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28811305/red-blood-cells-in-thrombosis
#8
James R Byrnes, Alisa S Wolberg
Red blood cells (RBCs) have historically been considered passive bystanders in thrombosis. However, clinical and epidemiological studies have associated quantitative and qualitative abnormalities in RBCs, including altered hematocrit, sickle cell disease, thalassemia, hemolytic anemias, and malaria, with both arterial and venous thrombosis. A growing body of mechanistic studies suggests RBCs can promote thrombus formation and enhance thrombus stability. These findings suggest RBCs may contribute to thrombosis pathophysiology and reveal potential strategies for therapeutically targeting RBCs to reduce thrombosis...
August 15, 2017: Blood
https://www.readbyqxmd.com/read/28810311/-retrospective-analysis-of-13-cases-of-nocardiosis
#9
H Q Yang, H Z Shi, Z H Tong
Objective: To evaluate the clinical features, chest radiological manifestations, microbiological examination and treatments of nocardial disease. Methods: A retrospective study was conducted to analyze the data of patients with nocardial infection admitted to Beijing Chaoyang Hospital from January 2010 to January 2016. Results: The 13 patients, 6 males and 7 females, aged (51±17) years. Twelve cases were diagnosed with pulmonary nocardiosis, and 1 with disseminated nocardial infection. Most of these patients had complications: autoimmune diseases in 3 (2 with autoimmune hemolytic anemia and 1 with systemic lupus erythematosus), and bronchiectasis in 6 patients...
August 12, 2017: Chinese Journal of Tuberculosis and Respiratory Diseases
https://www.readbyqxmd.com/read/28804921/serum-thymidine-kinase-1-canine-c-reactive-protein-haptoglobin-and-vitamin-d-concentrations-in-dogs-with-immune-mediated-hemolytic-anemia-thrombocytopenia-and-polyarthropathy
#10
M Grobman, H Outi, H Rindt, C Reinero
BACKGROUND: Relapses of immune-mediated hemolytic anemia (IMHA), thrombocytopenia (ITP), or polyarthropathy (IMPA) occur despite normal hematologic and cytologic parameters. Thymidine kinase 1 (TK1), canine C-reactive protein (c-CRP), haptoglobin (HPT), and 25-Hydroxyvitamin-D (25(OH)D) might be adjunct to current monitoring strategies. HYPOTHESIS/OBJECTIVES: Compare serum concentrations of TK1, c-CRP, HPT, and 25(OH)D in dogs with well- and poorly controlled primary IMHA, ITP, or IMPA...
August 14, 2017: Journal of Veterinary Internal Medicine
https://www.readbyqxmd.com/read/28801959/outcomes-of-patients-with-atypical-hemolytic-uremic-syndrome-with-native-and-transplanted-kidneys-treated-with-eculizumab-a-pooled-post-hoc-analysis
#11
Christophe M Legendre, Josep M Campistol, Thorsten Feldkamp, Giuseppe Remuzzi, John F Kincaid, Åsa Lommelé, Jimmy Wang, Laurent E Weekers, Neil S Sheerin
Atypical hemolytic uremic syndrome (aHUS) often leads to end-stage renal disease (ESRD) and kidney transplantation; graft loss rates are high due to disease recurrence. A post hoc analysis of four prospective clinical trials in aHUS was performed to evaluate eculizumab, a terminal complement inhibitor, in patients with native or transplanted kidneys. The trials included 26-week treatment and extension periods. Dialysis, transplant, and graft loss were evaluated. Study endpoints included complete thrombotic microangiopathy (TMA) response, TMA event-free status, hematologic and renal parameters, and adverse events...
August 12, 2017: Transplant International: Official Journal of the European Society for Organ Transplantation
https://www.readbyqxmd.com/read/28796682/a-guide-to-terminology-for-rh-immunoprophylaxis
#12
S Gerald Sandler, John T Queenan
Rh immunoprophylaxis for Rh-negative women requires an understanding of terminology used for Rh blood typing laboratory reports. The pathophysiology of Rh hemolytic disease of the fetus and newborn was elucidated by studies in rhesus monkeys. Subsequent studies revealed that the human blood group antigen responsible for Rh hemolytic disease of the newborn (D antigen) is related to, but different from, the rhesus monkey antigen. Weak expression of the D antigen on red cells, originally termed D, is currently reported by laboratories as a "serologic weak D phenotype," which can be further defined by RHD genotyping to be either an RHD weak D type or a partial D phenotype...
