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Hemolytic disease

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https://www.readbyqxmd.com/read/29914582/importance-of-case-age-in-the-purported-association-between-phylogenetics-and-hemolytic-uremic-syndrome-in-escherichia-coli-o157-h7-infections
#1
G A M Tarr, S Shringi, H N Oltean, J Mayer, P Rabinowitz, J Wakefield, P I Tarr, T E Besser, A I Phipps
Escherichia coli O157:H7 is the largest cause of hemolytic uremic syndrome (HUS). Previous studies proposed that HUS risk varies across the E. coli O157:H7 phylogenetic tree (hypervirulent clade 8), but the role of age in the association is unknown. We determined phylogenetic lineage of E. coli O157:H7 isolates from 1160 culture-confirmed E. coli O157:H7 cases reported in Washington State, 2004-2015. Using generalised estimating equations, we tested the association between phylogenetic lineage and HUS. Age was evaluated as an effect modifier...
June 19, 2018: Epidemiology and Infection
https://www.readbyqxmd.com/read/29911962/evans-syndrome-after-successful-immunosuppressant-free-living-donor-liver-transplant
#2
Amr Badawy, Toshimi Kaido, Yoshizawa Atsushi, Shintaro Yagi, Koichiro Hata, Naoko Kamo, Ken Fukumitsu, Hideaki Okajima, Shinji Uemoto
Evans syndrome is an uncommon disease characterized by a combination of autoimmune hemolytic anemia and autoimmune thrombocytopenia concomitantly or sequentially with a positive direct Coombs test in the absence of any underlying known cause. Here, we present a case of an adult patient who underwent living-donor liver transplant that was preceded by bone marrow transplant 20 years earlier from the same HLA identical donor and who received a single-agent immunosuppressive therapy for only 2 months as prophylaxis against graft-versus-host disease...
June 18, 2018: Experimental and Clinical Transplantation
https://www.readbyqxmd.com/read/29910743/squeezing-for-life-properties-of-red-blood-cell-deformability
#3
REVIEW
Rick Huisjes, Anna Bogdanova, Wouter W van Solinge, Raymond M Schiffelers, Lars Kaestner, Richard van Wijk
Deformability is an essential feature of blood cells (RBCs) that enables them to travel through even the smallest capillaries of the human body. Deformability is a function of (i) structural elements of cytoskeletal proteins, (ii) processes controlling intracellular ion and water handling and (iii) membrane surface-to-volume ratio. All these factors may be altered in various forms of hereditary hemolytic anemia, such as sickle cell disease, thalassemia, hereditary spherocytosis and hereditary xerocytosis. Although mutations are known as the primary causes of these congenital anemias, little is known about the resulting secondary processes that affect RBC deformability (such as secondary changes in RBC hydration, membrane protein phosphorylation, and RBC vesiculation)...
2018: Frontiers in Physiology
https://www.readbyqxmd.com/read/29907460/clinical-and-genetic-predictors-of-atypical-hemolytic-uremic-syndrome-phenotype-and-outcome
#4
Franz Schaefer, Gianluigi Ardissino, Gema Ariceta, Fadi Fakhouri, Marie Scully, Nicole Isbel, Åsa Lommelé, Varant Kupelian, Christoph Gasteyger, Larry A Greenbaum, Sally Johnson, Masayo Ogawa, Christoph Licht, Johan Vande Walle, Véronique Frémeaux-Bacchi
Atypical hemolytic uremic syndrome (aHUS) is a rare, genetic, life-threatening disease. The Global aHUS Registry collects real-world data on the natural history of the disease. Here we characterize end-stage renal disease (ESRD)-free survival, the rate of thrombotic microangiopathy, organ involvement and the genetic background of 851 patients in the registry, prior to eculizumab treatment. A sex-specific difference was apparent according to age at initial disease onset as the ratio of males to females was 1...
June 12, 2018: Kidney International
https://www.readbyqxmd.com/read/29905409/l-fabp-and-il-6-as-markers-of-chronic-kidney-damage-in-children-after-hemolytic-uremic-syndrome
#5
Katarzyna Lipiec, Piotr Adamczyk, Elżbieta Świętochowska, Katarzyna Ziora, Maria Szczepańska
BACKGROUND: Hemolytic-uremic syndrome (HUS) is a form of thrombotic microangiopathy, in the course of which some patients may develop chronic kidney disease (CKD). From a clinical point of view, it is important to search for markers that allow for early identification of patients at risk of a poor prognosis. OBJECTIVES: The study evaluated the serum and urine levels of liver-type fatty acid binding protein (L-FABP) and interleukin 6 (IL-6). MATERIAL AND METHODS: The study was conducted in 29 children with a history of HUS...
