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1. New data from the italian national register of congenital coagulopathies, 2016 annual survey

Author

Abbonizio F., Hassan H. J., Riccioni R., Santagostino E., Arcieri R., Giampaolo A., Contino L., Accorsi A., Caremani A., Ettorre P. C., Giordano P., Lassandro G., Valdre L., Notarangelo L., Aru A. B., Radossi P., Tagariello G., Cultrera D., Iannaccaro P., Santoro R., Biasoli C., Di Gregorio P., Daniele F., Testa S., Serino M. L., Castaman G., Linari S. S., Morfini M., Molinari A. C., Delios G., Cantori I., Franchini M., Caimi M. T., Mancuso M. E., Peyvandi F., Marietta M., Todisco A., Speciale V., Cerbone A. M., Di Minno G., Schiavulli M., Rocino A., Spiezia M. M., Zanon E., Gagliano F., Mansueto M. F., Siragusa S., Coppola A., Quintavalle G., Rivolta G. F., Tagliaferri A., Ambaglio C., Gamba G., Marchesini E., Oliovecchio E., Dragani A., Arbasi M. C., MacChi S., Vincenzi D., Sottilotta G., Pizzini A. M., Luciani M., De Cristofaro R., Baldacci E., Mazzucconi M. G., Santoro C., Mameli L. A., Coluccia A., Marino P., Borchiellini A., Schinco P. C., Messina M., Pollio B., Ricca I., Agostini P., Cristallo A. F., Barillari G., De Angelis V., Mosanghini M. E., Feola G., Bonetti E., Cesaro S., Gandini G., Giuffrida A., Tosetto A., Abbonizio, F., Hassan, H. J., Riccioni, R., Santagostino, E., Arcieri, R., Giampaolo, A., Contino, L., Accorsi, A., Caremani, A., Ettorre, P. C., Giordano, P., Lassandro, G., Valdre, L., Notarangelo, L., Aru, A. B., Radossi, P., Tagariello, G., Cultrera, D., Iannaccaro, P., Santoro, R., Biasoli, C., Di Gregorio, P., Daniele, F., Testa, S., Serino, M. L., Castaman, G., Linari, S. S., Morfini, M., Molinari, A. C., Delios, G., Cantori, I., Franchini, M., Caimi, M. T., Mancuso, M. E., Peyvandi, F., Marietta, M., Todisco, A., Speciale, V., Cerbone, A. M., Di Minno, G., Schiavulli, M., Rocino, A., Spiezia, M. M., Zanon, E., Gagliano, F., Mansueto, M. F., Siragusa, S., Coppola, A., Quintavalle, G., Rivolta, G. F., Tagliaferri, A., Ambaglio, C., Gamba, G., Marchesini, E., Oliovecchio, E., Dragani, A., Arbasi, M. C., Macchi, S., Vincenzi, D., Sottilotta, G., Pizzini, A. M., Luciani, M., De Cristofaro, R., Baldacci, E., Mazzucconi, M. G., Santoro, C., Mameli, L. A., Coluccia, A., Marino, P., Borchiellini, A., Schinco, P. C., Messina, M., Pollio, B., Ricca, I., Agostini, P., Cristallo, A. F., Barillari, G., De Angelis, V., Mosanghini, M. E., Feola, G., Bonetti, E., Cesaro, S., Gandini, G., Giuffrida, A., and Tosetto, A.