August 4, 2017: Obstetrics and Gynecology
https://www.readbyqxmd.com/read/28794069/phase-i-study-of-the-anti-cd22-immunotoxin-moxetumomab-pasudotox-for-childhood-acute-lymphoblastic-leukemia
#13
Alan S Wayne, Nirali N Shah, Deepa Bhojwani, Lewis B Silverman, James A Whitlock, Maryalice Stetler-Stevenson, Weili Sun, Meina Liang, Jie Yang, Robert J Kreitman, Mark C Lanasa, Ira Pastan
Novel therapies are needed to overcome chemotherapy resistance for children with relapsed/refractory acute lymphoblastic leukemia (ALL). Moxetumomab pasudotox is a recombinant anti-CD22 immunotoxin. A multicenter, phase I study was conducted to determine the maximum tolerated cumulative dose (MTCD) and evaluate safety, activity, pharmacokinetics, and immunogenicity of moxetumomab pasudotox in children, adolescents, and young adults with ALL (n=55). Moxetumomab pasudotox was administered as a 30-minute intravenous infusion at doses of 5 to 50 µg/kg every other day (QOD) for six (Cohorts A and B) or 10 (Cohort C) doses, on 21-day cycles...
August 9, 2017: Blood
https://www.readbyqxmd.com/read/28791912/coinheritance-of-high-oxygen-affinity-hb-helsinki-hbb-c-248a-t-%C3%AE-82-ef6-lys%C3%A2-met-with-hb-h-disease
#14
Shir-Ying Lee, Jia-Hui Goh, Karen M L Tan, Te-Chih Liu
Hb Helsinki [HBB: c.248A>T; β82(EF6)Lys→Met] is a high oxygen affinity hemoglobin (Hb) causing polycythemia, whereas Hb H (β4) disease causes mild to severe chronic hemolytic anemia. The clinical characteristics, gel electrophoresis, capillary electrophoresis (CE) and molecular genotyping of a case of Hb Helsinki coinherited with Hb H disease in an ethnic Malay is described, illustrating the interaction between the β-globin variant and coinheritance of three α gene deletions. The proband was asymptomatic, exhibited microcytosis and a normal with Hb value...
August 9, 2017: Hemoglobin
https://www.readbyqxmd.com/read/28791286/autoimmune-thrombotic-thrombocytopenic-purpura-two-rare-cases-associated-with-juvenile-idiopathic-arthritis-and-multiple-sclerosis
#15
Despoina Dimopoulou, Athina Dimosiari, Eudokia Mandala, Theodoros Dimitroulas, Alaxandros Garyfallos
Secondary thrombotic microangiopathies are associated with several underlying conditions, with most of them being resolved after the treatment of background disease. Thrombotic thrombocytopenic purpura (TTP) is a rare microangiopathy presenting with anemia, thrombocytopenia, and neurological deficits, occurring most often in various autoimmune diseases due to inhibition of ADAMTS13 by autoantibodies, as well as in pregnant women with or without an autoimmune substrate. In this article, we report two newly diagnosed TTP cases, who have not been published so far...
2017: Frontiers in Medicine
https://www.readbyqxmd.com/read/28783254/characterization-of-plasma-labile-heme-in-hemolytic-conditions
#16
Zélia Gouveia, Ana Rita Carlos, Xiaojing Yuan, Frederico Aires-da-Silva, Roland Stocker, Ghassan J Maghzal, Sónia S Leal, Cláudio M Gomes, Smilja Todorovic, Olga Iranzo, Susana Ramos, Ana Catarina Santos, Iqbal Hamza, João Gonçalves, Miguel P Soares
Extracellular hemoglobin (Hb), a byproduct of hemolysis, can release its prosthetic heme groups upon oxidation. This produces metabolically active heme that is exchangeable between acceptor proteins, macromolecules and low molecular weight ligands, termed here labile heme. As it accumulates in plasma labile heme acts in a pro-oxidant manner and regulates cellular metabolism while exerting pro-inflammatory and cytotoxic effects that foster the pathogenesis of hemolytic diseases. Here we developed and characterized a panel of heme-specific single domain antibodies (sdAbs) that together with a cellular-based heme reporter assay, allow for quantification and characterization of labile heme in plasma during hemolytic conditions...
