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1. Diagnosis and treatment of chronic synovitis in patients with haemophilia: consensus statements from the Italian Association of Haemophilia Centres

Author

Di Minno, Matteo Nicola Dario, Napolitano, Mariasanta, Giuffrida, Anna Chiara, Baldacci, Erminia, Carulli, Christian, Boccalandro, Elena, Bruno, Clarissa, Forneris, Eleonora, Ricca, Irene, Passeri, Walter, Martinelli, Marco, Rivolta, Gianna Franca, Solimeno, Luigi Piero, Martinoli, Carlo, Rocino, Angiola, Pasta, Gianluigi, Aru, Brigida, Balestri, Elena, Biasoli, Chiara, Cantori, Isabella, Coluccia, Antonella, Contino, Laura, Coppola, Antonio, Cultrera, Dorina, Daniele, Filomena, Ettorre, Cosimo Pietro, Giordano, Paola, Iannaccaro, Piergiorgio, Lassandro, Giuseppe, Linari, Silvia, Luciani, Matteo, Malcangi, Giuseppe, Mancuso, Maria Elisa, Marchesini, Emanuela, Marino, Renato, Mazucconi, Maria Gabriella, Molinari, Angelo Claudio, Notarangelo, Lucia Dora, Pasca, Samantha, Quintavalle, Gabriele, Radossi, Paolo, Raso, Simona, Sabatino, Vincenzo, Santagostino, Elena, Santoro, Cristina, Scaturro, Dalila, Zanon, Ezio, Di Minno, Matteo Nicola Dario, Napolitano, Mariasanta, Giuffrida, Anna Chiara, Baldacci, Erminia, Carulli, Christian, Boccalandro, Elena, Bruno, Clarissa, Forneris, Eleonora, Ricca, Irene, Passeri, Walter, Martinelli, Marco, Rivolta, Gianna Franca, Solimeno, Luigi Piero, Martinoli, Carlo, Rocino, Angiola, Pasta, Gianluigi, Aru, Brigida, Balestri, Elena, Biasoli, Chiara, Cantori, Isabella, Coluccia, Antonella, Contino, Laura, Coppola, Antonio, Cultrera, Dorina, Daniele, Filomena, Ettorre, Cosimo Pietro, Giordano, Paola, Iannaccaro, Piergiorgio, Lassandro, Giuseppe, Linari, Silvia, Luciani, Matteo, Malcangi, Giuseppe, Mancuso, Maria Elisa, Marchesini, Emanuela, Marino, Renato, Mazucconi, Maria Gabriella, Molinari, Angelo Claudio, Notarangelo, Lucia Dora, Pasca, Samantha, Quintavalle, Gabriele, Radossi, Paolo, Raso, Simona, Sabatino, Vincenzo, Santagostino, Elena, Santoro, Cristina, Scaturro, Dalila, Zanon, Ezio, and Matteo Nicola Dario Di Minno, Mariasanta Napolitano, Anna Chiara Giuffrida, Erminia Baldacci, Christian Carulli, Elena Boccalandro, Clarissa Bruno, Eleonora Forneris, Irene Ricca, Walter Passeri, Marco Martinelli, Gianna Franca Rivolta, Luigi Piero Solimeno, Carlo Martinoli, Angiola Rocino, Gianluigi Pasta, Dalila Scaturro

Subjects
haemophilia, haemophilic arthropathy, synovitis, Chronic Disease, Consensus, Hemophilia A, Humans, Italy, Synovitis, haemophilia, haemophilic arthropathy, synovitis, Hematology
Abstract

Although synovitis is recognized as a marker of joint disease activity, its periodic assessment is not included in routine clinical surveillance of patients with haemophilia (PwH). In order to evaluate the current knowledge and to identify controversial issues, a preliminary literature search by the Musculoskeletal Committee of the Italian Association of Haemophilia Centres (AICE) has been conducted. Statements have been established and sent to the Italian AICE members to collect their level of agreement or disagreement by a Delphi process. Thirty-seven consensus recommendations have been drafted. We found a general agreement on the indication to consider the presence of synovitis as a marker of joint disease activity in PwH. Accordingly, there was agreement on the indication to search for synovitis both in patients reporting joint pain and in asymptomatic ones, recognizing ultrasound as the most practical imaging technique to perform periodic joint screening. Interestingly, after detection of synovitis, there was agreement on the indication to modify the therapeutic approach, suggesting prophylaxis in patients treated on demand and tailoring treatment in patients already under prophylaxis. Whereas the need of an early consultation with a physiotherapist is recommended for PwH affected by chronic synovitis, the exact timing for an orthopaedic surgeon consultation is currently unknown.

