178 results on '"MOLINARI AC"'
Search Results
2. The epidemiology of sepsis in paediatric intensive care units in Brazil (the Sepsis PREvalence Assessment Database in Pediatric population, SPREAD PED): an observational study
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de Souza, Daniela Carla, primary, Gonçalves Martin, Joelma, additional, Soares Lanziotti, Vanessa, additional, de Oliveira, Cláudio Flauzino, additional, Tonial, Cristian, additional, de Carvalho, Werther Brunow, additional, Roberto Fioretto, José, additional, Pedro Piva, Jefferson, additional, Juan Troster, Eduardo, additional, Siqueira Bossa, Aline, additional, Gregorini, Flávia, additional, Ferreira, Josiane, additional, Lubarino, Juliana, additional, Biasi Cavalcanti, Alexandre, additional, Ribeiro Machado, Flávia, additional, Silva, SC, additional, Giacomazzi, J, additional, Boldrini, Domingos A., additional, Gilli, FH, additional, Castro, LC de, additional, Albuquerque, ML de, additional, Osorio, VCTG, additional, Elias, MLC, additional, Câmara, AA da, additional, Maia, M, additional, Carvalho, PB de, additional, Augusto, FM, additional, Figueiredo, RCCM de, additional, Ikino, EL, additional, Nogueres, FAS, additional, Almeida, RJ de, additional, Torreão, L, additional, Ramalho, J, additional, Camões, MMS, additional, Oliveira, CS de, additional, Serafim, VIIS, additional, Junior, J Colleti, additional, Almeida, CG de, additional, Costa, MHM da, additional, Valerio, JF, additional, Navajas, ME, additional, Riveiro, PM, additional, Lubiana, A, additional, Brito, ACLC, additional, Moretto, V, additional, Rachid, LMMD, additional, Valle, M, additional, Souza, PP de, additional, Victor, RPL, additional, Castro, NR, additional, Sakomura, T, additional, Molon, M, additional, Ferreira, AR, additional, Fonseca, JG da, additional, Cunali, VCA, additional, Chagas, VCA, additional, Belek, G, additional, Rocha, TS da, additional, Mello, LCFF de, additional, Machado, LM, additional, Moliterno, NV, additional, Duran, C, additional, Sanos, CLEB, additional, Ribeiro, GT, additional, Silva, PSL da, additional, Lipinski, R, additional, Soledade, A, additional, Branco, KC, additional, Teles, ACO, additional, Cruz, GF da, additional, Mendonça, MR de, additional, Malheiros, E, additional, Vicari, J, additional, Izidro, PNT, additional, Campos, NMP, additional, Pontes, TC, additional, Carneiro, CRF, additional, Imamura, AH, additional, Yoshioka, FM, additional, Duarte, MCMB, additional, Menezes, T, additional, Falconiere, C, additional, Matos, F, additional, Zeitel, RS, additional, Nogueras, CC, additional, Silva, ML da, additional, Aprille, M, additional, Pires, TYM, additional, Portella, AF, additional, Flores, PVG, additional, Alfradique, PP, additional, Nascimento, RC, additional, Saldanha, MO, additional, Alves, AT, additional, Almeida, WJ de, additional, Rodrigues, M, additional, Lopes Júnior, E, additional, Komka, MRP, additional, Nascimento, MAR, additional, Lopes, CRC, additional, Bernardi, TMC, additional, Falcão, RV, additional, Fraga, AR, additional, Machado, AA, additional, Godoy, JEF, additional, Barcellos, J, additional, Queiroz, RLS, additional, Almeida, VVS de, additional, Lira, JZG, additional, Alvo, M, additional, Peçanha, TCP, additional, Sousa, AM de, additional, Sanches, CS, additional, Wendhausen, AR, additional, Pinheiro, SR, additional, Orione, MA, additional, Minossi, AM, additional, Bresolin, NL, additional, Martins, CDF, additional, Cury, VF, additional, Quinet, RPB, additional, Netto, AL, additional, Limonge, R, additional, Tamari, SST, additional, Souza, SER de, additional, Veiga, R, additional, Couto, NGCB, additional, Ribeiro, MFP, additional, Aguiar, AK de, additional, Evangelista, M, additional, Krauzer, JR, additional, Muller, H, additional, Genu, DHS, additional, Alvares, PA, additional, Maciel, KL, additional, Valente, FT, additional, Centeville, M, additional, Espinheira, GJ, additional, Silva, A da, additional, Vasconcelos, PL, additional, Hädrich, AZ, additional, Sousa, ALDGC, additional, Gandra, GA, additional, Souto, ACA, additional, Sabatini, L, additional, João, PRD, additional, Machado, ABMP, additional, Fonseca, FR, additional, Resende, APA, additional, Silva, RV da, additional, Silva, TP da, additional, Barros, LLT, additional, Souza, CSV de, additional, Harada, KO, additional, Calçado, D, additional, Pinheiro, LSB, additional, Frota, MCM, additional, Pulcheri, LB, additional, Silva, LM e, additional, Santos, KJ dos, additional, Nuncio, FH de, additional, Gomes Júnior, IO, additional, Gomes, ACC, additional, Klitzke, MA, additional, Souza, FR de, additional, Carvalho, FSC, additional, Lima, FP de, additional, Braun Filho, LR, additional, Oliveira, MAG de, additional, Castilho, T, additional, Pistelli, IP, additional, Paccez, JD, additional, Cendon, C, additional, Sapolnick, R, additional, Fronza, D, additional, Toscan, C, additional, Pinelli, RM, additional, Alencar, JV, additional, Pace, HL di, additional, Ramos, AD, additional, Molinari, AC, additional, Assis, JF, additional, Chavarri, APCR, additional, Pereira, RC, additional, Freitas, CL, additional, Salmen, ICDM, additional, Guerini, RCM, additional, Asakura, J, additional, Pires, ACR, additional, Benvenuti, G, additional, Fernandes, LM, additional, Oliveira, NF, additional, Barros, HJB, additional, Nascimento, LCE, additional, Machado, MB, additional, Santos, AOR dos, additional, Silva, AC da, additional, Oliveira, ACE de, additional, Reinheimer, SKY, additional, Mello, PCS, additional, Araújo, IO, additional, Rodrigues, KLC, additional, Amari, MN, additional, Andersson, M, additional, Petrini, LMCM, additional, Luz, AKSF, additional, Serafim, ESS, additional, Barbosa, RF, additional, Souza, D, additional, Delgado, AF, additional, Guerra, ALP, additional, Afiune, JY, additional, Huber, J, additional, Casonato, S, additional, Silva, DCB da, additional, Araujo, OR de, additional, Fialho, FMD, additional, Soares, LFR, additional, Queiroz Júnior, AA de, additional, Sartorelli, A, additional, Zuccoli, ACP, additional, Castelani, M, additional, Silva, LAA da, additional, Lima, SRA, additional, Araújo, MJSL de, additional, Cruz, ACS da, additional, Sillero, PM, additional, Sgorlon, G, additional, Alves, TRS, additional, Muniz, MMB, additional, Pereira, RHP, additional, Gonçalves, NR, additional, Teixeira, RPV, additional, Vasconcelos, IA, additional, Rossetti, CT, additional, Cesar, RG, additional, Alves, MAJ, additional, Martins, MK, additional, Rocha, JAR, additional, Arruda, L, additional, Grillo, ALY, additional, and Barros, G, additional
- Published
- 2021
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3. High impact and severe outcomes of intracranial hemorrhage (ICH) in an Italian population of adult mild hemophiliac patients: data from the EMO.REC Registry
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Zanon, E, Pasca, S, Demartis, F, Tagliaferri, T, Santoro, C, Cantori, I, Molinari, Ac, Biasoli, C, Coppola, A, Castanan, G, Linari, S, Luciani, M, Sottilotta, G, Ricca, I, Pollio, B, Borchiellini, A, Tosetto, A, and Santagostino, E
- Published
- 2021
4. Population Parmacokinetics of rFXIII (catridecacog) and clinical outcomes of prophylaxis in an Italian population of patients with Factor XIII deficiency
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Zanon, E, Pasca, S, Sottilotta, G, Molinari, Ac, Banov, L, Ferretti, A, Di Gregorio, P, Pollio, B, Siboni, Sm, Palla, R, Peyvandi, F, Pizzuti, M, Notarangelo, Ld, Simioni, P, Cojutti, P, Biasioli, C, and Pea, F
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- 2021
5. Catridecacog in the treatment of an Italian population of patients with FXIII deficiency: from pharmacokinetics to clinical outcomes (The Italian rFXIII study)
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Zanon, E, Pasca, S, Sottilotta, G, Molinari, Ac, Banov, L, Ferretti, A, Di Gregorio, P, Pollio, B, Siboni, Sm, Palla, R, Peyvandi, F, Pizzuti, M, Notarangelo, Ld, Simioni, P, Cojutti, P, Biasioli, C, and Pea, F
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- 2021
6. The successful use of susoctocog-alfa (rpFVIII) in the treatment of nine elderly patients presenting acquired haemophilia A: a multicenter case series
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Zanon, E, Pasca, S, Borchiellini, A, Lodigiani, C, Molinari, Ac, Moscatelli, P, Ambaglio, C, Preti, Ps, and Simioni, P
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- 2020
7. An Italian Survey on the real-world use of rFXIII (catridecacog) in patients with factor XIII deficiency
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Zanon, E, Pasca, S, Sottilotta, G, Molinari, Ac, Banov, L, Ferretti, A, Di Gregorio, P, Pollio, B, Siboni, Sm, Palla, R, Spiezia, L, Pizzuti, M, Notarangelo, Ld, and Simioni, P
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- 2020
8. Real World Experience on use of susoctocog-alfa (rpFVIII) in the treatment of a population of Italian elderly patients with acquired haemophilia A
