The aims of severe perioperative bleeding management are three-fold. First, preoperative identification by anamesis and laboratory testing of those patients for whom the perioperative bleeding risk may be increased. Second, implementation of strategies for correcting preoperative anaemia and stabilisation of the macro- and microcirculations in order to optimise the patient’s tolerance to bleeding. Third, targeted procoagulant interventions to reduce the amount of bleeding, morbidity, mortality and costs. The purpose of these guidelines is to provide an overview of current knowledge on the subject with an assessment of the quality of the evidence in order to allow anaesthetists throughout Europe to integrate this knowledge into daily patient care wherever possible. The Guidelines Committee of the European Society of Anaesthesiology (ESA) formed a task force with members of scientific subcommittees and individual expert members of the ESA. Electronic databases were searched without language restrictions from the year 2000 until 2012. These searches produced 20 664 abstracts. Relevant systematic reviews with meta-analyses, randomised controlled trials, cohort studies, case-control studies and cross-sectional surveys were selected. At the suggestion of the ESA Guideline Committee, the Scottish Intercollegiate Guidelines Network (SIGN) grading system was initially used to assess the level of evidence and to grade recommendations. During the process of guideline development, the official position of the ESA changed to favour the Grading of Recommendations Assessment, Development and Evaluation (GRADE) system. This report includes general recommendations as well as specific recommendations in various fields of surgical interventions. The final draft guideline was posted on the ESA website for four weeks and the link was sent to all ESA members. Comments were collated and the guidelines amended as appropriate. When the final draft was complete, the Guidelines Committee and ESA Board ratified the guidelines.

https://epub.ub.uni-muenchen.de/24023/1/oa_24023.pdf

Management of severe perioperative bleeding Guidelines from the European Society of Anaesthesiology

WFH Guidelines for the Management of Hemophilia, 3rd edition

The management of perioperative bleeding involves multiple assessments and strategies to ensure appropriate patient care. Initially, it is important to identify those patients with an increased risk of perioperative bleeding. Next, strategies should be employed to correct preoperative anaemia and to

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Management of severe perioperative bleeding: guidelines from the European Society of Anaesthesiology: First update 2016.

BackgroundThe prevention of bleeding with adequately sustained levels of clotting factor, after a single therapeutic intervention and without the need for further medical intervention, represents an important goal in the treatment of hemophilia. MethodsWe infused a single-stranded adeno-associated viral (AAV) vector consisting of a bioengineered capsid, liver-specific promoter and factor IX Padua (factor IX–R338L) transgene at a dose of 5×1011 vector genomes per kilogram of body weight in 10 men with hemophilia B who had factor IX coagulant activity of 2% or less of the normal value. Laboratory values, bleeding frequency, and consumption of factor IX concentrate were prospectively evaluated after vector infusion and were compared with baseline values. ResultsNo serious adverse events occurred during or after vector infusion. Vector-derived factor IX coagulant activity was sustained in all the participants, with a mean (±SD) steady-state factor IX coagulant activity of 33.7±18.5% (range, 14 to 81). On cumu...

https://www.nejm.org/doi/pdf/10.1056/NEJMoa1708538

Hemophilia B Gene Therapy with a High-Specific-Activity Factor IX Variant.

BackgroundPatients with hemophilia A rely on exogenous factor VIII to prevent bleeding in joints, soft tissue, and the central nervous system. Although successful gene transfer has been reported in patients with hemophilia B, the large size of the factor VIII coding region has precluded improved outcomes with gene therapy in patients with hemophilia A. MethodsWe infused a single intravenous dose of a codon-optimized adeno-associated virus serotype 5 (AAV5) vector encoding a B-domain–deleted human factor VIII (AAV5-hFVIII-SQ) in nine men with severe hemophilia A. Participants were enrolled sequentially into one of three dose cohorts (low dose [one participant], intermediate dose [one participant], and high dose [seven participants]) and were followed through 52 weeks. ResultsFactor VIII activity levels remained at 3 IU or less per deciliter in the recipients of the low or intermediate dose. In the high-dose cohort, the factor VIII activity level was more than 5 IU per deciliter between weeks 2 and 9 after ...

https://www.nejm.org/doi/pdf/10.1056/NEJMoa1708483

AAV5–Factor VIII Gene Transfer in Severe Hemophilia A

Hemophilia is a rare disorder that is complex to diagnose and to manage. These evidence-based guidelines offer practical recommendations on the diagnosis and general management of hemophilia, as well as the management of complications including musculoskeletal issues, inhibitors, and transfusion-transmitted infections. By compiling these guidelines, the World Federation of Hemophilia aims to assist healthcare providers seeking to initiate and/or maintain hemophilia care programs, encourage practice harmonization around the world and, where recommendations lack adequate evidence, stimulate appropriate studies.

/pdf/guidelines-for-the-management-of-hemophilia-1x6t2d999b.pdf

Guidelines for the management of hemophilia.

Bleeding into the joints is the most common manifestation of severe haemophiliacs. Although it may resolve spontaneously or with treatment, some patients find that one particular joint has recurrent bleeding; this is termed a target joint. Recurrent bleeding prevents the joint from regaining its range of motion, muscle strength and normal appearance. These changes become permanent, leading eventually to osteoarthritis. A target joint requires urgent and comprehensive treatment, especially in young patients, if permanent damage is to be prevented. Treatment with factor concentrate prophylaxis and physiotherapy can help to prevent new bleeds and allow the synovitis to resolve, but for persistent synovitis, synovectomy is recommended. The target ankle joint is a special challenge as it often develops in very young children when the articular cartilage is susceptible and compliance with conservative treatment is difficult.

The target joint.

1Physiotherapy Department, Health Sciences Centre Winnipeg – Shared Health, Winnipeg, MB, Canada; 2Children’s Hospital Research Institute of Manitoba, Winnipeg, MB, Canada; 3Department of Pediatrics and Child Health, Rady Faculty of Health Sciences, University of Manitoba, Winnipeg, MB, Canada; 4Data Sciences Platform, George & Fay Yee Centre for Healthcare Innovation, Winnipeg, MB, Canada; 5Department of Pediatric Orthopedic Surgery, University of Manitoba, Winnipeg, MB, Canada; 6Child Health Program, Health Sciences Centre Winnipeg – Shared Health, Winnipeg, MB, Canada

K. Mulder

Papers

Guidelines for the management of hemophilia.

The target joint.

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