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251 results on '"Porta F"'

1. Learning rate selection in stochastic gradient methods based on line search strategies

Author: Franchini, G., Porta, F., Ruggiero, V., Trombini, I., and Zanni, L.
Subjects: variance reduced methods, Stochastic gradient methods, convolutional neural networks, learning rate selection, mini batch size selection
Published: 2023

2. GAU-PED study for early diagnosis of Gaucher disease in children with splenomegaly and cytopenia

Author: Pession, A., Di Rocco, M., Venturelli, F., Tappino, B., Morello, W., Santoro, N., Giordano, P., Filippini, B., Rinieri, S., Russo, G., Girardi, K., Ruggiero, A., Galea, E., Antonucci, R., Tovaglieri, N., Porta, F., Tartaglione, I., Giona, F., Fagioli, F., Burlina, A., Mura, R., Russo, B., Tornesello, A., Menna, G., Russo, D., Caniglia, M., Schettini, S., Onofrillo, D., Ladogana, S., and Civino, A.
Subjects: Cytopenia, Splenomegaly, Lysosomal storage disease, Gaucher disease, Thrombocytopenia
Published: 2023

3. Minimally invasive approach compared to resternotomy for mitral valve surgery in patients with prior cardiac surgery

Author: Olsthoorn, Jules R., Heuts, Samuel, Houterman, Saskia, Maessen, Jos G., Sardari Nia, Peyman, Bramer, S., van Boven, W. J. P., Vonk, A. B. A., Koene, B. M. J. A., Bekkers, J. A., Hoohenkerk, G. J. F., Markou, A. L. P., de Weger, A., Segers, P., Porta, F., Speekenbrink, R. G. H., Stooker, W., Li, W. W. L., Daeter, E. J., van der Kaaij, N. P., Douglas, Y., CTC, MUMC+: MA Med Staf Artsass CTC (9), RS: Carim - Vessels, MUMC+: MA Cardiothoracale Chirurgie (3), RS: Carim - V04 Surgical intervention, MUMC+: MA Med Staf Spec CTC (9), Cardio-thoracic surgery, ACS - Atherosclerosis & ischemic syndromes, ACS - Heart failure & arrhythmias, ACS - Microcirculation, and Cardiothoracic Surgery
Subjects: Pulmonary and Respiratory Medicine, Reoperation, Mitral valve surgery, Mitral Valve Insufficiency, General Medicine, Resternotomy, Sternotomy, Nationwide registry, Treatment Outcome, Minimally invasive mitral valve surgery, Humans, Minimally Invasive Surgical Procedures, Mitral Valve, Surgery, Cardiac Surgical Procedures, Cardiology and Cardiovascular Medicine, Netherlands, Retrospective Studies
Abstract: OBJECTIVES Mitral valve (MV) surgery after prior cardiac surgery is conventionally performed through resternotomy and associated with increased morbidity and mortality. Alternatively, MV can be approached minimally invasively [minimally invasive mitral valve surgery (MIMVS)], but longer-term follow-up of this approach for MV surgery after prior cardiac surgery is lacking. Therefore, the aim of the current study is to evaluate short- and mid-term outcomes of MIMVS versus MV surgery through resternotomy in patients with prior sternotomy, using a nationwide registry. METHODS Patients undergoing isolated MV surgery after prior cardiac surgery between 2013 and 2018 were included. Primary outcomes were short-term morbidity and mortality and mid-term survival. Cox proportional hazard analysis was used to investigate the association between surgical approach and mortality. Propensity score matching was used to correct for potential confounders. RESULTS In total, 290 patients underwent MV surgery after prior cardiac surgery, of whom 205 patients were operated through resternotomy and 85 patients through MIMVS. No significant differences in 30-day mortality (3.4% vs 2%, P = 0.99) were observed between both groups. Five-year survival was 86.3% in the resternotomy group, compared to 89.4% in the MIMVS group (log-rank P = 0.45). In the multivariable analysis, surgical approach showed no relation with mid-term mortality [hazard ratio 0.73 (0.34–1.60); P = 0.44]. A lower incidence of prolonged intubation and new-onset arrhythmia was observed in MIMVS. CONCLUSIONS MV surgery after prior cardiac surgery has excellent short- and mid-term results in the Netherlands, and MIMVS and resternotomy appear to be equally efficacious. MIMVS is associated with a lower incidence of new-onset arrhythmia and prolonged intubation.
Published: 2022

4. Neural network-based classification of ENG recordings in response to naturally evoked stimulation

Author: Coviello, A., Porta, F., Magarini, M., and Spagnolini, U.
Subjects: Electroneurogram, rat sciatic nerve, signal preprocessing and classification, neural networks, nerve cuff electrode
Published: 2022

5. Hematopoietic stem cell transplantation for Wiskott-Aldrich syndrome

Author: Albert, M.H., Slatter, M.A., Gennery, A.R., Gungor, T., Bakunina, K., Markovitch, B., Hazelaar, S., Sirait, T., Courteille, V., Aiuti, A., Aleinikova, O.V., Balashov, D., Bernardo, M.E., Bodova, I., Bruno, B., Cavazzana, M., Chiesa, R., Fischer, A., Hauck, F., Ifversen, M., Kalwak, K., Klein, C., Kulagin, A., Kupesiz, A., Kuskonmaz, B., Lindemans, C.A., Locatelli, F., Lum, S.H., Maschan, A., Meisel, R., Moshous, D., Porta, F., Sauer, M.G., Sedlacek, P., Schulz, A., Suarez, F., Vallee, T.C., Winiarski, J.H., Zecca, M., Neven, B., Veys, P., Lankester, A.C., EBMT, European Soc Immunodeficiencies ES, and Stem CELL Transplant Primary Immun
Subjects: Transplantation Conditioning, Immunology, Hematopoietic Stem Cell Transplantation, Graft vs Host Disease, Cell Biology, Hematology, Biochemistry, Tissue Donors, Wiskott-Aldrich Syndrome, Treatment Outcome, surgical procedures, operative, Settore MED/38 - PEDIATRIA GENERALE E SPECIALISTICA, immune system diseases, Child, Preschool, Humans, Busulfan, Retrospective Studies
Abstract: Allogeneic hematopoietic stem cell transplantation (HSCT) is a potentially curative treatment for patients affected by Wiskott-Aldrich syndrome (WAS). Reported HSCT outcomes have improved over time with respect to overall survival, but some studies have identified older age and HSCT from alternative donors as risk factors predicting poorer outcome. We analyzed 197 patients undergoing transplant at European Society for Blood and Marrow Transplantation centers between 2006 and 2017 who received conditioning as recommended by the Inborn Errors Working Party (IEWP): either busulfan (n = 103) or treosulfan (n = 94) combined with fludarabine ± thiotepa. After a median follow-up post-HSCT of 44.9 months, 176 patients were alive, resulting in a 3-year overall survival of 88.7% and chronic graft-versus-host disease (GVHD)-free survival (events include death, graft failure, and severe chronic GVHD) of 81.7%. Overall survival and chronic GVHD-free survival were not significantly affected by conditioning regimen (busulfan- vs treosulfan-based), donor type (matched sibling donor/matched family donor vs matched unrelated donor/mismatched unrelated donor vs mismatched family donor), or period of HSCT (2006-2013 vs 2014-2017). Patients aged
Published: 2022

6. Guidelines for the implementation of workforce planning (WFP) in project-driven environments

Author: Micheli, G. J. L., Martino, A., Porta, F., Cravello, A., Panaro, M., and Calabrese, A.
Subjects: Project management, workforce, planning, workforce, Project management, planning
Published: 2022

7. Effect of minimally invasive mitral valve surgery compared to sternotomy on short- and long-term outcomes: a retrospective multicentre interventional cohort study based on Netherlands Heart Registration

Author: Olsthoorn, Jules R., Heuts, Samuel, Houterman, Saskia, Maessen, Jos G., Sardari Nia, Peyman, Bramer, S., van Boven, W. J. P., Vonk, A. B. A., Koene, B. M. J. A., Bekkers, J. A., Hoohenkerk, G. J. F., Markou, A. L. P., Weger, A. De, Segers, P., Porta, F., Speekenbrink, R. G. H., Stooker, W., Li, W. W. L., Daeter, E. J., van der Kaaij, N. P., Vigano, G., RS: Carim - Vessels, MUMC+: MA Med Staf Artsass CTC (9), CTC, MUMC+: MA Cardiothoracale Chirurgie (3), RS: Carim - V04 Surgical intervention, MUMC+: MA Med Staf Spec CTC (9), Cardiothoracic Surgery, and ACS - Heart failure & arrhythmias
Subjects: REPAIR, Pulmonary and Respiratory Medicine, Other Research Radboud Institute for Health Sciences [Radboudumc 0], Mitral valve surgery, SOCIETY, General Medicine, Sternotomy, Cohort Studies, OPERATIONS, Nationwide registry, Treatment Outcome, Minimally invasive mitral valve surgery, VOLUME, Humans, Minimally Invasive Surgical Procedures, Mitral Valve, Surgery, Cardiology and Cardiovascular Medicine, Mitral valve repair, Netherlands, Retrospective Studies
Abstract: OBJECTIVES Minimally invasive mitral valve surgery (MIMVS) has been performed increasingly for the past 2 decades; however, large comparative studies on short- and long-term outcomes have been lacking. This study aims to compare short- and long-term outcomes of patients undergoing MIMVS versus median sternotomy (MST) based on real-world data, extracted from the Netherlands Heart Registration. METHODS Patients undergoing mitral valve surgery, with or without tricuspid valve, atrial septal closure and/or rhythm surgery between 2013 and 2018 were included. Primary outcomes were short-term morbidity and mortality and long-term survival. Propensity score matching analyses were performed. RESULTS In total, 2501 patients were included, 1776 were operated through MST and 725 using an MIMVS approach. After propensity matching, no significant differences in baseline characteristics persisted. There were no between-group differences in 30-day mortality (1.1% vs 0.7%, P = 0.58), 1-year mortality (2.6% vs 2.1%, P = 0.60) or perioperative stroke rate (1.1% vs 0.6%, P = 0.25) between MST and MIMVS, respectively. An increased rate of postoperative arrhythmia was observed in the MST group (31.3% vs 22.4%, P CONCLUSIONS The MIMVS approach is as safe as the sternotomy approach for the surgical treatment of mitral valve disease. However, it comes at a cost of a reduced repair rate and more reinterventions in the long term, in the real-world.
Published: 2022

8. External validation of existing prediction models of 30-day mortality after Transcatheter Aortic Valve Implantation (TAVI) in the Netherlands Heart Registration

Author: Al-Farra, Hatem, Abu-Hanna, Ameen, de Mol, Bas A. J. M., ter Burg, W. J., Houterman, Saskia, Henriques, José P. S., Ravelli, Anita C. J., Vis, M. M., Vos, J., ten Berg, J., Tonino, W. A. L., Schotborgh, C. E., Roolvink, V., Porta, F., Stoel, M., Kats, S., Amoroso, G., van der Werf, H. W., Stella, P. R., de Jaegere, P., Cardiology, Medical Informatics, APH - Aging & Later Life, APH - Methodology, Cardiothoracic Surgery, ACS - Atherosclerosis & ischemic syndromes, APH - Quality of Care, ARD - Amsterdam Reproduction and Development, ACS - Pulmonary hypertension & thrombosis, APH - Health Behaviors & Chronic Diseases, and ACS - Heart failure & arrhythmias
Subjects: medicine.medical_specialty, Transcatheter aortic, Brier skill score, 030204 cardiovascular system & hematology, Risk Assessment, Transcatheter Aortic Valve Replacement, 03 medical and health sciences, Transcatheter aortic valve implantation (TAVI), 0302 clinical medicine, Risk Factors, Prediction model, Internal medicine, Discrimination, medicine, Humans, 030212 general & internal medicine, Mortality, Netherlands, Heart Valve Prosthesis Implantation, Ejection fraction, business.industry, External validation, EuroSCORE, Aortic Valve Stenosis, Treatment Outcome, Brier score, Aortic Valve, Cohort, Calibration, Cardiology, France, Cardiology and Cardiovascular Medicine, business, Predictive modelling
Abstract: Background: Several mortality prediction models (MPM) are used for predicting early (30-day) mortality following transcatheter aortic valve implantation (TAVI). Little is known about their predictive performance in external TAVI populations. We aim to externally validate established MPMs on a large TAVI dataset from the Netherlands Heart Registration (NHR). Methods: We included data from NHR-patients who underwent TAVI during 2013–2017. We calculated the predicted mortalities per MPM. We assessed the predictive performance by discrimination (Area Under Receiver Operating-characteristic Curve, AU-ROC); the Area Under Precision-Recall Curve, AU-PRC; calibration (using calibration-intercept and calibration-slope); Brier Score and Brier Skill Score. We also assessed the predictive performance among subgroups: tertiles of mortality-risk for non-survivors, gender, and access-route. Results: We included 6177 TAVI-patients with an observed early-mortality rate of 4.5% (n = 280). We applied seven MPMs (STS, EuroSCORE-I, EuroSCORE-II, ACC-TAVI, FRANCE-2, OBSERVANT, and German-AV) on our cohort. The highest AU-ROCs were 0.64 (95%CI 0.61–0.67) for ACC-TAVI and 0.63 (95%CI 0.60–0.67) for FRANCE-2. All MPMs had a very low AU-PRC of ≤0.09. ACC-TAVI had the best calibration-intercept and calibration-slope. Brier Score values ranged between 0.043 and 0.063. Brier Skill Score ranged between −0.47 and 0.004. ACC-TAVI and FRANCE-2 predicted high mortality-risk better than other MPMs. ACC-TAVI outperformed other MPMs in different subgroups. Conclusion: The ACC-TAVI model has relatively the best predictive performance. However, all models have poor predictive performance. Because of the poor discrimination, miscalibration and limited accuracy of the models there is a need to update the existing models or develop new TAVI-specific models for local populations.
Published: 2020