August 7, 2017: FEBS Journal
https://www.readbyqxmd.com/read/28780243/clinical-and-molecular-characteristics-of-infective-%C3%AE-hemolytic-streptococcal-endocarditis
#17
Oddvar Oppegaard, Haima Mylvaganam, Steinar Skrede, Stina Jordal, Marte Glambek, Bård Reiakvam Kittang
Streptococcus pyogenes (S. pyogenes) and Streptococcus dysgalactiae subspecies equisimilis (SDSE) cause considerable morbidity and mortality, and show similarities in disease manifestations and pathogenic mechanisms. Their involvement in infective endocarditis, however, has not been well described. Invasive S. pyogenes and SDSE infections in Health Region Bergen, Norway, in the period 1999-2013 were reviewed, and sixteen cases of endocarditis were identified. The median duration of symptoms was 2.5days, the frequency of embolic events 50%, 38% received valve replacement and the 30-day mortality was 25%...
June 30, 2017: Diagnostic Microbiology and Infectious Disease
https://www.readbyqxmd.com/read/28780041/quinine-induced-thrombotic-microangiopathy-a-report-of%C3%A2-19%C3%A2-patients
#18
Evaren E Page, Dustin J Little, Sara K Vesely, James N George
BACKGROUND: Quinine can cause diverse and severe immune-mediated adverse reactions, including thrombotic microangiopathy (TMA). Our objective was to describe the presenting features and long-term outcomes of patients with quinine-induced TMA. STUDY DESIGN: A case series of 19 patients with quinine-induced TMA treated with plasma exchange. SETTING & PARTICIPANTS: Patients with quinine-induced TMA initially suspected of having thrombotic thrombocytopenic purpura (TTP) were identified among patients enrolled in the Oklahoma TTP-Hemolytic Uremic Syndrome Registry...
August 3, 2017: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
https://www.readbyqxmd.com/read/28777391/design-and-numerical-evaluation-of-an-axial-partial-assist-blood-pump-for-chinese-and-other-heart-failure-patients
#19
Guang-Mao Liu, Dong-Hai Jin, Jian-Ye Zhou, Xi-Hang Jiang, Han-Song Sun, Yan Zhang, Hai-Bo Chen, Sheng-Shou Hu, Xing-Min Gui
A fully implantable axial left ventricular assist device LAP31 was developed for Chinese or other heart failure patients who need partial support. Based on the 5-Lpm total cardiac blood output of Chinese without heart failure disease, the design point of LAP31 was set to a flow rate of 3 Lpm with 100-mmHg pressure head. To achieve the required pressure head and good hemolytic performance, a structure that includes a spindly rotor hub and a diffuser with splitter and cantilevered main blades was developed. Computational fluid dynamics (CFD) was used to analyze the hydraulic and hemodynamic performance of LAP31...
August 2, 2017: International Journal of Artificial Organs
https://www.readbyqxmd.com/read/28770911/targeting-%C3%AE-cys93-in-hemoglobin-s-with-an-antisickling-agent-possessing-dual-allosteric-and-antioxidant-effects
#20
Tigist Kassa, Michael Brad Strader, Akito Nakagawa, Warren M Zapol, Abdu I Alayash
Sickle cell disease (SCD) is an inherited blood disorder caused by a β globin gene mutation of hemoglobin (HbS). The polymerization of deoxyHbS and its subsequent aggregation (into long fibers) is the primary molecular event which leads to red blood cell (RBC) sickling and ultimately hemolytic anemia. We have recently suggested that HbS oxidative toxicity may also contribute to SCD pathophysiology due to its defective pseudoperoxidase activity. As a consequence, a persistently higher oxidized ferryl heme is formed which irreversibly oxidizes "hotspot" residues (particularly βCys93) causing protein unfolding and subsequent heme loss...
August 3, 2017: Metallomics: Integrated Biometal Science
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