Search Results - "Grady, Robert W."

Bone loss caused by iron overload in a murine model: importance of oxidative stress by Tsay, Jaime, Yang, Zheiwei, Ross, F. Patrick, Cunningham-Rundles, Susanna, Lin, Hong, Coleman, Rhima, Mayer-Kuckuk, Philipp, Doty, Stephen B., Grady, Robert W., Giardina, Patricia J., Boskey, Adele L., Vogiatzi, Maria G.

“…Osteoporosis is a frequent problem in disorders characterized by iron overload, such as the thalassemias and hereditary hemochromatosis. The exact role of iron…”
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Anemia, Ineffective Erythropoiesis, and Hepcidin: Interacting Factors in Abnormal Iron Metabolism Leading to Iron Overload in β-Thalassemia by Gardenghi, Sara, PhD, Grady, Robert W., PhD, Rivella, Stefano, PhD

“…β-Thalassemia is a genetic disorder caused by mutations in the β-globin gene and characterized by chronic anemia caused by ineffective erythropoiesis, and…”
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Bone Disease in Thalassemia: A Frequent and Still Unresolved Problem by Vogiatzi, Maria G, Macklin, Eric A, Fung, Ellen B, Cheung, Angela M, Vichinsky, Elliot, Olivieri, Nancy, Kirby, Melanie, Kwiatkowski, Janet L, Cunningham, Melody, Holm, Ingrid A, Lane, Joseph, Schneider, Robert, Fleisher, Martin, Grady, Robert W, Peterson, Charles C, Giardina, Patricia J

“…Adults with β thalassemia major frequently have low BMD, fractures, and bone pain. The purpose of this study was to determine the prevalence of low BMD,…”
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Therapeutic hemoglobin levels after gene transfer in β-thalassemia mice and in hematopoietic cells of β-thalassemia and sickle cells disease patients by Breda, Laura, Casu, Carla, Gardenghi, Sara, Bianchi, Nicoletta, Cartegni, Luca, Narla, Mohandas, Yazdanbakhsh, Karina, Musso, Marco, Manwani, Deepa, Little, Jane, Gardner, Lawrence B, Kleinert, Dorothy A, Prus, Eugenia, Fibach, Eitan, Grady, Robert W, Giardina, Patricia J, Gambari, Roberto, Rivella, Stefano

“…Preclinical and clinical studies demonstrate the feasibility of treating β-thalassemia and Sickle Cell Disease (SCD) by lentiviral-mediated transfer of the…”
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Toward optimizing the use of deferasirox: potential benefits of combined use with deferoxamine by Grady, Robert W, Galanello, Renzo, Randolph, Rachel E, Kleinert, Dorothy A, Dessi, Carlo, Giardina, Patricia J

“…Patients with β-thalassemia require iron chelation therapy to protect against progressive iron overload and non-transferrin-bound iron. Some patients fail to…”
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Enhanced erythropoiesis in Hfe-KO mice indicates a role for Hfe in the modulation of erythroid iron homeostasis by Ramos, Pedro, Guy, Ella, Chen, Nan, Proenca, Catia C., Gardenghi, Sara, Casu, Carla, Follenzi, Antonia, Van Rooijen, Nico, Grady, Robert W., de Sousa, Maria, Rivella, Stefano

“…In hereditary hemochromatosis, mutations in HFE lead to iron overload through abnormally low levels of hepcidin. In addition, HFE potentially modulates…”
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Macrophages support pathological erythropoiesis in polycythemia vera and β-thalassemia by Ramos, Pedro, Casu, Carla, Gardenghi, Sara, Breda, Laura, Crielaard, Bart J, Guy, Ella, Marongiu, Maria Franca, Gupta, Ritama, Levine, Ross L, Abdel-Wahab, Omar, Ebert, Benjamin L, Van Rooijen, Nico, Ghaffari, Saghi, Grady, Robert W, Giardina, Patricia J, Rivella, Stefano

“…Ramos et al. report a crucial role for macrophages in erythroblast development in mice. Under conditions that induce new red blood cell formation, macrophage…”
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Hepcidin as a therapeutic tool to limit iron overload and improve anemia in β-thalassemic mice by Gardenghi, Sara, Ramos, Pedro, Marongiu, Maria Franca, Melchiori, Luca, Breda, Laura, Guy, Ella, Muirhead, Kristen, Rao, Niva, Roy, Cindy N, Andrews, Nancy C, Nemeth, Elizabeta, Follenzi, Antonia, An, Xiuli, Mohandas, Narla, Ginzburg, Yelena, Rachmilewitz, Eliezer A, Giardina, Patricia J, Grady, Robert W, Rivella, Stefano

“…Excessive iron absorption is one of the main features of β-thalassemia and can lead to severe morbidity and mortality. Serial analyses of β-thalassemic mice…”
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Combined iron chelation therapy by Galanello, Renzo, Agus, Annalisa, Campus, Simona, Danjou, Fabrice, Giardina, Patricia J., Grady, Robert W.

“…Patients with thalassemia major accumulate body iron over time as a consequence of continuous red blood cell transfusions which cause hepatic, endocrine, and…”
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Ineffective erythropoiesis in β-thalassemia is characterized by increased iron absorption mediated by down-regulation of hepcidin and up-regulation of ferroportin by Gardenghi, Sara, Marongiu, Maria F., Ramos, Pedro, Guy, Ella, Breda, Laura, Chadburn, Amy, Liu, YiFang, Amariglio, Ninette, Rechavi, Gideon, Rachmilewitz, Eliezer A., Breuer, William, Cabantchik, Z. Ioav, Wrighting, Diedra M., Andrews, Nancy C., de Sousa, Maria, Giardina, Patricia J., Grady, Robert W., Rivella, Stefano

“…Progressive iron overload is the most salient and ultimately fatal complication of β-thalassemia. However, little is known about the relationship among…”
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Differences in the prevalence of growth, endocrine and vitamin D abnormalities among the various thalassaemia syndromes in North America by Vogiatzi, Maria G., Macklin, Eric A., Trachtenberg, Felicia L., Fung, Ellen B., Cheung, Angela M., Vichinsky, Elliott, Olivieri, Nancy, Kirby, Melody, Kwiatkowski, Janet L., Cunningham, Melody, Holm, Ingrid A., Fleisher, Martin, Grady, Robert W., Peterson, Charles M., Giardina, Patricia J.

