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Articles 1 - 12 of 12
Full-Text Articles in Medicine and Health Sciences
Treatment Of Dental Complications In Sickle Cell Disease., Priti Mulimani, Samir K. Ballas, Adinegara Bl Abas, Laxminarayan Karanth
Treatment Of Dental Complications In Sickle Cell Disease., Priti Mulimani, Samir K. Ballas, Adinegara Bl Abas, Laxminarayan Karanth
Cardeza Foundation for Hematologic Research
BACKGROUND: Sickle cell disease is the most common single gene disorder and the commonest haemoglobinopathy found with high prevalence in many populations across the world. Management of dental complications in people with sickle cell disease requires special consideration for three main reasons. Firstly, dental and oral tissues are affected by the blood disorder resulting in several oro-facial abnormalities. Secondly, living with a haemoglobinopathy and coping with its associated serious consequences may result in individuals neglecting their oral health care. Finally, the treatment of these oral complications must be adapted to the systemic condition and special needs of these individuals, in …
Pharmacological Interventions For Painful Sickle Cell Vaso-Occlusive Crises In Adults., Tess E. Cooper, Ian R. Hambleton, Samir K. Ballas, Brydee A. Johnston, Philip J. Wiffen
Pharmacological Interventions For Painful Sickle Cell Vaso-Occlusive Crises In Adults., Tess E. Cooper, Ian R. Hambleton, Samir K. Ballas, Brydee A. Johnston, Philip J. Wiffen
Cardeza Foundation for Hematologic Research
BACKGROUND: Sickle cell disease (SCD) is a group of inherited disorders of haemoglobin (Hb) structure in a person who has inherited two mutant globin genes (one from each parent), at least one of which is always the sickle mutation. It is estimated that between 5% and 7% of the world's population are carriers of the mutant Hb gene, and SCD is the most commonly inherited blood disorder. SCD is characterized by distorted sickle-shaped red blood cells. Manifestations of the disease are attributed to either haemolysis (premature red cell destruction) or vaso-occlusion (obstruction of blood flow, the most common manifestation). Shortened …
Validation Of A Miniaturized Permeability Assay Compatible With Crispr-Mediated Genome-Wide Screen., Claire Simonneau, Junning Yang, Xianguo Kong, Robert Kilker, Leonard Edelstein, Paolo Fortina, Eric R. Londin, Arie Horowitz
Validation Of A Miniaturized Permeability Assay Compatible With Crispr-Mediated Genome-Wide Screen., Claire Simonneau, Junning Yang, Xianguo Kong, Robert Kilker, Leonard Edelstein, Paolo Fortina, Eric R. Londin, Arie Horowitz
Cardeza Foundation for Hematologic Research
The impermeability of the luminal endothelial cell monolayer is crucial for the normal performance of the vascular and lymphatic systems. A key to this function is the integrity of the monolayer's intercellular junctions. The known repertoire of junction-regulating genes is incomplete. Current permeability assays are incompatible with high-throughput genome-wide screens that could identify these genes. To overcome these limitations, we designed a new permeability assay that consists of cell monolayers grown on ~150 μm microcarriers (MCs). Each MC functions as a miniature individual assay of permeability (MAP). We demonstrate that false-positive results can be minimized, and that MAP sensitivity to …
Functionalization Of Cd36 Cardiovascular Disease And Expression Associated Variants By Interdisciplinary High Throughput Analysis., Namrata Madan, Andrew R. Ghazi, Xianguo Kong, Edward S. Chen, Chad A. Shaw, Leonard C. Edelstein
Functionalization Of Cd36 Cardiovascular Disease And Expression Associated Variants By Interdisciplinary High Throughput Analysis., Namrata Madan, Andrew R. Ghazi, Xianguo Kong, Edward S. Chen, Chad A. Shaw, Leonard C. Edelstein
Department of Medicine Faculty Papers