Subjects
Blood coagulation disorder, Haemophilia B, Haemophilia A, Von Willebrand's disease, Register
Abstract

Background - In Italy, the National Register of Congenital Coagulopathies (NRCC) collects epidemiological and therapeutic data from patients affected by haemophilia A (HA), haemophilia B (HB), von Willebrand's disease (vWD) and other rare coagulation disorders. Here we present data from the 2016 annual survey. Materials and methods - Data are provided by the Italian Haemophilia Centres, on a voluntary basis. Information flows from every Centre to a web-based platform of the Italian Association of Haemophilia Centres, shared with the Italian National Institute of Health, in accordance with current privacy laws. Patients are classified by diagnosis, disease severity, age, gender and treatment-related complications. Results - In 2016, the total number of patients with congenital coagulopathies in the NRCC was 10,360: 39.8% of these patients had HA, 31.5% had vWD, 8.5% had HB, and 20.2% had less common factor deficiencies. The overall prevalence of HA and HB was 13.9/100,000 males and 3.0/100,000 males, respectively. The overall prevalence of vWD was 5.4/100,000 inhabitants. During 2016, 126 patients had current alloantibodies to factor VIII (FVIII) or factor IX (FIX) and were under treatment with bypassing agents and/or immune tolerance induction. Overall, 388 patients with a history of alloantibodies were recorded in the NRCC of whom 337 with severe HA and 12 with severe HB. Coagulation factor use, evaluated from treatment plans, was approximately 451,000,000 IU of FVIII for HA patients (7.5 IU/inhabitant), and approximately 53,000,000 IU of FIX for HB patients (0.9 IU/inhabitant). Discussion - The prevalences of HA and HB fall within the ranges reported in more developed countries; the consumption of FVIII and FIX was in line with that of other European countries (France, United Kingdom) and Canada. The NRCC, with its bleeding disorder dataset, is a helpful tool for shaping public health policies, as well as planning clinical and epidemiological research projects.

Published
2020

2. Emergency management in patients with haemophilia A and inhibitors on prophylaxis with emicizumab: AICE practical guidance in collaboration with SIBioC, SIMEU, SIMEUP, SIPMeL and SISET

Author

Castaman, G., Santoro, C., Coppola, A., Mancuso, M. E., Santoro, R. C., Bernardini, S., Pugliese, F. R., Lubrano, R., Golato, M., Tripodi, A., Rocino, A., Santagostino, E., Biasoli, C., Borchiellini, A., Catalano, A., Contino, L., Coluccia, A., Cultrera, D., De Cristofaro, R., Di Minno, G., Fabbri, A., Franchini, M., Gamba, G., Chiara, A., Gresele, P., Giampaolo, A., Hassan, H. J., Luciani, M., Marchesini, E., Marino, R., Mazzucconi, M. G., Molinari, A. C., Morfini, M., Notarangelo, L. D., Peccarisi, L., Peyvandi, F., Pollio, B., Rivolta, G. F., Ruggieri, M. P., Sargentini, V., Schiavoni, M., Sciacovelli, L., Serino, M. L., Siragusa, S., Tagliaferri, A., Testa, S., Tosetto, A., Zampogna, S., Zanon, E., Castaman, Giancarlo, Santoro, Cristina, Coppola, Antonio, Mancuso, Maria E, Santoro, Rita C, Bernardini, Sergio, Pugliese, Francesco R, Lubrano, Riccardo, Golato, Maria, Tripodi, Armando, Rocino, Angiola, Santagostino, Elena, Biasoli, Chiara, Borchiellini, Alessandra, Catalano, Alberto, Contino, Laura, Coluccia, Antonella, Cultrera, Dorina, De Cristofaro, Raimondo, Di Minno, Giovanni, Fabbri, Andrea, Franchini, Massimo, Gamba, Gabriella, Giuffrida, Anna Chiara, Gresele, Paolo, Giampaolo, Adele, Hassan, Hamisa J, Luciani, Matteo, Marchesini, Emanuela, Marino, Renato, Mazzucconi, Maria Gabriella, Molinari, Angelo C, Morfini, Massimo, Notarangelo, Lucia D, Peccarisi, Lucia, Peyvandi, Flora, Pollio, Berardino, Rivolta, Gianna Franca, Ruggieri, Maria Pia, Sargentini, Vittorio, Schiavoni, Mario, Sciacovelli, Laura, Serino, Maria Luisa, Siragusa, Sergio, Tagliaferri, Annarita, Testa, Sophie, Tosetto, Alberto, Zampogna, Stefania, Zanon, Ezio, Castaman, G., Santoro, C., Coppola, A., Mancuso, M. E., Santoro, R. C., Bernardini, S., Pugliese, F. R., Lubrano, R., Golato, M., Tripodi, A., Rocino, A., Santagostino, E., Biasoli, C., Borchiellini, A., Catalano, A., Contino, L., Coluccia, A., Cultrera, D., De Cristofaro, R., Di Minno, G., Fabbri, A., Franchini, M., Gamba, G., Chiara, A., Gresele, P., Giampaolo, A., Hassan, H. J., Luciani, M., Marchesini, E., Marino, R., Mazzucconi, M. G., Molinari, A. C., Morfini, M., Notarangelo, L. D., Peccarisi, L., Peyvandi, F., Pollio, B., Rivolta, G. F., Ruggieri, M. P., Sargentini, V., Schiavoni, M., Sciacovelli, L., Serino, M. L., Siragusa, S., Tagliaferri, A., Testa, S., Tosetto, A., Zampogna, S., and Zanon, E.

