title: [Haemophilia].
abstract: Haemophilia is an X-linked inherited clotting disorder with a prevalence of 1 per 5000 men. A deficiency of clotting factor VIII (FVIII; haemophilia A) or IX (FIX; haemophilia B) causes haemophilia patients to suffer from spontaneous bleeding and excessive blood-loss following surgery or trauma. Prophylactic administration of a factor VIII- or factor IX-concentrate is the standard treatment for children with severe haemophilia. Women who are carriers of the F8 or F9 gene mutation can have a lowered plasma concentration of factor VIII or IX, and thus suffer from a mild form of haemophilia. Drugs that have a negative influence on blood clotting, such as NSAIDs, can lead to life-threatening bleeding in haemophilia patients. One of the main complications of haemophilia treatment is the formation of inhibiting antibodies that inactivate FVIII or FIX. Haemophilia patients should be treated by a multidisciplinary team in a hospital with a haemophilia treatment centre.
authors.affiliation: Academisch Medisch Centrum, Emma Kinderziekenhuis, Amsterdam.
authors.forename: Janneke I
authors.initials: JI
authors.lastname: Loomans
authors.forename: Janske
authors.initials: J
authors.lastname: Lock
authors.forename: Marjolein
authors.initials: M
authors.lastname: Peters
authors.forename: Frank W G
authors.initials: FW
authors.lastname: Leebeek
authors.forename: Marjon H
authors.initials: MH
authors.lastname: Cnossen
authors.forename: Karin
authors.initials: K
authors.lastname: Fijnvandraat
mesh_terms: D001779:Blood Coagulation Factors; D005164:Factor IX / Q000235:genetics / Q000378:metabolism; D005169:Factor VIII / Q000235:genetics / Q000378:metabolism; D006467:Hemophilia A / Q000097:blood / Q000453:epidemiology / Q000235:genetics*; D002836:Hemophilia B / Q000097:blood / Q000453:epidemiology / Q000235:genetics*; D006487:Hemostasis; D006579:Heterozygote; D006801:Humans; D009154:Mutation
chemical_list: D001779:Blood Coagulation Factors; C025667:prothrombin complex concentrates; D005169:Factor VIII; D005164:Factor IX