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Perioperative management of severe congenital protein C deficiency

Watanabe, Kentaroa; Kato, Motohiroa,b; Ishimaru, Tetsuyac; Hiwatari, Mitsuterua; Suzuki, Tomonorid; Minosaki, Yoshihirod; Takita, Junkoa; Fujishiro, Junc; Oka, Akiraa

Blood Coagulation & Fibrinolysis: December 2017 - Volume 28 - Issue 8 - p 646–649
doi: 10.1097/MBC.0000000000000654
Case Report

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.

aDepartment of Pediatrics, The University of Tokyo

bDepartment of Cell Therapy and Transplantation Medicine, The University of Tokyo Hospital

cDepartment of Pediatric Surgery, The University of Tokyo, Tokyo

dNICU, Kawaguchi Municipal Medical Center, Kawaguchi, Japan

Correspondence to Kentaro Watanabe, 7-3-1, Hongo, Bunkyo-ku, Tokyo, 113-8655, Japan Tel: +81 3 5800 8659; fax: +81 3 3816 4108; e-mail:

Received 8 February, 2017

Revised 6 June, 2017

Accepted 19 June, 2017

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