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Hemolytic disease

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https://www.readbyqxmd.com/read/27913955/polysaccharides-from-arnebia-euchroma-ameliorated-endotoxic-fever-and-acute-lung-injury-in-rats-through-inhibiting-complement-system
#1
Ying-Ye Ou, Yun Jiang, Hong Li, Yun-Yi Zhang, Yan Lu, Dao-Feng Chen
Arnebiaeuchroma (Royle) Johnst (Ruanzicao) is a traditional Chinese herbal medicine (TCM). It is extensively used in China and other countries for treatment of inflammatory diseases. It is known that hyper-activated complement system involves in the fever and acute lung injury (ALI) in rats. In our preliminary studies, anti-complementary activity of crude Arnebiaeuchroma polysaccharides (CAEP) had been demonstrated in vitro. This study aimed to investigate the role and mechanism of crude Arnebiaeuchroma polysaccharides (CAEP) using two animal models, which relate with inappropriate activation of complement system...
December 3, 2016: Inflammation
https://www.readbyqxmd.com/read/27913548/warm-antibody-autoimmune-hemolytic-anemia
#2
Theodosia A Kalfa
Autoimmune hemolytic anemia (AIHA) is a rare and heterogeneous disease that affects 1 to 3/100 000 patients per year. AIHA caused by warm autoantibodies (w-AIHA), ie, antibodies that react with their antigens on the red blood cell optimally at 37°C, is the most common type, comprising ∼70% to 80% of all adult cases and ∼50% of pediatric cases. About half of the w-AIHA cases are called primary because no specific etiology can be found, whereas the rest are secondary to other recognizable underlying disorders...
December 2, 2016: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/27913516/immunoregulatory-networks-in-sickle-cell-alloimmunization
#3
Karina Yazdanbakhsh
Red blood cell (RBC) transfusions are critical for treatment and prevention of complications of sickle cell disease (SCD), and most SCD patients will receive 1 or more transfusions by age 20. However, SCD alloimmunization remains a serious complication of transfusions that can lead to life-threatening acute and delayed transfusion reactions. Alloimmunization rates are higher in SCD patients most likely due to RBC antigenic differences between largely white donors vs mainly African-American recipients and frequency of transfusions...
December 2, 2016: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/27913484/cold-agglutinin-disease
#4
Sigbjørn Berentsen
Primary chronic cold agglutinin disease (CAD) is a well-defined clinicopathologic entity in which a specific, clonal lymphoproliferative B-cell bone marrow disorder results in autoimmune hemolytic anemia. The immune hemolysis is entirely complement-dependent, predominantly mediated by activation of the classical pathway and phagocytosis of erythrocytes opsonized with complement protein C3b. Typical clinical features in CAD have diagnostic and therapeutic implications. Pharmacologic treatment should be offered to patients with symptom-producing anemia or disabling circulatory symptoms...
December 2, 2016: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/27913483/atypical-hemolytic-uremic-syndrome
#5
Vahid Afshar-Kharghan
Atypical hemolytic uremic syndrome (aHUS) is a thrombotic microangiopathy (TMA) that affects multiple organs and the kidneys in particular. aHUS can be sporadic or familial and is most commonly caused by dysregulation of the alternative complement pathway. The initial attack of aHUS can occur at any age, and is associated with a high rate of progression to end stage renal disease. Many aHUS patients relapse in the native or transplanted kidneys, and require close monitoring and long-term management. Availability of anticomplement therapy has revolutionized the management of aHUS, and can change the natural course of aHUS by inducing hematologic remission, improving or stabilizing kidney functions, and preventing graft failure...
December 2, 2016: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/27913482/update-on-the-diagnosis-and-management-of-paroxysmal-nocturnal-hemoglobinuria
#6
Charles J Parker
Once suspected, the diagnosis of paroxysmal nocturnal hemoglobinuria (PNH) is straightforward when flow cytometric analysis of the peripheral blood reveals a population of glycosyl phosphatidylinositol anchor protein-deficient cells. But PNH is clinically heterogeneous, with some patients having a disease process characterized by florid intravascular, complement-mediated hemolysis, whereas in others, bone marrow failure dominates the clinical picture with modest or even no evidence of hemolysis observed. The clinical heterogeneity is due to the close, though incompletely understood, relationship between PNH and immune-mediated bone marrow failure, and that PNH is an acquired, nonmalignant clonal disease of the hematopoietic stem cells...
