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4. 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
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6. The Histopathological Landscape of Synovitis in Hemophilic Arthropathy

Author

Gualtierotti, Roberta, Suffritti, Chiara, Pescia, Carlo, Giachi, Andrea, Spena, Silvia, Molfino, Federico, Lavorato, Stanislao, Semproni, Eleonora, Truma, Addolorata, Arcudi, Sara, Ciavarella, Alessandro, Maggioni, Marco, Gianelli, Umberto, Siboni, Simona Maria, Acquati Lozej, Jacopo, Solimeno, Luigi Piero, and Peyvandi, Flora

Published
2023
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8. Recommendations on multidisciplinary management of elective surgery in people with haemophilia.

Author

UCL - SSS/IREC/NMSK - Neuro-musculo-skeletal Lab, UCL - (SLuc) Service de médecine physique et de réadaptation motrice, Escobar, Miguel A, Brewer, Andrew, Caviglia, Horacio, Forsyth, Angela, Jimenez-Yuste, Victor, Laudenbach, Lori, Lobet, Sébastien, McLaughlin, Paul, Oyesiku, J O O, Rodriguez-Merchan, E Carlos, Shapiro, Amy, Solimeno, Luigi Piero, UCL - SSS/IREC/NMSK - Neuro-musculo-skeletal Lab, UCL - (SLuc) Service de médecine physique et de réadaptation motrice, Escobar, Miguel A, Brewer, Andrew, Caviglia, Horacio, Forsyth, Angela, Jimenez-Yuste, Victor, Laudenbach, Lori, Lobet, Sébastien, McLaughlin, Paul, Oyesiku, J O O, Rodriguez-Merchan, E Carlos, Shapiro, Amy, and Solimeno, Luigi Piero

Abstract

Planning and undertaking elective surgery in people with haemophilia (PWH) is most effective with the involvement of a specialist and experienced multidisciplinary team (MDT) at a haemophilia treatment centre. However, despite extensive best practice guidelines for surgery in PWH, there may exist a gap between guidelines and practical application. For this consensus review, an expert multidisciplinary panel comprising surgeons, haematologists, nurses, physiotherapists and a dental expert was assembled to develop practical approaches to implement the principles of multidisciplinary management of elective surgery for PWH. Careful preoperative planning is paramount for successful elective surgery, including dental examinations, physical assessment and prehabilitation, laboratory testing and the development of haemostasis and pain management plans. A coordinator may be appointed from the MDT to ensure that critical tasks are performed and milestones met to enable surgery to proceed. At all stages, the patient and their parent/caregiver, where appropriate, should be consulted to ensure that their expectations and functional goals are realistic and can be achieved. The planning phase should ensure that surgery proceeds without incident, but the surgical team should be ready to handle unanticipated events. Similarly, the broader MDT must be made aware of events in surgery that may require postoperative plans to be changed. Postoperative rehabilitation should begin soon after surgery, with attention paid to management of haemostasis and pain. Surgery in patients with inhibitors requires even more careful preparation and should only be undertaken by an MDT experienced in this area, at a specialized haemophilia treatment centre with a comprehensive care model.

Published
2018

9. 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

10. Major and Minor Classifications for Surgery in People With Hemophilia: A Literature Review.

Author

Solimeno, Luigi Piero, Escobar, Miguel A., Krassova, Snejana, and Seremetis, Stephanie

Abstract

Agents that control bleeding and the usage of bypassing agents have made surgery an option to consider in people with hemophilia. However, the lack of consistent definitions for major or minor surgery may lead to inconsistencies in patient management. This literature review has evaluated how surgical procedures in people with hemophilia were categorized as major or minor surgery and assessed the consistency across publications. After screening 926 potentially relevant articles, 547 were excluded and 379 full-text articles were reviewed. Ninety-five articles categorized major or minor surgical procedures; of these, 35 publications categorized three or more major or minor surgical procedures and were included for analysis. Seven (20%) publications provided varying criteria for defining major or minor surgery, five of which defined surgery according to the level of surgical invasiveness. Across all 35 publications, there was considerable variance in the categorization of major and minor surgical procedures and some overlap in surgical nomenclature (eg, type of synovectomy, arthroscopy, and central venous access device insertion/removals). The lack of consistent guidance when referring to major or minor surgery in people with hemophilia needs to be addressed. Clear and consistent definitions, achieved by consensus and promoted by relevant international hemophilia committees, are desirable, to provide guidance on appropriate treatment, to increase the accuracy of trial data and may confound the interpretation of surgical outcomes. [ABSTRACT FROM AUTHOR]

Published
2018
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12. Knee and Ankle Arthroplasty in Hemophilia.