9. Allogeneic hematopoietic stem cell transplantation in leukocyte adhesion deficiency type I and III

Author: Bakhtiar, S., Salzmann-Manrique, E., Blok, H.J., Eikema, D.J., Hazelaar, S., Ayas, M., Toren, A., Goldstein, G., Moshous, D., Locatelli, F., Merli, P., Michel, G., Ozturk, G., Schulz, A., Heilmann, C., Ifversen, M., Wynn, R.F., Aleinikova, O., Bertrand, Y., Tbakhi, A., Veys, P., Karakukcu, M., Kupesiz, A., Ghavamzadeh, A., Handgretinger, R., Unal, E., Perez-Martinez, A., Gokce, M., Porta, F., Aksu, T., Karasu, G., Badell, I., Ljungman, P., Skorobogatova, E., Yesilipek, A., Zuckerman, T., Bredius, R.R.G., Stepensky, P., Shadur, B., Slatter, M., Gennery, A.R., Albert, M.H., Bader, P., Lankester, A., Pediat Dis Working Party, and Inborn Errors Working Party EBMT
Subjects: surgical procedures, operative
Abstract: Type I and III leukocyte adhesion deficiencies (LADs) are primary immunodeficiency disorders resulting in early death due to infections and additional bleeding tendency in LAD-III. The curative treatment of LAD-I and LAD-III is allogeneic hematopoietic stem cell transplantation (allo-HSCT). In this retrospective multicenter study, data were collected using the European Society for Blood and Marrow Transplantation registry; we analyzed data from 84 LAD patients from 33 centers, all receiving an allo-HSCT from 2007 to 2017. The 3-year overall survival estimate (95% confidence interval [CI]) was 83% (74-92) for the entire cohort: 84% (75-94) and 75% (50-100) for LAD-I and LAD-III, respectively. We observed cumulative incidences (95% CI) of graft failure (GF) at 3 years of 17% (9%-26%) and grade II to IV acute graft-versus-host disease (aGVHD) at 100 days of 24% (15%-34%). The estimate (95% CI) at 3 years for GF- and GVHD-II to IV-free survival as event-free survival (EFS) was 56% (46-69) for the entire cohort; 58% (46-72) and 56% (23-88) for LAD-I and LAD-III, respectively. Grade II to IV acute GVHD was a relevant risk factor for death (hazard ratio 3.6; 95% CI 1.4-9.1; P = .006). Patients' age at transplant >= 13 months, transplantation from a nonsibling donor, and any serological cytomegalovirus mismatch in donor-recipient pairs were significantly associated with severe acute GVHD and inferior EFS. The choice of busulfan- or treosulfan-based conditioning, type of GVHD prophylaxis, and serotherapy did not impact overall survival, EFS, or aGVHD. An intrinsic inflammatory component of LAD may contribute to inflammatory complications during allo-HSCT, thus providing the rationale for considering anti-inflammatory therapy pretreatment.
Published: 2021

10. Insights into the expanding phenotypic spectrum of inherited disorders of biogenic amines

Author: Kuseyri Hübschmann, O. Horvath, G. Cortès-Saladelafont, E. Yıldız, Y. Mastrangelo, M. Pons, R. Friedman, J. Mercimek-Andrews, S. Wong, S.-N. Pearson, T.S. Zafeiriou, D.I. Kulhánek, J. Kurian, M.A. López-Laso, E. Oppebøen, M. Kılavuz, S. Wassenberg, T. Goez, H. Scholl-Bürgi, S. Porta, F. Honzík, T. Santer, R. Burlina, A. Sivri, H.S. Leuzzi, V. Hoffmann, G.F. Jeltsch, K. Hübschmann, D. Garbade, S.F. Assmann, B. Fung, C.-W. Guder, P. Hong, S.T.K. Karall, D. Kato, M. Kavecan, I. Koht, J.A. Kuster, A. Lücke, T. Manti, F. Mir, P. Mühlhausen, C. Önenli Mungan, H.N. Palacios, N.A.J. Ramos, J.A.F. Steel, D. Stevanović, G. Sykut-Cegielska, J. Verbeek, M.M. García-Cazorla, A. Opladen, T. iNTD Registry Study Group
Abstract: Inherited disorders of neurotransmitter metabolism are rare neurodevelopmental diseases presenting with movement disorders and global developmental delay. This study presents the results of the first standardized deep phenotyping approach and describes the clinical and biochemical presentation at disease onset as well as diagnostic approaches of 275 patients from the registry of the International Working Group on Neurotransmitter related Disorders. The results reveal an increased rate of prematurity, a high risk for being small for gestational age and for congenital microcephaly in some disorders. Age at diagnosis and the diagnostic delay are influenced by the diagnostic methods applied and by disease-specific symptoms. The timepoint of investigation was also a significant factor: delay to diagnosis has decreased in recent years, possibly due to novel diagnostic approaches or raised awareness. Although each disorder has a specific biochemical pattern, we observed confounding exceptions to the rule. The data provide comprehensive insights into the phenotypic spectrum of neurotransmitter disorders. © 2021, The Author(s).
Published: 2021

11. Correction to: Tocilizumab for patients with COVID-19 pneumonia. The single-arm TOCIVID-19 prospective trial (Journal of Translational Medicine, (2020), 18, 1, (405), 10.1186/s12967-020-02573-9)