Published
2021

2. HEALTH RELATED QUALITY OF LIFE AND PSYCHOPATHOLOGICAL SYMPTOMS IN PEOPLE WITH HEMOPHILIA, BLOODBORNE CO-INFECTIONS AND COMORBIDITIES: AN ITALIAN MULTICENTER OBSERVATIONAL STUDY

Author

Schiavoni, Mario, primary, PRUNETI, CARLO, additional, GUIDOTTI, SARA, additional, MOSCATELLO, ALESSANDRA, additional, GIORDANO, FRANCESCA, additional, COLUCCIA, ANTONELLA, additional, SANTORO, RITA CARLOTTA, additional, MANSUETO, MARIA FRANCESCA, additional, ZANON, EZIO, additional, MARINO, RENATO, additional, CANTORI, ISABELLA, additional, and DE CRISTOFARO, RAIMONDO, additional

Published
2023
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3. Pain assessment and management in Italian Haemophilia Centres

Author

Di Minno, Matteo N D, Santoro, Cristina, Corcione, Antonio, Di Minno, Giovanni, Martinelli, Marco, Mancuso, Maria E, Acone, Benedetto, Molinari, Angelo C, Passeri, Emilio V, Rocino, Angiola, Santoro, Rita C, Tagliaferri, Annarita, Mattia, Consalvo, Agostini, Paola, Andreoli, Ernesto, Ambaglio, Chiara, Arancio, Fabiola, Aru, Brigida, Baldacci, Erminia, Belfiore, Antonella, Bernasconi, Sayla, Biasoli, Chiara, Borchiellini, Alessandra, Brunetti, Gregorio, Cerbone, Annamaria, Caimi, Teresa, Cantori, Isabella, Coluccia, Antonella, Contino, Laura, Coppetelli, Ugo, Costanzi, Matteo, Cultrera, Dorina, De Cristofaro, Raimondo, Delios, Grazia, Di Gennaro, Leonardo, Di Gregorio, Patrizia, Ettorre, Cosimo Pietro, Fasulo, Rosamaria, Feola, Giulio, Ferretti, Antonietta, Franchini, Massimo, Frizziero, Antonio, Galletti, Lisa, Giuffrida, Gaetano, Giuffrida, Annachiara, Iannaccaro, Piergiorgio, Lassandro, Giuseppe, Linari, Silvia, Lione, Aurora, Lombardo, Rita, Luciani, Matteo, Macchi, Silvia, Maestrini, Giacomo, Malcangi, Giuseppe, Malizia, Raniero, Margaglione, Maurizio, Marietta, Marco, Marino, Renato, Massoud, Michela, Napolitano, Mariasanta, Nichele, Ilaria, Notarangelo, Lucia Dora, Pagano, Cinzia, Pasta, Gianluigi, Pollio, Berardino, Quintavalle, Gabriele, Ranalli, Paola, Raso, Simona, Ricca, Irene, Schiavoni, Mario, Schiavulli, Michele, Sottilotta, Gianluca, Todisco, Angela, Zanon, Ezio, Di Minno, Matteo N D, Santoro, Cristina, Corcione, Antonio, Di Minno, Giovanni, Martinelli, Marco, Mancuso, Maria E, Acone, Benedetto, Molinari, Angelo C, Passeri, Emilio V, Rocino, Angiola, Santoro, Rita C, Tagliaferri, Annarita, Mattia, Consalvo, Agostini, Paola, Andreoli, Ernesto, Ambaglio, Chiara, Arancio, Fabiola, Aru, Brigida, Baldacci, Erminia, Belfiore, Antonella, Bernasconi, Sayla, Biasoli, Chiara, Borchiellini, Alessandra, Brunetti, Gregorio, Cerbone, Annamaria, Caimi, Teresa, Cantori, Isabella, Coluccia, Antonella, Contino, Laura, Coppetelli, Ugo, Costanzi, Matteo, Cultrera, Dorina, De Cristofaro, Raimondo, Delios, Grazia, Di Gennaro, Leonardo, Di Gregorio, Patrizia, Ettorre, Cosimo Pietro, Fasulo, Rosamaria, Feola, Giulio, Ferretti, Antonietta, Franchini, Massimo, Frizziero, Antonio, Galletti, Lisa, Giuffrida, Gaetano, Giuffrida, Annachiara, Iannaccaro, Piergiorgio, Lassandro, Giuseppe, Linari, Silvia, Lione, Aurora, Lombardo, Rita, Luciani, Matteo, Macchi, Silvia, Maestrini, Giacomo, Malcangi, Giuseppe, Malizia, Raniero, Margaglione, Maurizio, Marietta, Marco, Marino, Renato, Massoud, Michela, Napolitano, Mariasanta, Nichele, Ilaria, Notarangelo, Lucia Dora, Pagano, Cinzia, Pasta, Gianluigi, Pollio, Berardino, Quintavalle, Gabriele, Ranalli, Paola, Raso, Simona, Ricca, Irene, Schiavoni, Mario, Schiavulli, Michele, Sottilotta, Gianluca, Todisco, Angela, and Zanon, Ezio

