Iron-dependent erythropoiesis in women with excessive menstrual blood losses and women with normal menses

In women of fertile age, iron loss consequent to excessive menstrual discharge is by far the most frequent cause of iron-deficient anemia. However, the relationship between menstrual discharge and iron loss is poorly understood. In this prospective study, total menstrual and iron losses were assayed in a large cohort of non-anemic women and women with excessive menstrual blood losses (menorrhagia) in order to provide data useful for intervention. One hundred and five Caucasian women aged 20–45 years were recruited. Blood cell count and serum ferritin (SF) levels were determined in each case before menses. Menstrual fluid losses (MFL) were determined using a standardized pads’ weight method.…

Residual vein thrombosis to establish duration of anticoagulation after a first episode of deep vein thrombosis: the Duration of Anticoagulation based on Compression UltraSonography (DACUS) study

Abstract Residual vein thrombosis (RVT) indicates a prothrombotic state and is useful for evaluating the optimal duration of oral anticoagulant treatment (OAT). Patients with a first episode of deep vein thrombosis, treated with OAT for 3 months, were managed according to RVT findings. Those with RVT were randomized to either stop or continue anticoagulants for 9 additional months, whereas in those without RVT, OAT was stopped. Outcomes were recurrent venous thromboembolism and/or major bleeding. Residual thrombosis was detected in 180 (69.8%) of 258 patients; recurrent events occurred in 27.2% of those who discontinued (25/92; 15.2% person-years) and 19.3% of those who continued OAT (17/88…

Prophylaxis In Congenital Factor VII Deficiency, Indications, Efficacy and Safety: Results of the STER

Abstract Abstract 665 Introduction Prophylaxis is considered a difficult endeavour in FVII deficiency, especially because of the very short FVII zymogen and FVIIa half-lives, mainly in childhood. The Seven Treatment Evaluation Registry (STER, www.targetseven.org) is a prospective observational, multi-centre, web-based registry concerned with the evaluation of treatments for spontaneous bleeding episodes, surgery and prophylaxis in patients with FVII deficiency. As regards prophylaxis, STER provides the frame for a structured and detailed data capture aiming at: a) identifying patients in whom prophylaxis is advisable, b) defining clinical settings in which prophylaxis is necessary and c) de…

Congenital Factor VII Deficiency

Women with congenital factor VII deficiency: clinical phenotype and treatment options from two international studies

Introduction A paucity of data exists on the incidence, diagnosis and treatment of bleeding in women with inherited factor VII (FVII) deficiency. Aim Here we report results of a comprehensive analysis from two international registries of patients with inherited FVII deficiency, depicting the clinical picture of this disorder in women and describing any gender-related differences. Methods A comprehensive analysis of two fully compatible, international registries of patients with inherited FVII deficiency (International Registry of Factor VII deficiency, IRF7; Seven Treatment Evaluation Registry, STER) was performed. Results In our cohort (N = 449; 215 male, 234 female), the higher prevalence…

Buffy coat-derived platelets cryopreserved using a new method: Results from in vitro studies

Abstract Cryopreservation for the long-term storage of platelets (PLTs) is a useful method to overcome the limits of platelet shortage. This is an in vitro prospective study to evaluate the count, viability, and function of buffy coat-derived pooled platelet concentrates (BC-PLTs), treated with dimethyl sulphoxide (DMSO) and cryopreserved (CRY BC-PLTs) at −80 °C with a modified Valeri method. PLTs were stored in 6% DMSO with a patented kit. Overall, 49 BC-PLTs from 245 healthy volunteer donors were prepared, cryopreserved, and analysed before and after 3, 6, and 9 months of storage. In flow cytometry, a statistically significant reduction in CD 42b (92.7 ± 4.29% at T0 vs. 23.6 ± 27.5% at T3…

Replacement Therapy for Surgery in FVII Deficiency: The Ster Experience Concerning 63 Interventions.

Abstract Abstract 1290 Poster Board I-312 Background Excessive bleeding represents a major complication of surgical interventions, and its control is especially relevant in patients with Congenital Bleeding Disorders. In FVII deficiency, scanty data is available in surgery to guide treatment strategies. Methods The STER (Seven Treatment Evaluation Registry) is a multi-centre, prospective, observational, web-based registry providing the frame for an extensive and structured data collection. Results As of Dec. 2008, sixty-three surgical operations (36 “major” and 27 “minor”) were performed in 54 subjects (29 females and 25 males) with a FVII deficiency (31 previously symptomatic; 9 with FVIIc…

Management of the Sponataneous Bleeding Episodes in Factor VII Deficiency. A Prospective Evaluation of the STER,

Abstract Abstract 3368 Introduction Patients with an inherited factor VII (FVII) deficiency may display a wide range of clinical phenotypes, from an asymptomatic condition to serious hemorrhagic episodes such as fatal central nervous system (CNS) or gastrointestinal (GI) bleeds (Mariani G et al. Thromb Haemost 2005; 93: 481–7). Symptomatic patients can be divided into two major categories: those with mild-to-moderate bleeding tendency and individuals with a severe bleeding tendency which may be more severe than that in hemophilia. The former group mainly experience mucosal bleeding, a clinical picture that mimics that of a platelet disorder and often does not call for treatment. In contrast…

Absolute lymphocyte count is unrelated to overall survival in newly diagnosed elderly patients with multiple myeloma treated with immunomodulatory drugs

The absolute lymphocyte count (ALC) has been widely studied in hematologic and solid malignancies as a marker of host antitumor immunity. Its significance has been evaluated in multiple myeloma (MM...