June 13, 2018: Advances in Clinical and Experimental Medicine: Official Organ Wroclaw Medical University
https://www.readbyqxmd.com/read/29905377/red-blood-cell-adhesion-to-heme-activated-endothelial-cells-reflects-clinical-phenotype-in-sickle-cell-disease
#6
Erdem Kucukal, Anton Ilich, Nigel S Key, Jane A Little, Umut A Gurkan
In sickle cell disease (SCD), 'disease severity' associates with increased RBC adhesion to quiescent endothelium, but the impact on activated endothelium is not known. Increased concentrations of free heme result from intravascular hemolysis in SCD. Heme is essential for aerobic metabolism, and plays an important role in numerous biological processes. Excess free heme induces reactive oxygen species generation and endothelial activation, which are associated with cardiovascular disorders including atherosclerosis, hypertension, and thrombosis...
June 15, 2018: American Journal of Hematology
https://www.readbyqxmd.com/read/29902448/postponing-early-intrauterine-transfusion-with-intravenous-immunoglobulin-treatment-the-petit-study-on-severe-hemolytic-disease-of-the-fetus-and-newborn
#7
Carolien Zwiers, Johanna G van der Bom, Inge L van Kamp, Nan Van Geloven, Enrico Lopriore, John Smoleniec, Roland Devlieger, Pauline E Sim, Marie Anne Ledingham, Eleonor Tiblad, Kenneth J Moise, Karl-Philip Gloning, Mark D Kilby, Timothy G Overton, Ditte S Jørgensen, Katrine V Schou, Bettina Paek, Martin Walker, Emma Parry, Dick Oepkes, Masja de Haas
BACKGROUND: Intrauterine transfusion for severe alloimmunization in pregnancy performed before 20 weeks' gestation is associated with a higher fetal death rate. Intravenous immunoglobulins may prevent hemolysis and could therefore be a non-invasive alternative for early transfusions. OBJECTIVE(S): We evaluated whether maternal treatment with intravenous immunoglobulins defers the development of severe fetal anemia and its consequences in a retrospective cohort to which 12 fetal therapy centers contributed...
June 11, 2018: American Journal of Obstetrics and Gynecology
https://www.readbyqxmd.com/read/29895518/disseminated-cryptococcosis-associated-with-administration-of-eculizumab
#8
Megan Clancy, Ryan McGhan, Jeremy Gitomer, Ana Marie Inocencio, Christina Aldrich, Roberto Iaderosa, Ryan Stevens
PURPOSE: A case report of fatal disseminated cryptococcosis in a patient treated with eculizumab is presented along with a review of literature suggesting a possible etiologic mechanism. SUMMARY: A 23-year-old man with a history of minimal change nephrotic syndrome was hospitalized for acute kidney injury and abdominal pain and swelling. He was found to have disseminated pneumococcal disease, including peritonitis, bacteremia, and pulmonic endocarditis. The patient developed evidence of microangiopathic hemolytic anemia, leading to a diagnosis of atypical hemolytic uremic syndrome, and was started on eculizumab...
June 12, 2018: American Journal of Health-system Pharmacy: AJHP
https://www.readbyqxmd.com/read/29893502/biocomputing-for-portable-resettable-and-quantitative-point-of-care-diagnostics-making-glucose-meter-a-logic-gate-responsive-device-to-many-targets-in-clinical-care
#9
JingJing Zhang, Yi Lu
While biocomputing is recognized to provide intelligent solution to complex biosensing projects, it remains challenging to transform biomolecular logic gates into a convenient, portable, resettable and quantitative sensing system for point-of-care (POC) diagnostics in a low-resource setting. To overcome these limitations, we herein report the first design and demonstration of biocomputing on personal glucose meters (PGM) by utilizing glucose and nicotinamide adenine dinucleotide as signal outputs, DNAzyme and protein enzymes as building blocks and demonstrating a general platform for installing logic-gate responses (YES, NOT, INHIBIT and OR) to a variety of biological species, such as cations (sodium), anions (citrate), organic metabolites (ADP, ATP) and enzymes (pyruvate kinase, alkaline phosphatase, alcohol dehydrogenases)...
