title: Orthopaedic surgery in haemophilia patients with inhibitors as the last resort.
abstract: Haemophilia patients with inhibitors commonly present much more severe musculoskeletal problems than patients without inhibitors. With haematological advances using recombinant activated factor VII (rFVIIa) and activated prothrombin complex concentrate (aPCC), it is now possible to perform orthopaedic procedures in these patients with a high rate of success. In Spain, the healthcare system sets no financial limits for the treatment of haemophilia, and children with haemophilia today have a much better musculoskeletal status than 30 years ago. However, the children of 30 years ago are now adults and are afflicted by severe articular deformities that frequently require the intervention of an orthopaedic surgeon. Our view is that the well-known concepts of prophylaxis in patients without inhibitors should be followed and applied to patients with inhibitors. In fact, prophylaxis seems to be the only way to prevent the development of haemophilic arthropathy in patients with haemophilia. Until this goal can be achieved, major and minor orthopaedic procedures will be necessary in patients with inhibitors more frequently than in patients without inhibitors.
authors.affiliation: Department of Orthopaedic Surgery, La Paz Univerisity Hospital, Madrid, Spain. rmerchan@arrakis.es
authors.forename: E C
authors.initials: EC
authors.lastname: Rodriguez-Merchan
authors.forename: M
authors.initials: M
authors.lastname: Quintana
authors.forename: V
authors.initials: V
authors.lastname: Jimenez-Yuste
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000818:Animals; D019774:Blood Coagulation Factor Inhibitors / Q000097:blood; D001779:Blood Coagulation Factors / Q000008:administration & dosage*; D016063:Blood Loss, Surgical / Q000517:prevention & control; D002648:Child; D003029:Coagulants / Q000008:administration & dosage; D004195:Disease Models, Animal; D018450:Disease Progression; D004621:Embolization, Therapeutic; D005164:Factor IX / Q000235:genetics / Q000627:therapeutic use; D015942:Factor VIIa / Q000008:administration & dosage; D006395:Hemarthrosis / Q000150:complications* / Q000517:prevention & control; D006467:Hemophilia A / Q000000981:diagnostic imaging / Q000503:physiopathology / Q000628:therapy*; D006801:Humans; D007270:Injections, Intra-Articular; D051379:Mice; D018345:Mice, Knockout; D008875:Middle Aged; D019637:Orthopedic Procedures / Q000379:methods*; D011788:Quality of Life; D011859:Radiography; D019275:Radiopharmaceuticals / Q000008:administration & dosage; D016059:Range of Motion, Articular; D011994:Recombinant Proteins / Q000008:administration & dosage; D013585:Synovitis / Q000209:etiology / Q000601:surgery*; D013997:Time Factors; D055815:Young Adult
chemical_list: D019774:Blood Coagulation Factor Inhibitors; D001779:Blood Coagulation Factors; D003029:Coagulants; D019275:Radiopharmaceuticals; D011994:Recombinant Proteins; C025667:prothrombin complex concentrates; D005164:Factor IX; C103587:recombinant FVIIa; D015942:Factor VIIa