Subjects
Factor VIII, FVIII inhibitor, Settore BIO/12, Antibodies, Bispecific, Antibodies, Monoclonal, Humanized, Factor VIII, Hemophilia A, Hemorrhage, Hemostatics, Humans, Italy, Quality of Life, FVIII inhibitors, Hemorrhage, Antibodies, Monoclonal, Humanized, Hemophilia A, Antibodies, Hemostatics, bypassing agents, emergency, emicizumab, haemophilia A, Italy, hemic and lymphatic diseases, Monoclonal, Emergency, Haemophilia A, Antibodies, Bispecific, Quality of Life, Humans, Bispecific, Bypassing agents, Emicizumab, Humanized, Bypassing agent, Haemostasis
Abstract

Emicizumab has been approved in several countries for regular prophylaxis in patients with congenital haemophilia A and FVIII inhibitors because it substantially reduces their bleeding risk and improves quality of life. However, although significantly less frequent, some breakthrough bleeds may still occur while on emicizumab, requiring treatment with bypassing or other haemostatic agents. Thrombotic complications have been reported with the associated use of activated prothrombin complex concentrates. In addition, when surgery/invasive procedures are needed while on emicizumab, their management requires multidisciplinary competences and direct supervision by experts in the use of this agent. Given this, and in order to expand the current knowledge on the use of emicizumab and concomitant haemostatic agents, and reduce the risk of complications in this setting, the Italian Association of Haemophilia Centres (AICE) here provides guidance on the management of breakthrough bleeds and surgery in emergency situations in patients with haemophilia A and inhibitors on emicizumab prophylaxis. This paper has been shared with other National Scientific Societies involved in the field.

Published
2020

3. Inhibitor development and mortality in non-severe hemophilia A

Author

Eckhardt, C. L., Loomans, J. I., van Velzen, A. S., Peters, M., Mauser-Bunschoten, E. P., Schwaab, R., Mazzucconi, M. G., Tagliaferri, A., Siegmund, B., Reitter-Pfoertner, S. E., van der Bom, J. G., Fijnvandraat, K., Kamphuisen, P. W., Peerlinck, K., Oldenburg, J., Santagostino, E., Astermark, J., Eckhardt, C. L, van Velzen, A. S, Streefkerk, N., Loomans, J. L., van Eijkelenburg, A., Jansen, A. J., Kruijt, C. C., van Tienoven, B., van Baar, A. C. G., Corten, I. W., Meijer, K., Nijziel, M. R., Dors, N., Hamulyak, K., Beckers, E., Brons, P. P., Laros-van Gorkom, B. A. P., van Heerde, W. L., Leebeek, F., Kruip, M., Cnossen, M. H., Mauser-Bunschoten, E., Fischer, K., Smiers, F. J., Hermans, C., Klamroth, R., Escuriola-Ettingshausen, C., Königs, C., Petrini, P., Holmström, M., Mäkipernaa, A., Male, C., Pabinger, I., Keenan, R. D., Liesner, R., Khair, K., Yee, T. T., Hart, D. P., Rangarajan, S., Mitchell, M., Thompson, G., Haya, S., Moret, A., Cid, A. R., Jimenez-Yuste, V., Mancuso, M. E., Mazzuconni, M. G., Santoro, C., Morfini, M., Castaman, G., Schinco, P., Rivolta, G. F., Platokouki, H., and McRae, S.

