The Hemophilia Registry of the Italian Association of Hemophilia centres

Introduction: National Hemophilia registries were found to be powerful instruments to support health care and research. A National Registry was mantained in Italy by the Ministry of Health (ISS) until 1999. In 2003 the Italian Association of Hemophilia Centres (AICE) started a new program aimed to build up a National Registry of congenital coagulation diseases. Methods: The AICE identified an expert panel to steer the registry program. A computer software to assist patient care management was developed, and all the AICE Hemophilia Centres 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. Draft of local and national reports are submitted to a validation step by all Hemophilia Centres Directors through a web-based procedure. The Registry covers sociodemographic, clinical, laboratoristic and treatment data. A subset of data is shared with the ISS. Results: Overall, data were collected six times by 45/51 Hemophilia Centres; 34 centres updated their patients' records up to June 2006. The database contains 6183 records (347 duplicates), 380 of which relating to dead patients. Database growth and missing data clearance showed a constantly positive trend over time. The database collects records of the following alive patients: Hemophilia A: 1368 severe (mean age = 31.25, range 0.75 – 83.08), 385 moderate and 810 mild; Hemophilia B: 207 severe (mean age = 28.08 range 1.83 – 74.83), 128 moderate and 169 mild; vWD: 1023 type 1, 315 type 2 and 92 type 3; Factor VII deficiency 187, other rare deficiencies 531. Median age at diagnosis was 2 year for severe and 16 year for mild hemophilia patients. Inhibitor patients were 213 (107 high responder, of which 34 undergoing immunotolerance treatment and 48 low responder, of which 40 transient). Conclusions: The AICE runs a National Registry intended to become a powerful tool for policy making and epidemiological research.