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

Sham Sunder Goyal, D. Vimal Bhardwaj, U. K. Shenoy, Bhavya Reddy

Research output: Contribution to journalArticle

4 Citations (Scopus)

Abstract

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.

Original languageEnglish
Pages (from-to)605-607
Number of pages3
JournalIndian Journal of Anaesthesia
Volume55
Issue number6
DOIs
Publication statusPublished - 01-12-2011
Externally publishedYes

All Science Journal Classification (ASJC) codes

  • Anesthesiology and Pain Medicine

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