Subjects
Factor IX, Italy, Humans, Pain Management, pain heamophilia, Hemophilia A, blood coagulation disorders, haemophilia, pain perception, pain management, surveys, Haemostasis and Thrombosis, Pain Measurement
Abstract

BACKGROUND: Although the widespread use of factor VIII/IX replacement therapy has significantly reduced the severity of arthropathy in persons with haemophilia (PWH), some develop degenerative joint changes, associated with significant pain. The aim of this survey was to investigate the management and perception of pain among Italian physicians who treat PWH. MATERIALS AND METHODS: Between September and October 2017, a questionnaire was distributed to 35 Italian haemophilia treatment centres (60 physicians). RESULTS: Fifty-three haemophilia specialists completed the survey. We found that there was good agreement (98.1%) on the need to investigate pain at each clinical visit, but there was heterogeneity in the opinions of haemophilia specialists with regards to the availability of validated guidelines (35.8%) and whether pain specialists should be a part of the comprehensive care team in daily clinical practice (58.5%). Haemophilia specialists also agreed pain should be evaluated using a rating scale validated in PWH (88.7%). Pain was mainly managed by the haemophilia specialists themselves, supported by a physiatrist and physiotherapist, while a pain specialist was only involved in 26.4% of cases. The combination of paracetamol with tramadol or codeine was the most common first-line treatment, while cyclo-oxygenase-2 inhibitors, non-steroidal anti-inflammatory drugs, and opioids were less commonly used. DISCUSSION: There are some unmet needs in Italy regarding pain management for PWH and the management of pain in these patients by haemophilia specialists. There is a lack of evidence-based guidelines for these specialists to use, as well as a reluctance to involve pain specialists. The lack of spontaneous reporting of pain by PWH, despite using pain relief, highlights the need for clinicians to actively ask patients about any pain they may be experiencing.

Published
2021

4. Intracranial Haemorrhage in Haemophilia Patients Is Still an Open Issue: The Final Results of the Italian EMO.REC Registry

Author

Zanon, Ezio, primary, Pasca, Samantha, additional, Demartis, Francesco, additional, Tagliaferri, Annarita, additional, Santoro, Cristina, additional, Cantori, Isabella, additional, Molinari, Angelo Claudio, additional, Biasoli, Chiara, additional, Coppola, Antonio, additional, Luciani, Matteo, additional, Sottilotta, Gianluca, additional, Ricca, Irene, additional, Pollio, Berardino, additional, Borchiellini, Alessandra, additional, Tosetto, Alberto, additional, Peyvandi, Flora, additional, Frigo, Anna Chiara, additional, and Simioni, Paolo, additional

Published
2022
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7. Comparison of quality of life, and emotional and functional profiles in older people with and without severe haemophilia

Author

Riva, Silvia, primary, Marchesini, Emanuela, additional, Oliovecchio, Emily, additional, Coppola, Antonio, additional, Santagostino, Elena, additional, Radossi, Paolo, additional, Castaman, Giancarlo, additional, Valdrè, Lelia, additional, Santoro, Cristina, additional, Tagliaferri, Annarita, additional, Ettorre, Cosimo Pietro, additional, Zanon, Ezio, additional, Barillari, Giovanni, additional, Cantori, Isabella, additional, Caimi, Teresa Maria, additional, Sottilotta, Gianluca, additional, Iorio, Alfonso, additional, and Mannucci, Pier Mannuccio, additional

Published
2021
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8. Diagnosis and treatment of chronic synovitis in patients with haemophilia: consensus statements from the Italian Association of Haemophilia Centres.

