Subject(s)
Anticoagulants/administration & dosage , Atrial Fibrillation/drug therapy , Stroke/epidemiology , Aged , Aged, 80 and over , Anticoagulants/adverse effects , Dabigatran/administration & dosage , Dabigatran/adverse effects , Female , Humans , Italy/epidemiology , Male , Propensity Score , Prospective Studies , Pyrazoles/administration & dosage , Pyrazoles/adverse effects , Pyridones/administration & dosage , Pyridones/adverse effects , Rivaroxaban/administration & dosage , Rivaroxaban/adverse effects , Stroke/prevention & controlABSTRACT
Treatment of patients with inherited bleeding disorders (PWIBD) in the emergency department (ED) is challenging. In 2010, a project was started involving all eight hemophilia centers (HC) and all 44 EDs of the Region of Emilia-Romagna (Italy) to improve emergency care for PWIBD. The project incorporates guidelines for emergency treatment, education for ED staff, and a dedicated Web site providing extensive information, proposing treatments, and sharing data with patients' electronic clinical records. A Web algorithm, accessible to PWIBD as well as ED and HC staff, suggests the first dose of concentrate for each type and severity of bleed or trauma. Following training courses in each ED, the network was activated. During 2012 and 2013, the site was visited 14,000 times, the EDs accessed the Web site 1,739 times, and used the algorithms 206 times. In two reference EDs, triage-assessment and triage-treatment times were reduced in 2013 and 2012 (27/20 and 110/71.5 minutes, respectively) and medical advice from the HC increased (54 vs. 24% cases). The main advantages of this system are better management of patients in ED (shorter triage-to-treatment times) and improved collaboration between HCs and EDs. The most critical point remaining is staff turnover in EDs, necessitating continual training.
Subject(s)
Algorithms , Emergency Medical Services/methods , Emergency Service, Hospital , Hemophilia A , Internet , Medical Records Systems, Computerized , Education, Medical, Continuing , Female , Hemophilia A/diagnosis , Hemophilia A/drug therapy , Humans , Italy , MaleABSTRACT
Although desmopressin (DDAVP) is considered as the treatment of choice for many patients with mild hemophilia A, several aspects of DDAVP therapy remain unclear, including the rate and type of response and the molecular determinants of its clinical efficacy. To investigate these issues, we retrospectively studied all patients with mild hemophilia A followed up at the Parma Hemophilia Center. A total of 75 patients were enrolled who underwent a DDAVP test, and out of whom, 76% (57/75) had a complete or partial response. Response to DDAVP was significantly correlated to the patients' age (median age of responders and nonresponders: 24 and 18 y, respectively; p = 0.04) and type of mutation (all the 10 patients with mutations in the promoter region were nonresponders). The median basal factor VIII (FVIII):C level was significantly lower in responders than in nonresponders (0.14 vs. 0.19 IU/mL, respectively; p = 0.01); this was mainly due to nonresponders with promoter region mutations who had higher basal FVIII:C levels. During the 12-year follow-up, 82 of 237 (35%) bleeding episodes occurring in 27 responder patients were treated with 246 DDAVP infusions with complete or partial efficacy in 92% (75/82). Overall, 142 events were managed with 253 prophylactic DDAVP infusions, which were hemostatically effective in 96% of cases. No severe adverse reactions to DDAVP administration were recorded during the study period. These results document the safety and efficacy of DDAVP as a treatment or prevention of bleeding in patients with mild hemophilia A, also in the context of home treatment, and encourage the more widespread use of this product.
