Successful living domino liver transplantation in a child with protein C deficiency

• DOI: 10.1111/petr.12446
• Publication Date: 2015-02-25T05:41:49Z
• Authors: Masatoshi Matsunami; Akira Ishiguro; Akinari Fukuda; Kengo Sasaki; Hajime Uchida; Takanobu Shigeta; Hiroyuki Kanazawa; Seisuke Sakamoto; Motoki Ohta; Hisaya Nakadate; Reiko Horikawa; Atsuko Nakazawa; Mika Ishige; Koichi Mizuta; Mureo Kasahara
• Keywords: Protein C deficiency; Alpha 1-antitrypsin deficiency; Purpura fulminans

Abstract PC is produced in the liver and inhibits blood coagulation by catalyzing active factors V VIII . deficiency causes abnormal clotting that difficult to regulate anticoagulative treatments. Four reports of treated with LTx have been published; however, no report DLT as a therapy for available. We describe case 23‐month‐old girl who received compound heterozygous deficiency. Her activity was below 5%. She developed intracranial lesion frequent refractory purpura fulminans. Both her parents had mutations genes were excluded living donors. Furthermore, she low priority on waiting list deceased‐donor transplantation. performed using from patient MSUD Activated concentrate safely supported perioperative period. After , maintained normal activities BCAA levels. This first successfully propose patients treatment option