Published
2015
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4. Addressing the impact of SARS-CoV-2 infection in persons with congenital bleeding disorders: The Italian MECCOVID-19 study

Author

Coluccia A., Marchesini E., Giuffrida A. C., Rivolta G. F., Ricca I., Zanon E., Luciani M., De Cristofaro R., Coppola A., Rocino A., Ambaglio C., Borchiellini A., Bonetti E., Caimi T. M., Carulli C., Contino L., Cultrera D., D'Attilio E., Delios G., Feola G., Giordano P., Giuffrida G., Grandone E., Lassandro G., Linari S., Margaglione M., Marino R., Molinari A. C., Napolitano M., Nichele I., Notarangelo L. D., Pasca S., Piscitelli L., Pollio B., Quintavalle G., Radossi P., Santoro C., Santoro R. C., Schiavulli M., Sottilotta G., Speciale V., Tagliaferri A., Valdre L., Coluccia A., Marchesini E., Giuffrida A.C., Rivolta G.F., Ricca I., Zanon E., Luciani M., De Cristofaro R., Coppola A., Rocino A., Ambaglio C., Borchiellini A., Bonetti E., Caimi T.M., Carulli C., Contino L., Cultrera D., D'Attilio E., Delios G., Feola G., Giordano P., Giuffrida G., Grandone E., Lassandro G., Linari S., Margaglione M., Marino R., Molinari A.C., Napolitano M., Nichele I., Notarangelo L.D., Pasca S., Piscitelli L., Pollio B., Quintavalle G., Radossi P., Santoro C., Santoro R.C., Schiavulli M., Sottilotta G., Speciale V., Tagliaferri A., and Valdre L.

Subjects
Adult, Inherited, Male, Pediatrics, medicine.medical_specialty, 2019-20 coronavirus outbreak, Coronavirus disease 2019 (COVID-19), congenital bleeding disorders, congenital bleeding disorder, Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), haemophilia, Hemorrhage, Haemophilia, Letter to the Editors, SARS‐CoV‐2, Young Adult, Blood Coagulation Disorders, Inherited, COVID‐19, COVID-19, epidemiology, observational study, SARS-CoV-2, Aged, Child, Preschool, Disease Management, Female, Humans, Italy, Middle Aged, Epidemiology, medicine, Young adult, Disease management (health), Child, Preschool, Letter to the Editor, Genetics (clinical), business.industry, Hematology, General Medicine, Blood Coagulation Disorders, medicine.disease, Observational study, business
Abstract

congenital bleeding disorders

Published
2021

6. Management of patients with severe haemophilia a without inhibitors on prophylaxis with emicizumab: AICE recommendations with focus on emergency in collaboration with SIBioC, SIMEU, SIMEUP, SIPMeL and SISET

Author

Coppola, A., Castaman, G., Santoro, R. C., Mancuso, M. E., Franchini, M., Marino, R., Rivolta, G. F., Santoro, C., Zanon, E., Sciacovelli, L., Manca, S., Lubrano, R., Golato, M., Tripodi, A., Rocino, A., Bernardini, S., Biasoli, C., Borchiellini, A., Cultrera, D., De Cristofaro, R., Di Minno, G., Gamba, G., Giampaolo, A., Giuffrida, A. C., Giuffrida, C., Gresele, P., Mazzucconi, M. G., Morfini, M., Hassan, H. J., Molinari, A. C., Pollio, B., Sargentini, V., Tagliaferri, A., Tosetto, A., and Zampogna, S.

