Sign in to save your search Sign in to your personal account. Pregnancy-related thrombosis in a woman with congenital afibrinogenemia: The etiology of intrahepatic hemorrhage is usually secondary to trauma. Author information Article notes Copyright and License information Disclaimer. The time course of plasma fibrinogen concentrations is described in Fig. Antimicrobial therapy was switched to imipenem, vancomycin, and amikacin.

Journal List Indian J Anaesth v. The most common clinical symptoms are mucocutaneous, soft-tissue, joint and genito-urinary spontaneous bleeding, traumatic, or surgical bleeding. Journal List Medicine Baltimore v. Case report A year-old female patient with a history of congenital afibrinogenemia. How to cite this URL:

Abstract Congenital afibrinogenemia is a very rare autosomal recessive disorder, results from mutation that affects plasma fibrinogen concentration. 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.

National Center for Biotechnology InformationU. Selective catheterization of the 2 branches of the right hepatic artery was successfully performed Fig. On day 6, abdominal CT scan showed abundant pelvic peritoneal effusion and diffuse infiltration of the peritoneum, suggesting a postoperative peritonitis.

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

Our website uses cookies to enhance your experience. Her laboratory tests showed: Unenhanced axial computed tomography CT scan and enhanced CT scan at arterial, portal, and later phases. A pharyngeal pack was put around the laryngeal inlet to prevent aspiration. HPB Surg ; Rare Bleeding Disorder Registry: Footnotes Source of Support: A high rate of syudy has been reported.


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Severe coagulopathy and inflammation are involved. In the postoperative period, no bleeding occurred, bowel transit was normal, and drainage tubes were removed quickly.

afibrinogenemia case study

The management of these patients is particularly challenging because they are not only at risk of thrombosis but also of bleeding. Afibrinogenemia is a caee coagulation disorder.

Congenital afibrinogenemia: a case report of a spontaneous hepatic hematoma

The thrombin time is also prolonged but more sensitive than PT or aPTT for quantitative and qualitative defects in fibrinogen. Genetic counselling and family studies should be part of a complete evaluation.

afibrinogenemia case study

The patients with inherited disorder of coagulation like congenital afibrinogenemia should be followed up by a comprehensive bleeding disorder care team. How to cite this article: Turk Pediatri Ars ; Sitemap What’s New Feedback Disclaimer.

The management of the intrahepatic hemorrhage is based on surgical techniques. The boy was posted for incision and drainage of haematoma by oromaxillofacial surgeons. Supportive treatment, damage afibeinogenemia surgery with packing associated with selective embolization was successfully performed. Afibrinogenemia has also been associated with afibrinogene,ia wound healing, wound dehiscence, spontaneous abortion and rarely intracranial haemorrhage.


Individuals with congenital afibrinogenemia should be managed by a comprehensive bleeding disorder care team experienced in diagnosing and managing inherited bleeding disorders.

Purchase access Subscribe to JN Learning for one year. Get free access to newly published articles Create a personal account or sign in to: Replacement therapy is the mainstay of treatment of bleeding episodes afibrinogwnemia these patients and plasma derived fibrinogen concentrate is the agent of choice.

Congenital afibrinogenemia: a case report of a spontaneous hepatic hematoma

Author information Article notes Copyright and License information Disclaimer. One case of perioperative management of liver transplantation after Budd Chiari syndrome in a patient with afibriniogenemia was also reported.

Laparotomy was performed with abundant cleaning of the peritoneal cavity and drainage plasmatic fibrinogen was 3. No significant vase of trauma or fever was noted.

afibrinogenemia case study

World J Gastroenterol ; 8:

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