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ELEVATA INCIDENZA DI LINFOMI INDOLENTI NON FOLLICOLARI NEI PAZIENTI CON CARENZA ACQUISITA DI INIBITORI DELLA C1 ESTERASI
No full textI linfomi della zona marginale sono malattie linfoproliferative rare, e rappresentano, nella popolazione generale, circa il 10% di tutti i linfomi non Hodgkin. I pazienti con carenza acquisita di inibitore della C1 esterasi, rappresentano una popolazione ad elevato rischio di sviluppare malattie linfoproliferative, si calcola infatti che il 33% di tali pazienti presenta o presenterà una malattia linfoproliferativa conclamata.
In questo studio abbiamo valutato una casistica di 72 pazienti affetti da carenza acquisita di inibitore della C1 esterasi seguiti nel nostro centro per 15 anni (range 1-24 anni) allo scopo di valutare la prevalenza della malattie linfoproliferative in tale popolazione e se particolari istotipi di malattia linfoproliferativa prevalgono in questa popolazione rispetto alla popolazione generale. I pazienti presentano una età mediana di 71 anni (range 64-79 anni), l’età mediana all’ insorgenza dei sintomi è di 57,5 anni (range 50-66 anni).
Ventiquattro pazienti presentavano malattie linfoproliferative. Venti pazienti presentavano malattie linfoproliferative indolenti di tipo non follicolare (il 75% costituito da linfomi marginali splenici), un paziente ha presentato un linfoma follicolare, mentre tre pazienti hanno presentato malattie linfoproliferative ad elevato grado di malignità (2 linfomi diffusi a grandi cellule B ed un linfoma mantellare). La malattia linfoproliferativa è stata diagnosticata all’ insorgenza dei sintomi di angioedema in 15 pazienti nei rimanenti durante il decorso dell’angioedema.
2 dei 24 pazienti sono in “watchful wait” pertanto allo stato attuale non ricevano alcun trattamento per la malattia linfoproliferativa. 13 pazienti hanno ricevuto un trattamento chemioterapico e due pazienti sono stati trattati con Rituximab. La sola splenectomia è stata effettuata in 3 pazienti.
Tutti i pazienti trattati per la malattia linfoproliferativa con trattamento chemio/immunoterapico o splenectomia hanno sperimentato un miglioramento dei sintomi clinici di angioedema o un miglioramento dei parametri complementari.
Il nostro studio suggerisce che, i pazienti con carenza acquisita di C1-inibitore hanno una elevata prevalenza di linfomi della zona marginale rispetto alla popolazione generale. L’ origine post-germinale di tali malattie linfoproliferative, suggerisce che la stimolazione antigenica continua svolge un ruolo fondamentale nella patogenesi di tale condizioni.
La proliferazione clonale B cellulare rappresenta un denominatore comune nei pazienti con carenza acquisita di C1-inibitore sia nei pazienti che presentano anticorpi inattivanti C1 inibitore che in quelli che sviluppano malattia linfoproliferativa.Marginal zone lymphoma represents about 10% of all non-Hodgkin lymphomas
(NHLs). 33% of patients with acquired angioedema (AAE) due to
acquired C1-inhibitor (C1-INH) deficiency (C1-INH-AAE) have or will
develop NHLs. C1-INH-AAE is a rare condition. We report the follow-up
of 72 C1-INH-AAE patients, followed for a median of 15 years (range 1–
24). Median age was 71 (range 64–79) years; median age at onset of
angioedema symptoms was 575 (range 50–66) years and it was 63 [range
45–80) years at diagnosis]. Twenty patients were diagnosed with low-grade
non-follicular B-cell lymphomas (75% were splenic MZL), one with follicular
and three with high-grade lymphomas (two diffuse large B-cell lymphomas
and one mantle cell lymphoma). Fifteen NHLs were diagnosed at
onset of AAE or thereafter (3 months to 7 years), eight had already been
diagnosed at onset of angioedema. Two of 24 patients remain on watchful
wait. Thirthen of 24 received chemotherapy, two received rituximab. Three
underwent splenectomy. All 18 patients receiving therapy for NHL experienced
post-treatment reduction in AAE symptoms. Our study suggests that
clonal B-cell proliferation is the pathology underlying AAE leading to production
of C1-INH-neutralizing autoantibodies and to NHLs. The postgerminal
centre origin of NHL suggests that immune stimulation may contribute
to lymphomagenesis
Going Beyond Counting First Authors in Author Co-citation Analysis
Full text linkThe present study examines one of the fundamental aspects of author co-citation analysis (ACA) - the way co-citation
counts are defined. Co-citation counting provides the data on which all subsequent statistical analyses and mappings
are based, and we compare ACA results based on two different types of co-citation counting - the traditional type that
only counts the first one among a cited work's authors on the one hand and a non-traditional type that takes into
account the first 5 authors of a cited work on the other hand. Results indicate that the picture produced through this non-traditional author co-citation counting contains more coherent author groups and is therefore considerably clearer. However, this picture represents fewer specialties in the research field being studied than that produced through the traditional first-author co-citation counting when the same number of top-ranked authors is selected and analyzed. Reasons for these effects are discussed
DGPS e paesaggi archeologici: il caso di studio della necropoli di Fehwet (Libia)
No full textThe study area corresponds to south-western Fezzan (Libya), a desert area with a few tiny villages and an almost total lack of roads. The first topographical maps of this area that were made available to the public and were used mostly by tourists visiting the country, were the ones made by the Russians on a scale of 1:200000 and 1:500000. These maps, as can be seen, are very difficult to read and are of almost no use for a detailed analysis of the area on a vast scale. Since 1996 our mission has been using Landsat TM satellite images, which are ideal for arge scale territorial survey work. The combined use of these images with pocket GPS has made it possible to effect highly effective survey work, with the development of extremely useful "specific distributive maps" , an example of which is shown here. Of course, in this type of work a certain approximation must be accepted because of the relatively low resolution of the images and the imprecision of the pocket GPS which may be off even 10-20 meters. Up until a few years ago the technology available did not seem to be adequate for intermediate scale surveys; the resolution was too low for the Landsat photos, the pocket GPS were not precise enough, and the use of the Total Station was too complicatedto the point of being prohibitive. Now, the use of the DGPS combined with the high resolution Ikonos images has made it possible to program medium to large scale relief surveys, like the one initiated at the necropolis of Fehwet
