21 research outputs found
Inherited bleeding disorders in pregnancy: rare coagulation factor defects
No full textInherited deficiencies of plasma proteins involved in blood coagulation generally lead to lifelong bleeding disorders. Rare bleeding disorders (RBDs), discussed in this chapter, represent 3–5 % of all the inherited coagulation deficiencies, with prevalence ranging from approximately 1:500,000 to 1:2,000,000 in the general population. Patients affected with bleeding disorders present a wide spectrum of clinical symptoms that vary from a mild or moderate bleeding tendency to severe episodes. Women with inherited bleeding disorders are particularly disadvantaged since, in addition to suffering from general bleeding symptoms, they are also at risk of bleeding complications from regular haemostatic challenges: menstruation, pregnancy and childbirth. Moreover, affected women may experience reduced quality of life caused by limitations in activities and work, and the impact on their reproductive life. Management of women with RBDs is difficult because of considerable inter-individual variation. Furthermore, reliable information on clinical management is scarce, with only a few long-term prospective studies of large cohorts providing evidence to guide diagnosis and treatment
Inherited bleeding disorders in pregnancy : rare coagulation factor defects
No full textInherited deficiencies of plasma proteins involved in blood coagulation generally lead to lifelong bleeding disorders. Rare bleeding disorders (RBDs), discussed in this chapter, represent 3–5 % of all the inherited coagulation deficiencies, with prevalence ranging from approximately 1:500,000 to 1:2,000,000 in the general population. Patients affected with bleeding disorders present a wide spectrum of clinical symptoms that vary from a mild or moderate bleeding tendency to severe episodes. Women with inherited bleeding disorders are particularly disadvantaged since, in addition to suffering from general bleeding symptoms, they are also at risk of bleeding complications from regular haemostatic challenges: menstruation, pregnancy and childbirth. Moreover, affected women may experience reduced quality of life caused by limitations in activities and work, and the impact on their reproductive life. Management of women with RBDs is difficult because of considerable inter-individual variation. Furthermore, reliable information on clinical management is scarce, with only a few long-term prospective studies of large cohorts providing evidence to guide diagnosis and treatment
Clinical efficacy and safety of DDAVP with or without tranexamic acid in inherited VWD: final results of the prospective and international study on 229 patients
No full text16. The real-life use of rFXIII (catridecacog) in patients with severe Factor XIII deficiency: an Italian survey.
No full textAn Italian Survey on the real-world use of rFXIII (catridecacog) in patients with factor XIII deficiency
No full textCancers in patients with von Willebrand disease: a survey from the Italian association of hemophilia centres.
No full textCatridecacog in the treatment of an Italian population of patients with FXIII deficiency: from pharmacokinetics to clinical outcomes (The Italian rFXIII study)
No full textCancers in patients with von Willebrand disease: a survey from the Italian association of hemophilia centres
No full textPopulation Parmacokinetics of rFXIII (catridecacog) and clinical outcomes of prophylaxis in an Italian population of patients with Factor XIII deficiency
No full textCancers in Patients with von Willebrand Disease: A Survey from the Italian Association of Haemophilia Centres
No full textBesides its essential role in hemostasis, there is growing evidence that von Willebrand factor (VWF) has an additional antitumor effect. To elucidate the clinical significance of this biological activity we conducted a retrospective study on cancers among Italian patients with von Willebrand disease (VWD) on behalf of the Italian Association of Haemophilia Centres (AICE). A questionnaire to collect demographic, clinical, and treatment data of VWD patients with cancer was sent to all the 54 Italian Haemophilia Treatment Centres (HTCs) members of AICE. Overall, 18 HTCs (33%) provided information on 92 VWD patients (61 alive and 31 deceased) with 106 cancers collected during the period 1981 to 2014. Of them, 19 (18%) were hematological cancers and 87 (82%) were solid cancers. A total of 61% of patients had type 1, 36% type 2 (12% type 2A, 14% type 2B, 9% type 2M, and 1% type 2N), and 3% type 3 VWD: this distribution was significantly different from that observed in the whole VWD population (79% type 1, 16% type 2 [8% type 2A, 4% type 2B, 2% type 2M, 2% type 2N], and 5% type 3; type 2 vs. non-type 2: p < 0.001). Overall, VWD patients with cancer underwent 52 invasive and 72 surgical procedures, were treated with VWF/factor VIII (FVIII) concentrates in 77 cases, with desmopressin (DDAVP) alone in 24 cases and with DDAVP and VWF/FVIII concentrates in 7 cases. Hemorrhagic complications were observed only rarely (2% of invasive procedures and radiotherapy and 6% of surgical interventions). The data collected by this survey document that a substantial number of cancers are recorded among VWD patients and that these patients are safely managed by HTC physicians through a multidisciplinary approach
