title: Surgery in patients with congenital factor VII deficiency: A single center experience.
abstract: BACKGROUND
  Congenital factor VII (FVII) deficiency is a rare hemorrhagic disorder that can cause excessive bleeding during and after surgery in affected patients. The recombinant form of activated factor VII (rFVIIa, NovoSeven® from Novo Nordisk, Bagsvaerd, Denmark), which was developed as a second-generation bypassing agent, has recently been used in the management of bleeding for patients with congenital FVII deficiency.
  
  
  METHODS
  We reviewed the results of 8 surgical procedures in 5 patients with congenital FVII deficiency at the Kyung Hee University Hospital, Gangdong, Seoul, Korea, between January 2008 and June 2010. We administrated rFVIIa preoperatively in six patients and postoperatively in five patients.
  
  
  RESULTS
  Between January 2008 and June 2010 at our center, 8 operations were performed successfully and no complications were observed in the 5 patients with congenital FVII deficiency. The median level of FVII activity was 2% (range, 0.6-7%). Four orthopedic procedures, 1 tonsillectomy, and 3 dental extractions were performed. The median duration of hospitalization was 8.5 days (range, 0-15 days). rFVIIa was administered at all procedures, except the dental extraction that was performed using only antifibrinolytic agents without any replacement. No bleeding or thrombogenic complications were observed in any case.
  
  
  CONCLUSIONS
  Patients with congenital FVII deficiency who require surgery can be treated efficiently and safely with rFVIIa or antifibrinolytic agents. rFVIIa was well tolerated and maintained effective hemostasis and showed good clinical outcome after the major surgery.
authors: Department of Pediatrics, Kyung Hee University Hospital at Gangdong, Seoul, Korea.
authors: Shin-Hee
authors: SH
authors: Kim
authors: Young Shil
authors: YS
authors: Park
authors: Kee-Hwan
authors: KH
authors: Kwon
authors: Jae Hoon
authors: JH
authors: Lee
authors: Kwang Chul
authors: KC
authors: Kim
authors: Myung Chul
authors: MC
authors: Yoo
keywords: Antifibrinolytic agent; Congenital FVII deficiency; Surgery; rFVIIa