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Zanon, E, Pasca, S, Borchiellini, A, Lodigiani, C, Molinari, Ac, Moscatelli, P, Ambaglio, C, Preti, Ps, and Simioni, P
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- 2020
9. 16. The real-life use of rFXIII (catridecacog) in patients with severe Factor XIII deficiency: an Italian survey
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Pasca, S, Sottilotta, G, Molinari, Ac, Banov, L, Ferretti, A, Di Gregorio, P, Pollio, B, Siboni, Sm, Palla, R, Spiezia, L, Pizzuti, M, Notarangelo, Ld, and Ezio Zanon, E
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- 2020
10. Intracranial hemorrhage an open issue: a 10-year Italian study on intracranial haemorrhage in haemophilia patients, the final results of the EMO.REC Registry
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Zanon, E, Pasca, S, Demartis, F, Tagliaferri, T, Santoro, C, Cantori, I, Molinari, Ac, Biasoli, C, Coppola, A, Castanan, G, Linari, S, Luciani, M, Sottilotta, G, Ricca, I, Pollio, B, Borchiellini, A, and Santagostino, E
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- 2020
11. Incidence, management and outcomes of intracranial haemorrhage in haemophilia patients: a 10-year Italian study - the final results of the EMO.REC Registry
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Zanon, E, Pasca, S, Demartis, F, Tagliaferri, T, Santoro, C, Cantori, I, Molinari, Ac, Biasoli, C, Coppola, A, Castanan, G, Linari, S, Luciani, M, Sottilotta, G, Ricca, I, Pollio, B, Borchiellini, A, and Santagostino, E
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- 2020
12. EMICIZUMAB PROPHYLAXIS IN HAEMOPHILIA A PATIENTS WITH INHIBITORS: A COST-EFFECTIVENESS AND BUDGET IMPACT ANALYSIS
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Cortesi, PA, Castaman, G, Trifiro, G, Ferrario, M, Improta, G, Mazzaglia, G, Molinari, AC, Mantovani, LG, Cortesi, P, Castaman, G, Trifiro, G, Ferrario, M, Improta, G, Mazzaglia, G, Molinari, A, and Mantovani, L
- Subjects
EHAEMOPHILIA A, COST-EFFECTIVENESS, BUDGET IMPACT - Published
- 2019
13. Two cases of successful Immune Tolerance Induction Rescue (ITI-R) with simoctocog alfa (rhFVIII) in hemophilia A patients and high-titer inhibitors
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Zanon, E, Pasca, S, Marino, R, and Molinari, Ac
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- 2018
14. Management of Chronic Childhood Immune Thrombocytopenic Purpura: AIEOP Consensus Guidelines
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De Mattia D, Del Vecchio GC, Russo G, De Santis A, Ramenghi U, Notarangelo L, Jankovic M, Molinari AC, Zecca M, Nobili B, Giordano P, AIEOP ITP Study Group, Acquaviva A, Amendola G, Baronci C, Binda S, Bisogno G, Bussetti C, Ciliberti A, Citterio M, Del Principe D, Farruggia P, Ladogana S, Magro S, Masera G, Menichelli A, Nardi M, Parodi E, Tucci F, Vimercati C., PESSION, ANDREA, DE MATTIA, D, DEL VECCHIO, Gc, Russo, G, DE SANTIS, A, Ramenghi, U, Notarangelo, L, Jankovic, M, Molinari, Ac, Zecca, M, Nobili, Bruno, Giordano, P, AIEOP ITP STUDY, Group, De Mattia D, Del Vecchio GC, Russo G, De Santis A, Ramenghi U, Notarangelo L, Jankovic M, Molinari AC, Zecca M, Nobili B, Giordano P, and AIEOP-ITP Study Group,Acquaviva A, Amendola G, Baronci C, Binda S, Bisogno G, Bussetti C, Ciliberti A, Citterio M, Del Principe D, Farruggia P, Ladogana S, Magro S, Masera G, Menichelli A, Nardi M, Parodi E, Pession A, Tucci F, Vimercati C.
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Pediatrics ,medicine.medical_specialty ,Platelet count ,Adolescent ,Consensus Development Conferences as Topic ,Prednisolone ,Rho(D) Immune Globulin ,medicine.medical_treatment ,conta piastrinica ,Splenectomy ,Platelet Transfusion ,management of pediatric thrombocytopenia ,idiopathic thrombocytopenic purpura ,platelet count ,trattamento della piastrinopenia pediatrica ,porpora trombocitopenica idiopatica ,Immune system ,immune system diseases ,hemic and lymphatic diseases ,medicine ,Pediatric thrombocytopenia ,Humans ,Platelet ,Idiopathic thrombocytopenic purpura ,Management of pediatric thrombocytopenia ,Thrombocytopenia ,Child ,Purpura, Thrombocytopenic, Idiopathic ,biology ,business.industry ,Immunoglobulins, Intravenous ,Infant ,Hematology ,General Medicine ,medicine.disease ,Thrombocytopenic purpura ,Purpura ,Chronic disease ,Platelet transfusion ,Child, Preschool ,Chronic Disease ,biology.protein ,Antibody ,medicine.symptom ,business ,Idiopathic thrombocytopenic purpura, Management of pediatric thrombocytopenia, Pediatric thrombocytopenia, Platelet count, Thrombocytopenia - Abstract
Background/Objective: The management of chronic childhood idiopathic thrombocytopenic purpura (ITP) is distinct from acute ITP. Similar to the publication on acute ITP guidelines, the AIEOP (Associazione Italiana di Ematologia e Oncologia Pediatrica) considered it appropriate to develop consensus guidelines for chronic childhood ITP to provide useful and shared information for physicians, healthcare professionals, parents and patients. Design/Methods: A preliminary, evidence-based document issued by a select group of AIEOP pediatric hematologists was discussed, modified and approved during a Consensus Conference according to procedures previously validated by the AIEOP Board. Results: The guidelines give prominence to the periodical reevaluation of all the etiological hypotheses of thrombocytopenia in relation to its clinical condition. The majority of chronic ITP children do not require treatment, especially if bleeding is absent or minimal. The treatment decision depends on several factors other than the platelet count, and treatment options are suggested in relation to the therapeutic scenarios. Recommendations are given regarding support for surgery, particular hemorrhagic conditions, daily activities/sports, as well as for vaccines and drugs. Experimental treatments are also discussed.
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- 2010
15. Perceived challenges and attitudes to regimen and product selection from Italian haemophilia treaters: the 2013 AICE survey
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Franchini, M, Coppola, A, Rocino, A, Zanon, E, Morfini, M, Italian Association of Haemophilia Centers AICE Working Group, 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, Maria Gabriella, 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, Testa, S., 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, Giovanni, 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.
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Haemophilia ,Pediatrics ,medicine.medical_specialty ,factor concentrates ,haemophilia treatment ,inhibitor ,prophylaxis ,survey ,viral safety ,Blood Coagulation Factors ,Health Care Surveys ,Hemophilia A ,Humans ,Italy ,Practice Patterns, Physicians' ,Surveys and Questionnaires ,Hospitals, Special ,Medical Staff, Hospital ,Practice Patterns ,NO ,Hospital ,Haemophilia A ,Medical Staff ,Medicine ,Genetics (clinical) ,Clotting factor ,Physicians' ,Special ,Treatment regimen ,business.industry ,Settore MED/09 - MEDICINA INTERNA ,Hematology ,General Medicine ,medicine.disease ,Blood coagulation factors ,Hospitals ,Clinical Practice ,Regimen ,Family medicine ,Product selection ,business - Abstract
Summary 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.
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- 2014
16. Paediatric arterial ischaemic stroke and cerebral sinovenous thrombosis: First report from the italian registry of pediatric thrombosis (R. I. T. I., Registro Italiano Trombosi Infantili)
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Suppiej, A, Gentilomo, C, Saracco, P, Sartori, S, Agostini, M, Bagna, R, Bassi, B, Giordano, P, Grassi, M, Guzzetta, A, Lasagni, D, Luciani, M, Molinari, Ac, Palmieri, A, Putti, Mc, Ramenghi, La, Rota, Ll, Sperlì, D, Laverda, Am, Simioni, P(1), Collaborators: Angriman M, Stroke working group of the Italian Registry of Pediatric Thrombosis., Aru, Ab, Barisone, E, Bartalena, L, Berta, M, Bertoni, E, Cancarini, P, Cavaliere, E, Celle, Me, Cerbone, Am, Cesaroni, E, Dalla Via, L, Dell'Oro, Mg, Di Rosa, G, Ferrari, Gm, Fiori, S, Gaffuri, M, Gallina, Mr, Gimmillaro, A, Grandone, E, Ladogana, S, Laforgia, N, La Piana, R, Maschio, F, Miniero, R, Nosadini, M, Panzeri, D, Petrucci, A, Piersigilli, F, Sala, D, Sangermani, R, Santoro, N, Tufano, A, Ventura, G, Vittorini, R., Suppiej, A., Gentilomo, C., Saracco, P., Sartori, S., Agostini, M., Bagna, R., Bassi, B., Giordano, P., Grassi, M., Guzzetta, A., Lasagni, D., Luciani, M., Molinari, A. C., Palmieri, A., Putti, M. C., Ramenghi, L. A., Rota, L. L., Sperli, D., Laverda, A. M., Simioni, P., Angriman, M., Aru, A. B., Barisone, E., Bartalena, L., Berta, M., Bertoni, E., Cancarini, P., Cavaliere, E., Celle, M. E., Cerbone, A. M., Cesaroni, E., Via, L. D., Dell'Oro, M. G., Di Rosa, G., Ferrari, G. M., Fiori, S., Gaffuri, M., Gallina, M. R., Gimmillaro, A., Grandone, E., Ladogana, S., Laforgia, N., La Piana, R., Maschio, F., Miniero, R., Nosadini, M., Panzeri, D., Petrucci, A., Piersigilli, F., Sala, D., Sangermani, R., Santoro, N., Tufano, A., Ventura, G., and Vittorini, R.
