Between 2000 and 2008, 11 major orthopaedic surgeries for 7 congenital haemophilia patients with inhibitors were performed by the first author as the primary doctor using recombinant activated factor VII (rFVIIa). Orthopaedic surgical treatments were performed for six surgeries for four high-responder haemophilia A patients, three surgeries for two high-responder haemophilia B patients and two surgeries for one low-responder haemophilia B patient. This low-responder patient is allergic to factor IX products, so he usually uses rFVIIa as a haemostatic agent. All of the surgeries were major, such as joint arthroplasty, arthroscopic synovectomy, and a combination of both, and excellent surgical results were achieved. Seven cases were controlled by bolus infusion of rFVIIa, and the other four cases were controlled by combined bolus and continuous infusion of rFVIIa. An anti-fibrolytic agent was used for all cases. There were no thrombogenic adverse effects, only two bleeding episodes. As for haemostatic control, nine surgeries were excellent, one was good and one was fair. This report is the largest clinical report on major orthopaedic surgeries at a single institute. We have concluded that the combination of bolus and continuous infusion of rFVIIa is safe and effective, and more convenient to administer than simple bolus infusion therapy to achieve haemostasis at peri-operative periods. In addition, our data also concurs with the data of several previous reports which showed that orthopaedic surgery for haemophilia patients with inhibitors by means of rFVIIa is safe and effective.
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http://dx.doi.org/10.1111/j.1365-2516.2009.02149.x | DOI Listing |
Adv Rehabil Sci Pract
January 2025
Vancouver Island Health Authority, Victoria, BC, Canada.
Spasticity affects up to 80% of individuals with cerebral palsy and can lead to pain and difficulties with performing activities of daily living. If left untreated, spasticity can progress to contracture and neuro-orthopedic deformities. Cryoneurolysis is an emerging and mini-invasive ultrasound-guided technique that causes secondary axonotmesis of peripheral nerves through the formation of an ice ball and may result in months to years of improved range of motion and reduced pain in patients with spasticity.
View Article and Find Full Text PDFTransl Pediatr
December 2024
Department of Hematology Center, National Key Clinical Discipline of Pediatric Hematology, National Key Discipline of Pediatrics (Capital Medical University), Key Laboratory of Major Diseases in Children, Ministry of Education, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China.
Background: The production of inhibitors is a serious complication that can arise during coagulation factor replacement therapy for hemophilia A (HA). The primary therapeutic strategy to eliminate inhibitors is immune tolerance induction (ITI), which is known to be an extremely challenging, prolonged, and costly treatment. With the widespread use of RNA sequencing (RNA-seq) to analyze differentially expressed genes (DEGs) across various treatment outcomes, there is potential for predicting ITI outcomes.
View Article and Find Full Text PDFArthroplast Today
December 2024
Rush University Medical Center, Chicago, IL, USA.
Avascular necrosis (AVN) of the femoral head accounts for up to 10% of all total hip arthroplasties performed annually. Typically associated with intravascular coagulation, AVN is extremely rare in patients with bleeding disorders such as hemophilia B. In this report, we describe the therapeutic management of a 46-year-old male with hemophilia B, presenting with chronic left hip pain and AVN of the femoral head.
View Article and Find Full Text PDFHemophilia A is a clotting disorder due to factor VIII deficiency, leading to prolonged bleeding. Acquired hemophilia A results from the immune system attacking factor VIII, typically occurring later in life. Factor V Leiden is a genetic mutation causing abnormal blood clot formation, primarily in veins.
View Article and Find Full Text PDFAnn Hematol
January 2025
Division of Hematology and Oncology, Department of Pediatrics, Faculty of Medicine Ramathibodi Hospital, Mahidol University, 270 Rama VI Road, Ratchathewi District, Bangkok, Thailand.
Bleeding assessment tools (BATs) are used by trained medical personnel for screening bleeding disorders on a one-to-one basis with patients; hence, they are time-consuming and limited in use for large-population screening. The aims of the study were to develop, validate, and demonstrate a Thai BAT mobile application (mBAT) for self-screening of bleeding disorders. mBAT was developed and validated using the paper-based Thai version of pediatric bleeding questionnaire (TPBQ).
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