1,721,007 research outputs found
Albumin-Fusion Recombinant FIX in the Management of People with Hemophilia B: An Evidence-Based Review
Full text linkAlbutrepenonacog-alfa (Idelvion(®), CSL Behring) is a recombinant fusion protein in which the recombinant FIX (rFIX) links a recombinant human albumin, extending the half-life of rFIX even beyond 100 hours. In 2016, this drug was approved worldwide for the treatment of pediatric and adult persons with hemophilia B (PWH-B). Its efficacy and safety were described in the PROLONG-9FP program and subsequently confirmed in the real-world practice, even if to date there are not many manuscripts that extensively and completely deal with the use of albutrepenonacog-alfa in daily practice, also evaluating its impact on the quality of life of patients treated with this drug; this review therefore aims to analyze all the publications currently available regarding the real-world use of this extended half-life concentrate, also noting which topics need further study and research
Very high risk of intracranial hemorrhage and severe outcomes in adult patients with mild hemophilia: Sub-analysis of the EMO.REC Registry
No full textComplex dental extractions in a patient with severe haemophilia A and inhibitors treated with activated prothrombin complex concentrate
No full textRegenerative Surgery with Dental Implant Rehabilitation in a Haemophiliac Patient
Full text linkThis study aimed to describe the first case of regenerative surgery in haemophiliac implant. Patients with haemophilia often present dental problems. A multidisciplinary approach is suggested in case of dental surgeries to reduce the high bleeding risk. A 41-year-old male patient with mild haemophilia A (FVIII 8.4%), presenting previous epistaxis, noncomplicated tooth extractions and traumatic haemartroses, all treated with single infusions of coagulation factor concentrates, was referred to the dental clinic of the Padua University Hospital based on the recommendation of his attending dentist. At first dental visit the patient reported intense pain in the right lower second molar, with impaired chewing function. After an endodontic unsuccessful treatment the element was judged as no longer recoverable. In agreement with the patient the dental element was then extracted, after a combined administration of recombinant factor VIII 3000 IU (35 IU/kg), and tranexamic acid 1,000 mg. The extraction was performed under local anaesthesia, paraperiosteal and truncular, moderate sedation, elevation of an envelope flap. After extraction, a preservation of the alveolus was carried out with bovine matrix bone graft covered with a resorbable membrane. Three months after the surgery a flapless implant was placed after a single infusion of factor VIII 2000 IU, tranexamic acid 1,000 mg, and a local para-periostal anaesthesia, without any complication. Oral surgeon and haematologist expert in coagulation diseases must therefore collaborate together to define a shared protocol for managing surgery in those patients
Real-Life Population Pharmacokinetics of Recombinant Factor XIII and Dosing Considerations for Preventing the Risk of Bleeding in Patients with FXIII Congenital Deficiency
No full textRecombinant factor XIII (rFXIII) at the recommended dosage of 35 IU/kg every 4 weeks is currently used for prophylaxis of bleeding in patients affected by FXIII deficiency. The aim of this study was to describe the population pharmacokinetics of rFXIII in patients with FXIII deficiency being treated with rFXIII in real-life and to assess, using Monte Carlo simulations, the attainment of defined FXIII concentration thresholds associated with prevention of the risk of bleeding over time
Haemophilia and Cancer: A Literature Review
Full text linkBackground: Opinions in the literature on the impact of cancer on patients with haemophilia are contradictory. There is a lack of data on the clinical presentation and management of cancer in patients with haemophilia (PWH). Methods: Papers were found following a comprehensive search in PubMed, Google Scholar, and Scopus using the terms “cancer” and “haemophilia” without time limits and using the English language as a filter. The references from all the retrieved original articles and reviews were assessed for additional relevant articles. Results: The emergence of malignancies is one of the important causes of morbidity and mortality in PWH. In the past decade, the literature mainly focused on the epidemiology and outcome of blood-borne cancers in the haemophilia patient group, as the incidence of hepatitis B virus (HBV), hepatitis C (HCV), and HIV infection were high among them. However, with the introduction of recombinant clotting factor concentrates (CFCs), physicians now pay attention to non-virus-related malignancies. Bleeding and thrombotic complications are important causes of morbidity and mortality in critically ill patients with cancer; replacement therapy with factor VIII or IX or others should be maintained during antitumour treatment. Conclusion: Overall, managing cancer in patients with haemophilia requires careful evaluation and individualised planning involving a multidisciplinary team of physicians experienced in haematology, oncology, and surgery
The sudden and unexpected appearance of inhibitors in a previously treated severe haemophilia B patient after the switch to albutrepenonacog alpha
No full textFlapless Dental Implant Surgery in Bleeding Disorders
Full text linkHemostasis disorders require particular attention in dental treatment. Dental implants are a very widespread and valid method for total rehabilitation. Flapless dental implant surgery is a minimally invasive treatment that allows the implants to be placed in the jaw bones with minimal surgical trauma. The aim of this study is to report the bleeding incidence in a group of patients with bleeding disorders treated with flapless implants. A total of 52 patients with bleeding disorders (46 in anticoagulant therapy; 4 with hemophilia; 2 with von Willebrandt disease) were treated with 188 flapless implant surgeries Anticoagulants were not discontinued. Patients with hemophilia and VWD were treated following specific protocols. Four late, easy to treat bleeding complications were reported (three mild bleeding, one ecchymosis). No additional sutures or other hemostatic measures were taken, no further infusions or transfusions were reported, and no severe bleeding complications requiring more than easy on-chair treatment, were reported. In conclusion, with adequate knowledge of the procedure and the pathology, dental implantology can be safely performed in patients with bleeding disorders
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