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Male ,Pediatrics ,AIS ,Children ,CSVT ,Italy ,Registry ,Thrombosis ,Adolescent ,Age of Onset ,Brain Ischemia ,Cerebral Arterial Diseases ,Child ,Child, Preschool ,Delayed Diagnosis ,Female ,Humans ,Infant ,Predictive Value of Tests ,Recurrence ,Registries ,Risk Factors ,Sinus Thrombosis, Intracranial ,Stroke ,Time Factors ,Treatment Outcome ,Hematology ,030204 cardiovascular system & hematology ,Sinus Thrombosis ,Lethargy ,0302 clinical medicine ,Medicine ,Predictive value of tests ,medicine.symptom ,medicine.medical_specialty ,NO ,03 medical and health sciences ,Preschool ,business.industry ,medicine.disease ,Intracranial ,Clinical trial ,Hemiparesis ,Etiology ,Age of onset ,business ,030217 neurology & neurosurgery - Abstract
SummaryData from large case series of children with cerebral thrombotic events are pivotal to improve prevention, early recognition and treatment of these conditions. The Italian Registry of Pediatric Thrombosis (R. I. T. I.) was established in 2007 by a multidisciplinary team, aiming for a better understanding of neonatal and paediatric thrombotic events in Italy and providing a preliminary source of data for the future development of specific clinical trials and diagnostic-therapeutic protocols. We analysed data relative to the paediatric cerebral thrombotic events of the R. I. T. I. which occurred between January 2007 and June 2012. In the study period, 79 arterial ischaemic stroke (AIS) events (49 in males) and 91 cerebral sinovenous thrombosis (CSVT) events (65 in males) were enrolled in the R. I. T. I. Mean age at onset was 4.5 years in AIS, and 7.1 years in CSVT. Most common modes of presentation were hemiparesis, seizures and speech disturbances in AIS, and headache, seizures and lethargy in CSVT. Most common etiologies were underlying chronic diseases, vasculopathy and cardiopathy in AIS, and underlying chronic diseases and infection in CSVT. Time to diagnosis exceeded 24 hours in 46 % AIS and 59 % CSVT. Overall data from the Italian Registry are in substantial agreement with those from the literature, despite small differences. Among these, a longer time to diagnosis compared to other registries and case series poses the accent to the need of an earlier recognition of paediatric cerebrovascular events in Italy, in order to enable prompt and effective treatment strategies.
- Published
- 2015
17. Consumption of clotting factors in severe haemophilia patients undergoing prophylaxis and on-demand treatment in Italy
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Giampaolo A, Abbonizio F, Puopolo M, Arcieri R, Mannucci PM, Hassan HJ, Accorsi A, Ettorre PC, Schiavoni M, Palareti G, Rodorigo G, Valdré L, Amaddii G, Billio A, Notarangelo L, Iannacaro P, Muleo P, Biasioli C, Rossi V, Alatri A, Testa S, Vincenzi D, Scapoli G, Morfini M, Molinari AC, Lapecorella M, Mariani G, Baudo F, Caimi MT, Federici AB, Gringeri A, Santagostino E, Marietta M, Coppola A, Perricone C, Schiavulli M, Miraglia E, Rocino A, Zanon E, Gagliano F, Mancuso G, Siragusa S, Rivolta F, Tagliaferri A, Gamba G, Iorio A, Oliovecchio E, Dragani A, Arbasi MC, Albertini P, Mancino A, Lombardo VT, Latella C, D'Incà M, Landolfi Raffaele, Biondo Francesca, Mazzucconi MG, Santoro Cristina, Mameli AL, Piseddu G, Schinco PC, Messina M, Rossetti G, Barillari G, Feola G, Franchini M, Gandini G, Castaman G, Rodeghiero F, Italian Association of Haemophilia Centres, DI MINNO, GIOVANNI, Giampaolo, A, Abbonizio, F, Puopolo, M, Arcieri, R, Mannucci, Pm, Hassan, Hj, Accorsi, A, Ettorre, Pc, Schiavoni, M, Palareti, G, Rodorigo, G, Valdré, L, Amaddii, G, Billio, A, Notarangelo, L, Iannacaro, P, Muleo, P, Biasioli, C, Rossi, V, Alatri, A, Testa, S, Vincenzi, D, Scapoli, G, Morfini, M, Molinari, Ac, Lapecorella, M, Mariani, G, Baudo, F, Caimi, Mt, Federici, Ab, Gringeri, A, Santagostino, E, Marietta, M, Coppola, A, DI MINNO, Giovanni, Perricone, C, Schiavulli, M, Miraglia, E, Rocino, A, Zanon, E, Gagliano, F, Mancuso, G, Siragusa, S, Rivolta, F, Tagliaferri, A, Gamba, G, Iorio, A, Oliovecchio, E, Dragani, A, Arbasi, Mc, Albertini, P, Mancino, A, Lombardo, Vt, Latella, C, D'Incà, M, Landolfi, Raffaele, Biondo, Francesca, Mazzucconi, Mg, Santoro, Cristina, Mameli, Al, Piseddu, G, Schinco, Pc, Messina, M, Rossetti, G, Barillari, G, Feola, G, Franchini, M, Gandini, G, Castaman, G, Rodeghiero, F, and Italian Association of Haemophilia, Centres
- Subjects
Clotting factor ,Male ,Pediatrics ,medicine.medical_specialty ,Factor VIII ,business.industry ,Haemophilia A ,Hematology ,Disease ,medicine.disease ,Haemophilia ,Hemophilia A ,Hemophilia B ,Factor IX ,On demand treatment ,Coagulation ,Italy ,Surveys and Questionnaires ,medicine ,Humans ,Female ,National registry ,business ,Coagulation Disorder - Abstract
Dear Sir, Treatment of haemophilia is costly, and it must be carefully monitored by physicians and health authorities, so as to improve the planning of the supply of factor concentrates obtained from the fractionation of human plasma or by recombinant technology. Therapy with coagulation factors can be used either prophylactically to prevent bleeding, which entails multiple weekly infusions, or on-demand, through infusion of the amount of factors determined by the severity of the disease and patient’s weight, to stop ongoing bleeding. According to a recent study, factor VIII (FVIII) consumption among persons with haemophilia A (HA) has increased in countries with high-income economies (Stonebraker et al., 2010). In Italy, haemophiliacs and other persons with congenital coagulation disorders are monitored by the National Registry of Congenital Coagulopathies (NRCC) established at the Istituto Superi=
- Published
- 2011
18. Replacement therapy with recombinant factor IX. A multicentre evaluation of current dosing practices in Italy
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Rocca A, Pizzinelli S, Oliovecchio E, Santagostino E, Rocino A, Iorio A, Contino L, Accorsi A, Ettorre C, Scaraggi FA, Rodorigo G, Valdrè L, Targhetta R, Tagariello G, Radossi P, Muleo G, Biasioli C, Morfini M, Molinari AC, Mariani G, Carloni MT, Cantori I, Mannucci PM, Coppola A, Schiavulli M, Zanon E, Tagliaferri A, Rivolta F, Marchesini E, Marcucci M, Dragani A, D'Inca M, Luciani M, Mazzucconi MG, Santoro C, Schinco P, Rossetti G, Barillari G, Gandini G, Castaman G., DI MINNO, GIOVANNI, Rocca, A, Pizzinelli, S, Oliovecchio, E, Santagostino, E, Rocino, A, Iorio, A, Contino, L, Accorsi, A, Ettorre, C, Scaraggi, Fa, Rodorigo, G, Valdrè, L, Targhetta, R, Tagariello, G, Radossi, P, Muleo, G, Biasioli, C, Morfini, M, Molinari, Ac, Mariani, G, Carloni, Mt, Cantori, I, Mannucci, Pm, DI MINNO, Giovanni, Coppola, A, Schiavulli, M, Zanon, E, Tagliaferri, A, Rivolta, F, Marchesini, E, Marcucci, M, Dragani, A, D'Inca, M, Luciani, M, Mazzucconi, Mg, Santoro, C, Schinco, P, Rossetti, G, Barillari, G, Gandini, G, and Castaman, G.