Author

Di Minno, Matteo Nicola Dario, Napolitano, Mariasanta, Giuffrida, Anna Chiara, Baldacci, Erminia, Carulli, Christian, Boccalandro, Elena, Bruno, Clarissa, Forneris, Eleonora, Ricca, Irene, Passeri, Walter, Martinelli, Marco, Rivolta, Gianna Franca, Solimeno, Luigi Piero, Martinoli, Carlo, Rocino, Angiola, Pasta, Gianluigi, Aru, Brigida, Balestri, Elena, Biasoli, Chiara, and Cantori, Isabella

Subjects
HEALTH facilities, SYNOVITIS, HEMOPHILIA, JOINT diseases, DIAGNOSIS, JOINT pain
Abstract

Summary: Although synovitis is recognized as a marker of joint disease activity, its periodic assessment is not included in routine clinical surveillance of patients with haemophilia (PwH). In order to evaluate the current knowledge and to identify controversial issues, a preliminary literature search by the Musculoskeletal Committee of the Italian Association of Haemophilia Centres (AICE) has been conducted. Statements have been established and sent to the Italian AICE members to collect their level of agreement or disagreement by a Delphi process. Thirty‐seven consensus recommendations have been drafted. We found a general agreement on the indication to consider the presence of synovitis as a marker of joint disease activity in PwH. Accordingly, there was agreement on the indication to search for synovitis both in patients reporting joint pain and in asymptomatic ones, recognizing ultrasound as the most practical imaging technique to perform periodic joint screening. Interestingly, after detection of synovitis, there was agreement on the indication to modify the therapeutic approach, suggesting prophylaxis in patients treated on demand and tailoring treatment in patients already under prophylaxis. Whereas the need of an early consultation with a physiotherapist is recommended for PwH affected by chronic synovitis, the exact timing for an orthopaedic surgeon consultation is currently unknown. [ABSTRACT FROM AUTHOR]

Published
2022
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9. Pain assessment and management in haemophilia: A survey among Italian patients and specialist physicians

Author

Tagliaferri, Annarita, Franchini, Massimo, Rivolta, Gianna F., Farace, Stefania, Quintavalle, Gabriele, Coppola, Antonio, Arbasi, Maria Cristina, Aru, Anna Brigida, Baldacci, Erminia, Bonetti, Elisa, Cantori, Isabella, Cerbone, Anna Maria, Cocco, Antonio, Coluccia, Antonella, Contino, Laura, Cultrera, Dorina, Daniele, Filomena, De Cristofaro, Raimondo, Di Minno, Giovanni, Di Minno, Matteo N. D., Di Perna, Caterina, Ettorre, Cosimo Pietro, Feola, Giulio, Gamba, Gabriella, Giordano, Paola, Giuffrida, Gaetano, Luciani, Matteo, Mancuso, Maria Elisa, Marino, Renato, Molinari, Angelo Claudio, Pasta, Gianluigi, Pedrazzi, Paola, Pizzini, Attilia Maria, Pollio, Berardino, Radossi, Paolo, Raso, Simona, Rodorigo, Giuseppina, Santoro, Rita, Schiavoni, Mario, Schinco, Piercarla, Sottilotta, Gianluca, Speciale, Vincenzo, Todisco, Angelamaria, Turello, Marina, Zanon, Ezio, Tagliaferri, Annarita, Franchini, Massimo, Rivolta, Gianna F., Farace, Stefania, Quintavalle, Gabriele, Coppola, Antonio, Arbasi, Maria Cristina, Aru, Anna Brigida, Baldacci, Erminia, Bonetti, Elisa, Cantori, Isabella, Cerbone, Anna Maria, Cocco, Antonio, Coluccia, Antonella, Contino, Laura, Cultrera, Dorina, Daniele, Filomena, De Cristofaro, Raimondo, Di Minno, Giovanni, Di Minno, Matteo N. D., Di Perna, Caterina, Ettorre, Cosimo Pietro, Feola, Giulio, Gamba, Gabriella, Giordano, Paola, Giuffrida, Gaetano, Luciani, Matteo, Mancuso, Maria Elisa, Marino, Renato, Molinari, Angelo Claudio, Pasta, Gianluigi, Pedrazzi, Paola, Pizzini, Attilia Maria, Pollio, Berardino, Radossi, Paolo, Raso, Simona, Rodorigo, Giuseppina, Santoro, Rita, Schiavoni, Mario, Schinco, Piercarla, Sottilotta, Gianluca, Speciale, Vincenzo, Todisco, Angelamaria, Turello, Marina, and Zanon, Ezio

