CASE REPORT
Year : 2011 | Volume
: 55 | Issue : 6 | Page : 605--607
Anaesthetic management of a child with congenital afibrinogenemia - A rare inherited coagulation disorder
Sham Sunder Goyal, D Vimal Bhardwaj, UK Shenoy, Bhavya Reddy
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
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.
How to cite this article:
Goyal SS, Bhardwaj D V, Shenoy U K, Reddy B. Anaesthetic management of a child with congenital afibrinogenemia - A rare inherited coagulation disorder.Indian J Anaesth 2011;55:605-607
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How to cite this URL:
Goyal SS, Bhardwaj D V, Shenoy U K, Reddy B. Anaesthetic management of a child with congenital afibrinogenemia - A rare inherited coagulation disorder. Indian J Anaesth [serial online] 2011 [cited 2013 May 19 ];55:605-607
Available from: http://www.ijaweb.org/article.asp?issn=0019-5049;year=2011;volume=55;issue=6;spage=605;epage=607;aulast=Goyal;type=0 |
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