- Abstract
Background: The in vivo recovery of recombinant factor IX (rFIX) is reported to be lower than that of plasma-derived products, with potential clinical implications for dosing. In clinical practice, a conversion (augmentation) factor is suggested to calculate the necessary doses of rFIX. The aim of this study was to assess the range of values for the conversion factor in usual clinical practice in Italy. Materials and methods: The study was questionnaire-based and proposed to all Italian Haemophilia centres treating patients with haemophilia B. Age, weight, dosage used in the last effective infusion, treatment regimen (prophylaxis versus on-demand), human immunodeficiency virus (HIV) and hepatitis C virus (HCV) status, and years of previous therapy with rFIX were recorded for patients with severe haemophilia B treated with rFIX. Mean, standard deviation, median and range were calculated for demographic and treatment data for the overall population and for subgroups. The conversion factor for the theoretical dosage of 40 IU/Kg was calculated. Results: Among 207 patients with severe haemophilia B being followed in 24 centres, 138 (66.7%) were being treated with rFIX. The sample of 207 patients represents 83.1% of the population of Italian patients with severe haemophilia B. The age range of the studied patients was 0-72 years (mean, 24 years) and the weight range was 3-108 kg (mean, 60 kg). Nineteen patients (14.4%) were positive for HIV and 51 (42.9%) were positive for HCV. The mean dosage of rFIX was 44 IU/Kg, with no significant difference between those receiving the product as prophylaxis or on-demand. A reduction in dosage was observed with increasing age (0.23 IU/kg/year). The mean value for the conversion factor was 1.10 ± 0.36 (median 1.00, range 0.51- 2.08), when estimated for the whole population. No effect of HIV and HCV status was found on the dose prescribed. No evident correlation was found with the underlying genetic mutation. Discussion: We found that dosing of rFIX in clinical practice is very close to that of plasmaderived FIX concentrates. As a consequence, dosing in the non-surgical setting should be started using the same criteria as those for plasma-derived FIX and treatment effectiveness verified on a clinical basis rather than relying on in vivo recovery assessments.
- Published
- 2011
19. Evidence-based recommendations on the treatment of von Willebrand disease in Italy
- Author
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Mannucci, Pm, Franchini, M, Castaman, G, Federici, Ab, Contino, L, Accorsi, A, Ciavarella, N, Schiavoni, M, Scaraggi, Fa, Rodorigo, G, Valdre, L, Targhetta, R, Tagariello, G, Radossi, P, Musso, R, Cultrera, D, Muleo, G, Iannacaro, P, Biasioli, C, Testa, S, Alatri, A, Vincenzi, D, Scapoli, G, Morfini, M, Molinari, Ac, Boeri, E, Caprino, D, Delios, G, Girotto, M, Mariani, G, Lapecorella, M, Carloni, Mt, Cantori, I, Santagostino, E, Gringeri, A, Marietta, M, Pedrazzoli, P, Di Minno, G, Coppola, A, Perricone, C, Schiavulli, M, Rocino, A, Berrettini, M, Zanon, E, Mancuso, G, Siragusa, S, Malato, A, Saccullo, G, Tagliaferri, A, Rivolta, F, Iorio, A, Oliovecchio, E, Ferrante, F, Dragani, A, Rossi, A, Mancino, A, Albertini, P, Macchi, S, D'Incà, M, De Rossi, G, Luciani, M, Landolfi, R, Mazzucconi, Maria Gabriella, Santoro, Cristina, Piseddu, G, Schinco, Pc, Rossetti, G, Barillari, G, Feola, G, Gandini, G., Mannucci, Pm, Franchini, M, Castaman, G, Federici, Ab, Contino, L, Accorsi, A, Ciavarella, N, Schiavoni, M, Scaraggi, Fa, Rodorigo, G, Valdre, L, Targhetta, R, Tagariello, G, Radossi, P, Musso, R, Cultrera, D, Muleo, G, Iannacaro, P, Biasioli, C, Testa, S, Alatri, A, Vincenzi, D, Scapoli, G, Morfini, M, Molinari, Ac, Boeri, E, Caprino, D, Delios, G, Girotto, M, Mariani, G, Lapecorella, M, Carloni, Mt, Cantori, I, Santagostino, E, Gringeri, A, Marietta, M, Pedrazzoli, P, DI MINNO, Giovanni, Coppola, A, Perricone, C, Schiavulli, M, Rocino, A, Berrettini, M, Zanon, E, Mancuso, G, Siragusa, S, Malato, A, Saccullo, G, Tagliaferri, A, Rivolta, F, Iorio, A, Oliovecchio, E, Ferrante, F, Dragani, A, Rossi, A, Mancino, A, Albertini, P, Macchi, S, D'Incà, M, De Rossi, G, Luciani, M, Landolfi, R, Mazzucconi, Mg, Santoro, C, Piseddu, G, Schinco, Pc, Rossetti, G, Barillari, G, Feola, G, and Gandini, G.
- Subjects
Male ,desmopressin ,Evidence-Based Medicine ,Factor VIII ,Hemostatics ,von willebrand disease ,von Willebrand Diseases ,von willebrand factor ,Treatment Outcome ,Italy ,von Willebrand Factor ,Humans ,Deamino Arginine Vasopressin ,Drug Therapy, Combination ,Female ,Original Article - Abstract
von Willebrand disease (VWD) is the most common hereditary bleeding disorder affecting both males and females. It arises from quantitative or qualitative defects of von Willebrand factor (VWF) and causes bleeding of mucous membranes and soft tissues. The aim of treatment is to correct the dual defect of haemostasis caused by the abnormal/reduced VWF and the concomitant deficiency of factor VIII (FVIII).This document contains evidence-based recommendations for the management of VWD compiled by AICE (the Italian Association of Haemophilia Centres). All the evidence supporting these recommendations are based on non-randomised comparative studies or case series, because randomised controlled clinical trials or meta-analyses are not available for this disease.Desmopressin (DDAVP) is the treatment of choice for patients with type 1 VWD with FVIII and VWF levels of 10 U/dL or more, while VWF/FVIII concentrates are indicated for those who are unresponsive or insufficiently responsive to DDAVP (severe type 1, type 2 and 3 VWD). VWF concentrates devoid of FVIII, not yet licensed in Italy, may be considered for short-term prophylaxis in elective surgery or for long-term secondary prophylaxis.
- Published
- 2009
20. Italian Registry of Haemophilia and Allied Disorders. Objectives, methodology and data analysis
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IORIO A, OLIOVECCHIO E, MORFINI M, MANNUCCI PM, CONTINO L, ACCORSI A, CIAVARELLA N, SCHIAVONI M, SCARAGGI FA, RODORIGO G, VALDRÉ L, TARGHETTA R, TAGARIELLO G, RADOSSI P, MUSSO R, CULTRERA D, MULEO G, IANNACARO P, BIASIOLI C, TESTA S, ALATRI A, VINCENZI D, SCAPOLI G, MOLINARI AC, BOERI E, CAPRINO D, MARIANI G, LAPECORELLA M, CARLONI MT, CANTORI I, SANTAGOSTINO E, GRINGERI A, FEDERICI AB, MARIETTA M, PEDRAZZOLI P, DI MINNO G, PERRICONE C, SCHIAVULLI M, ROCINO A, BERRETTINI M, ZANON E, MANCUSO G, TAGLIAFERRI A, RIVOLTA F, FERRANTE F, DRAGANI A, ROSSI A, ALBERTINI P, MACCHI S, D'INCA M, DE ROSSI G, LUCIANI M, LANDOLFI R, MAZZUCCONI MG, SANTORO C, PISEDDU G, CARLA SCHINCO P, ROSSETTI G, BARILLARI G, FEOLA G, GANDINI G, FRANCHINI M, CASTAMAN G., COPPOLA, Antonino, SIRAGUSA, Sergio, MALATO, Alessandra, SACCULLO, Giorgia, MANCINO, Antonio, IORIO A, OLIOVECCHIO E, MORFINI M, MANNUCCI PM, CONTINO L, ACCORSI A, CIAVARELLA N, SCHIAVONI M, SCARAGGI FA, RODORIGO G, VALDRÉ L, TARGHETTA R, TAGARIELLO G, RADOSSI P, MUSSO R, CULTRERA D, MULEO G, IANNACARO P, BIASIOLI C, TESTA S, ALATRI A, VINCENZI D, SCAPOLI G, MOLINARI AC, BOERI E, CAPRINO D, MARIANI G, LAPECORELLA M, CARLONI MT, CANTORI I, SANTAGOSTINO E, GRINGERI A, FEDERICI AB, MARIETTA M, PEDRAZZOLI P, DI MINNO G, COPPOLA A, PERRICONE C, SCHIAVULLI M, ROCINO A, BERRETTINI M, ZANON E, MANCUSO G, SIRAGUSA S, MALATO A, SACCULLO G, TAGLIAFERRI A, RIVOLTA F, FERRANTE F, DRAGANI A, ROSSI A, MANCINO A, ALBERTINI P, MACCHI S, D'INCA M, DE ROSSI G, LUCIANI M, LANDOLFI R, MAZZUCCONI MG, SANTORO C, PISEDDU G, CARLA SCHINCO P, ROSSETTI G, BARILLARI G, FEOLA G, GANDINI G, FRANCHINI M, and CASTAMAN G
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methodology, haemophilia ,Settore MED/15 - Malattie Del Sangue - Published
- 2008
21. Italian Registry of Haemophilia and Allied Disorders. Objectives, methodology and data analysis
- Author
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Iorio, A, Oliovecchio, E, Morfini, M, Mannucci, Pm, Contino, L, Accorsi, A, Ciavarella, N, Schiavoni, M, Scaraggi, Fa, Rodorigo, G, Valdré, L, Targhetta, R, Tagariello, G, Radossi, P, Musso, R, Cultrera, D, Muleo, G, Iannacaro, P, Biasioli, C, Testa, S, Alatri, A, Vincenzi, D, Scapoli, G, Molinari, Ac, Boeri, E, Caprino, D, Mariani, G, Lapecorella, M, Carloni, Mt, Cantori, I, Santagostino, E, Gringeri, A, Federici, Ab, Marietta, M, Pedrazzoli, P, Di Minno, G, Coppola, A, Perricone, C, Schiavulli, M, Rocino, A, Berrettini, M, Zanon, E, Mancuso, G, Siragusa, S, Malato, A, Saccullo, G, Tagliaferri, A, Rivolta, F, Ferrante, F, Dragani, A, Rossi, A, Mancino, A, Albertini, P, Macchi, S, Hassan, J, D'Inca, M, De Rossi, G, Luciani, M, Landolfi, R, Mazzucconi, Maria Gabriella, Santoro, Cristina, Piseddu, G, Carla Schinco, P, Rossetti, G, Barillari, G, Feola, G, Gandini, G, Franchini, M, Castaman, G., Iorio, A, Oliovecchio, E, Morfini, M, Mannucci, Pm, Contino, L, Accorsi, A, Ciavarella, N, Schiavoni, M, Scaraggi, Fa, Rodorigo, G, Valdré, L, Targhetta, R, Tagariello, G, Radossi, P, Musso, R, Cultrera, D, Muleo, G, Iannacaro, P, Biasioli, C, Testa, S, Alatri, A, Vincenzi, D, Scapoli, G, Molinari, Ac, Boeri, E, Caprino, D, Mariani, G, Lapecorella, M, Carloni, Mt, Cantori, I, Santagostino, E, Gringeri, A, Federici, Ab, Marietta, M, Pedrazzoli, P, DI MINNO, Giovanni, Coppola, A, Perricone, C, Schiavulli, M, Rocino, A, Berrettini, M, Zanon, E, Mancuso, G, Siragusa, S, Malato, A, Saccullo, G, Tagliaferri, A, Rivolta, F, Ferrante, F, Dragani, A, Rossi, A, Mancino, A, Albertini, P, Macchi, S, Hassan, J, D'Inca, M, De Rossi, G, Luciani, M, Landolfi, R, Mazzucconi, Mg, Santoro, C, Piseddu, G, Carla Schinco, P, Rossetti, G, Barillari, G, Feola, G, Gandini, G, Franchini, M, and Castaman, G.
