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https://www.readbyqxmd.com/read/28164595/differential-expression-of-cd52-cd14-and-hla-dr-on-cd4-monocytes-in-three-types-of-acquired-bone-marrow-failure-syndromes
#1
Yang Kai, Dai Yuanyuan, Guan Shihe, Mu Xuanxuan, Zhang Hao, Pan Ying, Wu Yuanyuan, Wang Aihua, Sun Beibei, TongYang, Zhou Tingdong
BACKGROUND: Aplastic anemia (AA), paroxysmal nocturnal hemoglobinuria (PNH), and myelodysplastic syndrome (MDS) are the common spectrums of acquired bone marrow failure syndromes (BMFs). Accurate and timely diagnosis is a significant clinical challenge because of the overlapping features. The pathogenesis is not fully understood, but several studies have suggested that defective monocyte functions play an important role. We aimed to find whether the different expressions of CD52, CD14 and HLA-DR on CD4+ monocytes would be helpful in the preliminary diagnosis of acquired BMFs...
September 1, 2016: Clinical Laboratory
https://www.readbyqxmd.com/read/28151558/whole-transcriptome-sequencing-identifies-increased-cxcr2-expression-in-pnh-granulocytes
#2
Kohei Hosokawa, Sachiko Kajigaya, Keyvan Keyvanfar, Wangmin Qiao, Yanling Xie, Angelique Biancotto, Danielle M Townsley, Xingmin Feng, Neal S Young
The aetiology of paroxysmal nocturnal haemoglobinuria (PNH) is a somatic mutation in the X-linked phosphatidylinositol glycan class A gene (PIGA), resulting in global deficiency of glycosyl phosphatidylinositol-anchored proteins (GPI-APs). This study applied RNA-sequencing to examine functional effects of the PIGA mutation in human granulocytes. CXCR2 expression was increased in GPI-AP(-) compared to GPI-AP(+) granulocytes. Macrophage migration inhibitory factor, a CXCR2 agonist, was significantly higher in plasma of PNH patients...
February 1, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28124080/serologic-response-to-meningococcal-vaccination-in-patients-with-paroxysmal-nocturnal-hemoglobinuria-pnh-chronically-treated-with-the-terminal-complement-inhibitor-eculizumab
#3
Ferras Alashkar, Colin Vance, Dörte Herich-Terhürne, Nicole Preising, Ulrich Dührsen, Alexander Röth
Eculizumab is indicated for the therapy of patients with symptomatic paroxysmal nocturnal hemoglobinuria (PNH). Due to inhibition of terminal complement cascade, patients on eculizumab are susceptible to Neisseria meningitidis infections. The two mainstays to reduce the risk of infection are vaccination and antibiotic prophylaxis. In this retrospective study, serologic response was analyzed after vaccination with a meningococcal vaccine in 23 PNH patients (median age 36 years; range 25 - 88 years; 15 males, 8 females) by measuring serum bactericidal assay (SBA) using rabbit complement (rSBA) titers against meningococcal serogroups A, C, W, and Y...
January 26, 2017: Annals of Hematology
https://www.readbyqxmd.com/read/28100318/-clinical-characteristics-of-clonal-evolution-after-immunosuppressive-therapy-in-children-with-severe-very-severe-aplastic-anemia
#4
Jing-Liao Zhang, Tian-Feng Liu, Li-Xian Chang, Xia Chen, Yuan-Yuan Ren, Cong-Cong Sun, Chao Liu, Wen-Bin An, Yang Wan, Xiao-Juan Chen, Wen-Yu Yang, Shu-Chun Wang, Ye Guo, Yao Zou, Yu-Mei Chen, Xiao-Fan Zhu
OBJECTIVE: To evaluate the clinical characteristics and risk factors of clonal evolution after immunosuppressive therapy (IST) in children with severe/very severe aplastic anemia (SAA/VSAA). METHODS: The clinical data of 231 children with newly-diagnosed SAA/VSAA who received IST were retrospectively studied. The incidence and risk factors of clonal evolution after IST were analyzed. RESULTS: The 5-year overall survival rate of the 231 patients was 82...
January 2017: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
https://www.readbyqxmd.com/read/28045484/development-of-autologous-c5-vaccine-nanoparticles-to-reduce-intravascular-hemolysis-in-vivo
#5
Lingjun Zhang, Wen Qiu, Stephen Crooke, Yan Li, Areeba Abid, Bin Xu, M G Finn, Feng Lin
The complement system is emerging as a new target for treating many diseases. For example, Eculizumab, a humanized monoclonal antibody against complement component 5 (C5), has been approved for paroxysmal nocturnal hemoglobinuria (PNH) in which patient erythrocytes are lysed by complement. In this study, we developed vaccines to elicit autologous anti-C5 antibody production in mice for complement inhibition. Immunization of mice with a conservative C5 xenoprotein raised high titers of IgG's against the xenogenous C5, but these antibodies did not reduce C5 activity in the blood...
