Models for institutional and professional accreditation of haemophilia centres in Italy.

The Health Commission of the Conference between the Italian State and Regions recognized the need to establish an institutional accreditation model for Haemophilia Centres (HCs) to be implemented by 21 Regions in order to provide patients with haemophilia and allied inherited coagulations disorders with high and uniform standards of care. The Italian National Blood Centre, on behalf of the Commission, convened a panel of clinicians, patients, experts, representatives from Regions and Ministry of Health. The agreed methodology included: systematic literature review and best practice collection, analysis of provisions and regulations of currently available services, priority setting, definition of principles and criteria for the development of recommendations on the optimal requirements for HCs.

Blood transfusion practice: a nationwide survey in Italy.

Background: Blood transfusion is a complex activity. Some of the components of this activity are implementation of standard procedures, evaluation of appropriateness of the blood use, methods for bedside identification of patients and the release of blood in emergencies, during out-of-routine hours and from hospitals lacking a Blood Centre. An overview about how these issues are managed in Italy could be of interest.

High-resolution analysis of von Willebrand factor multimeric composition defines a new variant of type I von Willebrand disease with aberrant structure but presence of all size multimers (type IC).

In Type I von Willebrand disease, the whole series of von Willebrand factor (vWF) multimers is present in plasma, but all are decreased in quantity. No structural abnormality of individual multimers has been demonstrated so far in these patients. We now describe five individuals, from two unrelated families, who had this form of the disease and in whom the complex banding pattern of each vWF multimer was markedly abnormal.

Efficacy of porcine factor VIII in the management of haemophiliacs with inhibitors.

We report our experience with polyelectrolyte-fractionated highly purified porcine factor VIII concentrate (Hyate:C). Porcine factor VIII concentrate was used to treat 14 haemorrhagic episodes including seven severe haemarthroses, three severe haematomata, two episodes of oral bleeding, one traumatic and the other after multiple dental extractions and two life-threatening intestinal haemorrhages. Altogether 60 infusions of Hyate:C were given to five haemophiliacs with inhibitors.

Evaluation of the effectiveness of DDAVP in surgery and in bleeding episodes in haemophilia and von Willebrand's disease. A study on 43 patients.

1-deamino-8-D-arginine-vasopressin (DDAVP), was used in a wide spectrum of clinical situations employing two different dosages (0.3 and 0.4 microgram/kg b.