Author

Solimeno, Luigi Piero and Pasta, Gianluigi

Subjects
*TOTAL knee replacement, *TOTAL ankle replacement, *ARTHROPLASTY, *KNEE surgery, *ANKLE surgery, *HEMOPHILIA
Abstract

Today, major surgical procedures can be safely performed in hemophilic patients with chronic arthropathy, using available factor concentrates. In this setting, total knee replacement is considered the "gold standard", while the use of total ankle replacement is still debated. Indeed, the unsatisfactory results obtained with the previous available design of implants did not raise enthusiasm as knee or hip replacement. Recently, the introduction of new implant designs and better reported outcomes have renewed the interest in total ankle replacement in people with hemophilia. In this review, the role of replacement surgery in the treatment of chronic hemophilic arthropathy will be described. [ABSTRACT FROM AUTHOR]

Published
2017
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13. Laying the foundations for gene therapy in Italy for patients with haemophilia A: A Delphi consensus study

Author

Giancarlo Castaman, Christian Carulli, Raimondo De Cristofaro, Marco Follino, Angelo Lupi, Maria Elisa Mancuso, Maria Francesca Mansueto, Angelo Claudio Molinari, Pietro Pasquetti, Cristina Santoro, Rita Carlotta Santoro, Sergio Siragusa, Luigi Piero Solimeno, Armando Tripodi, Ezio Zanon, Giovanni Di Minno, Castaman, Giancarlo, Carulli, Christian, De Cristofaro, Raimondo, Follino, Marco, Lupi, Angelo, Mancuso, Maria Elisa, Mansueto, Maria Francesca, Molinari, Angelo Claudio, Pasquetti, Pietro, Santoro, Cristina, Santoro, Rita Carlotta, Siragusa, Sergio, Solimeno, Luigi Piero, Tripodi, Armando, Zanon, Ezio, and Minno, Giovanni Di

Subjects
Delphi technique, Italy, consensus, genetic therapy, haemophilia A, patient care team, patient selection, Hematology, General Medicine, Genetics (clinical)
Abstract

IntroductionCurrent treatment for haemophilia A involves factor VIII replacement or non-replacement (emicizumab) therapies, neither of which permanently normalise factor VIII levels. Gene therapy using adeno-associated viral (AAV) vectors is an emerging long-term treatment strategy for people with severe haemophilia A (PwSHA) that is likely to be available for clinical use in the near future. AimThis article proposes practical guidelines for the assessment, treatment, and follow-up of potential PwSHA candidates for AAV-based gene therapy. MethodUsing the Delphi method, a working group of Italian stakeholders with expertise in and knowledge of the care of adults with haemophilia A analysed literature for AAV-based gene therapy and drafted a list of statements that were circulated to a panel of Italian peers. During two rounds of voting, panel members voted on their agreement with each statement to reach a consensus. ResultsThe Delphi process yielded 40 statements regarding haemophilia A gene therapy, across five topics: (1) organisational model; (2) multidisciplinary team; (3) patient engagement; (4) laboratory surveillance; and (5) patient follow-up and gene therapy outcomes. The consensus was reached for all 40 statements, with the second round of voting needed for five statements. ConclusionUse of the hub-and-spoke organisational model and multidisciplinary teams are expected to optimise patient selection for gene therapy, as well as the management of dosing and patient follow-up, patient engagement, laboratory surveillance, and patient expectations regarding outcomes. This approach should allow the benefits of AAV-based gene therapy for haemophilia A to be maximised.

Published
2023

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