“…Summary This study aimed to determine differences in the rates of growth, endocrine‐ and calcium‐related abnormalities in the various thalassemia syndromes in…”
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Effect of Transfusional Iron Overload on Immune Response by Cunningham-Rundles, Susanna, Giardina, Patricia J., Grady, Robert W., Califano, Claudia, McKenzie, Priscilla, De Sousa, Maria

“…Increased susceptibility to infectious disease is observed in persons with transfusion-dependent thalassemia and iron overload who experience increased…”
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Decreased differentiation of erythroid cells exacerbates ineffective erythropoiesis in β-thalassemia by Libani, Ilaria V., Guy, Ella C., Melchiori, Luca, Schiro, Raffaella, Ramos, Pedro, Breda, Laura, Scholzen, Thomas, Chadburn, Amy, Liu, YiFang, Kernbach, Margrit, Baron-Lühr, Bettina, Porotto, Matteo, de Sousa, Maria, Rachmilewitz, Eliezer A., Hood, John D., Cappellini, M. Domenica, Giardina, Patricia J., Grady, Robert W., Gerdes, Johannes, Rivella, Stefano

“…In β-thalassemia, the mechanism driving ineffective erythropoiesis (IE) is insufficiently understood. We analyzed mice affected by β-thalassemia and observed,…”
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Effectiveness and safety of ICL670 in iron-loaded patients with thalassaemia: a randomised, double-blind, placebo-controlled, dose-escalation trial by Nisbet-Brown, Eric, Olivieri, Nancy F, Giardina, Patricia J, Grady, Robert W, Neufeld, Ellis J, Séchaud, Romain, Krebs-Brown, Axel J, Anderson, Judith R, Alberti, Daniele, Sizer, Kurt C, Nathan, David G

“…Transfusional iron overload is a potentially fatal complication of the treatment of thalassaemia. We aimed to investigate short-term efficacy,…”
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Increased survival and reversion of iron-induced cardiac disease in patients with thalassemia major receiving intensive combined chelation therapy as compared to desferoxamine alone by Lai, Maria Eliana, Grady, Robert W., Vacquer, Stefania, Pepe, Alessia, Carta, Maria Paola, Bina, Patrizio, Sau, Franco, Cianciulli, Paolo, Maggio, Aurelio, Galanello, Renzo, Farci, Patrizia

“…Myocardial iron overload is the leading cause of death in patients with β-thalassemia major. An intensification monotherapy with deferoxamine (DFO) as well as…”
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Iron chelation therapy in thalassemia major: A systematic review with meta-analyses of 1520 patients included on randomized clinical trials by Maggio, Aurelio, Filosa, Aldo, Vitrano, Angela, Aloj, Giuseppina, Kattamis, Antonis, Ceci, Adriana, Fucharoen, Suthat, Cianciulli, Paolo, Grady, Robert W., Prossomariti, Luciano, Porter, John B., Iacono, Angela, Cappellini, Maria Domenica, Bonifazi, Fedele, Cassarà, Filippo, Harmatz, Paul, Wood, John, Gluud, Christian

“…The effectiveness of deferoxamine (DFO), deferiprone (DFP), or deferasirox (DFX) in thalassemia major was assessed. Outcomes were reported as means ± SD, mean…”
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Decreased hepcidin expression in murine β-thalassemia is associated with suppression of Bmp/Smad signaling by Parrow, Nermi L., Gardenghi, Sara, Ramos, Pedro, Casu, Carla, Grady, Robert W., Anderson, Erik R., Shah, Yatrik M., Li, Huihui, Ginzburg, Yelena Z., Fleming, Robert E., Rivella, Stefano

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Iron metabolism and ineffective erythropoiesis in β-thalassemia mouse models by Ramos, Pedro, Melchiori, Luca, Gardenghi, Sara, Van-Roijen, Nico, Grady, Robert W., Ginzburg, Yelena, Rivella, Stefano

“…β‐thalassemia is a disease associated with decreased β‐globin production leading to anemia, ineffective erythropoiesis, and iron overload. New mechanisms…”
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Hepcidin and Hfe in iron overload in β-thalassemia by Gardenghi, Sara, Ramos, Pedro, Follenzi, Antonia, Rao, Niva, Rachmilewitz, Eliezer A., Giardina, Patricia J., Grady, Robert W., Rivella, Stefano

“…Hepcidin (HAMP) negatively regulates iron absorption, degrading the iron exporter ferroportin at the level of enterocytes and macrophages. We showed that mice…”
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Phase Ib clinical trial of starch‐conjugated deferoxamine (40SD02): a novel long‐acting iron chelator by Harmatz, Paul, Grady, Robert W., Dragsten, Paul, Vichinsky, Elliott, Giardina, Patricia, Madden, Jacqueline, Jeng, Michael, Miller, Becky, Hanson, Gregory, Hedlund, Bo

“…Summary The most widely used drug for iron chelation is deferoxamine (DFO) mesylate. While effective in promoting iron excretion, it requires prolonged daily…”
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