Author: Perrone, F., Piccirillo, M. C., Ascierto, P. A., Salvarani, C., Parrella, R., Marata, A. M., Popoli, P., Ferraris, L., Marrocco-Trischitta, M. M., Ripamonti, D., Binda, F., Bonfanti, P., Squillace, N., Castelli, F., Muiesan, M. L., Lichtner, M., Calzetti, C., Salerno, N. D., Atripaldi, L., Cascella, M., Costantini, M., Dolci, G., Facciolongo, N. C., Fraganza, F., Massari, M., Montesarchio, V., Mussini, C., Negri, E. A., Botti, G., Cardone, C., Gargiulo, P., Gravina, A., Schettino, C., Arenare, L., Chiodini, P., Gallo, C., Vitale, M. G., Trojaniello, C., Palla, M., Bianchi, A. A. M., De Feo, G., Miscio, L., Chiodiniy, P., Froldi, M., Menicanti, L., Cuppone, M. T., Gobbo, G., Baldessari, C., Valenti, V., Castelvecchio, S., Poli, F., Giacomazzi, F., Piccinni, R., Annnunziata, M. L., Biondi, A., Bussolari, C., Mazzoleni, M., Giachi, A., Filtz, A., Manini, A., Poletti, E., Masserini, F., Conforti, F., Gaudiano, G., Favero, V., Moroni, A., Viva, T., Fancoli, F., Ferrari, D., Niro, D., Resta, M., Ballotta, A., Poli, M. D., Ranucci, M., Tebaldi, A., Gritti, G., Pasulo, L., Gaglio, L., Del Fabbro, R., Alborghetti, L., Giustinetti, G., Columpsi, P., Cazzaniga, M., Capici, S., Sala, L., Di Sciacca, R., Mosca, G., Pirozzi, M. R., Franceschini, F., Roccaro, A., Salvetti, M., Paini, A., Corda, L., Ricci, C., Tomasoni, L., Nasta, P., Lorenzotti, S., Odolini, S., Foca, E., Roldan, E. Q., Metra, M., Magrini, S., Borghetti, P., Latronico, N., Piva, S., Filippini, M., Tomasi, G., Zuccala, F., Cattaneo, S., Scolari, F., Bossini, N., Gaggiotti, M., Properzi, M., Del Borgo, C., Marocco, R., Belvisi, V., Tieghi, T., De Masi, M., Zuccala, P., Fabietti, P., Vetica, A., Mercurio, V. S., Carraro, A., Fondaco, L., Kertusha, B., Curtolo, A., Del Giudice, E., Lubrano, R., Zotti, M. G., Puorto, A., Ciuffreda, M., Sarni, A., Monteforte, G., Romeo, D., Viola, E., Damiani, C., Barone, A., Mantovani, B., Di Sanzo, D., Gentili, V., Carletti, M., Aiuti, M., Gallo, A., Meliante, P. G., Martellucci, S., Riggio, O., Cardinale, V., Ridola, L., Bragazzi, M. C., Gioia, S., Valenzi, E., Graziosi, C., Bina, N., Fasolo, M., Ricci, S., Gioacchini, M. T., Lucci, A., Corso, L., Tornese, D., Nijhawan, P., Equitani, F., Cosentino, C., Palladino, M., Leonetti, F., Leto, G., Gnessi, C., Campagna, G., Cesareo, R., Marrocco, F., Straface, G., Mecozzi, A., Cerbo, L., Isgro, V., Parrocchia, S., Visconti, G., Casati, G., Ariani, A., Donghi, L., Tacconelli, E., Bertoldi, M., Cattaneo, P., Lambertenghi, L., Motta, L., Omega, L., Albano, G., Scarano, F., De Rosa, A., Buglione, A., Lavoretano, S., Gaglione, G., De Marco, M., Sangiovanni, V., Fusco, F. M., Viglietti, R., Manzillo, E., Rescigno, C., Pisapia, R., Plamieri, G., Maraolo, A., Calabria, G., Catalano, M., Fiorentino, G., Annunziata, A., Polistina, G., Imitazione, P., Mollica, M., Esposito, V., D'Abraccio, M., Punzi, R., Bianco, V., Sbreglia, C., Del Vecchio, R. F., Bordonali, A., Franco, A., Salsi, P., Fontana, M., Virzi, G., Calderone, O., Molteni, A., Gennarini, S., Gnudi, U., Ricci, M. A., Titolo, G., Mensi, G., Vuotto, P., Gasperini, B., Mancini, M., Pasquini, Z., Spanu, P., Clementi, S., Pierini, S., Bokor, D., Gori, D., Ciofetti, M., Caimi, M., Bettazzi, L., Allevi, E., Furiani, S., Capitanio, C., Mastropasqua, B., Fara, C., Pulitano, G., Matsuno, J. S., Porta, F. D., Dolfini, V., Beyene, N. B., Bezzi, M., Novali, M., Viale, P., Tedeschi, S., Pascale, R., Bruno, R., Di Filippo, A., Sachs, M., Oggionni, T., Di Stefano, M., Mengoli, C., Facchini, C., De Nardo, D., Frausini, G., Mucci, L., Tedesco, S., Girolimetti, R., Manfredini, E., Di Carlo, A. M., Espinosa, E., Dennetta, D., Ticinesi, A., Meschi, T., Nouvenne, A., Norbiato, C., Vitale, F., Saracco, M., Codeluppi, M., Fronti, E., Ferrante, P., Nespola, G. A., Francisci, D., Tosti, A., Carbonelli, C. M., Greco, A., Tinti, M. G., Stellini, R., Appiani, C., Reghenzi, P., Poletti, V., Ravaglia, C., Tacconi, D., Malcontenti, C., Sainaghi, P. P., Landi, R., Vassia, V., Rizzi, E., Bellan, M., Rossati, A., Castello, L., Mastroianni, C. M., Russo, G., Toffoletto, F., Serino, F. S., Brollo, L., Momesso, E., Turati, M. L., Monforte, A. D., Marchetti, G., Boni, F., Teopompi, E., Trenti, C., Boracchia, L., Minelli, E., Ghidoni, G., Matei, A., Caruso, A., Arcoleo, G., Camarda, G., Catalano, F., Spatafora, M., Bettega, D., Andreoni, M., Teti, E., Sarmati, L., Di Lorenzo, A., Celeste, M., Baratto, F., Monticelli, J., Criveller, P., Antonini, A., Anselmo, Riccio, Castellano, M., Cappelli, C., Corvini, F., Zanini, B., Crippa, M., Ronconi, M., Costa, R., Casella, S., Brentana, L., Bernardi, L., Frascati, A., Panese, S., Presotto, F., Michieletto, L., Bernardi, C., Fusar, M., Agnoletti, V., Farina, M., Russo, Lavorini, F., Ginanni, R., Palmieri, F., Mosti, S., Amaglio, A., Cattaneo, A., Cirri, S., Montisci, A., Gallazzi, C., Cosseta, D., Baronio, B., Rampa, L., Maggi, P., Messina, V., Sabatti, M. C., Palumbo, M., Mazzone, A., Faggioli, P., Bussini, L., Fornaro, G., Volpato, F., Imperiale, D., Manno, E., Ferreri, E., Martelli, D., Verhovez, A., Giorgis, S., Faccio, L., Delli Quadri, R., Negro, C., Converso, M., Bosco, F., Amadosi, S., Prandini, P., Cocchi, S., Manfrin, V., Del Punta, V., Mazzola, G., Sportato, G., Romagnoli, M., Cristini, F., Facondini, F., Perin, T., Boschi, A., Meschiari, M., Guaraldi, G., Modica, S., Moneta, S., Boccalatte, D., Marchetti, V., Amadasi, S., Ebbreo, G., Dale, M., Tura, P., Rizzoni, D., Boari, G. E. M., Bonetti, S., Marini, E., Daniele, I., Grossi, P. A., Delfrate, N. W., Bernhart, O., Spizzo, G., Mahlknecht, K., Volkl, T., Di Pietro, M. A., Trezzi, M., Monacci, C., Peris, A., Bonizzoli, M., Cavanna, L., Moroni, C., Stroppa, E. M., Savio, M. C., Gatti, F., Bartolaminelli, C., Petrosillo, N., Donno, D. R., Taglietti, F., Topino, S., Chinello, P., Galati, V., D'Offizi, G., Taibi, C., Cimolato, B., Moroni, F., Palagano, N., Pelagatti, L., Seravalle, C., Landini, G., Amitrano, M., Raimondo, M., Mangiacapra, S., Romano, A., Atteno, M., Blanc, P., Suardi, L. R., Pallotto, C., Casinelli, K., Uccella, I., Harari, S., Caminati, A., Lipani, F., Di Perri, G., Calcagno, A., Calleri, G., Montrucchio, C., Caputo, A. M., Cozzio, S., Delle Donne, L., Bassetti, M., Malgorzata, M., Nicolini, L. A., Russo, C., Sepulcri, C., Beltramini, S., Mina, F., Puoti, M., Gandino, A., Langer, T., D'Amico, F., Berlendis, M., Rocchetti, C., Cettolo, F., Fausini, G., Bocchi, P., Cioni, G., Cappi, C., Corcione, S., De Rosa, F. G., Scabini, S., Canta, F., Mornese Pinna, S., Pensa, A., Rocco, M., Cirasa, M. T., Spinicci, M., Mencarini, J., Zammarchi, L., Cenderello, G., Sciole, K., Bassi, F., Bianchi, M., Frigerio, S., Spaziani, S., Nucera, A., Rizzardini, G., Cossu, M. V., Antivalle, M., Carpinteri, G., Macheda, S., Labate, D., Bottiroli, M., Erne, E. M., Cristina, Z., Di Biase, V., Malberti, F., Montani, G., Poisa, P., Bettini, D., Cauda, R., Ciccullo, A., Riccardi, N., Angheben, A., Turrini, M., Clerici, R., Gardellini, A., Liparulo, L., Rossini, T., Ucciferri, C., Cipollone, F., Vecchiet, J., Nico, A., Marra, L., Leone, A., Sdanganelli, A., Palmiotti, G. A., D'Alagni, G., Santantonio, T. A., Lo Caputo, S., Bottalico, I., Ponticiello, A., Di Perna, F., Bernardi, E., Beltrame, A., Bravi, S., David, M., Bernardi, P., Galante, D., Uccelli, M. C., Prestini, K., Drera, M., Zini, E., Peregrinelli, A., Blanzuoli, L., Benedetti, V., Calvi, R., Scaglione, N., Nallino, G., Bonazzi, M., Crespi, T., Masolin, T., Regazzetti, A., Cerri, M. C., Maffezzini, E., Piazza, M., Papetti, C., De Filippi, C., Roveda, E., Cipolla, G., Scozzafava, M., Crepaldi, M., Henchi, S., Vanoni, N., Repossi, A., Vezzoli, M., Scorletti, E., Perugini, O., Pasini, S. M., Pacetti, V., Ferrari, L., de Paduanis, G. A., del Duca, S., Dell'Ara, F., Brocchieri, A., Minoja, G., Storti, E., Pitagora, L., Costa, I., Delfanti, F., Orlandi, M., Ruggeri, R., Ruggieri, L., Livigni, S., Silengo, D., Ageno, W., Pedrini, L., Artiol, S., Morbidoni, L., De Donno, G., Ravagnani, V., Inglese, F., Scotton, P. G., Costantini, P., Delucchi, M., Clini, E., Ansuini, A., Baiocchi, M., Lain, G., Vincenzo, B., Rastelli, G., Doria, A., Vianello, A., Cattelan, A. M., Bindoli, S., Felicietti, M., Canetta, C., Scartabellati, A., Accordino, S., Ferrara, M., Cocco, L., Cirillo, F., Pace, E., De Caro, M., Alberico, M., Benigni, G., Damiano, T., Fusco, P., Iuorio, A., Torretta, G., Racagni, M., Muttini, S., Sala, G., Ghiringhelli, P., Chiumiento, F., Baccari, L., Bocchi, F., Benatti, F., Catellani, J., Coppola, M., Papi, A., Bosco, E., Lazzeri, C., Cesira, N., Puttini, C., Carli, T., Croci, L., Corridi, M., Arlotti, M., Guerrini, G., Cola, L., Romanelli, M., Bonifazi, M., Gasparini, S., Mei, F., Cerutti, E., Lacedonia, D., Santoro, A., Guidelli, G. M., Greco, S., Castellan, A., Infantino, G., Camici, L., Covani Frigieri, F. C., Pavoni, V., Migliori, L., Rossetti, B., Montagnini, F., Mauro, I., Genovese, E., Capuozzo, A., Vitiello, L., Sirignano, E., Gnesin, P., Servillo, G., Marinelli, A., Pasero, D., Babudieri, S., Madeddu, G., De Vito, A., Casadio, L., Ranghitta, M., Passalacqua, R., Fioravanti, A., Gentile, I., Buonomo, A. R., Scotto, R., Zappulo, E., Dell'Aquila, G., Bianchetti, A., Guerini, F., Vallone, A., Oppedisano, P., Pusterla, L., Giglio, O., Sartori, E., Zanardini, C., Gatti, P., Valiani, V., Piconi, S., Molteni, C., Dognini, G., Cosimo, F., Guarneri, L., Pulvirenti, F., Mondino, V., Traballi, G., Iemoli, E., Grisolia, A., Giorgi, R., Nucera, G., Raffaelli, V., Marino, P., Negro, E., Serati, L., Tamanini, S., Iacobello, C., Strano, G., Boglione, L., Catania, A., Gipponi, P., Di Cato, L., Panaccione, A., Vitale, G., Crippa, I. A., Giacomini, M., Basile, A., Bellone, A., Tundo, P., Buzzigoli, S., Palmiero, G., Magnaca, A., Silva, M., Ricci, M., Crespi, S., Pasquino, B., Consales, G., Bragantini, D., Mastroianni, F., Righetti, G., Scarafino, A., Bitetto, M., Franzetti, F., Piga, S., Delmonte, V., Carbonara, S., Losappio, R., Dejaco, C., Mastroianni, C., Del Bono, V., Gilioli, F., Barzan, D., De Struppi, S., Carlotto, A., Guadagnin, M. L., Girardis, M., Bertellini, E., Dentali, F., Foresta, G., Baratta, A., Viviani, R., Agrati, A. M., Perego, G. B., Montineri, A., Manuele, R., Bonfante, S., Aquilini, D., Prozzo, A., Santopuoli, D., Di Rosa, Z., Alborghetti, A., Peci, P., Bakhtadze, N., Pandini, C. S., Ashofarir, N., Casella, G., Spagnolli, W., Urru, S., Marchesoni, I., Caminiti, G., Argilloni, E., Danieli, E., Ghirardi, G., Antonioli, C. M., Lipari, A., Zavarise, P., Kokaly, F., Polati, E., Gottin, L., Lucernoni, P., De Conti, F., Marcon, E., Pontali, E., Vacca, E. B., Saffioti, C., Zunino, A., Pognuz, E. R., Berlot, G., Saltori, M., Tedesco, A., Agostini, C., Di Rosolini, M. A., Marino, F., Bellinzona, G., Grassi, W., Di Carlo, M., Scimonello, G., Nonini, S., Mondino, M., Mantovani, L. F., Tenti, E., Tropea, C. M. G., Di Stefano, D. E., Guelfi, P., Dagna, L., Morgana, G., Montemurro, L., Girelli, D., Crisafulli, E., Maroccia, A., Cemuschi, A. M., Bernasconi, M., Zummo, U., Barbato, V., Bevilacqua, S., Buonfanti, G., Canzanella, G., De Matteis, G., Florio, M., Martino, M., Ribecco, M. T., Romano, F., Savio, A., Sparavigna, L., Curvietto, M., Citarella, M., Nava, V., Maggioni, P., Magni, M., Iommelli, C., Bianco, A., Corsini, R., Valli, L., Ruggieri, M. P., Melica, T., Ferrari, A., Cicognini, D., Delliponti, M., Zuccarini, A., Ciani, S., Raffaeli, D., Donati, L., Cannizzo, S., Lui, S., Santini, L., Roncaglia, E., Mighali, P., Eisendle, F., Cerino, G., Citterio, C., Di Nunzio, C., Mancini, A., Lamonica, S., Resimini, S., Sarteschi, G., Pavei, C., Battistini, N., Gazzola, O. E., Miceli, M., Pontiggia, S., Lonati, V., Giannandrea, G., Sortino, C., Ravani, S., Uggeri, C., Jocolle, G., Bare, C., Baroni, I., De Candia, D., Fiorini, B., Chierico, K., Romeo, F., Bottega, R., Boccasile, L., Corsaro, A., Spadoni, C., Chiari, S., Ercolino, G., Dell'Uomo, V., Viri, S., Minato, M., Gazzola, L., Dorina, B., Gianelli, D., Maspero, S., Farinazzo, M., Zanini, P., Sangiovanni, A., Del Giudice, A., Dragonetti, M. M., Bordignon, S., Machiavelli, A. M., Chiodelli, G., Spatarella, M., Zenoni, D., Beretta, F. N., Santilli, G., Badagliacca, R., Angileri, M., Giannelli, L., Campomori, A., Maimone, P., Fadda, A., Faoro, S., Pisterna, A., Cacopardo, B., Marino, A., Pampaloni, A., Celesia, B. M., Cinnella, G., Labella, D., Caporusso, R. R., Danzi, M., Fiscon, M., Malena, M., Fendt, D., Nardi, S., Stobbione, P., Savi, M. L., De Monte, A., Scala, A., Liberato, N. L., Luchi, S., Vincenti, A., Cabrini, L., Pinelli, G., Brugioni, L., Potenza, D., Numis, F. G., Porta, G., D'Amico, M., Iengo, B., Angarano, G., Saracino, A., Blasi, L., De Negri, P., Angelici, S., Farina, A., Martino, G. P., Bitti, G., Tedeschi, A., De Ponti, S., Agostinone, A., Parruti, G., Consorte, A., Frattari, A., Filippelli, A., Pagliano, P., Masullo, A., Sellitto, C., Reta, M., Rossi, N., Raumer, L., Andreassi, S., Brancaleoni, P., Carai, A., Salerno, A. M., Marinangeli, F., Mariani, R., Ciccone, A., Meschini, C., Santoboni, G., Angrisani, C., Micarelli, D., Tarquini, G., Fregoni, V., Volta, C. A., Cherubini, A., Del Prete, M. S., Ciarrochi, E., Tasca, F., Ballarin, A., Bianchin, A., Flocco, R., Cuzzone, V., Carpinteri, M., Gallotti, P., Torre, F., Zannetti, P., Crapis, M., Venturini, S., Barattini, M., Gori, G., Mastroianni, A., De Stefano, G., Gilio, M., Rapisarda, G., Gulisano, L., Granata, M. L., Saglimbene, S., Montalto, M. T., Grasso, I., De Luca, S., Magro, G., Messina, F., Scapino, B., Abrate, P., Francisco, C., Pesce, L., Navarra, M., Agosti, M., Pagani, S., Piluso, M., Ricioppo, A., Tognella, S., Rovere, P., Vincenzi, M., Ghirardi, L., Generali, D., Ingrosso, M., Desiderio, E., Molaro, R., Vitiello, S., Lancione, L., Paone, T. C., Meli, A., Mainardi, S., Rastellino, V., Ursillo, A., di Grigoli, P., Bovetto, E., Stefanetto, I. M., Mazzola, F., Daniele, A., Bisio, C., Delnero, P., Morando, G., Nava, A., Francesco, L., Fiammengo, F., Regis, M., Roccatello, D., Sabato, E., Liccardi, M. M., Bretto, C., Lutri, L., Castenetto, E., Roberti, G., Guidi, M. F., Bini, F., Zappa, M. C., Trequattrini, T., Rivitti, R., Vigliarolo, R., Succu, A., Lilli, M., Serao, M., Giogre, G., Ruggieri, A., Flores, K., Vairo, G., Satira, R., Lingua, A., Spina, R., Nicastri, E., Maffongelli, G., Barreca, F., Scollet, S., Franchi, F., Fabbri, C., Minuz, P., Dalbeni, A., Zanatta, P., Gelormini, D., Mandelli, A., Galderisi, F., Zoia, E., Marchi, M. R., De Almeida Neves, N., Carbone, G., Di Caterino, E., Petrone, A., Usai, C. A., Bandiera, F., Monti, R., Hofer, A., Castiglione, G., Angeletti, C., Tarsia, P., Veronese, L., Artoni, P. D., Larussa, D., Fumagalli, R., Brioschi, P., Cerutti, A., Pasquino, P., Gilberto, F., Cantadori, L., Tomasoni, G., Tomasoni, L. R., Coppola, N., Spolveri, S., Pollastri, C., Fico, L., Principi, T., Pierantozzi, S., Fontana, C., Lubrano, G., Martinelli, L., Navalesi, P., Serra, E., Cogi, E., Manzi, A., Furino, E., Dasseni, N., Gentilini, C., Benatti, E., Pignatti, A., Aiello, G., Milia, M., Covesnon, M. G., Brianti, A., Francesco, C., Ilaria, B., Pagnozzi, F., Mietta, S., Rossi, A., Maroni, L., Borroni, V., Bellintani, C., Sgarabotto, C., Bizzotto, G., Bucci, L., Spagnuolo, G., Agostini, M., Caria, F. C., Testa, F., De Palma, R., Murdaca, G., Zanolini, G., Sala, N., Righini, E., Pontremoli, R., Aondio, G., Riccardi, F., De Cristoforo, M. G., De Michele, F., Storti, A., Perra, R., Deidda, S., Enrica, C., Valastro, F., Pierfranceschi, M. G., De Gennaro, F., Nardecchia, A. L., Castellini, M., Buetto, G., Ippoliti, G., Sicheri, D., Bottoli, M. G., De Arroyabe, B. M. L., Versaci, A., Di Cura Villa Giada Pallotti, C., Civita, M., Grio, M., Liuzzi, N., Molino, P., Pastorelli, M., Ricchiardi, A., Varbella, F., Zeme, A. D., Sighieri, C., Portale, G., Olivetti, A., Pagnoni, C., Moschini, G., Boni, S., Guerra, A., Scudellari, R., Vella, S., Inchiostro, S., Piazza, O., Guarino, S., Aldegheri, G., Napoli, G., Morettini, A., Caldini, E., Menicacci, L., Pieralli, F., Torrini, M., Poggesi, L., Visetti, E. M., Mangano, C., Visconti, S., Maietta, P., Banfi, E., Cartella, S., Venturi, B., Nuceri, A., Chiesa, E., Pacentra, E., Panzolato, G., Giannotti, M., Bianchi, C., Pietrangelo, A., Para, O., Rutili, M. S., Russo, R., Lanfranco, M., Scalabrino, E., Tafuri, A., Perfetti, E., Chiarello, T., Cancanelli, L., Otero, M., Pannella, G., Bellucci, F., Ferrero, G., Vico, C., Stillante, M. S., D'Andrea, G., Amoroso, F., Arcidiacono, A., Bella, A. M., Belsito, A., Berte, Y., Carubia, G., Caruso, M. G., Casella, O., Chiereleson, F., Costa, C., De Franco, D., Germana, G., Messina, A., Musumeci, D., Noto, C., Valenti, M., Sorrentino, C., Panico, R., Schettino, G., Piccoli, J., Pepe, A., De Rosa, F., Ottaviano, M., Marrazzo, G., Raponi, G., Diberardino, S., Bausi, S., Marini, S. F., Giubellino, E., Innocenti, G., Gugliemi, G., Maccari, D., and Baciu, I.
Subjects: tocilizumab, covid 19, pneumonia
Published: 2021

12. Hematopoietic stem cell transplantation for CD40 ligand deficiency : Results from an EBMT/ESID-IEWP-SCETIDE-PIDTC study