Subjects
FVIII, Laboratory monitoring, Haemophilia A, haemophilia A, 030204 cardiovascular system & hematology, Antibodies, Monoclonal, Humanized, Hemophilia A, Haemophilia, surgery, 03 medical and health sciences, 0302 clinical medicine, Antibodies, Bispecific, Health care, medicine, bleeding, emergency, emicizumab, Humans, In patient, Genetics (clinical), Emicizumab, Emergency management, business.industry, Hematology, General Medicine, medicine.disease, Severe haemophilia A, Medical emergency, business, 030215 immunology
Abstract

Introduction The factor VIII (FVIII)-mimetic bispecific monoclonal antibody, emicizumab, previously approved for prophylaxis in haemophilia A with inhibitors, has been recently licensed in several countries also in patients with severe haemophilia A (PWSHA) without inhibitors. The introduction of this innovative agent requires the development of specific pathways at Haemophilia Treatment Centres (HTC), particularly regarding laboratory testing and treatment of breakthrough bleeds and invasive procedures/surgeries, even more critical when patients are managed by non-specialist professionals. Limited literature data and clinical experience in PWSHA without inhibitors on emicizumab are currently available. Aim To promote awareness and overcome these challenges, the Italian Association of Haemophilia Centres (AICE) issued a guidance on the management of PWSHA without inhibitors on emicizumab prophylaxis, focused on emergency and shared with other National Scientific Societies in the field. Methods The document, drafted by an AICE expert panel and approved through online consultation, was further revised by a multidisciplinary working group, including members of 5 haemostasis, laboratory and emergency scientific societies. The final version was approved by the Council of each society. Results General recommendations about use of FVIII concentrates for the treatment of bleeding or haemostatic coverage of invasive procedures/surgeries and laboratory monitoring in PWSHA without inhibitors on emicizumab are provided. Specific issues of the management in the emergency room are focused, highlighting the need for direct involvement or formalized supervision by specialist HTC physicians. Conclusions This guidance provides a reference pathway to be implemented in the different healthcare organizations, especially for the challenging emergency management in this setting.

Published
2020

25. Cost-effectiveness analysis of late prophylaxis vs. on-demand treatment for severe haemophilia A in Italy

Author

Coppola, A., D'Ausilio, A., Aiello, A., Amoresano, S., Toumi, M., Mathew, P., Tagliaferri, A., Feola, G., Molinari, A. C., Santoro, C., Rivolta, G. F., Cultrera, D. B., Gagliano, F., Zanon, E., Mancuso, M. E., Valdrè, L., Mameli, L., Banov, L., Di Minno, G., Quintavalle, G., Mazzucconi, M. G., Negri, N., Santagostino, E., Coppola, A., D'Ausilio, A., Aiello, A., Amoresano, S., Toumi, M., Mathew, P., Tagliaferri, A., Feola, G., Molinari, A. C., Santoro, C., Rivolta, G. F., Cultrera, D. B., Gagliano, F., Zanon, E., Mancuso, M. E., Valdrè, L., Mameli, L., Banov, L., Di Minno, G., Quintavalle, G., Mazzucconi, M. G., Negri, N., and Santagostino, E.

Subjects
Pediatrics, medicine.medical_specialty, Cost-Benefit Analysis, Haemophilia A, haemophilia A, cost-effectiveness analysi, 030204 cardiovascular system & hematology, Hemophilia A, 03 medical and health sciences, 0302 clinical medicine, Quality of life, cost-effectiveness analysis, Italy, prophylaxis, Hematology, Genetics (clinical), Health care, medicine, Humans, Cost-Benefit Analysi, health care economics and organizations, Factor VIII, Cost–benefit analysis, business.industry, prophylaxi, General Medicine, Cost-effectiveness analysis, Markov Chain, medicine.disease, Markov Chains, Quality-adjusted life year, Relative risk, Quality of Life, Severe haemophilia A, business, 030215 immunology, Human
Abstract

