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https://www.readbyqxmd.com/read/28630122/eculizumab-blocks-vaccine-induced-opsonophagocytic-killing-of-meningococci-by-whole-blood-from-immunized-adults
#1
Monica Konar, Dan M Granoff
Eculizumab, a humanized anti-complement C5 monoclonal antibody for treatment of paroxysmal nocturnal hemoglobinuria (PNH) and atypical hemolytic uremic syndrome, blocks the terminal complement pathway required for serum bactericidal activity (SBA). Because treated patients are at >1000-fold increased risk of meningococcal disease, vaccination is recommended, but whether vaccination can protect by opsonophagocytic activity in the absence of SBA is not known. Meningococci were added to anticoagulated blood from 12 healthy adults vaccinated with meningococcal serogroup B and serogroup A,C,W,Y vaccines...
June 19, 2017: Blood
https://www.readbyqxmd.com/read/28626544/atypical-hemolytic-uremic-syndrome-a-brief-review
#2
REVIEW
Kuixing Zhang, Yuxin Lu, Kevin T Harley, Minh-Ha Tran
Atypical hemolytic uremic syndrome (aHUS) is a disease characterized by the triad of microangiopathic hemolytic anemia, thrombocytopenia and acute kidney injury. The histopathologic lesions of aHUS include thrombotic microangiopathy involving the glomerular capillaries and thrombosis involving arterioles or interlobar arteries. Extra-renal manifestations occur in up to 20% of patients. The majority of aHUS is caused by complement system defects impairing ordinary regulatory mechanisms. Activating events therefore lead to unbridled, ongoing complement activity producing widespread endothelial injury...
June 1, 2017: Hematology Reports
https://www.readbyqxmd.com/read/28626212/a-huge-subcutaneous-hematoma-in-an-adult-with-kasabach-merritt-syndrome
#3
Kuan-Lin Wu, Chiung-Ying Liao, Chen-Kuang Chang, Shang-Yun Ho, Yeu-Sheng Tyan, Yuan-Chun Huang
BACKGROUND Kasabach-Merritt syndrome is a potentially fatal disease that consists of hemangioma(s) with thrombocytopenia, microangiopathic hemolytic anemia, and coagulopathy. Extensive hemangiomatosis is rare. We present the radiological features and treatment strategy of a young adult suffering from Kasabach-Merritt syndrome with widespread hemangiomas and an infected huge hematoma in the right thigh. CASE REPORT A 33-year-old Taiwanese male presented with a painful 20-cm mass over his right thigh and gross hematuria for 2 days...
June 19, 2017: American Journal of Case Reports
https://www.readbyqxmd.com/read/28620416/antiphospholipid-syndrome-multiple-manifestations-in-a-single-patient-a-high-suspicion-is-still-needed
#4
Uroosa Ibrahim, Shiksha Kedia, Gwenalyn Garcia, Jean Paul Atallah
Antiphospholipid Syndrome (APS) is an autoimmune disorder with clinical and laboratory features of vascular thrombosis, pregnancy loss, and persistent antiphospholipid antibodies (aPLs). The pathophysiology is thought to involve the activation of endothelial cells, monocytes, platelets, and complement by aPLs. Disease can range from asymptomatic to rapidly fatal catastrophic APS. We present a case of a 34-year-old male referred for pancytopenia and splenomegaly. On examination, he had decreased sensation and 4/5 power in the left upper extremity...
2017: Case Reports in Medicine
https://www.readbyqxmd.com/read/28615230/rapid-rhd-zygosity-determination-using-digital-pcr
#5
Kelly A Sillence, Amr J Halawani, Wajnat A Tounsi, Kirsty A Clarke, Michele Kiernan, Tracey E Madgett, Neil D Avent
BACKGROUND: Paternal zygosity testing is used for determining homo- or hemizygosity of RHD in pregnancies that are at a risk of hemolytic disease of the fetus and newborn. At present, this is achieved by using real-time PCR or the Rhesus box PCR, which can be difficult to interpret and unreliable, particularly for black African populations. METHODS: DNA samples extracted from 58 blood donors were analyzed using 2 multiplex reactions for RHD-specific targets against a reference (AGO1)(2) to determine gene dosage by digital PCR...
