- Congenital afibrinogenemia
Congenital afibrinogenemia Classification and external resources ICD-10 D65 ICD-9 286.3 OMIM 202400 DiseasesDB 307 MedlinePlus 001313 eMedicine ped/3042 MeSH D000347
Congenital afibrinogenemia is a rare inherited blood disorder in which the blood does not clot normally due to a lack of or a malfunction involving fibrinogen, a protein necessary for coagulation. 
Fibrinogen is also known as Factor I. Its lack is inherited in an autosomic recessive way. It can express itself with excessive bleeding since birth (bleeding from umbilical cord, easy bruising, bleeding after circumcision) 
When a problem of fibrinogen is suspected, the following tests can be ordered:
Fibrinogen level in blood (total and clottable)
Condition Prothrombin time Partial thromboplastin time Bleeding time Platelet count Vitamin K deficiency or warfarin prolonged prolonged unaffected unaffected Disseminated intravascular coagulation prolonged prolonged prolonged decreased von Willebrand disease unaffected prolonged prolonged unaffected Haemophilia unaffected prolonged unaffected unaffected Aspirin unaffected unaffected prolonged unaffected Thrombocytopenia unaffected unaffected prolonged decreased Early Liver failure prolonged unaffected unaffected unaffected End-stage Liver failure prolonged prolonged prolonged decreased Uremia unaffected unaffected prolonged unaffected Congenital afibrinogenemia prolonged prolonged prolonged unaffected Factor V deficiency prolonged prolonged unaffected unaffected Factor X deficiency as seen in amyloid purpura prolonged prolonged unaffected unaffected Glanzmann's thrombasthenia unaffected unaffected prolonged unaffected Bernard-Soulier syndrome unaffected unaffected prolonged unaffected 
- ^ Neerman-Arbez M, de Moerloose P (2007). "Mutations in the fibrinogen gene cluster accounting for congenital afibrinogenemia: an update and report of 10 novel mutations". Hum. Mutat. 28 (6): 540–53. doi:10.1002/humu.20483. PMID 17295221.
- ^ National Hemophilia foundation http://www.hemophilia.org/NHFWeb/MainPgs/MainNHF.aspx?menuid=184&contentid=44&rptname=bleeding
- ^ Brick Wendy,MD, Burgess Rusell,MD, Dysfibrogenemia http://emedicine.medscape.com/article/199723-diagnosis
- ^ Kaplan QBook - USMLE Step 1 - 5th edition - page 254
Pathology: hematology · hematologic diseases of RBCs and megakaryocytes / MEP (D50-69,74, 280-287) Red
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