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

Author

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

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

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

Published
2020

8. Perceived challenges and attitudes to regimen and product selection from Italian haemophilia treaters: the 2013 AICE survey

Author

Franchini, M, Coppola, A, Rocino, A, Zanon, E, Morfini, M, 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.

Subjects
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.

Published
2014

10. Comorbidities in persons with haemophilia aged 60 years or more compared with age-matched people from the general population

Author

Marchesini, E., primary, Oliovecchio, E., additional, Coppola, A., additional, Santagostino, E., additional, Radossi, P., additional, Castaman, G., additional, Valdrè, L., additional, Santoro, C., additional, Tagliaferri, A., additional, Ettorre, C., additional, Zanon, E., additional, Barillari, G., additional, Cantori, I., additional, Caimi, T. M., additional, Sottilotta, G., additional, Iorio, A., additional, and Mannucci, P. M., additional

Published
2017
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11. Consumption of clotting factors in severe haemophilia patients undergoing prophylaxis and on-demand treatment in Italy

Author

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

12. Replacement therapy with recombinant factor IX. A multicentre evaluation of current dosing practices in Italy

Author

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

13. Mortality and causes of death in Italian persons with haemophilia, 1990-2007

Author

Tagliaferri, A, Rivolta, Gf, Iorio, A, Oliovecchio, E, Mancuso, Me, Morfini, M, Rocino, A, Mazzucconi, Mg, Franchini, M, Italian Association of Hemophilia Centers, Ciavarella, N, Scaraggi, A, Valdrè, L, Tagariello, G, Radossi, P, Muleo, G, Iannaccaro, Pg, Biasoli, C, Vincenzi, D, Serino, Ml, Linari, S, Molinari, C, Boeri, E, La Pecorella, M, Carloni, Mt, Santagostino, E, Di Minno, G, Coppola, A, Zanon, E, Spiezia, L, Di Perna, C, Marchesini, M, Marcucci, M, Dragani, A, Macchi, S, Albertini, P, D'Incà, M, Santoro, C, Biondo, F, Piseddu, G, Rossetti, G, Barillari, G, Gandini, Giorgio, Giuffrida, Anna Chiara, Castaman, G., Tagliaferri, A, Rivolta, Gf, Iorio, A, Oliovecchio, E, Mancuso, Me, Morfini, M, Rocino, A, Mazzucconi, Mg, Franchini, M, Ciavarella, N, Scaraggi, A, Valdrè, L, Tagariello, G, Radossi, P, Muleo, G, Iannaccaro, Pg, Biasoli, C, Vincenzi, D, Serino, Ml, Linari, S, Molinari, C, Boeri, E, La Pecorella, M, Carloni, Mt, Santagostino, E, DI MINNO, Giovanni, Coppola, A, Zanon, E, Spiezia, L, Di Perna, C, Marchesini, M, Marcucci, M, Dragani, A, Macchi, S, Albertini, P, D'Incà, M, Santoro, C, Biondo, F, Piseddu, G, Rossetti, G, Barillari, G, Gandini, G, Giuffrida, Ac, Castaman, G, and Italian Association of Hemophilia, Centers

Subjects
Pediatrics, retrospective study, HIV Infections, cause of death, Hepatitis C: complications, Hemophilia A: complications, hemophilia, Medicine, Young adult, Child, Genetics (clinical), Cause of death, education.field_of_study, Mortality rate, adult, article, Hematology, General Medicine, Hepatitis C, Middle Aged, Italy: epidemiology, aged, comorbidity, disease association, hepatitis C, human, Italy, life expectancy, major clinical study, male, mortality, priority journal, sex difference, HIV Infections: mortality, Child, Preschool, Female, medicine.medical_specialty, Adolescent, Population, Hepatitis C: mortality, Hemophilia A: mortality, Haemophilia, Hemophilia A, Hemophilia B, Young Adult, cancer, haemophilia, hcv, hiv, Humans, Cancer, HCV, HIV, Mortality, Preschool, education, Retrospective Studies, Hemophilia B: complications, HIV Infections: complications, business.industry, Retrospective cohort study, medicine.disease, Adult, Aged, Cause of Death, Hemophilia B: mortality, Life Expectancy, Male, Life expectancy, business
Abstract