Subjects
Adult, Male, medicine.medical_specialty, Adolescent, Analgesic, Haemophilia A, haemophilia, 030204 cardiovascular system & hematology, Haemophilia, Hemophilia A, 03 medical and health sciences, Young Adult, 0302 clinical medicine, Quality of life, Pain assessment, Physicians, Surveys and Questionnaires, Medicine, Humans, Pain Management, pain, survey, Joint bleeding, Child, Genetics (clinical), Aged, Pain Measurement, business.industry, Chronic pain, General Medicine, Hematology, Middle Aged, analgesic, medicine.disease, Italy, comprehensive care, Physical therapy, Female, patient, Chronic Pain, business, 030215 immunology, Pain therapy
Abstract

Introduction Persons with haemophilia (PWH) experience recurrent joint bleeding which leads from early synovitis to irreversible joint damage. Pain strongly affects patients' quality of life, as PWH suffer from acute pain associated with haemarthroses and chronic pain due to arthritic and degenerative complications. Aim To investigate pain issues among PWH and their treaters in Italy. Methods Persons with haemophilia and specialist physicians responded to a survey focused on pain characteristics, assessment, and management by phone call and online, respectively. Results One hundred and nineteen patients (76% severe haemophilia, 61% ≥18 years) and 44 physicians were involved. Pain was reported by 61% of PWH; among those who did not experience pain, 70% were children on prophylaxis. Patients described pain as chronic (71%), acute (69%) or postoperative (8%), and rated it as severe in 65% of cases. Clinicians reported lower percentages of patients with pain (46%), classified as chronic (58%), acute (33%) or postoperative (21%), half using specific scales. Pain was systematically investigated by treaters according to 36% of patients. Paracetamol was largely the most prescribed first-line pain therapy (89%), as well the most employed analgesic by PWH (51%), who also used non-steroidal anti-inflammatory drugs (24%), cyclo-oxygenase-2 inhibitors (21%) or opioids (26%). To manage pain, 61% of clinicians stated to collaborate with other specialists. Physiotherapy was often suggested but less frequently used by PWH. Conclusions Pain is under-recognized and unsatisfactorily addressed by haemophilia treatment centre (HTC) clinicians, with discrepant management compared to PWH responses. Education in systematic pain assessment and multidisciplinary treatment and development of management guidelines are highly needed.

