Surgery in patients with congenital factor VII deficiency: A single center experience.
10.5045/kjh.2012.47.4.281
- Author:
Shin Hee KIM
1
;
Young Shil PARK
;
Kee Hwan KWON
;
Jae Hoon LEE
;
Kwang Chul KIM
;
Myung Chul YOO
Author Information
1. Department of Pediatrics, Kyung Hee University Hospital at Gangdong, Seoul, Korea. pysmd@khnmc.or.kr
- Publication Type:Original Article
- Keywords:
Congenital FVII deficiency;
Surgery;
rFVIIa;
Antifibrinolytic agent
- MeSH:
Antifibrinolytic Agents;
Factor VII;
Factor VIIa;
Hemorrhage;
Hemorrhagic Disorders;
Hemostasis;
Hospitalization;
Humans;
Korea;
Orthopedic Procedures;
Recombinant Proteins;
Tonsillectomy
- From:Korean Journal of Hematology
2012;47(4):281-285
- CountryRepublic of Korea
- Language:English
-
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(R) 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. CONCLUSION: 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.
