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CASE REPORT
Year : 2011  |  Volume : 55  |  Issue : 6  |  Page : 605-607

Anaesthetic management of a child with congenital afibrinogenemia - A rare inherited coagulation disorder


Department of Anaesthesiology, Kasturba Medical College, Manipal, Karnataka, India

Correspondence Address:
Sham Sunder Goyal
Assistant Professor, Department of Anaesthesiology, KMC, Manipal 576 104, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0019-5049.90617

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Congenital afibrinogenemia is a very rare autosomal recessive disorder, results from mutation that affects plasma fibrinogen concentration. It is frequently associated with bleeding diathesis of varying severity. We describe the case of a 10-year-old child diagnosed of congenital afibrinogenemia who presented to hospital with subperiosteal haematoma and was posted for incision and drainage. Replacement therapy is the mainstay of treatment of bleeding episodes in this patient and plasma-derived fibrinogen concentrate is the agent of choice. Cryoprecipitate and fresh frozen plasma are alternative treatments. Appropriate amount of cryoprecipitate were transfused pre-operatively to the child. Individuals with congenital afibrinogenemia should be managed by a comprehensive bleeding disorder care team experienced in diagnosing and managing inherited bleeding disorders. Anaesthesiologist, surgeons and haematologist should work like a unit to manage the surgical emergencies.


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