Inherited bleeding disorders: results from the Italian Regional Haemophilia Centre of Pescara.
BACKGROUND
Inherited bleeding disorders registries can be useful to improve health care of these rare disorders and document their natural history.


METHODS
We analysed the epidemiological, diagnostic and therapeutic aspects of patients managed at an Italian Regional Haemophilia Centre, based in Pescara (in the Region of Abruzzo).


RESULTS
This Regional Haemophilia Centre currently follows 376 patients: 248 with rare clotting factor defects, 33 with von Willebrand's disease, 75 with haemophilia A and 20 with haemophilia B. Three patients with severe haemophilia A have developed inhibitors. Among all the haemophiliacs, the prevalence of hepatitis C virus infection is 21% while the prevalence of human immunodeficiency virus infection is 5.3%. Among the whole haemophilic population referring to the Pescara Haemophilia Centre, 87.4% are treated with recombinant factors while 12 patients with severe haemophilia are receiving primary prophylaxis.


CONCLUSIONS
In brief, an analysis of the epidemiological, clinical and therapeutic data collected at the Regional Haemophilia Centre of Pescara is a useful tool for monitoring and continuously improving the quality of care of patients with inherited bleeding disorders.
Dragani
Alfredo
A
Dipartimento di Ematologia, Centro Regionale delle Malattie Rare del Sangue e degli Organi Emopoietici, Ospedale Santo Spirito, Pescara, Italy. Alfredo.dragani@tin.it
Malizia
Raniero
R
Iuliani
Ornella
O
Di Marzio
Ilaria
I
Davì
Giovanni
G
D000293:Adolescent; D000328:Adult; D025861:Blood Coagulation Disorders, Inherited / Q000453:epidemiology* / Q000235:genetics / Q000628:therapy; D016913:Blood Component Transfusion / Q000706:statistics & numerical data; D002648:Child; D002675:Child, Preschool; D020022:Genetic Predisposition to Disease / Q000235:genetics; D006801:Humans; D007223:Infant; D007558:Italy / Q000453:epidemiology; D008875:Middle Aged; D020641:Polymorphism, Single Nucleotide / Q000235:genetics; D012042:Registries