Published
2018

11. Acquired inhibitors of clotting factors: AICE recommendations for diagnosis and management

Author

Franchini, Massimo, Castaman, Giancarlo, Coppola, Antonio, Santoro, Cristina, Zanon, Ezio, Di Minno, Giovanni, Morfini, Massimo, Santagostino, Elena, Rocino, Angiola, Abbate, Rosanna, Aru, Anna Brigida, Biasoli, Chiara, Borchiellini, Alessandra, Caimi, Teresa Maria, Cantori, Isabella, Cerbone, Anna Maria, Cesaro, Simone, Ciabatta, Carlo, Coluccia, Antonella, Contino, Laura, Cultrera, Dorina, De Cristofaro, Raimondo, Delios, Grazia, Di Minno, Matteo Nicola Dario, Dragani, Alfredo, Ettorre, Cosimo Pietro, Feola, Giulio, Gamba, Gabriella, Gandini, Giorgio, Giuffrida, Anna Chiara, Giuffrida, Gaetano, Gresele, Paolo, Hassan, Hamisa Jane, Lassandro, Giuseppe, Latella, Caterina, Linari, Silvia, Luciani, Matteo, Mancuso, Maria Elisa, Marchesini, Emanuela, Marino, Renato, Matino, Davide, Mazzucconi, Maria Gabriella, Messina, Maria, Milan, Marta, Molinari, Angelo Claudio, Napolitano, Mariasanta, Notarangelo, Lucia Dora, Peyvandi, Flora, Pollio, Berardino, Radossi, Paolo, Riccardi, Federica, Riva, Silvia, Rivolta, Gianna Franca, Rossetti, Gina, Santoro, Rita Carlotta, Serino, Maria Luisa, Schiavulli, Michele, Schiavoni, Mario, Schinco, Piercarla, Solimeno, Luigi Piero, Sottilotta, Gianluca, Targhetta, Roberto, Tagliaferri, Annarita, Testa, Sophie, Todisco, Angela, Turello, Marina, Valdrè, Lelia, Franchini M., Castaman G., Coppola A., Santoro C., Zanon E., Di Minno G., Morfini M., Santagostino E., Rocino A., Abbate R., Aru A.B., Biasoli C., Borchiellini A., Caimi T.M., Cantori I., Cerbone A.M., Cesaro S., Ciabatta C., Coluccia A., Contino L., Cultrera D., De Cristofaro R., Delios G., Di Minno M.N.D., Dragani A., Ettorre C.P., Feola G., Gamba G., Gandini G., Giuffrida A.C., Giuffrida G., Gresele P., Hassan H.J., Lassandro G., Latella C., Linari S., Luciani M., Mancuso M.E., Marchesini E., Marino R., Matino D., Mazzucconi M.G., Messina M., Milan M., Molinari A.C., Napolitano M., Notarangelo L.D., Peyvandi F., Pollio B., Radossi P., Riccardi F., Riva S., Rivolta G.F., Rossetti G., Santoro R.C., Serino M.L., Schiavulli M., Schiavoni M., Schinco P., Solimeno L.P., Sottilotta G., Targhetta R., Tagliaferri A., Testa S., Todisco A., Turello M., Valdre L., Franchini, Massimo, Castaman, Giancarlo, Coppola, Antonio, Santoro, Cristina, Zanon, Ezio, DI MINNO, Giovanni, Morfini, Massimo, Santagostino, Elena, Rocino, Angiola, Group Author: Abbate, Rosanna, Aru, Anna Brigida, Biasoli, Chiara, Borchiellini, Alessandra, Caimi, Teresa Maria, Cantori, Isabella, Cerbone, Anna Maria, Cesaro, Simone, Ciabatta, Carlo, Coluccia, Antonella, Contino, Laura, Cultrera, Dorina, De Cristofaro, Raimondo, Delios, Grazia, DI MINNO, matteo nicola dario, Dragani, Alfredo, Ettorre, Cosimo Pietro, Feola, Giulio, Gamba, Gabriella, Gandini, Giorgio, Giuffrida, Anna Chiara, Giuffrida, Gaetano, Gresele, Paolo, Hassan, Hamisa Jane, Lassandro, Giuseppe, Latella, Caterina, Linari, Silvia, Luciani, Matteo, Mancuso, Maria Elisa, Marchesini, Emanuela, Marino, Renato, Matino, Davide, Mazzucconi, Maria Gabriella, Messina, Maria, Milan, Marta, Molinari, Angelo Claudio, Napolitano, Mariasanta, Notarangelo, Lucia Dora, Peyvandi, Flora, Pollio, Berardino, Radossi, Paolo, Riccardi, Federica, Riva, Silvia, Rivolta, Gianna Franca, Rossetti, Gina, Santoro, Rita Carlotta, Serino, Maria Luisa, Schiavulli, Michele, Schiavoni, Mario, Schinco, Piercarla, Solimeno, Luigi Piero, Sottilotta, Gianluca, Targhetta, Roberto, Tagliaferri, Annarita, Testa, Sophie, Todisco, Angela, Turello, Marina, and Valdrè, Lelia

Subjects
Male, Autoantibodies, Blood Coagulation Factor Inhibitors, Female, Humans, Hemophilia A, Immunology and Allergy, Hematology, Blood Coagulation Factor Inhibitor, Recommendation, Autoantibodie, Human
Published
2015