Author: Ferrua, F., Galimberti, S., Courteille, V., Slatter, M.A., Booth, C., Moshous, D., Neven, B., Blanche, S., Cavazzana, M., Laberko, A., Shcherbina, A., Balashov, D., Soncini, E., Porta, F., Al-Mousa, H., Al-Saud, B., Al-Dhekri, H., Arnaout, R., Formankova, R., Bertrand, Y., Lange, A., Smart, J., Wolska-Kusnierz, B., Aquino, V.M., Dvorak, C.C., Fasth, A., Fouyssac, F., Heilmann, C., Hoenig, M., Schuetz, C., Kelecic, J., Bredius, R.G.M., Lankester, A.C., Lindemans, C.A., Suarez, F., Sullivan, K.E., Albert, M.H., Kalwak, K., Barlogis, V., Bhatia, M., Bordon, V., Czogala, W., Alonso, L., Dogu, F., Gozdzik, J., Ikinciogullari, A., Krivan, G., Ljungman, P., Meyts, I., Mustillo, P., Smith, A.R., Speckmann, C., Sundin, M., Keogh, S.J., Shaw, P.J., Boelens, J.J., Schulz, A.S., Sedlacek, P., Veys, P., Mahlaoui, N., Janda, A., Davies, E.G., Fischer, A., Cowan, M.J., Gennery, A.R., SCETIDE, PIDTC, EBMT, ESID IEWP, Ferrua, F, Galimberti, S, Courteille, V, Slatter, M, Booth, C, Moshous, D, Neven, B, Blanche, S, Laberko, A, Shcherbina, A, Balashov, D, Soncini, E, Porta, F, Al-Mousa, H, Al-Saud, B, Al-Dhekri, H, Arnaout, R, Formankova, R, Bertrand, Y, Lange, A, Smart, J, Wolska-Kusnierz, B, Aquino, V, Dvorak, C, Fasth, A, Fouyssac, F, Heilmann, C, Hoenig, M, Schuetz, C, Kelečić, J, Bredius, R, Lankester, A, Lindemans, C, Suarez, F, Sullivan, K, Albert, M, Kałwak, K, Barlogis, V, Bhatia, M, Bordon, V, Czogala, W, Alonso, L, Dogu, F, Gozdzik, J, Ikinciogullari, A, Kriván, G, Ljungman, P, Meyts, I, Mustillo, P, Smith, A, Speckmann, C, Sundin, M, Keogh, S, Shaw, P, Boelens, J, Schulz, A, Sedlacek, P, Veys, P, Mahlaoui, N, Janda, A, Davies, E, Fischer, A, Cowan, M, and Gennery, A
Subjects: 0301 basic medicine, Oncology, medicine.medical_specialty, medicine.medical_treatment, Immunology, Hematopoietic stem cell transplantation, X-Linked Combined Immunodeficiency Diseases, primary immunodeficiency, Donor lymphocyte infusion, 03 medical and health sciences, 0302 clinical medicine, Internal medicine, X-linked hyper-IgM syndrome, Humans, Medicine, Immunology and Allergy, Risk factor, Child, Prospective cohort study, business.industry, Infant, Newborn, Infant, medicine.disease, Treatment Outcome, 030104 developmental biology, medicine.anatomical_structure, Graft-versus-host disease, surgical procedures, operative, Supportive psychotherapy, Child, Preschool, hematopoietic stem cell transplantation, Primary immunodeficiency, Bone marrow, CD40 ligand, business, 030215 immunology
Abstract: BACKGROUND: CD40 ligand (CD40L) deficiency, an X-linked primary immunodeficiency, causes recurrent sinopulmonary, Pneumocystis and Cryptosporidium species infections. Long-term survival with supportive therapy is poor. Currently, the only curative treatment is hematopoietic stem cell transplantation (HSCT). OBJECTIVE: We performed an international collaborative study to improve patients' management, aiming to individualize risk factors and determine optimal HSCT characteristics. METHODS: We retrospectively collected data on 130 patients who underwent HSCT for CD40L deficiency between 1993-2015. We analyzed outcome and variables' relevance with respect to survival and cure. RESULTS: Overall survival (OS), event-free survival (EFS), and disease-free survival (DFS) were 78.2%, 58.1%, and 72.3% 5 years after HSCT. Results were better in transplantations performed in 2000 or later and in children less than 10 years old at the time of HSCT. Pre-existing organ damage negatively influenced outcome. Sclerosing cholangitis was the most important risk factor. After 2000, superior OS was achieved with matched donors. Use of myeloablative regimens and HSCT at 2 years or less from diagnosis associated with higher OS and DFS. EFS was best with matched sibling donors, myeloablative conditioning (MAC), and bone marrow-derived stem cells. Most rejections occurred after reduced-intensity or nonmyeloablative conditioning, which associated with poor donor cell engraftment. Mortality occurred mainly early after HSCT, predominantly from infections. Among survivors who ceased immunoglobulin replacement, T-lymphocyte chimerism was 50% or greater donor in 85.2%. CONCLUSION: HSCT is curative in patients with CD40L deficiency, with improved outcome if performed before organ damage development. MAC is associated with better OS, EFS, and DFS. Prospective studies are required to compare the risks of HSCT with those of lifelong supportive therapy. ispartof: JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY vol:143 issue:6 pages:2238-2253 ispartof: location:United States status: published
Published: 2019

13. Haploidentical transplantation and posttransplant cyclophosphamide for treating aplastic anemia patients: a report from the EBMT Severe Aplastic Anemia Working Party

Author: Prata, P.H., Eikema, D.J., Afansyev, B., Bosman, P., Smiers, F., Diez-Martin, J.L., Arrais-Rodrigues, C., Koc, Y., Poir?, X., Sirvent, A., Kr?ger, N., Porta, F., Holter, W., Bloor, A., Jubert, C., Ganser, A., Tanase, A., M?nard, A.L., Pioltelli, P., P?rez-Sim?n, J.A., Ho, A., Aljurf, M., Russell, N., Labussiere-Wallet, H., Kerre, T., Rocha, V., Soci?, G., Risitano, A., Dufour, C., Latour, R.P. de, SAA WP EBMT, Prata, P. H., Eikema, D. -J., Afansyev, B., Bosman, P., Smiers, F., Diez-Martin, J. L., Arrais-Rodrigues, C., Koc, Y., Poire, X., Sirvent, A., Kroger, N., Porta, F., Holter, W., Bloor, A., Jubert, C., Ganser, A., Tanase, A., Menard, A. -L., Pioltelli, P., Perez-Simon, J. A., Ho, A., Aljurf, M., Russell, N., Labussiere-Wallet, H., Kerre, T., Rocha, V., Socie, G., Risitano, A., Dufour, C., and Peffault de Latour, R.
Subjects: medicine.medical_specialty, Transplantation Conditioning, Cyclophosphamide, medicine.medical_treatment, Eltrombopag, Graft vs Host Disease, Gastroenterology, 03 medical and health sciences, chemistry.chemical_compound, 0302 clinical medicine, Internal medicine, Medicine, Humans, Cumulative incidence, Prospective Studies, Aplastic anemia, Prospective cohort study, Transplantation, Univariate analysis, Neutrophil Engraftment, business.industry, Hematopoietic Stem Cell Transplantation, Anemia, Aplastic, Immunosuppression, Hematology, medicine.disease, Europe, chemistry, 030220 oncology & carcinogenesis, Transplantation, Haploidentical, business, 030215 immunology, medicine.drug
Abstract: In Press., In the absence of an HLA-matched donor, the best treatment for acquired aplastic anemia patients refractory to immunosuppression is unclear. We collected and analyzed data from all acquired aplastic anemia patients who underwent a haploidentical transplantation with posttransplant cyclophosphamide in Europe from 2011 to 2017 (n = 33). The cumulative incidence of neutrophil engraftment was 67% (CI95%: 51–83%) at D +28 and was unaffected by age group, stem cell source, ATG use, or Baltimore conditioning regimen. The cumulative incidence of grades II–III acute GvHD was 23% at D +100, and limited chronic GvHD was 10% (0–20) at 2 years, without cases of grade IV acute or extensive chronic GvHD. Two-year overall survival was 78% (64–93), and 2-year graft-versus-host disease-free survival was 63% (46–81). In univariate analysis, the 2-year OS was higher among patients who received the Baltimore conditioning regimen (93% (81–100) versus 64% (41–87), p = 0.03), whereas age group, stem cell source, and ATG use had no effect. Our results using unmanipulated haploidentical transplantation and posttransplant cyclophosphamide for treating refractory AA patients are encouraging, but warrant confirmation in a prospective study with a larger number of patients and longer follow-up.
Published: 2019

14. Erratum: Consensus guideline for the diagnosis and treatment of tetrahydrobiopterin (BH4) deficiencies (Orphanet Journal of Rare Diseases (2020) 15: 126 DOI: 10.1186/s13023-020-01379-8)

Author: Opladen, T. López-Laso, E. Cortès-Saladelafont, E. Pearson, T.S. Sivri, H.S. Yildiz, Y. Assmann, B. Kurian, M.A. Leuzzi, V. Heales, S. Pope, S. Porta, F. García-Cazorla, A. Honzík, T. Pons, R. Regal, L. Goez, H. Artuch, R. Hoffmann, G.F. Horvath, G. Thöny, B. Scholl-Bürgi, S. Burlina, A. Verbeek, M.M. Mastrangelo, M. Friedman, J. Wassenberg, T. Jeltsch, K. Kulhánek, J. Kuseyri Hübschmann, O.
Abstract: Following the original article's publication [1] the authors asked for the correction of Fig. 2, since the names of the disease genes [GCH1 and PCBD1] in the figure published did not match the listed diseases [AR-GTPCHD and PCDD]. The correct Fig. 2 isshown below: In the context of the manuscript correction and inorder to match he text content, the words "apart from DHPRD" should be removed from the second row and second column of Table 4, as shown below: (Table Presented). © 2020 The Author(s). Reference.
Published: 2020

15. Consensus guideline for the diagnosis and treatment of tetrahydrobiopterin (BH4) deficiencies (vol 15, 126, 2020)

Author: Opladen T, López-Laso E, Cortés-Saladelafont E, Pearson TS, Sivri HS, Yildiz Y, Assmann B, Kurian MA, Leuzzi V, Heales S, Pope S, Porta F, Garcia-Cazorla A, Honzík T, Pons R, Regal L, Goez H, Artuch-Iriberri R, Hoffmann GF, Horvath G, Thöny B, Scholl-Bürgi S, Burlina A, Verbeek MM, Mastrangelo M, Friedman J, Wassenberg T, Jeltsch K, Kulhánek J, Kuseyri Hübschmann O, and International Working Group on Neurotransmitter related Disorders (iNTD)
Published: 2020

16. Consensus guideline for the diagnosis and treatment of tetrahydrobiopterin (BH4) deficiencies (vol 15, 126, 2020)

Author: Opladen, T, Lopez-Laso, E, Cortes-Saladelafont, E, Pearson, TS, Sivri, HS, Yildiz, Y, Assmann, B, Kurian, MA, Leuzzi, V, Heales, S, Pope, S, Porta, F, Garcia-Cazorla, A, Honzik, T, Pons, R, Regal, L, Goez, H, Artuch, R, Hoffmann, GF, Horvath, G, Thony, B, Scholl-Burgi, S, Burlina, A, Verbeek, MM, Mastrangelo, M, Friedman, J, Wassenberg, T, Jeltsch, K, Kulhanek, J, and Hubschmann, OK
Abstract: An amendment to this paper has been published and can be accessed via the original article.
Published: 2020

17. Ultrasonography in psoriatic arthritis: which sites should we scan?

Author: Zabotti A., Piga M., Canzoni M., Sakellariou G., Iagnocco A., Scire C. A., Adinolfi A., Azzolin I., Bandinelli F., Batticciotto A., Boffini N., Bortoluzzi A., Carrara G., Cavatorta F. P., Cagnotto G., Caprioli M., Colaci M., De Lucia O., Delle Sedie A., Denaro V., Di Matteo A., Di Sabatino V., Epis O. M., Hoxha A., Farina I., Germano G., Filippou G., Filippucci E., Focherini M. C., Gabba A., Idolazzi L., La Paglia G. M. C., Luccioli F., Macchioni P., Magnani M., Massarotti M., Mastaglio C., Navarini L., Parisi S., Diamanti A. P., Picerno V., Piras M., Porta F., Possemato N., Prevete I., Raffeiner B., Ramonda R., Ravagnani V., Rossi D., Rossi S., Scolieri P., Santoboni G., Scioscia C., Terenzi R., Tinazzi I., Toscano C., Venditti C., Volpe A., Vukatana G., Zanframundo G., Zabotti, A, Piga, M, Canzoni, M, Sakellariou, G, Iagnocco, A, Scire, C, Adinolfi, A, Azzolin, I, Bandinelli, F, Batticciotto, A, Boffini, N, Bortoluzzi, A, Carrara, G, Cavatorta, F, Cagnotto, G, Caprioli, M, Colaci, M, De Lucia, O, Delle Sedie, A, Denaro, V, Di Matteo, A, Di Sabatino, V, Epis, O, Hoxha, A, Farina, I, Germano, G, Filippou, G, Filippucci, E, Focherini, M, Gabba, A, Idolazzi, L, La Paglia, G, Luccioli, F, Macchioni, P, Magnani, M, Massarotti, M, Mastaglio, C, Navarini, L, Parisi, S, Diamanti, A, Picerno, V, Piras, M, Porta, F, Possemato, N, Prevete, I, Raffeiner, B, Ramonda, R, Ravagnani, V, Rossi, D, Rossi, S, Scolieri, P, Santoboni, G, Scioscia, C, Terenzi, R, Tinazzi, I, Toscano, C, Venditti, C, Volpe, A, Vukatana, G, and Zanframundo, G
Subjects: Genetics and Molecular Biology (all), medicine.medical_specialty, Immunology, Arthritis, Physical examination, outcomes research, psoriatic arthritis, ultrasonography, rheumatology, immunology and allergy, immunology, biochemistry, genetics and molecular biology (all), Disease, Biochemistry, Sensitivity and Specificity, General Biochemistry, Genetics and Molecular Biology, Rheumatology, Immunology and Allergy, Biochemistry, Genetics and Molecular Biology (all), NO, Tendons, 03 medical and health sciences, Psoriatic arthritis, 0302 clinical medicine, Internal medicine, medicine, Humans, 030212 general & internal medicine, Prospective cohort study, 030203 arthritis & rheumatology, medicine.diagnostic_test, business.industry, outcomes research, psoriatic arthritis, ultrasonography, Arthritis, Psoriatic, Bursa, Synovial, medicine.disease, Joints, Observational study, Differential diagnosis, business
Abstract: In psoriatic arthritis (PsA), ultrasonography (US) plays a growing role in the differential diagnosis and in monitoring treatment response.1 PsA is a heterogeneous disease with different domains and peculiar sites involved.2 Therefore, a dedicated US composite score is needed to monitor disease activity and to identify structural damage progression. A recently published Systematic Literature Review (SLR) identified only two US scores specifically developed for PsA (ie, 5TPD and PsA-Son) and, although these had a good sensitivity to detect inflammation and a good feasibility, they have not been validated in other series.1 3 4 Recently, the Study Group for US of the Italian Society of Rheumatology promoted the Ultrasound in PSoriatic Arthritis TREAtMent (UPSTREAM) study (registered at ClinicalTrial.gov, NCT03330769). UPSTREAM is a multicentre observational prospective cohort study and it represents the first …
Published: 2018

