title: Management of congenital quantitative fibrinogen disorders: a Delphi consensus.
abstract: BACKGROUND
  No evidence-based guidelines for the management of patients suffering from afibrinogenaemia and hypofibrinogenaemia are available.
  
  
  OBJECTIVE
  The aim of this study was to harmonize patient's care among invited haemophilia experts from Belgium, France and Switzerland. A Delphi-like methodology was used to reach a consensus on: prophylaxis, bleeding, surgery, pregnancy and thrombosis management.
  
  
  RESULTS
  The main final statements are as follows: (i) a secondary fibrinogen prophylaxis should be started after a first life-threatening bleeding in patients with afibrinogenaemia; (ii) during prophylaxis the target trough fibrinogen level should be 0.5 g L-1; (iii) if an adaptation of dosage is required, the frequency of infusions rather than the fibrinogen amount should be modified; (iv) afibrinogenaemic patients undergoing a surgery at high bleeding risk should receive fibrinogen concentrates regardless of the personal or family history of bleeding; (v) moderate hypofibrinogenaemic patients (i.e. ≥0.5 g L-1) without previous bleeding (despite haemostatic challenges) undergoing a surgery at low bleeding risk may not receive fibrinogen concentrates as prophylaxis; (vi) monitoring the trough fibrinogen levels should be performed at least once a month throughout the pregnancy and a foetal growth and placenta development close monitoring by ultrasound is recommended; (vii) fibrinogen replacement should be started concomitantly to the introduction of anticoagulation in afibrinogenaemic patients suffering from a venous thromboembolic event; and (viii) low-molecular-weight heparin is the anticoagulant of choice in case of venous thromboembolism.
  
  
  CONCLUSIONS
  The results of this initiative should help clinicians in the difficult management of patients with congenital fibrinogen disorders.
authors.affiliation: Division of Angiology and Haemostasis, University Hospitals and Faculty of Medicine, Geneva, Switzerland.
authors.forename: A
authors.initials: A
authors.lastname: Casini
authors.affiliation: Division of Angiology and Haemostasis, University Hospitals and Faculty of Medicine, Geneva, Switzerland.
authors.forename: P
authors.initials: P
authors.lastname: de Moerloose
mesh_terms: D000347:Afibrinogenemia / Q000150:complications*; D000013:Congenital Abnormalities / Q000276:immunology*; D019468:Disease Management; D005260:Female; D005340:Fibrinogen / Q000627:therapeutic use*; D006470:Hemorrhage / Q000188:drug therapy*; D006801:Humans; D011247:Pregnancy
chemical_list: D005340:Fibrinogen
keywords: afibrinogenaemia; bleeding; fibrinogen; hypofibrinogenaemia; pregnancy; thrombosis