congenital afibrinogenemia


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Noun1.congenital afibrinogenemia - a rare congenital disorder of blood coagulation in which no fibrinogen is found in the blood plasma
afibrinogenemia - the absence of fibrinogen in the plasma leading to prolonged bleeding
References in periodicals archive ?
The patient who presented to the Pediatric Hematology Outpatient clinic because of unstoppable gingival bleeding for the first time in 2006 was diagnosed with congenital afibrinogenemia with findings including a prothrombin time (>120 s) and an activated partial thromboplastin time (>120 s) which were too long to be measured and a fibrinogen level which was too low to be measured.
Splenic rupture is observed more commonly in congenital afibrinogenemia compared to the other congenital bleeding disorders.
Therefore, splenectomy should be performed, although bleeding is controlled with medical treatment in patients with congenital afibrinogenemia with splenic rupture, since the risk of recurrence is high (8, 9).

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