June 12, 2018: Angewandte Chemie
https://www.readbyqxmd.com/read/29889319/pediatric-thrombotic-thrombocytopenic-purpura
#10
REVIEW
Bérangère S Joly, Paul Coppo, Agnès Veyradier
Child-onset thrombotic thrombocytopenic purpura (TTP) is a rare entity of thrombotic microangiopathy (TMA). The pathophysiology of the disease is based on a severe functional deficiency of ADAMTS13 (activity <10%), the specific von Willebrand factor (VWF)-cleavage protease. This deficiency may be either acquired (associated anti-ADAMTS13 autoantibodies) or congenital (resulting from biallelic mutations of ADAMTS13 gene). ADAMTS13 deficiency is responsible for the accumulation of high molecular weight multimers of VWF and the formation of platelet thrombi in the microcirculation...
June 11, 2018: European Journal of Haematology
https://www.readbyqxmd.com/read/29888403/genotype-phenotype-correlations-of-low-frequency-variants-in-the-complement-system-in-renal-disease-and-age-related-macular-degeneration
#11
M J Geerlings, E B Volokhina, E K de Jong, N van de Kar, M Pauper, C B Hoyng, L P van den Heuvel, A I den Hollander
Genetic alterations in the complement system have been linked to a variety of diseases, including atypical hemolytic uremic syndrome (aHUS), C3 glomerulopathy (C3G), and age-related macular degeneration (AMD). We performed sequence analysis of the complement genes CFH, CFI, and C3 in 866 aHUS/C3G and 697 AMD patients. In total we identified 505 low frequency alleles, representing 121 unique variants, of which 51 are novel. CFH contained the largest number of unique low frequency variants (n=64; 53%), followed by C3 (n=32; 26%) and CFI (n=25; 21%)...
June 11, 2018: Clinical Genetics
https://www.readbyqxmd.com/read/29887684/b-l-basal-ganglia-lesions-in-a-child-leading-to-a-diagnosis-of-glucose-6-phosphate-dehydrogenase-deficiency
#12
Nidhi Prabhakar, Chirag K Ahuja, Niranjan Khandelwal
Bilateral basal ganglia lesions are a common non-specific finding seen in many diseases. One of the differential diagnoses for it, in a child, is kernicterus occurring due to hyperbilirubinemia. Glucose-6-phosphate dehydrogenase (G6PD) deficiency is a common cause of severe hyperbilirubinemia. A 1-year old child presented to the hospital with history of generalized dystonia in the previous 3 days. MRI showed evidence of symmetrical lesions in bilateral globus pallidus, which were hyperintense on T2/FLAIR and isointense on T1...
April 2018: Annals of Neurosciences
https://www.readbyqxmd.com/read/29885944/cold-agglutinin-disease-complicating-management-of-aortic-dissection
#13
James Bras, Kelsey Uminski, Arjuna Ponnampalam
BACKGROUND: Cold agglutinin disease is characterized by acrocyanosis, hemolytic anemia, and occasionally, frank hemoglobinuria. Although cold agglutinins are commonly detected, they are rarely clinically significant due to subphysiologic temperatures at which agglutination occurs. Cardiovascular surgical procedures requiring hypothermia present a unique challenge for these patients, requiring modification of the conduct of cardiopulmonary bypass and cardioplegia. CASE REPORT: Herein we report a case of a patient with a prior history of symptomatic cold agglutinin disease and type A aortic dissection, presenting with dilation of his known diseased ascending aorta, requiring semi-urgent repair...
April 2018: Transfusion and Apheresis Science
https://www.readbyqxmd.com/read/29884138/the-complex-treatment-including-rituximab-in-the-management-of-catastrophic-antiphospholid-syndrome-with-renal-involvement
#14
Aleksandra Rymarz, Stanisław Niemczyk
BACKGROUND: Catastrophic antiphospholipid syndrome (CAPS) is a rare, life-threatening form of antiphospholipid syndrome (APS) involving many organs and leading to their insufficiency. The pathogenesis of CAPS is associated with the presence of antiphospholipid antibodies (aPL). Typical therapy includes anticoagulation, glucocorticoids, therapeutic plasma exchanges and/or intravenous immunoglobulin. Despite this aggressive treatment, the mortality rate of 37% is still high. Novel therapeutic agents are required...
June 8, 2018: BMC Nephrology
https://www.readbyqxmd.com/read/29883245/fatal-meningococcemia-due-to-neisseria-meningitidis-serogroup-y-in-a-vaccinated-child-receiving-eculizumab
#15
Meltem Polat, Selçuk Yüksel, Nuriye Ünal Şahin
Life-threatening and fatal meningococcal infections have occurred in patients treated with eculizumab. Herein we describe an 11-year-old boy with atypical hemolytic uremic syndrome treated with eculizumab who developed fatal meningococcemia due to Neisseria meningitidis serogroup Y 16 months after receiving two doses of meningococcal conjugate vaccine (MenACWY-D) while on oral penicillin prophylaxis. Clinicians need to maintain a high index of suspicion for invasive meningococcal disease in patients taking eculizumab treatment, regardless of meningococcal vaccination or antimicrobial prophylaxis status...
