title: Rare coagulation disorders: fibrinogen, factor VII and factor XIII.
abstract: Rare coagulation disorders (RCDs) include the inherited deficiencies of fibrinogen, factor (F) II, FV, combined FV and VIII, FVII, FX, combined FVII and X, FXI, FXIII and combined congenital deficiency of vitamin K-dependent factors (VKCFDs). Despite their rarity, a deep comprehension of all these disorders is essential to really understand haemostasis. Indeed, even if they share some common features each RCD has some particularity which makes it unique. In this review, we focus on three disorders: fibrinogen, FVII and FXIII.
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
authors.affiliation: Hemophilia treatment Center, Montpellier, France.
authors.forename: J-F
authors.initials: JF
authors.lastname: Schved
authors.affiliation: Department of Hematology, Children's Hospital of Orange County, Orange, CA, USA.
authors.forename: D
authors.initials: D
authors.lastname: Nugent
mesh_terms: D000347:Afibrinogenemia / Q000175:diagnosis* / Q000188:drug therapy; D025861:Blood Coagulation Disorders, Inherited / Q000175:diagnosis* / Q000188:drug therapy; D005167:Factor VII / Q000627:therapeutic use; D005168:Factor VII Deficiency / Q000175:diagnosis* / Q000188:drug therapy; D005176:Factor XIII / Q000235:genetics / Q000627:therapeutic use; D005177:Factor XIII Deficiency / Q000175:diagnosis* / Q000188:drug therapy; D005340:Fibrinogen / Q000627:therapeutic use; D006801:Humans; D020125:Mutation, Missense; D012042:Registries
chemical_list: D005167:Factor VII; D005340:Fibrinogen; D005176:Factor XIII
keywords: bleeding; factor VII; factor XIII; fibrinogen