Although a number of studies have analysed so far the causes of death and the life expectancy in haemophilic populations, no investigations have been conducted among Italian haemophilia centres. Thus, the aim of this study was to investigate mortality, causes of deaths, life expectancy and co-morbidities in Italian persons with haemophilia (PWH). Data pertaining to a total of 443 PWH who died between 1980 and 2007 were retrospectively collected in the 30 centres who are members of the Italian Association of Haemophilia Centres that chose to participate. The mortality rate ratio standardized to the male Italian population (SMR) was reduced during the periods 1990-1999 and 2000-2007 such that during the latter, death rate overlapped that of the general population (SMR 1990-1999: 1.98 95% CI 1.54-2.51; SMR 2000-2007: 1.08 95% CI 0.83-1.40). Similarly, life expectancy in the whole haemophilic population increased in the same period (71.2 years in 2000-2007 vs. 64.0 in 1990-1999), approaching that of the general male population. While human immunodeficiency virus infection was the main cause of death (45%), 13% of deaths were caused by hepatitis C-associated complications. The results of this retrospective study show that in Italian PWH improvements in the quality of treatment and global medical care provided by specialized haemophilia centres resulted in a significantly increased life expectancy.

Published
2010

14. Comparison of the rates of joint arthroplasty in patients with severe factor VIII and IX deficiency: an index of different clinical severity of the 2 coagulation disorders

Author

Tagariello G, Iorio A, Santagostino E, Morfini M, Bisson R, Innocenti M, Mancuso ME, Mazzucconi MG, Pasta GL, Radossi P, Rodorigo G, Santoro C, Sartori R, Scaraggi A, Solimeno LP, Mannucci PM, Ciavarella N, Valdrè L, Targhetta R, Biasioli C, Vincenzi D, Molinari C, Boeri E, Mariani G, Lapecorella M, Coppola A, Schiavulli M, Rocino A, Mancuso G, Siragusa S, Malato A, Tagliaferri A, Rivolta F, Oliovecchio E, Marcucci M, Dragani A, Mancino A, D'Incà M, De Rossi G, Luciani M, Landolfi R, Piseddu G, Schinco PC, Rossetti G, Barillari G, Feola G, Gandini G, Giuffrida A, Castaman G., DI MINNO, GIOVANNI, Tagariello, G, Iorio, A, Santagostino, E, Morfini, M, Bisson, R, Innocenti, M, Mancuso, ME, Mazzucconi, MG, Pasta, GL, Radossi, P, Rodorigo, G, Santoro, C, Sartori, R, Scaraggi, A, Solimeno, LP, Mannucci, PM, Ciavarella, N, Valdrè, L, Targhetta, R, Biasioli, C, Vincenzi, D, Molinari, C, Boeri, E, Mariani, G, Lapecorella, M, di Minno, G, Coppola, A, Schiavulli, M, Rocino, A, Mancuso, G, Siragusa, S, Malato, A, Tagliaferri, A, Rivolta, F, Oliovecchio, E, Marcucci, M, Dragani, A, Mancino, A, D'Incà, M, De Rossi, G, Luciani, M, Landolfi, R, Piseddu, G, Schinco, PC, Rossetti, G, Barillari, G, Feola, G, Gandini, G, Giuffrida, A, Castaman, G, Mancuso, Me, Mazzucconi, Mg, Pasta, Gl, Solimeno, Lp, Mannucci, Pm, DI MINNO, Giovanni, Schinco, Pc, and Castaman, G.

Subjects
Adult, medicine.medical_specialty, Adolescent, medicine.medical_treatment, Immunology, Population, macromolecular substances, Gene mutation, Hemophilia A, Hemophilia B, Severity of Illness Index, Biochemistry, Arthroplasty, Settore MED/15 - Malattie Del Sangue, Cohort Studies, Young Adult, Risk Factors, hemic and lymphatic diseases, Internal medicine, Severity of illness, medicine, Coagulopathy, Humans, Child, education, Retrospective Studies, education.field_of_study, Hematology, hamophilia, arthroplasty, business.industry, Retrospective cohort study, Cell Biology, Odds ratio, Blood Coagulation Disorders, Middle Aged, medicine.disease, Surgery, Italy, Joint Diseases, business, Algorithms
Abstract