18. Tifus Murino en España durante el periodo 2004-2013

Author: Monteagudo-Monterde, S. and Segura-Porta, F.
Abstract: [ES] Objetivos: Estimar la incidencia y otros datos epidemiológicos del tifus murino en España. Material y métodos: Se analizaron aquellos pacientes cuyo diagnóstico al alta hospitalaria entre los años 2004-2013 se hubiera codificado en el CMBD, según el CIE 9-MC, como 081.0 (tifus endémico murino portado por pulgas). Resultados: Se diagnosticaron 56 pacientes (83,93% como diagnóstico principal y 16,07% como secundario) y 50 de ellos se detectaron en las Islas Canarias. La tasa de incidencia global por millón de habitantes y año, fue de 0,13 (IC95% de 0,10 a 0,17) en España. La media de edad fue de 49,75 años y un rango de 8-84 años. El riesgo relativo (RR) hombre-mujer fue 1,7:1. La mayoría de casos se concentraron en el tercer trimestre del año. Conclusiones: La incidencia del TM en España es baja y heterogénea. La enfermedad se detectó principalmente en Canarias, varones y el tercer trimestre del año.[EN] Objectives: To estimate the incidence and other epidemiological data of murine typhus in Spain. Material and methods: We analyzed those patients whose diagnosis at hospital discharge between 2004-2013 years had been coded in the Hospitalization Minimum Data Set, according to the ICD 9-MC, as 081.0 (Typhus, Endemic Flea-Borne). Main results: 56 patients were diagnosed (83.93% as main diagnosis and 16.07% as secondary) and 50 of them were detected in the Canary Islands. The global incidence rate per million inhabitants per year was 0.13 (CI95% from 0.10 to 0.17) in Spain.The mean age was 49.75 years and a range of 8-84 years. The relative risk (RR) male-female was 1.7: 1. The majority of cases were concentrated in the third quarter of the year. Conclusions: The incidence of TM in Spain is low and heterogeneous. The disease was detected mainly in the Canary Islands, males and the third quarter of the year. No
Published: 2018

19. Selection mechanisms for microstructures and reversible martensitic transformations

Author: Della Porta, F and Ball, J
Subjects: Material Sciences, Mathematics
Abstract: The work in this thesis is inspired by the fabrication of Zn45Au30Cu25. This is the first alloy undergoing ultra-reversible martensitic transformations and closely satisfying the cofactor conditions, particular conditions of geometric compatibility between phases, which were conjectured to influence reversibility. With the aim of better understanding reversibility, in this thesis we study the martensitic microstructures arising during thermal cycling in Zn45Au30Cu25, which are complex and different in every phase transformation cycle. Our study is developed in the context of continuum mechanics and nonlinear elasticity, and we use tools from nonlinear analysis. The first aim of this thesis is to advance our understanding of conditions of geometric compatibility between phases. To this end, first, we further investigate cofactor conditions and introduce a physically-based metric to measure how closely these are satisfied in real materials. Secondly, we introduce further conditions of compatibility and show that these are nearly satisfied by some twins in Zn45Au30Cu25. These might influence reversibility as they improve compatibility between high and low temperature phases. Martensitic phase transitions in Zn45Au30Cu25 are a complex phenomenon, especially because the crystalline structure of the material changes from a cubic to a monoclinic symmetry, and hence the energy of the system has twelve wells. There exist infinitely many energy-minimising microstructures, limiting our understanding of the phenomenon as well as our ability to predict it. Therefore, the second aim of this thesis is to find criteria to select physically-relevant energy minimisers. We introduce two criteria or selection mechanisms. The first involves a moving mask approximation, which allows one to describe some experimental observations on the dynamics, while the second is based on using vanishing interface energy. The moving mask approximation reflects the idea of a moving curtain covering and uncovering microstructures during the phase transition, as appears to be the case for Zn45Au30Cu25, and many other materials during thermally induced transformations. We show that the moving mask approximation can be framed in the context of a model for the dynamics of nonlinear elastic bodies. We prove that every macroscopic deformation gradient satisfying the moving mask approximation must be of the form 1 + a(x) ⊗ n(x), for a.e. x. With regards to vanishing interface energy, we consider a one-dimensional energy functional with three wells, which simplifies the physically relevant model for martensitic transformations, but at the same time highlights some key issues. Our energy functional admits infinitely many minimising gradient Young measures, representing energy-minimising microstructures. In order to select the physically relevant ones, we show that minimisers of a regularised energy, where the second derivatives are penalised, generate a unique minimising gradient Young measure as the perturbation vanishes. The results developed in this thesis are motivated by the study of Zn45Au30Cu25, but their relevance is not limited to this material. The results on the cofactor conditions developed here can help for the understanding of new alloys undergoing ultra-reversible transformations, and as a guideline for the fabrication of future materials. Furthermore, the selection mechanisms studied in this work can be useful in selecting physically relevant microstructures not only in Zn45Au30Cu25, but also in other materials undergoing martensitic transformations, and other phenomena where pattern formation is observed.
Published: 2018

20. TALASSO-DREPANOCITOSI DA MUTAZIONI BETA+: REALMENTE UN FENOTIPO 'MILD'?

Author: Agostini, A., Notarangelo, L. D., Gorio, C., Folsi, V., Casale, M., Perrotta, S., Samperi, P., Barone, A., Arcioni, F., Pegoraro, A., Burnelli, R., Nardi, M., Giona, F., Filippini, B., Giordano, P., Del-Vecchio, G. C., Casini, T., Masera, N., Colombatti, R., Sainati, L., Porta, F., and Russo, G.
Published: 2018

21. The nonlocal Cahn-Hilliard-Hele-Shaw system with logarithmic potential

Author: Della Porta, F., Giorgini, A., and Grasselli, M.
Published: 2018

22. Development and first validation of a disease activity score for gout

Author: Scirè, Ca1, Carrara, G2, Viroli, C3, Cimmino, Ma4, Taylor, Wj5, Manara, M2, Govoni, M6, Salaffi, F7, Punzi, L8, Montecucco, C9, Matucci Cerinic M10, Minisola, G11, Collaborators Ariani A, Study Group for the Kick Off of the Italian Network for Gout S. t. u. d. y., Galossi, A, Lauriti, C, Fracassi, E, Idolazzi, L, Bardelli, M, Selvi, E, Tirri, E, Furini, F, Inverardi, F, Calabrò, A, Porta, F, Bittelli, R, Venturino, F, Capsoni, F, Prevete, I, Sebastiani, G, Selmi, C, Fabbriciani, G, D'Avola, G, Botticella, G, Serale, F, Seminara, G, D'Alessandro, G, Santo, L, Longato, L, Zaccara, E, Sinigaglia, L, Atteritano, Marco, Broggini, M, Caprioli, M, Favero, M, Sallì, S, Scarati, M, Parisi, S, Malavolta, N, Corvaglia, S, Scarpato, S, Veneto, V., Scire, C, Carrara, G, Viroli, C, Cimmino, M, Taylor, W, Manara, M, Govoni, M, Salaffi, F, Punzi, L, Montecucco, C, Matucci-Cerinic, M, Minisola, G, Ariani, A, Galossi, A, Lauriti, C, Fracassi, E, Idolazzi, L, Bardelli, M, Selvi, E, Tirri, E, Furini, F, Inverardi, F, Calabro, A, Porta, F, Bittelli, R, Venturino, F, Capsoni, F, Prevete, I, Sebastiani, G, Selmi, C, Fabbriciani, G, D'Avola, G, Botticella, G, Serale, F, Seminara, G, D'Alessandro, G, Santo, L, Longato, L, Zaccara, E, Sinigaglia, L, Atteritano, M, Broggini, M, Caprioli, M, Favero, M, Salli, S, Scarati, M, Parisi, S, Malavolta, N, Corvaglia, S, Scarpato, S, Veneto, V, Scirè, Carlo A, Carrara, Greta, Viroli, Cinzia, Cimmino, Marco A., Taylor, William J., Manara, Maria, Govoni, Marcello, Salaffi, Fausto, Punzi, Leonardo, Montecucco, Carlomaurizio, Matucci-Cerinic, Marco, Minisola, Giovanni, Ariani, Alarico, Galossi, Alessandra, Lauriti, Ciro, Fracassi, Elena, Idolazzi, Luca, Bardelli, Marco, Selvi, Enrico, Tirri, Enrico, Furini, Federica, Inverardi, Flora, Calabrò, Andrea, Porta, Francesco, Bittelli, Raffaele, Venturino, Francesco, Capsoni, Franco, Prevete, Immacolata, Sebastiani, Giandomenico, Selmi, Carlo, Fabbriciani, Gianluigi, D'Avola, Giovanni, Botticella, Giulia, Serale, Francesca, Seminara, Giulia, D'Alessandro, Giuseppe, Santo, Leonardo, Longato, Lorena, Zaccara, Eleonora, Sinigaglia, Luigi, Atteritano, Marco, Broggini, Marco, Caprioli, Marta, Favero, Marta, Sallì, Salvatore, Scarati, Marco, Parisi, Simone, Malavolta, Nazzarena, Corvaglia, Stefania, Scarpato, Salvatore, and Veneto, Vittorio
Subjects: Male, medicine.medical_specialty, Visual analogue scale, Aged, Arthralgia, Factor Analysis, Statistical, Female, Follow-Up Studies, Gout, Humans, Joints, Linear Models, Middle Aged, Pain Measurement, Patient Reported Outcome Measures, Regression Analysis, Reproducibility of Results, Uric Acid, Disease Progression, Severity of Illness Index, NO, disease activity, gout, patient perspective, 03 medical and health sciences, 0302 clinical medicine, gout, Rheumatology, Discriminant function analysis, Linear regression, Criterion validity, medicine, 030212 general & internal medicine, 030203 arthritis & rheumatology, business.industry, Construct validity, Regression analysis, Statistical, medicine.disease, Linear discriminant analysis, patient perspective, Physical therapy, Rheumatology, Factor Analysis, business, Factor Analysis, disease activity
Abstract: Objective To develop a new composite disease activity score for gout and provide its first validation. Methods Disease activity has been defined as the ongoing presence of urate deposits that lead to acute arthritis and joint damage. Every measure for each Outcome Measures in Rheumatology core domain was considered. A 3-step approach (factor analysis, linear discriminant analysis, and linear regression) was applied to derive the Gout Activity Score (GAS). Decision to change treatment or 6-month flare count were used as the surrogate criteria of high disease activity. Baseline and 12-month followup data of 446 patients included in the Kick-Off of the Italian Network for Gout cohort were used. Construct- and criterion-related validity were tested. External validation on an independent sample is reported. Results Factor analysis identified 5 factors: patient-reported outcomes, joint examination, flares, tophi, and serum uric acid (sUA). Discriminant function analysis resulted in a correct classification of 79%. Linear regression analysis identified a first candidate GAS including 12-month flare count, sUA, visual analog scale (VAS) of pain, VAS global activity assessment, swollen and tender joint counts, and a cumulative measure of tophi. Alternative scores were also developed. The developed GAS demonstrated a good correlation with functional disability (criterion validity) and discrimination between patient- and physician-reported measures of active disease (construct validity). The results were reproduced in the external sample. Conclusion This study developed and validated a composite measure of disease activity in gout. Further testing is required to confirm its generalizability, responsiveness, and usefulness in assisting with clinical decisions.
Published: 2016

23. Seminario della Scuola di Dottorato in Archeologia

Author: Casadei, E., Oselini, V., and Porta, F.
Published: 2017

24. Rischio di recidiva nel linfoma di Hodgkin pediatrico: dalla esperienza AIEOP ad una strategia europea

Author: Mascarin, M., Bernasconi, S., Bertolini, P., Bianchi, M., Buffardi, S., Casale, F., Casini, T., Cellini, M., Cesaro, S., Civino, A., Consarino, C., Cosmi, C., D’Amico, S., De Santis, R., Facchini, E., Fagioli, F., Farruggia, P., Favre, C., Felici, L., Galimberti, D., Garaventa, A., Iaria, G., Indolfi, P., Locatelli, F., Moleti, M. L., Muggeo, P., Mura, R. M., Pericoli, R., Perruccio, K., Pierani, P., Pillon, M., Porta, F., Provenzi, M., Rinieri, S., Rondelli, R., Russo, G., Sala, A., Santoro, N., Sau, A., Sperli, D., Todesco, A., Tolva, A., Varasso, G., Verzegnassi, F., Vinti, L., Zecca, M., Lopci, E., Elia, C., Birri, S., Sabattini, E., D’Amore, E., and Burnelli, R.
Subjects: pediatria, linfoma hodgkin, risultati, linfoma hodgkin, pediatria, risultati
Published: 2017

25. Progetto di restauro, consolidamento e miglioramento sismico del Duomo di Pienza

Author: Podestà, S., Brignola, A., Luchini, C., Porta, F., and Romei, F.
Subjects: Duomo di Pienza, valutazione delle sicurezza sismica, meccanismi locali, Duomo di Pienza, miglioramento sismico, valutazione delle sicurezza sismica, meccanismi locali, miglioramento sismico
Published: 2017

26. La messa in sicurezza dei beni monumentali a seguito dell’evento sismico del 30 ottobre 2016: il caso di Norcia

Author: Podestà, S., Brignola, A., Scandolo, L., Luchini, C., Porta, F., and Romei, F.
Subjects: 2016 Central Italy Earthquake, Norcia, Temporary safety measures, 2016 Central Italy Earthquake, Temporary safety measures, Norcia, Cultural heritage buildings, Cultural heritage buildings
Published: 2017

27. Il linfoma di Hodgkin in età pediatrica: risultati a lungo termine del protocollo AIEOP-MH’89

Author: Robazza, M., Mascarin, M., Elia, C., Barra, S., Bianchi, M., Buffardi, S., Bunkheila, F., Caniglia, M., Casini, T., Cellini, M., Cesaro, Simone, Consarino, C., D’Amico, S., De Santis, R., Farruggia, P., Guerrini, G., Garaventa, A., Indolfi, P., Locatelli, F., Moleti, M. L., Nardi, M., Pession, A., Pierani, P., Porta, F., Provenzi, M., Riccardi, U., Rondelli, R., Sala, A., Santoro, N., Sau, A., Scarzello, G., Todesco, A., Zanazzo, G. A., Zecca, M., and Brunelli, R.
Subjects: chemioterapia, protocollo MH 89, Linfoma di Hodgkin, Linfoma di Hodgkin, protocollo MH 89, chemioterapia
Published: 2016

28. The Italian MSUS Study Group recommendations for the format and content of the report and documentation in musculoskeletal ultrasonography in rheumatology

