J Thromb Haemost ; 2: A rare cause of young myocardial infarct. Severe spontaneous arterial thrombotic manifestations in patients with inherited hypo- and afibrinogenemia. Privacy Policy Terms of Use. The time course of plasma fibrinogen concentrations is described in Fig. Fluid replacement therapy with crystalloids was started.

Needle aspiration of subperiosteal haematoma was done, collection up to 30 ml was drained and pressure dressing was done. Conclusions In conclusion, spontaneous hepatic hematoma can be observed in patients with afibrinogenemia and is a real challenge for clinicians. Cryoprecipitate and fresh frozen plasma are alternative treatments. The patients with inherited disorder of coagulation like congenital afibrinogenemia should be followed up by a comprehensive bleeding disorder care team. Purchase access Subscribe to the journal. In this article, we presented the case of a year old female with congenital afibrinogenemia who was admitted with a spontaneous intrahepatic hematoma. Am J Emerg Med ;

A year-old female patient with a history of congenital afibrinogenemia.

afibrinogenemia case study

Given the hemodynamic instability, surgical treatment was necessary using packing by applying the principles of damage control surgery. She had suffered from massive bleeding 7 years ago, diagnosed as hemoperitoneum complicating an ovarian cyst rupture.

Boy was shifted to postoperative ICU for further monitoring.

Surgeons, haematologist and anaesthesiologist should work like a unit to manage these types of surgical emergencies. Simultaneously, she presented an early ventilator acquired pneumonia methicillin sensitive Staphylococcus aureus.


[Congenital afibrinogenemia: about a case].

Active bleeding continued during the intervention and norepinephrine was introduced. Supportive treatment including transfusion and fibrinogen administration, associated with repeated packing surgeries and selective embolization, were successfully performed.

afibrinogenemia case study

Clinical features of sstudy bleeding, bleeding after minor trauma, or after surgery have been described as well as thrombo-embolic complications. How to cite this URL: No septic shock occurred. The proconvertin index is obtained during the same procedure by determining the percentage of thrombin formed during the first minute of activation before addition of proconvertin.

Management of spontaneous haemorrhage and rupture of hepatocellular adenomas. Thromb Haemost ; 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. Afibrinogenemia has an estimated prevalence of one for 1, We report the case of a year-old boy weighing 28 Kg, a diagnosed case of congenital afibrinogenemia who presented to hospital with swelling of mandible on right side.

Liver Int ; A high rate of consanguinity has been afibrinogenemla. Find articles by Yazine Mahjoub.

She was being treated monthly with fibrinogen concentrates, tranexamic acid during menstrual periodsand oral contraception. Sign in to make a comment Sign in to your personal account. Sign in to access your subscriptions Sign in to your personal account. The anaesthesiologist should work with surgeons and haematologists in unison to manage such patients who present for various surgical emergencies.


[Congenital afibrinogenemia: about a case].

Paradoxically, few patients with afibrinogenemia may also suffer from severe thromboembolic complications. Purchase access Subscribe to the journal. National Center for Biotechnology AfibrniogenemiaU. Individuals requiring plasma-derived coagulation factor concentrates should be immunised with the hepatitis A and hepatitis B vaccine.

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

Boy was immunised with hepatitis A and hepatitis B vaccine. The child gave history of easy bruisability, prolonged bleeding from pin prick sites that used to stop after applying sustained pressure for long time. Its estimated incidence is one to two cases per million births. Sitemap What’s New Feedback Disclaimer. On day 6, abdominal CT scan showed abundant pelvic peritoneal effusion and diffuse infiltration of the peritoneum, suggesting a postoperative peritonitis.

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. A rare cause of young myocardial infarct.

afibrinogenemia case study

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