Introduction Long-term regular administrations of factor VIII (FVIII) concentrate (prophylaxis) initiated at an early age prevents bleeding in patients with severe haemophilia A (HA). The 5-year prospective Italian POTTER study provided evidence of benefits in adolescents and adults of late prophylaxis (LP) vs. on-demand therapy (OD) in reducing bleeding episodes and joint morbidity and improving quality of life; however, costs were increased. Aim The aim of this study was to determine the cost-effectiveness of LP vs. OD with sucrose-formulated recombinant FVIII in adolescents and adults with severe HA in Italy. Methods A Markov model evaluated lifetime cost-effectiveness of LP vs. OD in patients with severe HA in Italy, from both the healthcare and societal perspectives. Clinical input parameters were taken from the POTTER study and published literature. Health utility values were assigned to each health state as measured by the joint disease severity Pettersson score. Costs were expressed in Euro (€) 2014, including drug and other medical costs. Sensitivity analyses were performed considering societal perspective (including productivity lost) and varying relative risk of bleeding episodes between regimens. Clinical outcomes and costs were discounted at 6% according to previous studies. Results Lifetime incremental discounted quality-adjusted life-years (QALYs) were +4.26, whereas incremental discounted costs were +€229,694 from a healthcare perspective, with estimated incremental cost-effectiveness ratios (ICERs) equal to €53,978/QALY. Sensitivity analyses confirmed the base-case results showing lower ICERs with the societal perspective. Conclusion Late prophylaxis vs. on-demand therapy results in a cost-effective approach with ICERs falling below the threshold considered acceptable in Italy.

Published
2017

26. Benefits of prophylaxis versus on-demand treatment in adolescents and adults with severe haemophilia A: The POTTER study

Author

Tagliaferri, Annarita, Feola, Giulio, Molinari, Angelo Claudio, Santoro, Cristina, Rivolta, Gianna Franca, Cultrera, Dorina Bianca, Gagliano, Fabio, Zanon, Ezio, Mancuso, Maria Elisa, Valdrè, Lelia, Mameli, Luciana, Amoresano, Susanna, Mathew, Prasad, Tagliaferri, A., Rivolta, G. F., Di Perna, C., Feola, G., Molinari, A. C., Banov, L., Santoro, C., Mazzucconi, M. G., Cultrera, D. B., Gagliano, F., Zanon, E., Mancuso, M. E., Santagostino, E., Valdrè, L., Rodorigo, G., Mameli, L., Piseddu, G., Coppola, A., Negri, N., Amoresano, S., Mathew, P., COPPOLA, ANTONIO, DI MINNO, GIOVANNI, Tagliaferri, Annarita, Feola, Giulio, Molinari, Angelo Claudio, Santoro, Cristina, Rivolta, Gianna Franca, Cultrera, Dorina Bianca, Gagliano, Fabio, Zanon, Ezio, Mancuso, Maria Elisa, Valdrè, Lelia, Mameli, Luciana, Amoresano, Susanna, Mathew, Prasad, Coppola, Antonio, Tagliaferri, A., Rivolta, G. F., Di Perna, C., Feola, G., Molinari, A. C., Banov, L., Santoro, C., Mazzucconi, M. G., Cultrera, D. B., Gagliano, F., Zanon, E., Mancuso, M. E., Santagostino, E., Valdrè, L., Rodorigo, G., Mameli, L., Piseddu, G., Coppola, A., DI MINNO, Giovanni, Negri, N., Amoresano, S., and Mathew, P.