Author: Iagnocco A, Porta F, CUOMO, Giovanna, Delle Sedie A, Filippucci E, Grassi W, Sakellariou G, Epis O, Adinolfi A, Ceccarelli F, De Lucia O, Di Geso L, Di Sabatino V, Gabba A, Gattamelata A, Gutierrez M, Massaro L, Massarotti M, Perricone C, Picerno V, Ravagnani V, Riente L, Scioscia C, Naredo E, Filippou G, Musculoskeletal Ultrasound Study Group of the Italian Society of Rheumatology, Iagnocco, A, Porta, F, Cuomo, Giovanna, Delle Sedie, A, Filippucci, E, Grassi, W, Sakellariou, G, Epis, O, Adinolfi, A, Ceccarelli, F, De Lucia, O, Di Geso, L, Di Sabatino, V, Gabba, A, Gattamelata, A, Gutierrez, M, Massaro, L, Massarotti, M, Perricone, C, Picerno, V, Ravagnani, V, Riente, L, Scioscia, C, Naredo, E, Filippou, G, and Musculoskeletal Ultrasound Study Group of the Italian Society of, Rheumatology
Subjects: Pathology, medicine.medical_specialty, Delphi Technique, Delphi method, Recommendations, Documentation, Rheumatology, Musculoskeletal ultrasonography, Internal medicine, Report, Rheumatic Diseases, Ultrasound, Medicine, Humans, Medical physics, Pharmacology (medical), recommendations, report, ultrasound, Grading (education), Musculoskeletal System, computer.programming_language, Ultrasonography, Response rate (survey), Modalities, business.industry, Italy, business, computer, Delphi
Abstract: Objective:The objective of this study was to draw up a set of recommendations for the format and content of the musculoskeletal ultrasonography (MSUS) report in rheumatology. METHODS: A panel of rheumatologists, members of the MSUS Study Group of the Italian Society of Rheumatology, met in order to identify the main discrepancies in the MSUS report. A set of 15 recommendations was then defined, aimed at resolving the main discrepancies. They consisted of information about the motivations for the MSUS examination, the equipment, the US modalities and scanning technique, a list of the examined structures and findings, the scoring/grading systems, the number of images and main findings to include and conclusions. Subsequently a Delphi-based procedure was started in order to obtain agreement on a core set of recommendations. Consensus for each recommendation was considered achieved when the percentage of agreement was >75%. RESULTS: Three complete rounds were performed. The response rate was 85.2% for the first round, 78.3% for the second and 88.9% for the third. Finally, consensus was obtained for 14 of 15 statements. These 14 statements represent the recommendations of the group for the format and content of the report and documentation in MSUS in rheumatology. CONCLUSION: To the best of our knowledge, our group has produced the first recommendations for the format and content of the report and documentation in MSUS in rheumatology. The report is an integral part of the MSUS examination and its use in a homogeneous form can help in the correct interpretation of the findings.
Published: 2013

29. PRIMO PROTOCOLLO PER NEUROBLASTOMA AD ALTO RISCHIO siop EUROPE NEUROBLASTOMA. REPORT AD INTERIM DELLA CASISTICA ITALIANA

Author: Luksch, R., Viscardi, E., Bianchi, M., Prete, A., Castellano, A., D’Angelo, P., Zanazzo, G., Moscheo, C., Manzitti, C., Vetrella, S., Tondo, A., Di Cataldo, A., Pierani, P., Bonetti, F., Pota, E., De Leonardis, F., Casazza, G., Porta, F., Provenzi, M., Cesaro, Simone, Bertolini, P., Galleni, B., and Garaventa, A.
Subjects: neuroblastoma, chemioterapia, SIOP, SIOP, chemioterapia, neuroblastoma
Published: 2015

30. C. Cimino, E. Carraro, L. Mussolin, E.S. d’Amore,S. Buffardi, A. Garaventa, A. Lombardi, R. De Santis, P. D’Angelo, P. Bulian, N. Santoro, F. Porta, R. Mura, A. Tondo, M. Cellini, A. Sala, P. Bertolini, K. Perruccio, M. Nardi, M. Piglione, S. Cesaro, M. Pillon, L. Lo Nigro

Author: Cimino, C., Carraro, E., Mussolin, L., D’Amore, E. S., Buffardi, S., Garaventa, A., Lombardi, A., De Santis, R., D’Angelo, P., Bulian, P., Santoro, N., Porta, F., Mura, R., Tondo, A., Cellini, M., Sala, A., Bertolini, P., Perruccio, K., Nardi, M., Piglione, M., Cesaro, Simone, Pillon, M., and Lo Nigro, L.
Subjects: pediatria, sopravvivenza, Linfomi non-Hodgkin, Linfomi non-Hodgkin, pediatria, sopravvivenza
Published: 2015

31. New Platinum(II) complexes in STAT3 inhibition: a promising tool for anticancer therapy

Author: Rimoldi, I., Porta, F., Villa, S., Gelain, A., Meneghetti, F., Barlocco, D., nicola ferri, Asai, A., and Facchetti, G.
Published: 2015

32. Outcome of hematopoietic stem cell transplantation for adenosine deaminase-deficient severe combined immunodeficiency

Author: Hassan, A., Booth, C., Brightwell, A., Allwood, Z., Veys, P., Rao, K., Honig, M., Friedrich, W., Gennery, A., Slatter, M., Bredius, R., Finocchi, A., Cancrini, C., Aiuti, A., Porta, F., Lanfranchi, A., Ridella, M., Steward, C., Filipovich, A., Marsh, R., Bordon, V., Al-Muhsen, S., Al-Mousa, H., Alsum, Z., Al-Dhekri, H., Ghonaium, A. al, Speckmann, C., Fischer, A., Mahlaoui, N., Nichols, K.E., Grunebaum, E., Zahrani, D. al, Roifman, C.M., Boelens, J., Davies, E.G., Cavazzana-Calvo, M., Notarangelo, L., Gaspar, H.B., European Grp Blood Marrow Transpla, European Soc Immunodeficiency, Hassan, A, Booth, C, Brightwell, A, Allwood, Z, Veys, P, Rao, K, Hönig, M, Friedrich, W, Gennery, A, Slatter, M, Bredius, R, Finocchi, A, Cancrini, C, Aiuti, Alessandro, Porta, F, Lanfranchi, A, Ridella, M, Steward, C, Filipovich, A, Marsh, R, Bordon, V, AL MUHSEN, S, AL MOUSA, H, Alsum, Z, AL DHEKRI, H, AL GHONAIUM, A, Speckmann, C, Fischer, A, Mahlaoui, N, Nichols, Ke, Grunebaum, E, AL ZAHRANI, D, Roifman, Cm, Boelens, J, Davies, Eg, CAVAZZANA CALVO, M, Notarangelo, L, and Gaspar, Hb
Subjects: Oncology, Male, Transplantation Conditioning, Adenosine Deaminase, Genetic enhancement, medicine.medical_treatment, T-Lymphocytes, Hematopoietic stem cell transplantation, Kaplan-Meier Estimate, Biochemistry, humoral, Adenosine deaminase, newborn, immune system diseases, Agammaglobulinemia, hemic and lymphatic diseases, Child, Immunity, Cellular, Hematology, biology, Histocompatibility Testing, Graft Survival, Hematopoietic Stem Cell Transplantation, surgical procedures, operative, Treatment Outcome, Child, Preschool, Female, Unrelated Donors, medicine.medical_specialty, Immunology, preschool, Internal medicine, medicine, Humans, Lymphocyte Count, Retrospective Studies, Settore MED/38 - Pediatria Generale e Specialistica, Severe combined immunodeficiency, agammaglobulinemia, transplantation conditioning, severe combined immunodeficiency, humans, retrospective studies, infant, newborn, child, child, preschool, kaplan-meier estimate, infant, lymphocyte count, histocompatibility testing, immunity, cellular, unrelated donors, graft survival, treatment outcome, myeloablative agonists, hematopoietic stem cell transplantation, siblings, adenosine deaminase, male, female, t-lymphocytes, immunity, humoral, business.industry, Siblings, Infant, Newborn, Infant, Cell Biology, Myeloablative Agonists, medicine.disease, immunity, Adenosine deaminase deficiency, Immunity, Humoral, Transplantation, biology.protein, Severe Combined Immunodeficiency, business, cellular
Abstract: Deficiency of the purine salvage enzyme adenosine deaminase leads to SCID (ADA-SCID). Hematopoietic cell transplantation (HCT) can lead to a permanent cure of SCID; however, little data are available on outcome of HCT for ADA-SCID in particular. In this multicenter retrospective study, we analyzed outcome of HCT in 106 patients with ADA-SCID who received a total of 119 transplants. HCT from matched sibling and family donors (MSDs, MFDs) had significantly better overall survival (86% and 81%) in comparison with HCT from matched unrelated (66%; P < .05) and haploidentical donors (43%; P < .001). Superior overall survival was also seen in patients who received unconditioned transplants in comparison with myeloablative procedures (81% vs 54%; P < .003), although in unconditioned haploidentical donor HCT, nonengraftment was a major problem. Long-term immune recovery showed that regardless of transplant type, overall T-cell numbers were similar, although a faster rate of T-cell recovery was observed after MSD/MFD HCT. Humoral immunity and donor B-cell engraftment was achieved in nearly all evaluable surviving patients and was seen even after unconditioned HCT. These data detail for the first time the outcomes of HCT for ADA-SCID and show that, if patients survive HCT, long-term cellular and humoral immune recovery is achieved.
Published: 2012

33. PROSPECTIVE STUDY OF 84 PATIENTS WITH WISKOTT-ALDRICH SYNDROME AND X-RECESSIVE PIASTRINOPENIA: RESULTS OF THE APPLICATION OF COMMON PROTOCOL FOR DIAGNOSIS AND TREATMENT OF WAS/XLT

Author: Soresina, A., Notarangelo, L. D., Ventura, A., Locatelli, F., Dufour, C., Galanello, R., Zanesco, L., Biondi, A., Mattia, D., Aiuti, A., Maurizio Arico', Azzari, C., Cornelli, P. E., Rossi, P., Martino, S., Specchia, F., Pignata, C., Izzi, G., Mazza, C., Moratto, D., Giliani, S., Rondelli, R., Pession, A., Ugazio, A. G., Plebani, A., Pietrogrande, M. C., Porta, F., Soresina, A., Notarangelo, L. D., Ventura, A., Locatelli, F., Dufour, C., Galanello, R., Zanesco, L., Biondi, A., De Mattia, D., Aiuti, A., Arico, M., Azzari, C., Cornelli, P. E., Rossi, P., Martino, S., Specchia, F., Pignata, Claudio, Izzi, G., Mazza, C., Moratto, D., Giliani, S., Rondelli, R., Pession, A., Ugazio, A. G., Plebani, A., Pietrogrande, M. C., and Porta, F.
Published: 2012

34. DIFETTO DI LINFOCITI Ccr4/Ccr6+ IN PAZIENTI CON SINDROME DI IPER IgE CORRELA CON RIDUZIONE DEI LINFOCITI Th17

Author: Guadrini L, Giacomelli M, Moratto M, Caracciolo S, Nigro S, Lougaris V, Pajno G, Porta F. Salpietro A, Monfredini C, Corrente S, Soresina A, Aiuti A, Cancrini C, Ragusa G, Gallizzi R, Tommasini A, Montin D, Specchia G. G, Baraldi E, Nespoli L, Duse M, Plebani A, Badolato R., RICCI, GIAMPAOLO, Guadrini L, Giacomelli M, Moratto M, Caracciolo S, Nigro S, Lougaris V, Pajno G, Porta F. Salpietro A, Monfredini C, Corrente S, Soresina A, Aiuti A, Cancrini C, Ragusa G, Gallizzi R, Tommasini A, Montin D, Ricci G, Specchia G.G, Baraldi E, Nespoli L, Duse M, Plebani A, and Badolato R
Subjects: SINDROME DA IPER IGE, LINFOCITI TH17
Published: 2012

35. Marriage and parenthood among childhood cancer survivors: a report from the Italian AIEOP Off-Therapy Registry

Author: Pivetta, E, Maule, Mm, Pisani, P, Zugna, D, Haupt, R, Jankovic, M, Aricò, M, Casale, F, Clerico, A, Cordero di Montezemolo, L, Kiren, V, Locatelli, F, Palumbo, G, Pession, A, Pillon, M, Santoro, N, Terenziani, M, Valsecchi, Mg, Dama, E, Magnani, C, Merletti, F, Pastore, G, Fagioli, F, Bona, G, Dini, G, Carnelli, V, Biondi, A, Zecca, M, Conter, V, Porta, F, Fedeli, F, Massimino, M, Nespoli, L, Roncarolo, Mg, Carli, M, Cesaro, S, Memo, L, Colleselli, P, Battisti, L, Tamaro, Paolo, Mascarin, M, Nocerino, A, Izzi, G, Paolucci, P, Borgna, Pc, Vecchi, V, Abate, Me, Acquaviva, A, Favre, C, Aversa, F, Pierani, P, Felici, L, Visani, G, Fioritoni, G, Foa, R, Riccardi, R, Frega, G, Poggi, V, Amendola, G, Filosa, A, Ladogana, S, Presta, G, Pozzi, S, De Mattia, D, Consarino, C, Nobile, F, Sperlì, D, D'Angelo, P, Marino, S, Gallisai, D, Targhetta, R., Pivetta, E, Maule, M, Pisani, P, Zugna, D, Haupt, R, Jankovic, M, Aricò, M, Casale, F, Clerico, A, Cordero di Montezemolo, L, Kiren, V, Locatelli, F, Palumbo, G, Pession, A, Pillon, M, Santoro, N, Terenziani, M, Valsecchi, M, Dama, E, Magnani, C, Merletti, F, Pastore, G, Maule, Mm, Valsecchi, Mg, Fagioli, F, Bona, G, Dini, G, Carnelli, V, Biondi, A, Zecca, M, Conter, V, Porta, F, Fedeli, F, Massimino, M, Nespoli, L, Roncarolo, Mg, Carli, M, Cesaro, S, Memo, L, Colleselli, P, Battisti, L, Tamaro, Paolo, Mascarin, M, Nocerino, A, Izzi, G, Paolucci, P, Borgna, Pc, Vecchi, V, Abate, Me, Acquaviva, A, Favre, C, Aversa, F, Pierani, P, Felici, L, Visani, G, Fioritoni, G, Foa, R, Riccardi, R, Frega, G, Poggi, V, Amendola, G, Filosa, A, Ladogana, S, Presta, G, Pozzi, S, De Mattia, D, Consarino, C, Nobile, F, Sperlì, D, D'Angelo, P, Marino, S, Gallisai, D, and Targhetta, R.
Subjects: Adult, Male, Parents, Pediatrics, medicine.medical_specialty, Total fertility rate, media_common.quotation_subject, Population, Editorials and Perspectives, Fertility, off-therapy, cancer survival, Cohort Studies, Quality of life, Marriage and parenthood, Medicine, Humans, cancer, cancer survivors, childhood, Registries, Survivors, Marriage, education, Child, media_common, education.field_of_study, business.industry, Infant, Newborn, Infant, Hematology, Original Articles, Middle Aged, childhood cancer, fertility, long-term survivors, marriage, quality of life, El Niño, Italy, Child, Preschool, Hematologic Neoplasms, Cohort, Marital status, Female, business, Cohort study, Follow-Up Studies
Abstract: Background The aim of this study was to describe the patterns of marriage and parenthood in a cohort of childhood cancer survivors included in the Off-Therapy Registry maintained by the Italian Association of Pediatric Hematology and Oncology. Design and Methods We analyzed a cohort of 6,044 patients diagnosed with cancer between 1960 and 1998, while aged 0 to 14 years and who were 18 years old or older by December 2003. They were followed up through the regional vital statistics registers until death or the end of follow up (October 30, 2006), whichever occurred first, and their marital status and date of birth of their children were recorded. The cumulative probabilities of being married and having a first child were computed by gender and compared by tumor type within the cohort. Marriage and fertility rates (the latter defined as the number of live births per woman-year) were compared with those of the Italian population of the same age, gender, area of residence and calendar period by means of the observed to expected (O/E) ratios. Results During the follow-up period, 4,633 (77%) subjects had not married. The marriage O/E ratios were 0.56 (95% CI: 0.51-0.61) and 0.70 (95% CI: 0.65-0.76) among men and women, respectively. Overall, 263 men had 367 liveborn children, and 473 women had 697 liveborn children. The female fertility O/E ratio was 0.57 (95% CI: 0.53-0.62) overall, and 1.08 (95% CI: 0.99-1.17) when analyses were restricted to married/cohabiting women Conclusions Childhood cancer survivors are less likely to marry and to have children than the general population, confirming the life-long impact of their previous disease on their social behavior and choices. The inclusion of counseling in the strategies of management and long-term surveillance of childhood cancer patients could be beneficial to survivors as they approach adulthood. ©2011 Ferrata Storti Foundation.
Published: 2011

