AFIBRINOGENEMIA CASE STUDY

Proconvertin was also determined by the method. Create a free personal account to access your subscriptions, sign up for alerts, and more. Sign in to save your search Sign in to your personal account. Open in a separate window. Sign in to customize your interests Sign in to your personal account. Parents were counselled about the involved risks in the surgery and its outcome. Abdominal computed tomography CT confirmed this suspicion of hematoma Fig.

Thrombo-embolic prophylaxis by low molecular weight heparin was introduced on day 7, after prophylactic intermittent legs compression during the 1st week. Privacy Policy Terms of Use. 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. Successive bleeding and thrombotic complications in a patient with afibrinogenemia: Rabe F, Salomon E. The etiology of intrahepatic hemorrhage is usually secondary to trauma.

Successive bleeding and thrombotic complications in a patient with afibrinogenemia: Please review our privacy policy.

[Congenital afibrinogenemia: about a case].

There was no history of trauma. The thrombin time is also prolonged but more sensitive than PT or aPTT for quantitative and qualitative defects in fibrinogen. Find articles by Herve Dupont. Genetic counselling and family studies should be part of a complete evaluation.

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Congenital afibrinogenemia: a case report of a spontaneous hepatic hematoma

Recessively inherited coagulation disorders. No significant history of trauma or fever was noted. Related articles Afibrinogenemia antiplatelet therapy myocardial infarction. Such as splenic rupture, it should be considered in patients with abdominal pain, acute abdomen, and hypotension or shock.

No septic shock occurred. Three grams of fibrinogen concentrates and tranexamic acid were administered at the beginning of the 3rd surgery.

Congenital afibrinogenemia: a case report of a spontaneous hepatic hematoma

Create a free personal account to access your subscriptions, sign up caee alerts, and more. Replacement therapy is the mainstay of treatment of bleeding episodes in sthdy patients and plasma derived fibrinogen concentrate is the agent of choice.

The boy was given a titrated dose of 1. HPB Oxford ; Replacement therapy is the mainstay of treatment of bleeding episodes in this patient and plasma-derived fibrinogen concentrate is the agent of choice.

The removal of abdominal packing was possible on day 4, without any abdominal bleeding plasmatic fibrinogen 1.

afibrinogenemia case study

Perioperative coagulation management in a patient with afibrinogenemia undergoing liver transplantation. Liver Int ; Laparotomy was performed with abundant cleaning of the peritoneal cavity and drainage plasmatic fibrinogen was 3.

J Thromb Haemost ; 9: Purulent peritoneal effusion was infected with methicillin resistant Staphylococcus haemolyticus allowing de-scalation to vancomycin alone for 10 days.

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afibrinogdnemia Fibrinogen replacement therapy for congenital fibrinogen deficiency. Br J Haematol ; She had suffered from massive bleeding 7 years ago, diagnosed as hemoperitoneum complicating an ovarian cyst rupture. Aetio-pathogenesis and the management casr spontaneous liver bleeding in the West: Afibrinogenemia has an estimated prevalence of one for 1, 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.

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

Boy was shifted to postoperative ICU for further monitoring. Get free access to newly published articles. Sign in to customize your interests Sign in to your personal account.

afibrinogenemia case study

Author information Article notes Copyright and License information Disclaimer. In our patient, after transfusion of four units of cryoprecipitate, the bleeding parameters improved except for fibrinogen assay and INR being 1.

Abdominal computed tomography CT confirmed this suspicion of hematoma Fig.