Subjects
Adult, Male, Pediatrics, medicine.medical_specialty, Haemophilia, Time Factors, Adolescent, Cost-Benefit Analysis, Health-related quality of life, 030204 cardiovascular system & hematology, Hemophilia A, Severity of Illness Index, Drug Administration Schedule, Drug Costs, Hemostatics, 03 medical and health sciences, Young Adult, 0302 clinical medicine, Quality of life, Haemophilic arthropathy, On demand, Hemarthrosis, Clinical endpoint, Medicine, Humans, Prospective Studies, Prophylaxi, Child, Bleeding episodes, Factor VIII, business.industry, Bleeding, Age Factors, Secondary prophylaxis, Hematology, Middle Aged, medicine.disease, On demand treatment, Treatment Outcome, Italy, Quality of Life, Severe haemophilia A, business, 030215 immunology
Abstract

SummaryRigorous evidence is lacking on long-term outcomes of factor VIII (FVIII) prophylaxis initiated in adolescent or adult patients with severe haemophilia A. The prospective, open-label Prophylaxis versus On-demand Therapy Through Economic Report (POTTER) study (Clinical-Trials.gov NCT01159587) compared long-term late secondary prophylaxis (recombinant FVIII-FS 20–30 IU/kg thrice weekly) with on-demand treatment in patients aged 12 to 55 years with severe haemophilia A. The annual number of joint bleeding episodes (primary endpoint), total bleeding episodes, orthopaedic and radiologic (Pettersson) scores, health-related quality of life (HRQoL), pharmacoeconomic impact, and safety were evaluated over a > 5-year period (2004–2010). Fifty-eight patients were enrolled at 11 centres in Italy; 53 (27 prophylaxis, 26 on demand) were evaluated and stratified into 2 age subgroups (12–25 and 26–55 years). Patients receiving prophylaxis experienced a significantly lower number of joint bleeding episodes vs the on–demand group (annualised bleeding rate, 1.97 vs 16.80 and 2.46 vs 16.71 in younger and older patients, respectively; p=0.0043). Results were similar for total bleeding episodes. Prophylaxis was associated with significantly fewer target joints (p< 0.001), better orthopaedic (p=0.0019) and Pettersson (p=0.0177) scores, better HRQoL, and fewer days of everyday activities lost (p< 0.0001) but required significantly higher FVIII product consumption. The POTTER study is the first prospective, controlled trial documenting long-term benefits of late secondary prophylaxis in adolescents and adults with severe haemophilia A. The benefits of reduced bleeding frequency, improved joint status, and HRQoL may offset the higher FVIII consumption and costs.

Published
2015

32. Anthracycline drugs and MDR expression in human leukemia

Author

Pogliani, E, Belotti, D, Rivolta, G, Maffe, P, Corneo, G, Pogliani E. M., Belotti D., Rivolta G. F., Maffe P. F., Corneo G., Pogliani, E, Belotti, D, Rivolta, G, Maffe, P, Corneo, G, Pogliani E. M., Belotti D., Rivolta G. F., Maffe P. F., and Corneo G.

Abstract

We investigated the expression of P-glycoprotein (P-gp) in 50 adults with de novo acute myeloid leukemia (AML) at the initial diagnosis in order to further define the relationship between the presence of P-gp on leukemic cells and the efficacy of two different anthracycline drugs, Daunorubicin (DNR) and Idarubicin (IRR), in terms of remission, induction and survival. We found that 30 (60%) of the 50 patients were negative for P-gp expression (group 1) and 20 patients (40%) were positive (group 2) for P-gp expression by MRK16 MoAb using a cut of 10% positive cells. Among the 50 patients, 35 (70%) obtained complete remission (CR); depending on P-gp expression the CR rate was 80% for group 1 and 45% for group 2 (p<0.005). The median duration of overall survival (OS) was 20 months for patients in group 1, compared to 10 months for patients in group 2 (p<0.005). Regarding the anthracycline used, no difference in CR has been observed in patients of group 1 (75% CTR with DNR versus 90% CR with IDR); on the contrary in group 2 we observed 40% CR with DNR versus 70% CR with IDR (p<0.005). No significant difference has been achieved in group 1 terms of median duration of overall survival between DNR and IDR regimen; on the contrary the median duration of OS in patients of group 2 treated with IDR regimen was significantly longer than DNR regimen (p<0.005). These results confirm the prognostic value of P-gp expression in AML at diagnosis and we suggest that Idarubicin could be a valid anthracycline drug for reversing multidrug resistance.

