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COLLEGIO DI DIREZIONE - Azienda Ospedaliera di Parma

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were tested by using the Pearson correlation coefficient. Adjusted multiple logistic regression models were applied to<br />

assess the relationships between mCAC, FEV1, MLA, and emphysema extent and all-cause mortality and CVEs.<br />

Results: The final study cohort consisted of 1159 smokers. There were no significant correlations between mCAC score<br />

and FEV1 (r = -0.03, P = .4), MLA (r = -0.01, P = .7), or emphysema extent (r = 0.02, P = .6). An mCAC score greater<br />

than 400 was the only factor that was independently associated with both all-cause mortality (odds ratio [OR]: 3.73;<br />

95% confidence interval [CI]: 1.05, 13.32; P = .04) and CVEs (OR: 2.87; 95% CI: 1.13, 7.27; P = .03). Conclusion:<br />

mCAC is a better pre<strong>di</strong>ctor of CVE and all-cause mortality than FEV1 and emphysema extent and may contribute to the<br />

identification of high-risk in<strong>di</strong>viduals in a lung cancer screening setting.<br />

79) Tagliaferri, A; Di Perna, C; Riccar<strong>di</strong>, F; Pattacini, C; Rivolta, GF; Franchini, M (2012) The natural<br />

history of mild haemophilia: a 30-year single centre experience HAEMOPHILIA 18(2):166-174 IF=2.364<br />

[Article]<br />

Although up to 50% of all haemophilic patients followed at haemophilia treatment centres (HTCs) are affected by a<br />

mild factor VIII (FVIII) or factor IX (FIX) defect, published data regar<strong>di</strong>ng the natural history of these <strong>di</strong>sorders are<br />

scarce. To fill this lack of information, a retrospective single centre study was conducted. All cases with mild<br />

haemophilia (75 A and 7 B) followed at the regional reference HTC of <strong>Parma</strong> were evaluated. The patients me<strong>di</strong>an age<br />

at <strong>di</strong>agnosis was 11.5 years and their me<strong>di</strong>an age at first blee<strong>di</strong>ng was 5.5 years; 95% of patients had a history of<br />

haemorrhagic problems during their life. Twenty-three percent of patients were infected by HCV, and none by HIV.<br />

Genetic analysis was performed in 80 patients (97% haemophilia A and 100% haemophilia B) and 21 <strong>di</strong>fferent<br />

mutations were characterized. Eleven percent of patients had never received treatment, whereas 67% were treated with<br />

plasma-derived or recombinant FVIII/FIX concentrates (4% developed inhibitors). desmopressin (DDAVP) was used in<br />

80% of the haemophilia A patients. The response to DDAVP was closely related to the patients genetic profile, as 60%<br />

of non-responders had a mutation in the F8 promoter region. Patients with mild haemophilia may experience a variety<br />

of me<strong>di</strong>cal problems, sometimes challenging for the physicians, during their lifetime. The HTCs play an important role<br />

in the management of these patients, whose <strong>di</strong>agnosis is often delayed. The HTCs should improve patients knowledge<br />

and consideration of their <strong>di</strong>sease and encourage them to maintain regular contact with their haemophilia care provider.<br />

80) Tagliaferri, A; Di Perna, C; Santoro, C; Schinco, P; Santoro, R; Rossetti, G; Coppola, A; Morfini, M;<br />

Franchini, M (2012) Cancers in patients with hemophilia: a retrospective study from the Italian Association<br />

of Hemophilia Centers JOURNAL OF THROMBOSIS AND HAEMOSTASIS 10(1):90-95 IF=5.439<br />

[Article]<br />

. Background: The increased life expectancy of the hemophilia population, primarily as a result of advances in factor<br />

replacement therapy, has enabled hemophiliacs to reach an older age. Consequently, age-related <strong>di</strong>seases, such as<br />

car<strong>di</strong>ovascular <strong>di</strong>sorders and cancers, are being increasingly recognized in such patients. However, only few data are<br />

available on such co-morbi<strong>di</strong>ties, their management and impact on the primary blee<strong>di</strong>ng <strong>di</strong>sorders. Objectives: With the<br />

aim of investigating several still unclear issues regar<strong>di</strong>ng cancers in hemophilia patients, we conducted, on behalf the<br />

Italian Association of Hemophilia Centers (AICE), a study on cancers among Italian hemophiliacs. Patients: Data<br />

pertaining to 122 hemophiliacs with 127 cancers between 1980 and 2010 were retrospectively collected in 21 centers of<br />

the AICE which chose to participate. Results: Sixty-nine percent of cancers were recorded during the decade 20012010.

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