title: The Italian multiregional thalassemia registry: Centers characteristics, services, and patients' population.
abstract: OBJECTIVE
  The prognosis of beta-Thalassemia major and other congenital hemoglobinopathies has profoundly changed over the last decades. Moreover, only few countries in Europe provide dedicated services and the description of the measures for patients monitoring and treatment is overall very scarce. The HTA-Thal project is aimed to identify the services available in Italy and to collect epidemiological and clinical data on the thalassemic population (HTA-Thal Registry).
  
  
  METHODS
  A map of the existing centers was created and two electronic questionnaires were completed with information on the services and patients.
  
  
  RESULTS
  On 182 centers identified, 60 completed the two questionnaires. Centers resulted to be extremely heterogeneous in terms of size, age of patients in care, and services availability. The transition of pediatric patients to adult centers was not guaranteed. Thousand eight hundred and seventy-three beta-Thalassemia major patients (of which 259 pediatrics), regularly transfused, were registered. Deferasirox is the most used chelator as monotherapy (616 patients) and its use prevails in younger patients. A higher number of patients (847 patients) use Deferoxamine, either alone (448 patients) or in combination with DFP (399 patients), while 782 patients use Deferiprone alone (383 patients) or in combination (399 patients). 31.6 and 66.6% of centers were not equipped for specialized visits or local MRI, respectively. Centers with 30-80 patients show the high percentage of patients appropriately monitored when compared to smaller or bigger centers.
  
  
  CONCLUSIONS
  This analysis confirms the importance of patients' registries for the collection of large datasets and the need for dedicated 'specialized centers' equipped to provide the best standard treatment to patients.
authors: a Fondazione per la Ricerca Farmacologica Gianni Benzi Onlus , Via Abate Eustasio 30, 70010 Valenzano (BA) , Italy.
authors: Rosa
authors: R
authors: Conte
authors: a Fondazione per la Ricerca Farmacologica Gianni Benzi Onlus , Via Abate Eustasio 30, 70010 Valenzano (BA) , Italy.
authors: Lucia
authors: L
authors: Ruggieri
authors: b Consorzio per Valutazioni Biologiche e Farmacologiche , Via L. Porta 14, 27100 Pavia , Italy.
authors: Arianna
authors: A
authors: Gambino
authors: a Fondazione per la Ricerca Farmacologica Gianni Benzi Onlus , Via Abate Eustasio 30, 70010 Valenzano (BA) , Italy.
authors: Franco
authors: F
authors: Bartoloni
authors: c Fondazione Salvatore Maugeri , Via S. Maugeri, 4, 27100 Pavia , Italy.
authors: Paola
authors: P
authors: Baiardi
authors: b Consorzio per Valutazioni Biologiche e Farmacologiche , Via L. Porta 14, 27100 Pavia , Italy.
authors: Donato
authors: D
authors: Bonifazi
authors: a Fondazione per la Ricerca Farmacologica Gianni Benzi Onlus , Via Abate Eustasio 30, 70010 Valenzano (BA) , Italy.
authors: Fedele
authors: F
authors: Bonifazi
authors: b Consorzio per Valutazioni Biologiche e Farmacologiche , Via L. Porta 14, 27100 Pavia , Italy.
authors: Mariagrazia
authors: M
authors: Felisi
authors: a Fondazione per la Ricerca Farmacologica Gianni Benzi Onlus , Via Abate Eustasio 30, 70010 Valenzano (BA) , Italy.
authors: Viviana
authors: V
authors: Giannuzzi
authors: b Consorzio per Valutazioni Biologiche e Farmacologiche , Via L. Porta 14, 27100 Pavia , Italy.
authors: Rosa
authors: R
authors: Padula
authors: d Fondazione G. Monasterio C.N.R.- Regione Toscana , Via Moruzzi 1, 56124 Pisa , Italy.
authors: Alessia
authors: A
authors: Pepe
authors: e Azienda Ospedaliera Padova Centro Leucemie Oncoematologia Pediatrica , Via N. Giustiniani 3, 35128 Padova , Italy.
authors: Maria Caterina
authors: MC
authors: Putti
authors: f Azienda Ospedaliera Universitaria Consorziale Policlinico di Bari, Unità Operativa di Pediatria Generale e Specializtica "Federico Vecchio" , P.zza G. Cesare, 11, 70124 Bari , Italy.
authors: Giovanni Carlo
authors: GC
authors: Del Vecchio
authors: g Azienda Ospedaliera Ospedali Riuniti Villa Sofia Cervello , Via Trabucco, 180, 90146 Palermo , Italy.
authors: Aurelio
authors: A
authors: Maggio
authors: h Azienda Ospedaliera "A. Cardarelli" UOS Talassemia Pediatrica e Emoglobinopatie Pediatriche , Via A. Cardarelli, 9, 80131 Napoli , Italy.
authors: Aldo
authors: A
authors: Filosa
authors: i Fondazione "Leonardo Giambrone" per la Guarigione dalla Thalassemia , Via Savona, 56, 81030 Castelvolturno (CE) , Italy.
authors: Angela
authors: A
authors: Iacono
authors: b Consorzio per Valutazioni Biologiche e Farmacologiche , Via L. Porta 14, 27100 Pavia , Italy.
authors: Laura
authors: L
authors: Mangiarini
authors: a Fondazione per la Ricerca Farmacologica Gianni Benzi Onlus , Via Abate Eustasio 30, 70010 Valenzano (BA) , Italy.|b Consorzio per Valutazioni Biologiche e Farmacologiche , Via L. Porta 14, 27100 Pavia , Italy.
authors: Adriana
authors: A
authors: Ceci
mesh_terms: D000328:Adult; D000554:Ambulatory Care Facilities / Q000706:statistics & numerical data*; D001803:Blood Transfusion / Q000706:statistics & numerical data; D015913:Chelation Therapy / Q000379:methods*; D000077543:Deferiprone; D003676:Deferoxamine / Q000627:therapeutic use; D005260:Female; D006801:Humans; D007502:Iron Chelating Agents / Q000627:therapeutic use; D007558:Italy / Q000453:epidemiology; D008297:Male; D011379:Prognosis; D011728:Pyridones / Q000627:therapeutic use; D012042:Registries; D011795:Surveys and Questionnaires; D013789:Thalassemia / Q000453:epidemiology* / Q000628:therapy*
chemical_list: D007502:Iron Chelating Agents; D011728:Pyridones; D000077543:Deferiprone; D003676:Deferoxamine
keywords: Beta-Thalassemia; Chelation; Hemoglobinopathies centers; MRI; Registries; Transfusion