Search results for "hemophilia"
showing 4 items of 94 documents
Post-synoviorthesis rehabilitation in haemophilia.
2001
The efficacy of radiosynoviorthesis in the management of chronic haemophilic synovitis has been proven. Like replacement therapy, however, this procedure has no effect on muscle strength or endurance, range of movement, coordination, or the patient's physical condition in general. All the factors just enumerated are essential for the recovery and maintenance of the haemophiliac's quality of life. That is why rehabilitation and physiotherapy are so important, since no medical therapy or surgical procedure are by themselves capable of preventing disabilities.
F9 missense mutations impairing factor IX activation are associated with pleiotropic plasma phenotypes.
2022
Background Circulating dysfunctional factor IX (FIX) might modulate distribution of infused FIX in hemophilia B (HB) patients. Recurrent substitutions at FIX activation sites (R191-R226, >300 patients) are associated with variable FIX activity and antigen (FIXag) levels. Objectives To investigate the (1) expression of a complete panel of missense mutations at FIX activation sites and (2) contribution of F9 genotypes on the FIX pharmacokinetics (PK). Methods We checked FIX activity and antigen and activity assays in plasma and after recombinant expression of FIX variants and performed an analysis of infused FIX PK parameters in patients (n = 30), mostly enrolled in the F9 Genotype and PK HB …
Ultrasound evaluation of joint damage and disease activity in adult patients with severe haemophilia A using the HEAD‐US system
2021
Introduction The Haemophilia Early Arthropathy Detection with Ultrasound (HEAD-US) system and scoring scale has proven to be an accurate and time-efficient imaging method for identifying joint damage in patients with haemophilia. Aim Observational, multicentre, cross-sectional study conducted in 8 centres in Spain that assessed the joint status of adult patients with severe haemophilia A (SHA) using HEAD-US. Methods Joint status of the elbow, knee and ankle was evaluated in adults with SHA receiving on-demand (OD) treatment, or primary (PP), secondary (SP), tertiary (TP) or intermittent (IP) prophylaxis. Results Of the 95 patients enrolled, 87 received prophylaxis (6.3% PP, 38.9% SP, 43.2% …
Dental treatment of patients with coagulation factor alterations: An update
2007
Hemostasia is a defense mechanism that protects vascular integrity, avoids blood loss, and maintains blood fluidity throughout the circulatory system. The biochemical processes leading to blood clot formation are complex, and alterations can appear at any point within the chain of events. While a range of alterations can affect the coagulation factors, some are more common than others in the general population, including congenital (hemophilia A and B, Von Willebrand’s disease) and acquired disorders (anticoagulant drugs). Such diseases require special consideration in the context of dental treatment, and therefore must be known to dental professionals. Interconsultation with the hematologi…