12. Rate and appropriateness of polypharmacy in older patients with hemophilia compared with age-matched controls

Author

Mannucci, Pier Mannuccio, primary, Nobili, Alessandro, additional, Marchesini, Emanuela, additional, Oliovecchio, Emily, additional, Cortesi, Laura, additional, Coppola, Antonio, additional, Santagostino, Elena, additional, Radossi, Paolo, additional, Castaman, Giancarlo, additional, Valdrè, Lelia, additional, Santoro, Cristina, additional, Tagliaferri, Annarita, additional, Ettorre, Cosimo, additional, Zanon, Ezio, additional, Barillari, Giovanni, additional, Cantori, Isabella, additional, Caimi, Teresa Maria, additional, Sottilotta, Gianluca, additional, Peyvandi, Flora, additional, and Iorio, Alfonso, additional

Published
2018
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13. Activated prothrombin complex concentrate (FEIBA® ) in acquired haemophilia A: a large multicentre Italian study - the FAIR Registry

Author

Zanon, Ezio, primary, Pasca, Samantha, additional, Santoro, Cristina, additional, Gamba, Gabriella, additional, Siragusa, Sergio M., additional, Rocino, Angiola, additional, Cantori, Isabella, additional, Federici, Augusto B., additional, Mameli, Luciana, additional, Giuffrida, Gaetano, additional, Falanga, Anna, additional, Lodigiani, Corrado, additional, Santoro, Rita C., additional, Milan, Marta, additional, Ambaglio, Chiara, additional, Napolitano, Mariasanta, additional, and Mazzucconi, Maria G., additional

Published
2018
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14. Identification of ten novel mutations in factor VIII gene: A study of a cohort of 52 haemophilia A patients

Author

Santacroce, Rosa, primary, Leccese, Angelica, additional, Trunzo, Roberta, additional, Lassandro, Giuseppe, additional, Giordano, Paola, additional, Ettorre, Cosimo, additional, Antoncecchi, Stefano, additional, Cantori, Isabella, additional, Dragani, Alfredo, additional, Belvini, Donata, additional, Salviato, Roberta, additional, and Margaglione, Maurizio, additional

Published
2015
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15. Activated prothrombin complex concentrate (FEIBA®) in acquired haemophilia A: a large multicentre Italian study – the FAIR Registry.

Author

Zanon, Ezio, Pasca, Samantha, Santoro, Cristina, Gamba, Gabriella, Siragusa, Sergio M., Rocino, Angiola, Cantori, Isabella, Federici, Augusto B., Mameli, Luciana, Giuffrida, Gaetano, Falanga, Anna, Lodigiani, Corrado, Santoro, Rita C., Milan, Marta, Ambaglio, Chiara, Napolitano, Mariasanta, and Mazzucconi, Maria G.

Abstract

The article presents a study which aimed to assess dosage, duration of treatment, as well as the effectiveness and safety of activated prothrombin complex concentrate (aPCC) in patients with Acquired haemophilia A (AHA), a rare bleeding disorder caused by the spontaneous development of auto-antibodies against coagulation factor VIII. Statistical analysis showed results that may be useful in clinical practice, but controlled trials needed to confirm the data obtained.

Published
2019
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18. Selection and transplantation of autologous CD34[sup +] B-lineage negative cells in advanced-phase multiple myeloma patients: a pilot study.

Author

Lemoli, Roberto M., Martinelli, Giovanni, Olivieri, Attilio, Motta, Maria Rosa, Rizzi, Simonetta, Terragna, Carolina, Leopardi, Giuliana, Benni, MonicaA, Ronconi, Sonia, Cantori, Isabella, Rondelli, Damiano, Mangianti, Serena, Leoni, Pietro, Montanari, Mauro, Cavo, Michele, and Tura, Sante

Subjects
MULTIPLE myeloma, CELL transplantation, BLOOD cells, CD antigens
Abstract