- Subjects
Adult ,Male ,Pediatrics ,medicine.medical_specialty ,Adolescent ,Haemophilia A ,MEDLINE ,Haemophilia ,Severity of Illness Index ,haemophilia ,registry ,von willebrand's disease ,von willebrand’s disease ,Blood Coagulation Disorders, Inherited ,Von willebrand ,Severity of illness ,Health care ,Computer software ,medicine ,Humans ,Haemophilia B ,Registries ,Age of Onset ,Child ,Genetics (clinical) ,Societies, Medical ,Aged ,business.industry ,Infant ,Hematology ,General Medicine ,Middle Aged ,medicine.disease ,Databases as Topic ,Italy ,Child, Preschool ,Data Interpretation, Statistical ,Female ,Medical emergency ,business - Abstract
National haemophilia registries are powerful instruments to support health care and research. A national registry was established in Italy by the Ministry of Health until 1999. Since 2003 the Italian Association of Haemophilia Centres (AICE) started a new programme aiming at building up the Italian Registry of Haemophilia and Allied Disorders. The AICE identified an expert panel to steer the registry. A computer software to assist patient management was developed and all the AICE-affiliated haemophilia treatment centres (HTC) were prompted to adopt it. Twice a year a predefined set of anonymized data is centralized and merged into a national database. Duplicated entries are managed through a confidentiality sparing mechanism. The database covers sociodemographic, clinical, laboratory and treatment data. A subset of data are shared with the Ministry of Health (Istituto Superiore di Sanita,ISS).Overall, data were collected six times by 43 of 49 HTC; 41 centres updated their patients' records up to December 2006. The database contains 6632 unique records, 442 of them referring to dead patients. Database growth and missing data clearance showed a constantly positive trend over time. The database has collected records of the following alive patients - haemophilia A: 1364 severe, 398 moderate and 935 mild; haemophilia B: 231 severe, 138 moderate and 204 mild; von Willebrand's disease: 1208 type 1, 346 type 2 and 96 type 3. Inhibitor patients were 296 (of which 194 high responders and 65 low responders).The Italian registry run by AICE adds to the list of the available national haemophilia registries and is intended to establish treatment guidelines and foster research projects in Italy.
- Published
- 2008
22. Cerebral te in Italian infants and children: data from the Italian registry of childhood thrombosis (RITI)
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Gentilomo, C, Saracco, P, Bagna, R, Bassi, B, Agostini, M, Giordano, P, Ramenghi, La, Molinari, Ac, Laverda, Am, Grassi, M, Putti, Mc, Suppiej, A, Sartori, S, and Simioni, P
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NO - Published
- 2013
23. Impact on healthcare costs and quality of life of secondary prophylaxis in adolescent and adult patients with severe haemophilia A: the POTTER study
- Author
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Tagliaferri, A, Coppola, A, Feola, G, Molinari, Ac, Santoro, Cristina, Gagliano, F, Cultrera, D, Mancuso, Me, Zanon, E, Valdre, L, Piseddu, G, and Amoresano, S.
- Published
- 2013
24. Factor VIII products and inhibitor development in severe hemophilia A
- Author
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Gouw SC, van der Bom JG, Ljung R, Escuriola C, Cid AR, Claeyssens-Donadel S, van Geet C, Kenet G, Mäkipernaa A, Molinari AC, Muntean W, Kobelt R, Rivard G, Santagostino E, Thomas A, and van den Berg HM
- Subjects
hemic and lymphatic diseases - Abstract
For previously untreated children with severe hemophilia A, it is unclear whether the type of factor VIII product administered and switching among products are associated with the development of clinically relevant inhibitory antibodies (inhibitor development).
- Published
- 2013
25. Prevention of venous thromboembolism in patients with cancer: Guidelines of the Italian Society for Haemostasis and Thrombosis (SISET)
- Author
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Siragusa S, Armani U, Carpenedo M, Falanga A, Fulfaro F, Imberti D, Laurora R, Molinari AC, Prisco D, Silingardi M, Verso M, Visona A, Siragusa, S, Armani, U, Carpenedo, M, Falanga, A, Fulfaro, F, Imberti, D, Laurora, R, Molinari, A, Prisco, D, Silingardi, M, Verso, M, and Visona, A
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Prevention of venous thromboembolism (VTE) - Abstract
Background: Prevention of venous thromboembolism (VTE) in cancer patients remains controversial in most clinical settings. Purpose: The Italian Society for Haemostasis and Thrombosis (SISET) commissioned a project to develop clinical practice guidelines for the prevention of VTE in patients with malignancy. Methods: Key questions concerning the prevention of VTE in patients with malignancy were formulated by a multidisciplinary working group consisting of experts in clinical medicine and research. After a systematic review and discussion of the literature, recommendations were formulated and graded according to the supporting evidence. For those questions for which the literature search did not find any definitive answers (due to absence of evidence, low quality evidence and/or contradictory evidence), a formal consensus method was used instead to issue clinical recommendations. Results: The search for "VTE prevention" resulted in 1021 citations; 69 articles were selected and 24 were used for drafting clinical recommendations. Four areas were graded A to C: 1) Need of prevention (pharmacological and/or mechanical) in cancer patients undergoing major abdominal or pelvic surgery and in 2) those with an acute medical disease requiring hospitalization and who are bedridden. Avoid prevention in 3) cancer patients with a central venous catheter and 4) those on chemotherapy, radiotherapy or hormonal therapy, except patients with multiple myeloma treated with thalidomide/lenalidomide plus high-dose dexamethasone, and those with gastrointestinal or lung cancer. Six areas were considered to be clinically important, but lacked evidence from the literature and thus required a formal consensus (grade D): 1) need of prevention during chemo-radiotherapy or hormonal therapy in patients with previous VTE; 2) optimal duration of pharmacological prevention in patients who are hospitalized/bedridden for acute medical illness; 3) optimal duration of pharmacological prevention in patients undergoing major surgery other than abdominal and pelvic; 4) optimal duration of pharmacological prevention in myeloma patients receiving thalidomide plus dexamethasone; 5) presence of cerebral metastasis as a contraindication to pharmacological prevention; 6) prevention in cancer patients undergoing surgery by laparoscopic procedures lasting> 30 min. Conclusion: Results of the systematic literature review and an explicit approach to consensus techniques have led to recommendations for the most clinically important issues in the prevention of VTE in cancer patients. (C) 2011 Elsevier Ltd. All rights reserved. Background: Prevention of venous thromboembolism (VTE) in cancer patients remains controversial in most clinical settings. Purpose: The Italian Society for Haemostasis and Thrombosis (SISET) commissioned a project to develop clinical practice guidelines for the prevention of VTE in patients with malignancy. Methods: Key questions concerning the prevention of VTE in patients with malignancy were formulated by a multidisciplinary working group consisting of experts in clinical medicine and research. After a systematic review and discussion of the literature, recommendations were formulated and graded according to the supporting evidence. For those questions for which the literature search did not find any definitive answers (due to absence of evidence, low quality evidence and/or contradictory evidence), a formal consensus method was used instead to issue clinical recommendations. Results: The search for "VTE prevention" resulted in 1021 citations; 69 articles were selected and 24 were used for drafting clinical recommendations. Four areas were graded A to C: 1) Need of prevention (pharmacological and/or mechanical) in cancer patients undergoing major abdominal or pelvic surgery and in 2) those with an acute medical disease requiring hospitalization and who are bedridden. Avoid prevention in 3) cancer patients with a central venous catheter and 4) those on chemotherapy, radiotherapy or hormonal therapy, except patients with multiple myeloma treated with thalidomide/lenalidomide plus high-dose dexamethasone, and those with gastrointestinal or lung cancer. Six areas were considered to be clinically important, but lacked evidence from the literature and thus required a formal consensus (grade D): 1) need of prevention during chemo- radiotherapy or hormonal therapy in patients with previous VTE; 2) optimal duration of pharmacological prevention in patients who are hospitalized/bedridden for acute medical illness; 3) optimal duration of pharmacological prevention in patients undergoing major surgery other than abdominal and pelvic; 4) optimal duration of pharmacological prevention in myeloma patients receiving thalidomide plus dexamethasone; 5) presence of cerebral metastasis as a contraindication to pharmacological prevention; 6) prevention in cancer patients undergoing surgery by laparoscopic procedures lasting > 30 min. Conclusion: Results of the systematic literature review and an explicit approach to consensus techniques have led to recommendations for the most clinically important issues in the prevention of VTE in cancer patients. © 2012 Elsevier Ltd. All rights reserved.
