Appropriate amount of cryoprecipitate were transfused pre-operatively to the child. Published online Jul HPB Surg ; On day 6, abdominal CT scan showed abundant pelvic peritoneal effusion and diffuse infiltration of the peritoneum, suggesting a postoperative peritonitis. Rabe F, Salomon E. Cryoprecipitate and fresh frozen plasma are alternative treatments that should be used only when fibrinogen concentrate is not available.

Post operatively, repeat coagulation revealed INR 1. Replacement therapy is the mainstay of treatment of bleeding episodes in this patient and plasma-derived fibrinogen concentrate is the agent of choice. Unenhanced axial computed tomography CT scan and enhanced CT scan at arterial, portal, and later phases. Needle aspiration of subperiosteal haematoma was done, collection up to 30 ml was drained and pressure dressing was done. Frontal views of hepatic artery angiography after surgical packing white arrow showing no arterial bleeding or tumoral blush but a mass effect performed by intrahepatic hematoma black arrow and subcapsular hematoma. Recessively inherited coagulation disorders. A pharyngeal pack was put around the laryngeal inlet to prevent aspiration.

Since there was no evidence of active bleed, no further cryoprecipitate was transfused. We are presenting a case of year-old male patient of congenital afibrinogenemia who had two episodes myocardial infarction in a span of two years.

She had suffered from massive bleeding 7 years ago, diagnosed as hemoperitoneum complicating an ovarian cyst rupture. Genetic counselling and family studies should be part of a complete evaluation.


[Congenital afibrinogenemia: about a case].

Antimicrobial therapy was switched to imipenem, vancomycin, and amikacin. Nil Conflict of Interest: These patients may be asymptomatic in their early infancy but some of them may present late with variable clinical events, ranging from moderate to minimal bleeding to cataclysmic haemorrhage.

Individuals requiring plasma-derived coagulation factor czse should be immunised with the hepatitis A and hepatitis B vaccine. Liver Transpl ; The boy was closely followed-up for three days, postoperatively, in postoperative ICU and also in the ward. How to cite this article: Footnotes Source of Support: Pathol Biol ; 8: Finally, fibrinogen plays a vital role in the process of inflammation, which is primarily mediated by its interaction with leucocytes through the surface receptors.

Author information Article notes Copyright and License information Disclaimer. No significant history of trauma or fever was noted.

Create a personal account to register for email alerts with links to free full-text articles. Pregnancy-related thrombosis in a woman afibrinogenemiq congenital afibrinogenemia: Thromb Haemost ; In the preceding paper, Prichard 1 has reviewed the published cases, and in this communication we report an additional case.

Congenital afibrinogenemia: a case report of a spontaneous hepatic hematoma

Neuromuscular blockade was achieved by i. Am J Hematol afibribogenemia 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 case study

afibrinogenemja Privacy Policy Terms of Use. At recent hospitalisation, the boy presented with throbbing pain and progressive swelling of right side of mandible for the past six days.


afibrinogenemia case study

No thrombo-embolic adverse event occurred. The child gave history of easy bruisability, prolonged bleeding from pin prick sites that used to stop after applying sustained pressure for long time. At the end of afibrinogenemiaa surgery, pharyngeal pack was removed and any bleeding points inside the oral cavity were inspected, residual neuromuscular blockade was reversed with 1.

Proconvertin was also determined by the method. On clinical examination, her abdomen was tender but not tense, she was afebrile and hemodynamically stable.

Congenital afibrinogenemia: a case report of a spontaneous hepatic hematoma

Inherited abnormalities of fibrinogen. Semin Thromb Hemost ; Congenital afibrinogenemia was diagnosed at the time of birth due to prolonged bleeding from the umbilical cord and was kept in neonatal intensive afibdinogenemia unit NICU.

afibrinogenemia case study

Fibrinogen casr therapy for congenital fibrinogen deficiency. Anaesthesiologist, surgeons and haematologist should work like a unit to manage the surgical emergencies. Sign in to customize your interests Sign in to your personal account.