Zobrazeno 1 - 10
of 65
pro vyhledávání: '"M. Domenica Cappellini"'
Publikováno v:
Mediterranean Journal of Hematology and Infectious Diseases, Vol 1, Iss 1, Pp e2009029-e2009029 (2009)
As the life expectancy of β-thalassemia patients has markedly improved over the last decade, several new complications are being recognized. The presence of a high incidence of thromboembolic events, mainly in thalassemia intermedia patients, has le
Externí odkaz:
https://doaj.org/article/1959172124c84f58bd5efc86237c874d
Publikováno v:
Mediterranean Journal of Hematology and Infectious Diseases, Vol 1, Iss 1, Pp e2009029-e2009029 (2009)
As the life expectancy of β-thalassemia patients has markedly improved over the last decade, several new complications are being recognized. The presence of a high incidence of thromboembolic events, mainly in thalassemia intermedia patients, has le
Externí odkaz:
https://doaj.org/article/ae9286a44f1348329bb1b2349aed550f
Publikováno v:
New England Journal of Medicine. 384:727-743
Autor:
Vip Viprakasit, Frank Richard, Vania Manolova, Franz Dürrenberger, John B. Porter, Thomas D. Coates, Ali T. Taher, Maciej W Garbowski, M. Domenica Cappellini, Antonis Kattamis
Publikováno v:
Expert review of hematology. 14(7)
In β-thalassemia, imbalanced globin synthesis causes reduced red blood cell survival and ineffective erythropoiesis. Suppressed hepcidin levels increase ferroportin-mediated iron transport in enterocytes, causing increased iron absorption and potent
Autor:
Angela Yen, Damiano Rondelli, la Fuente Josu de, Amanda M. Li, Jennifer Domm, Montalembert Mariane de, Tony W. Ho, Donna A. Wall, Brenda K. Eustace, Juergen Foell, Sujit Sheth, Haydar Frangoul, Rupert Handgretinger, Akshay Sharma, Sandeep Soni, Antonis Kattamis, Andrew Kernytsky, Franco Locatelli, Stephan A. Grupp, M. Domenica Cappellini, Julie A. Lekstrom-Himes, Selim Corbacioglu, David Altshuler, Markus Y. Mapara, Yi-Shan Chen, Martin H. Steinberg
Transfusion-dependent β-thalassemia (TDT) and sickle cell disease (SCD) are severe monogenic diseases with severe and potentially life-threatening manifestations. BCL11A is a transcription factor that represses γ-globin expression and fetal hemoglo
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::294f2984b8848005c8f160412c9178cd
https://hdl.handle.net/10807/228473
https://hdl.handle.net/10807/228473
Autor:
Antonis Kattamis, Soo-Min Lim, Silverio Perrotta, Jeevan K Shetty, Yesim Aydinok, Matthew L. Sherman, Kevin H. Kuo, Ersi Voskaridou, John Porter, Gian Luca Forni, Vip Viprakasit, Peter G. Linde, Abderrahmane Laadem, Pencho Georgiev, Jun Zou, Mohamed Bejaoui, Lee-Ping Chew, Meng-Yao Lu, Efthymia Vlachaki, Penka Ganeva, Believe Investigators, Ashutosh Lal, Olivier Hermine, Idit Pazgal-Kobrowski, Antonio Piga, Farrukh Shah, M. Domenica Cappellini, Liana Gercheva, Alexis A. Thompson, Ali T Taher, Adisak Tantiworawit, Raffaella Origa, Ellis J Neufeld, Abderrahim Khelif, P Joy Ho, Thomas D. Coates, Jennie Zhang, Hong-Keng Liew, Ping Chong Bee, Dimana Miteva, Tatiana Zinger
BACKGROUND Patients with transfusion-dependent ?-thalassemia need regular red-cell transfusions. Luspatercept, a recombinant fusion protein that binds to select transforming growth factor ? superfamily ligands, may enhance erythroid maturation and re
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::e4fe5db0b5c20202be3afeae19191200
http://hdl.handle.net/11591/430144
http://hdl.handle.net/11591/430144
Publikováno v:
New England Journal of Medicine. 384:2165-2166
Autor:
M. Domenica Cappellini, Irene Motta
Publikováno v:
Hematology. 2017:278-283
β-Thalassemias are characterized by reduced production of β-globin chain, resulting in α/β-chain unbalance and precipitation of α-globin–heme complexes and determining ineffective erythropoiesis. Ineffective erythropoiesis, chronic hemolytic a
Autor:
Jingjing Zhang, Stephanie Fowler, Christophe Lechauve, Mondira Kundu, Heather Tillman, Abdullah Freiwan, Irene Motta, Paola Delbini, Eugene Khandros, Kalin Mayberry, M. Domenica Cappellini, Christopher S. Thom, Mitchell J. Weiss, Emilio Boada Romero, Julia Keith
Publikováno v:
Sci Transl Med
In β-thalassemia, accumulated free α-globin forms intracellular precipitates that impair erythroid cell maturation and viability. Protein quality control systems mitigate β-thalassemia pathophysiology by degrading toxic free α-globin, although th
Autor:
Antonis Kattamis, M. Domenica Cappellini, John B. Porter, Ali T. Taher, Renzo Galanello, Vip Viprakasit, Khaled M. Musallam
Publikováno v:
European Journal of Internal Medicine. 28:91-96
Background Patients with non-transfusion-dependent thalassemia (NTDT) present with a spectrum of disease severities. Since there are multiple pathophysiologies in such patients, tailoring treatment remains essential. Therefore, one simple, reliable t