Data from the Italian Hemophilia Centres were collected to perform a retrospective survey of joint arthroplasty in patients with severe hemophilia. Twenty-nine of 49 hemophilia centers reported that 328 of the 347 operations were carried out in 253 patients with severe hemophilia A (HA) and 19 in 15 patients with severe hemophilia B (HB). When results were normalized to the whole Italian hemophilia population (1770 severe HA and 319 severe HB), patients with HA had a 3-fold higher risk of undergoing joint arthroplasty (odds ratio [OR], 3.38; 95% confidence interval [CI], 1.97-5.77; P < .001). These results were confirmed after adjustment for age, HIV, hepatitis C virus (HCV), and inhibitor in a Cox regression model (HR, 2.65; 95% CI, 1.62-4.33; P < .001). The survival analysis of time to joint arthroplasty in the subset of patients with severe HA was not affected by the severity of factor VIII (FVIII) gene mutations. A systematic review of literature articles reporting joint arthroplasties in HA and HB showed that the proportion of HA patients who had undergone arthroplasties was higher than that of HB patients, in agreement with the findings in our Italian cohort. These data suggest that the 2 inherited coagulation disorders have a different severity of clinical phenotype.

Published
2009

15. Evidence-based recommendations on the treatment of von Willebrand disease in Italy

Author

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

16. Italian Registry of Haemophilia and Allied Disorders. Objectives, methodology and data analysis

Author

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

Subjects
methodology, haemophilia, Settore MED/15 - Malattie Del Sangue
Published
2008

17. Italian Registry of Haemophilia and Allied Disorders. Objectives, methodology and data analysis

Author

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

23. Replacement therapy with recombinant factor IX . A multicentre evaluation of current dosing practices in Italy

Author

Rocca, A., Pizzinelli, S., Oliovecchio, E., Santagostino, E., Rocino, A., Iorio, A., Hoc Study Group, Italian A. D., Mazzucconi, Maria Gabriella, and Santoro, Cristina

Subjects
Adult, Male, Adolescent, HIV Infections, Hepacivirus, Hemophilia B, recovery, 0011-10, 2010, 2450, blood transfus doi 10, factor ix, haemophilia b, recombinant fix, Humans, Aged, Age Factors, HIV, Original Articles, Middle Aged, Hepatitis C, Recombinant Proteins, Female, Follow-Up Studies
Abstract

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.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.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.We found that dosing of rFIX in clinical practice is very close to that of plasma-derived 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
2010

25. Uncovered needs in the management of inherited bleeding disorders in Italy

Author

Arcieri, R, Molinari, A, Farace, S, Mazza, G, Garnero, A, Calizzani, G, Giordano, P, Oliovecchio, E, Mantovani, L, Manzoli, L, Giangrande, P, Molinari, AC, Arcieri, R, Molinari, A, Farace, S, Mazza, G, Garnero, A, Calizzani, G, Giordano, P, Oliovecchio, E, Mantovani, L, Manzoli, L, Giangrande, P, and Molinari, AC

Published
2014

30. Uncovered needs in the management of inherited bleeding disorders in Italy

Author

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

Subjects
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

31. Definition of an organisational model for the prevention and reduction of health and social impacts of inherited bleeding disorders

Author

Calizzani, Gabriele, Menichini, Ivana, Candura, Fabio, Lanzoni, Monica, Profili, Samantha, Tamburrini, Maria Rita, Fortino, Antonio, Vaglio, Stefania, Marano, Giuseppe, Facco, Giuseppina, Oliovecchio, Emily, Franchini, Massimo, Coppola, Antonio, Arcieri, Romano, Bon, Cinzia, Saia, Mario, Nuti, Sabina, Morfini, Massimo, Liumbruno, Giancarlo M., Di Minno, Giovanni, Grazzini, Giuliano, Calizzani, G, Menichini, I, Candura, F, Lanzoni, M, Profili, S, Tamburrini, Mr, Fortino, A, Vaglio, S, Marano, G, Facco, G, Oliovecchio, E, Franchini, M, Coppola, A, Arcieri, R, Bon, C, Saia, M, Nuti, S, Morfini, M, Liumbruno, Gm, DI MINNO, Giovanni, and Grazzini, G.

