Congenital afibrinogenemia is a rare coagulopathy, exhibits autosomal recessive inheritance with a male to female ratio 1: A few hours later, she exhibits tachycardia, increased abdominal pain, and hypotension. She was being treated monthly with fibrinogen concentrates, tranexamic acid during menstrual periods , and oral contraception. Needle aspiration of subperiosteal haematoma was done, collection up to 30 ml was drained and pressure dressing was done. Semin Thromb Hemost ; Create a free personal account to download free article PDFs, sign up for alerts, and more.

Severe hemorrhages in patients with afibrinogenemia can be treated with fibrinogen concentrates, cryoprecipitate, and fresh frozen plasma, but fibrinogen concentrates are the main option. J Thromb Haemost ; 9: Antifibrinolytics are useful along with fibrinogen replacement for mucosal bleeding, particularly oronasopharynx to decrease the frequency of rebleeding. Create a free personal account to access your subscriptions, sign up for alerts, and more. Since there was no evidence of active bleed, no further cryoprecipitate was transfused. Boy was shifted to postoperative ICU for further monitoring.

[Congenital afibrinogenemia: about a case].

The diagnosis of hepatocellular carcinoma has been discarded on CT images. Afibrinogenemia has an estimated prevalence of one for 1, Needle aspiration of subperiosteal haematoma was done, collection up to 30 ml was drained and pressure dressing was done.

Coagulation factor activity and clinical bleeding severity in rare bleeding disorders: Her genetic fibrinogen mutation was allele 1: Published by Wolters Kluwer Health, Inc. Spontaneous splenic rupture in a patient with congenital afibrinogenemia.


Congenital afibrinogenemia: a case report of a spontaneous hepatic hematoma

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.

Abdominal computed tomography CT confirmed this suspicion of hematoma Fig. Published online Jul Get free access to newly published articles Create a personal account or sign in to: Clinical features of spontaneous bleeding, bleeding after minor trauma, or after surgery have been described as well as stufy complications. Antimicrobial therapy was switched to imipenem, vancomycin, and amikacin.

Finally, fibrinogen plays a vital role in the process of inflammation, which is primarily mediated by its interaction with leucocytes through the surface receptors. Am J Emerg Med ; Cryoprecipitate and fresh frozen plasma are alternative treatments.

Pathol Biol ; 8: Post operatively, repeat coagulation revealed INR 1. No septic shock occurred. In conclusion, spontaneous hepatic hematoma can be observed in patients with afibrinogenemia and is a real challenge for clinicians. This article has been cited by 1 Congenital afibrinogenemia: In view of patient’s deranged coagulation profile, it was planned to transfuse four units of cryoprecipitate overnight.

afibrinogenemia case study

Congenital afibrinogenemia presenting as antenatal intracranial bleed: The anaesthesiologist should work with surgeons and haematologists in unison afibriinogenemia manage such patients who present for various surgical emergencies. Case report A year-old female patient with a history of congenital afibrinogenemia. Anaesthesiologist, surgeons czse haematologist should work like a unit to manage the surgical emergencies. J Thromb Haemost ; 9: Vasc Health Risk Manag ; 5: In our patient, after transfusion of four units of cryoprecipitate, the bleeding parameters improved except for fibrinogen assay and INR being 1.


afibrinogenemia case study

Such as splenic rupture, it should be considered in patients with abdominal pain, acute abdomen, and hypotension or shock. Successive bleeding and thrombotic complications in agibrinogenemia patient with afibrinogenemia: Her laboratory tests showed: Open in a separate window. The local hospital where the young women was 1st admitted did not assesse fibrinogen activity but we can hypothesize that it was sfudy than 0.

Create a free personal account to download free article PDFs, sign up for alerts, customize your interests, and more. The authors have no funding and conflicts of interest to disclose.

afibrinogenemia case study

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