CD36 is a platelet membrane glycoprotein whose engagement with oxidized low-density lipoprotein (oxLDL) results in platelet activation. The CD36 gene has been associated with platelet count, platelet volume, as well as lipid levels and CVD risk by genome-wide association studies. Platelet CD36 expression levels have been shown to be associated with both the platelet oxLDL response and an elevated risk of thrombo-embolism. Several genomic variants have been identified as associated with platelet CD36 levels, however none have been conclusively demonstrated to be causative. We screened 81 expression quantitative trait loci (eQTL) single nucleotide polymorphisms (SNPs) associated with platelet CD36 expression …
Use Of Plasmapheresis And Immunosuppressants To Treat Diffuse Alveolar Hemorrhage In A Patient With Granulomatosis With Polyangiitis., Yasar Sattar, Ammu Thampi Susheela, Waqas Ullah, Norina Usman, Fnu Zafrullah
Use Of Plasmapheresis And Immunosuppressants To Treat Diffuse Alveolar Hemorrhage In A Patient With Granulomatosis With Polyangiitis., Yasar Sattar, Ammu Thampi Susheela, Waqas Ullah, Norina Usman, Fnu Zafrullah
Abington Jefferson Health Papers
Granulomatosis with polyangiitis (GPA) is a systemic granulomatous inflammatory disease characterized by small-to-medium vessel vasculitis due to Central Anti-Neutrophil Cytoplasmic Antibody (C-ANCA). GPA commonly involves the lungs and the kidneys. Among the pulmonary manifestations, diffuse alveolar hemorrhage (DHA) is a rare presentation of GPA that can present with hemoptysis leading to acute onset of anemia and hemodynamic instability. An active diagnostic workup including serologic titer of C-ANCA, imaging, intensive care, and aggressive immunosuppression is the key to DAH management. We report a case of DAH secondary to GPA that presented with hemoptysis leading to severe anemia, initially resuscitated symptomatically and …
Horizontal Rna Transfer Goes Deep: Platelet Consumption And Microrna Utilization By Vascular Smooth Muscle Cells., Lawrence E. Goldfinger, Leonard C. Edelstein
Horizontal Rna Transfer Goes Deep: Platelet Consumption And Microrna Utilization By Vascular Smooth Muscle Cells., Lawrence E. Goldfinger, Leonard C. Edelstein
Department of Medicine Faculty Papers
No abstract provided.
A Prospective, Multi-Center, Randomized Comparison Of Iron Isomaltoside 1000 Versus Iron Sucrose In Patients With Iron Deficiency Anemia; The Ferwon-Ida Trial., Michael Auerbach, David Henry, Richard J. Derman, Maureen M. Achebe, Lars L. Thomsen, John Glaspy
A Prospective, Multi-Center, Randomized Comparison Of Iron Isomaltoside 1000 Versus Iron Sucrose In Patients With Iron Deficiency Anemia; The Ferwon-Ida Trial., Michael Auerbach, David Henry, Richard J. Derman, Maureen M. Achebe, Lars L. Thomsen, John Glaspy
Global Health Articles
Iron deficiency anemia (IDA) is prevalent, and intravenous iron, especially if given in a single dose, may result in better adherence compared with oral iron. The present trial (FERWON-IDA) is part of the FERWON program with iron isomaltoside 1000/ferric derisomaltose (IIM), evaluating safety and efficacy of high dose IIM in IDA patients of mixed etiologies. This was a randomized, open-label, comparative, multi-center trial conducted in the USA. The IDA patients were randomized 2:1 to a single dose of 1000 mg IIM, or iron sucrose (IS) administered as 200 mg intravenous injections, up to five times. The co-primary endpoints were adjudicated …
Gpvi Inhibitor As Antitumor Gateway Drug., Lawrence E. Goldfinger
Gpvi Inhibitor As Antitumor Gateway Drug., Lawrence E. Goldfinger
Department of Medicine Faculty Papers
In this issue ofBlood, Volz et al establish a potential antitumor strategy byexploiting the selective requirement for platelets to maintain vascular in-tegrity within the tumor microenvironment.1Their work demonstrates, forthefirst time, that functional inhibition of platelet-specific surface receptorglycoprotein (GP) VI, using F(ab9)2fragments to avoid platelet clearance,increases intratumoral hemorrhage and concomitant tumor cell apoptosis, aswell as enhanced accumulation of chemotherapeutic drugs. These effectswork additively to inhibit tumor growth, achieving results similar to thoseachieved by platelet depletion.
