Hematopoietic Stem Cell Transplantation in Thalassemia and Sickle Cell Disease. Unicenter Experience in a Multi-Ethnic Population.

Hematopoietic stem cell transplantation (HSCT) still remains the only definitive cure currently available for patients with thalassemia and sickle cell anemia. Results of transplant in thalassemia and in sickle cell anemia have steadily improved over the last two decades due to improvements in preventive strategies, and effective control of transplant-related complications. From 2004 through 2009, 145 consecutive patients with thalassemia and sickle cell anemia, ethnically heterogeneous from Mediterranean and Middle East countries, were given HSCT in the International Center for Transplantation in Thalassemia and Sickle Cella Anemia in Rome. This experience is characterized by two peculiarities: patients were ethnically very heterogeneous and the vast majority of these patients were not regularly transfesed/chelated and therefore were highly sensitized due to RBC transfusions without leukodepletion filters. Consequently, they could have a high risk of graft rejection as a result of sensitization to HLA antigens. The Rome experience of SCT in patients with thalassemia and sickle cell anemia confirmed the results obtained in Pesaro, and most importantly showed the reproducibility of these results in other centers

Acute mediastinitis: multidetector computed tomography findings following cardiac surgery

Mediastinite pós-cirurgias torácicas é definida como a infecção dos órgãos e tecidos do espaço mediastinal, ocorrendo em 0,4% a 5% dos casos. A gravidade da infecção pós-operatória varia desde infecção de tecidos superficiais da parede torácica até mediastinite fulminante com envolvimento esternal. O critério diagnóstico da tomografia computadorizada para mediastinite aguda pós-cirúrgica é a presença de coleção mediastinal, podendo estar associada ou não a anormalidades periesternais como edema/borramento de partes moles, separação dos segmentos esternais com reabsorção óssea marginal, esclerose e osteomielite. Achados associados incluem linfonodomegalias, consolidações pulmonares e derrame pleural e pericárdico. Pequenas coleções e gás mediastinais podem ser usualmente encontradas em pós-operatório recente de cirurgias torácicas sem a presença de infecções, limitando a eficácia da tomografia computadorizada nas duas primeiras semanas. Após esse período, a tomografia alcança quase 100% de sensibilidade e especificidade. Pacientes com suspeita clínica de mediastinite devem ser submetidos a exame de tomografia para pesquisa de coleções, identificando a extensão da doença e sua natureza. A versão de multidetectores propicia recursos de reconstruções em diversos planos e janelas, contribuindo especialmente para o estudo do esterno.Postoperative mediastinitis is defined as an infection of the organs and tissues in the mediastinal space, with an incidence ranging between 0.4% and 5% of cases. This disease severity varies from infection of superficial tissues in the chest wall to fulminant mediastinitis with sternal involvement. Diagnostic criterion for postoperative detection of acute mediastinitis at computed tomography is the presence of fluid collections and gas in the mediastinal space, which might or might not be associated with peristernal abnormalities such as edema of soft tissues, separation of sternal segments with marginal bone resorption, sclerosis and osteomyelitis. Other associated findings include lymphadenomegaly, pulmonary consolidation and pleural/pericardial effusion. Some of these findings, such as mediastinal gas and small fluid collections can be typically found in the absence of infection, early in the period following thoracic surgery where the effectiveness of computed tomography is limited. After approximately two weeks, computed tomography achieves almost 100% sensitivity and specificity. Patients with clinical suspicion of mediastinitis should be submitted to computed tomography for investigating the presence of fluid collections to identify the extent and nature of the disease. Multidetector computed tomography allows 3D images reconstruction, contributing particularly to the evaluation of the sternum

IUC Independent Policy Report: At the End of the End of History

The IUC Independent Policy Report was drafted by the IUC Legal Standards Research Group, organized by a Steering Committee chaired by Ugo Mattei (International University College of Turin), coordinated by Edoardo Reviglio (International University College of Turin) and Giuseppe Mastruzzo (International University College of Turin), and composed by Franco Bassanini (University of Rome “La Sapienza”), Guido Calabresi (Yale University), Antoine Garapon (Institut des Hautes Etudes sur la Justice, Paris), and Tibor Varady (Central European University, Budapest). Contributors include Eugenio Barcellona (Eastern Piedmont University), Mauro Bussani (University of Trieste), Giuliano G. Castellano (Ecole Polytechnique Preg/CRG), Moussa Djir´e (Bamako University), Liu Guanghua (Lanzhou University), Golnoosh Hakimdavar (University of Turin), John Haskell (SOAS), Jedidiah J. Kroncke (Yale Law School), Andrea Lollini (Bologna University), Alberto Lucarelli (Federico II University), Boris N. Mamlyuk, (University of Turin), Alberto Monti (Bocconi University), Sergio Ariel Muro (Torquato di Tella University), Domenico Nicol`o (Mediterranean University of Reggio Calabria), and Nicola Sartori (University of Michigan). The IUC Independent Policy Report argues for a radical change of perspective, capable of restoring the supremacy of the law over the economic system. It is not only about finance, nor is it only about economics or policy. In this sense a transnational set of normative principles is needed in order to establish a global legal system capable of controlling economic processes, rather than being controlled by them. Within this framework a series of policy proposals are presented in order to effectively implement a new system of global standards. The current Western standard of living is unsustainable. Should the rest share the model of development of the West, our planet will simply not be capable of resisting the growth in consumption and pollution. Within this fundamental setting of scarcity in resources, using the rhetoric of the end of history as the polar star for growth, development and ultimately happiness of the whole world is simply a cynical lie. We argue here for the beginning of a necessary process aimed at the development of a legal system that is much less about creating an effcient backbone for an exploitive economy and much more about a vision of civilization, justice and respect where the laws of nature and those of humans converge in a sustainable long-term philosophy. Principles of justice, responsibility and long term environmental protection, rather than short term economic contingency and strong interests must set the legal agenda. A new governance and bottom-up inclusive integration of knowledge-based economies (wherever located), which is crucial to the very survival of humankind, cannot happen without defning new terms of a widely accepted standard of long term justice in the transnational context, hence the urgency to conceive legitimate transnational legal structures and possibly some apparatus of “superlegality.” The report is composed of fve sections. After having presented the pitfalls of the prevailing theoretical apparatus, an alternative cultural grid upon which policy actions should be shaped is presented. In this sense several normative proposals - revisiting the key characteristics of the current system - are offered aiming at acquiring a wider perspective over the actual global crisis

L'economia politica e l'evoluzione del capitalismo negli ultimi quarant'anni

Convegno in onore di Giorgio Lunghini

IUC Independent Policy Report: At the End of the End of History: Global Legal Standards: Part of the Solution or Part of the Problem?

This draft was presented at the seminar, Global Standards in the 21st Century, organized by the G8 Presidency in Rome at the Ministry of Economy and Finance and Villa Madama on the 11-12th of May 2009. The IUC Independent Policy Report was drafted by the IUC Legal Standards Research Group, organized by a Steering Committee chaired by Ugo Mattei (International University College of Turin), coordinated by Edoardo Reviglio (International University College of Turin) and Giuseppe Mastruzzo (International University College of Turin),The IUC Independent Policy Report prepared by a group of lawyers at the International University College of Turin was presented at the meeting convened by the G8 Presidency in Rome on May 12, 2009.The IUC Independent Policy Report was drafted by the IUC Legal Standards Research Group, organized by a Steering Committee chaired by Ugo Mattei (International University College of Turin), coordinated by Edoardo Reviglio (International University College of Turin) and Giuseppe Mastruzzo (International University College of Turin), and composed by Franco Bassanini (University of Rome “La Sapienza”), Guido Calabresi (Yale University), Antoine Garapon (Institut des Hautes Etudes sur la Justice, Paris), and Tibor Varady (Central European University, Budapest). Contributors include Eugenio Barcellona (Eastern Piedmont University), Mauro Bussani (University of Trieste), Giuliano G. Castellano (Ecole Polytechnique Preg/CRG), Moussa Djir´e (Bamako University), Liu Guanghua (Lanzhou University), Golnoosh Hakimdavar (University of Turin), John Haskell (SOAS), Jedidiah J. Kroncke (Yale Law School), Andrea Lollini (Bologna University), Alberto Lucarelli (Federico II University), Boris N. Mamlyuk, (University of Turin), Alberto Monti (Bocconi University), Sergio Ariel Muro (Torquato di Tella University), Domenico Nicol`o (Mediterranean University of Reggio Calabria), and Nicola Sartori (University of Michigan). The IUC Independent Policy Report argues for a radical change of perspective, capable of restoring the supremacy of the law over the economic system. It is not only about finance, nor is it only about economics or policy. In this sense a transnational set of normative principles is needed in order to establish a global legal system capable of controlling economic processes, rather than being controlled by them. Within this framework a series of policy proposals are presented in order to effectively implement a new system of global standards. The current Western standard of living is unsustainable. Should the rest share the model of development of the West, our planet will simply not be capable of resisting the growth in consumption and pollution. Within this fundamental setting of scarcity in resources, using the rhetoric of the end of history as the polar star for growth, development and ultimately happiness of the whole world is simply a cynical lie. We argue here for the beginning of a necessary process aimed at the development of a legal system that is much less about creating an effcient backbone for an exploitive economy and much more about a vision of civilization, justice and respect where the laws of nature and those of humans converge in a sustainable long-term philosophy. Principles of justice, responsibility and long term environmental protection, rather than short term economic contingency and strong interests must set the legal agenda. A new governance and bottom-up inclusive integration of knowledge-based economies (wherever located), which is crucial to the very survival of humankind, cannot happen without defning new terms of a widely accepted standard of long term justice in the transnational context, hence the urgency to conceive legitimate transnational legal structures and possibly some apparatus of “superlegality.” The report is composed of fve sections. After having presented the pitfalls of the prevailing theoretical apparatus, an alternative cultural grid upon which policy actions should be shaped is presented. In this sense several normative proposals - revisiting the key characteristics of the current system - are offered aiming at acquiring a wider perspective over the actual global crisi

Correction of beta-thalassemia major by gene transfer in haematopoietic progenitors of pediatric patients

Beta-thalassemia is a common monogenic disorder due to mutations in the beta-globin gene and gene therapy, based on autologous transplantation of genetically corrected haematopoietic stem cells (HSCs), holds the promise to treat patients lacking a compatible bone marrow (BM) donor. We recently showed correction of murine beta-thalassemia by gene transfer in HSCs with the GLOBE lentiviral vector (LV), expressing a transcriptionally regulated human beta-globin gene. Here, we report successful correction of thalassemia major in human cells, by studying a large cohort of pediatric patients of diverse ethnic origin, carriers of different mutations and all candidates to BM transplantation. Extensive characterization of BM-derived CD34(+) cells before and following gene transfer shows the achievement of high frequency of transduction, restoration of haemoglobin A synthesis, rescue from apoptosis and correction of ineffective erythropoiesis. The procedure does not significantly affect the differentiating potential and the relative proportion of haematopoietic progenitors. Analysis of vector integrations shows preferential targeting of transcriptionally active regions, without bias for cancer-related genes. Overall, these results provide a solid rationale for a future clinical translation

AGILE TGFS AND GLOBAL LIGHTNING ACTIVITY

[1] The AGILE satellite detects Terrestrial Gamma-ray Flashes (TGFs) in the 0.35–100 MeV energy range using its Mini-Calorimeter (MCAL) instrument with an average detection rate of 10 TGFs/month. Thanks to its Low Earth Orbit with only 2.5 degree of inclination, AGILE guarantees an unprecedented exposure above the equator, where both lightning activity and TGF detection peak. Here we report the comparison between the AGILE TGFs detected between March 2009 and February 2010 and full climatology lightning worldwide distribution based on satellite optical observations from LIS (Lightning Imaging Sensor) and OTD (Optical Transient Detector) instruments. This approach is complementary to the one-to-one TGF/lightning correlations by ground-based sferics measurements. Based on mono and bi-dimensional Kolmogorov-Smirnov tests, we show that the AGILE TGFs and time-averaged global lightning in the equatorial area are not drawn from the same distribution. However, we find significant regional differences in the degree of correlation as well as in the TGF/lightning ratio. In the case of south east Asia we find a 87% probability for the TGF and lightning being samples of the same distribution. This result supports the idea that the physical conditions at play in TGF generation can have strong geographical and climatological modulation. Based on the assumption that the observed range of TGF/flash ratio holds at all latitudes we can estimate a global rate of ≃ 220 ÷ 570 TGFs per day. The observed TGF/flash geographical modulation as well as the TGF global rate estimate are in agreement with previous observations