“Summary. General guidelines exist for the use of recombinant activated factor VII (rFVIIa) to maintain haemostasis during surgery in congenital haemophilia A and B patients with high responding inhibitors
(CHwI). Individual surgical plans are required and based upon historical therapy response, adverse events and anticipated procedure. Surgical interventions are feasible, yet it remains unclear how many US hemophilia treatment centres (HTCs) perform procedures in this fragile population. To better understand the US HTC surgical experience in CHwI patients and the number/types of procedures performed, a 21-question survey was sent to 133 US HTCs, with follow-up for response clarification and to non-responders. 98/133 HTCs (74%) responded, with 87 currently treating CHwI patients. In the last decade, 76/85 HTCs performed 994 surgeries on CHwI patients. Sites were experienced selleck chemicals llc in the following procedures: central line insertion/removal (73 HTCs), dental (58), orthopaedic (52), abdominal (23), cardiovascular (14) and otolaryngologic (11). Experience with
orthopaedic surgeries included synovectomies – arthroscopic (23 HTCs), radioisotopic (22), and open (7); joint replacement (18); fracture repair (14); and arthrodesis (8). Treatment modalities included rFVIIa bolus (83 HTCs) or continuous infusions (9), plasma-derived activated prothrombin complex 上海皓元医药股份有限公司 concentrate (pd-aPCC) (55), antifibrinolytics (51), topical haemostatic agents (29), factor VIII (16) and fibrin sealants (14). Protocols for bypassing agents were used by 31/92 (33%) HTCs. SRT1720 molecular weight Most US HTCs surveyed care for CHwI patients (74%) and have experience in minor surgery; fewer HTCs reported complex orthopaedic surgical experience. Identification
of best practices and surgical barriers is required to guide future initiatives to support these patients. “
“This chapter contains sections titled: Introduction Inherited deficiencies of fibrinolytic system Congenital PAI-1 deficiency α2-Plasmin inhibitor (α2-PI) deficiency Inherited deficiencies of contact factor or kallikrein–kinin system Inherited platelet function disorders Bleeding disorders related to inherited vascular abnormalities Ehlers–Danlos syndrome vascular type (type IV) Hereditary hemorrhagic telangiectasia (Osler–Weber–Rendu syndrome) Conclusion Resources References “
“Summary. Menorrhagia, heavy menstrual bleeding, is a common condition that has a substantial impact on the lives of many women. The objective measurement of menorrhagia is often impractical; therefore diagnosis and treatment are usually based on the direct perception of the woman. Menstrual problems are likely to be worse in women with bleeding disorders, as they are more likely to have heavy and painful menstrual periods and ovulation bleeding and pain.