December 2, 2016: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/27906107/previously-undiagnosed-hereditary-spherocytosis-in-a-patient-with-jaundice-and-pyelonephritis-a-case-report
#7
Yuki Tateno, Ryoji Suzuki, Yukihiro Kitamura
BACKGROUND: Hereditary spherocytosis is autosomal dominant inherited extravascular hemolytic disorder and is the commonest cause of inherited hemolysis in northern Europe and the United States. The classical clinical features of hereditary spherocytosis are anemia, jaundice, and splenomegaly. However, all of these classical features are not always revealed in the case of mild hemolysis or when hemolysis is well compensated. Patients with hereditary spherocytosis may remain undiagnosed for years if their hemolysis is mild...
December 1, 2016: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/27904864/nephrotic-range-proteinuria-and-peripheral-edema-in-a-child-not-only-idiopathic-nephrotic-syndrome
#8
Valentina Dolcemascolo, Marina Vivarelli, Manuela Colucci, Francesca Diomedi-Camassei, Rossella Piras, Marta Alberti, Francesco Emma
Hemolytic uremic syndrome (HUS) is defined by the simultaneous occurrence of hemolytic anemia, thrombocytopenia, and acute kidney injury due to thrombotic microangiopathy (TMA) mainly occurring in renal and cerebral microvessels. Although the most common cause of HUS in children is Shiga toxin-producing Escherichia coli, atypical forms in which Shiga toxin is not the trigger may occur. Research over the last few years has shown that complement dysregulation secondary to mutations of genes coding for proteins involved in the regulation of the alternative pathway of complement account for most forms of atypical HUS (aHUS)...
September 2016: Case Reports in Nephrology and Dialysis
https://www.readbyqxmd.com/read/27902998/autoimmune-cytopenias-diagnosis-management
#9
Christian P Nixon, Joseph D Sweeney
The autoimmune cytopenias are a related group of disorders in which differentiated hematopoietic cells are destroyed by the immune system. Single lineage disease is characterized by the production of autoantibodies against red cells (autoimmune hemolytic anemia [AIHA]), platelets (autoimmune thrombocytopenia [ITP]) and neutrophils (autoimmune neutropenia [AIN]) whereas multilineage disease may include various combinations of these conditions. Central to the genesis of this disease is the breakdown of central and/or peripheral tolerance, and the subsequent production of autoantibodies by both tissue and circulating self-reactive B lymphocytes with support from T helper lymphocytes...
December 1, 2016: Rhode Island Medical Journal
https://www.readbyqxmd.com/read/27889586/surfactant-effect-on-the-physicochemical-characteristics-of-cationic-solid-lipid-nanoparticles
#10
Chiara Botto, Nicolò Mauro, Erika Amore, Elisabetta Martorana, Gaetano Giammona, Maria Luisa Bondì
Solid lipid nanoparticles (SLNs) may be considered as a new approach for therapeutics for many diseases. In addition to drug delivery, their use as non-viral vectors for gene delivery can be obtained by including cationic lipids, which provide a positive surface potential that favors binding to the nucleic acids as DNA, siRNA, miRNA, etc. In fact, the addition of cationic surfactants is indispensable for obtaining nanoparticles with surface positive charge. In this study, three different cationic lipids (dioctadecyl dimethyl ammonium bromide, cetyltrimethyl ammonium bromide, cetylpyridinium chloride) and Brij 76 as nonionic surfactant were employed to formulate Precirol ATO 5 based cSLN using pEGFP-LUC as model plasmid...
November 23, 2016: International Journal of Pharmaceutics
https://www.readbyqxmd.com/read/27883972/rheumatic-heart-disease-in-a-developing-country-incidence-and-trend-monastir-tunisia-2000-2013
#11
Asma Sriha Belguith, Afifa Koubaa Abdelkafi, Sana El Mhamdi, Manel Ben Fredj, Hela Abroug, Arwa Ben Salah, Inès Bouanene, Fahima Hassine, Amal Amara, Sana Bhiri, Abdelkarim Derbel, Habib Gamra, Faouzi Maatouk, Mohamed Soussi Soltani
BACKGROUND: The penicillin therapy of β hemolytic streptococcal pharyngitis has aided in the decrease of rheumatic heart disease (RHD) in developing countries. Tunisia is an endemic area, however, and incidence of RHD is weakly documented. We aimed at establishing the standardized incidence rate (SIR) of RHD in Monastir governorate and at determining RHD prevalence among hospitalized patients in two cardiology departments. METHODS: From the regional register of Monastir Hospital morbidity, we have selected newly diagnosed patients with RHD, residents of Monastir, and hospitalized to the 2 cardiology departments between 2000 and 2013 (2001 not included)...
