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

Fingerprint

Inherited Blood Coagulation Disorders
Afibrinogenemia
Anesthetics
Hemorrhage
Fibrinogen
Disease Susceptibility
Hematoma
Drainage
Emergencies
Therapeutics
Mutation

All Science Journal Classification (ASJC) codes

  • Anesthesiology and Pain Medicine

Cite this

Goyal, Sham Sunder ; Vimal Bhardwaj, D. ; Shenoy, U. K. ; Reddy, Bhavya. / Anaesthetic management of a child with congenital afibrinogenemia - a rare inherited coagulation disorder. In: Indian Journal of Anaesthesia. 2011 ; Vol. 55, No. 6. pp. 605-607.
@article{4282576056904dd0b8222150fe849a67,
title = "Anaesthetic management of a child with congenital afibrinogenemia - a rare inherited coagulation disorder",
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.",
author = "Goyal, {Sham Sunder} and {Vimal Bhardwaj}, D. and Shenoy, {U. K.} and Bhavya Reddy",
year = "2011",
month = "12",
day = "1",
doi = "10.4103/0019-5049.90617",
language = "English",
volume = "55",
pages = "605--607",
journal = "Indian Journal of Anaesthesia",
issn = "0019-5049",
publisher = "Medknow Publications and Media Pvt. Ltd",
number = "6",

}

Anaesthetic management of a child with congenital afibrinogenemia - a rare inherited coagulation disorder. / Goyal, Sham Sunder; Vimal Bhardwaj, D.; Shenoy, U. K.; Reddy, Bhavya.

In: Indian Journal of Anaesthesia, Vol. 55, No. 6, 01.12.2011, p. 605-607.

Research output: Contribution to journalArticle

TY - JOUR

T1 - Anaesthetic management of a child with congenital afibrinogenemia - a rare inherited coagulation disorder

AU - Goyal, Sham Sunder

AU - Vimal Bhardwaj, D.

AU - Shenoy, U. K.

AU - Reddy, Bhavya

PY - 2011/12/1

Y1 - 2011/12/1

N2 - 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.

AB - 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.

UR - http://www.scopus.com/inward/record.url?scp=84855446776&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=84855446776&partnerID=8YFLogxK

U2 - 10.4103/0019-5049.90617

DO - 10.4103/0019-5049.90617

M3 - Article

AN - SCOPUS:84855446776

VL - 55

SP - 605

EP - 607

JO - Indian Journal of Anaesthesia

JF - Indian Journal of Anaesthesia

SN - 0019-5049

IS - 6

ER -