Variations on the Author
Full text link“Variations on the Author” discusses two of Eduardo Coutinho’s recent films (Um Dia na Vida, from 2010, and Últimas Conversas, posthumously released in 2015) and their contribution to the general question of documentary authorship. The director’s filmography is characterized by a consistent yet self-effacing form of authorial self-inscription: Coutinho often features as an interviewer that rather than express opinions propels discourses; an interviewer that is good at listening. This mode of self-inscription characterizes him as an author who is not expressive but who is nonetheless markedly present on the screen. In Um Dia na Vida, however, Coutinho is completely absent form the image, while Últimas Conversas, on the contrary, includes a confessional prologue that moves the director from the margins to the center of his films. This article examines the ways in which these works stand out in the filmography of a director who offers new insights into the notion of cinematic authorship
Appropriate Similarity Measures for Author Cocitation Analysis
Full text linkWe provide a number of new insights into the methodological discussion about author cocitation analysis. We first argue that the use of the Pearson correlation for measuring the similarity between authors’ cocitation profiles is not very satisfactory. We then discuss what kind of similarity measures may be used as an alternative to the Pearson correlation. We consider three similarity measures in particular. One is the well-known cosine. The other two similarity measures have not been used before in the bibliometric literature. Finally, we show by means of an example that our findings have a high practical relevance.information science;Pearson correlation;cosine;similarity measure;author cocitation analysis
Optimization of a Darrieus Vertical-Axis Wind Turbine Using Blade Element Momentum theory and Evolutionary Algorithms
Full text linkWind turbine design procedures usually involve the adoption of the blade element e momentum theory.
Nevertheless, its use is limited by the lack of extended database regarding the aerodynamic coefficients
for most used airfoils. In the present work, an extended database generation procedure for symmetric
profiles is discussed and validated with the aim of adopting numerical optimization methods for verticalaxis
wind turbine design.
Evolutionary algorithms are thereby utilized to provide optimal configurations for different design
objectives. The pure performance and the annual energy production are here considered in order to
show the capabilities of the numerical code. A relevant increase in performance is achieved for all the
obtained results, showing that the numerical optimization can be successfully adopted in vertical-axis
wind turbine design procedures
Iron overload and thalassemia
No full textFor nearly 30 years, patients with transfusional iron overload have depended on nightly deferoxamine infusions for iron chelation. Despite dramatic gains in life expectancy in the deferoxamine era for patients with transfusion-dependent anemias, the leading cause of death for young adults with thalassemia major and related disorders has been cardiac disease from myocardial iron deposition. Strategies to reduce cardiac disease by improving chelation regimens have been of the highest priority. These strategies have included development of novel oral iron chelators to improve compliance, improved assessment of cardiac iron status, and careful epidemiologic assessment of European outcomes with deferiprone, an oral alternative chelator available for about a decade. Each of these strategies is now bearing fruit. The novel oral chelator deferasirox was recently approved by the Food and Drug Administration (FDA); a randomized clinical trial demonstrates that deferasirox at 20 to 30 mg/kg/d can maintain or improve hepatic iron in thalassemia as well as deferoxamine. A randomized trial based on cardiac T2* magnetic resonance imaging (MRI) suggests that deferiprone can unload myocardial iron faster than deferoxamine. Retrospective epidemiologic data suggest dramatic reductions in cardiac events and mortality in Italian subjects exposed to deferiprone compared with deferoxamine. These developments herald a new era for iron chelation, but many unanswered questions remain
Dispelling the Myths Behind First-author Citation Counts
Full text linkWe conducted a full-scale evaluative citation analysis study of scholars in the XML research field to explore just how different from each other author rankings resulting from different citation counting methods actually are, and to demonstrate the capability of emerging data and tools on the Web in supporting more realistic citation counting methods. Our results contest some common arguments for the continued
use of first-author citation counts in the evaluation of scholars, such as high correlations between author rankings by first-author citation counts and other citation
counting methods, and high costs of using more realistic citation counting methods that are not well-supported by the ISI databases. It is argued that increasingly available digital full text research papers make it possible for citation analysis studies to go beyond what the ISI databases have directly supported and to employ more
sophisticated methods
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