Adherence to treatment regimen and bleeding rates in a prospective cohort of youth and young adults on low-dose daily prophylaxis for severe hemophilia A

ConclusionsThis prospective longitudinal study in youth and young adults shows that LDDP may be associated with low ABRs, adequate adherence and HRQoL comparable to previously reported.
Source: BMC Hematology - Category: Hematology Source Type: research

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April 01, 2020 -- The U.S. Food and Drug Administration today approved Sevenfact [coagulation factor VIIa (recombinant)-jncw] for the treatment and control of bleeding episodes occurring in adults and adolescents 12 years of age and older with...
Source: - New Drug Approvals - Category: Drugs & Pharmacology Source Type: news
Conclusion: Fascial therapy does not appear to produce elbow hemarthrosis in patients with hemophilia. This treatment can improve joint pain, range of motion, and elbow status in patients with hemophilia. PMID: 32223585 [PubMed - as supplied by publisher]
Source: Physiotherapy Theory and Practice - Category: Physiotherapy Authors: Tags: Physiother Theory Pract Source Type: research
Conclusions: Low self-esteem in hemophilia patients indicates the importance of lifelong psychosocial support. Patients with pathologic HJHS are at risk of low-esteem. Using OSIQ with HJHS during follow-up of hemophilia patients may be useful for management.
Source: Journal of Pediatric Hematology Oncology - Category: Hematology Tags: Online Articles: Original Articles Source Type: research
Abstract Von Willebrand factor (VWF) and coagulation factor VIII (FVIII) circulate as a complex in plasma and have a major role in the hemostatic system. VWF has a dual role in hemostasis. It promotes platelet adhesion by anchoring the platelets to the subendothelial matrix of damaged vessels and it protects FVIII from proteolytic degradation. Moreover, VWF is an acute phase protein that has multiple roles in vascular inflammation and is massively secreted from Weibel-Palade bodies upon endothelial cell activation. Activated FVIII on the other hand, together with coagulation factor IX forms the tenase complex, an ...
Source: Sub-Cellular Biochemistry - Category: Biochemistry Authors: Tags: Subcell Biochem Source Type: research
CONCLUSIONS: after 12 weeks of observation, both approaches significantly reduced bleeds per animal and increased the proportion of bleed-free animals compared to controls (43% vs. 0%, respectively [AAV]; 75% vs. 8%, respectively [injection]). Both approaches resulted in an anti-FVIII inhibitory response in 20-37% of treated animals, similar to HA patients. Inhibitory antibodies were refractory to clinical improvement (reduction of bleeds) only in the AAV-based prophylaxis. An integrated model-based analysis of data on FVIII exposure and bleeding events was performed. This predicted the bleeding risk at any given circulati...
Source: Thrombosis and Haemostasis - Category: Hematology Authors: Tags: J Thromb Haemost Source Type: research
"Whatever Works": Innovations in the Treatment of Hemophilia in the United States 1783-1950. Can Bull Med Hist. 2020 Mar 20;:e386092019 Authors: Rushton AR Abstract Treatment of the bleeding disorder hemophilia in the nineteenth century was empirical, based on clinical experience. Medications, transfusions of human or animal blood, and injections of blood sera were utilized in an attempt to halt life-threatening hemorrhages. After 1900, the application of clinical laboratory science facilitated the utilization of anti-coagulated blood and donor blood compatibility tests for safer emergency t...
Source: Medical History - Category: History of Medicine Authors: Tags: Can Bull Med Hist Source Type: research
CONCLUSIONS: Not all of the seven included trials analysed the effects of the interventions on our primary outcomes (mood and personal well-being, coping strategies and QoL). Three trials were conducted in the 1970s and 1980s using techniques of auto-hypnosis or relaxation and, in accordance with the needs and therapeutic possibilities of the time, they focused on secondary outcomes, e.g. frequency of bleeding (physical health) and adherence to the intervention. The four newer trials assessed psycho-educational interventions all mediated by the use of technologies (DVD or computer) and often created according to age needs ...
Source: Cochrane Database of Systematic Reviews - Category: General Medicine Authors: Tags: Cochrane Database Syst Rev Source Type: research
We present the case of a young male with hemophilia B who was diagnosed with severe symptomatic mitral regurgitation and underwent successful robotic mitral valve repair. To our knowledge, this is the first report of a patient with hemophilia B who underwent robotic mitral valve repair .
Source: Journal of Cardiac Surgery - Category: Cardiovascular & Thoracic Surgery Authors: Tags: CASE REPORT Source Type: research
CONCLUSION:  This extension study demonstrated the long-term safety and efficacy of weekly rIX-FP in pediatric patients. Additionally, it showed that adequate bleed protection can be achieved with 10- or 14-day rIX-FP regimens in selected pediatric patients while maintaining safety. PMID: 32185782 [PubMed - as supplied by publisher]
Source: Thrombosis and Haemostasis - Category: Hematology Authors: Tags: Thromb Haemost Source Type: research
AbstractAn 88-year-old man with congenital hemophilia A developed end-stage renal disease due to microscopic polyangiitis. He was at risk for catheter-related infection because he was taking immunosuppressive agents for the treatment of polyangiitis. He was also unable to manipulate the peritoneal dialysis device. Therefore, hemodialysis using an arteriovenous fistula was induced for renal replacement therapy. Recombinant coagulation factor VIII (1000  IU) was administered via the venous chamber of the hemodialysis circuit 10 min before the end of each hemodialysis session, and nafamostat mesylate (25 mg/h) ...
Source: CEN Case Reports - Category: Urology & Nephrology Source Type: research
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