June 14, 2017: Clinical Chemistry
https://www.readbyqxmd.com/read/28614372/intestinal-organoids-model-human-responses-to-infection-by-commensal-and-shiga-toxin-producing-escherichia-coli
#6
Sayali S Karve, Suman Pradhan, Doyle V Ward, Alison A Weiss
Infection with Shiga toxin (Stx) producing Escherichia coli O157:H7 can cause the potentially fatal complication hemolytic uremic syndrome, and currently only supportive therapy is available. Lack of suitable animal models has hindered study of this disease. Induced human intestinal organoids (iHIOs), generated by in vitro differentiation of pluripotent stem cells, represent differentiated human intestinal tissue. We show that iHIOs with addition of human neutrophils can model E. coli intestinal infection and innate cellular responses...
2017: PloS One
https://www.readbyqxmd.com/read/28614243/different-types-of-glomerulonephritis-associated-with-the-dysregulation-of-the-complement-alternative-pathway-in-2-brothers-a-case-report
#7
Pei Chen, Li Zhu, Feng Yu, Sha-Sha Han, Si-Jun Meng, Wei-Yi Guo, Hong Zhang, Yan Song
RATIONALE: C3 glomerulonephritis (C3GN) and complement-mediated hemolytic uremic syndrome (HUS) both result from the abnormal regulation of the complement system. A significant number of patients with C3GN or complement-mediated HUS have mutations of more than 1 complement protein. This discovery has had a major impact on identifying the underlying cause of familial C3GN or complement-mediated HUS. PATIENT CONCERNS: We report the cases of 2 brothers (herein referred to as patient II-1 and patient II-9), both with complement disorders that differed in their clinical and genetic features...
June 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28611569/bacterial-ulcerative-esophagitis-in-an-immunocompetent-patient
#8
Jung A Shin, Young Bae Lee, In Cheol Yoon, Hyeon Ju Jeong, Taejung Kwon, Hong Sub Lee
Bacterial esophagitis is a very rare condition usually occurring in patients with immunosuppression. To our best knowledge, bacterial esophagitis without underlying immunosuppressive disease has not been reported. We report an immunocompetent patient with bacterial esophagitis caused by B-hemolytic Streptococcus which resulted in an esophageal stricture. A 68-year-old female was admitted for odynophagia which had developed several days before. Upper endoscopy revealed extensive ulceration covered by whitish exudates with submucosal edema at the proximal esophagus...
January 2017: Case Reports in Gastroenterology
https://www.readbyqxmd.com/read/28605374/laboratory-techniques-used-to-maintain-and-differentiate-biotypes-of-vibrio-cholerae-clinical-and-environmental-isolates
#9
Kyle D Brumfield, Bailey M Carignan, Jordan N Ray, Panagiota E Jumpre, Mike S Son
The aquatic Gram-negative bacterium Vibrio cholerae is the etiological agent of the infectious gastrointestinal disease cholera. Due to the global prevalence and severity of this disease, V. cholerae has been extensively studied in both environmental and laboratory settings, requiring proper maintenance and culturing techniques. Classical and El Tor are two main biotypes that compose the V. cholerae O1 serogroup, each displaying unique genotypic and phenotypic characteristics that provide reliable mechanisms for biotype characterization, and require distinct virulence inducing culturing conditions...
May 30, 2017: Journal of Visualized Experiments: JoVE
https://www.readbyqxmd.com/read/28600054/prenatal-treatment-of-severe-fetal-hemolytic-disease-due-to-anti-m-alloimmunization-by-serial-intrauterine-transfusions
#10
Lin Li, Linhuan Huang, Guangping Luo, Yanmin Luo, Qun Fang
OBJECTIVE: Fetal hemolytic disease is a common cause of fetal hydrops and fetal morbidity and mortality. Despite its relatively low frequency, the anti-M IgG antibody is one of the causes of severe fetal anemia and intrauterine death; only a few cases have been reported. CASE REPORT: This is a case of a pregnant woman with a history of three intrauterine deaths. A diagnosis of severe fetal anemia attributed to anti-M alloimmunization was confirmed in her fifth pregnancy...