Subject(s)
Deamino Arginine Vasopressin/therapeutic use , Hemophilia A/drug therapy , Adolescent , Adult , Aged , Child , Child, Preschool , Deamino Arginine Vasopressin/adverse effects , Hemophilia A/blood , Hemophilia A/genetics , Humans , Middle Aged , Retrospective Studies , Young AdultABSTRACT
BACKGROUND: The optimal duration of oral anticoagulation in patients with idiopathic venous thromboembolism is uncertain. Testing of D-dimer levels may play a role in the assessment of the need for prolonged anticoagulation. METHODS: We performed D-dimer testing 1 month after the discontinuation of anticoagulation in patients with a first unprovoked proximal deep-vein thrombosis or pulmonary embolism who had received a vitamin K antagonist for at least 3 months. Patients with a normal D-dimer level did not resume anticoagulation, whereas those with an abnormal D-dimer level were randomly assigned either to resume or to discontinue treatment. The study outcome was the composite of recurrent venous thromboembolism and major bleeding during an average follow-up of 1.4 years. RESULTS: The D-dimer assay was abnormal in 223 of 608 patients (36.7%). A total of 18 events occurred among the 120 patients who stopped anticoagulation (15.0%), as compared with 3 events among the 103 patients who resumed anticoagulation (2.9%), for an adjusted hazard ratio of 4.26 (95% confidence interval [CI], 1.23 to 14.6; P=0.02). Thromboembolism recurred in 24 of 385 patients with a normal D-dimer level (6.2%). Among patients who stopped anticoagulation, the adjusted hazard ratio for recurrent thromboembolism among those with an abnormal D-dimer level, as compared with those with a normal D-dimer level, was 2.27 (95% CI, 1.15 to 4.46; P=0.02). CONCLUSIONS: Patients with an abnormal D-dimer level 1 month after the discontinuation of anticoagulation have a significant incidence of recurrent venous thromboembolism, which is reduced by the resumption of anticoagulation. The optimal course of anticoagulation in patients with a normal D-dimer level has not been clearly established. (ClinicalTrials.gov number, NCT00264277 [ClinicalTrials.gov].).
Subject(s)
Anticoagulants/administration & dosage , Fibrin Fibrinogen Degradation Products/analysis , Pulmonary Embolism/drug therapy , Venous Thrombosis/drug therapy , Acenocoumarol/administration & dosage , Adult , Aged , Aged, 80 and over , Antiphospholipid Syndrome/diagnosis , Antithrombins/deficiency , Drug Administration Schedule , Follow-Up Studies , Hemorrhage/chemically induced , Humans , Middle Aged , Proportional Hazards Models , Prospective Studies , Pulmonary Embolism/blood , Pulmonary Embolism/diagnosis , Recurrence , Survival Analysis , Ultrasonography , Venous Thrombosis/blood , Venous Thrombosis/diagnostic imaging , Vitamin K/antagonists & inhibitors , Warfarin/administration & dosageSubject(s)
Coagulants/therapeutic use , Hemarthrosis/prevention & control , Hemophilia A/drug therapy , Adolescent , Adult , Aged , Child , Coagulants/administration & dosage , Coagulants/economics , Cost of Illness , Cost-Benefit Analysis , Drug Administration Schedule , Health Care Costs , Hemarthrosis/economics , Hemarthrosis/etiology , Hemophilia A/complications , Hemophilia A/economics , Humans , Italy , Middle Aged , Retrospective StudiesSubject(s)
Blood Coagulation Disorders, Inherited/complications , Orthopedic Procedures/adverse effects , Thromboembolism/etiology , Adolescent , Adult , Aged , Blood Coagulation Factors/therapeutic use , Child , Female , Humans , Intraoperative Complications , Male , Middle Aged , Perioperative Care , Retrospective Studies , Thromboembolism/prevention & controlABSTRACT
BACKGROUND AND OBJECTIVES: To evaluate the efficacy and safety of the factor VIII/von Willebrand factor concentrate Haemate-P as replacement therapy in patients with von Willebrand's disease (VWD) undergoing surgical or invasive procedures. DESIGN AND METHODS: Between January 1996 and October 2002, 26 patients (12 males and 14 females, median age 41.5 years, range 9-80 years), followed at three Italian Hemophilia Centers (Trento, Verona and Parma), with VWD type 1 (19 cases) and VWD type 2B (7 cases), underwent 43 surgical or invasive procedures: major surgery (14 cases), minor surgery (11 cases), dental extractions (11 cases), invasive diagnostic procedures (7 cases). Replacement therapy with factor VIII/von Willebrand factor concentrate (Haemate-P) was administered in the surgical setting as perioperative prophylaxis against excessive bleeding. RESULTS: The mean total dose (range) of Haemate-P used for major surgery was 284.1 IU VWF:RCo/kg (range 125.0-976.4), for minor surgery it was 120.8 IU VWF:RCo/kg (range 42.9-173.3), for dental extractions it was 38.4 IU VWF:RCo/kg (range 23.5-100.0) and for invasive procedures it was 87.3 VWF:RCo/kg (range 27.3-160.0). We recorded one bleeding episode 3 days after multiple dental extractions in a patient with severe periodontal disease; this bleeding was controlled with 2 further administrations of concentrate. We did not observe thrombotic episodes or other side effects following infusion of the concentrate. INTERPRETATION AND CONCLUSIONS: In conclusion, Haemate-P was effective and safe in preventing excessive bleeding after major and minor surgery or invasive procedures in VWD patients.
