Lo Studio “Emorragia cerebrale nell’emofilia A, B e negli altri difetti rari congeniti della coagulazione: epidemilogia e terapia” è uno studio osservazionale retrospettivo condotto dal Dott. Ezio Zanon in collaborazione con i Centri Emofilia AICE. Lo studio si è concluso con la pubblicazione dei risultati sulla rivista scientifica Haemophilia

Successivamente si è dato inizio ad uno Studio multicentrico nazionale osservazionale, non interventistico, retrospettivo, prospettico denominato EMOREC (VAI ALLO STUDIO IN CORSO)

Zanon

Dott. Ezio Zanon

Centro Emofilia
Azienda Ospedaliera di Padova

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CRF-studio ICH

Intracranial haemorrhage in the Italian population of haemophilia patients with and without inhibitors

E. ZANON, A. IORIO, A. ROCINO, A. ARTONI, R. SANTORO, A. TAGLIAFERRI,
A. COPPOLA, G. CASTAMAN, P. M. MANNUCCI, and THE ITALIAN ASSOCIATION OF HEMOPHILIA CENTERS

Intracranial haemorrhage (ICH) is the most serious bleeding symptom in haemophiliacs, resulting in high rates of mortality and disabling sequelae. The Association of Italian Haemophilia Centres carried out a retrospective survey (1987–2008) of ICH occurring in haemophiliacs with the goals to establish: (i) incidence, location of bleeding, death rate and disabling sequels; (ii) risk factors for ICH; and (iii) treatment used during the acute phase of ICH and for recurrence prevention. A total of 112 ICH episodes had occurred in 88 patients (78 haemophilia A, 10 haemophilia B), 24 of whom experienced recurrences. The cumulative hazard of ICH for the whole cohort over the entire follow-up period was 26.7 per 1000 patients, and the annualized rate of ICH was 2.50 events per 1000 patients (95% CI 1.90– 3.31). The risk of ICH was higher in the youngest children (24.4 per 1000, 95% CI 12.7–47.0 in the first year of age and 14.9, 95% CI 7.1–31.4 in the second year of age) and then progressively rose again after the age of 40. Univariate, bivariate (age-adjusted) and multivariate analysis investigating the effects of patient characteristics on ICH occurrence showed that haemo- philia severity and inhibitor status were strongly asso- ciated with ICH [severe vs. mild, HR 3.96 (2.39–6.57); inhibitor vs. non-inhibitor 2.52 (1.46–4.35)]. HCV infection was also associated with the risk of ICH [HR 1.83 (1.25–2.69)]. Therapeutic suggestions based upon our experience to control ICH recurrence are provided.

Full paper published in Haemophilia (2011), 1-7
© 2011 Blackwell Publishing Ltd