title: Replacement therapy for invasive procedures in patients  with haemophilia: literature review, European survey and  recommendations.
abstract: Although most surgical and invasive procedures can be performed safely in patients with haemophilia, the optimal level and duration of replacement therapy required to prevent bleeding complications have not been established conclusively. For providing more insight into optimal therapy during invasive procedures, a literature review of surgical procedures in patients with haemophilia was conducted. Concomitantly, current practice was surveyed in 26 European Haemophilia Comprehensive Care Centres, representing 15 different countries. The review identified 110 original papers published between 1965 and 2007. Of these, only two studies were randomized controlled trials. Target levels and the duration of replacement therapy in the published studies were as follows. For major orthopaedic surgery: preoperative targets were 80-90%; postoperative targets showed a high degree of variation, with trough levels ranging from 20% to 80%, duration 10-14 days; for liver biopsy, 70-100%, 1-7 days; tonsillectomy: 90-100%, 5-11 days; indwelling venous access device insertion: 100%, 3-10 days; circumcision: 50-60%, 2-4 days; dental surgery: 30-50%, single treatment. With the exception of dental surgery, current practice in Europe, as assessed by the survey, was largely in agreement with published data. In conclusion, this study provides both a comprehensive review and a large survey of replacement therapy in patients with haemophilia undergoing invasive procedures; these data have informed the consensus practical treatment recommendations made in this paper. This study highlights the need for better-designed studies in order to better define minimal haemostatic levels of replacement therapy and optimal treatment duration.
authors.affiliation: Haemostasis - Thrombosis Unit, Division of haematology, Cliniques Universitaires Saint-Luc, Université Catholique de Louvain, Bruxelles, Belgium. cedric.hermans@uclouvain.be
authors.forename: C
authors.initials: C
authors.lastname: Hermans
authors.forename: C
authors.initials: C
authors.lastname: Altisent
authors.forename: A
authors.initials: A
authors.lastname: Batorova
authors.forename: H
authors.initials: H
authors.lastname: Chambost
authors.forename: P
authors.initials: P
authors.lastname: De Moerloose
authors.forename: A
authors.initials: A
authors.lastname: Karafoulidou
authors.forename: R
authors.initials: R
authors.lastname: Klamroth
authors.forename: M
authors.initials: M
authors.lastname: Richards
authors.forename: B
authors.initials: B
authors.lastname: White
authors.forename: G
authors.initials: G
authors.lastname: Dolan
mesh_terms: D020878:Device Removal / Q000379:methods*; D019538:Health Care Surveys; D006467:Hemophilia A / Q000150:complications / Q000601:surgery*; D002836:Hemophilia B / Q000150:complications / Q000601:surgery*; D006488:Hemostasis, Surgical / Q000379:methods*; D006490:Hemostatics / Q000008:administration & dosage; D006801:Humans; D019106:Postoperative Hemorrhage / Q000517:prevention & control / Q000601:surgery*; D017410:Practice Guidelines as Topic / Q000592:standards
chemical_list: D006490:Hemostatics