Perioperative management of severe congenital protein C deficiency.

Abstract

: Perioperative care of congenital protein C deficiency has not been well established. Here, we describe a patient with congenital protein C deficiency who underwent laparoscopic fundoplication and gastrostomy at 2 years of age. Preoperatively, we stopped warfarin, administered fresh frozen plasma, and activated protein C. These procedures were performed without bleeding or clotting events, and at 3 days after the procedures, we restarted warfarin. Several episodes of abdominal hemorrhage and purpura fulminans occurred 2-4 weeks postoperatively, and the events were managed conservatively. We conclude that an invasive procedure can be performed in patients with protein C deficiency with appropriate supportive therapy, and postoperative observation for a sufficient length of time is essential to minimize the risk of complications.

• Authors: Kentaro Watanabe, Motohiro Kato, Tetsuya Ishimaru, Mitsuteru Hiwatari, Tomonori Suzuki, Yoshihiro Minosaki, Junko Takita, Jun Fujishiro, Akira Oka
• Journal: Blood coagulation & fibrinolysis : an international journal in haemostasis and thrombosis (Blood Coagul Fibrinolysis) Vol. 28 Issue 8 Pg. 646-649 (Dec 2017) ISSN: 1473-5733 [Electronic] England
• PMID: 28737523 (Publication Type: Case Reports, Journal Article)

Chemical References

• Protein C
• Warfarin

Topics

• Child, Preschool
• Fundoplication
• Humans
• Perioperative Care
• Plasma
• Protein C (administration & dosage)
• Protein C Deficiency (complications, congenital, therapy)
• Warfarin (therapeutic use)