November 14, 2016: International Journal of Cardiology
https://www.readbyqxmd.com/read/27880834/overexpressed-proteins-in-hypervirulent-clade-8-and-clade-6-strains-of-escherichia-coli-o157-h7-compared-to-e-coli-o157-h7-edl933-clade-3-strain
#12
Natalia Amigo, Qi Zhang, Ariel Amadio, Qunjie Zhang, Wanderson M Silva, Baiyuan Cui, Zhongjian Chen, Mariano Larzabal, Jinlong Bei, Angel Cataldi
Escherichia coli O157:H7 is responsible for severe diarrhea and hemolytic uremic syndrome (HUS), and predominantly affects children under 5 years. The major virulence traits are Shiga toxins, necessary to develop HUS and the Type III Secretion System (T3SS) through which bacteria translocate effector proteins directly into the host cell. By SNPs typing, E. coli O157:H7 was separated into nine different clades. Clade 8 and clade 6 strains were more frequently associated with severe disease and HUS. In this study, we aimed to identify differentially expressed proteins in two strains of E...
2016: PloS One
https://www.readbyqxmd.com/read/27879544/immune-hemolytic-anemia-paroxysmal-cold-hemoglobinuria-preceding-burkitt-lymphoma-in-a-12-year-old-child
#13
Majd T Ghanim, Brian Berman
Autoimmune hemolytic anemia (AIHA) in childhood, including paroxysmal cold hemoglobinuria, is an uncommon, potentially life-threatening disorder. AIHA is a recognized complication of several varieties of lymphoproliferative disorders, including high-grade B-cell lymphoma, but it has not been associated with Burkitt lymphoma in people without an underlying immunodeficiency. When AIHA occurs in association with lymphoproliferative disorders, it may precede or accompany the diagnosis of malignant disease or herald relapse...
November 22, 2016: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/27879540/severe-hemolytic-anemia-following-intravenous-immunoglobulin-in-an-infant-with-kawasaki-disease
#14
Vlad Tocan, Akari Inaba, Tamami Kurano, Motoshi Sonoda, Keiji Soebijanto, Hideki Nakayama
Severe hemolytic anemia (HA) is an uncommon adverse reaction of intravenous immunoglobulin (IVIg) administration. Previous reports assume that antibodies contained in IVIg preparations are the cause of hemolysis. We report a 10-month-old infant with Kawasaki disease who was treated with high-dose IVIg and developed severe HA. The patient's Rh blood type was D+C+c+E-e+. He developed anti-C and anti-e antibodies following treatment with IVIg, and, after considering all possible causes of hemolysis, we concluded that this was a case of autoimmune HA induced by immunoglobulin treatment...
November 22, 2016: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/27879189/gemcitabine-induced-hemolytic-uremic-syndrome-treated-with-eculizumab-or-plasmapheresis-two-case-reports%C3%A2
#15
María Esperanza López Rubio, Raquel Rodado Martínez, María Luisa Illescas, Encarnación Mateo Bosch, Mercedes Martínez Díaz, Lourdes de la Vara Inesta, Basilio Cabezuelo, María Elisa Morales Albuja, Eladio Lucas Guillén, Luisa Jimeno García
BACKGROUND: Drug-induced hemolytic-uremic syndrome (HUS) has shown good response to eculizumab (ECU). We present 2 cases of patients with gemcitabine-induced HUS (GEM-HUS), one of whom was treated with ECU and the other with conventional treatment. Patient 1: A 74-year-old male with resected adenocarcinoma of the pancreas started adjuvant treatment with GEM, but after 5 months GEM was discontinued due to acute kidney injury and severe hypertension. Laboratory analyses identified microangiopathic hemolytic anemia (MHA) and thrombocytopenia...