- Published
- 2012
26. The Italian registry of childhood thrombosis (RITI – registro italiano trombosi infantile) on behalf of the group for the Italian registry of childhood thrombosis (GIRTI)
- Author
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Gentilomo, C, Saracco, P, Agostini, M, Bagna, R, Bassi, Bianca, Giordano, P, Laverda, Am, Molinari, Ac, Ramenghi, La, and Simioni, P.
- Published
- 2011
27. A PRACTICAL APPROACH TO DIAGNOSIS AND TREATMENT OF SYMPTOMATIC THROMBOEMBOLIC EVENTS IN CHILDREN WITH ACUTE LYMPHOBLASTIC LEUKEMIA: RECOMMENDATIONS OF THE 'COAGULATION DEFECTS' AIEOP WORKING GROUP
- Author
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Giordano, FRANCESCO PAOLO, DEL VECCHIO GC, Saracco, P, Zecca, M, Molinari, Ac, DE MATTIA, D., DEL PRINCIPE, D, Jankovic, M, Nobili, B, Nardi, M, Ramenghi, U, Russo, G, and Santoro, N
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Catheterization, Central Venous ,pediatric ,Heparin ,Thromboembolism ,acute lymphoblastic leukemia ,tromboembolism ,Humans ,Thrombolytic Therapy ,Heparin, Low-Molecular-Weight ,Precursor Cell Lymphoblastic Leukemia-Lymphoma ,Child - Abstract
Intensified treatments with multi-drug regimens are responsible for the continuously increasing survival of children with acute lymphoblastic leukaemia. However, together with the widespread use of central venous lines, they are also considered the main risk factors for the growing number of thromboembolic complications in this population. The rate of thrombosis that was observed in 17 prospective studies was 5.2%. Due to the high survival rate, it is relevant to apply strategies to the long term survivors who overcome the disease but who experience thromboembolic complications. Specific treatment includes anticoagulants, especially unfractionated heparin and low molecular weight heparins, and thrombolytic drugs in few cases. Guidelines for the treatment of thrombosis in childhood only became available recently, but they do not include specific clinical subsets such as children with acute lymphoblastic leukaemia. The problems involved in scheduling thrombosis treatment in children with malignancy have recently been discussed, however the paper does not provide practical diagnostic schemes or treatment schedules. Some important questions regarding optimal prevention and treatment are still unanswered. Moreover, antithrombotic therapy in these patients is quite challenging owing to the higher risk of bleeding. We believe it would be possible to propose reasoned appropriate recommendations for treating thrombosis in children with acute lymphoblastic leukaemia, looking forward for the effects of recent patents. This paper is an attempt to provide a practical guide to the diagnosis and treatment of thrombotic events in children with acute lymphoblastic leukaemia, and it is aimed at physicians who have no specific knowledge of the diagnosis and management of thrombosis and haemostasis alterations in children.
- Published
- 2007
28. Aumentata proteolisi del fattore von Willebrand in corso di terapia della leucemia linfoblastica acuta del bambino
- Author
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Perutelli, P, Amato, S, De Mattia, D, Del Vecchio GC, Giordano, P, Russo, Giovanna, Saracco, P, and Molinari, Ac
- Published
- 2004
29. Conditions associated with infections of indwelling central venous catheters in cancer patients: a summary
- Author
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Castagnola, E, Molinari, Ac, Fratino, G, and Viscoli, Claudio
- Published
- 2003
30. Alterazioni emostatiche trombofiliche in bambini con LLA
- Author
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Giordano, P, Del Vecchio GC, Saracco, P, Molinari, Ac, Altomare, M, Piacente, L, Perutelli, P, Crescenzio, N, Russo, Giovanna, Santoro, N, and De Mattia, D.
- Published
- 2003
31. Changing pattern of pathogens causing broviac catheter related bactaeremias in children with cancer
- Author
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Castagnola, E, Garaventa, A, Viscoli, Claudio, Carrega, G, Nantron, M, Molinari, Ac, Moroni, C, and Giacchino, R.
- Published
- 1995
32. Metabolic and Genetic Risk Factors for Migraine in Children
- Author
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Bottini, F, primary, Celle, ME, additional, Calevo, MG, additional, Amato, S, additional, Minniti, G, additional, Montaldi, L, additional, Di Pasquale, D, additional, Cerone, R, additional, Veneselli, E, additional, and Molinari, AC, additional
- Published
- 2006
- Full Text
- View/download PDF
33. Recombinant human granulocyte-macrophage colony-stimulating factor (rhGM-CSF) and rhG-CSF in the treatment of a child with severe chronic neutropenia [letter]
- Author
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Mori, PG, primary, Pasino, M, additional, Dufour, C, additional, Boeri, E, additional, and Molinari, AC, additional
- Published
- 1994
- Full Text
- View/download PDF
34. Hemophilia A: carrier detection and prenatal diagnosis by DNA analysis
- Author
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Pecorara, M, Casarino, L, Mori, PG, Morfini, M, Mancuso, G, Scrivano, AM, Boeri, E, Molinari, AC, De Biasi, R, and Ciavarella, N
- Abstract
In this study, we used DNA polymorphisms for carrier detection and prenatal diagnosis of hemophilia A in a large group of Italian families. The restriction fragment length polymorphisms (RFLPs) investigated were the intragenic polymorphic Bc/I site within the factor VIII gene; the extragenic multiallelic Taq I system at the St14 locus; and the extragenic Bg/II site at the DX13 locus. The factor VIII probe was informative in 30%, St14 in 82%, and DX13 in 60% of obligate carriers. The combination of factor VIII-Bc/I and St14-Taq I showed that 91% of obligate carriers were heterozygotes for one or both; with all three probes, only 4% of obligate carriers were noninformative. In families clearly segregating for hemophilia A, RFLP analysis allowed us to define the carrier status for the hemophilia A gene in all 27 women tested. RFLP analysis allowed us to exclude the carrier status in 39 of 45 female relatives of sporadic patients. The combination of RFLP analysis and biological assay of factor VIII allowed us to identify a de novo mutation in the maternal grandfather in 7 of 12 of the families with sporadic cases, for which members of three generations were available for study. Nine of 10 couples requesting prenatal diagnosis provided informative RFLP DNA pattern. Carrier status was excluded in two women, two fetuses were shown to be female, and prenatal diagnosis was carried out in five pregnancies by DNA analysis. Prenatal testing was successful in three instances and failed in two because a sufficient amount of chorionic villous DNA was not obtained for the analysis.
- Published
- 1987
- Full Text
- View/download PDF
35. Complicanze infettive e chirurgiche in corso di trattamento per leucemia non linfoblastica acuta
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Haupt, R, Rizzo, A, Moroni, C, Molinari, Ac, Fabbri, A, Franchini, E, Garre', Ml, Squazzini, G, and Viscoli, Claudio
- Published
- 1986
36. Evidence for a 1980 HTLV-III infection in a currently asymptomatic B haemophiliac in Italy
- Author
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Varnier, Oliviero, Melioli, G, Merli, A, Schito, Gc, Tudor, G, Boeri, E, Molinari, Ac, and Mori, Pg
- Published
- 1985
37. Timing of booster immunizations with hepatitis B vaccine
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Molinari, AC, primary, Arigliani, R, additional, Rosanda, C, additional, Favareto, F, additional, and Mori, PG, additional
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- 1987
- Full Text
- View/download PDF
38. Hepatitis B virus infection in patients with antibody to hepatitis B surface antigen
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Molinari, AC, primary, Mori, PG, additional, Boeri, E, additional, Barabino, A, additional, Pasino, M, additional, Bertorello, CF, additional, and Lanteri, MP, additional
- Published
- 1985
- Full Text
- View/download PDF
39. Systemic Catheter-Related Venous Thromboembolism in Children: Data From the Italian Registry of Pediatric Thrombosis
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Lasagni, D., Nosadini, M., Molinari, A. C., Saracco, P., Pelizza, M. F., Piersigilli, F., Putti, M. C., Gaffuri, M., Giordano, P., Lorenzoni, G., Francavilla, A., Trapani, S., Luciani, M., Suppiej, A., Tufano, A., Tormene, D., Martinato, M., Gregori, D., Sartori, S., Simioni, P., Agostini, M., Bassi, B., Bertoni, E., Casani, A., Farinasso, D., Gallo, E., Gentilomo, C., Grassi, M., Lunetta, F., Magarotto, M., Maschio, F., Palmieri, A., Pettenazzo, A., Sangermani, R., Laverda, A., Lasagni, D, Nosadini, M, Molinari, Ac, Saracco, P, Pelizza, Mf, Piersigilli, F, Putti, Mc, Gaffuri, M, Giordano, P, Lorenzoni, G, Francavilla, A, Trapani, S, Luciani, M, Suppiej, A, Tufano, A, Tormene, D, Martinato, M, Gregori, D, Sartori, S, Simioni, P, Systemic Thromboses Working Group of the Italian Registry of Pediatric Thrombosis (RITI Registro Italiano Trombosi, Infantili)., UCL - SSS/IREC/SLUC - Pôle St.-Luc, and UCL - (SLuc) Service de néonatologie
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central venous catheter (CVC) ,pediatric ,children ,Pediatrics, Perinatology and Child Health ,catheter-complications ,heparin ,registry ,thrombosis ,Socio-culturale ,equipment and supplies - Abstract
BackgroundCentral venous catheters (CVCs) represent one of the main risk factors for venous thrombotic events (VTEs) in children.MethodsWe studied the Italian Registry of Pediatric Thrombosis (RITI) with regard to systemic radiologically confirmed CVC-related VTEs (CVC-VTEs) occurred during 6.5 years in children aged 29 days to 18 years.ResultsA total of 78 CVC-VTEs were included, which occurred in 76 patients (40/76, 53% males). CVC-VTEs comprised 67 non-cardiac VTEs (86%) and 11 intracardiac thrombotic events (ICTEs) (14%); the median age at onset was 19 and 17 months, respectively. The most frequent reason for CVC insertion was supportive therapy. The catheters were placed percutaneously in 85% of cases (56/66) and surgically in the remaining 15% (10/66). Peripherally inserted central catheters (PICCs) were used in 47% (31/66) cases, partially implanted catheters in 42% (28/66), non-implantable catheters in 7% (5/66), and totally implanted catheters (Port) in 2% (1/66). CVC-VTEs were symptomatic in 77% of cases (60/78), while in the remaining 23%, they were incidentally detected on the imaging performed for the underlying condition. The median time between CVC insertion and the onset of symptoms was 10 days in non-cardiac VTEs and 39 days in ICTEs. Doppler ultrasound was the diagnostic technique most frequently used. The venous compartment most frequently affected was the veins of the lower extremities (52%, 43/73). Anti-thrombotic treatment was administered in 96% of CVC-VTEs (75/78). About 2.6% (2/76) of patients experienced a second thrombotic event. At discharge, post-thrombotic syndrome was reported in 13.5% (5/37) events with available data, CVC replacement in 10.8% (4/47), and ischemic necrosis with toe finger amputation in 2.7% (1/37). Three patients died due to an underlying condition; no CVC-VTE-related deaths were reported.ConclusionsWe have carried out a registry-based study on CVC-VTEs in the children in Italy, providing the data that may help improve the detection and management of this CVC-related complication.
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- 2022
40. Central Venous access Devices in Pediatric Malignancies: A Position Paper of Italian Association of Pediatric Hematology and Oncology
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Paola Muggeo, Simone Cesaro, Giulio Andrea Zanazzo, Maria Pia Cicalese, Alessandro Inserra, Assunta Tornesello, Angelo Claudio Molinari, Valeria Grillenzoni, Angelica Barone, Alessandro Crocoli, Viviana Durante, Mauro Pittiruti, Crocoli, A, Tornesello, A, Pittiruti, M, Barone, A, Muggeo, P, Inserra, A, Molinari, Ac, Grillenzoni, V, Durante, V, Cicalese, Mp, Zanazzo, Ga, and Cesaro, S.
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Catheterization, Central Venous ,medicine.medical_specialty ,pediatric malignancy ,complications ,medicine.medical_treatment ,Guidelines as Topic ,Pediatrics ,central venous atheter ,Neoplasms ,Internal medicine ,Central Venous Catheters ,Humans ,Medicine ,Child ,Intensive care medicine ,Hematology ,business.industry ,Neoplasms therapy ,Venous access ,Italy ,Nephrology ,Settore MED/20 ,Position paper ,Surgery ,Pediatric hematology ,business ,Central venous catheter - Abstract
Introduction Treatment of pediatric malignancies is becoming progressively more complex, implying the adoption of multimodal therapies. A reliable, long-lasting venous access represents one of the critical requirements for the success of those treatments. Recent technical innovations—such as minimally invasive procedures for placement, new devices and novel materials—have rapidly spread for clinical use in adult patients, but are still not consistently used in the pediatric population. Methods The Supportive Therapy Working Group of Italian Association of Hematology and Oncology (AIEOP) reviewed medical literature focusing on new aspects of central venous access devices (VADs) in pediatric patients affected by oncohematological diseases. Results Appropriate recommendations for clinical use in these patients have been discussed and formulated. Conclusions The importance of the correct choice, management and use of VADs in pediatric oncohematological patients is a necessary prerequisite for an adequate standard of care, also considering the increased chances of cure and the longer life expectancy of those patients with modern therapies.
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- 2014
41. Uncovered needs in the management of inherited bleeding disorders in Italy
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Romano, Arcieri, Angelo C, Molinari, Stefania, Farace, Giuseppe, Mazza, Alberto, Garnero, Gabriele, Calizzani, Paola, Giordano, Emily, Oliovecchio, Lorenzo, Mantovani, Lamberto, Manzoli, Paul, Giangrande, Arcieri, R, Molinari, Ac, Farace, S, Mazza, G, Garnero, A, Calizzani, G, Giordano, P, Oliovecchio, E, Mantovani, LORENZO GIOVANNI, Manzoli, L, Giangrande, P., Molinari, A, Mantovani, L, and Giangrande, P
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Registrie ,bleeding disorders ,Haemophilia ,The model for the prevention and reduction of health and social impacts of inherited bleeding disorders in Italy ,Comprehensive Care Centre ,Socio-culturale ,Ambientale ,Hematology ,Hemophilia A ,Hospitals, Special ,Haemophilia Treatment Centre ,Italy ,Humans ,Immunology and Allergy ,Registries ,Delivery of Health Care ,Human - Published
- 2014
42. Present and future challenges in the treatment of haemophilia: the patient's perspective
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Romano, Arcieri, Angelo C, Molinari, Stefania, Farace, Giuseppe, Mazza, Alberto, Garnero, Gabriele, Calizzani, Paola, Giordano, Emily, Oliovecchio, Lorenzo, Mantovani, Lamberto, Manzoli, Paul, Giangrande, Arcieri, R, Molinari, Ac, Farace, S, Mazza, G, Garnero, A, Calizzani, G, Giordano, P, Oliovecchio, E, Mantovani, LORENZO GIOVANNI, Manzoli, L, Giangrande, P., Molinari, A, Mantovani, L, and Giangrande, P
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Comprehensive care ,Drug Therapy ,Sustainability ,Factor VIII demand ,haemophilia treatment, factor VIII demand, comprehensive care, sustainability ,Humans ,Socio-culturale ,Haemophilia treatment ,Review ,Hemophilia A - Published
- 2013
43. Development and definition of a simplified scanning procedure and scoring method for Haemophilia Early Arthropathy Detection with Ultrasound (HEAD-US)
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Ornella Della Casa Alberighi, Ermelinda Graziano, Angelo Claudio Molinari, Gianluigi Pasta, Elena Santagostino, Annarita Tagliaferri, Giuseppe Russo, Giovanni Di Minno, Carlo Martinoli, Massimo Morfini, Alberto Tagliafico, Martinoli, C, Della Casa Alberighi, O, DI MINNO, Giovanni, Graziano, E, Molinari, Ac, Pasta, G, Russo, G, Santagostino, E, Tagliaferri, A, Tagliafico, A, and Morfini, M.
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Adult ,medicine.medical_specialty ,Adolescent ,Early signs ,Elbow ,030204 cardiovascular system & hematology ,Hemophilia A ,Haemophilia ,Severity of Illness Index ,Young Adult ,03 medical and health sciences ,0302 clinical medicine ,Synovitis ,Hemarthrosis ,Arthropathy ,medicine ,Humans ,Knee ,Medical physics ,Child ,Aged ,Ultrasonography ,Observer Variation ,Haemophilic arthropathy ,business.industry ,Ultrasound ,Hematology ,Middle Aged ,medicine.disease ,medicine.anatomical_structure ,Child, Preschool ,Physical therapy ,Joints ,Ankle ,Joint Diseases ,business ,030215 immunology - Abstract
SummaryThe aim of this study was to develop a simplified ultrasound scanning procedure and scoring method, named Haemophilia Early Arthropathy Detection with UltraSound [HEAD-US], to evaluate joints of patients with haemophilic arthropathy. After an initial consensus-based process involving a multidisciplinary panel of experts, three comprehensive and evidence-based US scanning procedures to image the elbow, knee and ankle were established with the aim to increase sensitivity in detection of early signs of joint involvement while keeping the technique easy and quick to perform. Each procedure included systematic evaluation of synovial recesses and selection of a single osteochondral surface for damage analysis. Based on expert consensus, a simplified scoring system based on an additive scale was created to define the joint status and, in perspective, to offer a tool to evaluate disease progression and monitor the result of treatment in follow-up studies.
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- 2013
44. Management of bleeding and of invasive procedures in patients with platelet disorders and/or thrombocytopenia: Guidelines of the Italian Society for Haemostasis and Thrombosis (SISET)
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Esther Diana Rossi, Marco Cattaneo, Sergio Siragusa, Carlo L. Balduini, E. De Candia, Alberto Tosetto, G. Mariani, Angelo Claudio Molinari, Tosetto, A, Balduini, CL, Cattaneo, M, De Candia, E, Mariani, G, Molinari, AC, Rossi, E, and Siragusa, S
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Male ,medicine.medical_specialty ,Platelet disorder ,MEDLINE ,Settore MED/15 - Malattie Del Sangue ,platelet, transfusion ,medicine ,Humans ,Platelet ,Aprotinin ,Intensive care medicine ,Adverse effect ,Desmopressin ,business.industry ,Settore MED/09 - MEDICINA INTERNA ,Hematology ,bleeding ,medicine.disease ,Thrombosis ,Thrombocytopenia ,Surgery ,Bleeding diathesis ,Italy ,Surgical Procedures, Operative ,platelet defects ,Female ,Blood Platelet Disorders ,business ,medicine.drug - Abstract
The optimal management of bleeding or its prophylaxis in patients with disorders of platelet count or function is controversial. The bleeding diathesis of these patients is usually mild to moderate: therefore, transfusion of platelet concentrates may be inappropriate, as potential adverse effects might outweigh its benefit. The availability of several anti-hemorrhagic drugs further compounds this problem, mainly because the efficacy/suitability of the various treatment options in different clinical manifestations is not well defined. In these guidelines, promoted by the Italian Society for Studies on Haemostasis and Thrombosis (Società Italiana per lo Studio dell'Emostasi e della Trombosi [SISET]), we aim at offering the best available evidence to help the physicians involved in the management of patients with disorders of platelet count or function. Literature review and appraisal of available evidence are discussed for different clinical settings and for different available treatments, including platelet concentrates (PC), recombinant activated factor VII, desmopressin, antifibrinolytics, aprotinin and local hemostatic agents.