Replacement therapy for bleeding episodes in factor VII deficiency: A prospective evaluation

Patients with inherited factor VII (FVII) deficiency display different clinical phenotypes requiring ad hoc management. This study evaluated treatments for spontaneous and traumatic bleeding using data from the Seven Treatment Evaluation Registry (STER). One-hundred one bleeds were analysed in 75 patients (41 females; FVII coagulant activity <1-20%). Bleeds were grouped as haemarthroses (n=30), muscle/subcutaneous haematomas (n=16), epistaxis (n=12), gum bleeding (n=13), menorrhagia (n=16), central nervous system (CNS; n=9), gastrointestinal (GI; n=2) and other (n=3). Of 93 evaluable episodes, 76 were treated with recombinant, activated FVII (rFVIIa), eight with fresh frozen plasma (FFP), s…

Adverse Events in Treatment of Inherited Factor VII Deficiency: Final Analysis of the STER

Abstract Abstract 2279 Introduction No evidence-based guidelines are available for the treatment of Factor VII deficiency. Replacement therapy (RT) is still influenced by different factors as rarity of the disorder, availability and supply of products and economic reasons. All RTs are not exempt of side effects and scanty data is available about the safety of the products currently used. Aim of this study was to analyze adverse events (AEs) of RTs for congenital Factor VII deficiency, as reported in Seven Treatment Evaluation Registry (STER). Design and Methods FVII deficiency patients treated for bleeding episodes, prophylaxis, and surgery were investigated for RT-related AEs over a period…

Prophylaxis in congenital factor VII deficiency: indications, efficacy and safety. Results from the Seven Treatment Evaluation Registry (STER)

WOS: 000319897700018

Impact of psychological risk factors on disaster rescue operations: the case of Italian volunteers.

This paper reports on research into the characteristics of emergency rescue workers, with a focus on psychological distress. We present the results of a study of volunteers from the Italian national civil protection programme. A questionnaire was administered to 2,576 subjects from all over Italy. Most respondents were men, mostly unemployed, whose average level of education was higher than that among most Italian volunteers. Many were poorly endowed with basic skills, with large differences according to geography. Many expected to convert their volunteer activities into permanent jobs. Using this data, we employed a logistical regression model to analyse the risk of low, moderate and sever…

Role of clinical and laboratory parameters for treatment choice in patients with inherited FVII deficiency undergoing surgical procedures: evidence from the STER registry

Perioperative bleeding is a major concern in patients with factor VII (FVII) deficiency. Evaluating data of 95 FVII-deficient patients undergoing 110 surgical procedures (61 major, 49 minor), we assessed the impact of type of surgery, bleeding phenotype and FVII coagulant activity (FVII:C) levels on perioperative replacement therapy (RT). Compared to those with higher FVII:C levels, patients with &lt;3% FVII:C received a higher number of RT doses (8 vs. 2, P = 0·003) for a longer RT duration (3 days vs. 1 day, P = 0·001), with no difference in RT dose. Similarly, patients with a history of major bleeds received a higher number of RT doses (8·5 vs. 2-3, P = 0·013) for a longer RT duration (2…

Buffy coat-derived platelets cryopreserved using a new method: Results from a pivotal clinical trial on thrombocytopenic patients with acute leukaemia

Abstract The administration of cryopreserved platelets (PLTs) may overcome the limits of platelet shortage and availability, especially during some seasons or in specific contexts like rural areas. After in vitro validation studies, ad hoc prepared buffy coat-derived pooled platelet concentrates (BC-PLTs), treated with dimethyl sulphoxide (DMSO) and cryopreserved (CRY BC-PLTs) at -80 °C with a modified Valeri method, were transfused in patients with severe thrombocytopenia secondary to chemotherapy for acute leukaemia (AL). Five inpatients were enrolled in the pivotal clinical trial NCT02032134: 4 males and 1 female with a mean age of 71 years (range: 65–80). Four patients were diagnosed wi…

THE ESTIMATION OF THE COSTS OF INSULARITY THROUGH A REGRESSIVE ECONOMETRIC MODEL APPLIED TO SICILY

The geographic nature of the islands determines a peculiar condition of peripherality and specific accessibility problems often associated with the presence of structural delays in development processes. Data on the gaps due to insularity, as the case of Sicily clearly shows, returns an alarming picture that highlights employment imbalances, high poverty, high costs for transport, widespread margins, reduced internationalization and a decisive infrastructural inequality. In this work we have tried to offer a contribution to the meager debate on insularity, starting from the measurement of the disadvantage of insularity in economic terms. For Sicily, an estimate of the costs related to the i…

Replacement therapy in inherited factor VII deficiency: occurrence of adverse events and relation with surgery

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Recombinant, activated factor VII for surgery in factor VII deficiency: a prospective evaluation - the surgical STER

Excessive bleeding represents a major complication of surgical interventions and its control is especially relevant in patients with Congenital Bleeding Disorders (CBD). In factor VII (FVII) deficiency, scanty data on surgery is available to guide treatment strategies. The STER (Seven Treatment Evaluation Registry) is a multi-centre, prospective, observational, web-based study protocol providing the frame for a structured and detailed data collection. Inhibitor occurrence was checked in a centralized fashion. Forty-one surgical operations (24 'major' and 17 'minor') were performed in 34 subjects with a carefully characterized FVII deficiency under the coverage of recombinant activated Facto…