The feasibility of sequential positive and negative selection of mobilized CD34+ B-lineage negative cells to achieve tumour-free autografts in multiple myeloma (MM) patients was evaluated. Peripheral blood stem cells (PBSC) of 14 patients with advanced disease were mobilized. CD34+ cells were enriched in 12 of the patients by the avidin–biotin immunoabsorption technique. Subsequently, CD10+, CD19+, CD20+ and CD56+ cells (B-lin cells) were removed by immunomagnetic depletion. Minimal residual disease (MRD) was detected by flow cytometry and PCR-based molecular analysis of the patient specific IgH complementary-determining region III (CDRIII). Positive selection of stem cells produced a median recovery of 54.7% of the initial content of CD34+ cells (median purity 71.9%). Negative depletion of B-lineage cells reduced the number of CD34+ cells to 33.3% of the baseline value (median purity 72.7%). However, long-term culture assays showed the recovery of >60% of primitive haemopoietic progenitor cells after depletion of the B-lineage-positive cells. All evaluable patients had detectable disease in PBSC collections. The first step of positive selection of CD34+ cells resulted in >2 logs of tumour cell purging. However, molecular assessment showed the persistence of the disease in 6/7 cases. Immunofluorescence analysis demonstrated 1 additional log of B-cell purging by negative depletion. More importantly, molecular evaluation of IgH CDRIII region showed the disappearance of myeloma cells in 6/7 patients. 12 patients received a median of 3.9 × 106 CD34+ B-lin- cells/kg after conditioning with high-dose melphalan and showed a rapid reconstitution of haemopoiesis. These results were similar to two similar cohorts of patients who received either unmanipulated PBSC or positively selected CD34+ cells after the same... [ABSTRACT FROM AUTHOR]

Published
1999
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19. Activated prothrombin complex concentrate (FEIBA ® ) in acquired haemophilia A: a large multicentre Italian study - the FAIR Registry.

Author

Zanon E, Pasca S, Santoro C, Gamba G, Siragusa SM, Rocino A, Cantori I, Federici AB, Mameli L, Giuffrida G, Falanga A, Lodigiani C, Santoro RC, Milan M, Ambaglio C, Napolitano M, and Mazzucconi MG

Subjects
Blood Coagulation Factors adverse effects, Female, Humans, Italy, Male, Prospective Studies, Retrospective Studies, Blood Coagulation Factors administration & dosage, Hemophilia A drug therapy, Registries
Published
2019
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20. Perceived challenges and attitudes to regimen and product selection from Italian haemophilia treaters: the 2013 AICE survey.

Author

Franchini M, Coppola A, Rocino A, Zanon E, Morfini M, Accorsi A, Aru AB, Biasoli C, Cantori I, Castaman G, Cesaro S, Ciabatta C, De Cristofaro R, Delios G, Di Minno G, D'Incà M, Dragani A, Ettorre CP, Gagliano F, Gamba G, Gandini G, Giordano P, Giuffrida G, Gresele P, Latella C, Luciani M, Margaglione M, Marietta M, Mazzucconi MG, Messina M, Molinari AC, Notarangelo LD, Oliovecchio E, Peyvandi F, Piseddu G, Rossetti G, Rossi V, Santagostino E, Schiavoni M, Schinco P, Serino ML, Tagliaferri A, and Testa S

Subjects
Blood Coagulation Factors therapeutic use, Health Care Surveys, Hemophilia A drug therapy, Humans, Italy, Surveys and Questionnaires, Hemophilia A epidemiology, Hospitals, Special, Medical Staff, Hospital, Practice Patterns, Physicians' statistics & numerical data
Abstract

Despite great advances in haemophilia care in the last 20 years, a number of questions on haemophilia therapy remain unanswered. These debated issues primarily involve the choice of the product type (plasma-derived vs. recombinant) for patients with different characteristics: specifically, if they were infected by blood-borne virus infections, and if they bear high or low risk of inhibitor development. In addition, the most appropriate treatment regimen in non-inhibitor and inhibitor patients compel physicians operating at the haemophilia treatment centres (HTCs) to take important therapeutic decisions, which are often based on their personal clinical experience rather than on evidence-based recommendations from published literature data. To know the opinion on the most controversial aspects in haemophilia care of Italian expert physicians, who are responsible for common clinical practice and therapeutic decisions, we have conducted a survey among the Directors of HTCs affiliated to the Italian Association of Haemophilia Centres (AICE). A questionnaire, consisting of 19 questions covering the most important topics related to haemophilia treatment, was sent to the Directors of all 52 Italian HTCs. Forty Directors out of 52 (76.9%) responded, accounting for the large majority of HTCs affiliated to the AICE throughout Italy. The results of this survey provide for the first time a picture of the attitudes towards clotting factor concentrate use and product selection of clinicians working at Italian HTCs., (© 2014 John Wiley & Sons Ltd.)

Published
2014
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