January 12, 2017: ACS Chemical Biology
https://www.readbyqxmd.com/read/28039017/development-of-pro-inflammatory-phenotype-in-monocytes-after-engulfing-hb-activated-platelets-in-hemolytic-disorders
#6
Rashi Singhal, Sheetal Chawla, Deepak K Rathore, Angika Bhasym, Gowtham K Annarapu, Vandana Sharma, Tulika Seth, Prasenjit Guchhait
Monocytes and macrophage combat infections and maintain homeostatic balance by engulfing microbes and apoptotic cells, and releasing inflammatory cytokines. Studies have described that these cells develop anti-inflammatory properties upon recycling the free-hemoglobin (Hb) in hemolytic conditions. While investigating the phenotype of monocytes in two hemolytic disorders-paroxysmal nocturnal hemoglobinuria (PNH) and sickle cell disease (SCD), we observed a high number of pro-inflammatory (CD14(+)CD16(hi)) monocytes in these patients...
December 28, 2016: Clinical Immunology: the Official Journal of the Clinical Immunology Society
https://www.readbyqxmd.com/read/28028953/opinions-of-old-age-according-to-residents-of-polish-public-nursing-homes-and-members-of-the-universities-of-the-third-age
#7
Mateusz Cybulski, Elżbieta Krajewska-Kułak, Jacek Jamiołkowski
BACKGROUND: Statistical data from the turn of the 21st century shows a significant increase in the average human life span and, what follows, an extension of old age. OBJECTIVES: The aim of this study was to become familiar with the opinions submitted by respondents regarding aging and old age, health problems connected with aging and preferred health behaviors. MATERIAL AND METHODS: The research was conducted between January 3rd 2013 and February 15th 2014 on a group of 200 residents of public nursing homes and 200 members of the University of the Third Age using a questionnaire created by the authors...
September 2016: Advances in Clinical and Experimental Medicine: Official Organ Wroclaw Medical University
https://www.readbyqxmd.com/read/28006835/cramp-fasciculation-syndrome-associated-with-monofocal-motor-neuropathy
#8
Nicolas J Dubuisson, Vincent Van Pesch, Peter Y K Van den Bergh
INTRODUCTION: Cramp-fasciculation syndrome (CFS) is a peripheral nerve hyperexcitability disorder (PNH), which could be caused by inflammatory neuropathy. CASE REPORT: We describe a 51-year-old woman who presented with a 4-5 year history of fasciculations and painful cramping of the right thenar eminence. Electrophysiological studies showed motor conduction block in the right median nerve between the axilla and the elbow with fasciculation potentials and cramp discharges on EMG in the right abductor pollicis brevis muscle...
December 22, 2016: Muscle & Nerve
https://www.readbyqxmd.com/read/27990389/laryngeal-involvement-with-fatal-outcome-in-progressive-nodular-histiocytosis-a-rare-case-report
#9
Aarti Salunke, Vasudha Belgaumkar, Ravindranath Chavan, Rinkesh Dobariya
Progressive nodular histiocytosis (PNH) represents a very rare type of non-Langerhans cell histiocytosis. It is characterized by progressive appearance of papules and nodules without spontaneous resolution. We report a 60-year-old patient with novel clinical features in the form of extensive noduloulcerative lesions, ichthyotic patches, and laryngeal involvement culminating in fatal outcome prior to therapeutic intervention. Although the presenting features were baffling, histopathology and immunohistochemistry clinched the diagnosis of PNH...
November 2016: Indian Dermatology Online Journal
https://www.readbyqxmd.com/read/27986761/resistance-training-interventions-across-the-cancer-control-continuum-a-systematic-review-of-the-implementation-of-resistance-training-principles
#10
C M Fairman, P N Hyde, B C Focht
OBJECTIVES: The primary purpose of this systematic review is to examine the extant resistance training (RT) cancer research to evaluate the proportion of RT interventions that: (1) implemented key RT training principles (specificity, progression, overload) and (2) explicitly reported relevant RT prescription components (frequency, intensity, sets, reps). DESIGN: A qualitative systematic review was performed by two reviewers (CMF and PNH) who inspected the titles and abstracts to determine eligibility for this systematic review...