36. The Italian MSUS Study Group Recommendations for the format and content of the report and documentation in MSUS in Rheumatology

Author: Iagnocco, A., Porta, F., Cuomo, G., Delle Sedie, A., Filippucci, E., Grassi, W., Sakellariou, G., Epis, O., Adinolfi, A., Ceccarelli, F., De Lucia, O., Di Geso, L., Di Sabatino, V., Gabba, A., Gattamelata, A., Gutierrez, M., Massaro, L., Massarotti, M., Perricone, C., Picerno, V., Ravagnani, V., Riente, Lucrezia, Scioscia, C., Naredo, E., and Filippou, G.
Published: 2014

37. indicazioni e risultati del trapianto allogenico in età pediatrica

Author: Prete, A., Rondelli, R., Zecca, M., Fagioli, F., Lanino, E., Messina, C., Rovelli, A., Porta, F., Iori, A. P., Favre, C., Rabusin, M., Ripaldi, M., Caselli, D., Lo Nigro, L., Cesaro, Simone, Caniglia, M., Di Bartolomeo, P., Ziino, O., Orofino, M. G., Paolucci, P., Pession, A., and Locatelli, F.
Subjects: RISULTATI, PEDIATRIA, TRAPIANO DI MIDOLLO ALLOGENICO
Published: 2014

38. Unireuma Reumatologia per studenti e medici di medicina generale

Author: Adami, S., Afeltra, A., Alessandri, C., Alunno, A., Atteritano, M., Bagnato, G., Bandinelli, F., Bartoloni, E., Bellucci, E., Bombardieri, S., Calabrò, A., Camellini, D., Cantarini, L., Cantatore, F. P., Caporali, R., Carli, L., Cauli, A., Cimmino, M. A., Cipriani, P., Conti, F., Corrado, A., Cuomo, G., Cutolo, M., De Vita, S., Ferraccioli, G., Ferrante, A., Ferri, C., Galeazzi, M., Gatti, D., Generini, S., Gerli, R., Gerloni, V., Gerosa, M., Giacomelli, R., Giuggioli, D., Govoni, M., Grassi, W., Gremese, E., Iaiani, G., Iannone, F., Ibba, V., La Montagna, G., Lapadula, G., Mameli, A., Margiotta, D. P., Mathieu, A., Matucci Cerinic, M., Meliconi, R., Meroni, M., Meroni, P. L., Minniti, A., Montecucco, C., Mosca, M., Mura, V., Passiu, G., Perniola, S., Perosa, F., Perricone, R., Porta, F., Priori, R., Pulsatelli, L., Punzi, L., Rossini, M., Ruscitti, P., Salaffi, F., Scrivo, R., Sebastiani, M., Spadaro, A., Sulli, A., Triolo, G., Vacca, A., Vadacca, M., Valentini, G., Valesini, G., Viapiana, O., and Zampogna, G.
Published: 2014

39. Optimization of the procedure for obtaining DPSCs by means of fragile fracture in clean room conditions

Author: Paganelli, C., Lanfranchi, A., Porta, F., Valdivia-Gandur, I., Arroyo-Bote, S., Martinez-Arroyo, J., Martinez-Osorio, J., Carvalho-Lobato, P., Tallón-Walton, V., and Maria Cristina Manzanares Céspedes
Published: 2014

40. Survival of children with cancer in Italy, 1989-98. A report from the hospital based registry of the Italian Association of Pediatric Haematology and Oncology (AIEOP)

Author: Pession A, Dama E, Rondelli R, Magnani C, De Rosa M, Locatelli F, Fagioli F, Haupt R, Jankovic M, Terracini B, Merletti F, Pastore G, Italian Association of Paediatric Haematology, Oncology Madon E, Dini G, Carnelli V, Fedeli F, Fossati Bellani F, Masera G, Cornelli PE, Porta F, Dorizzi A, Nespoli A, Carli M, Marradi P, Rodeghiero F, Musi L, Mascarin M, Nocerino A, Izzi G, Paolucci P, Ambrosioni G, Picci P, Borgna Pignatti C, Bernini G, Morgese G, Favre C, Aversa F, Pierani P, Di Marzio A, Foà R, De Rossi G, Donfrancesco A, Castello MA, Casale F, Poggi V, Auricchio S, Antonelli P, Ladogana S, De Mattia D, Magro S, Nobile F, Aricò M, Schilirò G, Gallisai D, Argiolu F., TAMARO, PAOLO, Pession, A, Dama, E, Rondelli, R, Magnani, C, De Rosa, M, Locatelli, F, Fagioli, F, Haupt, R, Jankovic, M, Terracini, B, Merletti, F, Pastore, G, Italian Association of Paediatric, Haematology, Oncology Madon, E, Dini, G, Carnelli, V, Fedeli, F, Fossati Bellani, F, Masera, G, Cornelli, Pe, Porta, F, Dorizzi, A, Nespoli, A, Carli, M, Marradi, P, Rodeghiero, F, Musi, L, Tamaro, Paolo, Mascarin, M, Nocerino, A, Izzi, G, Paolucci, P, Ambrosioni, G, Picci, P, Borgna Pignatti, C, Bernini, G, Morgese, G, Favre, C, Aversa, F, Pierani, P, Di Marzio, A, Foà, R, De Rossi, G, Donfrancesco, A, Castello, Ma, Casale, F, Poggi, V, Auricchio, S, Antonelli, P, Ladogana, S, De Mattia, D, Magro, S, Nobile, F, Aricò, M, Schilirò, G, Gallisai, D, Argiolu, F., Pession A, Dama E, Rondelli R, Magnani C, De Rosa M, Locatelli F, Fagioli F, Haupt R, Jankovic M, Terracini B, Merletti F, Pastore G, and on behalf of the Italian Association of Paediatric Haematology and Oncology
Subjects: Oncology, Male, Cancer Research, medicine.medical_specialty, Pediatrics, Adolescent, Lymphoproliferative disorders, Cancer registration, survival, children cancer, Internal medicine, Neuroblastoma, Neoplasms, medicine, Humans, Survivors, Sex Distribution, Child, Survival rate, Childhood cancer, Gender, Italy, Survival, Hematology, business.industry, Hazard ratio, Cancer, Infant, medicine.disease, Lymphoma, El Niño, Child, Preschool, Female, business, Epidemiologic Methods
Abstract: We describe the survival patterns of 10,791 Italian children (age 0-14) diagnosed with cancer during 1989-1998 and who were included in the hospital-based registry of the Italian Association of Paediatric Haematology and Oncology. Five-year cumulative survival percentages were 76% for lymphoproliferative disorders and 68% for solid tumours. Survival rates in 1994-1998 significantly improved for acute lymphocytic leukaemia (ALL), acute non-lymphocytic leukaemia, Hodgkin's lymphoma and Wilms' tumour. Gender and age were determinants of survival for some specific types of cancer. Girls with ALL and neuroblastoma exhibited a significant advantage (hazard ratio HR 0.72, 0.62-0.83) and disadvantage (HR 0.73, 0.59-0.90) over boys, respectively. Children with a Wilms' tumour diagnosed above age 3 had a worse prognosis than younger children (HR 2.3, 1.4-4.1). The persisting gender-related difference in survival rate for ALL requires understanding as to whether it is attributable to delays in the adoption of more recent therapeutic protocols, while the corresponding findings for Wilms' tumour and neuroblastoma deserve further biological interpretation.
Published: 2008

41. Dizionario della critica militante

Author: LEONELLI, Giuseppe, LA PORTA F., Leonelli, Giuseppe, and LA PORTA, F.
Subjects: Letteratura italiana, dizionario, critica, Novecento
Abstract: Il capitolo disegna lo stato della critica letteraria italiana di estrazione militante, ma anche accademica,con sensibilità militante, negli anni Ottanta del Novecento e s'interroga sul futuro della critica.
Published: 2007

42. Reduced cytokines release and myocardial damage in coronary artery bypass

Author: Colagrande, L, Porta, F, Martino, A, Sangalli, F, Avalli, L, FORMICA, FRANCESCO, PAOLINI, GIOVANNI, Colagrande, L, Formica, F, Porta, F, Martino, A, Sangalli, F, Avalli, L, and Paolini, G
Subjects: Arginine, cabg, cardioplegia, myocardial damage, inflammatory citokines, MED/23 - CHIRURGIA CARDIACA
Abstract: BACKGROUND: Recently, L-arginine has been added to cardioplegia to limit myocardial ischemic damage. The mechanism of action is related to the production of nitric oxide, with vasodilatation and reduction of endothelial dysfunction. Our prospective randomized study on coronary artery bypass patients investigates the effect of L-arginine on myocardial stress as expressed by myocardial cytokines release and myocardial ischemia in terms of troponin T concentration. METHODS: Coronary artery surgery patients were randomly assigned to receive 7.5 g L-arginine in 500 mL of cardioplegic solution (group A). Group B was used as control. Cold blood 4:1 anterograde and retrograde cardioplegia with warm induction was administered. Blood samples were collected from the retrograde coronary sinus catheter to determine interleukin-2 receptor, interleukin-6, and tumor necrosis factor levels. Serum samples at different time points were also analyzed to measure myocardial ischemia markers. Hemodynamic and echocardiographic evaluations were obtained perioperatively. RESULTS: Sixty-five patients were enrolled (group A, treated with L-arginine, n = 33; group B, control, n = 32). Wedge pressure and intensive care unit stay were significantly reduced in group A (p = 0.023 and p = 0.03, respectively). Cytokines levels were lower in group A, with a significance for interleukin-6 (p = 0.026); troponin T was reduced in treated patients (0.33 versus 0.57 ng/mL at 18 hours: p = 0.009). CONCLUSIONS: Coronary artery surgery patients benefit from L-arginine cardioplegia supplementation in terms of reduced inflammatory reaction, limitation of myocardial ischemia, and better hemodynamic performance. Moreover, a clinical advantage is evident in terms of a shorter intensive care unit stay in patients treated with L-arginine.
Published: 2006

43. Pequeñas y Medianas Empresas en América Latina e Internacionalización. Apertura, Liberalización y Políticas

Author: PIETROBELLI, Carlo, PORTA F, MOORI KOENIG V., Pietrobelli, Carlo, Porta, F, and MOORI KOENIG, V.
Published: 2005

44. Trapianto di cellule staminali emopoietiche allogenico in pazienti pediatrici con leucemia mieloide acuta in RC2:studio del gruppo trapianto di cellule staminali emopoietiche e terapia cellulare dell'AIEOP

Author: Fagioli F., Zecca M., Locatelli F., Uderzo C., Dini G., Cesaro S., Berger M., Di Bartolomeo P., Favre C., Andolina M., Porta F., Arcese W., Rondelli R., Messina C., PESSION, ANDREA, Fagioli F., Zecca M., Locatelli F., Uderzo C., Dini G., Cesaro S., Berger M., Di Bartolomeo P., Pession A., Favre C., Andolina M., Porta F., Arcese W., Rondelli R., and Messina C.
Published: 2005

45. L-arginine effects on myocardial stress in cardiac surgery: Preliminary results

Author: Colagrande L, Francesco Formica, Porta F, Brustia M, Avalli L, Sangalli F, Muratore M, Paolini G, Colagrande, L, Formica, F, Porta, F, Brustia, M, Avalli, L, Sangalli, F, Muratore, M, and Paolini, G
Subjects: Arginine, cardioplegic solution, inflammatory citokines, MED/23 - CHIRURGIA CARDIACA
Abstract: BACKGROUND: L-arginine in addition to cardioplegia stimulates the release of nitric oxide and increases coronary blood flow, decreasing platelet activation and leukocyte adhesion. The aim of our study was to determine the feasibility and the efficacy of the addition of L-arginine to antegrade and retrograde blood cardioplegia in reducing myocardial damage and stress. METHODS: Twenty-eight consecutive patients who underwent coronary artery bypass grafting were randomized to receive 7.5 g of L-arginine in 500 ml of cardioplegic solution. To assess safety of use of L-arginine, hemodynamic evaluation was performed before sternum opening, at sternum closure, and 1 hour after arrival in the intensive care unit to measure cardiac index, systemic and pulmonary vascular resistances, and pulmonary capillary wedge pressure. Moreover, transesophageal echocardiography was performed to assess myocardial contractility. To determine the effects on myocardial stress, blood samples were taken from the retrograde coronary sinus catheter for lactate, interleukin (IL)-2 receptor, IL-6 and tumor necrosis factor (TNF)-alpha levels. Serum samples (preoperatively, 2, 18 and 42 hours after aortic cross-clamping removal) were also analyzed to measure creatine phosphokinase, creatine kinase-MB mass, cardiac troponin T, platelets, and leukocytes. RESULTS: We found statistical differences for IL-2 receptor, IL-6, TNF-alpha, platelets and leukocytes, in favor of the treated group, and decreasing trends in creatine kinase-MB mass and troponin T levels. CONCLUSIONS: The present study shows the positive effects of the addition of L-arginine to cardioplegia. Reduced IL-2 receptor, IL-6 and TNF-alpha indicate a decrease in myocardial stress. Safety of Larginine is related to lower values of systemic vascular resistances and pulmonary capillary wedge pressure observed in group A postoperatively that could improve the patient's outcome in terms of a reduced need for inotropic support. Moreover, the decrease in platelet and leukocyte count in the treated group might express a reduced no-reflow phenomenon and a better reperfusion, limiting endothelial injury from oxygen radical production.
Published: 2005