Published
1996

33. LRP1/ CD91 is up-regulated in monocytes from patients with haemophilia A: a single-centre analysis.

Author

Franchini, M., Urbani, S., Amadei, B., Rivolta, G. F., Perna, C. Di, Riccardi, F., Frattini, F., Crestani, S., Bonfanti, C., Formentini, A., Quintavalla, R., and Tagliaferri, A.

Subjects
HEMOPHILIA, LIPOPROTEINS, GENETIC regulation, VIRAL hepatitis, CROSS-sectional method
Abstract

The low-density lipoprotein receptor-related protein 1 ( LRP1) is an ubiquitously expressed endocytic receptor that, among its several functions, is involved in the catabolism of coagulation factor VIII ( FVIII) and in the regulation of its plasma concentrations. Although LRP1/ CD91 polymorphisms have been associated with increased FVIII levels and a consequent thrombotic risk, no data are available on LRP1/ CD91 expression in patients with inherited FVIII deficiency. With the aim of elucidating this issue, 45 consecutive patients with haemophilia A ( HA) (18 severe, 5 moderate and 22 mild HA) were enrolled in this cross-sectional, single-centre survey. The LRP1/ CD91 mean fluorescence intensity ( MFI) in monocytes from HA patients was significantly higher than that detected in 90 healthy blood donors (105 vs. 67, P < 0.001). This over-expression was independent of hepatitis C virus infection status and varied according to the severity of the haemophilia, being higher in patients with more severe FVIII deficiency. In conclusion, our study documents for the first time that LRP1/ CD91 is over-expressed on monocytes from HA patients, with the intensity of expression varying according to the severity of the FVIII deficiency. Further studies are needed to assess the clinical implications of these findings. [ABSTRACT FROM AUTHOR]

Published
2013
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34. A web-based clinical record ‘xl’Emofilia®’ for outpatients with haemophilia and allied disorders in the Region of Emilia-Romagna: features and pilot use.

Author

PATTACINI, C., RIVOLTA, G. F., DI PERNA, C., RICCARDI, F., and TAGLIAFERRI, A.

Subjects
*BLOOD coagulation disorders, *HEMOPHILIA, *EMERGENCY medical services, *MEDICAL emergencies, *BLOOD diseases
Abstract

The treatment of haemophilia in developed countries is based on home self-infusion of concentrates. Improving communication between haemophilia centres (HC) and patients is very important. The Hub Centre (Parma) designed a new outpatient clinical record, ‘xl’Emofilia®’, as part of a project ‘Web Connections of the Region’s HC’ funded by Emilia-Romagna Health Authority. It is a web-based application suited to the needs of HC, which shares the databases of the region’s HC, integrated with regional and national registries that can be accessed from anywhere. Data are managed with the ‘https’ protocol. Significant innovations are ‘pathways’ that help with the entry of data and ‘problem list’, which is a summary (updated automatically) of the patient’s clinically significant data that can be consulted at a glance. With a ‘web identity’ (a personal USB key for secure web access), patients can record bleeds and home infusions, consult their own data and allow access to their general practitioners or in emergency departments anywhere in the world (also in English language). In December 2006, the HC started to use ‘xl’Emofilia®’ and 673 clinical records are now active. Since April 2007, 50 pilot patients have been trained and are successfully using the system. A questionnaire administered to these patients on their level of satisfaction with the system and its ease of use gave excellent results. Our web-based system facilitates communication between patients and HC, improves the quality of care and enables patients to use these information at any time and from anywhere in the world. [ABSTRACT FROM AUTHOR]

Published
2009
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