November 23, 2016: Clinical Nephrology
https://www.readbyqxmd.com/read/27876749/anti-fungal-activity-of-ctn-15-34-the-c-terminal-peptide-fragment-of-crotalicidin-a-rattlesnake-venom-gland-cathelicidin
#16
Carolina Sidrim P Cavalcante, Cláudio B Falcão, Raquel Os Fontenelle, David Andreu, Gandhi Rádis-Baptista
Crotalicidin (Ctn), a 34-residue cathelicidin from a South American rattlesnake, and its fragment (Ctn[15-34]) have shown anti-infective and cytotoxic activities against Gram-negative bacteria and certain tumor lines, respectively. The extent of such effects has been related to physicochemical characteristics such as helicity and hydrophobicity. We now report the anti-fungal activity of Ctn and its fragments (Ctn[1-14]) and (Ctn[15-34]). MIC determination and luminescent cell viability assays were used to evaluate the anti-infective activity of Ctn and its fragments (Ctn[1-14]) and (Ctn[15-34]) as anti-fungal agents against opportunistic yeast and dermatophytes...
November 23, 2016: Journal of Antibiotics
https://www.readbyqxmd.com/read/27872630/control-of-wilt-and-rot-pathogens-of-tomato-by-antagonistic-pink-pigmented-facultative-methylotrophic-delftia-lacustris-and-bacillus-spp
#17
Veeranan Janahiraman, Rangasamy Anandham, Soon W Kwon, Subbiah Sundaram, Veeranan Karthik Pandi, Ramasamy Krishnamoorthy, Kiyoon Kim, Sandipan Samaddar, Tongmin Sa
The studies on the biocontrol potential of pink pigmented facultative methylotrophic (PPFM) bacteria other than the genus Methylobacterium are scarce. In the present study, we report three facultative methylotrophic isolates; PPO-1, PPT-1, and PPB-1, respectively, identified as Delftia lacustris, Bacillus subtilis, and Bacillus cereus by 16S rRNA gene sequence analysis. Hemolytic activity was tested to investigate the potential pathogenicity of isolates to plants and humans, the results indicates that the isolates PPO-1, PPT-1, and PPB-1 are not pathogenic strains...
2016: Frontiers in Plant Science
https://www.readbyqxmd.com/read/27871907/prognostic-value-of-t786c-and-g894t-enos-polymorphisms-in-sickle-cell-disease
#18
Iakovos Armenis, Vassiliki Kalotychou, Revekka Tzanetea, Panagoula Kollia, Zoi Kontogeorgiou, Dimitra Anastasopoulou, Marina Mantzourani, Michael Samarkos, Konstantinos Pantos, Kostas Konstantopoulos, Ioannis Rombos
Endothelial Nitric Oxide Synthase (eNOS) is crucial for vascular homeostasis. Polymorphisms T786C and G894T are reportedly affecting eNOS regulation and have been related to various diseases. Sickle Cell Disease (SCD), a clinically diverse chronic hemolytic anemia, implies impaired nitric oxide bioavailability. Aim of this study was to determine eNOS genotype for T786C and G894T polymorphisms in Greek patients with SCD and to elucidate its functional consequences and possible effects on clinical phenotype. Seventy nine steady state cases, most of them compound heterozygous for Sickle cell anemia/beta thalassemia and 48 controls were enrolled...
November 18, 2016: Nitric Oxide: Biology and Chemistry
https://www.readbyqxmd.com/read/27869428/-hemolytic-anemia-as-the-first-clinical-presentation-of-wilson-disease-a-pediatric-case
#19
José A Henao C, Kathia Valverde Muñoz, María L Ávila A
: Wilson disease is an autosomal recessive disorder of the copper's hepatic metabolism; it results in toxicity due to accumulation of the mineral. The hemolytic anemia is present in 17% at some point of the disease, although it is a rare initial clinical presentation. CASE REPORT: 11 years old boy who presented with negative Coombs hemolytic anemia and elevation of liver enzymes. The possibility of Wilson disease was considered, which was confirmed with the finding of a Kayser-Fleischer ring in the eye exam...
December 1, 2016: Archivos Argentinos de Pediatría
https://www.readbyqxmd.com/read/27869414/prevalence-and-clinical-course-of-typical-hemolytic-uremic-syndrome-among-sibling
#20
Alfredo Eymann, Paula Coccia, Claudia Raddavero, Gabriela Lafi, Verónica Ferraris, José Ramírez, Jorge Ferraris
Introduction: Hemolytic uremic syndrome (HUS) isaninfectious disease caused by Shiga toxin-producing Escherichia coli. The objective of this study was to assess the risk of transmission and clinical course between siblings with typical HUS. Population and methods: Medical records of children with typical HUS between 1997 and 2012 were reviewed. Sibling pairs were established as inclusion criteria. A severity score was defined. Results: A total of 133 patients with HUS were recorded; 40 had siblings and 4 progressed to HUS (10%)...
December 1, 2016: Archivos Argentinos de Pediatría
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