46. POSTERIOR REVERSIBLE ENCEPHALOPATHY SYNDROME (PRES) AND EPILEPSY: CLINICAL AND EEG FEATURES IN 18 CHILDREN

Author: Milito, G., Fazzi, E., Accorsi, P., Gambara, S., Molinaro, Anna, Schumacher, F., Notarangelo, L. D., Cavagnini, S., Porta, F., Giordano, L., Société Européenne de Neurologie Pediatrique, G., Milito, E., Fazzi, P., Accorsi, S., Gambara, Molinaro, Anna, F., Schumacher, L. D., Notarangelo, S., Cavagnini, F., Porta, and L., Giordano
Subjects: Posterior Reversible Encephalopathy Syndrome, Brain MRI, antiepileptic treatment, epilepsy, EEG
Published: 2013

47. Diffusion and applications of musculoskeletal ultrasound in Italian Rheumatology Units

Author: Iagnocco, A, Ceccarelli, F, Cuomo, G, Delle Sedie, A, Filippou, G, Filippucci, E, Grassi, W, Porta, F, Sakellariou, G, Perricone, C, and Musculoskeletal Ultrasound Study Group of the Italian Society of Rheumatology
Published: 2013

48. Unrelated donor marrow transplantation in childhood: a report from the Associazione Italiana Ematologia e Oncologia Pediatrica (AIEOP) and the Gruppo Italiano per il Trapianto Midollo Osseo (GITMO)

Author: Dini, G., Cancedda, R., Giorgiani, G., Porta, F., Messina, C., Uderzo, C., Andrea Pession, Fagioli, F., La Nasa, G., Arcese, W., Pollichieni, S., Zecca, M., Lanino, E., Mazzolari, E., Cesaro, S., Balduzzi, A., Rondelli, R., Vassallo, E., Cappelli, B., Locatelli, F., Dini, G, Cancedda, R, Giorgiani, G, Porta, F, Messina, C, Uderzo, C, Pession, A, Fagioli, F, La Nasa, G, Arcese, W, Pollichieni, S, Zecca, M, Lanino, E, Mazzolari, E, Cesaro, S, Balduzzi, A, Rondelli, R, Vassallo, E, Cappelli, B, and Locatelli, F
Subjects: Homologous, Tissue and Organ Procurement, Adolescent, Lymphoma, Acute Disease, Blast Crisis, Humans, Retrospective Studies, Child, Transplantation, Homologous, Italy, Tissue Donors, Registries, Metabolism, Inborn Errors, Leukemia, Histocompatibility Testing, Treatment Outcome, Graft vs Host Disease, Chronic Disease, Lymphoma, Non-Hodgkin, Societies, Medical, Bone Marrow Transplantation, trapianto di midollo osseo, donatore alternativo, pediatrico, pediatrico, Non-Hodgkin, Medical, Transplantation, Inborn Errors, donatore alternativo, Metabolism, trapianto di midollo osseo, hematopoietic stem cell transplantation, unrelated donor, childhood, Societies, Settore MED/15 - Malattie del Sangue
Abstract: BACKGROUND AND OBJECTIVES: Unrelated donor bone marrow transplant (UD-BMT) has become an attractive, alternative source of hematopoietic cells for patients lacking a matched sibling. The aim of this paper is to report on 520 patients below 19 years of age undergoing UD BMT in 31 Italian centers between September 1989 and December 2001, and to focus on the results achieved in the 423 patients grafted before December 2000. DESIGNS AND METHODS: In 1989 the Italian Bone Marrow Transplant Group (GITMO) and the Italian Association for Pediatric Hematology and Oncology (AIEOP) established the Italian Bone Marrow Donor Registry (IBMDR) to facilitate donor search and marrow procurement for patients lacking an HLA identical sibling. By the end of December 2001, 296,720 HLA-A, B typed volunteer donors had been cumulatively registered and 3,411 searches had been activated for Italian patients. At least one HLA-A, B, DRB1 matched donor was found for 54% of the patients and 520 UD BMTs were performed in patients below 19 years of age before December 2001. Since 1999 more than 90% of the patients < or = 14 years old, and more than 50% of the patients 15-18 years old undergoing UD BMT have been treated in AIEOP institutions. In 50% of the cases donors were found in the IBMDR, and in 50% they were found in 14 other Registries. The average time from search activation to transplant was 6 months for diseases other than chronic myeloid leukemia (CML), while for CML it was 8.7 months. RESULTS: Actuarial 100-day transplant-related mortality (TRM) was 32% in patients grafted between 1989 and 1997, and 21% for patients grafted after 1998 (p = 0.003). Twenty-eight per cent of the patients developed grade III or IV acute graft-versus-host disease (GvHD), and 20% developed extensive chronic GvHD. The rate of disease-free survival at three years was 37% for patients with acute lymphoblastic leukemia, 38% for acute myeloid leukemia or myelodysplastic syndrome patients, 59% for patients with inborn errors, and 51% for patients with CML. INTERPRETATION AND CONCLUSIONS: We conclude that the IBMDR has benefited a substantial number of patients lacking a matched sibling and has facilitated the recruitment of UDs into the international donor pool. Results show a positive trend after 1998, mainly due to a decrease in transplant-related-mortality. BACKGROUND AND OBJECTIVES: Unrelated donor bone marrow transplant (UD-BMT) has become an attractive, alternative source of hematopoietic cells for patients lacking a matched sibling. The aim of this paper is to report on 520 patients below 19 years of age undergoing UD BMT in 31 Italian centers between September 1989 and December 2001, and to focus on the results achieved in the 423 patients grafted before December 2000. DESIGNS AND METHODS: In 1989 the Italian Bone Marrow Transplant Group (GITMO) and the Italian Association for Pediatric Hematology and Oncology (AIEOP) established the Italian Bone Marrow Donor Registry (IBMDR) to facilitate donor search and marrow procurement for patients lacking an HLA identical sibling. By the end of December 2001, 296,720 HLA-A, B typed volunteer donors had been cumulatively registered and 3,411 searches had been activated for Italian patients. At least one HLA-A, B, DRB1 matched donor was found for 54% of the patients and 520 UD BMTs were performed in patients below 19 years of age before December 2001. Since 1999 more than 90% of the patients < or = 14 years old, and more than 50% of the patients 15-18 years old undergoing UD BMT have been treated in AIEOP institutions. In 50% of the cases donors were found in the IBMDR, and in 50% they were found in 14 other Registries. The average time from search activation to transplant was 6 months for diseases other than chronic myeloid leukemia (CML), while for CML it was 8.7 months. RESULTS: Actuarial 100-day transplant-related mortality (TRM) was 32% in patients grafted between 1989 and 1997, and 21% for patients grafted after 1998 (p = 0.003). Twenty-eight per cent of the patients developed grade III or IV acute graft-versus-host disease (GvHD), and 20% developed extensive chronic GvHD. The rate of disease-free survival at three years was 37% for patients with acute lymphoblastic leukemia, 38% for acute myeloid leukemia or myelodysplastic syndrome patients, 59% for patients with inborn errors, and 51% for patients with CML. INTERPRETATION AND CONCLUSIONS: We conclude that the IBMDR has benefited a substantial number of patients lacking a matched sibling and has facilitated the recruitment of UDs into the international donor pool. Results show a positive trend after 1998, mainly due to a decrease in transplant-related-mortality.
Published: 2002

49. Transplantation in patients with SCID: mismatched related stem cells or unrelated cord blood?

Author: Fernandes, Jf, Rocha, V, Labopin, M, Neven, B, Moshous, D, Gennery, Ar, Friedrich, W, Porta, F, Diaz de Heredia, C, Wall, D, Bertrand, Y, Veys, P, Slatter, M, Schulz, A, Chan, Kw, Grimley, M, Ayas, M, Gungor, T, Ebell, W, Bonfim, C, Kalwak, K, Taupin, P, Blanche, S, Gaspar, Hb, Landais, P, Fischer, A, Gluckman, E, Cavazzana Calvo, M, Eurocord, Inborn Errors Working Party of European Group for Blood, Marrow Transplantation: Ahmed, A, Auiti, A, Biffi, A, Cant, A, Fasth, A, Gennery, A, Hassan, A, Lankester, A, O'Mera, A, Plabani, A, Rovelli, A, Salmon, A, Scarselli, A, Thrasher, A, Van Royen, A, Villa, A, Wawer, A, Wahadneh, A, Worth, A, Belohradsky, B, Wolska, B, Gaspar, B, Bonfirm, C, Booth, C, Klein, C, Messina, C, Peters, C, Steward, C, Lindemans, C, Schuetz, C, de Heredia Rubio CD, Bensoussan, D, Gleadow, D, Lilic, D, Gambineri, Eleonora, Smith, E, Aerts, F, Caracseghi, F, Roberts, G, Davies, G, Al Mousa, H, Jossanc, H, Ozsahim, H, Hirsch, I, Meyts, I, Tezcan, I, Mueller, I, Andresc, I, Boelens, J, Fernandes, J, Folloni, J, Keuhl, J, Reichenbach, J, Stary, J, Wachowiak, J, Xu Bayford, J, Cunha, Jm, Ehlert, J, Rao, K, Sykora, K, Andais, L, Brown, L, Dal Cortivo, L, Griffith, L, Notarangelo, L, Abinun, M, Albert, M, Bierings, M, Bouchet, M, Cavazzana, M, Hirschfield, M, Cowan, M, Hoenig, M, Loubser, M, Roncarolo, M, Sauer, M, Schneider, M, Verstegen, M, Schroeder, M, Essink, M, Yesilipek, M, Entz Werle, N, Mahlaoui, N, Schlautmann, N, Taylor, N, Vanroyen, N, Walffraat, N, Sanal, O, Amrolia, P, Bordigoni, P, De Coppi, P, Frange, P, Orchard, P, Sedlacek, P, Shaw, P, Stephensky, P, Bacchetta, R, Bredius, R, Formankova, R, Gale, R, Seger, R, Wynn, R, Corbacioglu, S, Ehl, S, Hacein Bey, S, Hambleton, S, Mohsen, S, Mueller, S, Pai, Sy, Espanol, T, Flood, T, Guengoer, T, Bordon, V, Ormoor, V, Pashano, V, Courteille, V, Czogala, W, Qasim, W, Camci, Y, and Nademi, Z.
Subjects: Male, medicine.medical_specialty, Transplantation Conditioning, medicine.medical_treatment, Immunology, Graft vs Host Disease, Kaplan-Meier Estimate, Cord Blood Stem Cell Transplantation, Hematopoietic stem cell transplantation, Biochemistry, Gastroenterology, SCID HSCT, Internal medicine, medicine, Humans, Proportional Hazards Models, Retrospective Studies, Preparative Regimen, Severe combined immunodeficiency, business.industry, Umbilical Cord Blood Transplantation, Incidence, Hematopoietic Stem Cell Transplantation, Infant, Newborn, Infant, Cell Biology, Hematology, medicine.disease, Surgery, Transplantation, Treatment Outcome, Child, Preschool, Histocompatibility, Cord blood, Female, Severe Combined Immunodeficiency, business
Abstract: Pediatric patients with SCID constitute medical emergencies. In the absence of an HLA-identical hematopoietic stem cell (HSC) donor, mismatched related-donor transplantation (MMRDT) or unrelated-donor umbilical cord blood transplantation (UCBT) are valuable treatment options. To help transplantation centers choose the best treatment option, we retrospectively compared outcomes after 175 MMRDTs and 74 UCBTs in patients with SCID or Omenn syndrome. Median follow-up time was 83 months and 58 months for UCBT and MMRDT, respectively. Most UCB recipients received a myeloablative conditioning regimen; most MMRDT recipients did not. UCB recipients presented a higher frequency of complete donor chimerism (P = .04) and faster total lymphocyte count recovery (P = .04) without any statistically significance with the preparative regimen they received. The MMRDT and UCBT groups did not differ in terms of T-cell engraftment, CD4+ and CD3+ cell recoveries, while Ig replacement therapy was discontinued sooner after UCBT (adjusted P = .02). There was a trend toward a greater incidence of grades II-IV acute GVHD (P = .06) and more chronic GVHD (P = .03) after UCBT. The estimated 5-year overall survival rates were 62% ± 4% after MMRDT and 57% ± 6% after UCBT. For children with SCID and no HLA-identical sibling donor, both UCBT and MMRDT represent available HSC sources for transplantation with quite similar outcomes.
Published: 2012

50. Ultrasound assessment of the elbow

Author: Radunovic, G., Vlad, V., Micu, M. C., Nestorova, R., Petranova, T., Porta, F., and Annamaria Iagnocco
Subjects: ltrasound, anatomy, elbow, epicondylitis, synovitis, ulnar nerve entrapment, ultrasound, Rheumatic Diseases, Elbow Joint, Humans, Joint Diseases, Ultrasonography
Abstract: Ultrasonography of the elbow is a very helpful and reliable diagnostic procedure for a broad spectrum of rheumatic and orthopedic conditions, representing a possible substitute to magnetic resonance imaging for evaluation of soft tissues of the elbow. Musculoskeletal ultrasound (US) shows many advantages over other imaging modalities, probably the most important being its capability to perform a dynamic assessment of musculoskeletal elements with patient's partnership and observation during examination. In addition, ultrasonography is cost effective, easy available, and has excellent and multiplanar capability to visualize superficial soft tissue structures. Among all imaging procedures, US is highly accepted by patients. US assessment of the elbow requires good operator experience in the assessment of normal anatomy, and suitable high-quality equipment. US of the elbow provides detailed information including joint effusions, medial and lateral epicondylitis, tears of the distal biceps and triceps tendons, radial and ulnar collateral ligament tears, ulnar nerve entrapment, cubital or olecranon bursitis and intra-articular loose bodies. The aim of this paper is to review the screening technique and the basic normal and pathological findings in elbow US.
Published: 2012

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