Pathol Biol ; 8: 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. Create a free personal account to download free article PDFs, sign up for alerts, and more. Footnotes Source of Support: Semin Thromb Hemost ; The management of the intrahepatic hemorrhage is based on surgical techniques. Sign in to download free article PDFs Sign in to access your subscriptions Sign in to your personal account.

Sitemap What’s New Feedback Disclaimer. Cryoprecipitate and fresh frozen plasma are alternative treatments that should be used only when fibrinogen concentrate is not available. A high rate of consanguinity has been reported. Liver Int ; Intra abdominal haemorrhage due to a ruptured corpus luteum cyst in a girl with congenital afibrinogenemia.

The removal of abdominal packing was possible on day 4, without any abdominal bleeding plasmatic fibrinogen 1. Author information Afibrinogenemai notes Copyright and License information Disclaimer.

Correlating clinical manifestations with factor levels in rare bleeding disorders: Given the hemodynamic instability, surgical treatment was necessary using packing by applying the principles of damage control surgery. Treatment of congenital fibrinogen deficiency: Spontaneous splenic rupture in a patient with congenital afibrinogenemia.

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


Finally, fibrinogen plays a vital role in the process of inflammation, which is primarily mediated by its interaction with leucocytes through the surface receptors.

[Congenital afibrinogenemia: about a case].

The patient was managed conservatively with antiplatelet therapy and thrombolytic therapy was not given due to high risk for bleeding. Create a personal account to register for email aibrinogenemia with links to free full-text articles.

The boy was posted for incision and drainage of haematoma by oromaxillofacial surgeons. Journal List Indian J Anaesth v. Please review our privacy policy.

afibrinogenemia case study

Author information Copyright and License information Disclaimer. World J Gastroenterol ; 8: Create a free personal account to access your subscriptions, sign up for alerts, and more. Congenital afibrinogenemia is a rare coagulopathy, exhibits autosomal recessive inheritance with a male to female ratio 1: Parents were counselled about the involved risks in the surgery and its outcome. A pharyngeal pack was put around the laryngeal inlet to prevent aspiration. Discussion 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.

afibrinogenemia case study

Individuals requiring plasma-derived coagulation factor concentrates should be immunised with the hepatitis A and hepatitis B vaccine. Fibrinogen replacement therapy for congenital fibrinogen deficiency. The patients with inherited disorder of coagulation like congenital afibrinogenemia should be followed up by a comprehensive bleeding disorder care team.


[Congenital afibrinogenemia: about a case].

Published online Jul Liver Transpl ; Inhibition of local fibrinolysis allows maintenance of the clot and decreases the frequency of rebleeding. The local hospital where the young women was 1st admitted did not assesse fibrinogen activity but we can hypothesize that it was lower than 0.

Frontal views of hepatic caee angiography after surgical packing white arrow showing no arterial bleeding or tumoral blush but a mass effect performed by intrahepatic hematoma black arrow and subcapsular hematoma. Etiology and management of hemmorrhage in spontaneous liver rupture: None, Conflict of Interest: Simultaneously, she presented an early ventilator acquired pneumonia methicillin sensitive Staphylococcus aureus.

Selective catheterization of the 2 branches of the right hepatic afibginogenemia was successfully performed Fig. Articles from Medicine are provided here courtesy of Wolters Kluwer Health.

Congenital afibrinogenemia: a case report of a spontaneous hepatic hematoma

Inherited abnormalities of fibrinogen. She was being treated monthly with fibrinogen concentrates, tranexamic acid during menstrual periodsand oral contraception. Her genetic fibrinogen mutation was allele 1: