AFIBRINOGENEMIA CASE STUDY

Create a free personal account to download free article PDFs, sign up for alerts, customize your interests, and more. Correlating clinical manifestations with factor levels in rare bleeding disorders: Cryoprecipitate and fresh frozen plasma are alternative treatments. She was admitted to the emergency department of a local hospital with acute abdominal pain and nausea. Abstract Congenital afibrinogenemia is a very rare autosomal recessive disorder, results from mutation that affects plasma fibrinogen concentration. Congenital afibrinogenemia is a very rare autosomal recessive disorder, results from mutation that affects plasma fibrinogen concentration. Boy was shifted to postoperative ICU for further monitoring.

Coagulation factor activity and clinical bleeding severity in rare bleeding disorders: Successive bleeding and thrombotic complications in a patient with afibrinogenemia: Severe coagulopathy and inflammation are involved. Such as splenic rupture, it should be considered in patients with abdominal pain, acute abdomen, and hypotension or shock. National Center for Biotechnology Information , U.

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afibrinogenemia case study

Please review our privacy policy. Such as splenic rupture, it should be considered in patients with abdominal pain, acute abdomen, and hypotension or shock. A few hours later, she exhibits tachycardia, increased abdominal pain, and hypotension. On day 6, abdominal CT scan showed abundant pelvic peritoneal effusion and diffuse infiltration of the peritoneum, suggesting a postoperative peritonitis.

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Register for email alerts with links to free full-text articles Access PDFs of free articles Manage your interests Save searches and receive search alerts. In the operating room, anaesthetic induction with increasing concentration of sevoflurane in oxygen was done.

The boy was born out of consanguineous marriage and elder sibling had similar bleeding problems with history of multiple blood transfusions several times. Perioperative coagulation management in a patient with afibrinogenemia undergoing liver transplantation.

[Congenital afibrinogenemia: about a case].

Taslimi R, Golshani K. Related articles Afibrinogenemia antiplatelet therapy myocardial infarction.

afibrinogenemia case study

The working diagnosis was subperiosteal haematoma. Coagulation factor activity and clinical bleeding severity in rare bleeding disorders: Boy was shifted to postoperative ICU for further monitoring. Conclusions In conclusion, spontaneous hepatic hematoma can be observed in patients with afibrinogenemia and is a real challenge for clinicians.

One case of perioperative management of liver transplantation after Budd Chiari syndrome in a patient with afibriniogenemia was also reported. None, Conflict of Interest: How to cite this article: Rabe F, Salomon E.

Congenital afibrinogenemia: a case report of a spontaneous hepatic hematoma

The boy was given a titrated dose of 1. Report of a Case. Congenital afibrinogenemia is a rare coagulopathy, exhibits autosomal recessive inheritance with a male to female ratio 1: Author information Copyright and License information Disclaimer.

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The etiology of intrahepatic hemorrhage is usually secondary to trauma. Afibrinogenemia has also been associated with poor wound healing, wound dehiscence, spontaneous abortion and rarely intracranial haemorrhage.

afibrinogenemia case study

Fibrinogen and the risk of thrombosis. Singapore Med J ; Simultaneously, she presented an early ventilator acquired pneumonia methicillin sensitive Staphylococcus aureus. Antifibrinolytics are useful along with fibrinogen replacement for mucosal bleeding, particularly oronasopharynx to decrease the frequency of rebleeding.

Congenital afibrinogenemia: a case report of a spontaneous hepatic hematoma

Footnotes Source of Support: Cryoprecipitate and fresh frozen plasma are alternative treatments that should be used only when fibrinogen concentrate is not available. Supportive treatment including transfusion and fibrinogen administration, associated with repeated packing surgeries and selective embolization, were successfully performed.

World J Gastroenterol ; 8: At recent hospitalisation, the boy presented with throbbing pain and progressive swelling of right side of mandible for the past six days.