December 16, 2016: British Journal of Sports Medicine
https://www.readbyqxmd.com/read/27936436/spectroscopic-single-crystal-x-ray-hirshfeld-in-vitro-and-in-silico-biological-evaluation-of-a-new-series-of-potent-thiazole-nucleus-integrated-with-pyrazoline-scaffolds
#11
Vinutha V Salian, Badiadka Narayana, Balladka K Sarojini, Madan S Kumar, Govinahalli S Nagananda, Kullaiah Byrappa, Avinash K Kudva
In the present study, the spectroscopic characterization of a new series of substituted thiazole linked pyrazoline scaffolds 4a-l was performed. The formation of 4a-l from the intermediate 3-(4-chlorophenyl)-5-[4-(propan-2-yl)phenyl]-4,5-dihydro-1H-pyrazole-1-carbothioamide 2 and substituted 2-bromo-1-phenylethanone 3a-l was evidenced through the changes in FTIR, (1)H NMR, (13)C NMR, LCMS data. The X-ray diffraction studies revealed that compound 2 and 4g crystallized in monoclinic crystal system with P21/n space group...
March 5, 2017: Spectrochimica Acta. Part A, Molecular and Biomolecular Spectroscopy
https://www.readbyqxmd.com/read/27936141/hbs-binding-to-gp1b%C3%AE-activates-platelets-in-sickle-cell-disease
#12
Gowtham K Annarapu, Rashi Singhal, Avinash Gupta, Sheetal Chawla, Harish Batra, Tulika Seth, Prasenjit Guchhait
Intravascular hemolysis increases the risk of thrombosis in hemolytic disorders. Our previous study showed that the binding of adult hemoglobin (HbA) to glycoprotein (GP) 1bα induced the activation of platelets. The elevated plasma Hb or platelet surface bound Hb positively correlated with platelet activation in patients with paroxysmal nocturnal hemoglobinuria (PNH). Furthermore, this study shows that the sickle Hb [HbS, occurs due to single nucleotide polymorphism at A>T of β-globin gene of Hb and causes sickle cell disease (SCD)] also bound to GP1bα and activated platelets in a concentration-dependent manner...
2016: PloS One
https://www.readbyqxmd.com/read/27913482/update-on-the-diagnosis-and-management-of-paroxysmal-nocturnal-hemoglobinuria
#13
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/27911366/a-multimodal-imaging-and-stimulation-based-method-of-evaluating-connectivity-related-brain-excitability-in-patients-with-epilepsy
#14
Mouhsin M Shafi, Susan Whitfield-Gabrieli, Catherine J Chu, Alvaro Pascual-Leone, Bernard S Chang
Resting-state functional connectivity MRI (rs-fcMRI) is a technique that identifies connectivity between different brain regions based on correlations over time in the blood-oxygenation level dependent signal. rs-fcMRI has been applied extensively to identify abnormalities in brain connectivity in different neurologic and psychiatric diseases. However, the relationship among rs-fcMRI connectivity abnormalities, brain electrophysiology and disease state is unknown, in part because the causal significance of alterations in functional connectivity in disease pathophysiology has not been established...
November 13, 2016: Journal of Visualized Experiments: JoVE
https://www.readbyqxmd.com/read/27903532/acquired-somatic-mutations-in-pnh-reveal-long-term-maintenance-of-adaptive-nk-cells-independent-from-hspc
#15
Marcus A F Corat, Heinrich Schlums, Chuanfeng Wu, Jakob Theorell, Diego A Espinoza, Stephanie E Sellers, Danielle M Townsley, Neal S Young, Yenan T Bryceson, Cynthia E Dunbar, Thomas Winkler
Natural killer (NK) cells have long been considered short-lived effectors of innate immunity. However, recent animal models and human studies suggest that subsets of NK cells have adaptive features. We investigate clonal relationships of various NK cell subsets, including the adaptive population, by taking advantage of naturally occurring X-linked somatic PIGA mutations in hematopoietic stem and progenitor cells (HSPC) from patients with paroxysmal nocturnal hemoglobinuria (PNH). The affected HSPCs and their progeny lack expression of glycosylphosphatidylinositol (GPI) anchors on their cell surfaces, allowing quantification of PIGA-mutant (GPI-negative) HSPC-derived peripheral blood cell populations...
November 30, 2016: Blood
https://www.readbyqxmd.com/read/27837250/development-of-a-disease-specific-quality-of-life-questionnaire-for-patients-with-aplastic-anemia-and-or-paroxysmal-nocturnal-hemoglobinuria-qlq-aa-pnh-report-on-phases-i-and-ii
#16
Martha Groth, Susanne Singer, Cathrin Niedeggen, Andrea Petermann-Meyer, Alexander Röth, Hubert Schrezenmeier, Britta Höchsmann, Tim H Brümmendorf, Jens Panse
Acquired aplastic anemia (AA) and paroxysmal nocturnal hemoglobinuria (PNH) are interrelated ultra-rare diseases. Quality of life (QoL) evaluation tools used in studies for AA and PNH are unspecific and designed for cancer patients (e.g., the European Organization for Research and Treatment of Cancer Quality of Life Questionnaire, EORTC QLQ-C30). Given the complexity of AA and PNH, variation in symptoms and treatments, younger age of many patients, and the fact that AA and PNH are not classified as malignant diseases, it is likely that cancer-specific questionnaires are inappropriate...