June 2017: Taiwanese Journal of Obstetrics & Gynecology
https://www.readbyqxmd.com/read/28596661/what-is-it-really-anti-g-or-anti-d-plus-anti-c-clinical-significance-in-antenatal-mothers
#11
Soumya Das, Shamee Shastry, M Murugesan, Poornima Baliga B, Shamee Shastry
G antigen of Rh blood group system is present either along with D and/or C positive red cells. Hence, [serologically anti-G presents with the similar picture as that of multiple antibodies (anti-D + anti-C). Differentiating them is important as anti-D + anti-C causes severe hemolytic disease of the fetus and newborn than anti-G. In pregnancies with anti-G alone, alloimmunization due to D antigen could be prevented by prophylactic administration of RhIg. Differentiating between anti-D + C from anti-G in alloimmunized pregnant mothers becomes essential...
June 2017: Indian Journal of Hematology & Blood Transfusion
https://www.readbyqxmd.com/read/28596380/identification-of-oxidative-modifications-of-hemopexin-and-their-predicted-physiological-relevance
#12
Peter Hahl, Rachel Hunt, Edward S Bjes, Andrew Skaff, J Andrew Keightley, Ann Smith
Hemopexin protects against heme toxicity in hemolytic diseases and conditions, sepsis and sickle cell disease. This protection is sustained by heme-hemopexin complexes present in biological fluids that resist oxidative damage during heme-driven inflammation. However, apo-hemopexin is vulnerable to inactivation by reactive nitrogen and oxygen species that covalently modify amino acids. The consequent nitration of amino acids is considered a selective and specific effect reflecting biological events. Using liquid chromatography-tandem mass spectrometry, we discovered low endogenous levels of tyrosine nitration in the peptide YYCFQGNQFLR of human hemopexin, which was similarly nitrated in rabbit and rat hemopexins...
June 8, 2017: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/28588179/childhood-wilson-disease-bangladesh-perspective
#13
M Rukunuzzaman, A B Karim, M Nurullah, F Sultana, M W Mazumder, M A Rahman, S B Billah, F Begum, M Oliullah
Wilson's disease (WD) is an autosomal recessive disorder affecting copper metabolism causing copper induced damage to various organs. In children liver is commonly involved. Central nervous system, eyes, RBC, kidneys, brain and bones may also be affected. Aim of the study is to evaluate clinical & laboratory profile of Wilson's disease in children. This cross sectional descriptive study was conducted at the department of Paediatric Gastroenterology and Nutrition, Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, Bangladesh, from January 2011 to December, 2013...
April 2017: Mymensingh Medical Journal: MMJ
https://www.readbyqxmd.com/read/28583115/tuberculosis-arthritis-of-the-sternoclavicular-joint-after-uncomplicated-falciparum-malaria-a-case-report
#14
Boundia Djiba, Baidy Sy Kane, Mamadou Alpha Diallo, Khadim Diongue, Ngoné Diaba Diack, Hamidou Deme, Mouhamed Dieng, Maimouna Sow, Daouda Ndiaye, Abdoulaye Pouye
BACKGROUND: Malaria and tuberculosis are co-endemic in many developing countries. However their associations are rarely reported. Yet, it has been suggested that a pathological process may link the two diseases. CASE PRESENTATION: A 20-year-old female patient was admitted in the internal medicine service of Aristide Le Dantec Hospital for uncomplicated malaria. She was previously treated for autoimmune hemolytic anaemia using prednisone at 5 mg per day. Clinical examination showed swelling in front of the sternoclavicular joint...
June 5, 2017: Annals of Clinical Microbiology and Antimicrobials
https://www.readbyqxmd.com/read/28580721/persistent-hemolytic-disease-of-the-fetus-and-newborn-hdfn-associated-with-passive-acquisition-of-anti-d-in-maternal-breast-milk
#15
Marissa Li, John C Blaustein
BACKGROUND: Anti-D is a well-documented, significant cause of hemolytic disease of the fetus and newborn (HDFN), but its presence in breast milk is not routinely described. Theoretically, breast milk containing anti-D could have the potential to exacerbate HDFN if ingested by the affected infant. STUDY DESIGN AND METHODS: This is a case report of a 28-week premature male neonate with hydrops fetalis born to a 32-year-old woman (gravidity 3/parity 3) with anti-D and anti-G...