Subjects
Male, Aging, The model for the prevention and reduction of health and social impacts of inherited bleeding disorders in Italy, clinical pathway, performance indicators, Community Networks, Blood Coagulation Disorders, Inherited, Cost of Illness, Italy, Socioeconomic Factors, Models, Organizational, Humans, haemophilia care, Female
Abstract

Due to the increase in life expectancy, patients with haemophilia and other inherited bleeding disorders are experiencing age-related comorbidities that present new challenges. In order to meet current and emerging needs, a model for healthcare pathways was developed through a project funded by the Italian Ministry of Health. The project aimed to prevent or reduce the social-health burden of the disease and its complications.The National Blood Centre appointed a panel of experts comprising clinicians, patients, National and Regional Health Authority representatives. Following an analysis of the scientific and regulatory references, the panel drafted a technical proposal containing recommendations for Regional Health Authorities, which has been formally submitted to the Ministry of Health. Finally, a set of indicators to monitor haemophilia care provision has been defined.In the technical document, the panel of experts proposed the adoption of health policy recommendations summarised in areas, such as: multidisciplinary integrated approach for optimal healthcare provision; networking and protocols for emergency care; home therapy; registries/databases; replacement therapy supply and distribution; recruitment and training of experts in bleeding disorders. The recommendations became the content of proposal of agreement between the Government and the Regions. Monitoring and evaluation of haemophilia care through the set of established indicators was partially performed due to limited available data.The project provided recommendations for the clinical and organisational management of patient with haemophilia. A particular concern was given to those areas that play a critical role in the comorbidities and complications prevention. Recommendations are expected to harmonise healthcare care delivery across regional networks and building the foundation for the national haemophilia network.

Published
2014

32. Present and future challenges in the treatment of haemophilia: the patient's perspective

Author

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

Subjects
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

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

Author

Mannucci PM, Nobili A, Marchesini E, Oliovecchio E, Cortesi L, Coppola A, Santagostino E, Radossi P, Castaman G, Valdrè L, Santoro C, Tagliaferri A, Ettorre C, Zanon E, Barillari G, Cantori I, Caimi TM, Sottilotta G, Peyvandi F, and Iorio A

Subjects
Age Factors, Female, Hemophilia A pathology, Humans, Male, Prevalence, Risk Factors, Hemophilia A drug therapy, Polypharmacy
Abstract

Background: In older people, multiple chronic ailments lead to the intake of multiple medications (polypharmacy) that carry a number of negative consequences (adverse events, prescription and intake errors, poor adherence, higher mortality). Because ageing patients with haemophilia (PWHs) may be particularly at risk due to their pre-existing multiple comorbidities (arthropathy, liver disease), we chose to analyse the pattern of chronic drug intake in a cohort of PWHs aged 60 years or more., Patients and Methods: S + PHERA is a multicentre observational study, with the broad goal to evaluate prospectively the health status and medication intake in 102 older patients with severe haemophilia A or B compared with 204 age- and residence-matched controls chosen randomly from the same general practices of PWHs. The rate of potential drug-drug interactions (PDDI) was evaluated as a proxy of prescription appropriateness., Results: After excluding replacement therapies and antiviral drugs, PWHs took in average less daily drugs than controls (2.4 ± 2.5 vs 3.0 ± 2.4) and had a lower rate of polypharmacy. Moreover, their prevalence of PDDI was lower (16.7% vs 27%)., Conclusions: The rate of polypharmacy and the appropriateness of medications other than those for haemophilia and related comorbidities are acceptable in Italian PWHs, and better than those in their age peers without haemophilia, perhaps owing to drug tailoring and deprescribing by the specialized haemophilia centres at the time of regular visits. However, the PWHs investigated herewith were relatively young and the rate of polypharmacy and related PDDIs may become more prominent and crucial when older ages are reached, suggesting the need of continuous surveillance on prescribed drugs and the risk of drug-drug interactions., (© 2018 John Wiley & Sons Ltd.)

Published
2018
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36. Definition of an organisational model for the prevention and reduction of health and social impacts of inherited bleeding disorders.

Author

Calizzani G, Menichini I, Candura F, Lanzoni M, Profili S, Tamburrini MR, Fortino A, Vaglio S, Marano G, Facco G, Oliovecchio E, Franchini M, Coppola A, Arcieri R, Bon C, Saia M, Nuti S, Morfini M, Liumbruno GM, Di Minno G, and Grazzini G

Subjects
Community Networks standards, Cost of Illness, Female, Humans, Italy, Male, Aging, Blood Coagulation Disorders, Inherited economics, Blood Coagulation Disorders, Inherited therapy, Community Networks economics, Community Networks organization & administration, Models, Organizational, Socioeconomic Factors
Abstract