February 2017: Annals of Hematology
https://www.readbyqxmd.com/read/27812245/paroxysmal-nocturnal-hemoglobinuria-from-bench-to-bed
#17
REVIEW
Amrallah A Mohammed, Hani El-Tanni, Tariq Al-Malki Atiah, Arwa Al-Malki Atiah, Marwan Al-Malki Atiah, Ayman A Rasmy
Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired hemolytic anemia with highly variable clinical symptoms making the diagnosis and prediction of its outcome difficult. It is caused by the expansion of a hematopoietic progenitor cell that has acquired a mutation in the X-linked phosphatidylinositol glycan class A (PIGA) gene that results in deficiency of the glycosylphosphatidylinositol anchor structure responsible for fixing a wide spectrum of proteins particularly CD55 and CD59. The clinical features of this disease arise as a result of complement-mediated hemolysis in unprotected red cells, leukocytes, and platelets as well as the release of free hemoglobin...
December 2016: Indian Journal of Hematology & Blood Transfusion
https://www.readbyqxmd.com/read/27780113/the-dysfunction-of-platelets-in-paroxysmal-nocturnal-hemoglobinuria
#18
Rong Fu, Yinping Meng, Yihao Wang, Hui Liu, Yi Liu, Lijuan Li, Shaoxue Ding, Guojin Wang, Jia Song, Zonghong Shao
INTRODUCTION: Thrombosis is a dangerous complication of paroxysmal nocturnal hemoglobinuria (PNH) and has a high mortality rate. However, the mechanism underlying the development of thrombosis in PNH remains unclear. To explore this, platelet function and serum complement activity were investigated in 14 patients with classical PNH, 11 with PNH aplastic anemia (AA) and 30 healthy controls. MATERIAL AND METHODS: Serum concentrations of the terminal complement complex (sC5b-9) were determined by enzyme-linked immunofluorescence assay (ELISA), and the levels of C5b-9, CD61 and CD62p on platelet membranes were determined by flow cytometry...
December 2016: Thrombosis Research
https://www.readbyqxmd.com/read/27775713/small-molecule-factor-d-inhibitors-targeting-the-alternative-complement-pathway
#19
Jürgen Maibaum, Sha-Mei Liao, Anna Vulpetti, Nils Ostermann, Stefan Randl, Simon Rüdisser, Edwige Lorthiois, Paul Erbel, Bernd Kinzel, Fabrice A Kolb, Samuel Barbieri, Julia Wagner, Corinne Durand, Kamal Fettis, Solene Dussauge, Nicola Hughes, Omar Delgado, Ulrich Hommel, Ty Gould, Aengus Mac Sweeney, Bernd Gerhartz, Frederic Cumin, Stefanie Flohr, Anna Schubart, Bruce Jaffee, Richard Harrison, Antonio Maria Risitano, Jörg Eder, Karen Anderson
Complement is a key component of the innate immune system, recognizing pathogens and promoting their elimination. Complement component 3 (C3) is the central component of the system. Activation of C3 can be initiated by three distinct routes-the classical, the lectin and the alternative pathways-with the alternative pathway also acting as an amplification loop for the other two pathways. The protease factor D (FD) is essential for this amplification process, which, when dysregulated, predisposes individuals to diverse disorders including age-related macular degeneration and paroxysmal nocturnal hemoglobinuria (PNH)...
December 2016: Nature Chemical Biology
https://www.readbyqxmd.com/read/27766064/characteristics-of-taiwanese-patients-of-pnh-in-the-international-pnh-registry
#20
Wen-Chien Chou, Wei-Han Huang, Ming-Chung Wang, Chao-Sung Chang, Shih-Peng Yeh, Tzeon-Jye Chiou, Yeu-Chin Chen, Tseng-Hsi Lin, Ming-Ching Shen
BACKGROUND: Paroxysmal nocturnal hemoglobinuria (PNH) is a rare and acquired hematopoietic stem cell disease, with florid clinical presentations. Although this disease has been characterized in the western countries, its clinical and laboratory features in Taiwan have not yet been reported. RESULTS: As a part of an international prospective, non-interventional, observational registration trial of PNH, we have analyzed 63 patients recruited between 2009 and 2015 in Taiwan, with comparison to the 3857 patients in the rest of the world (ROW)...
2016: Thrombosis Journal
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