June 5, 2017: Transfusion
https://www.readbyqxmd.com/read/28578979/hiv-associated-kidney-diseases-clarifying-concordance-between-renal-failure-in-hiv-infection-and-histopathologic-manifestations-at-kidney-biopsy
#16
REVIEW
Carla L Ellis
Patients with HIV infection have a wide spectrum of renal diseases. Some are known to be the direct effect of the viral infection while others are renal diseases that also occur in uninfected populations. HIV associated nephropathy (HIVAN) is considered to be a subtype of primary focal and segmental glomerulosclerosis that is distinct in HIV infected patients. It is more frequent in the African-American population and associated with mutations of the apolipoprotein L1 (APOL1) gene. HIV associated immune complex kidney disease (HIVICD) encompasses a spectrum of HIV associated renal diseases characterized by the presence of immune complex deposition within glomeruli...
May 5, 2017: Seminars in Diagnostic Pathology
https://www.readbyqxmd.com/read/28571698/a-novel-antimicrobial-peptide-against-dental-caries-associated-bacteria
#17
Long Chen, Lili Jia, Qiang Zhang, Xirui Zhou, Zhuqing Liu, Bingjie Li, Zhentai Zhu, Fenwei Wang, Changyuan Yu, Qian Zhang, Feng Chen, Shi-Zhong Luo
Dental caries, a highly prevalent oral disease, is primarily caused by pathogenic bacteria infection, and most of them are anaerobic. Herein, we investigated the activity of a designed antimicrobial peptide ZXR-2, and found it showed broad-spectrum activity against a variety of Gram-positive and Gram-negative oral bacteria, particularly the caries-related taxa Streptococcus mutans. Time-course killing assays indicated that ZXR-2 killed most bacterial cells within 5 min at 4 × MIC. The mechanism of ZXR-2 involved disruption of cell membranes, as observed by scanning electron microscopy...
May 29, 2017: Anaerobe
https://www.readbyqxmd.com/read/28568311/defining-the-clinical-relevance-of-red-blood-cell-autoantibodies-by-monocyte-monolayer-assay
#18
Marina C A V Conrado, Amanda N D'Avila, Juliana B Vieira, Silvia L Bonifacio, Francisco C A Gomes, Marcia R Dezan, Valeria B Oliveira, Ingrid H Ribeiro, Luciana T C M Tucunduva, Alfredo Mendrone-Júnior, Vanderson Rocha, Carla L Dinardo
BACKGROUND: The Monocyte Monolayer Assay (MMA) is an in vitro simulation of red blood cell (RBC) alloantibody behavior. It has been classically applied to predict the risks of post-transfusion hemolytic reactions when transfusing incompatible RBC units. Quantifying erythrophagocytosis by MMA may be an interesting option for situations where there is doubt whether a RBC autoantibody is mediating significant hemolysis. Here, we present three situations involving RBC autoantibodies in which the MMA was decisive for clarifying the diagnosis and choosing the best clinical treatment...
May 31, 2017: Journal of Clinical Laboratory Analysis
https://www.readbyqxmd.com/read/28567595/do-alpha-thalassemia-fetal-hemoglobin-and-the-ugt1a1-polymorphism-have-an-influence-on-serum-bilirubin-levels-and-cholelithiasis-in-patients-with-sickle-cell-disease
#19
Laura Alencastro de Azevedo, Joyce Bonazzoni, Sandrine Comparsi Wagner, Mariela Granero Farias, Christina M Bittar, Liane Daudt, Simone Martins de Castro
BACKGROUND: Increased destruction of erythrocytes in patients with sickle cell disease results in chronic hyperbilirubinemia and leads to the formation of gallstones. OBJECTIVES: The objective of this study was to determine the combined influence of alpha thalassemia, fetal hemoglobin, and the UGT1A1 polymorphism on serum bilirubin levels and cholelithiasis in patients with sickle cell disease. METHODS: We analyzed 72 patients treated in the outpatient hematology unit of the Clinical Hospital of Porto Alegre...
May 31, 2017: Molecular Diagnosis & Therapy
https://www.readbyqxmd.com/read/28554826/oxidative-pathways-in-the-sickle-cell-and-beyond
#20
REVIEW
Abdu I Alayash
Polymerization of deoxy sickle cell hemoglobin (HbS) is well recognized as the primary event that triggers the classic cycles of sickling/unsickling of patients red blood cells (RBCs). RBCs are also subjected to continuous endogenous and exogenous oxidative onslaughts resulting in hemolytic rate increases which contribute to the evolution of vasculopathies associated with this disease. Compared to steady-state conditions, the occurrences of vaso-occlusive crises increase the levels of both RBC-derived microparticles as well as extracellular Hb in circulation...
May 20, 2017: Blood Cells, Molecules & Diseases
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