title: Primary prophylaxis in haemophilia care: Guideline update 2016.
abstract: This paper reviews the current status on recommendations or guidelines for primary prophylaxis based on recent published papers from organizations or group of experts as well as some original key papers. A rather uniform view exists that prophylaxis should be initiated at an early age before or after no more than a single joint bleed and, if possible, preferably be continued for life. The dose and dose frequency of prophylaxis is dependent on the goal of treatment, bleeding phenotype, compliance, venous access and economic resources in the health care system and should be tailored individually based on clinical outcome and pharmacokinetics. For children, the effectiveness of prophylaxis is more dependent on maintaining minimum trough levels than in adults. Novel extended half-life products are being introduced, which should not affect the decision on when to start prophylaxis nor the initial dose, but which may be helpful for patients with difficult venous access and which may enable higher trough levels of factor VIII.
authors.affiliation: Van Creveld Kliniek, University Medical Center Utrecht, Utrecht, The Netherlands.
authors.forename: Kathelijn
authors.initials: K
authors.lastname: Fischer
authors.affiliation: Lund University, Dept. of Clinical Sciences Lund, Paediatrics, Lund, Sweden; Skåne University Hospital, Department of Paediatrics, Malmö Centre for Thrombosis and Haemostasis, Malmö, Sweden. Electronic address: Rolf.Ljung@med.lu.se.
authors.forename: Rolf
authors.initials: R
authors.lastname: Ljung
mesh_terms: D005164:Factor IX / Q000627:therapeutic use; D005169:Factor VIII / Q000627:therapeutic use; D006467:Hemophilia A / Q000517:prevention & control*; D002836:Hemophilia B / Q000517:prevention & control*; D006801:Humans; D017410:Practice Guidelines as Topic
chemical_list: D005169:Factor VIII; D005164:Factor IX
keywords: Factor IX; Factor VIII; Haemophilia A; Haemophilia B; Prophylaxis