Author information Copyright and License information Disclaimer. Supportive treatment, damage control surgery with packing associated with selective embolization was successfully performed. Fluid replacement therapy with crystalloids was started. World J Gastroenterol ; 8: Sign in to make a comment Sign in to your personal account. The diagnosis of hepatocellular carcinoma has been discarded on CT images. Am J Hematol ;

Enhanced axial CT showing early leak of contrast material at the arterial phase due to arterial bleeding with increasing leak at the portal and later phases. Fluid replacement therapy with crystalloids was started. The patient was discharged to surgical ward on day Etiology and management of hemmorrhage in spontaneous liver rupture: Thromb Haemost ; She was being treated monthly with fibrinogen concentrates, tranexamic acid during menstrual periods , and oral contraception.

Laparotomy was performed with abundant cleaning of the peritoneal cavity and drainage plasmatic fibrinogen was 3. Case report A year-old female patient with a history of congenital afibrinogenemia.

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

Post operatively, repeat afibrinogneemia revealed INR 1. Management of spontaneous haemorrhage and rupture of hepatocellular adenomas. Fibrinogen and the risk of thrombosis. Cryoprecipitate and fresh frozen plasma are alternative treatments that should be used only when fibrinogen concentrate is not available.

A pharyngeal pack was put around the laryngeal inlet to prevent aspiration. Simultaneously, she presented an afibrlnogenemia ventilator acquired pneumonia methicillin sensitive Staphylococcus aureus.


Congenital afibrinogenemia: a case report of a spontaneous hepatic hematoma

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

Conclusions In conclusion, spontaneous hepatic hematoma can be observed in patients with afibrinogenemia and is a real challenge for clinicians. Proconvertin was also afibrinogenemua by the method. Sign in to download free article PDFs Sign in to access your subscriptions Sign in to your personal account.

afibrinogenemia case study

Boy was immunised with hepatitis A and hepatitis B vaccine. Peyvandi F, Mannucci PM. On day 2, the patient underwent further surgery to remove abdominal packing but subcapsular hepatic hematoma was still bleeding and another packing therapy was necessary.

[Congenital afibrinogenemia: about a case].

Abdominal computed tomography CT confirmed this suspicion of hematoma Fig. The reason being, fibrinogen concentrate in blood takes time of 18 to 36 hours to reach at its peak level after transfusion.

Each vial contains mg fibrinogen; mg human albumin, mg L-arginine, mg of sodium chloride and mg of sodium citrate. Arch Pediatr ; None, Conflict of Interest: Patients should avoid aspirin and other drugs that affect platelet afibrinogenwmia.

Unenhanced studg computed tomography CT scan and enhanced CT scan at arterial, portal, and later phases. In the postoperative period, no bleeding occurred, bowel transit was normal, and drainage tubes were removed quickly. The etiology of intrahepatic hemorrhage is usually secondary to trauma.


The management of the intrahepatic hemorrhage is based on surgical techniques.

A few hours later, she exhibits tachycardia, increased abdominal pain, and hypotension. In the operating room, anaesthetic induction with increasing concentration of sevoflurane in oxygen was done. The time course of plasma fibrinogen concentrations is described in Fig. Active bleeding continued during the intervention and norepinephrine was introduced.

The boy is a member of haemophiliac society, but the genetic counselling was not done for him.

afibrinogenemia case study

Treatment of congenital fibrinogen deficiency: Congenital afibrinogenemia is a rare coagulopathy, exhibits autosomal recessive inheritance with a male to female ratio 1: Recurrent myocardial infarction in a case of congenital Afibrinogenemia. Congenital afibrinogenemia presenting as antenatal intracranial bleed: Find articles by Yazine Mahjoub. Selective catheterization of the 2 branches of the right hepatic artery was successfully performed Fig.

Vasc Health Risk Manag ; 5:

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