Introduction: Due to the increase in life expectancy, patients with haemophilia and other inherited bleeding disorders are experiencing age-related comorbidities that present new challenges. In order to meet current and emerging needs, a model for healthcare pathways was developed through a project funded by the Italian Ministry of Health. The project aimed to prevent or reduce the social-health burden of the disease and its complications., Material and Methods: The National Blood Centre appointed a panel of experts comprising clinicians, patients, National and Regional Health Authority representatives. Following an analysis of the scientific and regulatory references, the panel drafted a technical proposal containing recommendations for Regional Health Authorities, which has been formally submitted to the Ministry of Health. Finally, a set of indicators to monitor haemophilia care provision has been defined., Results: In the technical document, the panel of experts proposed the adoption of health policy recommendations summarised in areas, such as: multidisciplinary integrated approach for optimal healthcare provision; networking and protocols for emergency care; home therapy; registries/databases; replacement therapy supply and distribution; recruitment and training of experts in bleeding disorders. The recommendations became the content of proposal of agreement between the Government and the Regions. Monitoring and evaluation of haemophilia care through the set of established indicators was partially performed due to limited available data., Conclusions: The project provided recommendations for the clinical and organisational management of patient with haemophilia. A particular concern was given to those areas that play a critical role in the comorbidities and complications prevention. Recommendations are expected to harmonise healthcare care delivery across regional networks and building the foundation for the national haemophilia network.

Published
2014
Full Text
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37. Current status of Italian Registries on inherited bleeding disorders.

Author

Hassan HJ, Morfini M, Taruscio D, Abbonizio F, Giampaolo A, Kodra Y, Oliovecchio E, and Vittozzi L

Subjects
Adolescent, Adult, Blood Coagulation Disorders, Inherited classification, Child, Child, Preschool, Female, Humans, Italy epidemiology, Male, Blood Coagulation Disorders, Inherited epidemiology, Blood Coagulation Disorders, Inherited therapy, Registries standards
Published
2014
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38. Perceived challenges and attitudes to regimen and product selection from Italian haemophilia treaters: the 2013 AICE survey.

Author

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

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

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

Published
2014
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40. Management of inherited von Willebrand disease in Italy: results from the retrospective study on 1234 patients.

Author

Federici AB, Bucciarelli P, Castaman G, Baronciani L, Canciani MT, Mazzucconi MG, Morfini M, Rocino A, Schiavoni M, Oliovecchio E, Iorio A, and Mannucci PM

Subjects
Adolescent, Adult, Aged, Aged, 80 and over, Child, Child, Preschool, Deamino Arginine Vasopressin therapeutic use, Factor VIII therapeutic use, Female, Gene Frequency, Hemostatics therapeutic use, Humans, Infant, Italy, Male, Middle Aged, Retrospective Studies, Young Adult, von Willebrand Diseases diagnosis, von Willebrand Factor therapeutic use, Mutation, von Willebrand Diseases drug therapy, von Willebrand Diseases genetics, von Willebrand Factor genetics
Abstract

Von Willebrand disease (VWD) is the most common inherited bleeding disorder and is due to quantitative and/or qualitative defects of von Willebrand factor (VWF). Despite the improved knowledge of the disease, detailed data on VWD types requiring specific treatments have not been reported thus far. To determine the number and types of VWD requiring therapy with desmopressin (DDAVP) and/or VWF/FVIII concentrates in Italy, a national registry on VWD (RENAWI) was organized. Only 16 of 48 centers included VWD in the RENAWI with diagnoses performed locally. Patients with uncertain results were retested by two expert laboratories using multimeric analysis and mutations of the VWF gene. A total of 1234 of 1529 (81%) cases satisfied the inclusion criteria and could be classified as VWD1 (63%), VWD2A (7%), VWD2B (6%), VWD2M (18%), VWD2N (1%), and VWD3 (5%). VWD types were also confirmed by DNA analyses and occur in young adults (83%), mainly in women (58%). Mucosal bleedings (32 to 57%) are more frequent than hematomas (13%) or hemarthrosis (6%). Most patients were exposed to an infusion trial with desmopressin (DDAVP) and found responsive with the following rates: VWD1 (69%), VWD2A (26%), VWD2M (29%), and VWD2N (71%). However, DDAVP was not always used to manage bleeding in all responsive patients and VWF/FVIII concentrates were given instead of or together with DDAVP in VWD1 (30%), VWD2A (84%), VWD2B (62%), VWD2M (63%), VWD2N (30%), and VWD3 (91%). Data of the RENAWI showed that correct VWD identification and classification might be difficult in many Italian centers. Therefore, evidence-based studies should be organized only in well-characterized patients tested by laboratories that are expert in the clinical, laboratory, and molecular markers of VWD., (© Thieme Medical Publishers.)

Published
2011
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