Quantitative Sensory Testing In Children With Sickle Cell Disease: Additional Insights And Future Possibilities., Robin E. Miller, Dawn S. Brown, Scott W. Keith, Sarah E. Hegarty, Yamaja Setty, Claudia M. Campbell, Suzanne M. Mccahan, Suhita Gayen-Betal, Hal Byck, Marie Stuart
Quantitative Sensory Testing In Children With Sickle Cell Disease: Additional Insights And Future Possibilities., Robin E. Miller, Dawn S. Brown, Scott W. Keith, Sarah E. Hegarty, Yamaja Setty, Claudia M. Campbell, Suzanne M. Mccahan, Suhita Gayen-Betal, Hal Byck, Marie Stuart
Department of Pharmacology and Experimental Therapeutics Faculty Papers
Quantitative sensory testing (QST) is used in a variety of pain disorders to characterize pain and predict prognosis and response to specific therapies. In this study, we aimed to confirm results in the literature documenting altered QST thresholds in sickle cell disease (SCD) and assess the test-retest reliability of results over time. Fifty-seven SCD and 60 control subjects aged 8-20 years underwent heat and cold detection and pain threshold testing using a Medoc TSAII. Participants were tested at baseline and 3 months; SCD subjects were additionally tested at 6 months. An important facet of our study was the development and …
Survival Probability In Patients With Sickle Cell Anemia Using The Competitive Risk Statistical Model., Emilia Matos Do Nascimento, Clarisse Lopes De Castro Lobo, Basilio De Bragança Pereira, Samir K. Ballas
Survival Probability In Patients With Sickle Cell Anemia Using The Competitive Risk Statistical Model., Emilia Matos Do Nascimento, Clarisse Lopes De Castro Lobo, Basilio De Bragança Pereira, Samir K. Ballas
Cardeza Foundation for Hematologic Research
The clinical picture of patients with sickle cell anemia (SCA) is associated with several complications some of which could be fatal. The objective of this study is to analyze the causes of death and the effect of sex and age on survival of Brazilian patients with SCA. Data of patients with SCA who were seen and followed at HEMORIO for 15 years were retrospectively collected and analyzed. Statistical modeling was performed using survival analysis in the presence of competing risks estimating the covariate effects on a sub-distribution hazard function. Eight models were implemented, one for each cause of death. The …
Sickle Cell Disease And Variation In The Par4 Receptor, Matthew Lankiewicz, Steven E. Mckenzie, Leonard Edelstein, Stephanie Renna
Sickle Cell Disease And Variation In The Par4 Receptor, Matthew Lankiewicz, Steven E. Mckenzie, Leonard Edelstein, Stephanie Renna
Phase 1
Sickle cell disease disproportionately affects African Americans in the U.S. Much can still be learned regarding determinants of frequency and severity of painful vaso-occlusive episodes in these patients. It has been reported that a variant in PAR4 (protease-activated receptor 4) has a unique distribution among African Americans. One variant (Thr120) is hyperactive, while the other (Ala120) is hypoactive. This receptor is present on platelets, vascular cells, and nociceptors. We wish ultimately to test the hypothesis that sickle cell patients with the hyperactive PAR4 receptor have greater pain severity. A genotype-phenotype correlation would have prognostic value. An adequately powered study to …
Transcriptional Activation Of The Mir-17-92 Cluster Is Involved In The Growth-Promoting Effects Of Myb In Human Ph-Positive Leukemia Cells., Manuela Spagnuolo, Giulia Regazzo, Marco De Dominici, Andrea Sacconi, Andrea Pelosi, Etleva Korita, Francesco Marchesi, Francesco Pisani, Alessandra Magenta, Valentina Lulli, Iole Cordone, Andrea Mengarelli, Sabrina Strano, Giovanni Blandino, Maria G. Rizzo, Bruno Calabretta
Transcriptional Activation Of The Mir-17-92 Cluster Is Involved In The Growth-Promoting Effects Of Myb In Human Ph-Positive Leukemia Cells., Manuela Spagnuolo, Giulia Regazzo, Marco De Dominici, Andrea Sacconi, Andrea Pelosi, Etleva Korita, Francesco Marchesi, Francesco Pisani, Alessandra Magenta, Valentina Lulli, Iole Cordone, Andrea Mengarelli, Sabrina Strano, Giovanni Blandino, Maria G. Rizzo, Bruno Calabretta
Department of Cancer Biology Faculty Papers
MicroRNAs, non-coding regulators of gene expression, are likely to function as important downstream effectors of many transcription factors including MYB. Optimal levels of MYB are required for transformation/maintenance of BCR-ABL-expressing cells. We investigated whether MYB silencing modulates microRNA expression in Philadelphia-positive (Ph+) leukemia cells and if MYB-regulated microRNAs are important for the "MYB addiction" of these cells. Thirty-five microRNAs were modulated by MYB silencing in lymphoid and erythromyeloid chronic myeloid leukemia-blast crisis BV173 and K562 cells; 15 of these were concordantly modulated in both lines. We focused on the miR-17-92 cluster because of its oncogenic role in tumors and found …