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- 2009
45. Gene therapy for people with Haemophilia B: a proposed care delivery model in Italy.
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Castaman G, Di Minno G, Simioni P, Molinari AC, Siragusa S, Baldacci E, La Mura V, Lupi A, Grazzi EF, and Peyvandi F
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Background: Gene therapy is designed to provide people with haemophilia B with a steady and elevated factor IX activity, thereby strengthening protection and relieving the burden of replacement therapy frequent infusions. The European Medicines Agency has approved a gene therapy for the severe and moderately severe forms of haemophilia B that uses the factor IX Padua variant (etranacogene dezaparvovec). The aim is to provide a document dedicated to haemophilia B gene therapy and give a comprehensive overview of the topic., Methods: An Italian group of experts in haemophilia has carried out a narrative review of the literature and has discussed during a virtual meeting several key aspects of the delivery of this treatment in Italy. The discussion covered the organisational model, the role of the multidisciplinary team, the laboratory surveillance and the patient journey, from the follow-up to the identification of safety issues and outcome measures., Results: This article highlights the need to follow the Hub and Spoke organisational model and sheds light on the role of each professional figure within the multidisciplinary teams to favour patient engagement, management, and retention. Moreover, this article stresses the need to perform laboratory tests for patient screening and follow-up and proposes a checklist to help patient identification. Finally, the needs of Italian haemophilia centres have been considered to ensure an efficient implementation of the care delivery model., Conclusion: It is crucial to ensure that centres are appropriately organized, equipped and trained to adequately select patients, deliver the gene therapy and perform follow-up., (Copyright © 2024. Published by Elsevier Inc.)
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- 2024
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46. Prolongated Activated Partial Thromboplastin Time (aPTT) in Pediatric Patients before Surgery-Crying Wolf: Lupus (Anticoagulant) Does Not Always Threaten Children.
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Martini T, Santoro RC, Banov L, Ierardi A, Leotta M, Strangio A, Svahn J, and Molinari AC
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A prolonged preoperatory aPTT in children is often the cause of a delay of scheduled surgeries and the repetition of multiple blood tests, with the consequent wasting of resources and significant discomfort for children and parents. The aim of this review is to analyze the situations in which an isolated prolongation of aPTT is found during preoperative evaluation in children, especially when it is due to the presence of antiphospholipid antibodies, providing the readers with the keys to interpret this situation and the possibility to correctly evaluate the hemorrhagic risk of a patient.
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- 2024
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47. The value-based healthcare approach to haemophilia: Development of outcome measures for the evaluation of care of people with haemophilia.
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Cortesi PA, Fornari C, Conti S, Pollio B, Boccalandro E, Buzzi A, Carulli C, Coppola A, De Cristofaro R, Di Minno MND, Dolan G, Ferri Grazzi E, Fornari A, Gualtierotti R, Hermans C, Jiménez-Juste V, Kenet G, Lupi A, Martinoli C, Mansueto MF, Nicolò G, Tagliaferri A, Gringeri A, Molinari AC, Mantovani LG, and Castaman G
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- Humans, Quality of Life, Cross-Sectional Studies, Value-Based Health Care, Outcome Assessment, Health Care, Hemophilia A drug therapy
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Introduction: Considering the advances in haemophilia management and treatment observed in the last decades, a new set of value-based outcome indicators is needed to assess the quality of care and the impact of these medical innovations., Aim: The Value-Based Healthcare in Haemophilia project aimed to define a set of clinical outcome indicators (COIs) and patient-reported outcome indicators (PROIs) to assess quality of care in haemophilia in high-income countries with a value-based approach to inform and guide the decision-making process., Methods: A Value-based healthcare approach based on the available literature, current guidelines and the involvement of a multidisciplinary group of experts was applied to generate a set of indicators to assess the quality of care of haemophilia., Results: A final list of three COIs and five PROIs was created and validated. The identified COIs focus on two domains: musculoskeletal health and function, and safety. The identified PROIs cover five domains: bleeding frequency, pain, mobility and physical activities, Health-Related Quality of Life and satisfaction. Finally, two composite outcomes, one based on COIs, and one based on PROIs, were proposed as synthetic outcome indicators of quality of care., Conclusion: The presented standard set of health outcome indicators provides the basis for harmonised longitudinal and cross-sectional monitoring and comparison. The implementation of this value-based approach would enable a more robust assessment of quality of care in haemophilia, within a framework of continuous treatment improvements with potential added value for patients. Moreover, proposed COIs and PROIs should be reviewed and updated routinely., (© 2024 John Wiley & Sons Ltd.)
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- 2024
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48. Endovascular repair of ascending aortic pathologies in patients unfit for open surgery: case series and literature review.
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Ferraresi M, Katsarou M, Luigi Molinari AC, Segreti S, and Rossi G
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The number of vascular centers performing endovascular repair of ascending aortic disease is constantly increasing. Accordingly to the guidelines, open surgical repair remains the gold standard for these pathologies. However, approximately one quarter of patients are deemed unfit for open surgery. In this study, we describe three cases of ascending thoracic endovascular aortic repair (TEVAR) performed at our center. All the patients were deemed unfit for open surgery by the aortic team. Two patients had an ascending aortic pseudoaneurysm, and the third had a focal type A aortic dissection. In two cases, we used two abdominal aortic cuffs deployed from zone 0B to zone 0C, with no need for supra-aortic trunk debranching. In one case, we performed a "reverse" extrathoracic debranching, and we deployed a thoracic endograft from zone 0B to zone 2. Complications included one minor stroke and one inguinal hematoma. In one patient with an infected pseudoaneurysm, we performed ascending TEVAR as a bridge strategy for open repair. This patient developed a type Ia endoleak; however, clinical stabilization and infection control were obtained, and he was able to undergo heart surgery successfully. He underwent a second reintervention to treat superior mesenteric embolic occlusion. At 2 years of follow-up, all three patients were alive. Our preliminary experience demonstrates the technical feasibility and clinical appropriateness of ascending TEVAR using standard, commercially available endografts. However, no consensus has been reached regarding some critical aspects, such as the development of a standardized technique or the efficacy of the currently available devices. The improvements in graft design and the adoption of the "aortic team" approach could help in the near future to standardize the procedure, establish appropriate indications, and ensure good clinical outcomes., Competing Interests: None., (© 2024 The Authors.)
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- 2024
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49. Corrigendum: The new Italian registry of infantile thrombosis (RITI): a reflection on its journey, challenges and pitfalls.
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Pelizza MF, Martinato M, Rosati A, Nosadini M, Saracco P, Giordano P, Luciani M, Ilardi L, Lasagni D, Molinari AC, Bagna R, Palmieri A, Ramenghi LA, Grassi M, Magarotto M, Magnetti F, Francavilla A, Indolfi G, Suppiej A, Gentilomo C, Restelli R, Tufano A, Tormene D, Pin JN, Tona C, Meneghesso D, Rota L, Conti M, Russo G, Lorenzoni G, Gregori D, Sartori S, and Simioni P
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[This corrects the article DOI: 10.3389/fped.2023.1094246.]., (© 2024 Pelizza, Martinato, Rosati, Nosadini, Saracco, Giordano, Luciani, Ilardi, Lasagni, Molinari, Bagna, Palmieri, Ramenghi, Grassi, Magarotto, Magnetti, Francavilla, Indolfi, Suppiej, Gentilomo, Restelli, Tufano, Tormene, Pin, Tona, Meneghesso, Rota, Conti, Russo, Lorenzoni, Gregori, Sartori, Simioni and Collaborators of the R.I.T.I. (Italian and Registry of Infantile Thrombosis).)
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- 2024
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50. Lupus Anticoagulant Detection under the Magnifying Glass.
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Molinari AC, Martini T, Banov L, Ierardi A, Leotta M, Strangio A, and Santoro RC
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Diagnosis of antiphospholipid syndrome (APS) requires the presence of a clinical criterion (thrombosis and/or pregnancy morbidity), combined with persistently circulating antiphospholipid antibodies (aPL). Lupus anticoagulant (LA) is one of the three laboratory parameters (the others being antibodies to either cardiolipin or β2-glycoprotein I) that defines this rare but potentially devastating condition. For the search for aCL and aβ2-GP-I, traditionally measured with immunological solid-phase assays (ELISA), several different assays and detection techniques are currently available, thus making these tests relatively reliable and widespread. On the other hand, LA detection is based on functional coagulation procedures that are characterized by poor standardization, difficulties in interpreting the results, and interference by several drugs commonly used in the clinical settings in which LA search is appropriate. This article aims to review the current state of the art and the challenges that clinicians and laboratories incur in the detection of LA.
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- 2023
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