June 8, 2018: Human Vaccines & Immunotherapeutics
https://www.readbyqxmd.com/read/29881224/infectious-flexor-hand-tenosynovitis-state-of-knowledge-a-study-of-120-cases
#16
REVIEW
William Mamane, Stenley Lippmann, Dan Israel, Reeta Ramdhian-Wihlm, Michael Temam, Virginie Mas, Jérome Pierrart, Emmanuel H Masmejean
Introduction: Since Kanavel in 1905, knowledge of phlegmon of flexor tendon sheaths of the fingers have evolved over the twentieth century. This serious infection is 20% of infections of the hand and may have adverse consequences for the function of the finger and even beyond, of the hand. Amputation is always a risk. Frequently face this type of infection, we conducted a retrospective study and made an inventory of knowledge in order to consolidate and improve the overall care. Materials & Methods: The study was retrospective and cross, focused on 120 patients operated on at Hand Surgery Unit, during 4 years...
June 2018: Journal of Orthopaedics
https://www.readbyqxmd.com/read/29880417/acute-neonatal-bilirubin-encephalopathy-in-the-state-of-utah-2009-2018
#17
Robert D Christensen, Archana M Agarwal, Tracy I George, Vinod K Bhutani, Hassan M Yaish
Herein we report a case series of seven newborn infants, all apparently well at birth, who in the period since 2009 were cared for in the State of Utah with acute bilirubin encephalopathy (ABE). This report summarizes our attempts to define common features of these seven through a state-wide voluntary registry, as a step toward devising new means of preventing such cases in the future. In previous reports of ABE, many of the affected neonates had no clearly defined explanation for their progressive hyperbilirubinemia...
May 23, 2018: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/29874792/isolation-of-marine-bacillus-sp-with-antagonistic-and-organic-substances-degrading-activities-and-its-potential-application-as-a-fish-probiotic
#18
Shuxin Zhou, Yu Xia, Chongmiao Zhu, Weihua Chu
We report on the isolation and characterization of an acid- and bile-tolerant bacterial strain, Bacillus sp. YB1701 with antibacterial and quorum-quenching activity. Strain YB1701 was isolated from coastal sediment samples and characterized by biochemical tests and 16S rRNA sequencing. In vitro study indicated that strain YB1701 can survive at pH 2.0 for up to 3 h and tolerate bile up to 2.0% concentration even after 12 h of exposure. Strain YB1701 showed antimicrobial activity against fish pathogens Aeromonas hydrophila and Vibrio parahemolyticus using an agar well diffusion assay...
June 5, 2018: Marine Drugs
https://www.readbyqxmd.com/read/29874251/a-new-biosensor-for-noninvasive-determination-of-fetal-rhd-status-in-maternal-blood-of-rhd-negative-pregnant-women
#19
Ebru Dündar Yenilmez, Umut Kökbaş, Kezban Kartlaşmış, Levent Kayrın, Abdullah Tuli
Prenatal detection of the fetal RHD status can be useful in the management of RhD incompatibility to identify fetuses at risk of hemolytic disease. Hemolytic disease causes morbidity and mortality of the fetus in the neonatal period. The routine use of antenatal and postnatal anti-D prophylaxis has reduced the incidence of hemolytic disease of the fetus and newborn. This study describe the detection of fetal RhD antigens in blood of RhD negative pregnant women using a nanopolymer coated electrochemical biosensor for medical diagnosis...
2018: PloS One
https://www.readbyqxmd.com/read/29871018/management-of-hcv-related-liver-disease-in-hemophilia-and-thalassemia
#20
Maria Grazia Rumi, Vito Di Marco, Massimo Colombo
Chronic infection with the hepatitis C virus (HCV) has long been the dominant complication of substitution therapy in patients with inherited blood disorders and the cause of anticipated death due to end-stage liver disease. In hemophilia, transmission of HCV with clotting factors concentrates started to be curbed in the mid-1980s following the adoption of procedures of virus inactivation of concentrates based on heat, whereas in the 1990s treatment of HCV infection with interferon monotherapy was attempted, however, with little success...
May 2018: Seminars in Liver Disease
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