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Prophylactic recombinant factor VIIa administration to an infant with congenital systemic juvenile xanthogranuloma
Author(s) -
DE SANTIAGO JESUS,
MARTINEZGARCIA ERNESTO,
GIRON JORGE,
SALCEDO CONCEPCIÓN,
PÉREZGALLARDO ANTONIO
Publication year - 2006
Publication title -
pediatric anesthesia
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.704
H-Index - 82
eISSN - 1460-9592
pISSN - 1155-5645
DOI - 10.1111/j.1460-9592.2006.02009.x
Subject(s) - medicine , juvenile xanthogranuloma , coagulopathy , recombinant factor viia , refractory (planetary science) , surgery , consumptive coagulopathy , anesthesia , immunology , physics , histiocyte , astrobiology
Summary We report the case of an infant affected with congenital systemic juvenile xanthogranuloma scheduled for central venous access system implantation (Port‐a‐Cath TM ) and a liver and bone marrow biopsy. The patient had impaired liver function, thrombocytopenia, and coagulopathy which was refractory to daily fresh‐frozen plasma and platelet infusions: 80 μ g·kg −1 dose −1 of recombinant factor VIIa (rFVIIa) was administered i.v. every 2 h starting 30 min before the procedure and ending 6 h afterwards. Very minor bleeding was observed during the procedure. In conclusion, rFVIIa therapy was effective as prophylaxis for both invasive procedures in this patient with a coagulopathy which was refractory to other different therapies.