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Management of surgical procedures in children with severe FV deficiency: experience of 13 surgeries
Author(s) -
Mathias M.,
Tunstall O.,
Khair K.,
Liesner R.
Publication year - 2013
Publication title -
haemophilia
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.213
H-Index - 92
eISSN - 1365-2516
pISSN - 1351-8216
DOI - 10.1111/hae.12053
Subject(s) - medicine , fresh frozen plasma , pediatrics , surgical procedures , surgery , platelet
Summary Homozygous severe factor V ( FV ) deficiency has a prevalence of around one per million. Even in patients with FV levels of <0.01 IU mL −1 there appears to be a variation in bleeding phenotype in that there is a subgroup of affected individuals who present in later childhood and have a relatively mild bleeding phenotype, but there are children who present as neonates with intracerebral bleeding events and who have a much more severe bleeding phenotype. The only available current FV replacement is in the form of fresh frozen plasma ( FFP ) or solvent detergent FFP . We present here our experience with surgical haemostatic cover for 13 surgeries in three children with severe FV deficiency.