Cryoprecipitate has been used as a source of fibrinogen due to the non availability of fibrinogen concentrate in our set up; each concentrate of cryoprecipitate contains mg of fibrinogen. We describe the case of a year-old child diagnosed of congenital afibrinogenemia who presented to hospital with subperiosteal haematoma and was posted for incision and drainage. Three grams of fibrinogen concentrates and tranexamic acid were administered at the beginning of the 3rd surgery. The hypothesis of hepatic adenoma was initially preferred in the context of a young woman with estrogen—progestin contraception and no history of liver cirrhosis. Semin Thromb Hemost ; Each vial contains mg fibrinogen; mg human albumin, mg L-arginine, mg of sodium chloride and mg of sodium citrate.

Support Center Support Center. National Center for Biotechnology Information , U. Given the hemodynamic instability, surgical treatment was necessary using packing by applying the principles of damage control surgery. Mice expressing a mutant form of fibrinogen that cannot support fibrin formation exhibit compromised antimicrobial host defense. Inhibition of local fibrinolysis allows maintenance of the clot and decreases the frequency of rebleeding. Treatment of congenital fibrinogen deficiency: Register for email alerts with links to free full-text articles Access PDFs of free articles Manage your interests Save searches and receive search alerts.

afibrinogenemia case study

Since there was no evidence of active bleed, no further cryoprecipitate was transfused. Cryoprecipitate has been used as a source of fibrinogen due to the non availability of fibrinogen concentrate in our set up; each concentrate of cryoprecipitate contains mg of fibrinogen. The patient also sustained injury to left infraorbital and maxillary area two months back causing ecchymosis that needed hospitalisation and was treated with two units of fresh frozen plasma.


The child gave history of easy bruisability, prolonged bleeding from pin prick sites that used to stop after applying sustained pressure for long time. Find articles by Yazine Mahjoub. Fibrinogen and the risk of thrombosis.

afibrinogenemia case study

Replacement therapy is the mainstay of treatment of bleeding episodes in these patients and plasma derived fibrinogen concentrate is the agent of choice.

Supportive treatment, damage control surgery with packing associated with selective embolization was successfully performed.

afibrinogenemia case study

Sitemap What’s New Feedback Disclaimer. Perioperative coagulation management in a patient with afibrinogenemia undergoing liver transplantation.

[Congenital afibrinogenemia: about a case].

The boy is a member of haemophiliac society, but the genetic counselling was not done for him. Finally, fibrinogen plays a vital role in the process afibrinogenemiw inflammation, which is primarily mediated by its interaction with leucocytes through the surface receptors.

Published online Jul J Thromb Haemost ; 2: Privacy Policy Terms of Use. It is also a ligand for intercellular adhesion molecule-1 and is an important mediator of cell—cell interaction and adhesion.

Severe hemorrhages in patients with afibrinogenemia can be treated with fibrinogen concentrates, cryoprecipitate, and fresh frozen plasma, but fibrinogen afibrunogenemia are the main option.

Congenital afibrinogenemia: a case report of a spontaneous hepatic hematoma

Author information Article notes Copyright and License information Disclaimer. This is an open access article distributed under the Creative Commons Attribution License 4. Afibrinogenemia is a rare coagulation disorder. One case of perioperative management of liver transplantation after Budd Chiari syndrome in a patient afibrinogwnemia afibriniogenemia was also reported.

AFIBRINOGENEMIA: Report of a Case | JAMA Pediatrics | JAMA Network

The thrombin time is also prolonged but more sensitive than PT or aPTT for quantitative and qualitative defects in fibrinogen. A pharyngeal pack was put around the laryngeal inlet to prevent aspiration. Severe coagulopathy and inflammation are involved. Frontal views of hepatic artery angiography after surgical packing white arrow showing no arterial bleeding or tumoral blush but afubrinogenemia mass effect performed by intrahepatic hematoma black arrow and subcapsular hematoma.


Create a free personal account to download free article PDFs, sign up for alerts, customize your interests, and more. Recurrent myocardial infarction in a case of congenital Afibrinogenemia.

Pathol Biol ; 8: The most common clinical symptoms are mucocutaneous, soft-tissue, joint and genito-urinary spontaneous bleeding, traumatic, or surgical bleeding. Cryoprecipitate and fresh frozen plasma are alternative treatments that should be used only when fibrinogen concentrate is not available.

[Congenital afibrinogenemia: about a case].

afiibrinogenemia Congenital afibrinogenemia, cryoprecipitate, fibrinogen. Support Center Support Center. To the best of our acknowledge, this is the 1st case of spontaneous intrahepatic bleeding reported in patient with congenital afibrinogenemia whereas several cases of splenic ruptures were reported.

The patient was discharged to surgical ward on day How to cite this URL: The surgeon decided to implement abdominal packing as damage control therapy, to ensure safe conditions before transferring the patient.